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ANATOMI DAN FISIOLOGI

Anatomi ginjal 1) Makroskopis Ginjal terletak dibagian belakang abdomen atas, dibelakang peritonium, didepan dua kosta terakhir dan tiga otot-otot besar (transversus abdominis, kuadratus lumborum dan psoas mayor). Ginjal pada orang dewasa penjangnya sampai 13 cm, lebarnya 6 cm dan berat kedua ginjal kurang dari 1% berat seluruh tubuh atau ginjal beratnya antara 120-150 gram. Bentuknya seperti biji kacang, jumlahnya ada 2 buah yaitu kiri dan kanan, ginjal kiri lebih besar dari ginjal kanan dan pada umumnya ginjal laki-laki lebih panjang dari pada ginjal wanita. Ginjal dipertahankan dalam posisi tersebut oleh bantalan lemak yang tebal. Potongan longitudinal ginjal memperlihatkan dua daerah yang berbeda yaitu korteks dan medulla.

Medulla terbagi menjadi baji segitiga yang disebut piramid. Piramid-piramid tersebut dikelilingi oleh bagian korteks dan tersusun dari segmen-segmen tubulus dan duktus pengumpul nefron. Papila atau apeks dari tiap piramid membentuk duktus papilaris bellini yang terbentuk dari kesatuan bagian terminal dari banyak duktus pengumpul (Price,1995 : 773).

2) Mikroskopis Tiap tubulus ginjal dan glumerulusnya membentuk satu kesatuan (nefron). Nefron adalah unit fungsional ginjal. Dalam setiap ginjal terdapat sekitar satu juta nefron. Setiap nefron terdiri dari kapsula bowman, tumbai kapiler glomerulus, tubulus kontortus proksimal, lengkung henle dan tubulus kontortus distal, yang mengosongkan diri keduktus pengumpul. (Price, 1995) 3) Vaskularisasi ginjal Arteri renalis dicabangkan dari aorta abdominalis kira-kira setinggi vertebra lumbalis II. Vena renalis menyalurkan darah kedalam vena kavainferior yang terletak disebelah kanan garis tengah. Saat arteri renalis masuk kedalam hilus, arteri tersebut bercabang menjadi arteri interlobaris yang berjalan diantara piramid selanjutnya membentuk arteri arkuata kemudian membentuk arteriola interlobularis yang tersusun paralel dalam korteks. Arteri interlobularis ini kemudian membentuk arteriola aferen pada glomerulus (Price, 1995).

Glomeruli bersatu membentuk arteriola aferen yang kemudian bercabang membentuk sistem portal kapiler yang mengelilingi tubulus dan disebut kapiler peritubular. Darah yang mengalir melalui sistem portal ini akan dialirkan kedalam jalinan vena selanjutnya menuju vena interlobularis, vena arkuarta, vena interlobaris, dan vena renalis untuk akhirnya mencapai vena

cava inferior. Ginjal dilalui oleh sekitar 1200 ml darah permenit suatu volume yang sama dengan 20-25% curah jantung (5000 ml/menit) lebih dari 90% darah yang masuk keginjal berada pada korteks sedangkan sisanya dialirkan ke medulla. Sifat khusus aliran darah ginjal adalah otoregulasi aliran darah melalui ginjal arteiol afferen mempunyai kapasitas intrinsik yang dapat merubah resistensinya sebagai respon terhadap perubahan tekanan darah arteri dengan demikian mempertahankan aliran darah ginjal dan filtrasi glomerulus tetap konstan ( Price, 1995). 4) Persarafan pada ginjal Menurut Price (1995) “Ginjal mendapat persarafan dari nervus renalis (vasomotor), saraf ini berfungsi untuk mengatur jumlah darah yang masuk kedalam ginjal, saraf ini berjalan bersamaan

dengan pembuluh darah yang masuk ke ginjal”.

b. Fisiologi ginjal Menurut Syaifuddin (1995) “Fungsi ginjal yaitu mengeluarkan zat-zat toksik atau racun; mempertahankan keseimbangan cairan; mempertahankan keseimbangan kadar asam dan basa dari cairan tubuh; mempertahankan keseimbangan garam-garam dan zat-zat lain dalam tubuh; mengeluarkan sisa metabolisme hasil akhir sari protein ureum, kreatinin dan amoniak”. Tiga tahap pembentukan urine :

1) Filtrasi glomerular Pembentukan kemih dimulai dengan filtrasi plasma pada glomerulus, seperti kapiler tubuh lainnya, kapiler glumerulus secara relatif bersifat impermiabel terhadap protein plasma yang besar dan cukup permabel terhadap air dan larutan yang lebih kecil seperti elektrolit, asam amino, glukosa,

dan sisa nitrogen. Aliran darah ginjal (RBF = Renal Blood Flow) adalah sekitar 25% dari curah jantung atau sekitar 1200 ml/menit. Sekitar seperlima dari plasma atau sekitar 125 ml/menit dialirkan melalui glomerulus ke kapsula bowman. Ini dikenal dengan laju filtrasi glomerulus (GFR =

Glomerular Filtration Rate). Gerakan masuk ke kapsula bowman’s disebut filtrat. Tekanan filtrasi

berasal dari perbedaan tekanan yang terdapat antara kapiler glomerulus dan kapsula bowman’s, tekanan hidrostatik darah dalam kapiler glomerulus mempermudah filtrasi dan kekuatan ini

dilawan oleh tekanan hidrostatik filtrat dalam kapsula bowman’s serta tekanan osmotik koloid

darah. Filtrasi glomerulus tidak hanya dipengaruhi oleh tekanan-tekanan koloid diatas namun juga

oleh permeabilitas dinding kapiler. 2) Reabsorpsi Zat-zat yang difilltrasi ginjal dibagi dalam 3 bagian yaitu : non elektrolit, elektrolit dan air. Setelah filtrasi langkah kedua adalah reabsorpsi selektif zat-zat tersebut kembali lagi zat-zat yang sudah difiltrasi. 3) Sekresi Sekresi tubular melibatkan transfor aktif molekul-molekul dari aliran darah melalui tubulus kedalam filtrat. Banyak substansi yang disekresi tidak terjadi secara alamiah dalam tubuh (misalnya penisilin). Substansi yang secara alamiah terjadi dalam tubuh termasuk asam urat dan kalium serta ion-ion hidrogen. Pada tubulus distalis, transfor aktif natrium sistem carier yang juga telibat dalam sekresi hidrogen dan ion-ion kalium tubular. Dalam hubungan ini, tiap kali carier membawa natrium keluar dari

cairan tubular, cariernya bisa hidrogen atau ion kalium kedalam cairan tubular “perjalanannya

kembali” jadi, untuk setiap ion natrium yang diabsorpsi, hidrogen atau kalium harus disekresi dan sebaliknya. Pilihan kation yang akan disekresi tergantung pada konsentrasi cairan ekstratubular (CES) dari ion-ion ini (hidrogen dan kalium). Pengetahuan tentang pertukaran kation dalam tubulus distalis ini membantu kita memahami beberapa hubungan yang dimiliki elektrolit dengan lainnya. Sebagai contoh, kita dapat mengerti mengapa bloker aldosteron dapat menyebabkan hiperkalemia atau mengapa pada awalnya dapat terjadi penurunan kalium plasma ketika asidosis berat dikoreksi secara theurapeutik.

HORSESOE KIDNEY (GINJAL TAPAL KUDA)

  • A. Definisi

Ginjal tapal kuda merupakan jenis yang paling umum dari fusi anomali ginjal.

Ginjal tapal kuda adalah penyatuan kutub kutub ginjal (biasanya bagian bawah). Mereka saling berhubungan melalui istmus yang berupa parenkim ginjal atau berupa jaringan fibrous (band). Letak ginjal tapal kuda lebih rendah daripada posisi yang normal, dan istmus letaknya setinggi vertebra lumbal 4 5.

  • B. Etiologi

Dua teori tentang embrio dari ginjal tapal kuda telah diusulkan. Ajaran klasik

fusi mekanik berpendapat bahwa ginjal tapal kuda terbentuk selama organogenesis, ketika kutub inferior dari sentuhan ginjal awal, menggabungkan di garis tengah lebih rendah. Teori fusi mekanik berlaku untuk ginjal tapal kuda dengan isthmus berserat. Atau, studi lebih baru postulat bahwa fusi abnormal dari jaringan yang berhubungan dengan isthmus parenchymatous dari beberapa ginjal tapal kuda adalah hasil dari peristiwa teratogenik melibatkan migrasi abnormal sel-sel nephrogenic posterior, yang kemudian bersatu untuk membentuk isthmus. Kejadian teratogenik mungkin juga berhubungan dengan peningkatan insiden anomali kongenital terkait dan neoplasias tertentu, seperti tumor Wilms dan tumor karsinoid terkait dengan isthmus dari ginjal tapal kuda.

  • C. Epidemiologi

Ginjal tapal kuda merupakan anomali yang tidak jarang dijumpai.Di dalam autopsi didapati rata-rata 1 di dalam 600 800 kasus. Pada umumnya penggabungan terjadi pada pole bawah, akan tetapi pada + 10% kasus terjadi pada pole atas. Pada laki-laki lebih sering, terjadi dari pada wanita dengan perbandingan 2:1.

D.

Patofisiologi

Ginjal terbentuk dari metanephros pada minggu kelima dari kehidupan embryonal. Ginjal tapal kuda terjadi sebagai akibat penyatuan dari renal blastema (nephroblast = tunas ginjal) pada minggu ke-8 sampai ke-10 kehidupan embryo, biasanya pada pole bawahnya di dekat daerah bifurcatio aortae.

Dalam pertumbuhannya, ginjal bergerak menuju ke-cranial sambil berputar 90 derajat, tetapi apabila terjadi penyatuan pada pole bawahnya maka ginjal tersebut tidak akan mencapai tempatnya yang normal, terhalang pada isthmusnya oleh arteri messenterica superior. Karena kedua pole bawahnya bersatu, maka masing-masing ginjal tidak dapat melakukan rotasi 90 derajat, sehingga pelvis renalis yang seharusnya menghadap ke medial jadi menghadap ke depan dan letak ureter di depan isthmus. Juga letak kedua ginjal menjadi lebih berdekatan dan sumbu memanjangnya arahnya sejajar atau menguncup ke bawah.

Letak ginjal normal di dalam cavum abdominis pada posisi berdiri di antara vertebra lumbalis I dan vertebra lumbalis N dimana ginjal kanan biasanya lebih rendah dari kiri. Sumbu memanjang kedua ginjal membentuk sudut yang menguncup ke-cranial. Pembuluh darah arterial yang pergi ke ginjal berasal dari bagian bawah aorta abdominalis atau dari arteri ilaca communis, bahkan kadang-kadang terdapat arteri renalis yang multipel yang dapat mengakibatkan kesulitan dalam melaksanakan pembedahan.

Untuk menentukan horseshoe kidney secara radiologis, Gutierrez membuat dan mengukur besarnya sudut "pyelographic triangle" dari suatu foto Ro ginjal dengan cara menarik sebuah garis horizontal di antara kedua crista iliaca dan garis horizontal lainnya melalui discus intervertebra lumbalis II dan III. Dari titik potong garis pertama dengan columna vertebralis dan kedua titik potong garis kedua dengan calyc ginjal yang paling caudal dan medial ditarik garis sehingga terbentuk sudut yang membuka ke arah cranial. Pada gambaran ginjal normal besarnya sudut tersebut 90 derajat, sedangkan pada horseshoe kidney 20 derajat.

  • E. Manifestasi Klinis

Gejala-gejala klinis yang terjadi disebabkan oleh adanya tekanan pada ureter oleh bagian yang menghubungkan kedua ginjal (isthmus), yang mengakibatkan terjadinya obstruksi aliran kemih. Gejalanya bisa berupa haematuria dan kolik abdomen yang disebabkan hidronephrose, penyakit infeksi pada ginjal dan batu ginjal. Dilaporkan 2 kasus, seorang laki-laki bangsa Indonesia berusia 28 tahun dan seorang wanita bangsa Indonesia berusia 39 tahun, yang mempunyai horseshoe

kidney. Kedua penderita mengalami haematuria dan nyeri pinggang sehabis melakukan kegiatan fisik yang berat. Gambaran radiologis memberi kesan bahwa isthmus pada penderita laki-laki terdiri dari jaringan ikat (fibrous tissue), sedangkan pada penderita wanita jaringan ginjal (parenchymatous tissue). Walaupun demikian konfirmasi untuk ini sebaiknya dengan arteriografi.

Jika tidak menimbulkan komplikasi, anomali ini tidak menunjukkan gejala, dan secara tak sengaja hanya terdeteksi pada saat dilakukan pemeriksaan pencitraan saluran kemih untuk mencari anomali di tempat lain. Keluhan muncul jika disertai obstruksi pada uretropelvic junction atau refluk vesiko ureter (VUR) berupa nyeri atau timbulnya massa pada pinggang. Obstruksi dan VUR dapat menimbulkan infeksi dan batu saluran kemih. Pada PIV tampak ginjal menyatu pada bagian kaudal dengan sumbu mengarah dari kranio lateral ke kaudo medial. Kadang kadang dijumpai adanya dilatasi pelvikalises. Untuk mencari adanya VUR dapat dilakukan pemeriksaan refluks studi.

a)

Presentasi

Hampir sepertiga dari pasien dengan ginjal tapal kuda tetap asimtomatik, dan ginjal tapal kuda adalah temuan insidental selama pemeriksaan radiologi. Pada anak-anak, infeksi saluran kencing adalah gejala yang umum. Namun, gejala mungkin samar-samar. Daripada nyeri panggul, sakit perut, dan gejala gastrointestinal seperti mual, distensi perut dan kepenuhan bisa mendominasi. Ginjal tapal kuda kemungkinan terjadi bila trauma perut karena tidak dilindungi oleh tulang rusuk dan dapat dikompresi atau patah di kolom vertebral lumbar oleh pukulan perut.

Dalam seri otopsi, kelainan ini lebih banyak terjadi pada anak-anak karena anomali kongenital yang berkaitan dengan ginjal tapal kuda tidak kompatibel dengan kelangsungan hidup jangka panjang. Kelainan ini hidup bersama dalam sistem kardiovaskuler, gastrointestinal, dan tulang terjadi pada sampai 85% dari pasien. Ini termasuk cacat ventriculoseptal, hemivertebrae dengan scoliosis, myelomeningocele,

dan

colobomata

dari

b.

Dengan

keberadaan

obstruksi

UPJ,

symphysiotomy

(pembagian

isthmus)

pernah dianjurkan secara rutin setelah pyeloplasty untuk memperbaiki drainase. Namun, prosedur ini dikaitkan dengan peningkatan risiko perdarahan, fistula, dan infark ginjal. Juga, karena pembuluh darah abnormal, ginjal kembali ke posisi semula setelah pembagian isthmus. Karena itu, symphysiotomy jarang, jika pernah, ditunjukkan dalam hubungannya dengan pyeloplasty.

  • F. Pemeriksaan penunjang

    • 1. Studi Laboratorium

Setelah ginjal tapal kuda didiagnosis atau dicurigai, laboratorium lebih lanjut dan evaluasi pencitraan harus dilakukan untuk menilai status ginjal dan untuk mencari

penyebab yang dapat diobati patologi ginjal. Urine dengan kultur urin harus dilakukan. Kelainan sedimen urin harus dievaluasi sebagai indikasi klinis. Infeksi harus dirawat. Serum kimia dengan kreatinin dianjurkan untuk menentukan fungsi ginjal

  • 2. STUDI IMAGING

Pyelography intravena (IVP) dan CT scan (CT scan dari perut dan panggul, dengan

dan tanpa kontras intravena) adalah studi radiologis terbaik awal untuk menentukan fungsi anatomi ginjal.CT scan ini menunjukkan isthmus dari ginjal tapal kuda. CT scan atau ultrasonografi sangat membantu untuk keberadaan batu, massa, atau hidronefrosis.

G.

Tatalaksana

1) TERAPI MEDIS

Ginjal tapal kuda rentan terhadap penyakit ginjal medis. Evaluasi metabolik harus dilakukan karena penyebab metabolik untuk penyakit batu ginjal kurang umum pada pasien dengan ginjal tapal kuda dibandingkan pada populasi umum dengan penyakit batu ginjal. Bila kelainan metabolik diidentifikasi maka harus dirawat. evaluasi metabolik termasuk batu ginjal24-jam studi penilaian risiko dan serum, termasuk kalsium, asam urat, dan fosfor.

Terapi

Pengobatan bedah didasarkan pada proses penyakit dan indikasi operasi standar. Pasokan anomali vaskular pada ginjal harus disimpan di garis depan dalam pikiran dokter bedah saat merencanakan pendekatan bedah. Umumnya, irisan garis tengah perut menyediakan akses ke kedua sisi ginjal tapal kuda dan pembuluh darah.

  • H. HASIL DAN PROGNOSIS

Ginjal tapal kuda tidak menyulitkan kehamilan atau persalinan. Yang penting, perhatikan bahwa kehadiran ginjal tapal kuda saja tidak mempengaruhi kelangsungan hidup. Seperti disebutkan di atas, ginjal tapal kuda memang memiliki kecenderungan yang lebih tinggi untuk menjadi sakit. Oleh karena itu, kelangsungan hidup tergantung

pada proses penyakit yang ginjal tapal kuda mungkin terpengaruh pelabuhan atau mengembangkan.

DAFTAR PUSTAKA

pada proses penyakit yang ginjal tapal kuda mungkin terpengaruh pelabuhan atau mengembangkan. <a href=http://translate.googleusercontent.com/translate_c?hl=id&prev=/search% 3Fq%3Dhorseshoe%2Bkidney%26hl%3Did%26biw%3D1024%26bih%3D416 %26prmd%3Divns&rurl=translate.google.co.id&sl=en&u=http://emedicine.med scape.com/article/441510- treatment&usg=ALkJrhh7DJqJtGIKlSXuoS95MQampwkYhA - a1128 DAFTAR PUSTAKA 1. Guyton,C Arthur dan Hall, jhon E. anatomi dan fisiologis ginjal“buku ajar fisiologi kedokteran Edisi 11”. Jakarta: EGC, 1997. Halaman 324-326 2. Purnomo, Basuki B. 2003. Dasar – dasar Urologi . Edisi kedua. Malang : Sagung Seto. 125 – 126 3. www.medicastore.com  Horseshoe Kidney  Akses : 25 mei 2011 4. http://www.kalbe.co.id/files/cdk/files/cdk_028_masalah_penyakit_ginjal_dan saluran air_kemih_di_indonesia.pdf  akses : 25 mei 2011 5. http://www.urologyhealth.org/  Akses : 25 mei 2011 6. www.emedicine.com  urology  Akses : 25 mei 2011 Horseshoe kidney http://en.wikipedia.or g /wiki/Horseshoe_kidney - mw- hea dhttp://en.wikipedia.org/wiki/Horseshoe_kidney - p-search Horseshoe kidney , also known as ren arcuatus (in latin), renal fusion or super kidney, is a congenital disorder affecting about 1 in 500 people . In this disorder, the patient's kidneys fuse together to form a horseshoe - shape during development in the womb. The fused part is the isthmus of the horseshoe kidney. Fusion abnormalities of the kidney can be categorized into two groups: horseshoe kidney and crossed fused ectopia. The 'horseshoe kidney' is the most common renal fusion anomaly. Presentation " id="pdf-obj-5-53" src="pdf-obj-5-53.jpg">

Horseshoe kidney, also known as ren arcuatus (in latin), renal fusion or super kidney, is a congenital disorder affecting about 1 in 500 people. [1]

In this disorder, the patient's kidneys fuse together to form a horseshoe-shape during development in the womb. The fused part is the isthmus of the horseshoe kidney.

Fusion abnormalities of the kidney can be categorized into two groups: horseshoe kidney and crossed fused ectopia. The 'horseshoe kidney' is the most common renal fusion anomaly.

Presentation

Axial CT image of the abdomen showing a horseshoe kidney.

Axial CT image of the abdomen showing a horseshoe kidney.

In patients with this condition, the central portion of the kidney may be found just inferior to the inferior mesenteric artery because the normal embryologic ascent of the kidneys is arrested by its presence in people with central fusion of the kidneys. [2] Horseshoe kidney is often asymptomatic, though persons affected by this condition may experiencenausea, abdominal discomfort, kidney stones and urinary tract infections at greater frequency than those without renal fusion. There is currently no cure for renal fusion other than symptomatic treatment. People with a horseshoe kidney must drink lots of water (more than most people), to keep from having a kidney stone or other symptoms from happening. [citation needed]

[edit]Associated conditions

IVP showing Horseshoe kidney

IVP showing Horseshoe kidney

While most cases of horseshoe kidneys are asymptomatic and discovered upon autopsy, the condition may increase the risk for:

  • Kidney obstruction - abnormal placement of ureter may lead to obstruction and dilation of the kidney.

  • Kidney infections - associated with vesicoureteral reflux.

  • Kidney stones - deviant orientation of kidneys combined with slow urine flow and kidney obstruction may lead to kidney stones.

[edit]Notable persons with this condition

Both Robert Rowan and Mel Gibson are affected with this condition

Ginjal tapal kuda, juga dikenal sebagai ren arcuatus (di latin), fusi ginjal atau ginjal super, adalah kelainan bawaan yang mempengaruhi sekitar 1 dari 500 orang [1].

Dalam gangguan ini, ginjal pasien sekering bersama untuk membentuk bentuk tapal kuda selama perkembangan di dalam rahim. Bagian menyatu adalah tanah genting dari ginjal tapal kuda.

Fusion kelainan ginjal dapat dikategorikan menjadi dua kelompok: ginjal tapal kuda dan menyeberangi ectopia menyatu. The 'ginjal tapal kuda' adalah anomali fusi yang paling umum ginjal.

Presentasi

Axial CT gambar perut menunjukkan ginjal tapal kuda.

Pada pasien dengan kondisi ini, bagian tengah ginjal dapat ditemukan hanya kalah dengan arteri mesenterika inferior karena pendakian embryologic normal ginjal ditangkap oleh kehadirannya pada orang dengan fusi pusat ginjal [2] ginjal Horseshoe. Adalah sering tanpa gejala, meskipun orang-orang yang terpengaruh oleh kondisi ini mungkin experiencenausea, ketidaknyamanan perut, batu ginjal dan infeksi saluran kemih pada frekuensi yang lebih besar daripada mereka yang tidak fusi ginjal. Saat ini tidak ada obat untuk fusi ginjal selain pengobatan simtomatik. Orang dengan ginjal tapal kuda harus minum banyak air (lebih dari kebanyakan orang), untuk menjaga dari memiliki batu ginjal atau gejala lain terjadi. [Rujukan?]

[Sunting] Associated kondisi

IVP menunjukkan ginjal Horseshoe

Sementara sebagian besar kasus ginjal tapal kuda tidak menunjukkan gejala dan ditemukan setelah otopsi, kondisi dapat meningkatkan risiko untuk:

• Ginjal obstruksi - penempatan abnormal ureter dapat menyebabkan obstruksi dan pelebaran ginjal.

• Ginjal Infeksi - terkait dengan refluks vesicoureteral.

Batu ginjal • - orientasi menyimpang ginjal dikombinasikan dengan aliran urin lambat dan obstruksi ginjal dapat menyebabkan batu ginjal.

• Kanker ginjal - peningkatan risiko kanker ginjal, terutama tumor Wilms ', karsinoma sel transisional, dan laporan kasus sesekali tumor karsinoid [3] Meskipun peningkatan risiko, risiko secara keseluruhan masih relatif rendah ..

Prevalensi ginjal tapal kuda pada wanita dengan Sindrom Turner adalah sekitar 15%. [4]

Hal ini dapat dikaitkan dengan trisomi 18 [5].

Background

The horseshoe kidney is the most common type of renal fusion anomaly. It consists of two distinct functioning kidneys on each side of the midline, connected at the lower poles by an isthmus of functioning renal parenchyma or fibrous tissue that crosses the midline of the body.

Epidemiology

Frequency

Horseshoe kidney occurs in 1 per 400-800 live births. The true incidence probably lies somewhere between these two extremes. Horseshoe kidney is twice as common in males as in females. No genetic determinant is known, although it has been reported in identical twins and in siblings within the same family.

Etiology

Two theories regarding the embryogenesis of the horseshoe kidney have been proposed. The classic teaching of mechanical fusion holds that the horseshoe kidney is formed during organogenesis, when the inferior poles of these early kidneys touch, fusing in the lower midline. The theory of mechanical fusion is valid for horseshoe kidneys with a fibrous isthmus. Alternatively, more recent studies postulate that the abnormal fusion of tissue associated with the parenchymatous isthmus of some horseshoe kidneys is the result of a teratogenic event involving the abnormal migration of posterior nephrogenic cells, which then coalesce to form the isthmus. This teratogenic event may also be

responsible for the increased incidence of related congenital anomalies and of certain neoplasias, such as Wilms tumor and carcinoid tumor associated with the isthmus of the horseshoe kidney.

Pathophysiology

By itself, the horseshoe kidney does not produce symptoms. However, by virtue of its embryogenesis and anatomy, it is predisposed to a higher incidence of disease than the normal kidney. The variable blood supply, presence of the isthmus, high insertion point, and abnormal course of the ureters all contribute to these problems. Because of these embryogenic and anatomical factors, the rates of hydronephrosis, stone formation, infection, and certain cancers are higher, resulting in a diseased horseshoe kidney (see image below).

responsible for the increased incidence of related congenital anomalies and of certain neoplasias, such as <aWilms tumor and carcinoid tumor associated with the isthmus of the horseshoe kidney. Pathophysiology By itself, the horseshoe kidney does not produce symptoms. However, by virtue of its embryogenesis and anatomy, it is predisposed to a higher incidence of disease than the normal kidney. The variable blood supply, presence of the isthmus, high insertion point, and abnormal course of the ureters all contribute to these problems. Because of these embryogenic and anatomical factors, the rates of hydronephrosis , stone formation, infection, and certain cancers are higher, resulting in a diseased horseshoe kidney (see image below). Excretory urogram shows a horseshoe kidney with left hydronephrosis. The most common associated finding in horseshoe kidney is ureteropelvic junction (UPJ) obstruction , which occurs in up to 35% of patients. It causes most problems. Obstruction is due to the high insertion of the ureter into the renal pelvis. The crossing of the ureter over the isthmus may also contribute to obstruction. Nonobstructive dilatation must be distinguished from obstructive dilatation using diuresis radioisotope renal scans. The prevalence of stones in the horseshoe kidney ranges from 20-60%. Stone disease is thought to be due to the associated hydronephrosis or UPJ obstruction that causes urinary stasis, which hinders stone passage. Metabolic factors, as in the normal population, have also been suggested as contributing to stone formation in these patients. The orientation of the calyces also impairs drainage, resulting in stasis. These kidneys appear dilated or abnormal on most imaging studies, although the radionuclide scans are generally accepted as being diagnostic. Urinary stasis and stone disease also predispose the horseshoe kidney to infection, which occurs in 27-41% of patients. Ascending infection from vesicoureteral reflux is another cause of infection in the horseshoe kidney. Certain cancers are more common in the horseshoe kidney . T his is thought to be due to teratogenic factors present at birth and the susceptibility of the diseased horseshoe kidney to certain cancers. Renal cell carcinoma is the most common renal cancer in horseshoe kidney, accounting for 45% of tumors . T he incidence of renal cell cancer in the horseshoe kidney is no different from that of the normal kidney. Transitional cell cancer and sarcoma account for 20% and 7% of tumors, respectively. The relative risk of transitional cell carcinoma in the horseshoe kidney is increased 3- to 4-fold. This is thought to be due to chronic obstruction, stones, and/or infection in the affected kidneys. The incidence of both Wilms and carcinoid tumors is also higher in the horseshoe kidney. Examination of these tumors may provide an insight into the development and " id="pdf-obj-9-13" src="pdf-obj-9-13.jpg">

Excretory urogram shows a horseshoe kidney with left

hydronephrosis.

The most common associated finding in horseshoe kidney is ureteropelvic junction (UPJ) obstruction, which occurs in up to 35% of patients. It causes most problems. Obstruction is due to the high insertion of the ureter into the renal pelvis. The crossing of the ureter over the isthmus may also contribute to obstruction. Nonobstructive dilatation must be distinguished from obstructive dilatation using diuresis radioisotope renal scans. The prevalence of stones in the horseshoe kidney ranges from 20-60%. Stone disease is thought to be due to the associated hydronephrosis or UPJ obstruction that causes urinary stasis, which hinders stone passage. Metabolic factors, as in the normal population, have also been suggested as contributing to stone formation in these patients. The orientation of the calyces also impairs drainage, resulting in stasis. These kidneys appear dilated or abnormal on most imaging studies, although the radionuclide scans are generally accepted as being diagnostic. Urinary stasis and stone disease also predispose the horseshoe kidney to infection, which occurs in 27-41% of patients. Ascending infection from vesicoureteral reflux is another cause of infection in the horseshoe kidney. Certain cancers are more common in the horseshoe kidney. [1] This is thought to be due to teratogenic factors present at birth and the susceptibility of the diseased horseshoe kidney to certain cancers. Renal cell carcinoma is the most common renal cancer in horseshoe kidney, accounting for 45% of tumors. [2, 3] The incidence of renal cell cancer in the horseshoe kidney is no different from that of the normal kidney. Transitional cell cancer and sarcoma account for 20% and 7% of tumors, respectively. The relative risk of transitional cell carcinoma in the horseshoe kidney is increased 3- to 4-fold. This is thought to be due to chronic obstruction, stones, and/or infection in the affected kidneys. The incidence of both Wilms and carcinoid tumors is also higher in the horseshoe kidney. Examination of these tumors may provide an insight into the development and

embryogenesis of the horseshoe kidney and the predilection of these two tumors to form in the horseshoe kidney. Wilms tumor accounts for 28% of malignant lesions. The relative risk of Wilms tumor is increased 2-fold. Half of these arise from the isthmus. Renal carcinoids are rare, with only 32 reported cases. Of the 32 cases, 5 of these renal carcinoids arose in a horseshoe kidney. The relative risk of a carcinoid tumor in a patient with a horseshoe kidney is 62 times that found in the normal population. Of the 5 reported carcinoid tumors reported in patients with horseshoe kidneys, 3 have originated in or have involved the isthmus. The location of these tumors in the isthmus may be explained by the embryogenesis involving abnormal migration of posterior nephrogenic cells, leading to the formation of the isthmus. This is a teratogenic event, which may explain this increased incidence of tumor within the isthmus. This theory may also explain the greater incidence of Wilms tumor in the isthmus. When compared with carcinoid tumor arising in a normal kidney, those that arise in a horseshoe kidney follow a more benign course.

Presentation

Nearly one third of patients with a horseshoe kidney remain asymptomatic, and the horseshoe kidney is an incidental finding during radiological examination. Physical examination may reveal a midline lower-abdominal mass.

Symptoms, when present, are usually due to obstruction, stones, or infection. In children, urinary tract infection is the most common presenting symptom. Clinical signs of disease are similar to those that would be found in a normal kidney. However, symptoms may be vague. Instead of flank pain, abdominal pain, and gastrointestinal symptoms such as nausea, abdominal distension and fullness may predominate. The Rovsing sign, consisting of abdominal pain, nausea, and vomiting with hyperextension of the spine, is rare. The horseshoe kidney may be predisposed to blunt abdominal trauma because it is unprotected by the rib cage and may be compressed or fractured across the lumbar vertebral column by an abdominal blow. This can occur during a car crash when the victim is restrained by a seat belt; the kidney is compressed between the belt and the spine.

Associated genitourinary anomalies in horseshoe kidney are common and occur in as many as two thirds of patients. Vesicoureteral reflux is present in approximately half the patients. Ureteral duplication is present in 10%.Hypospadias or undescended testis is present in 4% of males. Bicornuate uterus or septate vagina is present in 7% of females.

Other anomalies occur in other organ systems. In autopsy series, these abnormalities are more prevalent in children because the congenital anomalies associated with horseshoe kidney are incompatible with long-term survival. Therefore, the incidence of other anomalies is greater in those who die at birth or early infancy than in those who reach adulthood. These coexisting abnormalities in the cardiovascular, gastrointestinal, and skeletal systems occur in up to 85% of patients. These include ventriculoseptal defects, hemivertebrae with scoliosis, myelomeningocele, and colobomata of the iris. Horseshoe kidney is associated with known genetic syndromes, including Turner syndrome, oral- cranial-digital syndrome, and trisomies 18 and E.

Autosomal-dominant polycystic kidney disease has also been found in the horseshoe kidney, with 20 such cases reported in the literature.

Relevant Anatomy

Horseshoe kidneys may be found at any location along the path of normal renal ascent from the pelvis to the mid abdomen. The kidneys may be lower than normal because the isthmus is tethered by the inferior mesenteric artery during renal ascent. The isthmus usually lies anterior to the great vessels, at the level of the third to fifth lumbar vertebra. Rarely, it is posterior to these vessels or runs between them.

The vascular supply is variable and originates from the aorta, the iliac arteries, and the inferior mesenteric artery. Bilateral single renal hilar arteries occur in 30% of cases, and various combinations of single and multiple renal hilar and isthmus vessels are seen in 70% of cases. The isthmus of the kidney may not have a separate blood supply or, in 65% of cases, is supplied by a single vessel from the aorta. The blood supply to the isthmus may arise from the common iliac or inferior mesenteric arteries.

The collecting system has a characteristic appearance on intravenous urography because of an incomplete inward rotation of the renal pelvis, which faces anterior. The axis of the collecting system is deviated inward at the lower poles because of the lower pole's connection with the isthmus. The ureter may have a high insertion point into the renal pelvis and may cross anteriorly over the isthmus as it descends to the bladder. Rarely does the collecting system cross the isthmus to the contralateral kidney.

Contraindications

In the presence of UPJ obstruction, symphysiotomy (division of the isthmus) was once recommended routinely after pyeloplasty to improve drainage. However, this procedure is associated with an increased risk of hemorrhage, fistula, and renal infarction. Also, because of their abnormal vasculature, the kidneys return to their original position after division of the isthmus. Because of this, symphysiotomy is rarely, if ever, indicated in conjunction with pyeloplasty.

The presence of obstruction or hydronephrosis precludes treatment of kidney stones using extracorporeal shockwave lithotripsy (ESWL)

Laboratory Studies

Once a horseshoe kidney is diagnosed or suspected, further laboratory and imaging

evaluation should be performed to assess the status of the kidneys and to look for treatable causes of renal pathology. Urinalysis with urine culture should be performed. Abnormalities of the urine sediment

should be evaluated as clinically indicated. Infection should be treated. Serum chemistry with creatinine is recommended to determine baseline renal function

Imaging Studies

Intravenous pyelography (IVP) protocol CT scanning (CT scanning of the abdomen and pelvis, with and without intravenous contrast) is the best initial radiologic study to determine anatomy and relative renal function. See CT scan below.

This CT scan demonstrates the isthmus of a horseshoe kidney. Note the uptake of contrast in

This CT scan demonstrates the isthmus of a horseshoe kidney. Note the uptake of contrast in the isthmus.

Abdominal and pelvic CT scanning or renal ultrasonography is helpful to screen for the presence of stones, masses, or hydronephrosis

Other Tests

Further studies are performed as indicated and tailored to the clinical situation. These include dedicated CT scanning of the kidneys with and without intravenous contrast, diuresis renal scanning to assess renal function and drainage, and voiding cystourethrography to rule out concomitant vesicoureteral reflux. The frequency of vesicoureteral reflux in association with horseshoe kidney warrants voiding cystourethrography in all pediatric patients.

Medical Therapy

The horseshoe kidney is susceptible to medical renal disease. These diseases, if present, are treated as indicated. A metabolic evaluation should be performed because metabolic causes for kidney stone disease are no less common in the patient with horseshoe kidney than in the general population with kidney stone disease. Any identified metabolic abnormality should be treated. Metabolic evaluation includes a 24- hour stone risk assessment and serum studies, including calcium, uric acid, and phosphorous

Surgical Therapy

Surgical treatment is based on the disease process and standard surgical indications. The anomalous vascular supply to the kidney should be kept at the forefront of the surgeon's mind when planning the surgical approach. Generally, the midline abdominal incision provides access to both sides of the horseshoe kidney and vessels.

Ureteropelvic junction obstruction

UPJ obstruction is usually treated with open pyeloureteroplasty or ureterocalicostomy. With the advent of and surgeon familiarization with laparoscopic techniques, the preferred approach has become laparoscopic dismembered pyeloplasty. More recently, robotic dismembered pyeloplasty using the da Vinci surgical system has been successfully performed. [5, 6] Endopyelotomy is an option but yields success rates that are inferior to those of open or laparoscopic techniques. [7]

In bilateral UPJ obstruction, the midline transperitoneal incision provides access to both sides of the horseshoe kidney and the vessels. Symphysiotomy (division of the isthmus) is controversial and was recommended routinely after pyeloplasty to improve drainage. However, it is associated with an increased risk of hemorrhage, fistula, and renal infarction. In addition, after division of the isthmus, the kidneys, because of their abnormal vasculature, return to their original position. Therefore, symphysiotomy is rarely, if ever, indicated in conjunction with pyeloplasty.

Kidney stones

Kidney stones can be treated with ESWL, endoscopy, or open surgery. If pyeloplasty is to be performed, stones can be removed concomitantly with a flexible nephroscope and stone basket. [8] Treatment decisions and indications are similar to those for a normal kidney. The presence of untreated obstruction or hydronephrosis precludes ESWL. Concomitant percutaneous nephrostolithotomy with endopyelotomy has been used successfully in the treatment of stones with obstruction. Alternatively, successful

retrograde therapy of the UPJ with an "Accusize" cutting-balloon dilator followed by ESWL has also been

retrograde therapy of the UPJ with an "Accusize" cutting-balloon dilator followed by ESWL has also been performed.

retrograde therapy of the UPJ with an "Accusize" cutting-balloon dilator followed by ESWL has also been

Renal tumors

Guided by the angiographic findings, interruption of the blood supply of the tissue to be resected is the first step. This prevents significant hemorrhage during dissection and tissue removal. In cancer surgery, the isthmus usually needs to be divided to gain access to the tumor and surrounding lymph nodes.

Abdominal aneurysmectomy

Problems may arise when operating on the great vessels, eg, with abdominal aortic aneurysm repair. Horseshoe kidney complicates aortic aneurysm surgery in 1 out of 200 cases. The most important aspect of abdominal aneurysm repair in association with horseshoe kidney is appropriate surgical management of the common renal artery anomalies. Survival in these patients is predicated on preservation of renal function. Renal artery continuity can be established via branch grafts or reimplantation into the aortic graft. The approach can be midline-abdominal or retroperitoneal through a low-left thoracoabdominal incision. Successful endoluminal transfemoral repair via stent placement has also been described.

Renal transplantation

Horseshoe kidneys can be used for transplantation. They can be transplanted into a single recipient en bloc or can be divided and transplanted into two individuals. Dividing the isthmus can increase the risk of urinary fistula.

The donor’s medical history must be obtained to preclude complications such as

hydronephrosis, renal calculi, and urinary tract infection. The decision to transplant a horseshoe kidney en bloc depends on renal isthmus morphology and vascular anatomy, as well as the medical and functional status of the kidney.

Preoperative Details

Because of the anomalous and variable vascular supply to the horseshoe kidney, authorities recommend preoperative arteriography to delineate the vascular anatomy in addition to the routine diagnostic procedures. Accessory and aberrant arteries to the parenchyma and the tumor are the rule

Intraoperative Details

Because of the anomalous vasculature, the blood supply to the kidney must be identified and preserved.

Outcome and Prognosis

The horseshoe kidney does not complicate pregnancy or delivery. Importantly, note that the presence of the horseshoe kidney alone does not affect survival. As mentioned above, the horseshoe kidney does have a higher propensity to become diseased. Therefore, survival depends on the disease process that the affected horseshoe kidney may harbor or develop.

Future and Controversies

Despite the increased incidence of Wilms tumor in children with horseshoe kidney and carcinoid tumor in adults with horseshoe kidney, no recommendations regarding periodic surveillance to detect occult malignancies have been made.

Performing periodic renal ultrasonography in children with horseshoe kidney seems prudent for early detection of Wilms tumor. Periodic renal ultrasonography in the adult may also be prudent, especially in patients with hydronephrosis. All patients with horseshoe kidneys and stones should undergo 24-hour urine tests for kidney stone prophylaxis. Otherwise, surveillance should be performed as indicated based on the clinical situation.

 

Overview

Horseshoe kidney is a condition in which the kidneys are fused together at the lower end or base. By fusing, they form a "U" shape, which gives it the name "horseshoe."

Horseshoe kidney occurs during fetal development, as the kidneys move into their normal position in

Turner syndrome a genetic disorder seen in girls that causes them to be shorter than others and to

the flank area (area around the side, just above the waist). Horseshoe kidney occurs in about one in 500 children.

Horseshoe kidney can occur alone or in combination with other disorders. The most common

o

disorders seen with horseshoe kidney include:

o

not mature sexually as they grow into adulthood. Sixty percent of girls with Turner syndrome have horseshoe kidneys. Trisomy 18 a serious chromosome abnormality involving defects in nearly all organ systems, including horseshoe kidney in 20 percent of children affected.

 
 

In-Depth

What complications are associated with horseshoe kidney?

One-third of people with horseshoe kidneys have at least one other complication involving the

cardiovascular system, the central nervous system or the genitourinary system (which is the reproductive organs and urinary system) such as the following:

kidney stones - crystals and proteins that form stones in the kidney that may lead to a urinary tract obstruction.

hydronephrosis - enlargement of the kidneys that is usually the result of a urinary tract obstruction.

Wilm's tumor - an embryonic (newly-formed) tumor of the kidneys that usually occurs during early

childhood. renal cancer or polycystic kidney disease

various cardiovascular, gastrointestinal conditions or skeletal problems

 

What are the symptoms of horseshoe kidney?

While each child may experience symptoms differently, the most common symptoms of horseshoe kidney include:

urinary tract infection - usually uncommon in children under 5 years and unlikely in boys at any age.

flank (around the side, just above the waist) pain

kidney stones - if the stones remain in the kidney, your child may have no symptoms. If the stones

o

pass through her urinary tract, she could experience the following symptoms:

o

restlessness

o

sweating

o

nausea and/or vomiting

abdominal mass

o

blood in urine

o

changes in urinary frequency

o

chills

o

fever

o

cloudy urine

hydronephrosis - occurs when there is a urinary tract obstruction and the kidney(s) become enlarged

o

and potentially damaged. Symptoms of hydronephrosis may include the following:

o

poor weight gain

o

decreased urination

o

urinary tract infection

 

About one-third of children with horseshoe kidney have no symptoms.

Tests

How is horseshoe kidney diagnosed?

If your child is not experiencing symptoms, she may not need diagnosis or treatment. If she is experiencing symptoms, her physician may order one or more of the following diagnostic tests:

 

renal ultrasound - This is an imaging technique that uses a computer and high-frequency sound

waves to create images of blood vessels, tissues and organs. Physicians are able to view internal organs as they function, and assess blood flow through various vessels. voiding cystourethrogram (VCUG) - This is a specific x-ray that examines the your child's urinary

tract. A catheter (hollow tube) is placed in her urethra (tube that drains urine from the bladder to the outside of the body) and her bladder is filled with a liquid dye. X-ray images are taken as her bladder fills and empties. The images show if there is any reverse flow of urine into the ureters and kidneys. intravenous pyelogram (IVP) - This is an imaging technique that uses an x-ray to see the structures of

the urinary tract. An intravenous contrast of dye is given so that the structures can be seen on film. This technique reveals the rate and path of your child's urine flow through the urinary tract. blood and urine tests - These determine how well your child's kidneys may be functioning.

 
 

Treatment & Care

There is no known cure for a horseshoe kidney, but if your child has complications, her symptoms will be treated. Treatment approaches may include:

antibiotics (to treat an underlying infection)

surgical intervention (for symptomatic kidney stones)

 

If your child has no symptoms, she may not need to be treated. If your child has hydronephrosis, your doctor may want to discuss several other non-surgical or surgical treatment options.

Ikhtisar

Ginjal tapal kuda adalah suatu kondisi di mana ginjal digabungkan bersama di ujung bawah atau dasar. Dengan sekering, mereka membentuk "U" bentuk, yang memberikan nama "tapal kuda."

• ginjal Horseshoe terjadi selama perkembangan janin, seperti ginjal pindah ke

posisi normal mereka di daerah panggul (daerah di sekitar sisi, tepat di atas pinggang).

• ginjal Horseshoe terjadi pada sekitar satu dari 500 anak.

• ginjal Horseshoe dapat terjadi sendiri atau dalam kombinasi dengan gangguan

lainnya. Penyakit yang paling sering terlihat dengan ginjal tapal kuda meliputi:

o Sindrom Turner kelainan genetik terlihat pada perempuan yang menyebabkan mereka untuk menjadi lebih pendek daripada yang lain dan tidak matang secara seksual saat mereka tumbuh menjadi dewasa. Enam puluh persen dari anak perempuan dengan sindrom Turner memiliki ginjal tapal kuda.

o Trisomi 18 kromosom yang serius yang melibatkan kelainan cacat pada sistem organ hampir semua, termasuk ginjal tapal kuda di 20 persen anak-anak yang terkena dampak.

In-Depth Apa komplikasi yang berhubungan dengan ginjal tapal kuda?

Satu-sepertiga orang dengan ginjal tapal kuda memiliki setidaknya satu komplikasi lain yang melibatkan sistem kardiovaskular, sistem saraf pusat atau sistem genitourinari (yang merupakan organ reproduksi dan sistem kemih) seperti berikut:

• batu ginjal - kristal dan protein yang membentuk batu di ginjal yang dapat menyebabkan obstruksi saluran kemih.

• hidronefrosis - pembesaran ginjal yang biasanya merupakan hasil dari obstruksi saluran kemih.

• tumor Wilm itu - sebuah embrio (yang baru terbentuk) tumor ginjal yang biasanya terjadi pada anak usia dini.

• ginjal kanker atau penyakit ginjal polikistik

• hidrosefali dan / atau spina bifida • berbagai kardiovaskular, gastrointestinal kondisi atau masalah tulang

Apa saja gejala ginjal tapal kuda?

Sementara setiap anak mungkin mengalami gejala yang berbeda, gejala yang paling umum dari ginjal tapal kuda meliputi:

• Infeksi saluran kemih - biasanya jarang terjadi pada anak di bawah 5 tahun dan tidak mungkin anak laki-laki pada usia berapa pun.

• batu ginjal - jika batu tetap di ginjal, anak Anda mungkin tidak memiliki gejala. Jika batu melewati saluran kemih, dia bisa mengalami gejala berikut:

o panggul (sekitar sisi, tepat di atas pinggang) sakit o gelisah

o berkeringat o mual dan / atau muntah o darah dalam urin o perubahan frekuensi kencing o menggigil o Demam o berawan urin

• hidronefrosis - terjadi ketika ada obstruksi saluran kemih dan ginjal (s) membesar dan berpotensi rusak. Gejala hidronefrosis mungkin termasuk yang berikut:

o perut massal o miskin berat badan o kencing berkurang o Infeksi saluran kemih Sekitar sepertiga dari anak-anak dengan ginjal tapal kuda tidak memiliki gejala.

Hubungi Kami Tes Bagaimana ginjal tapal kuda didiagnosa?

Jika anak Anda tidak mengalami gejala, ia mungkin tidak perlu diagnosis atau pengobatan. Jika dia mengalami gejala, dokter nya dapat memerintahkan salah satu atau lebih dari tes diagnostik berikut:

• USG ginjal - Ini adalah teknik pencitraan yang menggunakan komputer dan frekuensi tinggi gelombang suara untuk membuat gambar pembuluh darah, jaringan dan organ. Dokter dapat melihat organ-organ internal saat mereka berfungsi, dan menilai aliran darah melalui berbagai kapal.

• berkemih cystourethrogram (VCUG) - Ini adalah khusus x-ray yang meneliti saluran kemih pada anak Anda. Sebuah kateter (tabung hampa) ditempatkan dalam uretra nya (tabung yang mengalirkan urin dari kandung kemih ke luar tubuh) dan kandung kemih nya diisi dengan cairan pewarna. X-ray diambil sebagai kandung kemihnya mengisi dan mengosongkan. Gambar menunjukkan jika ada arus balik urin ke ureter dan ginjal.

• intravena pyelogram (IVP) - Ini adalah teknik pencitraan yang menggunakan x- ray untuk melihat struktur dari saluran kemih. Sebuah kontras intravena zat

pewarna yang diberikan sehingga struktur dapat dilihat pada film. Teknik ini mengungkapkan tingkat dan jalur aliran urin anak Anda melalui saluran kemih.

• tes darah dan urin - ini menentukan seberapa baik ginjal anak Anda mungkin akan berfungsi.

Pengobatan & Perawatan

Tidak ada obat dikenal untuk ginjal tapal kuda, tetapi jika anak Anda memiliki komplikasi, gejala akan diperlakukan. Pendekatan pengobatan mungkin termasuk:

• antibiotik (untuk mengobati infeksi yang mendasari) • bedah intervensi (untuk batu ginjal gejala)

Jika anak Anda tidak memiliki gejala, dia tidak mungkin perlu diobati.

Jika anak Anda memiliki hidronefrosis, dokter Anda mungkin ingin mendiskusikan beberapa pilihan lain pengobatan non-bedah atau bedah.

http://radiopaedia.org/articles/horseshoe_kidney

Horseshoe kidney

Dr Alexandra Stanislavsky and Dr Frank Gaillard et al.view revision history A horseshoe kidney is the most common type of renal fusion anomaly (see developmental renal anomalies), and not only alters the imaging appearance, but also renders the kidneys susceptible to trauma, stone formation and transitional cell carcinoma of the renal pelvis.

Epidemiology

Horseshoe kidneys are found in approximately 1 in 400-500 adults and are more frequently encountered in males ( M:F 2:1 ) 1-3 . The vast majority of cases are sporadic, except for those associated with genetic syndromes (see below) 3 .

Clinical presentation

Horseshoe kidneys are, in themselves, asymptomatic and thus they are usually identified incidentally. They are however prone to a number of complications as a result of poor drainage, which may lead to clinical presentation. These complications include:

infection and pyeloureteritis cystica

increased incidence of malignancy

o

o

increased susceptibility to trauma

Associations

Horseshoe kidneys are frequently associated with both genitourinary and non-genitourinary malformations, and are also seen as part of a number of syndromes 3 :

chromosomal / aneupliodic anomalies

o

o

o

o

Turner syndrome - up to 7% have a horseshoe kidney

o

o

o

Edward syndrome (trisomy 18) - up to 20% have a horseshoe kidney

Patau syndrome (trisomy 13)

non-aneupliodic anomalies

o

o

o

Pathology

Embryology

A horseshoe kidney is formed by fusion across the midline of two distinct functioning kidneys, one on each side of the midline. They are connected by an isthmus of functioning renal parenchyma or fibrous tissue. In the vast majority of cases the fusion is between the lower poles (90%). In the remainder the superior or both the superior or inferior poles are fused. This latter configuration is referred to as a sigmoid kidney 3 .

The normal ascent of the kidneys is impaired by they inferior mesenteric artery (IMA) which hooks over the isthmus.

As a result of this fusion the inferior pole of each kidney point medially (the reverse of the normal renal axis). The ureters leave the kidneys and pass anterior to the isthmus, which is typically located immediately below the inferior mesenteric artery.

Also due to the halted ascent, renal vascular anomalies are common, and the renal arteries do not arise from the normal level.

Radiographic features

Ultrasound

Unless aware of the typical appearances of a horseshoe kidney, the abnormally rotated and inferiorly located kidney results in poor visualisation of the inferior pole and underestimation of the length. This is especially the case if the patient is scanned prone, and is an additional argument for scanning patients supine with left and right decubitus positions 2 .

Alternatively the renal tissue located anterior the aorta may be mistaken for retroperitoneal tissue, such as may be seen in lymphoma or metastatic nodal enlargement 2 .

CT and MRI

Both CT and MRI demonstrate renal tissue of normal imaging appearance, but with abnormal configuration. Enhancement is normal, and excretory phase imaging may be used to assess the collecting system.

Treatment and prognosis

Horseshoe kidneys in themselves do not require any treatment, and patients have normal life expectancy. It is however important to recognise their presence prior to abdominal surgery or renal intervention for one of their many complications (see above).

Differential diagnosis

When visualised with cross-sectional imaging (CT or MRI) there is essentially no differential. On ultrasound care must be taken to not mistaken a horseshoe kidney for a midline retroperitoneal mass, or to underestimate the length of the kidney.

Other entities to be aware of, from purely and nomenclature point of view include :

Tapal kuda ginjal Dr Alexandra Stanislavsky dan Dr Frank Gaillard et sejarah al.view revisi

Sebuah ginjal tapal kuda adalah jenis yang paling umum dari anomali fusi ginjal (lihat anomali perkembangan ginjal), dan tidak hanya mengubah penampilan pencitraan, tetapi juga membuat ginjal rentan terhadap trauma, pembentukan batu dan karsinoma sel transisional dari pelvis ginjal.

Epidemiologi

Ginjal tapal kuda yang ditemukan pada sekitar 1 pada orang dewasa 400-500 dan lebih sering ditemui pada laki-laki (M: F 2:1) 1-3. Sebagian besar kasus yang sporadis, kecuali yang terkait dengan sindrom genetik (lihat di bawah) 3.

Klinis Presentasi

Ginjal tapal kuda adalah, dalam diri mereka sendiri, tanpa gejala dan dengan demikian mereka biasanya diidentifikasi kebetulan. Namun mereka rentan

terhadap sejumlah komplikasi akibat drainase yang buruk, yang dapat menyebabkan presentasi klinis. Komplikasi ini meliputi:

• hidronefrosis, sekunder untuk obstruksi persimpangan pelviureteric • infeksi dan pyeloureteritis cystica • ginjal kalkuli • peningkatan kejadian keganasan o Wilms tumor o karsinoma sel transisional (TCC)

• peningkatan kerentanan terhadap trauma

Asosiasi

Ginjal tapal kuda ini sering berhubungan dengan malformasi baik genitourinari dan non-genitourinari, dan juga dipandang sebagai bagian dari sejumlah sindrom 3:

• kromosom / aneupliodic anomali

o sindrom Down o Sindrom Turner - hingga 7% memiliki ginjal tapal kuda

o Edward syndrome (trisomi 18) - sampai dengan 20% memiliki ginjal tapal kuda

o Sindrom Patau (trisomi 13) • non-aneupliodic anomali o Ellis-van Creveld sindrom 2 o Fanconi anemia 1 o Goltz sindrom o Kabuki sindrom o Pallister-Hall sindrom o VACTERL asosiasi Patologi Embriologi

Sebuah ginjal tapal kuda dibentuk oleh fusi di garis tengah dua ginjal berfungsi yang berbeda, satu di setiap sisi garis tengah. Mereka dihubungkan oleh sebuah tanah genting dari fungsi ginjal parenkim atau jaringan fibrosa. Dalam sebagian besar kasus fusi antara kutub lebih rendah (90%). Dalam sisanya kutub superior

atau keduanya yang superior atau inferior menyatu. Konfigurasi terakhir ini disebut sebagai 3 ginjal sigmoid.

Pendakian normal ginjal terganggu oleh mereka arteri mesenterika inferior (IMA) yang kait atas tanah genting.

Sebagai hasil dari fusi kutub inferior setiap titik ginjal medial (kebalikan dari sumbu ginjal normal). Ureter meninggalkan ginjal dan lulus anterior ke tanah genting, yang biasanya terletak langsung di bawah arteri mesenterika inferior.

Juga karena pendakian dihentikan, anomali vaskular ginjal yang umum, dan arteri ginjal tidak muncul dari tingkat normal.

Radiografi fitur

Ultrasound

Kecuali menyadari penampilan khas dari ginjal tapal kuda, secara tidak normal dan hasil diputar ginjal inferior terletak di visualisasi miskin tiang rendah dan meremehkan panjang. Hal ini terutama terjadi jika pasien dipindai rawan, dan merupakan argumen tambahan untuk pemindaian pasien terlentang dengan posisi decubitus kiri dan kanan 2.

Atau jaringan ginjal terletak anterior aorta mungkin keliru untuk jaringan retroperitoneal, seperti dapat dilihat pada limfoma atau metastasis pembesaran nodal 2.

CT dan MRI

Kedua CT dan MRI menunjukkan jaringan ginjal penampilan pencitraan normal, tetapi dengan konfigurasi yang abnormal. Peningkatan adalah normal, dan ekskretoris pencitraan fase dapat digunakan untuk menilai sistem pengumpulan.

Pengobatan dan prognosis

Ginjal tapal kuda dalam diri mereka tidak memerlukan pengobatan apapun, dan pasien memiliki harapan hidup yang normal. Hal tersebut adalah penting untuk mengenali kehadiran mereka sebelum operasi perut atau intervensi ginjal untuk salah satu dari mereka banyak komplikasi (lihat di atas).

Diagnosis

Ketika divisualisasikan dengan cross-sectional imaging (CT atau MRI) ada dasarnya diferensial tidak. Pada perawatan USG harus diambil untuk tidak salah ginjal tapal kuda untuk sebuah massa retroperitoneal garis tengah, atau meremehkan panjang ginjal.

Entitas lain yang harus diperhatikan, dari titik murni dan nomenklatur pandang meliputi:

• lintas menyatu ginjal ectopia • panggul ginjal

• malrotated ginjal

Abstract

Background

this study represents a case series to evaluate how successful is the rigid percutaneous nephroscopy as a tool for clearance of all stones in various locations in horseshoe kidneys.

Methods

Between 2005 and 2009, we carried out PCNL (percutaneous nephrolithotomy) for calculi in horseshoe kidneys in 21 renal units (17 patients) in our department. The indications were large stone burden in 18 units and failed SWL(shock wave lithotripsy) in 3 renal units. All procedures were done under general anesthesia; using fluoroscopic guidance for localization and standard alkan dilatation followed by rigid nephroscopy and stone extraction with or without stone disintegration. We analyzed our results regarding the site and number of the required access, the intra and postoperative complications, the presence of any residual stones, as well as their location.

Results

The procedure was completed, using a single access tract in 20 renal units, with the site of puncture being the upper calyx in nine units and the posterior middle calyx in eleven units. Only in one renal unit, two access tracts (an upper and a lower calyceal) were required for completion and a supracostal puncture was required in another case. There was no significant intraoperative bleeding and no blood transfusion was required in any patient. A pelvic perforation occurred in one case, requiring longer PCN (percutaneous nephrostomy) drainage. One patient with infection stones suffered urosepsis postoperatively which was successfully managed. Three cases had residual stones, all located in the renal isthmus, all residuals were un approachable with the rigid instrument; resulting in a overall stone-free rate of 85.7% at discharge.

Conclusion

Percutaneous nephrolithotomy is generally safe and successful in the management of stones in horseshoe kidneys. However, location of the stones in these patients is crucial to decide the proper tool for optimal stone clearance result.

Background

Horseshoe kidney occurs in about 1 in 400 persons [1]. As with other fusion anomalies, it is found more commonly in males. During embryogenesis, fusion of the lower poles prevents normal ascent and causes malrotation with anterior displacement of the collecting system. Insertion of the ureter on the renal pelvis is displaced superiorly and laterally, probably as the result of incomplete renal rotation. It is associated with a significant rate of ureteropelvic obstruction. These factors contribute to impaired drainage with stasis,

infection and predispose to calculus formation. The incidence of stone formation in horseshoe kidneys has been reported to be approximately 20%[2].

Since the reports of Wickham and Kellet in 1981 and Clayman in 1983, percutaneous extraction of stones in horseshoe kidneys has been widely adopted as the standard of care for stones greater than 2 cm or when shock wave lithotripsy fails. While the atypical anatomical orientation of the calices and renal pelvis makes spontaneous passage of stones less likely; orientation of the calices and vessels renders percutaneous puncture of horseshoe kidney relatively safe[3].

Janetschek and Kunzel described three different arterial patterns supplying the horseshoe kidney: normal renal arteries, accessory arteries originating from different levels and entering renal hilum and aberrant arteries entering directly the poles or the isthmus of the kidney. Except for some of the arteries to the isthmus, there were no vessel on the dorsal aspect of the kidney; hence the risk of arterial bleeding is deemed not higher than with normal kidneys [4].

In horseshoe kidney, the frontal plane lies more or less in the sagittal plane of the body. Consequently, the posterior row of calices point dorsomedially and the ventral row dorsolaterally and the renal pelvis is in a ventral position. In some kidneys there are also calices to the isthmus. These always lie within a coronal plane and point medially [4] Generally, the orientation of the collecting system offers surprisingly good access to percutaneous nephrolithotomy. The calices pointing dorsally are entered by direct puncture; where as access to the calices in the isthmus is gained across the pelvis. The anatomical situation results in a lower and medial position of nephrostomy tract, whose orientation is more or less dorsoventral.

In this study we tried to critically evaluate our stone clearance success rates using rigid nephroscope only, in urolithiasis in a horse shoe kidney candidate for percutaneous stone extractions.

Methods

Cairo university hospitals ethical committee granted ethical approval for the study. Our centre is a national tertiary referral centre for urological patients. Between 2005 and 2009, out of our patients records for percutaneous nephrolithitomy we identified a total of 17 patients (21 renal units) underwent percutaneous renal surgery for renal stones in horseshoe kidneys. In eighteen renal unit had a stone burden more than 2.5 cm; as measured by the length of the maximum diameter and were offered PCNL as a primary management. Three patients with stones less than 2 cm undertook PCNL, following failure of fragmentation after 3 sessions of SWL.

All patients were evaluated clinically and underwent routine laboratory investigations. Patients with positive urine cultures were started on an appropriate antibiotic. KUB and IVU were required for planning of the percutaneous access (Figures 1, 2) and US was done to assess the degree of hydronephrosis and the parenchymal thickness. CT angiography was performed only in the first 10 patients to delineate the vascular anatomy and its relation to the percutaneous access in all renal units, all vessels were found to enter from the medial aspect of the kidney and apart for some of the arteries to the isthmus, there were no vessels on the dorsal surface of the kidney.

<a href=Figure 1. KUB showing stones in a horseshoe kidney with stones extending in the isthmus . " id="pdf-obj-24-41" src="pdf-obj-24-41.jpg">

Figure 1. KUB showing stones in a horseshoe kidney with stones extending

in the isthmus.

<a href=Figure 2. CT scan showing isthmic stone in a horseshoe kidney . " id="pdf-obj-25-3" src="pdf-obj-25-3.jpg">

Figure 2. CT scan showing isthmic stone in a horseshoe kidney.

The procedure

After obtaining the patients' informed consent to carry out the surgery including their approval for potential use their anonyms medical data from our data base for research and audit purposes. a dose of perioperitave antibiotic is administered with the induction of general anesthesia. Cystoscopy and ureteric catheterisation are initially performed in the lithotomy position. In case of impacted stone in the pelvis or pelviureteric junction, an opened tipped 6F ureteric catheter is passed over a J-tip guide wire. The patient is then turned to the prone position. Bolsters are placed underneath the patient's abdomen in order to fix the kidney, pushing it posteriorly and limiting its movement during respiration.

With the C-arm in the vertical position, the pelvicalyceal system is opacified and distended with contrast material through the ureteric catheter. An 18-Gauge puncture needle is advanced in a straight line towards the desired calyx. The puncture site is always medial to posterior axillary line, with an angle of 70-90 degrees with the horizontal plane directed towards the targeted calyx. Due to the downward and medial displacement of the calyces, examination with C-arm at 90 degrees provides a direct end-on view of the posterior calyx. The C-arm is then rotated 30 degrees towards the surgeon & the depth of needle penetration is monitored fluoroscopically. The site of the puncture depends on the location and number of the stones as well as the orientation of the pelvicalyceal system. Whenever possible, the middle followed by the upper calyx are chosen to access to the collecting system. In patients with normal renal anatomy. Due to downward displacement of horseshoe kidneys, upper polar accesses are usually achieved through an infracostal puncture which is relatively safe away from the pleura.

Once the puncture was made, a guide wire is then passed into the collecting system, followed by dilation of the tract using the standard telescopic metal Alken set over a central rod. A 30 Fr. Amplatz sheath is then inserted (Figure 3). Because the tract is almost vertical, oblique fluoroscopy is needed to guide tract dilatation and placement of an operating sheath.

<a href=Figure 3. preoperative IVU . " id="pdf-obj-25-21" src="pdf-obj-25-21.jpg">

Figure 3. preoperative IVU.

Nephroscopy is then carried out, using a rigid nephroscope. Following stone visualization, fragmentation and extraction were completed with the use of the Pneumatic Swiss lithoclast and stone grasper forceps. Because of the relatively long almost perpendicular distance from the skin to the renal pelvis in horseshoe kidneys, a middle calyceal puncture is usually preferred in cases with multiple stones, especially if lower calyceal or isthmic. An additional lower calyceal puncture was only required in one case with lower calyceal and isthmic stones, where it allowed for retrieval of the lower calyceal stones by compensating for the distance factor, but it failed to allow us retrieving the isthmic stones as the rigidity and length of the tract does not permit maneuverability of the rigid nephroscope inside the collecting system. The procedure is left tubeless, whenever possible.

A KUB is performed 24 hours later to detect any missed stones and an antegrade or retrograde study is carried out prior to removal of the ureteric catheter or the nephrostomy tube to exclude significant extravasation. Complications and stone-free rates were recorded. Complications were characterized as

major if they required additional intervention or resulted in a prolonged hospital stay or minor if they could be managed conservatively with no additional intervention or morbidity.

Results

Patients' age ranged between 21-51 years with a mean age of 34 years. Overall, of the 17 patients, there were 13 males and 4 females (21 renal units). Two patients had history of prior renal surgery (pyelolithotomy in one and hemi-nephrectomy on the opposite side in another case). Three patients had tried SWL with failure of fragmentation after 3 sessions. All patients had normal kidney functions and none suffered from any bleeding disorder. Eleven renal units (52.4%) had only pelvic stones, 6 units (28.6%) had pelvic and lower calyceal stones, three cases (14.2%) had stones in the pelvis, lower calyx and isthmus and in one case (4.8%) stones were upper calyceal only. The stone size was calculated by measuring the length of the maximum stone diameter. In cases of multiple stones, the length of the maximum diameter of each stone was added to calculate the size. Eleven renal units (52.4%) had stone sizes from 2-6 cm, 10 units (47.6%) had stones > 6 cm.

The procedure was carried out through a single access tract in 20 out of the 21 endoscopies (95.2%). However, two access tracts were needed in 1 renal unit (4.8%). In 9 units (42.85%), the upper calyx alone was the site of puncture. Posterior middle calyx alone was chosen in 11 cases (52.4%). Two punctures were used for completion of the procedure in 1 case (4.8%) and the combination was an upper and lower calyceal approach. Supracostal puncture was done in one case (4.8%). The operative time ranged from 30 min to 160 min (mean 70 min).

Intra-operative bleeding was not significant; none of the cases required blood transfusion intra or postoperatively. Calyceal neck injury occurred in one case, causing minor bleeding that slightly impaired the vision, but the procedure was completed with successful stone clearance. A major pelvic perforation occurred in one case during tract dilatation and was managed conservatively with prolonged drainage with a nephrostomy tube. Antegrade pyelography on day 5 revealed no extravasation.

Residual stones were left in three cases (14.3%). In all cases, they were located in the renal isthmus (0.5-1.5 Cm). In two cases, they were recognized intra-operatively with fluoroscopy and in the third, in the postoperative KUB as it lied over the shadow of the spine. They were all inaccessible from the calyx of entry, as the long nephroscope barely reached the renal isthmus and the perpendicular access did not allow manipulation inside the collecting system. We believe a flexible nephroscope would have achieved complete successful clearance in the two cases recognized intra-operatively; however, unfortunately it was not available in our institute at that time period. A direct isthmic puncture was deemed too hazardous. These three cases were offered post-operative SWL vs conservative management and they all chose to be placed under follow-up (Figure 4).

<a href=Figure 4. intraoperative fluoroscopic image showing access to the lower pole . " id="pdf-obj-26-25" src="pdf-obj-26-25.jpg">

Figure 4. intraoperative fluoroscopic image showing access to the lower

pole.

Two cases among the 21 renal units (9.5%) suffered self-limited leakage from the nephroscopy site for 12-

  • 24 hours. None of them required secondary intervention. Post-operative urosepsis occurred in a young

female with bilateral infection stones. In spite of pre-operative antibiotics, she developed post-operative persistent fever and leucocytosis with profound hypotension. After exclusion of any perirenal collection related to the procedure She was referred to the ICU and responded to hemodynamic support and precise antimicrobial therapy. No bowel injury was encountered in our study; a fact that we attributed to the very medial location of the percutaneous tract, in comparison to standard PCNL; in which bowel injury is also considered uncommon. None of the cases included in our study required secondary intervention.

Discussion

Various treatment modalities have been used to treat stones in horseshoe kidneys, including shock wave lithotripsy (SWL), ureteroscopy, PCNL, and open surgery. Although adequate fragmentation can be achieved by SWL, yet the anatomic abnormalities may prevent fragment passage in a substantial number of patients. The overall stone-free rate has been only around 53% (range 50% to 79%)[2]. Alternatively, PCNL has been used successfully to remove calculi from horseshoe kidneys. The upper pole and mid renal, but not the lower pole calyces, which are located posteriorly, are recommended. The inferior lie of the kidneys places most upper pole calyces below the twelfth rib, thereby making a supracostal puncture a relatively safe access.

The use of PCNL in the treatment of stones in horseshoe kidneys has received little attention in published series. Most reported on a small number of patients. The largest series in English published studies included

  • 47 patients with 60 renal units treated collected from data over a 17 years period by Stephanie et al[5].

Another study by Mansoura University presented their experience on 34 patients with 45 stone-bearing horseshoe kidneys treated by PCNL in a period of 8 years [6]. table 1 shows the results of percutaneous nephrolithotomy in horseshoe kidneys in different studies.

Table 1. Results of different studies done on percutaneous nephrolithotomy in horseshoe kidney

The percentage of upper pole access in previous studies ranged from 62% to 81%. This is because it allows access to the upper pole calices, renal pelvis, lower pole calices, pelviureteric junction and proximal ureter. Furthermore, it can decrease blood loss because the long axis of nephroscope is aligned with the long axis of the kidney, thereby minimizing nephroscope torque on renal tissue during manipulation. Unfortunately, upper pole access results in an unusually long tract, and the instruments may not reach the lower and medial calyces. In our work, the access to the kidney was through upper calyx in 42.8% of cases; whereas a middle calyceal puncture was resorted to in 52.4% of cases. This can be attributed to the fact that 42.8% of our renal units had lower calyceal stones. A middle calyceal access was expected to shorten the distance to the lower calyx substantially.

Three of our patients had residual isthmic stones; that were inaccessible using the rigid nephroscope. We believe flexible nephroscopy to be a vital part of rendering patients stone-free during PCNL in anomalous horseshoe kidneys, especially if associated with isthmic stones. Even though flexible nephroscope was not available in our unit, our stone-free rate (85.7%) is still comparable to other working groups with rates ranging from 72% to 87.5%.

Conclusion

Percutaneous nephrolithotomy is a safe and effective method in treating stones in horseshoe kidney. The procedure offers the highest likelihood of rendering patients stone free. However, patients with stones in an isthmic location the availability of flexible nephroscope might achieve a better clearance.

Latar belakang Penelitian ini merupakan serangkaian kasus untuk mengevaluasi seberapa sukses adalah nephroscopy perkutan kaku sebagai alat untuk izin dari semua batu di berbagai lokasi di ginjal tapal kuda. Metode Antara 2005 dan 2009, kami melakukan PCNL (percutaneous nephrolithotomy) untuk kalkuli dalam ginjal tapal kuda di 21 unit ginjal (17 pasien) di departemen kami. Indikasi adalah beban batu besar di 18 unit dan gagal SWL (shock wave lithotripsy) dalam 3 unit ginjal. Semua prosedur dilakukan di bawah anestesi umum, menggunakan bimbingan fluoroscopic untuk lokalisasi dan dilatasi Alkan standar yang diikuti oleh nephroscopy kaku dan ekstraksi batu dengan atau tanpa disintegrasi batu. Kami menganalisis hasil kami mengenai situs dan jumlah akses yang diperlukan, komplikasi intra dan pasca operasi, kehadiran setiap batu sisa, serta lokasi mereka. Hasil Prosedur selesai, menggunakan saluran akses tunggal dalam 20 unit ginjal, dengan lokasi tusukan menjadi kelopak bagian atas di sembilan unit dan tengah posterior kelopak di sebelas unit. Hanya dalam satu unit ginjal, saluran akses dua (bagian atas dan bagian calyceal rendah) yang diperlukan untuk penyelesaian dan tusukan supracostal diperlukan dalam kasus lain. Tidak ada perdarahan intraoperatif signifikan dan tidak ada transfusi darah yang diperlukan dalam setiap pasien. Sebuah perforasi panggul terjadi dalam satu kasus, yang membutuhkan lebih lama PCN (percutaneous nefrostomi) drainase. Satu pasien dengan batu infeksi pasca operasi mengalami urosepsis yang berhasil dikelola. Tiga kasus memiliki batu sisa, semua terletak di tanah genting ginjal, semua residu yang didekati un dengan instrumen yang kaku, sehingga tingkat batu-bebas keseluruhan 85,7% pada debit. Kesimpulan Percutaneous nephrolithotomy umumnya aman dan sukses dalam pengelolaan batu pada ginjal tapal kuda. Namun, lokasi dari batu pada pasien-pasien sangat penting untuk menentukan alat yang tepat untuk hasil pembersihan batu yang optimal. Latar belakang Ginjal tapal kuda terjadi pada sekitar 1 dari 400 orang [1]. Seperti dengan anomali fusi lainnya, ditemukan lebih umum pada laki-laki. Selama embriogenesis, fusi dari kutub rendah mencegah pendakian normal dan menyebabkan malrotation dengan perpindahan anterior dari sistem pengumpulan. Penyisipan ureter pada pelvis ginjal dipindahkan superior dan lateral, mungkin sebagai akibat dari rotasi ginjal tidak lengkap. Hal ini terkait dengan tingkat signifikan obstruksi ureteropelvic. Faktor-faktor ini berkontribusi terhadap drainase terganggu dengan stasis, infeksi dan menyebabkan rentan terhadap pembentukan kalkulus. Insiden pembentukan batu dalam ginjal tapal kuda telah dilaporkan menjadi sekitar 20% [2]. Karena laporan Wickham dan Kellet pada tahun 1981 dan pada tahun 1983 Clayman, perkutan ekstraksi batu di ginjal tapal kuda telah diadopsi secara luas sebagai standar perawatan untuk batu yang lebih besar dari 2 cm atau ketika gelombang kejut lithotripsy gagal. Sementara orientasi anatomi atipikal dari kalises dan pelvis ginjal membuat perjalanan spontan batu kecil kemungkinannya, orientasi kalises dan pembuluh membuat perkutan tusuk ginjal tapal kuda relatif aman [3]. Janetschek dan Künzel menggambarkan tiga pola yang berbeda arteri memasok ginjal tapal kuda: arteri ginjal normal, arteri aksesori yang berasal dari berbagai tingkat dan memasuki hilus ginjal dan arteri menyimpang masuk langsung kutub atau tanah genting ginjal. Kecuali untuk beberapa arteri tanah genting, tidak ada kapal pada aspek dorsal ginjal, maka risiko perdarahan arteri dianggap tidak lebih tinggi dibandingkan dengan ginjal normal [4]. Dalam ginjal tapal kuda, bidang frontal terletak lebih atau kurang di pesawat sagittal dari tubuh. Akibatnya, baris posterior kalises menunjuk dorsomedially dan baris ventral dorsolaterally dan pelvis ginjal berada dalam posisi ventral. Dalam beberapa ginjal ada juga kalises ke tanah genting. Ini selalu terletak dalam pesawat koronal dan titik medial [4] Umumnya, orientasi sistem pengumpulan menawarkan akses mengejutkan baik untuk perkutan nephrolithotomy. Para kalises menunjuk punggung yang dimasukkan oleh tusukan langsung, di mana sebagai akses ke kalises di tanah genting diperoleh di seluruh panggul. Hasil Situasi anatomi dalam posisi yang lebih rendah dan medial nefrostomi saluran, yang orientasinya lebih atau kurang dorsoventral.

Dalam penelitian ini kami mencoba untuk mengevaluasi secara kritis batu kami tingkat keberhasilan izin menggunakan nephroscope kaku saja, di dalam urolithiasis calon kuda sepatu ginjal untuk perkutan ekstraksi batu. Metode Kairo universitas rumah sakit komite etik memberikan persetujuan etika untuk penelitian. Center kami adalah pusat rujukan nasional tersier untuk pasien urologis. Antara 2005 dan 2009, dari catatan kami pasien untuk perkutan nephrolithitomy kami mengidentifikasi total 17 pasien (21 unit ginjal) menjalani operasi ginjal perkutan untuk batu ginjal di ginjal tapal kuda. Dalam delapan belas Unit ginjal memiliki beban batu lebih dari 2,5 cm, yang diukur dengan panjang diameter maksimum dan ditawarkan PCNL sebagai manajemen utama. Tiga pasien dengan batu kurang dari 2 cm melakukan PCNL, kegagalan berikut fragmentasi setelah 3 sesi SWL. Semua pasien dievaluasi klinis dan menjalani pemeriksaan laboratorium rutin. Pasien dengan kultur urin positif dimulai pada antibiotik yang sesuai. KUB dan IVU yang diperlukan untuk perencanaan akses perkutan (Angka 1, 2) dan AS dilakukan untuk menilai tingkat hidronefrosis dan ketebalan parenkim. CT angiography dilakukan hanya dalam 10 pasien pertama yang menggambarkan anatomi pembuluh darah dan hubungannya dengan akses perkutan di seluruh unit ginjal, semua kapal ditemukan masuk dari aspek medial ginjal dan terpisah untuk beberapa dari arteri ke tanah genting , tidak ada pembuluh pada permukaan dorsal ginjal. Gambar 1. KUB menunjukkan batu di ginjal tapal kuda dengan batu memanjang di tanah genting. Gambar 2. CT scan menunjukkan batu isthmic dalam ginjal tapal kuda. Prosedur Setelah memperoleh informed consent pasien untuk melaksanakan operasi termasuk persetujuan mereka untuk menggunakan potensi mereka anonim data medis dari basis data kami untuk tujuan penelitian dan audit. dosis antibiotik perioperitave diberikan dengan induksi anestesi umum. Sistoskopi dan kateterisasi ureter yang awalnya dilakukan dalam posisi litotomi. Dalam kasus batu berdampak pada persimpangan panggul atau pelviureteric, yang dibuka berujung kateter ureter 6F dilewatkan melalui kawat penuntun J- tip. Pasien kemudian berbalik ke posisi tengkurap. Guling ditempatkan di bawah perut pasien dalam rangka untuk memperbaiki ginjal, mendorongnya posterior dan membatasi gerakannya selama respirasi. Dengan lengan C-dalam posisi vertikal, sistem pelvicalyceal yang opacifier dan buncit dengan bahan kontras melalui kateter ureter. Sebuah tusukan jarum 18-Gauge yang maju dalam garis lurus ke arah kelopak yang diinginkan. Situs tusukan selalu medial aksila posterior dengan garis, dengan sudut 70-90 derajat dengan bidang horizontal diarahkan menuju tampuk ditargetkan. Karena perpindahan ke bawah dan medial calyces, pemeriksaan dengan C-lengan pada 90 derajat memberikan end-on langsung pandangan kelopak posterior. The C-lengan kemudian diputar 30 derajat ke arah ahli bedah & kedalaman penetrasi jarum dimonitor fluoroscopically. Situs dari tusukan tergantung pada lokasi dan jumlah batu serta orientasi sistem pelvicalyceal. Bila mungkin, tengah diikuti oleh kelopak bagian atas yang dipilih untuk mengakses ke sistem pengumpul. Pada pasien dengan anatomi ginjal normal. Karena perpindahan ke bawah ginjal tapal kuda, akses kutub atas biasanya dicapai melalui tusukan yg terletak di bawah tulang rusuk yang relatif aman jauh dari pleura. Setelah menusuk dibuat, kawat pemandu kemudian dilewatkan ke dalam sistem pengumpulan, diikuti dengan pelebaran saluran menggunakan Alken logam standar teleskopik terbenam di batang tengah. Sebuah 30 Fr. Amplatz selubung tersebut kemudian dimasukkan (Gambar 3). Karena saluran hampir vertikal, miring fluoroskopi diperlukan untuk memandu dilatasi saluran dan penempatan selubung operasi. Gambar 3. preoperative IVU. Nephroscopy kemudian dilakukan, dengan menggunakan nephroscope kaku. Batu visualisasi berikut, fragmentasi dan ekstraksi telah diselesaikan dengan penggunaan lithoclast Pneumatic Swiss dan tamak batu forsep. Karena jarak hampir tegak lurus relatif panjang dari kulit ke pelvis ginjal dalam ginjal tapal kuda, tusukan calyceal menengah biasanya lebih disukai dalam kasus-kasus dengan batu beberapa, terutama jika lebih rendah calyceal atau isthmic. Sebuah tusukan calyceal tambahan rendah hanya diperlukan dalam satu kasus dengan calyceal rendah dan batu isthmic, di mana ia diperbolehkan untuk pengambilan batu calyceal rendah dengan kompensasi untuk faktor jarak, tetapi gagal untuk

memungkinkan kita mengambil batu isthmic sebagai kekakuan dan panjang dari saluran tersebut tidak memungkinkan manuver dari nephroscope kaku dalam sistem pengumpulan. Prosedur ini meninggalkan tubeless, bila memungkinkan. Sebuah KUB dilakukan 24 jam kemudian untuk mendeteksi adanya batu terjawab dan studi antegrade atau retrograde dilakukan sebelum penghapusan kateter ureter atau tabung nefrostomi untuk mengecualikan ekstravasasi signifikan. Komplikasi dan batu-bebas tarif dicatat. Komplikasi yang dicirikan sebagai besar jika mereka diperlukan intervensi tambahan atau menghasilkan tinggal di rumah sakit yang berkepanjangan atau kecil jika mereka dapat dikelola secara konservatif tanpa intervensi tambahan atau morbiditas. Hasil Usia pasien berkisar antara 21-51 tahun dengan usia rata-rata 34 tahun. Secara keseluruhan, dari 17 pasien, ada 13 laki-laki dan 4 perempuan (21 unit ginjal). Dua pasien memiliki riwayat operasi ginjal sebelum (pyelolithotomy dalam satu dan hemi-nephrectomy di sisi berlawanan dalam kasus lain). Tiga pasien telah mencoba SWL dengan kegagalan fragmentasi setelah 3 sesi. Semua pasien memiliki fungsi ginjal normal dan tidak menderita gangguan pendarahan. Sebelas unit ginjal (52,4%) hanya memiliki batu panggul, 6 unit (28,6%) telah panggul dan batu calyceal rendah, tiga kasus (14,2%) memiliki batu di panggul, kelopak rendah dan isthmus dan dalam satu kasus (4,8%) batu adalah atas calyceal saja. Ukuran batu dihitung dengan mengukur panjang dari diameter batu maksimal. Dalam kasus batu ganda, panjang diameter maksimum masing-masing batu ditambahkan untuk menghitung ukuran. Sebelas unit ginjal (52,4%) memiliki ukuran batu dari 2-6 cm, 10 unit (47,6%) memiliki batu> 6 cm. Prosedur ini dilakukan melalui saluran akses tunggal dalam 20 keluar dari 21 endoscopies (95,2%). Namun, dua saluran akses yang diperlukan dalam 1 unit ginjal (4,8%). Pada 9 unit (42,85%), kelopak bagian atas saja adalah tempat tusukan. Posterior tengah kelopak saja terpilih dalam 11 kasus (52,4%). Dua tusukan yang digunakan untuk menyelesaikan prosedur dalam 1 kasus (4,8%) dan kombinasi itu pendekatan calyceal atas dan bawah. Tusuk Supracostal dilakukan dalam satu kasus (4,8%). Waktu operasi berkisar antara 30 menit sampai 160 menit (rata-rata 70 menit). Intra-operatif perdarahan tidak signifikan, tidak ada satupun kasus yang dibutuhkan darah intra transfusi atau pasca operasi. Cedera leher Calyceal terjadi dalam satu kasus, menyebabkan pendarahan kecil yang sedikit gangguan penglihatan, tetapi prosedur selesai dengan izin batu sukses. Sebuah perforasi panggul besar terjadi dalam satu kasus selama dilatasi saluran dan dikelola secara konservatif dengan drainase berkepanjangan dengan tabung nefrostomi. Pyelography antegrade pada hari ke 5 mengungkapkan ekstravasasi tidak. Sisa batu yang tersisa di tiga kasus (14,3%). Dalam semua kasus, mereka berada di tanah genting ginjal (0,5- 1,5 Cm). Dalam dua kasus, mereka diakui intra-bedah dengan fluoroskopi dan ketiga, dalam KUB pascaoperasi karena berbohong atas bayangan tulang belakang. Mereka semua dapat diakses dari kelopak masuk, sebagai nephroscope panjang hampir mencapai tanah genting ginjal dan akses tegak lurus tidak memungkinkan manipulasi dalam sistem pengumpulan. Kami percaya nephroscope fleksibel akan mencapai sukses izin lengkap dalam dua kasus diakui intra-bedah, namun, sayangnya itu tidak tersedia di lembaga kami pada jangka waktu tersebut. Sebuah tusukan isthmic langsung dianggap terlalu berbahaya. Ketiga kasus yang ditawarkan pasca-operasi SWL manajemen vs konservatif dan mereka semua memilih untuk ditempatkan di bawah tindak lanjut (Gambar 4). Gambar 4. intraoperatif fluoroscopic gambar yang menunjukkan akses ke tiang yang lebih rendah. Dua kasus di antara 21 unit ginjal (9,5%) mengalami self-terbatas kebocoran dari situs nephroscopy selama 12-24 jam. Tak satu pun dari mereka diperlukan intervensi sekunder. Pasca-operasi urosepsis terjadi pada seorang wanita muda dengan batu infeksi bilateral. Terlepas dari pra-operatif antibiotik, ia mengembangkan pasca operasi demam gigih dan leukositosis dengan hipotensi yang mendalam. Setelah mengesampingkan setiap koleksi perirenal terkait dengan prosedur Dia dirujuk ke ICU dan menanggapi dukungan hemodinamik dan terapi antimikroba yang tepat. Tidak ada cedera usus ditemui dalam penelitian kami, sebuah kenyataan bahwa kita dikaitkan dengan lokasi yang sangat medial saluran perkutan, dibandingkan dengan PCNL standar, di mana cedera usus juga dianggap biasa. Tak satu pun dari kasus- kasus yang termasuk dalam penelitian kami diperlukan intervensi sekunder.

Diskusi Berbagai modalitas pengobatan telah digunakan untuk mengobati batu pada ginjal tapal kuda, termasuk gelombang kejut lithotripsy (SWL), ureteroscopy, PCNL, dan operasi terbuka. Meskipun fragmentasi yang memadai dapat dicapai dengan SWL, namun kelainan anatomi dapat mencegah bagian fragmen dalam sejumlah besar pasien. Tingkat batu bebas keseluruhan telah hanya sekitar 53% (kisaran 50% sampai 79%) [2]. Atau, PCNL telah berhasil digunakan untuk menghilangkan kalkuli dari ginjal tapal kuda. Tiang atas dan pertengahan ginjal, tapi bukan calyces tiang yang lebih rendah, yang terletak posterior, yang dianjurkan. Kebohongan inferior ginjal menempatkan calyces tiang paling atas di bawah tulang rusuk kedua belas, sehingga membuat tusukan supracostal akses yang relatif aman. Penggunaan PCNL dalam pengobatan batu ginjal di tapal kuda telah mendapat sedikit perhatian dalam seri diterbitkan. Kebanyakan melaporkan pada sejumlah kecil pasien. Seri terbesar dalam penelitian yang diterbitkan Inggris termasuk 47 pasien dengan 60 unit ginjal diperlakukan dikumpulkan dari data selama periode 17 tahun oleh Stephanie et al [5]. Studi lain oleh Universitas Mansoura mempresentasikan pengalaman mereka pada 34 pasien dengan 45 batu ginjal tapal kuda bantalan dirawat oleh PCNL dalam jangka waktu 8 tahun [6]. Tabel 1 menunjukkan hasil perkutan nephrolithotomy di ginjal tapal kuda dalam studi yang berbeda. Tabel 1. Hasil studi yang berbeda dilakukan pada perkutan nephrolithotomy di ginjal tapal kuda Persentase akses tiang atas dalam studi sebelumnya berkisar antara 62% sampai 81%. Hal ini karena memungkinkan akses ke kalises tiang atas, pelvis ginjal, kalises tiang lebih rendah, persimpangan pelviureteric dan ureter proksimal. Selain itu, dapat menurunkan kehilangan darah karena sumbu panjang nephroscope sejajar dengan sumbu panjang ginjal, sehingga meminimalkan torsi nephroscope pada jaringan ginjal selama manipulasi. Sayangnya, hasil akses atas tiang dalam saluran sangat panjang, dan instrumen tidak mungkin mencapai calyces rendah dan medial. Dalam pekerjaan kami, akses ke ginjal adalah melalui kelopak atas dalam 42,8% kasus, sedangkan tusukan calyceal menengah yang terpaksa dalam 52,4% kasus. Hal ini dapat dikaitkan dengan fakta bahwa 42,8% dari unit ginjal kita memiliki batu calyceal rendah. Sebuah akses calyceal menengah diharapkan untuk memperpendek jarak ke kelopak bawah substansial. Tiga dari pasien kami memiliki batu isthmic sisa, yang dapat diakses dengan menggunakan nephroscope kaku. Kami percaya nephroscopy fleksibel untuk menjadi bagian penting dari render pasien batu-gratis selama PCNL dalam ginjal tapal kuda anomali, terutama jika dikaitkan dengan batu isthmic. Meskipun nephroscope fleksibel tidak tersedia di unit kami, batu-free rate kami (85,7%) masih sebanding dengan kelompok kerja lain dengan berkisar antara 72% sampai 87,5%. Kesimpulan Percutaneous nephrolithotomy adalah metode yang aman dan efektif dalam mengobati batu di ginjal tapal kuda. Prosedur ini menawarkan kemungkinan tertinggi render pasien batu gratis. Namun, pasien dengan batu di lokasi isthmic ketersediaan nephroscope fleksibel dapat mencapai clearance yang lebih baik.

Competing interests

The authors declare that they have no competing interests.

Authors' contributions

AK: data collection and querying the hospital records. AS: data collection & participation in writing the manuscript. ME: design the study &writing the manuscript. AE: drafting the manuscript. TO: participation in the design of the study &interpretation of the results. ME: general supervision. All authors read and approved the final manuscript

References

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N Engl J Med 1959, 261:684. PubMed Abstract

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J Urol 2003, 170(1):48-51. PubMed Abstract | Publisher Full Text

1. Glenn JF: Analysis of 51 patients with horseshoe kidney. N Engl J Med 1959, 261:. PubMed Abstract 2. Raj GV, Auge BrianK, Weizer AlonZ, Denstedt JohnD, et al .: Percutaneous management of calculi within horseshoe kidneys. J Urol 2003, 170 (1) : 48-51 . PubMed Abstract | Publisher Full Text 3. Yohannes P, Smith AD: The endourological management of complications associated with horseshoe kidney. J Urol 2002, 168 (1) : 5-8. Review PubMed Abstract | Publisher Full Text 4. Janetschek G, Kunzel KH: Percutaneous nephrolithotomy in horseshoe kidneys. Applied anatomy and clinical experience. Brit J Urol 1988, 62: 117-122. PubMed Abstract | Publisher Full Text 5. Symons StephanieJ, Anil Ramachandran, Abraham Kurien, Ramen Baiysha, Desai MaheshR: Urolithiasis in the horseshoe kidney: a single-centre experience. BJUI Int 2008, 102 (11) : 1676-80. Publisher Full Text 6. Shokeir AA, El-Nahas AR, Shoma AM, Eraky I, El-Kenawy M, Mokhtar A, El-Kappany H: Percutaneous nephrolithotomy in treatment of large stones within horseshoe kidneys. Urology 2004, 64 (3) : 426-9. PubMed Abstract | Publisher Full Text 7. Jones DJ, Wickham JA, Kellett MJ: Percutaneous nephrolithotomy for calculi in horseshoe kidneys. J Urol 1991, 145: 481-3. PubMed Abstract 8. Al-Otaibi K, Hosking DH: Percutaneous stone removal in horseshoe kidneys. J Urol 1999, 162: 674-7. PubMed Abstract | Publisher Full Text 9. Lingeman JE, Saw KC: Percutaneous operative procedures in horseshoe kidneys. J Urol 1999, 161 (suppl) : 371. abstract 1436 Publisher Full Text http://wikis.lib.ncsu.edu/index.php/Horseshoe_Kidney " id="pdf-obj-32-31" src="pdf-obj-32-31.jpg">
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J Urol 2002, 168(1):5-8.

Review

1. Glenn JF: Analysis of 51 patients with horseshoe kidney. N Engl J Med 1959, 261:. PubMed Abstract 2. Raj GV, Auge BrianK, Weizer AlonZ, Denstedt JohnD, et al .: Percutaneous management of calculi within horseshoe kidneys. J Urol 2003, 170 (1) : 48-51 . PubMed Abstract | Publisher Full Text 3. Yohannes P, Smith AD: The endourological management of complications associated with horseshoe kidney. J Urol 2002, 168 (1) : 5-8. Review PubMed Abstract | Publisher Full Text 4. Janetschek G, Kunzel KH: Percutaneous nephrolithotomy in horseshoe kidneys. Applied anatomy and clinical experience. Brit J Urol 1988, 62: 117-122. PubMed Abstract | Publisher Full Text 5. Symons StephanieJ, Anil Ramachandran, Abraham Kurien, Ramen Baiysha, Desai MaheshR: Urolithiasis in the horseshoe kidney: a single-centre experience. BJUI Int 2008, 102 (11) : 1676-80. Publisher Full Text 6. Shokeir AA, El-Nahas AR, Shoma AM, Eraky I, El-Kenawy M, Mokhtar A, El-Kappany H: Percutaneous nephrolithotomy in treatment of large stones within horseshoe kidneys. Urology 2004, 64 (3) : 426-9. PubMed Abstract | Publisher Full Text 7. Jones DJ, Wickham JA, Kellett MJ: Percutaneous nephrolithotomy for calculi in horseshoe kidneys. J Urol 1991, 145: 481-3. PubMed Abstract 8. Al-Otaibi K, Hosking DH: Percutaneous stone removal in horseshoe kidneys. J Urol 1999, 162: 674-7. PubMed Abstract | Publisher Full Text 9. Lingeman JE, Saw KC: Percutaneous operative procedures in horseshoe kidneys. J Urol 1999, 161 (suppl) : 371. abstract 1436 Publisher Full Text http://wikis.lib.ncsu.edu/index.php/Horseshoe_Kidney " id="pdf-obj-32-49" src="pdf-obj-32-49.jpg">
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Brit J Urol 1988, 62:117-122. PubMed Abstract | Publisher Full Text

1. Glenn JF: Analysis of 51 patients with horseshoe kidney. N Engl J Med 1959, 261:. PubMed Abstract 2. Raj GV, Auge BrianK, Weizer AlonZ, Denstedt JohnD, et al .: Percutaneous management of calculi within horseshoe kidneys. J Urol 2003, 170 (1) : 48-51 . PubMed Abstract | Publisher Full Text 3. Yohannes P, Smith AD: The endourological management of complications associated with horseshoe kidney. J Urol 2002, 168 (1) : 5-8. Review PubMed Abstract | Publisher Full Text 4. Janetschek G, Kunzel KH: Percutaneous nephrolithotomy in horseshoe kidneys. Applied anatomy and clinical experience. Brit J Urol 1988, 62: 117-122. PubMed Abstract | Publisher Full Text 5. Symons StephanieJ, Anil Ramachandran, Abraham Kurien, Ramen Baiysha, Desai MaheshR: Urolithiasis in the horseshoe kidney: a single-centre experience. BJUI Int 2008, 102 (11) : 1676-80. Publisher Full Text 6. Shokeir AA, El-Nahas AR, Shoma AM, Eraky I, El-Kenawy M, Mokhtar A, El-Kappany H: Percutaneous nephrolithotomy in treatment of large stones within horseshoe kidneys. Urology 2004, 64 (3) : 426-9. PubMed Abstract | Publisher Full Text 7. Jones DJ, Wickham JA, Kellett MJ: Percutaneous nephrolithotomy for calculi in horseshoe kidneys. J Urol 1991, 145: 481-3. PubMed Abstract 8. Al-Otaibi K, Hosking DH: Percutaneous stone removal in horseshoe kidneys. J Urol 1999, 162: 674-7. PubMed Abstract | Publisher Full Text 9. Lingeman JE, Saw KC: Percutaneous operative procedures in horseshoe kidneys. J Urol 1999, 161 (suppl) : 371. abstract 1436 Publisher Full Text http://wikis.lib.ncsu.edu/index.php/Horseshoe_Kidney " id="pdf-obj-32-62" src="pdf-obj-32-62.jpg">
  • 5. Symons StephanieJ, Anil Ramachandran, Abraham Kurien, Ramen Baiysha, Desai MaheshR:Urolithiasis in the horseshoe kidney: a single-centre experience.

BJUI Int 2008, 102(11):1676-80. Publisher Full Text

1. Glenn JF: Analysis of 51 patients with horseshoe kidney. N Engl J Med 1959, 261:. PubMed Abstract 2. Raj GV, Auge BrianK, Weizer AlonZ, Denstedt JohnD, et al .: Percutaneous management of calculi within horseshoe kidneys. J Urol 2003, 170 (1) : 48-51 . PubMed Abstract | Publisher Full Text 3. Yohannes P, Smith AD: The endourological management of complications associated with horseshoe kidney. J Urol 2002, 168 (1) : 5-8. Review PubMed Abstract | Publisher Full Text 4. Janetschek G, Kunzel KH: Percutaneous nephrolithotomy in horseshoe kidneys. Applied anatomy and clinical experience. Brit J Urol 1988, 62: 117-122. PubMed Abstract | Publisher Full Text 5. Symons StephanieJ, Anil Ramachandran, Abraham Kurien, Ramen Baiysha, Desai MaheshR: Urolithiasis in the horseshoe kidney: a single-centre experience. BJUI Int 2008, 102 (11) : 1676-80. Publisher Full Text 6. Shokeir AA, El-Nahas AR, Shoma AM, Eraky I, El-Kenawy M, Mokhtar A, El-Kappany H: Percutaneous nephrolithotomy in treatment of large stones within horseshoe kidneys. Urology 2004, 64 (3) : 426-9. PubMed Abstract | Publisher Full Text 7. Jones DJ, Wickham JA, Kellett MJ: Percutaneous nephrolithotomy for calculi in horseshoe kidneys. J Urol 1991, 145: 481-3. PubMed Abstract 8. Al-Otaibi K, Hosking DH: Percutaneous stone removal in horseshoe kidneys. J Urol 1999, 162: 674-7. PubMed Abstract | Publisher Full Text 9. Lingeman JE, Saw KC: Percutaneous operative procedures in horseshoe kidneys. J Urol 1999, 161 (suppl) : 371. abstract 1436 Publisher Full Text http://wikis.lib.ncsu.edu/index.php/Horseshoe_Kidney " id="pdf-obj-32-75" src="pdf-obj-32-75.jpg">
  • 6. Shokeir AA, El-Nahas AR, Shoma AM, Eraky I, El-Kenawy M, Mokhtar A, El-Kappany H:Percutaneous nephrolithotomy in treatment of large stones within horseshoe kidneys.

Urology 2004, 64(3):426-9. PubMed Abstract | Publisher Full Text

1. Glenn JF: Analysis of 51 patients with horseshoe kidney. N Engl J Med 1959, 261:. PubMed Abstract 2. Raj GV, Auge BrianK, Weizer AlonZ, Denstedt JohnD, et al .: Percutaneous management of calculi within horseshoe kidneys. J Urol 2003, 170 (1) : 48-51 . PubMed Abstract | Publisher Full Text 3. Yohannes P, Smith AD: The endourological management of complications associated with horseshoe kidney. J Urol 2002, 168 (1) : 5-8. Review PubMed Abstract | Publisher Full Text 4. Janetschek G, Kunzel KH: Percutaneous nephrolithotomy in horseshoe kidneys. Applied anatomy and clinical experience. Brit J Urol 1988, 62: 117-122. PubMed Abstract | Publisher Full Text 5. Symons StephanieJ, Anil Ramachandran, Abraham Kurien, Ramen Baiysha, Desai MaheshR: Urolithiasis in the horseshoe kidney: a single-centre experience. BJUI Int 2008, 102 (11) : 1676-80. Publisher Full Text 6. Shokeir AA, El-Nahas AR, Shoma AM, Eraky I, El-Kenawy M, Mokhtar A, El-Kappany H: Percutaneous nephrolithotomy in treatment of large stones within horseshoe kidneys. Urology 2004, 64 (3) : 426-9. PubMed Abstract | Publisher Full Text 7. Jones DJ, Wickham JA, Kellett MJ: Percutaneous nephrolithotomy for calculi in horseshoe kidneys. J Urol 1991, 145: 481-3. PubMed Abstract 8. Al-Otaibi K, Hosking DH: Percutaneous stone removal in horseshoe kidneys. J Urol 1999, 162: 674-7. PubMed Abstract | Publisher Full Text 9. Lingeman JE, Saw KC: Percutaneous operative procedures in horseshoe kidneys. J Urol 1999, 161 (suppl) : 371. abstract 1436 Publisher Full Text http://wikis.lib.ncsu.edu/index.php/Horseshoe_Kidney " id="pdf-obj-32-90" src="pdf-obj-32-90.jpg">
  • 7. Jones DJ, Wickham JA, Kellett MJ: Percutaneous nephrolithotomy for calculi in horseshoe kidneys.

J Urol 1991, 145:481-3. PubMed Abstract

  • 8. Al-Otaibi K, Hosking DH: Percutaneous stone removal in horseshoe kidneys.

J Urol 1999, 162:674-7. PubMed Abstract | Publisher Full Text

1. Glenn JF: Analysis of 51 patients with horseshoe kidney. N Engl J Med 1959, 261:. PubMed Abstract 2. Raj GV, Auge BrianK, Weizer AlonZ, Denstedt JohnD, et al .: Percutaneous management of calculi within horseshoe kidneys. J Urol 2003, 170 (1) : 48-51 . PubMed Abstract | Publisher Full Text 3. Yohannes P, Smith AD: The endourological management of complications associated with horseshoe kidney. J Urol 2002, 168 (1) : 5-8. Review PubMed Abstract | Publisher Full Text 4. Janetschek G, Kunzel KH: Percutaneous nephrolithotomy in horseshoe kidneys. Applied anatomy and clinical experience. Brit J Urol 1988, 62: 117-122. PubMed Abstract | Publisher Full Text 5. Symons StephanieJ, Anil Ramachandran, Abraham Kurien, Ramen Baiysha, Desai MaheshR: Urolithiasis in the horseshoe kidney: a single-centre experience. BJUI Int 2008, 102 (11) : 1676-80. Publisher Full Text 6. Shokeir AA, El-Nahas AR, Shoma AM, Eraky I, El-Kenawy M, Mokhtar A, El-Kappany H: Percutaneous nephrolithotomy in treatment of large stones within horseshoe kidneys. Urology 2004, 64 (3) : 426-9. PubMed Abstract | Publisher Full Text 7. Jones DJ, Wickham JA, Kellett MJ: Percutaneous nephrolithotomy for calculi in horseshoe kidneys. J Urol 1991, 145: 481-3. PubMed Abstract 8. Al-Otaibi K, Hosking DH: Percutaneous stone removal in horseshoe kidneys. J Urol 1999, 162: 674-7. PubMed Abstract | Publisher Full Text 9. Lingeman JE, Saw KC: Percutaneous operative procedures in horseshoe kidneys. J Urol 1999, 161 (suppl) : 371. abstract 1436 Publisher Full Text http://wikis.lib.ncsu.edu/index.php/Horseshoe_Kidney " id="pdf-obj-32-112" src="pdf-obj-32-112.jpg">
  • 9. Lingeman JE, Saw KC: Percutaneous operative procedures in horseshoe kidneys.

J Urol 1999, 161(suppl):371.

abstract 1436

1. Glenn JF: Analysis of 51 patients with horseshoe kidney. N Engl J Med 1959, 261:. PubMed Abstract 2. Raj GV, Auge BrianK, Weizer AlonZ, Denstedt JohnD, et al .: Percutaneous management of calculi within horseshoe kidneys. J Urol 2003, 170 (1) : 48-51 . PubMed Abstract | Publisher Full Text 3. Yohannes P, Smith AD: The endourological management of complications associated with horseshoe kidney. J Urol 2002, 168 (1) : 5-8. Review PubMed Abstract | Publisher Full Text 4. Janetschek G, Kunzel KH: Percutaneous nephrolithotomy in horseshoe kidneys. Applied anatomy and clinical experience. Brit J Urol 1988, 62: 117-122. PubMed Abstract | Publisher Full Text 5. Symons StephanieJ, Anil Ramachandran, Abraham Kurien, Ramen Baiysha, Desai MaheshR: Urolithiasis in the horseshoe kidney: a single-centre experience. BJUI Int 2008, 102 (11) : 1676-80. Publisher Full Text 6. Shokeir AA, El-Nahas AR, Shoma AM, Eraky I, El-Kenawy M, Mokhtar A, El-Kappany H: Percutaneous nephrolithotomy in treatment of large stones within horseshoe kidneys. Urology 2004, 64 (3) : 426-9. PubMed Abstract | Publisher Full Text 7. Jones DJ, Wickham JA, Kellett MJ: Percutaneous nephrolithotomy for calculi in horseshoe kidneys. J Urol 1991, 145: 481-3. PubMed Abstract 8. Al-Otaibi K, Hosking DH: Percutaneous stone removal in horseshoe kidneys. J Urol 1999, 162: 674-7. PubMed Abstract | Publisher Full Text 9. Lingeman JE, Saw KC: Percutaneous operative procedures in horseshoe kidneys. J Urol 1999, 161 (suppl) : 371. abstract 1436 Publisher Full Text http://wikis.lib.ncsu.edu/index.php/Horseshoe_Kidney " id="pdf-obj-32-128" src="pdf-obj-32-128.jpg">

http://wikis.lib.ncsu.edu/index.php/Horseshoe_Kidney

Horseshoe Kidney

Introduction : What is Horseshoe Kidney?

Horseshoe Kidney Introduction : What is Horseshoe Kidney? Frequency Horseshoe kidney occurs approximately 1 per 500

Frequency

Horseshoe kidney occurs approximately 1 per 500 live births and appears to be twice as common in males as in women. There has been no known genetic determinants, yet it has been reported in identical twins and in siblings within the same family. Although present at birth horseshoe kidney usually causes no signs or symptoms and often goes undetected for years. It is possible that during a physical examination it could present as a midline lower abdominal mass, but on average it is identifiable during radiological examinations.

Etiology

There are two theories that have been proposed regarding the embryogenesis of horseshoe kidneys. The classic teaching of mechanical fusion holds that the horseshoe kidney is formed during organogenesis when the inferior poles of the early kidneys come in contact fusing in the lower midline. This mechanical fusion seems valid for horseshoe kidneys with a fibrous isthmus.

The explanation for lateral fusion is that, during early embryonic life, lateral flexion of the lumbo-sacral spine may push one of the developing kidneys toward the midline and can lead to asymmetric fusion. In later embryonic life, the ascent of the fused kidney is hindered by the inferior mesenteric artery, with the isthmus of the horseshoe kidney becoming trapped under it. As a result, the horseshoe kidney always lies at a position that is lower than normal.

More recent studies have proven the second theory of abnormal fusion of tissue associated, with the parenchymatous isthmus. Some horseshoe kidneys are the result of a teratogenic event involving the abnormal migration of posterior nephrogenic cells which then unite to form the isthmus. The teratogenic event may also be responsible for the

increased occurrence of related congenital anomalies and conditions associated with horseshoe kidney.

Numerous agents have been implicated as renal teratogens in animal studies such as Vitamin A. Other experiments suggest ethanol is a teratogenic for the urinary tract later in gestation and this also induces premature cell death. In humans, glucose (a mother with diabetes) and angiotensin a converting enzyme inhibitors used for maternal hypertension are recognized as renal teratogens as well.

Anatomy/Positioning

increased occurrence of related congenital anomalies and conditions associated with horseshoe kidney. Numerous agents have been

Horseshoe kidney occurs during fetal development as the kidneys move into their normal position in the flank area around the side, just above the waist. As the kidneys of the fetus rise from the pelvic area they fuse together forming a "U" shape, hence the name and the appearance of a "horseshoe".Technically, the term horseshoe is reserved for cases in which most of each kidney lies on one side of the spine. It includes symmetric horseshoe kidney (midline fusion) or asymmetric horseshoe kidney (L- shaped kidney). They may be positioned lower than normal because the isthmus is hindered by the inferior mesenteric artery during renal ascent. The isthmus usually lies anterior to the great vessels at the level of the third to fifth lumbar vertebrae.

In less than 90% of cases, fusion in horseshoe kidney occurs along the lower pole. This region of fusion, called the isthmus, is usually composed of renal parenchymal tissue. However, in many instances, it may consist of fibrous tissue. The ureters usually pass anterior to the isthmus, and they may have a high insertion point in the renal pelvis.

In the midline fusion anomaly, the kidneys are symmetric, with each of the lower poles of the kidneys uniting at the midline. In the lateral fusion anomaly, one kidney is more

vertical, while the other kidney is more horizontal; the isthmus lies slightly toward one side. In rare cases, the upper poles fuse, reversing the horseshoe appearance.

Symptoms

  • Asymptomatic

  • Urinary Tract Infections

  • Kidney Stones

  • Hematuria

  • Vague abdominal pain

  • Palpable lump in the abdomen

  • Severe pain attributed to hydronephrosis

Testing

  • Intravenous pyelogram (IVP): Intravenous contrast dye is given so structures can be seen on film.

  • Computed tomography (CT) scanning: CT scanning combines special x-ray equipment producing images of internal organs, bone, soft tissue and blood vessels.

  • Magnetic resonance imaging (MRI): a radiology technique that uses magnetism, radio waves, and a computer to produce images of body structures, images.

  • Scintigraphy: a diagnostic test in which a two-dimensional picture of a body radiation source is obtained through the use of radioisotopes.

  • Ultrasonography: a diagnostic used to visualize subcutaneous body structures such as internal organs for possible pathology and to assess blood flow.

  • Angiography: an X-ray exam of the arteries and veins to diagnose blockages and other blood vessel problems.

  • Urine cultures and blood tests: used to determine how well the kidneys function.

Complications  Infections (Urinary and General)  Reflux  Stone Disease  Tumors (Benign and Malignant)

Complications

  • Infections (Urinary and General)

  • Reflux

  • Stone Disease

  • Tumors (Benign and Malignant)

  • Trauma

Treatment

There is no cure for horseshoe kidneys. Treatment is usually focused toward the accompanying diseases (ex. urinary tract infection and hydronephrosis). Most people have no loss of regular kidney function and require no treatment. Antibiotics may be used to treat underlying infections; however, occasionally surgery is necessary only when extreme complications such as failure to respond to antibiotics or threats of poor blood supply take place.

Transplants

Because of the continuing organ shortage, kidneys with atypical anatomy are frequently considered for transplantation. These kidneys are frequently accompanied by vascular and urinary tract abnormalities and there has always been a concern for primary nonfunction based on technical failure. Because of the technical challenges involved in transplanting this type of kidney and their low frequency, some surgeons seem to be reluctant to use horseshoe kidneys for transplantation. Horseshoe kidneys can be

transplanted into a single recipient in tact, or divided to benefit two patients. Research has reported technical and enduring effectiveness with transplant of horseshoe kidneys.

Frekuensi

Ginjal tapal kuda terjadi sekitar 1 per 500 kelahiran hidup dan tampaknya dua kali lebih umum pada laki-laki seperti pada wanita. Belum ada faktor genetik diketahui, namun telah dilaporkan pada kembar identik dan saudara kandung dalam keluarga yang sama. Meskipun hadir pada saat lahir ginjal tapal kuda biasanya tidak menimbulkan tanda-tanda atau gejala dan sering tidak terdeteksi selama bertahun-tahun. Ada kemungkinan bahwa selama pemeriksaan fisik bisa hadir sebagai massa perut garis tengah yang lebih rendah, tetapi rata-rata itu diidentifikasi selama pemeriksaan radiologi.

Etiologi

Ada dua teori yang telah diajukan mengenai embriogenesis ginjal tapal kuda. Ajaran klasik fusi mekanik menyatakan bahwa ginjal tapal kuda terbentuk selama organogenesis ketika kutub inferior ginjal awal datang dalam kontak sekering di garis tengah yang lebih rendah. Ini fusi mekanik tampaknya berlaku untuk ginjal tapal kuda dengan isthmus berserat.

Penjelasan untuk fusi lateral adalah bahwa, selama hidup embrio awal, fleksi lateral tulang belakang lumbo-sacral dapat mendorong salah satu ginjal berkembang ke arah garis tengah dan dapat menyebabkan fusi asimetris. Di kemudian hari embrio, pendakian dari ginjal menyatu terhalang oleh arteri mesenterika inferior, dengan tanah genting dari ginjal tapal kuda menjadi terperangkap di bawahnya. Akibatnya, ginjal tapal kuda selalu berada pada posisi yang lebih rendah dari normal.

Kajian yang lebih mutakhir telah membuktikan teori kedua fusi abnormal jaringan terkait, dengan genting parenchymatous. Beberapa ginjal tapal kuda adalah hasil dari peristiwa teratogenik melibatkan migrasi abnormal sel nephrogenic posterior yang kemudian bersatu untuk membentuk tanah genting. Acara teratogenik mungkin juga bertanggung jawab atas terjadinya peningkatan anomali kongenital terkait dan kondisi yang berhubungan dengan ginjal tapal kuda.

Sejumlah agen telah terlibat sebagai teratogen ginjal dalam studi hewan seperti eksperimen Vitamin A. lain menunjukkan etanol adalah teratogenik untuk saluran kemih kemudian dalam kehamilan dan ini juga menginduksi kematian sel prematur. Pada manusia, glukosa (seorang ibu dengan diabetes) dan angiotensin converting enzyme inhibitor suatu digunakan untuk hipertensi ibu diakui sebagai teratogen ginjal juga.

Anatomi / Positioning

Ginjal tapal kuda terjadi selama perkembangan janin ginjal pindah ke posisi normal mereka di daerah panggul sekitar sisi, tepat di atas pinggang. Sebagai

ginjal dari kenaikan janin dari daerah panggul mereka sekering bersama membentuk "U" bentuk, maka nama dan penampilan dari "tapal kuda". Secara teknis, tapal kuda istilah dicadangkan untuk kasus-kasus di mana sebagian besar dari setiap ginjal terletak pada satu sisi tulang belakang. Ini mencakup ginjal tapal kuda simetris (garis tengah fusi) atau ginjal tapal kuda asimetris (L- berbentuk ginjal). Mereka dapat diposisikan lebih rendah dari normal karena tanah genting terhalang oleh arteri mesenterika inferior selama pendakian ginjal. Tanah genting biasanya terletak anterior ke pembuluh besar di tingkat vertebra lumbalis ketiga untuk kelima.

Dalam waktu kurang dari 90% dari kasus, fusi di ginjal tapal kuda terjadi di sepanjang tiang yang lebih rendah. Ini daerah fusi, yang disebut tanah genting, biasanya terdiri dari jaringan parenkim ginjal. Namun, dalam banyak kasus, mungkin terdiri dari jaringan fibrosa. Ureter biasanya melewati anterior ke tanah genting, dan mereka mungkin memiliki titik penyisipan tinggi dalam pelvis ginjal.

Dalam anomali fusi garis tengah, ginjal yang simetris, dengan masing-masing kutub bawah ginjal menyatukan di garis tengah. Dalam anomali fusi lateral, satu ginjal lebih vertikal, sedangkan ginjal lainnya lebih horizontal, tanah genting terletak sedikit ke arah satu sisi. Dalam kasus yang jarang terjadi, sekering tiang atas, membalikkan penampilan tapal kuda.

Gejala

Asimtomatik • • Infeksi Saluran Kemih • Batu Ginjal • Hematuria • sakit perut Vague • teraba benjolan di perut

• Nyeri berat dikaitkan dengan hidronefrosis

Pengujian

• pyelogram intravena (IVP): pewarna kontras intravena diberikan sehingga

struktur dapat dilihat pada film.

• Computed tomography (CT) scan: CT scan menggabungkan khusus x-ray peralatan menghasilkan gambar dari organ, tulang, jaringan lunak dan pembuluh darah.

• Magnetic resonance imaging (MRI): teknik radiologi yang menggunakan

magnet, gelombang radio, dan komputer untuk menghasilkan gambar struktur tubuh, gambar.

• Skintigrafi: tes diagnostik di mana gambar dua dimensi dari sumber radiasi

benda diperoleh melalui penggunaan radioisotop.

• Ultrasonografi: diagnostik yang digunakan untuk memvisualisasikan struktur

tubuh subkutan seperti organ internal untuk patologi mungkin dan untuk

menilai aliran darah.

• Angiografi: ujian X-ray dari arteri dan vena untuk mendiagnosa penyumbatan darah dan masalah lainnya kapal.

• Urine kultur dan tes darah: digunakan untuk menentukan seberapa baik fungsi

ginjal.

Komplikasi

• Infeksi (Kemih dan Umum) • Reflux

• Penyakit Batu • Tumor (jinak dan ganas) • Trauma

Pengobatan

Tidak ada obat untuk ginjal tapal kuda. Pengobatan biasanya difokuskan terhadap penyakit penyerta (infeksi saluran kemih ex. dan hidronefrosis). Kebanyakan orang tidak kehilangan fungsi ginjal secara teratur dan tidak memerlukan pengobatan. Antibiotik dapat digunakan untuk mengobati infeksi yang mendasari, namun, kadang-kadang diperlukan operasi hanya bila komplikasi ekstrim seperti kegagalan untuk merespon terhadap antibiotik atau ancaman suplai darah yang buruk terjadi.

Transplantasi

Karena kekurangan organ terus, ginjal dengan anatomi atipikal sering dipertimbangkan untuk transplantasi. Ini ginjal sering disertai dengan kelainan saluran pembuluh darah dan urin dan ada selalu menjadi perhatian bagi nonfunction primer berdasarkan pada kegagalan teknis. Karena tantangan teknis yang terlibat dalam transplantasi jenis ini ginjal dan frekuensi rendah, beberapa ahli bedah tampaknya enggan untuk menggunakan ginjal tapal kuda untuk transplantasi. Ginjal tapal kuda dapat dipindahkan ke satu penerima dalam bijaksana, atau dibagi dua untuk menguntungkan pasien. Penelitian telah melaporkan efektivitas teknis dan abadi dengan transplantasi ginjal tapal kuda.