ATRESIA ANI Etiologi Kelainan bawaan anus yang disebabkan oleh gangguan pertumbuhan, fusi dan pembentukan anus

dari tonjolan embriogenik. Pada kelainan bawaan anus umumnya tidak ada kelainan rektum, sfingter dan otot dasar panggul. Namun demikian pada agenesis anus, sfingter internus mungkin tidak memadai. Kelainan bawaan rektum terjadi karena gangguan pemisahan kloaka menjadi rektum dan sinus urogenital sehingga biasanya disertai dengan gangguan perkembangan septum urorektal yang memisahkannya. Dalam hal ini terjadi fistula antara saluran kemih dan saluran genital. Atresia ani (imperforate anus) Penatalaksanaan atresia ani dilakukan sesuai dengan letak ujung atresia terhadap otot panggul. Untuk itu dibuat pembagian sebagai berikut: Atresia ani letak rendah (translevator) Rektum menembus m. Levator anus sehingga jarak antara kulit dan ujung rektum paling jauh 1 cm. Dapat berupa stenosis anus yang hanya membutuhkan dilatasi membran atau merupakan membran anus tipis yang dapat dibuka segera setelah anak lahir. Agenesis anus yang disertai fistula perineum juga dapat ditangani segera setelah anak lahir. Atresia ani letak tinggi (supralevator) Rektum tidak mencapai m. Levator anus, dengan jarak antara ujung buntu rektum sampai kulit perineum lebih dari 1 cm. Biasanya disertai dengan fistula kesaluran kencing atau genital. Untuk menentukan golongan malformasi anorektal digunakan cara invertogram. Invertogram adalah teknik pengambilan foto untuk menilai jarak puntung distal rektum terhadap marka anus dikulit peritonium. Pada teknik bayi diletakkan terbalik (kepala dibawah) atau tidur terlungkup (prone), dengan sinar horizontal diarahkan ke trohanter mayor. Dinilai ujung udara yang ada didistal rektum ke marka anus. Klasifikasi (Wingspread 1981) Penggolongan anatomis malformasi anorektal: Laki laki Golongan I: Tindakan: 1. Fistel urine Kolostomi neonatus pada usia 2. Atresia rekti 4 6 bulan 3. Perineum datar 4. Tanpa fistel udara> 1cm dari kulit pada invertogram Golongan II: Tindakan: 1. Fistel perineum Operasi definitif neonatus tanpa

2. Membran anal kolonostomi 3. Stenosis ani 4. Bucket handle 5. Tanpa fistel, udara 1cm dari kulit pada invertogram Golongan II: Tindakan: 1. Fistel perineum Operasi definitif neonatus tanpa 2. Stenosis ani kolonostomi 3. Tanpa fistel, udara 1 cm dari kulit pada invertogram. Tidak ada evakuasi sehingga perlu segera dilakukan kolostomi. Golongan II 1. Fistel perineum Terdapat lubang antara vulva dan tempat dimana lokasi anus normal. Dapat berbentuk anus anterior, tulang anus tampak normal, tetapi marka anus yang rapat ada diposteriornya. Umumnya menimbulkan obstipasi. 2. Stenosis ani Lubang anus terletak dilokasi normal, tetapi sangat sempit. Evakuasi feses tidak lancar sebaiknya cepat dilakukan operasi definitif. 3. Tanpa fistel Udara > 1 cm dari kulit pada invertogram. Tidak ada evakuasi sehingga perlu segera dilakukan kolostomi. b. Laki laki Perlu diperhatikan hal hal sebagai berikut: - Perineum : bentuk dan adanya fistel - Urine : dicari ada tidaknya butir butir mekonium diurin Golongan I 1. Fistel urine Tampak mekonium keluar dari orificium urethra eksternum. Fistula dapat terjadi keuretra maupun vesika urinaria. Cara praktis untuk membedakan lokasi fistel ialah dengan memasang kateter urine. Bila kateter terpasang dan urine jernih, berarti fistel terletak diuretra yang terhalang kateter. Bila kateter urine mengandung mekonium, berarti fistel kevesika urinaria. Evakuasi feses tidak lancar dan penderita memerlukan kolostomi segara. 2. Atresia rekti Kelainan dimana anus tampak normal, tetapi pada pemeriksaan colok dubur jari tidak dapat masuk lebih dari 1 2 cm, sehingga tidak ada evakuasi feses sehingga perlu segera dilakukan kolostomi. 3. Perineum datar Menunjukkan bahwa otot yang berfungsi untuk kontinensi tidak terbentuk sempurna.

4. Tanpa fistel Udara > 1 cm dari kulit pada invertogram. Tidak ada evakuasi sehingga perlu segera dilakukan kolostomi. Golongan II 1. Fistel perineum Sama dengan wanita 2. Membran anal Anus tertutup selaput tipis dan sering tampak bayangan jalan mekonium dibawah kulit. Evakuasi feses tidak ada. Perlu secepatnya dilakukan terapi definitif. 3. Stenosis ani Lubang anus terletak dilokasi normal, tetapi sangat sempit. Evakuasi feses tidak lancar sebaiknya cepat dilakukan operasi definitif. 4. Bucket handle Daerah lokasi anus normal tertutup kulit yang berbentuk gagang ember. Evakuasi feses tidak ada. Perlu secepatnya dilakukan terapi definitif. 5. Tanpa fistel Udara > 1 cm dari kulit pada invertogram. Tidak ada evakuasi sehingga perlu segera dilakukan kolostomi. Pada 10 20% penderita fistula harus dilakukan pemeriksaan radiologis invertogram. Penatalaksanaan Prinsip pengobatan operatif pada malformasi anorektal dengan tindakan bedah yang disebutkan diseksi postero sagital atau plastik anorektal posterosagital. Kolostomi merupakan perlindungan sementara. Ada dua tempat kolostomi yang dianjurkan dipakai pada neonatus dan bayi yaitu transversokolostomi (kolostomi dikolon transversum) dan sigmoidostomi (kolostomi disigmoid). Bentuk kolostomi yang mudah dan aman adalah stoma laras ganda (Double barrel). Teknik operatif definitif (Posterior Sagital Ano-Rekto-Plasti) Prinsip operasi: 1. Bayi diletakkan tengkurap 2. Sayatan dilakukan diperineum pada garis tengah, mulai dari ujung koksigeus sampai batas anterior marka anus. 3. Tetap bekerja digaris tengah untuk mencegah merusak saraf. 4. Ahli bedah harus memperhatikan preservasi seluruh otot dasar panggul. 5. Tidak menimbulkan trauma struktur lain. Prognosis 1. Dengan menggunakan kalsifikasi diatas dapat dievaluasi fungsi klinis: a. Kontrol feses dan kebiasaan buang air besar b. Sensibilitas rektum c. Kekuatan kontraksi otot sfingter pada colok dubur

2. Evaluasi psikologis Fungsi kontinensia tidak hanya tergantung pada kekuatan sfingter atau sensasi saja, tetapi tergantung juga pada bantuan orang tua dan kooperasi serta keadaan mental penderita. DAFTAR PUSTAKA Sjamsuhidayat R (2000), Anorektum, Buku Ajar Bedah, Edisi revisi, EGC, Jakarta, hal 901 908. Moritz M.Z (2003), Operative Pediatric Surgery, Mc. Grow Hill Professional, United State. Lawrence W (2003), Anorectal Anomalies, Current Diagnosis & Treatment, edisi 11, Mc. Graw Hill Professional, United States, hal 1324 1327. Reksoprodjo S, Malformasi Anorektal, Kumpulan Ilmu Bedah, FKUI, Jakarta hal 134 139

Atresia Ani
Posted on Februari 23, 2010 by ilmubedah

Atresia Ani adalah kelainan kongenital yang dikenal sebagai anus imperforate meliputi anus, rectum atau keduanya (Betz. Ed 3 tahun 2002) Atresia ani atau anus imperforate adalah tidak terjadinya perforasi membran yang memisahkan bagian entoderm mengakibatkan pembentukan lubang anus yang tidak sempurna. Anus tampak rata atau sedikit cekung ke dalam atau kadang berbentuk anus namun tidak berhubungan langsung dengan rectum. Atresia Ani merupakan kelainan bawaan (kongenital), tidak adanya lubang atau saluran anus (Donna L. Wong, 520 : 2003). Atresia berasal dari bahasa Yunani, a artinya tidak ada, trepis artinya nutrisi atau makanan. Dalam istilah kedokteran atresia itu sendiri adalah keadaan tidak adanya atau tertutupnya lubang badan normal atau organ tubular secara kongenital disebut juga clausura. Dengan kata lain tidak adanya lubang di tempat yang seharusnya berlubang atau buntunya saluran atau rongga tubuh, hal ini bisa terjadi karena bawaan sejak lahir atau terjadi kemudian karena proses penyakit yang mengenai saluran itu. Atresia dapat terjadi pada seluruh saluran tubuh, misalnya atresia ani. Atresia ani yaitu tidak berlubangnya dubur. Atresia ani memiliki nama lain yaitu anus imperforata. Jika atresia terjadi maka hampir selalu memerlukan tindakan operasi untuk membuat saluran seperti keadaan normalnya Menurut Ladd dan Gross (1966) anus imperforata dalam 4 golongan, yaitu: 1. Stenosis rektum yang lebih rendah atau pada anus 2. Membran anus yang menetap 3. Anus imperforata dan ujung rektum yang buntu terletak pada bermacammacam jarak dari peritoneum 4. Lubang anus yang terpisah dengan ujung 2. Etiologi Atresia dapat disebabkan oleh beberapa faktor, antara lain: 1. Putusnya saluran pencernaan dari atas dengan daerah dubur sehingga bayi lahir tanpa lubang dubur 2. Kegagalan pertumbuhan saat bayi dalam kandungan berusia 12 minggu/3 bulan

3. Adanya gangguan atau berhentinya perkembangan embriologik didaerah usus, rektum bagian distal serta traktus urogenitalis, yang terjadi antara minggu keempat sampai keenam usia kehamilan. 3. Patofisiologi Atresia ani atau anus imperforate dapat disebabkan karena : 1) Kelainan ini terjadi karena kegagalan pembentukan septum urorektal secara komplit karena gangguan pertumbuhan, fusi atau pembentukan anus dari tonjolan embrionik 2) Putusnya saluran pencernaan dari atas dengan daerah dubur, sehingga bayi lahir tanpa lubang dubur 3) Gangguan organogenesis dalam kandungan penyebab atresia ani, karena ada kegagalan pertumbuhan saat bayi dalam kandungan berusia 12 minggu atau tiga bulan 4) Berkaitan dengan sindrom down 5) Atresia ani adalah suatu kelainan bawaan

What is Hirschsprung's disease?

Hirschsprung's (HURSH-sprungz) disease, or HD, is a disease of the large intestine. The large intestine is also sometimes called the colon. The word bowel can refer to the large and small intestines. Hirschsprung's disease usually occurs in children. It causes constipation, which means that bowel movements are difficult. Some children with Hirschsprung's disease can't have bowel movements at all. The stool creates a blockage in the intestine. If Hirschsprung's disease is not treated, stool can fill up the large intestine. This can cause serious problems like infection, bursting of the colon, and even death. Most parents feel frightened when they learn that their child has a serious disease. This information will help you understand Hirschsprung's disease and how you and the doctor can help your child.

Why does Hirschsprung's disease cause constipation?

Normally, muscles in the intestine push stool to the anus, where stool leaves the body. Special nerve cells in the intestine, called ganglion cells, make the muscles push. A person with Hirschsprung's disease does not have these nerve cells in the last part of the large intestine. Healthy large intestine: Nerve cells are found throughout the intestine.

Hirschsprung's disease large intestine: Nerve cells are missing from the last part of the intestine.

In a person with Hirschsprung's disease, the healthy muscles of the intestine push the stool until it reaches the part without the nerve cells. At this point, the stool stops moving. New stool then begins to stack up behind it. Sometimes the ganglion cells are missing from the whole large intestine and even parts of the small intestine before it. When the diseased section reaches to or includes the small intestine, it is called long-segment disease. When the diseased section includes only part of the large intestine, it is called short-segment disease.

What causes Hirschsprung's disease?

Hirschsprung's disease develops before a child is born. Normally, nerve cells grow in the baby's intestine soon after the baby begins to grow in the womb. These nerve cells grow down from the top of the intestine all the way to the anus. With Hirschsprung's disease, the nerve cells stop growing before they reach the end. No one knows why the nerve cells stop growing. But we do know that it's not the mother's fault. Hirschsprung's disease isn't caused by anything the mother did while she was pregnant.

Some children with Hirschsprung's disease have other health problems, such as Down's syndrome and other rare disorders.

If I have more children, will they have Hirschsprung's disease too?

In some cases, Hirschsprung's disease is hereditary, which means mothers and fathers could pass it to their children. This can happen even if the parents don't have Hirschsprung's disease. If you have one child with Hirschsprung's disease, you could have more children with the disease. Talk to your doctor about the risks.

What are the symptoms of Hirschsprung's disease??

Symptoms of Hirschsprung's disease usually show up in very young children. But sometimes they don't appear until the person is a teenager or an adult. The symptoms are a little different for different ages. Symptoms in Newborns Newborns with Hirschsprung's disease don't have their first bowel movement when they should. These babies may also throw up a green liquid called bileafter eating and their abdomens may swell. Discomfort from gas or constipation might make them fussy. Sometimes, babies with Hirschsprung's disease develop infections in their intestines. Symptoms in Young Children Most children with Hirschsprung's disease have always had severe problems with constipation. Some also have more diarrhea than usual. Children with Hirschsprung's disease might also have anemia, a shortage of red blood cells, because blood is lost in the stool. Also, many babies with Hirschsprung's disease grow and develop more slowly than they should. Symptoms in Teenagers and Adults Like younger children, teenagers and adults with Hirschsprung's disease usually have had severe constipation all their lives. They might also have anemia. Those with anemia look pale and tire easily

How does the doctor find out if Hirschsprung's disease is the problem?
To find out if a person has Hirschsprung's disease, the doctor will do one or more tests:

barium enema (BAR-ee-um EN-uh-muh) x ray manometry (ma-NOM-eh-tree) biopsy (BY-op-see)

Barium Enema X Ray An x ray is a black-and-white picture of the inside of the body. The picture is taken with a special machine that uses a small amount of radiation. For a barium enema x ray, the doctor puts barium

through the anus into the intestine before taking the picture. Barium is a liquid that makes the intestine show up better on the x ray. In some cases, instead of barium another liquid, called Gastrografin, may be used. Gastrografin is also sometimes used in newborns to help remove a hard first stool. Gastrografin causes water to be pulled into the intestine, and the extra water softens the stool. In places where the nerve cells are missing, the intestine looks too narrow. If a narrow large intestine shows on the x ray, the doctor knows Hirschsprung's disease might be the problem. More tests will help the doctor know for sure. Other tests to diagnose Hirschsprung's disease are manometry and biopsy: Manometry The doctor inflates a small balloon inside the rectum. Normally, the analmuscle will relax. If it doesn't, Hirschsprung's disease may be the problem. This test is most often done in older children and adults. Biopsy This is the most accurate test for Hirschsprung's disease. The doctor removes and looks at a tiny piece of the intestine under a microscope. If the nerve cells are missing, Hirschsprung's disease is the problem. The doctor may do one or all of these tests. It depends on the child.

What is the treatment for Hirschsprung's disease??

Pull-through Surgery Hirschsprung's disease is treated with surgery. The surgery is called a pull-through operation. There are three common ways to do a pull-through, and they are called the Swenson, the Soave, and the Duhamel procedures. Each is done a little differently, but all involve taking out the part of the intestine that doesn't work and connecting the healthy part that's left to the anus. After pull-through surgery, the child has a working intestine. Before surgery: The diseased section is the part of the intestine that doesn't work.

Step 1: The doctor removes the diseased section.

Step 2: The healthy section is attached to the rectum or anus.

Colostomy and Ileostomy Often, the pull-through can be done right after the diagnosis. However, children who have been very sick may first need surgery called an ostomy. This surgery helps the child get healthy before having the pull-through. Some doctors do an ostomy in every child before doing the pull-through. In an ostomy, the doctor takes out the diseased part of the intestine. Then the doctor cuts a small hole in the baby's abdomen. The hole is called astoma. The doctor connects the top part of the intestine to the stoma. Stool leaves the body through the stoma while the bottom part of the intestine heals. Stool goes into a bag attached to the skin around the stoma. You will need to empty this bag several times a day. Step 1: The doctor takes out most of the diseased part of the intestine.

Step 2: The doctor attaches the healthy part of the intestine to the stoma (a hole in the abdomen).

If the doctor removes the entire large intestine and connects the small intestine to the stoma, the surgery is called an ileostomy. If the doctor leaves part of the large intestine and connects that to the stoma, the surgery is called a colostomy. Later, the doctor will do the pull-through. The doctor disconnects the intestine from the stoma and attaches it just above the anus. The stoma isn't needed any more, so the doctor either sews it up during surgery or waits about 6 weeks to make sure that the pull-through worked.

What will my child's life be like after surgery?

Ostomy Most babies are more comfortable after having an ostomy because they can pass gas more easily and aren't constipated anymore. Older children will be more comfortable, too, but they may have some trouble getting used to an ostomy. They will need to learn how to take care of the stoma and how to change the bag that collects stool. They may be worried about being different from their friends. Most children can lead a normal life after surgery. Nurses at the hospital can teach you and your child how to care for a stoma and can talk to you about your worries.

Adjusting After Pull-through After a pull-through, 9 out of 10 children pass stool normally. Some children may have diarrhea for a while, and babies may develop a nasty diaper rash. Eventually the stool will become more solid and the child will need to go to the bathroom less often. Toilet training may be delayed, as the child learns how to use the bottom muscles only after pull-through surgery. Older children might stain their underwear for a while after the surgery. It is not their fault. They can't control this problem, but it improves with time. Some children become constipated because 1 in 10 children with Hirschsprung's disease has difficulty moving stool through the part of the colon without nerve cells. A mild laxative may also be helpful. Ask your doctor for suggestions. Diet and Nutrition Drinking plenty of liquids is important after surgery for Hirschsprung's disease. One job of the large intestine is to collect the water and salts the body needs. Since your child's intestine is shorter now, it absorbs less. Your child will need to drink more to make sure his body gets enough fluids. An infant who has long-segment disease requiring an ileostomy may need special tube feedings. The shortened intestine does not allow the bloodstream enough time to absorb nutrients from food before it is pushed out of the body as stool. Tube feedings that deliver nutrients can make up for what is lost. Eating high-fiber foods like cereal and bran muffins can help reduce constipation and diarrhea. Infection Infections can be very dangerous for a child with Hirschsprung's disease. Infection of the large and small intestines is called enterocolitis. It can happen before or after surgery to treat Hirschsprung's disease. Here are some of the signs to look for:

fever swollen abdomen vomiting diarrhea bleeding from the rectum sluggishness

Call your doctor immediately if your child shows any of these signs. If the problem is enterocolitis, your child may be admitted to the hospital. In the hospital, an intravenous (I.V.) line may be needed to keep body fluids up and to deliver antibiotics to fight the infection. The large

intestine will be rinsed regularly with a mild salt water solution until all remaining stool has been removed. The rinse may also contain antibiotics to kill bacteria. When the child has recovered from the infection, the doctor may advise surgery. If the child has not had the pull-through surgery yet, the doctor may prepare for it by doing a (colostomy or ileostomy before the child leaves the hospital. If the child has already had a pull-through operation, the doctor may correct the obstruction with surgery. Enterocolitis can be life threatening, so watch for the signs and call your doctor immediately if they occur. Long-segment Hirschsprung's disease Sometimes Hirschsprung's disease affects most or all of the large intestine, plus some of the small intestine. Children with long-segment Hirschsprung's disease can be treated with pull-through surgery, but there is a risk of complications such as infection, diarrhea, and diaper rash afterward. Parents need to pay close attention to their child's health. Also, since some, most, or all of the intestine is removed, drinking a lot of fluid is important.

Hirschsprung's Disease At A Glance

Hirschsprung's disease is a disease of the large intestine. Hirschsprung's disease develops in children before they are born. It is not caused by anything the mother did while pregnant. Symptoms of Hirschsprung's disease include
o o o o o
delayed first bowel movement in newborns swollen abdomen and vomiting constipation since birth slow growth and development anemia

Children with Hirschsprung's disease may get an infection, called enterocolitis, which can cause fever and diarrhea. Hirschsprung's disease is a serious disease that needs to be treated right away. Hirschsprung's disease is treated with pull-through surgery or, sometimes, ostomy. After treatment, most children with Hirschsprung's disease lead normal lives.

The skin is the largest organ of the body, with a total area of about 20 square feet. The skin protects us from microbes and the elements, helps regulate body temperature, and permits the sensations of touch, heat, and cold. Skin has three layers:

The epidermis, the outermost layer of skin, provides a waterproof barrier and creates our skin tone. The dermis, beneath the epidermis, contains tough connective tissue, hair follicles, and sweat glands. The deeper subcutaneous tissue (hypodermis) is made of fat and connective tissue.

The skin's color is created by special cells called melanocytes, which produce the pigment melanin. Melanocytes are located in the epidermis

The vagina is an elastic, muscular canal with a soft, flexible lining that provides lubrication and sensation. The vagina connects the uterus to the outside world. The vulva and labia form the entrance, and the cervixof the uterus protrudes into the vagina, forming the interior end. The vagina receives the penis during sexual intercourse and also serves as a conduit for menstrual flow from the uterus. During childbirth, the baby passes through the vagina (birth canal). The hymen is a thin membrane of tissue that surrounds and narrows the vaginal opening. It may be torn or ruptured by sexual activity or by exercise. The urinary bladder is a muscular sac in the pelvis, just above and behind the pubic bone. When empty, the bladder is about the size and shape of a pear. Urine is made in the kidneys, and travels down two tubes called ureters to the bladder. The bladder stores urine, allowing urination to be infrequent and voluntary. The bladder is lined by layers of muscle tissue that stretch to accommodate urine. The normal capacity of the bladder is 400 to 600 mL. During urination, the bladder muscles contract, and two sphincters (valves) open to allow urine to flow out. Urine exits the bladder into theurethra, which carries urine out of the body. Because it passes through the penis, the urethra is longer in men (8 inches) than in women (1.5 inches). The abdomen (commonly called the belly) is the body space between the thorax (chest) and pelvis. The diaphragm forms the upper surface of the abdomen. At the level of the pelvic bones, the abdomen ends and the pelvis begins. The abdomen contains all the digestive organs, including the stomach, small and large intestines, pancreas, liver, and gallbladder. These organs are held together loosely by connecting tissues (mesentery) that allow them to expand and to slide against each other. The abdomen also contains the kidneys and spleen. Many important blood vessels travel through the abdomen, including the aorta, inferior vena cava, and dozens of their smaller branches. In the front, the abdomen is protected by a thin, tough layer of tissue called fascia. In front of the fascia are the abdominal muscles and skin. In the rear of the abdomen are the back muscles.