Atresia Ani

Pembimbing:
dr. Guntur Surya A, Sp.B, Sp.BA

Hashina Zulfa
Thesar Waldi
Devina R Hapsari
Identitas Pasien
Nama : By. Ny. NN
Jenis Kelamin : Laki-laki
Umur : 1,5 jam
Tgl Lahir : 4 September 2019 pk 23.30
Alamat : Kalitengah, Klaten
Tgl Masuk RS : 5 September 2019 pk 01.00
Keluhan Utama
Tidak memiliki anus
Anamnesis
Riwayat Penyakit Sekarang:
Bayi kiriman BPM dengan keterangan tidak memiliki anus. Demam (-), muntah (-),
kejang (-)

Riwayat Persalinan:
Bayi lahir dari ibu usia 21 th P1A0 UK 35-36 mgg. Bayi lahir secara spontan langsung
menangis pada 4 September 2019 pk 23.30 dengan BB 1450 gram, PB 39 cm, LK 29 cm,
LD 23 cm, LP 21 cm, LLA 7 cm, A/S 8/9
Saat lahir bidan mendapati bayi tidak memiliki anus, tidak tampak adanya BAB, tampak
penis lengket pada kulit dengan adanya lubang di bagian bawah penis. Ibu riwayat
coitus (+) 5 hari yll
Anamnesis
Riwayat Penyakit Keluarga:

- Keluhan serupa disangkal

- Kelainan kongenital atau penyakit bawaan lainnya disangkal
Pemeriksaan Fisik
KU : gerak kurang aktif, menangis kuat Status anal
HR 131 kpm I: tampak anal dimple (+), anus (-), black
RR 55 kpm ribbon (-), fistula perianal (-), feses/
T 36 C meconium (-)
SpO2 98-99% P: NT (-)

Status abdomen Status Urogenital

I: distensi (-) I: tampak batang penis (+) lengket
A: peristaltik (+) ventral dengan skrotum, tampak lubang
P: supel (+) pada ventral penis
P: timpani (+)
Pemeriksaan Fisik
Pemeriksaan Fisik (5/9 pk 04.00)
KU : hipoaktif, menangis tidak kuat Sindrom :
● Kelopak mata kiri masih menyatu
T : 36 C
● High palatum
CNS : S4, letargi ● Dagu kecil
● Hipospadia penoscrotal
CVS : HR 129 kpm, bising (-)
● Anal dimple
Resp: RR 72 kpm, retraksi ringan, O2 ● Sindaktili jari kaki kanan
NCPAP FiO2 30%, PEEP 5,5, SpO2 94%
GIT : supel, BU (+)
GUT : BAK via DC, tampak batang penis
lengket dengan ventral scrotum, lubang
pada ventral penis
Integ: anal dimple (+), fistula perianal (-)
Diagnosis
Atresia ani tanpa fistula

Susp hipospadia penoscrotal

Susp sindrom

BBLSR, KB, SMK, spontan

Manajemen
Awasi KU/VS/acute abdomen
Termoregulasi
IVFD D10% 80cc bolus, 4,8 cc/jam
O2 NCPAP FiO2 30% PEEP 6
Ampicillin (50 mg/kg/12 jam) → 75 mg/12 jam
Gentamisin (4,5 mg/kg/36 jam) → 6,5 mg/36 jam
Pasang OGT No 8 → keluar cairan kekuningan
Pasang DC pada lubang di bawah penis → keluar urin
Lab lengkap, urin rutin, stercobilin urin
Babygram
Knee chest position usia 20-24 jam
Jika letak tinggi → pro TCS (colostomy)
● HMD Grade II
● Konfigurasi cor normal
● Tak tampak kelainan pada
abdomen
● Udara terdistal dari sistema
usus berada di cranial
pubococcygeal line
● Jarak anal dengan udara
sistema usus terdistal 5,5 cm
● Kesan: atresia ani high level
 Nama Hasil Nilai Rujukan
Pemeriksaan

Lekosit 8.1 10^3/uL 5-30.1

Eritrosit 3.99 10^6/uL 3.7-6.6

Pemeriksaan Penunjang Hemoglobin 16.2 g/dL 14.9-23.7

Lab (5/9/2019)
Hematokrit 46.3 % 44-72

MCV 116 fL 80-99

MCH 40.6 pg 27-31

MCHC 35 g/dl 33-37

Trombosit 313 10^3/µL 150-450

RDW 14.9 % 10-15

 Nama Hasil Nilai Rujukan
Pemeriksaan

Neutrofil 79.9 % 40-80

Limfosit 16.6 % 34

Pemeriksaan Penunjang MXD 3.5 % 1-12

Lab (5/9/2019)
RDW 14.9 % 10-15

MPV 7,3

Golongan darah O

Anti HIV (non VCT) NR NR

HBsAg Negatif Negatif

Anti HCV Negatif Negatif

 Nama Hasil Nilai Rujukan
Pemeriksaan

MAKROSKOPIS

Warna Kuning

Pemeriksaan Penunjang Jernih

Urinalisis (5/9/2019)
KIMIA

Protein (-) (-)

Glukosa (-) (-)

Keton (-) (-)

Bilirubin (-) (-)

pH 7.0 5-8

Nitrit (-) (-)

Berat Jenis 1.015

 Nama Hasil Nilai Rujukan
Pemeriksaan

KIMIA

Urobilinogen (-) (-)

Pemeriksaan Penunjang Blood (-) (-)

Urinalisis (5/9/2019)
Leukosit (-) (-)

SEDIMEN

Epitel Positif (2+)

Eritrosit 0

Leukosit 0-1

Silinder 0

Kristal 0

Bakteri (+)
Pembahasan
ATRESIA ANI
Anorectal Malformation
● Anorectal malformations (ARMs) are birth defects in which the anus is
absent or malformed
● Average incidence worldwide is 1 in 4000 to 5000 live births, slight male
preponderance
● Most frequent defect in male pts is anorectal atresia with a rectourethral
fistula, and anorectal atresia with a rectovestibular fistula in females.
● ARM without a fistula → unusual, only occurs in 5% of pts, associated with
Down syndrome
● ARM usually requires immediate surgery to open a passage for feces,
unless a fistula can be relied on, or until corrective surgery takes place
Embryology
● Cloaca formed at 21 days Allantois joining anteriorly and hindgut joining
posteriorly
● Urorectal septum divides cloaca into anterior urogenital cavity & posterior
rectoanal cavity
● Urorectal septum touches the cloacal membrane divides it into anterior
urogenital membrane & posterior anal membrane
● These membranes will breakdown to produce the anterior urogenital &
posterior anal openings
Types (male)
1. Rectoperineal fistula (traditionally known as low defect), a.k.a covered
anus, bucket-handle malformations. Diagnosis is established by perineal
inspection
Types (male)
2. Rectourethral fistula
● Most frequent defect in males
● Fistula may be located at the
lower (bulbar) or the higher
(prostatic) part of the urethra
● Occasionally the infant passes
meconium through the urethra
Types (male)
3. Recto-bladder neck fistula
● Rectum opens into the
bladder neck
● Poor prognosis for bowel
control (anal muscles
frequently are poorly
developed) flat perineum
● Entire pelvis seems to be
underdeveloped
Types (male)
4. Imperforate anus without fistula
● Well-developed sacrum
● Good prognosis in terms of bowel function
● About ½ of the pts with no fistula also have
Down syndrome
5. Rectal atresia
● Extremely unusual
● Lumen of the rectum is totally/partially
interrupted
Types (female)
1. Rectoperineal fistula ~ equivalent to the perineal
fistula described in the male patient
2. Rectovestibular fistula
● The most common defect in girls, has excellent
functional prognosis
● Clinical exam shows normal urethral opening,
normal vagina, with a 3rd hole in the vestibule
(rectovestibular fistula)
Types (female)
3. Imperforate anus without fistula
● Carries the same therapeutic & prognostic
implications as described in male patients
4. Persistent cloaca
● Cloaca: a defect in which the rectum,
vagina, and urinary tract meet and fuse
(creating a single common channel)
● Should be suspected in females with
imperforate anus and small-looking
genitalia
Classification
Classification
Algorithm for the Treatment
Imaging
After 24hrs of birth obtain cross table lateral radiograph, patient is in prone
position
Management
1. Thorough perineal inspection
a. If meconium is visualized evidence of a rectoperineal fistula
b. If there is meconium in the urine → rectourinary fistula
2. During the first 24hrs, newborn should receive IV fluids, antibiotics, and
NG decompression to prevent aspiration
3. Decision to perform colostomy or a primary operation should be made
within the first 24 hours of life
4. The first 24 hours should also be used to rule out serious associated
defects
5. After 24 hours, if there is no meconium on the perineum, we recommend
obtaining a cross-table lateral radiograph with the patient in the prone
position.
Management
•Signs of a perineal fistula (+) → anoplasty should be performed during the
first 48 hours of life, without a protective colostomy

•Invertogram, air in the rectum is located below the coccyx → PSARP

•If rectal gas does not extend beyond the coccyx, or meconium in the urine (+),
or abnormal sacrum, or flat bottom, or cloaca in females → colostomy. Then
perform a PSARP 1-2 months later (if the neonate gains weight appropriately)
Colostomy
● Colostomy → a portion of the large intestine is
removed or bypassed, the remaining portion of
the functioning large intestine (colon) is brought
through the abdominal wall. The objective is to
allow elimination of bowel contents
● There are 3 locations where colostomy can be
made:
1.Ascending
2.Descending
3.Transverse
Colostomy
There are 3 types of stomas that can be created for a colostomy:
1.End-stoma
2.Loop-stoma
3.Loop-end stoma
PSARP (Posterior sagittal anorectoplasty)
•PSARP (posterior sagittal anorectoplasty) is a
technique for the repair of high anorectal
malformations. It is based upon complete exposure
of the anorectal region, whereas the patient is
positioned in a prone jackknife position and the pelvis
elevated.
•Exact location of the anus & the anal muscles
complex was determined using electrical stimulation
Anal Dilatation
● Started 2 weeks after the repair with a dilator
that fits gently into the anus
● Performed 2x daily by the parents, and the size
of the dilator is increased weekly until the rectum
reaches the desired size, which depends on the
patient’s age
● Once the desired size is reached, the colostomy
can be closed
● The frequency of dilatation can be reduced once
there is no resistance using the final dilator size
Evaluation of the outcome
Krickenbeck classification is used to standardize the methodology for evaluation of
outcome of patients with ARM
Terimakasih