ANORECTAL

MALFORMATIONS
NM RIKA TRISMAYANTI SpB,SpBA(K)
ANORECTAL MALFORMATIONS

MALE MALE
INCIDENCE
• Worldwide 1 : 5000 live births
• A genetic predisposition
• Female  Rectovestibular fistula
• Male  Rectourethral fistula
• Down syndrome
Di seluruh dunia 1: 5000 kelahiran hidup
Kecenderungan genetik
Wanita  Fistula rektovestibular
Laki-laki  Fistula rektouretral
Sindrom Down
CLASSIFICATION

MALES FEMALES
• Perineal fistula • Perineal fistula
• Rectourethral fistula • Vestibular fistula
Bulbar • Persistent cloaca
Prostatic ≤ 3 cm common channel
≥ 3 cm common channel
• Rectobladder neck fistula
• Imperforated anus without
• Imperforate anus without fistula fistula
• Rectal atresia • Rectal atresia
• Complex defects • Complex defects
MALES
• Fistula perineum
• Fistula rektouretral
• Yg berhubungan dgn bengkak
• Prostatis
• Fistula leher rectobladder
• Anus imperforate tanpa fistula
• Atresia rektal
• Cacat kompleks
FEMALES
• Fistula perineum
• Fistula vestibular
• Kloaka persisten
• ≤ 3 cm saluran umum
• ≥ 3 cm saluran umum
• Anus yang tidak berlubang
tanpa fistula
• Atresia rektal
• Cacat kompleks
 ASSOCIATED MALFORMATION
• Cardiovascular anomalies : ASD, TF, VSD
• Gastrointestinal anomalies : Tracheoesophageal, duodenal, Hirschprung disease
• Spinal, sacral, and vertebral anomalies : Lumbosacral (hemivertebrae, scoliosis, butterfly
vertebrae, hemisacrum), Spinal (tethered cord, spinal lipomas, syringomyelia,
myelomeningocele)
• Genitourinary anomalies : Vesicoureteric reflux, renal agenesis and dysplasia,
cryptorchidism, hypospadias
• Gynecologic anomalies : hydrocolpos
• VACTERL

• Anomali kardiovaskular: ASD, TF, VSD

• Anomali gastrointestinal: Tracheoesophageal, duodenal, penyakit Hirschprung
• Anomali tulang belakang, sakral, dan vertebral: Lumbosakral (hemivertebrae,
skoliosis, vertebra kupu-kupu, hemisakrum), Tulang belakang (tali pusat, lipoma
tulang belakang, syringomyelia, myelomeningocele)
• Anomali genitourinari: refluks vesikoureterik, agenesis dan displasia ginjal,
kriptorkismus, hipospadia
• Anomali ginekologi: hidrokolpos
• VACTERL
ALGORITHM FOR THE TREATMENT OF
MALE NEWBORN
ALGORITHM FOR THE TREATMENT OF
FEMALE NEWBORN
IMAGING STUDIES

• Babygram
• Invertogram
• Cross-table lateral radiograph
LIMITED PSARP

• Perineal fistula
• Perform in the first 48 hours of life
• In newborns who cannot undergo the procedure because they are ill,
have significant associated anomalies  dilatation (repair in 2-3
months)

• Fistula perineum
• Lakukan dalam 48 jam pertama kehidupan
• Pada bayi baru lahir yang tidak dapat menjalani prosedur karena
sakit, memiliki kelainan terkait yang signifikan  dilatasi (perbaikan
dalam 2-3 bulan)
COLOSTOMY

• A first stage in a newborn with a high anomaly

• Devided colostomy, loop colostomy
• Management after colostomy : high pressure distal colostography
(visualize the true extent of the rectum and the presence of a
rectourinary fistula

• Tahap pertama pada bayi baru lahir dengan anomali tinggi

• Kolostomi terbagi, kolostomi loop
• Penatalaksanaan setelah kolostomi: kolostografi distal tekanan tinggi
(visualisasikan luas sebenarnya dari rektum dan adanya fistula
rektourinari)
 GENERAL PRINCIPLES OF
POSTOPERATIVE CARE
• Oral feedings may begin when the child is awake
• Antibiotics
• In males who had a rectourethral fistula, the urinary catheter should be left for 7 days
• Cloaca repair  urinary catheter left for 2-3 weeks
• A Dilatation program :
2 weeks after surgery
Twice a day
Every week, the size of the dilator is increased by one unit until the desired size is
reached
• Pemberian makanan secara oral dapat dimulai saat anak terjaga
• Antibiotik
• Pada pria yang mengalami fistula rektouretral, kateter urin harus dibiarkan selama
7 hari
• Perbaikan kloaka  kateter urin dibiarkan selama 2-3 minggu
• Program Dilatasi:
2 minggu setelah operasi
Dua kali sehari
Setiap minggu, ukuran dilator bertambah satu unit hingga ukuran yang diinginkan
tercapai
GENERAL PRINCIPLES OF
POSTOPERATIVE CARE
• Once the correct size is reached, the colostomy can be closed, which is usually 8-
12 weeks after the reconstruction
• Dilatations must continue after closure
• Once a day for 1 month, twice a week for a month, and than once a week for 3
month
• Multiple bowel movements + perineal excoration  Constipating Diet  after 3-6
months a more regular bowel movement pattern develops

• Setelah ukuran yang tepat tercapai, kolostomi dapat ditutup, biasanya 8-12
minggu setelah rekonstruksi
• Dilatasi harus berlanjut setelah penutupan
• Sekali sehari selama 1 bulan, dua kali seminggu selama sebulan, dan dari sekali
seminggu selama 3 bulan
• Buang air besar multipel + ekskorasi perineum  Diet sembelit  setelah 3-6
bulan pola buang air besar lebih teratur berkembang
OUTCOMES

• Complication
Wound infection, retraction
Incontinence, stricture, fibrosis
Constipation
• Komplikasi
Infeksi luka, retraksi
Inkontinensia, striktur, fibrosis
Sembelit