Anorectal malformations Dr.Bassam Alabbasi

Introduction: -Assurbanipal library: (when a woman bears a child whose anus is closed then the whole land will suffer the want of food). - becoming less common. -imperforate anus wrong name ? Most have fistulous connection. - Correction is difficult :- 1- loss of relation to sphincter. 2- abnormal muscle development. 3- abnormal nerve supply.

Incidence:- - About 1-3000 to 1-5000 live birth. - Males affected slightly more than female. -high lesion =male/low lesion =female - Most cases sporadically. - Risk of inheritance only 1%.

:Classification Different sub type classified according to:- 1-relation of terminal bowel to pelvic floor muscle(levator ani). 2- presence or absence of fistula(skin or uro genital). 3- conditions needs colostomy , others not need.

1- High type : blind end rectum above levator ani muscle a-with fistula to -bladder or urethra in male -vagina in female. b-without fistula(anorectal agenesis or atresia). 2-low type: blind end rectum below levator ani muscle. a-with fistula to- skin (cutaneous fistula) - vestibule. b-without fistula (anal agenesis) 3- cloaca in female(rectum ,urethra and vagina in one opening or channal called urogenital sinus)

International classification

Associated anomalies:- - Carful system review(very important) . More than 60% of cases have second anomalies. : Genito urinary anomalies -50% of cases and its include:- 1-neuropathic bladder. 2-vesicoureteral reflux. 3-ureterocel. 4-ureteric duplication. 5-renal agenesis. 6-bladder or cloacal exstophy. 7-miscelaneous.

Cardiovascular anomalies:- Skeletal anomalies:- -20% of cases:- 1- vertebral. 2-spinal dysraphism. 3-sacral anomalies. 4-spinal cord and pelvic nerve dysfunction. 5- pelvic floor dusfunction. Cardiovascular anomalies:- -12% of cases and it may be life threatening:- 1- V.S.D. 2-A.S.D. 3-A-V Canal. 4-Right aortic arch.

Gastro intestinal anomalies:- - 10% of cases:- 1-Congenital cystic colon(C.C.C.). 2-Dudenal Artesia. 3-esophageal Artesia. 4-intestinal artesian. Syndromes or associations:- 1- Down syndrome. 2- V.A.C.T.E.R.L. association. 3- Currarino triade.

Clinical presentations -intestinal obstruction. -simple inspection(diagnostic)/level(difficult). - Condition of anal dimple(well formed=low/flat=high). -voiding of meconium per urethra(in male) high type. - sometime cutaneous fistula filled with meconium low type. -in female carful genital examination essential (no.of opening). 3 opening: suggest vestibular fistula. 2 opening : suggest agenesis. 1 opening : diagnostic for cloacae with common channel(urogenital sinus).

Radiology Determined: 1-relation of rectum to sphincter muscle. 2-associated anomalies. 1-x-ray of the spine and chest (sacral , VATER). 2-Lateral invertogram ( pubococcegeal line) 18-24hr.after birth. -gas shadow above this line suggest high type. - gas shadow below suggest low type. 3-lateral decubitus X-ray. 4-M.R.I. 5- ultrasound. 6-Echocardiography. 7- M.C.U.

TREATMENT AND PROGNOSIS Satisfactory results=efficient continence. general considerations: 1-perineal fistula=low lesion = good prognosis. 2-meconium in urine (in male) = high type = colostomy. 3-in female=search for fistula=mostly local surgery = good prognosis. if no fistula = colostomy. 4-sacral anomalies + neurogenic bladder = poor sphincter action = poor prognosis.

Surgical technique - Colostomy at birth. Low lesion: High lesion: good long term outlook but tendency to constipation - A perineal anoplasty =cutaneous fistula. - anal stenosis &imperforate anal membrane=simple incision &dilatation. -vestibular fistula =transpositioning anoplasty few monthes later. High lesion: - Colostomy at birth. 3-4 month later definitive surgery using posterior sagital anorectoplasty(P.S.A.R.P. pullthrogh) Or penna technique.

- Recently laparoscopic pull through using nerve stimulator to identify anal sphincter , -the sphincter is not well developed and nerve supply is deficient= continence not good. - 2 months later colostomy closed. - Regular anal dilatation.

Complications Early: 1-wound infection. 2- colostomy complications. 3-recto urinary fistula . 4- neurogenic bladder. 5- anal stenosis. 6- rectal mucosal prolaps. Late: 1-constipation. 2-megarectum. 3- soiling (incontinence)

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