1. Anorectal malformations Dr.Bassam Alabbasi

2. 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.

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

4. :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.

5. 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)

6. International classification

8. 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.

9. 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.

10. 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.

11. 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).

12. 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.

13. 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.

14. 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.

17. - 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.

18. 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)

19. Any questions