She was born with multiple congenital anomalies including anterior abdominal wall defect, bowel herniation, and vestibular anus. (6,7) She had craniofacial manifestations including a 'coarse' face with a flat profile, high forehead with temporal-frontal balding, sparse eyebrows and lashes, shallow supraorbital ridges, upslanting palpebral fissures, hypertelorism, and upturned nares.
However, this infant was born with some other congenital anomalies of gut rotation including anterior abdominal wall defect, bowel herniation, and vestibular anus described only in the severest forms of PKS.
However, there is still no consensus among paediatric surgeons on what constitutes the best definitive management of children born with a vestibular anus
. In the Indian scenario the opinion is very much divided particularly between ASARP and PSARP.
108 girls with vestibular anus on regular dilatation plan till PSARP or anal transposition; 6 girls with ectopic anterior anus on regular dilatation plan till PSARP or anal transposition.
Anal transposition was done for vestibular anus in females as single stage without prior colostomy in 84 patients.
Procedure done as 2nd Stage or Single Stage after Weight Gain 1 Abdominoperineal 20 of total 690 pull through boys (17) and girls (3) = (2.8%) 2 Anal transposition as 84/690 single stage without (12.1%) prior colostomy in 84 girl patients 3 Anal transposition 6/690 (0.8%) ectopic anterior anus in females as single stage 4 PSARP was done for 24/690 (3.4%) vestibular anus in females as second stage with prior colostomy 5 PSARP for males as 160/690 (23.1%) = Total second stage with 294/690 prior colostomy (42.6%) RESULTS
This approach is recommended as an optimal technique for treatment of vestibular anus
in girls by experienced surgeons with large series of cases and our results agrees with the published literature.