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J Indian Assoc Pediatr Surg. 2017 Apr-Jun; 22(2): 67–68.
PMCID: PMC5379875

Anorectal Malformation: Issues Beyond Definitive Surgery

figure JIAPS-22-67-g001
Prema Menon

Dear Friends,

Greetings from Chandigarh!

It is a great honor to write the editorial for this issue specially dedicated to anorectal malformations (ARMs).

Several books, book chapters, and papers have been written on this malformation. However, the following concerns still remain: What is the best age and time to perform the definitive surgery, should we be performing primary repair to the extent possible, is a laparoscopic approach better in high anomalies, are we looking for and managing associated anomalies from birth onward proactively, and what are the long-term problems we face in our country.

Is primary posterior sagittal anorectoplasty (PSARP) in male intermediate and high ARMs worth the effort? Our recent analysis suggests that the answer is YES, i.e., these newborns seem to have better continence. Anesthetic expertise, especially in emergency hours, the general condition of the newborn to withstand surgery in the prone position, and most importantly, the experience and ability of the surgeon have to be kept in mind while doing a primary repair. The overall hospital costs can be brought down, the parents and child are spared the trauma of a colostomy, and the much-needed hospital beds and operation theater times are available in busy government institutes such as ours where no patient is turned away. It should neither be done by someone in training nor it is advisable in rectal atresia where there are high chances of wound contamination and dehiscence as the suture line is not at the anal verge.

We continue to perform primary PSARP in all females with vestibular fistula between 3 months and 1 year of age with excellent results. Although often called “decompressing” by those in training, you realize that the child is straining at stools since birth. With time, the narrow fistula causes hypertrophy of the proximal colon. In late referrals, with fecalith formation, often proportional to the age of the child and the food she takes, it is safer to perform the definitive surgery with a covering colostomy. We make all efforts to keep the bowel decompressed by fistula dilatation, laxatives, and rectal/fistula wash right from the day of diagnosis. Good preoperative bowel preparation, i.e. total gut irrigation, may have to be given for more than one cycle. For assessing this, apart from clinical examination, a simple plain radiograph of the abdomen is useful. Congenital pouch colon should be ruled out by a contrast enema in the presence of a reasonably good size vestibular opening in a constipated child.

While the management of female low ARMs and nearly all male ARMs is relatively straightforward, the management of cloaca requires detailed investigations and expertise. It is not a surgery for the novice as the available sphincter mechanism for urinary and fecal continence should be preserved. Similarly, managing a child with congenital pouch colon throws up many challenges.

The associated anomalies are often forgotten. While gastrointestinal tract anomalies are picked up early, urological anomalies are often missed or ignored. We have encountered a few children with ARM lost to follow-up and coming at 10–12 years of age with uremia due to neurogenic bladder and vesicoureteric reflux in a single-scarred kidney. It is our practice to perform an ultrasound of kidney ureter bladder and an echocardiogram at the first presentation. Sacral anomalies should be documented. A raised blood urea creatinine report even in the first 3 days of life should be given importance and nephrotoxic drugs must be avoided until further investigations show the presence of normal bilateral kidneys. Due to the high incidence of vesicoureteric reflux in high anomalies, uroprophylaxis should be started until a micturating cystourethrogram can be done.

While we may have done the procedure excellently, fecal continence is not achieved in a commiserate manner. Reporting of continence has to be honest so that others are not mislead. Management of constipation, soiling, etc., should start early in life. These symptoms are often elicited only by specific questions by a sympathetic listener. Examination of the underwear for soiling and perineal excoriation gives a fairly good idea of the problems faced by these children. Dietary modifications, medications such as loperamide, daily saline enemas given retrograde or antegrade, and the ability of the parents to deal with the problem play an important role in achieving social continence. An important aspect often neglected is the psychological effect of the disease on the patients and the caregiver. We should understand the disease implications on the quality of life of these children when they interact with peers, especially as they grow into adults and face life independently. They should be handed over at the appropriate time to adult surgeons, nephrologists, etc., as required or as many pediatric surgeons seem to be doing in our country, continue to take care of them well into adulthood!

These and several other problematic areas are highlighted in this issue. I thank the contributors from all across our country for sharing their vast experience and all the paper reviewers for sparing their valuable time. We should be proud of our legacy. Many Indian pediatric surgeons have made significant contributions on this subject in the past. I hope we will do the same in the future also, discovering innovative ways to improve continence.

Namaskar!


Articles from Journal of Indian Association of Pediatric Surgeons are provided here courtesy of Wolters Kluwer -- Medknow Publications