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quote from "Pediatric Colorectal Conundrums: Case Studies: From Fundamental to Advanced (Pediatric Colorectal Surgery)" by Marc Levitt, Thomas Xu, Hussein Wissanji - "Classically, it had been considered that an ileoanal pull-through should not be performed until urinary continence was achieved. The main reason for this recommendation was to avoid severe irritation of the perineal skin due to frequent stooling after proctocolectomy which could be improved if the child could sit on the potty. However, the treatment of perineal skin and in the medical management of hypermotility with loperamide and resins in combination with irrigations have so improved that waiting on the pull-through is no longer recommended. In fact, waiting until a child is older can be problematic as the child then is more aware of passage of stool, and may withhold, and in certain cases this has led to proctalgia fugax, a severe perineal sphincter spasm."

quote from "Pediatric Colorectal Conundrums: Case Studies: From Fundamental to Advanced (Pediatric Colorectal Surgery)" by Marc Levitt, Thomas Xu, Hussein Wissanji - "A colonic resection based solely on a frozen section must be avoided in patients who have an aganglionic zone proximal to the left colon. This is because proximal to the splenic flexure you cannot expect to see hypertrophic nerves (the sacral plexus does not innervate that bowel). Therefore, you are completely dependent upon your pathologist identifying ganglion cells on frozen section which is prone to sampling bias /false negatives. This is due to artifact in the tissue that can occur from the freezing process. Consequently, you risk resection of good bowel that was incorrectly deemed aganglionic by frozen section."

case from Dr Usman Akram What will be the best plan according to you in this case ?

quote from "Pediatric Colorectal Conundrums: Case Studies: From Fundamental to Advanced (Pediatric Colorectal Surgery)" by Marc Levitt, Thomas Xu, Hussein Wissanji - A technically correct high-pressure distal colostogram should provide data on the following six key components: 1. The mucous fistula site. 2. The amount of bowel length available for the pull-through (AP view). 3. The rectal end and communication with the urinary tract (if present) (lateral view). 4. The tip of the sacrum. 5. The urethra and bladder (if possible). 6. An anal marker.

quote from "Pediatric Colorectal Conundrums: Case Studies: From Fundamental to Advanced (Pediatric Colorectal Surgery)" by Marc Levitt, Thomas Xu, Hussein Wissanji - 1. For all anal stenosis (and rectal atresia) cases you must check for a presacral mass. 2. For any such presacral mass, always make sure there is no dural component (pelvic plus spinal MRI) and, if present, involve neurosurgery. 3. If Currarino syndrome, genetic testing is warranted for the patient and first-degree relatives. 4. For management, removal of the mass is key which will likely solve much of the constipation. 5. If the anus is narrow that must be treated; an anal canal sparing technique can accomplish this. Occasionally, dilation alone can work. 6. With mass removal the colon should improve, although in such a case antegrade flush access with a Malone may be beneficial to promote daily colonic emptying. 7. Whether the colon will recover is a key question, but it can be decided later whether a colon resection is required.

Regarding PSARP for recto-bulbar fistula quote from "Pediatric Colorectal Conundrums: Case Studies: From Fundamental to Advanced (Pediatric Colorectal Surgery)" by Marc Levitt, Thomas Xu, Hussein Wissanji - "The key maneuver required is to open the posterior rectal wall and carefully inspect the anterior wall for any connections and, if a fistula is found, then carefully mobilize the anterior rectum off the urethra."