10-Year-Old Male Presents with Severe Left-sided Abdominal Pain and Discharge from Anus

• Ten-year-old male presents to the ED with severe left sided abdominal pain and discharge from the anus.
• He is febrile and tachycardic. He is admitted and ultimately diagnosed with Shigella colitis with overflow diarrhea into the efferent limb of his loop transverse colostomy. Also, he was a Syrian refugee who immigrated to Canada. No prior medical records available.
• Per the medical and surgical history from the family he was born full term and spend several weeks in the NICU.
• He passed stool within 24 h of life.
• Anus never looked normal and he was discharged home with plan for family to perform dilations after birth.
• At age 2, he had his first laparotomy and surgery on “the bladder”.
• At age 3.5, he had second laparotomy and creation of a colostomy.
• At age 4, he had a third laparotomy and revision of the colostomy—presumably loop transverse colostomy.
• At age 8, he had laparotomy, pull-through, and PSARP complicated by perineal wound infection and significant scarring.
• At age 8.5 he had appendicitis with open appendectomy.
• On exam, he had multiple abdominal scars.
• On perineal exam, he had severely scarred anus with multiple incisions surrounding the anus. He had a hypotonic anal sphincter that passed a size 6 Hegar. His sacral ratio was 0.58. He had a lower GI study through the anus that showed irregular margins of the rectum and colon to the level of the colostomy and he had a posterior rectocele.
• CT abdomen and pelvis showed a presacral thin walled mass.
• He had a transverse colon preserved and normal cecum.
• MRI pelvis and spine showed a thickened rectum/sigmoid and thin walled presacral cyst not communicating with the spinal canal thought to represent dermoid.
• EUA was performed using the Pena stimulator that showed no movement.
• Anoplasty was performed by opening the shelf anteriorly and could then pass a Hegar 15.
• Resection of the presacral mass with the tip of the coccyx was performed through a posterior approach.
• Pathology confirmed an epidermoid cyst.
• Contrast study was performed through the colostomy. The left colon and rectum are patent without stenosis, anterior to the rectal wall there was a small contrast extravasation and posterior rectal wall rectocele was identified.
• He had an anorectal manometry study, because the patient would like his stoma closed.
• Manometry showed hypotensive anal sphincter and no proper continence mechanism.

Takeaways

• It is important for all the members of the family to have AP and lateral x-rays of the sacrum. If any of them have hemisacrum, MRI should be obtained.
• Patient will mostly likely be fecally incontinent. A neomalone can be created at the same time as the colostomy closure. The neomalone should not be used for 1 mo following surgery. After the colostomy is closed, bowel management will need to be implemented to keep the patient clean.