2 Case Presentation
A 36-week male neonate was referred to the pediatric surgery service on the second day of life due to failure to pass meconium, abdominal distension, and bilious emesis.
The patient was born to a 23-year-old healthy primipara Iranian mother without a history of abortion via normal vaginal delivery. Her birth weight was 2500 grams.
On physical examination, the primary vital signs were in the normal range, bowel sounds were decreased, and the rectum was empty.
Initial abdominopelvic radiography revealed Thumb-sized intestinal loops with no gas in the rectum [Figure 1A]. Small-bowel contrast series confirmed dilated proximal blind end [Figure 1B].
Baseline biochemical and blood gases analyses were in the normal range. On the third day, with a diagnosis of intestinal obstruction, the patient underwent exploratory laparotomy after obtaining informed consent from the parent. We identified Ileal atresia with a proximal dilated, blind-ending1 cm distal bud attached to the ileocecal region (Type IIIa). [Figure 2] The colon appeared as an unused microcolon throughout. After resecting the proximal dilated ileal pouch and ileocecal part, surgery was followed by an end-to-end ileo- ascending colonic anastomosis.
Permanent Hematoxylin & Eosin (H&E) pathological examination revealed the absence of ganglion cells in the cecum, the appendix, and the rectum and the abundance of ganglia in the ileum [Figure 3].
Due to the persistence of abdominal distention, no defecation on postoperative days, and a histopathologic report that was compatible with total segment HD, the patient was a candidate for re-exploration. [Figure 4]
Our findings were a new colon and a functional intestinal obstruction at the anastomosis site with no anastomotic leak.
After taking multiple levels of frozen section biopsies to reaffirm the total colonic aganglionosis, the patient underwent an ileostomy and colonic mucus fistula bypass
The final permanent pathology studies by calretinin test disclosed total aganglionic colon HD.
Swenson pull-through surgery with removal of all colon and ileoanal anastomosis was performed on three month old. The case tolerated oral feeding after five days and was discharged from the hospital in good condition.