A Case Report of a Child with Constipation Diagnosed with Acquired Myenteric Hypoganglionosis

Abstract

Background and Clinical Significance: Acquired myenteric hypoganglionosis is a rare dysmotility disorder that can present in childhood and adulthood, characterized by a reduced number of ganglion cells within Auerbach's plexus. Due to the rarity of the pathology, few case reports of acquired myenteric hypoganglionosis in adolescents have been described. This case report explores the presentation, risk factors, and surgical complications associated with the ultimate diagnosis of myenteric hypoganglionosis. Case Presentation: We present a case of a 12-year-old male with a history of constipation and achalasia, presenting with constipation and abdominal distention, who underwent a colonoscopy, which was converted to an exploratory laparotomy with loop ileostomy creation due to persistent significant abdominal distention. This was complicated by colonic perforation, most likely secondary to stercoral colitis, requiring takeback to the operating room on postoperative day 11 for an exploratory laparotomy with bowel resection and mucous fistula creation. The patient was then referred to Boston Children's Hospital for motility studies, which revealed poor colonic motility and plans to reassess motility in 1 year. Conclusions: Although rare, it is important to have high clinical suspicion for acquired myenteric hypoganglionosis in children, especially males, with severe constipation.

Keywords: acquired myenteric hypoganglionosis; case report; constipation; pediatric surgery.

Figure 1

KUB on patient presentation, revealing a dilated transverse colon with significant stool burden.

Figure 2

CT scan on patient presentation, revealing a colon dilated to 6.34 cm in diameter, with significant stool burden.

Figure 3

Dilated transverse colon in the initial exploratory laparotomy.

Figure 4

Arrow indicates perforation of the ascending colon (with surgeon’s finger inside) and stool in the peritoneum upon takeback.