Case Report: 7-Year-Old Boy with Incarcerated Internal Hernia Leading to Extensive Intestinal Necrosis Due to a Large Congenital Mesenteric Defect.

We present a case of a 7-year-old boy with acute abdominal symptoms initially misdiagnosed as constipation. Delayed imaging diagnostics revealed an ileus with contorted small intestine, so laparotomy was indicated. An acute bowel obstruction was found based on an incarcerated internal hernia. Small and large bowel segments were incarcerated into a large mesenteric defect leading to extended intestinal necrosis. About 30 cm of necrotic small bowel and 15 cm of large intestine were resected, two primary anastomoses were performed. The mesenteric defect was closed with two running sutures. The boy's clinical outcome was very good. Two aspects are discussed: the initial clinical misdiagnosis of acute bowel obstruction in a child leading to a delay of diagnostics and therapy on the one hand and the origin of mesenteric defects on the other. In children with abdominal pain, ultrasound must be performed as soon as possible and pediatric surgeons have to be involved early. There should be an awareness of the fact, that mesenteric defects and other congenital malformations can occur more often than we suspect it. In the case of an internal hernia, a misjudgement of the clinical condition may be very harmful for the patient and can lead to a short bowel syndrome or even death.

Associations of Mucosal Nerve Fiber Innervation Density with Hirschsprung-Associated Enterocolitis: A Retrospective Three-Center Cohort Study.

OBJECTIVE: Hirschsprung's disease (HSCR) is a congenital intestinal neurodevelopmental disorder characterized by the absence of enteric ganglion cells in the distal colon. Although Hirschsprung-associated enterocolitis (HAEC) is the most frequent life-threatening complication in HSCR, to date reliable biomarkers predicting the likelihood of HAEC are yet to be established. We established a three-center retrospective study including 104 HSCR patients surgically treated between 1998 and 2019. MATERIALS AND METHODS: Patient-derived cryopreserved or paraffin-preserved colonic tissue at surgery was analyzed via ßIII-tubulin immunohistochemistry. We subsequently determined extrinsic mucosal nerve fiber density in resected rectosigmoid specimens and classified HSCR patients accordingly into nerve fiber-high or fiber-low groups. We compared the distribution of clinical parameters obtained from medical records between the fiber-high (n = 36) and fiber-low (n = 68) patient groups. We assessed the association between fiber phenotype and enterocolitis using univariate and multivariate logistic regression adjusted for age at operation. RESULTS: Enterocolitis was more prevalent in patients with sparse mucosal nerve fiber innervation (fiber-low phenotype, 87%) compared with the fiber-high phenotype (13%; p = 0.002). In addition, patients developing enterocolitis had a younger age at surgery (3 vs. 7 months; p = 0.016). In the univariate analysis, the odds for enterocolitis development in the fiber-low phenotype was 5.26 (95% confidence interval [CI], 1.67-16.59; p = 0.005) and 4.01 (95% CI, 1.22-13.17; p = 0.022) when adjusted for age. CONCLUSION: Here, we showed that HSCR patients with a low mucosal nerve fiber innervation grade in the distal aganglionic colon have a higher risk of developing HAEC. Consequently, histopathologic analysis of the nerve fiber innervation grade could serve as a novel sensitive prognostic marker associated with the development of enterocolitis in HSCR patients.