A rare association of ileal atresia and total colonic Hirschsprung's disease

Daib, A.; Lindolsi, Maha; Hellal, Y.; Boughdir, Malek; Abdallah, Rabiaa Ben; Kaabar, N

doi:10.1016/j.epsc.2021.102111

Cited by 1 publication

(3 citation statements)

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“…Intestinal atresia is a congenital disease characterized by complete occlusion of the intestinal lumen ( 6 ). Causes of JIA have been attributed to intrauterine vascular events involving the mesenteric vessel branches of the midgut, leading to bowel necrosis and resorption ( 1 , 4 ). Intestinal atresia has been classified into four types based on the Grosfeld classification system ( Table 1 ).…”

Section: Discussionmentioning

confidence: 99%

“…In contrast to JIA, which is thought to be due to a vascular defect in the intrauterine period, HD occurs due to the absence of ganglion cells in the myenteric (Auerbach) and submucosal (Meissner) plexuses, which is regarded as a neurogenic origin for intestinal obstruction ( 7 , 8 ). Concomitant HD with ileal atresia can be explained through a possible common intrauterine vascular accident with loss of bowel that, in turn, may act as a barrier for neuromeric cells caudal migration, leading to colonic aganglionosis ( 4 , 9 ). HD is reported to occur in 1 in 5,000 newborns ( 2 ).…”

Section: Discussionmentioning

confidence: 99%

“…The incidence of intestinal obtrusion ranges from 1 in 5,000 to 1 in 14,000 live births, and occurs equally in men and women ( 1 – 3 ). Additionally, JIA is associated with other concomitant malformations, such as cardiac anomalies, gastroschisis, cystic fibrosis, and other conditions ( 1 , 4 , 5 ). Concomitant ileal atresia and Hirschsprung disease (HD) is an extremely rare entity, with an incidence ranging from 0.25 to 0.8% ( 4 ).…”

Section: Introductionmentioning

confidence: 99%

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Case Report: Jejunoileal Atresia With Persistent Poor Bowel Function Can Occur After Surgical Correction for Hirschsprung Disease

et al. 2022

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Jejunoileal atresia (JIA) is one of the common etiologies of intestinal obtrusion in neonates. However, cases of concomitant ileal atresia and Hirschsprung disease (HD) rarely occur. We report the case of a male infant who had JIA concomitantly with HD that was re-anastomosed. The patient underwent an exploratory laparotomy to resect the dilated terminal ileum. Subsequently, owing to a significantly dilated proximal bowel, he underwent a second exploratory laparotomy. However, he continued to have feeding intolerance postoperatively. He had colonic aganglionosis and was diagnosed with HD. A third laparotomy was then performed. Additionally, he had recurrent episodes of gram-negative bacteremia, especially candida parapsilosis fungemia, despite receiving antibiotics and antifungal, and there were no identifiable underlying genetic or immunological causes. Finally, the patient had recurrent episodes of hypoglycemia, central hypothyroidism, and multiple organ failure and died at the age of 7 months. The concomitant ileal atresia and HD was thought to be due to a common intrauterine vascular accident, together with loss of bowel, thereby acting as a barrier for the caudal migration of neuromeric cells and leading to colonic aganglionosis. In this case, ileal atresia was associated with colonic aganglionosis, central hypothyroidism, and persistent bacteremia, which is a unique finding. In cases of JIA, persistent poor bowel function after surgical correction of concomitant HD should be considered.

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Section: Discussionmentioning

confidence: 99%