Isolated ileal perforation in infancy: a lethal initial presentation of Hirschsprung’s disease

  • Fadi Iskandarani | iskandaranifadi@gmail.com Faculty of Medicine, Lebanese University, Hadath, Beirut; Sahel General Hospital, Ghobeiri, Beirut, Lebanon.
  • Chawki Hammoud Faculty of Medicine, Lebanese University, Hadath, Beirut; Sahel General Hospital, Ghobeiri, Beirut, Lebanon.
  • Sarah Srour Faculty of Medicine, Lebanese University, Hadath, Beirut; Sahel General Hospital, Ghobeiri, Beirut, Lebanon.
  • Gloria Pelizzo Pediatric Surgery Unit, Children’s Hospital, Istituto Mediterraneo di Eccellenza Pediatrica, Palermo, Italy.
  • Ghassan Nakib Department of Pediatric Surgery, Medclinic Middle East, Mediclinic City Hospital Dubai, United Arab Emirates.
  • Valeria Calcaterra Pediatric Unit, Department of Maternal and Children’s Health, Fondazione IRCCS Policlinico San Matteo, Pavia; Department of Internal Medicine, University of Pavia, Italy.
  • Amir Khanafer Faculty of Medicine, Lebanese University, Hadath, Beirut; Sahel General Hospital, Ghobeiri, Beirut, Lebanon.

Abstract

A rare case of ileal perforation, as a fatal initial presentation of total colonic aganglionosis (TCA) in infancy is reported. A 10-week-old boy, was brought to the emergency department with symptoms of complicated intestinal obstruction. He looked ill, was lethargic, markedly dehydrated and had a severely distended abdomen. An abdominal X-ray revealed multiple air fluid levels seen in a distended small intestine. During exploratory laparotomy the ileum was massively dilated with distal segment perforation. Ileal perforation repair was performed. A totally collapsed microcolon was identified. Biopsies were taken from the high rectum, sigmoid and hepatic flexure. Appendectomy and ileostomy were performed. All biopsies, as well as the appendix, showed absence of ganglion cells. Despite this procedure the patient progressively deteriorated and later died due to sepsis. Ileal perforation in infants is a rare, but potentially fatal initial presentation of TCA. Early detection is essential to prevent life-threatening complications.

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Published
2017-06-26
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Section
Case Reports & Letters
Keywords:
Hirschsprung's disease, total colonic aganglionosis, ileal perforation, initial presentation, infant
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How to Cite
Iskandarani, F., Hammoud, C., Srour, S., Pelizzo, G., Nakib, G., Calcaterra, V., & Khanafer, A. (2017). Isolated ileal perforation in infancy: a lethal initial presentation of Hirschsprung’s disease. Pediatric Reports, 9(2). https://doi.org/10.4081/pr.2017.7084

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