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Pediatric Reports
Volume 9
Issue 2
10.4081/pr.2017.7084
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Case Report

Isolated Ileal Perforation in Infancy: A Lethal Initial Presentation of Hirschsprung’s Disease

by
Fadi Iskandarani
1,*,
Chawki Hammoud
1,
Sarah Srour
1,
Gloria Pelizzo
1,
Ghassan Nakib
1,
Valeria Calcaterra
1 and
Amir Khanafer
1
1
Faculty of Medicine, Lebanese University, Hadath, Beirut; Sahel General Hospital, Ghobeiri, Beirut, Lebanon
2
Pediatric Surgery Unit, Children’s Hospital, Istituto Mediterraneo di Eccellenza Pediatrica, Palermo, Italy
3
Department of Pediatric Surgery, Medclinic Middle East, Mediclinic City Hospital Dubai, UAE
4
Pediatric Unit, Department of Maternal and Children’s Health, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy
5
Department of Internal Medicine, University of Pavia, Lebanon, Italy
*
Author to whom correspondence should be addressed.
Pediatr. Rep. 2017, 9(2), 7084; https://doi.org/10.4081/pr.2017.7084
Submission received: 8 February 2017 / Revised: 8 February 2017 / Accepted: 8 February 2017 / Published: 26 June 2017

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.
Keywords: Hirschsprung's disease; total colonic aganglionosis; ileal perforation; initial presentation; infant Hirschsprung's disease; total colonic aganglionosis; ileal perforation; initial presentation; infant

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MDPI and ACS Style

Iskandarani, F.; Hammoud, C.; Srour, S.; Pelizzo, G.; Nakib, G.; Calcaterra, V.; Khanafer, A. Isolated Ileal Perforation in Infancy: A Lethal Initial Presentation of Hirschsprung’s Disease. Pediatr. Rep. 2017, 9, 7084. https://doi.org/10.4081/pr.2017.7084

AMA Style

Iskandarani F, Hammoud C, Srour S, Pelizzo G, Nakib G, Calcaterra V, Khanafer A. Isolated Ileal Perforation in Infancy: A Lethal Initial Presentation of Hirschsprung’s Disease. Pediatric Reports. 2017; 9(2):7084. https://doi.org/10.4081/pr.2017.7084

Chicago/Turabian Style

Iskandarani, Fadi, Chawki Hammoud, Sarah Srour, Gloria Pelizzo, Ghassan Nakib, Valeria Calcaterra, and Amir Khanafer. 2017. "Isolated Ileal Perforation in Infancy: A Lethal Initial Presentation of Hirschsprung’s Disease" Pediatric Reports 9, no. 2: 7084. https://doi.org/10.4081/pr.2017.7084

APA Style

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), 7084. https://doi.org/10.4081/pr.2017.7084

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