Isolated ileal perforation in infancy: a lethal initial presentation of Hirschsprung’s disease
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 s...
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| Format: | Article |
| Language: | English |
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MDPI AG
2017-06-01
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| Series: | Pediatric Reports |
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| Online Access: | http://www.pagepress.org/journals/index.php/pr/article/view/7084 |
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| author | Fadi Iskandarani Chawki Hammoud Sarah Srour Gloria Pelizzo Ghassan Nakib Valeria Calcaterra Amir Khanafer |
| author_facet | Fadi Iskandarani Chawki Hammoud Sarah Srour Gloria Pelizzo Ghassan Nakib Valeria Calcaterra Amir Khanafer |
| author_sort | Fadi Iskandarani |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-14T03:45:51Z |
| format | Article |
| id | doaj.art-0d80785551b341a7a4aadcca9d3f3b11 |
| institution | Directory Open Access Journal |
| issn | 2036-749X 2036-7503 |
| language | English |
| last_indexed | 2024-12-14T03:45:51Z |
| publishDate | 2017-06-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Pediatric Reports |
| spelling | doaj.art-0d80785551b341a7a4aadcca9d3f3b112022-12-21T23:18:22ZengMDPI AGPediatric Reports2036-749X2036-75032017-06-019210.4081/pr.2017.70843756Isolated ileal perforation in infancy: a lethal initial presentation of Hirschsprung’s diseaseFadi Iskandarani0Chawki Hammoud1Sarah Srour2Gloria Pelizzo3Ghassan Nakib4Valeria Calcaterra5Amir Khanafer6Faculty of Medicine, Lebanese University, Hadath, Beirut; Sahel General Hospital, Ghobeiri, BeirutFaculty of Medicine, Lebanese University, Hadath, Beirut; Sahel General Hospital, Ghobeiri, BeirutFaculty of Medicine, Lebanese University, Hadath, Beirut; Sahel General Hospital, Ghobeiri, BeirutPediatric Surgery Unit, Children’s Hospital, Istituto Mediterraneo di Eccellenza Pediatrica, PalermoDepartment of Pediatric Surgery, Medclinic Middle East, Mediclinic City Hospital DubaiPediatric Unit, Department of Maternal and Children’s Health, Fondazione IRCCS Policlinico San Matteo, Pavia; Department of Internal Medicine, University of PaviaFaculty of Medicine, Lebanese University, Hadath, Beirut; Sahel General Hospital, Ghobeiri, BeirutA 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.http://www.pagepress.org/journals/index.php/pr/article/view/7084Hirschsprung's diseasetotal colonic aganglionosisileal perforationinitial presentationinfant |
| spellingShingle | Fadi Iskandarani Chawki Hammoud Sarah Srour Gloria Pelizzo Ghassan Nakib Valeria Calcaterra Amir Khanafer Isolated ileal perforation in infancy: a lethal initial presentation of Hirschsprung’s disease Pediatric Reports Hirschsprung's disease total colonic aganglionosis ileal perforation initial presentation infant |
| title | Isolated ileal perforation in infancy: a lethal initial presentation of Hirschsprung’s disease |
| title_full | Isolated ileal perforation in infancy: a lethal initial presentation of Hirschsprung’s disease |
| title_fullStr | Isolated ileal perforation in infancy: a lethal initial presentation of Hirschsprung’s disease |
| title_full_unstemmed | Isolated ileal perforation in infancy: a lethal initial presentation of Hirschsprung’s disease |
| title_short | Isolated ileal perforation in infancy: a lethal initial presentation of Hirschsprung’s disease |
| title_sort | isolated ileal perforation in infancy a lethal initial presentation of hirschsprung s disease |
| topic | Hirschsprung's disease total colonic aganglionosis ileal perforation initial presentation infant |
| url | http://www.pagepress.org/journals/index.php/pr/article/view/7084 |
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