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Published on: October 29, 2014
Hereditary Multiple Intestinal Atresia: A Case Report and Review of the Literature
Maher M Al-Zaiem1, Rawan S Alsamli2, Ethar A Alsulami2
1Department of Pediatric Surgery, Maternity and Children Hospital, Makkah, SAU.
Insights
Hereditary multiple intestinal atresia (HMIA) is a rare condition affecting the entire gastrointestinal tract. This case report details a neonate diagnosed with HMIA, highlighting diagnostic and management strategies.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Medical Genetics
Background:
- Hereditary multiple intestinal atresia (HMIA) is an extremely rare congenital anomaly.
- It affects the gastrointestinal tract from the pylorus to the rectum, presenting significant surgical challenges.
Observation:
- A three-day-old female infant presented with symptoms of upper intestinal obstruction.
- Radiological imaging revealed pyloric obstruction, intra-abdominal calcifications, and rectal atresia, strongly suggesting HMIA.
- Exploratory laparotomy confirmed extensive intestinal atresias from the pylorus through the small bowel, a cord-like colon, and rectal atresia.
Findings:
- Surgical intervention involved multiple resections of atretic segments with subsequent anastomoses.
- A terminal ileostomy and a trans-anastomotic tube were utilized for management.
- The case illustrates the complex clinical presentation and surgical management of HMIA.
Implications:
- This case contributes to the limited literature on Hereditary multiple intestinal atresia.
- It underscores the importance of early diagnosis and aggressive surgical management in neonates with HMIA.
- Further research into the genetic basis and long-term outcomes of HMIA is warranted.
Abstract:
Hereditary multiple intestinal atresia (HMIA) is a rare form of intestinal atresia that affects the gastrointestinal tract from the pylorus to the rectum. A few cases have been reported in the literature. Here, we report the case of a three-day-old girl who was referred to our hospital as a case of upper intestinal obstruction. After initial resuscitation, the radiological examination revealed pyloric obstruction, multiple intra-abdominal calcifications, and rectal atresia making the diagnosis of HMIA most likely. Exploratory laparotomy revealed multiple intestinal atresia, the first started at the pylorus, the second was at the level of the duodenojejunal junction, and there were also multiple small bowel atresias. The colon was a cord-like structure, and there was rectal atresia. Multiple resections of the atretic intestinal segment followed by multiple anastomoses, terminal ileostomy, and the use of a trans-anastomotic tube were performed. In this study, the clinical picture, radiological findings, and management are described and compared to the findings reported in the literature.
