Al-Zaiem Maher M, Alsamli Rawan S, Alsulami Ethar A, Mohammed Ruba F, Almatrafi Mohammad I
Department of Pediatric Surgery, Maternity and Children Hospital, Makkah, SAU.
College of Medicine and Surgery, Umm Al-Qura University, Makkah, SAU.
Cureus. 2022 Oct 30;14(10):e30870. doi: 10.7759/cureus.30870. eCollection 2022 Oct.
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.
遗传性多发性肠闭锁（HMIA）是一种罕见的肠闭锁形式，可累及从幽门到直肠的胃肠道。文献中已有少数病例报道。在此，我们报告一例3日龄女婴，因上消化道梗阻转诊至我院。初步复苏后，影像学检查显示幽门梗阻、腹腔内多发钙化及直肠闭锁，高度怀疑为HMIA。剖腹探查发现多处肠闭锁，第一处始于幽门，第二处位于十二指肠空肠交界处，还有多处小肠闭锁。结肠呈条索状，存在直肠闭锁。对闭锁肠段进行了多次切除，随后进行了多次吻合、末端回肠造口术，并使用了经吻合口管。本研究描述了该病例的临床表现、影像学检查结果及治疗方法，并与文献报道的结果进行了比较。
