Olutoye Oluyinka O, Hammond J D, Gilley Jamie, Beckman Ross M, Bulathsinghala Marie, Keswani Sonya S, Davies Jonathan, Mazziotti Mark V, Donepudi Roopali, Belfort Michael A, King Alice, Ketwaroo Pamela M, Lee Timothy C
Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX, USA.
Division of Neonatology, Texas Children's Hospital, Baylor College of Medicine, Houston, TX, USA.
J Pediatr Surg Case Rep. 2023 Jun;93. doi: 10.1016/j.epsc.2023.102654. Epub 2023 Apr 28.
Malrotation of the intestinal tract is a congenital malformation commonly found either incidentally or after affected individuals develop signs and symptoms of intestinal obstruction. Malrotation is prone to midgut volvulus that can cause intestinal obstruction and lead to ischemia and necrosis requiring emergent surgical intervention. Rare instances of midgut volvulus have been reported in the literature and carry a high mortality given the difficulty in establishing a diagnosis prior to development of signs of intestinal ischemia and necrosis. Advancements in imaging have made it possible to diagnose malrotation earlier, raising the question of optimal timing of delivery, especially in cases of prenatally diagnosed midgut volvulus. In these cases, the risks of premature birth must be weighed against the risks of fetal intestinal ischemia and potential fetal demise.
This case report details an interesting presentation of intestinal malrotation with suspected midgut volvulus found on prenatal imaging at 33 weeks and 4 days' gestation. This prompted delivery of the infant at 34 weeks and 2 days' gestation with urgent operative management, within 3 hours of life, after diagnosis was confirmed postnatally. Intraoperatively, the infant was confirmed to have midgut volvulus without bowel ischemia, the intestines were reduced, and a Ladd procedure was performed without incident. The infant recovered postoperatively without complication, tolerated advancement to full volume feeds and was discharged on day of life 18.
Successful management of fetal malrotation with midgut volvulus may be accomplished by early access to a multi-disciplinary team of professionals, prompt postnatal confirmation of diagnosis, and urgent correction to minimize the risk of complications.
肠道旋转不良是一种先天性畸形，通常在偶然情况下被发现，或者在受影响个体出现肠梗阻症状后被发现。旋转不良易发生中肠扭转，可导致肠梗阻，并引发缺血和坏死，需要紧急手术干预。文献中报道了罕见的中肠扭转病例，鉴于在肠道缺血和坏死迹象出现之前难以确诊，其死亡率很高。影像学的进步使得更早诊断旋转不良成为可能，这就引发了关于最佳分娩时机的问题，尤其是在产前诊断为中肠扭转的情况下。在这些病例中，必须权衡早产风险与胎儿肠道缺血风险以及潜在的胎儿死亡风险。
本病例报告详细描述了一例有趣的肠道旋转不良病例，在妊娠33周零4天时的产前影像学检查中发现疑似中肠扭转。这促使在妊娠34周零2天时分娩婴儿，并在出生后确诊后3小时内进行紧急手术治疗。术中证实婴儿患有中肠扭转但无肠缺血，肠道复位，并顺利进行了Ladd手术。婴儿术后恢复良好，无并发症，耐受全量喂养，于出生后第18天出院。
通过尽早联系多学科专业团队、产后迅速确诊以及紧急纠正，可成功处理胎儿旋转不良合并中肠扭转，以尽量降低并发症风险。
