Krishnamurthy Kritika, El Hussein Siba, Omarzai Yumna
Mount Sinai Medical Center, Department of Pathology. Miami Beach, Miami, FL, United States of America.
Mount Sinai Medical Center, Department of Pathology and Herbert Wertheim College of Medicine Florida International University. Miami Beach, Miami, FL, United States of America.
Autops Case Rep. 2018 Sep 14;8(3):e2018037. doi: 10.4322/acr.2018.037. eCollection 2018 Jul-Sep.
Small intestinal volvulus (SBV) is the abnormal twisting of bowel around the axis of its mesentery, leading to obstruction and vascular compromise, resulting in bowel ischemia and necrosis which are life-threatening. Risk factors include malformation, malrotation, and adhesions. Its rare incidence and vague clinical presentation make it a difficult diagnosis, more so in a nonverbal patient who cannot express his pain, which is the first and most prominent symptom. Studies suggest an increased frequency of intestinal obstruction in cerebral palsy patients. There are no reported cases of small intestinal volvulus in association with cerebral palsy. We present a case of a 21-year-old man with severe cerebral palsy and kyphoscoliosis. The patient presented to the emergency room with respiratory distress and abdominal distension. An acute abdomen was noted. Abdominal X-rays revealed gas patterns suggestive of small intestinal obstruction. The patient rapidly deteriorated, and resuscitation attempts were unsuccessful. Autopsy revealed peritoneal cavity filled with extensively dilated and thin-walled loops of small intestine. Twisting of the small intestine, showing 360° rotation around the mesenteric root in a clockwise manner at two separate sites, was noted. On bowel dissection, mucosal folds were absent, and mucosa was green with patchy areas of hemorrhage consistent with ischemic necrosis. There was no evidence of any malformation, malrotation or adhesions. Small intestinal volvulus is a rare entity with a nonspecific clinical presentation that poses a diagnostic challenge. This autopsy highlights the need to maintain a high index of suspicion for small intestinal volvulus in cases of bowel obstruction in cerebral palsy patients to expedite surgery and prevent mortality. The primary caregivers of non-verbal cerebral palsy patients living outside of healthcare facilities need to be trained in recognition of life-threatening medical emergencies such as gastrointestinal obstruction and seek emergent attention at the earliest to prevent treatment delays.
小肠扭转(SBV)是指肠管围绕肠系膜轴异常扭转,导致肠梗阻和血管受压,进而引起肠缺血和坏死,危及生命。危险因素包括畸形、肠旋转不良和粘连。其发病率低且临床表现不典型,难以诊断,对于无法表达疼痛(这是首要且最突出症状)的无语言能力患者更是如此。研究表明脑瘫患者肠梗阻的发生率增加。目前尚无小肠扭转合并脑瘫的报道病例。我们报告一例21岁患有严重脑瘫和脊柱后凸侧弯的男性患者。该患者因呼吸窘迫和腹胀被送往急诊室。检查发现有急腹症。腹部X线显示气体分布提示小肠梗阻。患者病情迅速恶化,复苏尝试未成功。尸检发现腹腔内充满广泛扩张且壁薄的小肠肠袢。可见小肠扭转,在两个不同部位围绕肠系膜根部顺时针旋转360°。对肠管进行解剖时,未见黏膜皱襞,黏膜呈绿色并有散在出血区,符合缺血坏死表现。未发现任何畸形、肠旋转不良或粘连的证据。小肠扭转是一种罕见疾病,临床表现不具特异性,诊断具有挑战性。此次尸检强调,对于脑瘫患者肠梗阻病例,需高度怀疑小肠扭转,以便加快手术并预防死亡。生活在医疗机构外的无语言能力脑瘫患者的主要照料者需要接受培训,识别诸如胃肠道梗阻等危及生命的医疗紧急情况,并尽早寻求紧急救治以防止治疗延误。