Destro Francesca, Maestri Luciano, Meroni Milena, Campari Alessandro, Pederiva Federica, Costanzo Sara, Del Re Giulia, Roveri Margherita, Zuccotti Gianvincenzo, Calcaterra Valeria, Pelizzo Gloria
Pediatric Surgery Department, "Vittore Buzzi" Children's Hospital, 20154 Milano, Italy.
Pediatric Radiology Department, "Vittore Buzzi" Children's Hospital, 20154 Milano, Italy.
Children (Basel). 2021 Oct 30;8(11):982. doi: 10.3390/children8110982.
Colonic volvulus (CV) is a rare but potentially life-threatening condition with unclear etiopathogenesis. To date, less than 80 pediatric cases have been described. Hirschsprung's disease (HD) is associated with CV in 17% of cases, representing a significant risk factor. Non-HD CV is an even more complex entity. The aim of this study is to describe a series of patients with CV to accentuate some peculiar aspects of this disease. We performed a retrospective study (period: 2012-2021) collecting information of patients with CV. Data analyzed included: demographics, medical history, presenting symptoms and radiological and surgical details. Eleven patients (12.5 ± 2.8 years; 7F/4M) had CV (eight sigmoid, two transverse colon, one total colon). Five patients had associated anomalies and three had HD. A two-step approach with volvulus endoscopic/radiological detorsion followed by intestinal resection was attempted in eight cases (one endoscopic approach failed). Three patients required surgery at admission. At follow-up, two patients developed recurrent intestinal obstruction, one of whom also had anastomotic stenosis. Colonic volvulus is a challenging condition that requires prompt patient care. A missed diagnosis could lead to severe complications. The evaluation of the patient should include a careful histological examination (searching for HD and alpha-actin deficiency), immunologic and metabolic screening, neurological tests and detection of chronic intestinal pseudo-obstruction (CIPO). Lifelong follow-up is mandatory for the early recognition and treatment of progressive diseases involving the proximal gastrointestinal tract.
结肠扭转（CV）是一种罕见但可能危及生命的疾病，其病因发病机制尚不清楚。迄今为止，已报道的儿科病例不到80例。17%的病例中，先天性巨结肠病（HD）与CV相关，这是一个重要的危险因素。非HD型CV是一个更为复杂的实体。本研究的目的是描述一系列CV患者，以突出这种疾病的一些特殊方面。我们进行了一项回顾性研究（时间段：2012 - 2021年），收集CV患者的信息。分析的数据包括：人口统计学、病史、症状表现以及放射学和手术细节。11名患者（12.5 ± 2.8岁；7名女性/4名男性）患有CV（8例为乙状结肠扭转，2例为横结肠扭转，1例为全结肠扭转）。5名患者伴有其他异常，3名患者患有HD。8例患者尝试了两步法，即先进行扭转的内镜/放射学复位，然后进行肠切除术（1例内镜复位失败）。3名患者入院时即需要手术。在随访中，2名患者出现复发性肠梗阻，其中1例还伴有吻合口狭窄。结肠扭转是一种具有挑战性的疾病，需要对患者进行及时治疗。漏诊可能导致严重并发症。对患者的评估应包括仔细的组织学检查（寻找HD和α-肌动蛋白缺乏症）、免疫和代谢筛查、神经学检查以及慢性假性肠梗阻（CIPO）的检测。对于涉及近端胃肠道的进行性疾病，必须进行终身随访，以便早期识别和治疗。
