Ko Dayoung, Yang Hee-Beom, Youn Joong, Kim Hyun-Young
Department of Pediatric Surgery, Seoul National University Children's Hospital, Seoul 03080, Korea.
Department of Surgery, Seoul National University Bundang Hospital, Seongnam-si 13620, Korea.
J Clin Med. 2021 May 28;10(11):2376. doi: 10.3390/jcm10112376.
Chronic intestinal pseudo-obstruction (CIPO) is an extremely rare condition with symptoms of recurrent intestinal obstruction without any lesions. The outcomes of pediatric CIPO and predictors for the outcomes have not yet been well established. We analyzed the clinical outcomes and associated factors for the outcomes of pediatric CIPO. We retrospectively reviewed 66 primary CIPO patients diagnosed between January 1985 and December 2017. We evaluated parenteral nutrition (PN) factors such as PN duration, PN use over 6 months, home PN, and mortality as outcomes. We selected onset age, presence of urologic symptoms, pathologic type, and involvement extent as predictors. The early-onset CIPO was found in 63.6%, and 21.2% of the patients presenting with urologic symptoms. Of the 66 patients, 47 and 11 had neuropathy and myopathy, respectively. The generalized involvement type accounted for 83.3% of the cases. At the last follow-up, 24.2% of the patients required home PN management. The mean duration of PN was 11.8 ± 21.0 months. The overall mortality rate of primary CIPO was 18.2%. PN factors were predicted by the urologic symptoms and extent of involvement. However, mortality was predicted by pathologic type. The onset age was not significantly associated with the outcomes. CIPO with urologic symptoms and generalized CIPO had poor PN outcomes. Myopathy is suggested as a predictor of mortality in children with primary CIPO.
慢性假性肠梗阻（CIPO）是一种极为罕见的疾病，具有反复肠梗阻症状但无任何病变。小儿CIPO的预后及预后预测因素尚未完全明确。我们分析了小儿CIPO的临床预后及相关影响因素。我们回顾性分析了1985年1月至2017年12月期间诊断的66例原发性CIPO患者。我们将肠外营养（PN）相关因素如PN持续时间、6个月以上PN使用情况、家庭PN以及死亡率作为预后指标。我们选择发病年龄、泌尿系统症状的存在、病理类型和受累范围作为预测因素。发现63.6%为早发型CIPO，21.2%的患者伴有泌尿系统症状。66例患者中，分别有47例和11例患有神经病变和肌病。广泛受累类型占病例的83.3%。在最后一次随访时，24.2%的患者需要家庭PN管理。PN的平均持续时间为11.8±21.0个月。原发性CIPO的总体死亡率为18.2%。PN相关因素可由泌尿系统症状和受累程度预测。然而，死亡率由病理类型预测。发病年龄与预后无显著相关性。伴有泌尿系统症状的CIPO和广泛性CIPO的PN预后较差。肌病被认为是原发性CIPO患儿死亡率的一个预测因素。
