Department of Paediatric Surgery, Temple Street Children University Hospital, Dublin, Ireland.
Department of Paediatric Surgery, Temple Street Children University Hospital, Dublin, Ireland.
J Pediatr Surg. 2021 Sep;56(9):1583-1589. doi: 10.1016/j.jpedsurg.2020.12.017. Epub 2021 Jan 1.
Exomphalos major (EM) is associated with significant morbidity and even mortality with an important risk of long-term pulmonary disease.
To assess the outcomes of exomphalos in a single tertiary pediatric unit and to identify prognostic factors for patients with respiratory insufficiency who still require ventilatory assistance at six months.
All infants admitted to our institution over a 10-year period (2005 to 2015) with exomphalos were retrospectively reviewed. EM was defined when the abdominal wall defect measured >= 5 cm and/or contained liver within the sac. Data were collected on patient demographics, prenatal course and imaging, birth information, immediate and long-term outcomes. Those with long-term respiratory insufficiency were identified as the primary outcome and reviewed to assess prognostic factors. A p value of ≤0.05 was regarded as significant. Data are quoted as median(range).
A total of 46 infants were diagnosed with exomphalos during the study period, with most (n = 30, 65%) defined as exomphalos major. Respiratory complications occurred in 16 (35%) with 8 (50%) of these requiring long-term (≥6 months) mechanical ventilation and 5 (31%) required a tracheostomy. On univariate analysis, resuscitation at birth (p = 0.0004), birth weight <3000 g (p = 0.008), use of nitric oxide (p = 0.004), high frequency oscillatory ventilation (HFOV) (p = 0.001), pulmonary hypoplasia (p<0.0001) and pulmonary hypertension (PHTN) (p = 0.02) were significantly associated with respiratory insufficiency. The strongest predictive model for ventilation support at six months was resuscitation at birth in combination with PH (OR = 1.57). Five infants (11%) died at 5(1-122) days.
In patients with EM, the presence of pulmonary hypertension along with resuscitation at birth are the most important prognostic factors for long-term respiratory insufficiency. Acknowledgement of these factors allows for better parental counselling regarding respiratory outcomes.
巨大脐膨出（EM）与显著的发病率相关，甚至导致死亡，且存在重要的长期肺部疾病风险。
评估单一三级儿科单位中脐膨出的结局，并确定仍需要 6 个月通气支持的呼吸功能不全患者的预后因素。
回顾性分析我院在 10 年间（2005 年至 2015 年）收治的所有患有脐膨出的婴儿。当腹壁缺陷≥5cm 且/或囊内含有肝脏时定义为巨大脐膨出。收集患者人口统计学、产前过程和影像学、出生信息、近期和长期结局的数据。将有长期呼吸功能不全的患者确定为主要结局，并进行回顾性评估预后因素。p 值≤0.05 被认为有统计学意义。数据以中位数（范围）表示。
研究期间共有 46 例婴儿被诊断为脐膨出，其中大多数（n=30，65%）被定义为巨大脐膨出。16 例（35%）发生呼吸并发症，其中 8 例（50%）需要长期（≥6 个月）机械通气，5 例（31%）需要气管切开术。单因素分析显示，出生时复苏（p=0.0004）、出生体重<3000g（p=0.008）、一氧化氮（p=0.004）、高频振荡通气（HFOV）（p=0.001）、肺发育不良（p<0.0001）和肺动脉高压（PHTN）（p=0.02）与呼吸功能不全显著相关。六个月时通气支持的最强预测模型是出生时复苏与 PH 的联合（OR=1.57）。5 例婴儿（11%）在 5（1-122）天死亡。
在患有 EM 的患者中，肺动脉高压的存在以及出生时的复苏是长期呼吸功能不全最重要的预后因素。认识到这些因素可以更好地为父母提供关于呼吸结局的咨询。
