微囊型先天性肺气道畸形伴胎儿水肿：类固醇与开放胎儿切除术的比较。

Microcystic congenital pulmonary airway malformation with hydrops fetalis: steroids vs open fetal resection.

机构信息

Department of Surgery, University of California at San Francisco, San Francisco, CA 94143-0570, USA.

出版信息

J Pediatr Surg. 2012 Jan;47(1):36-9. doi: 10.1016/j.jpedsurg.2011.10.015.

DOI:10.1016/j.jpedsurg.2011.10.015

Abstract

BACKGROUND/PURPOSE: Congenital pulmonary airway malformations (CPAM) are rare lesions often diagnosed during routine prenatal ultrasound. The presence of hydrops fetalis is an indicator of poor prognosis. Here we present a retrospective review of fetuses undergoing either open fetal surgery or steroids for predominantly microcystic CPAM with hydrops fetalis.

METHOD

A retrospective review of patients undergoing open fetal surgery or steroids for CPAM at our institution was performed. The primary outcome was survival.

RESULTS

A retrospective review of all patients referred to our institution with the diagnosis of CPAM was performed. Fetuses with predominantly microcystic CPAM and the presence of hydrops fetalis treated with steroid or surgery were included. Thirteen patients were treated with steroids, and 11 patients underwent open fetal surgery. In the steroid group 12 (92%) of 13 fetuses survived to delivery versus 9 (82%) of 11 in the open fetal surgery group. Only 5 (56%) of 9 of the patients in the open fetal surgery group survived to neonatal discharge compared to 10 (83%) of 12 in the steroid group.

CONCLUSIONS

In the present retrospective study, improved survival was seen in fetuses with hydrops fetalis and predominantly microcystic CPAM treated with steroids when compared with open fetal surgery. Steroids should be considered for first-line therapy in these cases.

摘要

背景/目的：先天性肺气道畸形（CPAM）是一种罕见的病变，通常在常规产前超声检查中诊断。胎儿水肿的存在是预后不良的指标。在此，我们回顾性分析了接受开放胎儿手术或类固醇治疗主要为微囊型 CPAM 合并胎儿水肿的病例。

方法

对我院行开放胎儿手术或类固醇治疗 CPAM 的患者进行回顾性分析。主要结局为存活率。

结果

对我院诊断为 CPAM 的所有患者进行了回顾性分析。纳入主要为微囊型 CPAM 且存在胎儿水肿的患者，给予类固醇或手术治疗。13 例患者接受类固醇治疗，11 例患者行开放胎儿手术。在类固醇组，13 例胎儿中有 12 例（92%）存活至分娩，而在开放胎儿手术组中，11 例中有 9 例（82%）存活至分娩。在开放胎儿手术组中，只有 5 例（56%）患者存活至新生儿出院，而在类固醇组中，12 例中有 10 例（83%）存活至新生儿出院。