Background

Severe primary fetal hydrothorax (PFH) and fetal lung lesions (FLL) such as congenital pulmonary airway malformation (CPAM) and Bronchopulmonary sequestration (BPS) are often treated by thoraco-amniotic shunt (TAS).

Objectives

To compare short and long-term outcome of fetuses treated by TAS due to FLL to those treated due to PFH.

Method

A retrospective analysis was performed for all fetuses treated by TAS, between the years 2004–2015, evaluating the short and long term neurodevelopmental outcome. Long term neurodevelopment was additionally analyzed prospectively by Vineland adaptive behavioral scale (VABS) standardized questionnaires.

Results

38 fetuses were treated by 52 TAS insertions; of which 13 (35%) due to FLL and 25 due to PFH. Perinatal survival was high (87.9%) with 3 neonatal death and one termination of pregnancy (TOP). High survival rate persisted even in cases requiring recurrent shunt insertion (80% survival). There was no significant difference in short or long term outcome including perinatal survival (84% Vs 90%, P = 0.64) and hydrops resolution (91% Vs 63%, p = 0.19). Long term outcome, including rate of neurodevelopmental abnormalities (23.5% Vs 20%) and VABS score (91.3 ± 13.3 Vs 96.4 ± 14.7), were similar for both groups.

Conclusion

TAS insertion is effective and resulting in high perinatal survival even in cases when sequential insertion is needed. Short and long- term outcome of neonates with FLL treated by TAS are comparable to neonates treated due to PFH.

中文翻译：

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通过胸-羊膜分流术治疗产前胸部异常、短期和长期神经发育结果：单中心经验

背景

严重的原发性胎儿胸腔积液 (PFH) 和胎儿肺部病变 (FLL)，例如先天性肺气道畸形 (CPAM) 和支气管肺隔离症 (BPS)，通常采用胸膜分流术 (TAS) 治疗。

目标

比较因 FLL 而接受 TAS 治疗的胎儿与因 PFH 治疗的胎儿的短期和长期结果。

方法

对 2004-2015 年间接受 TAS 治疗的所有胎儿进行回顾性分析，评估短期和长期神经发育结果。还通过 Vineland 适应性行为量表 (VABS) 标准化问卷对长期神经发育进行了前瞻性分析。

结果

38 名胎儿接受了 52 次 TAS 插入治疗；其中 13 例（35%）是由于 FLL，25 例是由于 PFH。围产期存活率高（87.9%），有 3 例新生儿死亡和 1 例终止妊娠（TOP）。即使在需要反复插入分流器的情况下（80% 的存活率），也能保持高存活率。短期或长期结果无显着差异，包括围产期存活率（84% 比 90%，P  = 0.64）和水肿消退（91% 比 63%，p  = 0.19）。两组的长期结果，包括神经发育异常率（23.5% 比 20%）和 VABS 评分（91.3 ± 13.3 比 96.4 ± 14.7）相似。

结论

TAS 插入是有效的，即使在需要顺序插入的情况下，也能提高围产期存活率。接受 TAS 治疗的 FLL 新生儿的短期和长期结果与接受 PFH 治疗的新生儿相当。