Muntean Ancuta, Cazacu Ramona, Ade-Ajayi Niyi, Patel Shailesh B, Nicolaides Kypros, Davenport Mark
Departments of Paediatric Surgery, Kings College Hospital, London, UK.
Harris Birthright Research Centre for Fetal Medicine, Kings College Hospital, London, UK.
J Pediatr Surg. 2023 Feb;58(2):213-217. doi: 10.1016/j.jpedsurg.2022.10.028. Epub 2022 Oct 23.
Insertion of a thoraco amniotic shunt (TAS) during fetal life is a therapeutic option where there is a high risk of death secondary to large congenital lung malformations (CLM). The aim of this study is to present our center's long-term experience.
Retrospective single center review of the period (Jan 2000-Dec 2020). We included all fetuses that underwent TAS insertion for CLM with detailed analysis of those live newborns managed in our center. Data are quoted as median (range).
Thirty one fetuses underwent 37 TAS insertions at a 25 (20-30) weeks gestational age. This was successful on 1st attempt in 30 (97%) fetuses. In 6 cases a 2nd shunt was required at 6.5 (2-10) weeks following the 1st insertion. Twenty-eight survived to be born. Sixteen (9 male) infants were delivered in our center at 39 (36-41) weeks gestational age and birth weight of 3.1 (2.6-4.2) kg. All infants underwent surgery at 2 (0-535) days (emergency surgery, n = 9; expedited n = 4; elective surgery, n = 3). Final histopathology findings were CPAM Type 1 (n = 14, n.b. associated with mucinous adenocarcinoma, n = 1), CPAM Type 2 (n = 1) and an extralobar sequestration (n = 1). Postoperative stay was 16 (1-70) days with survival in 15/16 (94%). One infant died at 1 day of life secondary to a combination of pulmonary hypoplasia and hypertension. Median follow up period was 10.7 (0.4-20.4) years. Nine (60%) children developed a degree of chest wall deformity though none have required surgical intervention. Clinically, 14/15 (93%) have otherwise normal lung function without limitations of activity, sporting or otherwise. One child has a modest exercise limitation (FVC - 70% predicted).
TAS insertion is associated with high perinatal survival and should be considered in fetuses at risk of hydrops secondary to large cystic lung malformation. Their long term outcome is excellent although most have a mild degree of chest wall deformity.
对于因大型先天性肺畸形（CLM）继发死亡风险较高的情况，在胎儿期插入胸羊膜分流管（TAS）是一种治疗选择。本研究的目的是介绍我们中心的长期经验。
对2000年1月至2020年12月期间进行回顾性单中心研究。我们纳入了所有因CLM接受TAS插入的胎儿，并对在我们中心管理的存活新生儿进行详细分析。数据以中位数（范围）表示。
31例胎儿在孕25（20 - 30）周时接受了37次TAS插入。30例（97%）胎儿首次尝试即成功。6例在首次插入后6.5（2 - 10）周需要再次插入分流管。28例存活至出生。16例（9例男性）婴儿在孕39（36 - 41）周时在我们中心分娩，出生体重为3.1（2.6 - 4.2）kg。所有婴儿在2（0 - 535）天接受手术（急诊手术9例；加急手术4例；择期手术3例）。最终组织病理学结果为1型先天性肺气道畸形（CPAM）（14例，其中1例伴有黏液腺癌）、2型CPAM（1例）和肺叶外隔离症（1例）。术后住院时间为16（1 - 70）天，16例中有15例（94%）存活。1例婴儿在出生1天时因肺发育不全和高血压合并症死亡。中位随访期为10.7（0.4 - 20.4）年。9例（60%）儿童出现一定程度的胸壁畸形，不过均无需手术干预。临床上，15例中有14例（93%）肺功能正常，活动、运动等均无限制。1例儿童有轻度运动受限（预测用力肺活量 - 70%）。
插入TAS与围产期高存活率相关，对于因大型囊性肺畸形继发有水肿风险的胎儿应予以考虑。尽管大多数有轻度胸壁畸形，但他们的长期预后良好。
