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The long-term outcome following thoraco-amniotic shunting for congenital lung malformations

      HIGHLIGHTS

      • Antenatal thoracoamniotic shunt insertion for large congenital lung malformations is well tolerated in fetuses with or at risk of hydrops.
      • postnatal survival and long-term outcome is excellent.

      Abstract

      Aim of the Study

      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.

      Methods

      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).

      Main results

      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).

      Conclusions

      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.

      Keywords

      Abbreviations:

      CPAM (congenital pulmonary airway malformation), CCAM (congenital cystic adenomatoid malformation), BPS (bronchopulmonary sequestration), TAS (thoraco-amniotic shunt), CPAP (continuous positive airway pressure)
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