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


      • 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.


      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.


      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).


      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.



      CPAM (congenital pulmonary airway malformation), CCAM (congenital cystic adenomatoid malformation), BPS (bronchopulmonary sequestration), TAS (thoraco-amniotic shunt), CPAP (continuous positive airway pressure)
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Journal of Pediatric Surgery
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Stocker J.T.
        Congenital pulmonary airway malformation: a new name and expanded classification of congenital cystic adenomatoid malformation of the lung.
        Histopathology. 2002; 41: 424-431
        • Paramalingam S.
        • Parkinson E.
        • Sellars M.
        • et al.
        Congenital segmental emphysema: an evolving lesion.
        Eur J Pediatr Surg. 2010; 20: 78-81
        • Adzick N.S.
        • Flake A.W.
        • Crombleholme T.M.
        Management of congenital lung lesions.
        Semin Pediatr Surg. 2003; 12: 10-16
        • Adzick N.S.
        • Harrison M.R.
        • Crombleholme T.M.
        • et al.
        Fetal lung lesions: management and outcome.
        Am J Obstet Gynecol. 1998; 179: 884-889
        • Davenport M.
        • Warne S.A.
        • Cacciaguerra S.
        • et al.
        Current outcome of antenally diagnosed cystic lung disease.
        J Pediatr Surg. 2004; 39: 549-556
        • Nicolaides K.H.
        • Blott M.
        • Greenough A.
        Chronic drainage of fetal pulmonary cyst.
        Lancet. 1987 Mar 14; 1: 618
        • Blott M.
        • Nicolaides K.H.
        • Greenough A.
        Postnatal respiratory function after chronic drainage of fetal pulmonary cyst.
        Am J Obstet Gynecol. 1988; 159: 858-859
        • Booth P.
        • Nicolaides K.H.
        • Greenough A.
        • et al.
        Pleuro-amniotic shunting for fetal chylothorax.
        Early Hum Dev. 1987; 15: 365-367
        • Nicolaides K.H.
        • Azar G.B.
        Thoraco-Amniotic Shunting.
        Fetal Diagn Ther. 1990; 5: 153-164
      1. National Institute for Health and Care Excellence (NICE). Interventional procedures guidance Insertion of pleuro–amniotic shunt for fetal pleural effusion 2006.

        • Cavoretto P.
        • Molina F.
        • Poggi S.
        • et al.
        Prenatal diagnosis and outcome of echogenic fetal lung lesions.
        Ultrasound Obstet Gynecol. 2008; 32: 769-783
        • Kemp J.
        • Davenport M.
        • Pernet A.
        Antenatally diagnosed surgical anomalies: the psychological effect of parental antenatal counseling.
        J Pediatr Surg. 1998; 33: 1376-1379
        • Singh R.
        • Davenport M.
        The argument for operative approach to asymptomatic lung lesions.
        Semin Pediatr Surg. 2015; 24: 187-195
        • Thompson H.
        • Jones C.
        • Pardy C.
        • Kufeji D.
        • Nichols E.
        • Murphy F.
        • Davenport M
        Application of the Clavien-Dindo classification to a pediatric surgical network.
        J Pediatr Surg. 2020; 55: 312-315
        • Muntean A.
        • Banias L.E.
        • Ade-Ajayi N.
        • et al.
        Neonatal congenital pulmonary airway malformation associated with mucinous adenocarcinoma and KRAS mutations.
        J Pediatr Surg. 2022; 43
        • Grethel E.J.
        • Wagner A.J.
        • Clifton M.S.
        • et al.
        Fetal intervention for mass lesions and hydrops improves outcome: a 15-year experience.
        J Pediatr Surg. 2007; 42: 117-123
        • Yinon Y.
        • Grisaru-Granovsky S.
        • Chaddha V.
        • et al.
        Perinatal outcome following fetal chest shunt insertion for pleural effusion.
        Ultrasound Obstet Gynecol. 2010; 36: 58-64
        • Wilson R.D.
        • Baxter J.K.
        • Johnson M.P.
        • et al.
        Thoracoamniotic shunts: fetal treatment of pleural effusions and congenital cystic adenomatoid malformations.
        Fetal Diagn Ther. 2004; 19: 413-420
        • Brown R.
        • Nicolaides K.
        Constriction band of the arm following insertion of a pleuro-amniotic shunt.
        Ultrasound Obstet Gynecol. 2000; 15: 439-440
        • Peranteau W.H.
        • Scott Adzick N.
        • Boelig M.M.
        • et al.
        Thoracoamniotic shunts for the management of fetal lung lesions and pleural effusions: a single-institution review and predictors of survival in 75 cases.
        J Pediatr Surg. 2015; 50: 301-305
        • Abbasi N.
        • Windrim R.
        • Keunen J.
        • et al.
        Perinatal outcome in fetuses with dislodged thoraco-amniotic shunts.
        Fetal Diagn Ther. 2021; 48: 430-439
        • Schrey S.
        • Kelly E.N.
        • Langer J.C.
        • et al.
        Fetal thoracoamniotic shunting for large macrocystic congenital cystic adenomatoid malformations of the lung.
        Ultrasound Obstet Gynecol. 2012; 39: 515-520
        • Litwińska M.
        • Litwińska E.
        • Janiak K.
        • et al.
        Thoracoamniotic shunts in macrocystic lung lesions: case series and review of the literature.
        Fetal Diagn Ther. 2017; 41: 179-183
        • Rosenblat O.
        • Furman Y.
        • Kimhi G.
        • et al.
        In-utero treatment of prenatal thoracic abnormalities by thoraco-amniotic shunts, short and long term neuro developmental outcome: a single center experience.
        J Pediatr Surg. 2022; 57: 364-368
        • Witlox R.S G M
        • et al.
        Long-term neurodevelopmental and respiratory outcome after intrauterine therapy for fetal thoracic abnormalities.
        Fetal Diag Therap. 2019; 45: 162-167
        • Kelly E.N.
        • Seaward G.
        • Ye X.Y.
        • Windrim R.
        • Van Mieghem T.
        • Keunen J.
        • Abbasi N.
        • Chitayat D.
        • Ryan G
        Short- and long-term outcome following thoracoamniotic shunting for fetal hydrothorax.
        Ultrasound Obstet Gynecol. 2021; 57: 624-630
        • Dunn A.
        • Pearce K.
        • Callister R.
        • et al.
        Exercise capacity is not decreased in children who have undergone lung resection early in life for congenital thoracic malformations compared to healthy age-matched children.
        Pediatr Pulmonol. 2017; 52: 1340-1348
        • Merchant A.M.
        • Peranteau W.
        • Wilson R.D.
        • et al.
        Postnatal chest wall deformities after fetal thoracoamniotic shunting for congenital cystic adenomatoid malformation.
        Fetal Diagn Ther. 2007; 22: 435-439