Fetal Sclerotherapy for Hydropic Congenital Cystic Adenomatoid Malformations of the Lung Refractory to Steroids: A Case Report and Review of the Literature.

Nimrah Abbasi, Anne-Maude Morency, Jacob C Langer, Priscilla P L Chiu, Rose Chami, Rory Windrim, Johannes Keunen, Gareth Seaward, Greg Ryan

Journal: Fetal diagnosis and therapy 2020;47(1):24-33

PMID: 31112955

Abstract

Microcystic congenital cystic adenomatoid malformations (CCAM), when associated with hydrops, carry a dismal prognosis. Options for treatment are limited and experimental, including antenatal corticosteroids, open fetal surgery, laser ablation and, more recently, sclerotherapy. We describe a case of a large, predominantly microcystic CCAM in a hydropic fetus treated successfully with direct interstitial injection of a sclerosant agent (3% sodium tetradecyl sulfate) at 23+3 weeks gestation, after multiple failed courses of steroids. Elective thoracoscopic right lower lobectomy was performed at 1 year of life and there have been no respiratory or other medical morbidities since. A literature review of fetal lung masses treated with sclerosants antenatally reveals that sclerotherapy may represent a novel treatment option for large hydropic microcystic CCAMs, which are unresponsive to corticosteroids. Further studies are required to evaluate the utility and safety of fetal sclerotherapy, as this may represent an alternative minimally invasive treatment option to fetal lobectomy.

© 2019 S. Karger AG, Basel.

Address: Fetal Medicine Unit, Ontario Fetal Centre, Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynaecology, Mount Sinai Hospital, Toronto, Ontario, Canada, [email protected].; University of Toronto, Toronto, Ontario, Canada, [email protected].; Fetal Medicine Unit, Ontario Fetal Centre, Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynaecology, Mount Sinai Hospital, Toronto, Ontario, Canada.; University of Toronto, Toronto, Ontario, Canada.; Division of General and Thoracic Surgery, Department of Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada.; Department of Paediatric Laboratory Medicine, The Hospital for Sick Children, Toronto, Ontario, Canada.

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