A case report of sirolimus use in early fetal management of lymphatic malformation.

Autor: Seront E; Institut Roi Albert II, Department of Medical Oncology, Center for Vascular Anomalies, Saint-Luc University Hospital, UCLouvain, VASCERN VASCA European Reference Centre, Brussels, Belgium., Biard JM; Fetal Medicine Unit, Department of Obstetrics, Center for Vascular Anomalies, Saint-Luc University Hospital, UCLouvain, VASCERN VASCA European Reference Centre, Brussels, Belgium., Van Damme A; Institut Roi Albert II, Department of Pediatric Hematology & Oncology, Center for Vascular Anomalies, Saint-Luc University Hospital, UCLouvain, VASCERN VASCA European Reference Centre, Brussels, Belgium., Revencu N; Center for Human Genetics, Center for Vascular Anomalies, Saint-Luc University Hospital, UCLouvain, VASCERN VASCA European Reference Centre, Brussels, Belgium., Lengelé B; Division of Plastic Surgery, Center for Vascular Anomalies, Saint-Luc University Hospital, UCLouvain, VASCERN VASCA European Reference Centre, Brussels, Belgium., Schmitz S; Division of ENT, Center for Vascular Anomalies, Saint-Luc University Hospital, UCLouvain, VASCERN VASCA European Reference Centre, Brussels, Belgium., de Toeuf C; Division of ENT, Center for Vascular Anomalies, Saint-Luc University Hospital, UCLouvain, VASCERN VASCA European Reference Centre, Brussels, Belgium., Clapuyt P; Department of Pediatric Radiology, Center for Vascular Anomalies, Saint-Luc University Hospital, UCLouvain, VASCERN VASCA European Reference Centre, Brussels, Belgium., Veyckemans F; Department of Anesthesiology, Center for Vascular Anomalies, Saint-Luc University Hospital, UCLouvain, VASCERN VASCA European Reference Centre, Brussels, Belgium., Prégardien C; Department of Anesthesiology, Center for Vascular Anomalies, Saint-Luc University Hospital, UCLouvain, VASCERN VASCA European Reference Centre, Brussels, Belgium., Vikkula M; Human Molecular Genetics, de Duve Institute, UCLouvain, Brussels, Belgium & Center for Vascular Anomalies, Saint-Luc University Hospital, UCLouvain, VASCERN VASCA European Reference Centre, Brussels, Belgium., Bernard P; Fetal Medicine Unit, Department of Obstetrics, Center for Vascular Anomalies, Saint-Luc University Hospital, UCLouvain, VASCERN VASCA European Reference Centre, Brussels, Belgium., Boon LM; Division of Plastic Surgery, Center for Vascular Anomalies, Saint-Luc University Hospital, UCLouvain, VASCERN VASCA European Reference Centre, Brussels, Belgium. laurence.boon@saintluc.uclouvain.be.
Jazyk: angličtina
Zdroj: Nature cardiovascular research [Nat Cardiovasc Res] 2023 Jun; Vol. 2 (6), pp. 595-599. Date of Electronic Publication: 2023 Jun 01.
DOI: 10.1038/s44161-023-00280-4
Abstrakt: Sirolimus, by targeting the mammalian target of rapamycin (mTOR) pathway, has demonstrated efficacy on lymphatic malformations (LMs) in adults and neonates. The current hypothesis is that the earlier the lesion is treated, the better it responds. This has prompted the idea that sirolimus administration might be efficacious to treat fetal LMs as well. Here we report a successful management of a cervicofacial fetal LM with sirolimus taken orally by the mother from the 22nd week of pregnancy until 2 weeks before planned delivery. Repeated cordocentesis recorded a 30% transplacental crossing of sirolimus. Continuation of sirolimus after birth allowed resection of the residual mass. We have followed the physical and neurological evolution of the child for 6 years since the fetal administration of sirolimus. We conclude that early administration of sirolimus during pregnancy with maternal serum monitoring may be proposed to high-risk fetal LMs in selected cases.
(© 2023. The Author(s), under exclusive licence to Springer Nature Limited.)
Databáze: MEDLINE
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