Maternal Sirolimus Therapy for Fetal Extensive Lymphatic Cystic Malformation: Selection Criteria, Management, and Outcomes in a Series of Six Cases

Guibaud L, Bordas-Fournel M, Cabet S, Le Vaillant C, Bruel A, Lefrançois T, Megier C, Benachi A, Sohier M, Gallois Y, Simon T, …, Fraissenon A · 2026 · Prenatal Diagnosis · Atlas ID GUIB2026

What this study shows

Maternal rapamycin (sirolimus) therapy for extensive fetal lymphatic malformations. Prenatal mTOR inhibition feasible with partial/marked lesion regression in 5/6 newborns; transplacental transfer observed. Observational case series (n=6); confounding by indication not excluded.

Abstract

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Maternal sirolimus therapy has emerged as a potential prenatal treatment for extensive fetal lymphatic malformations, particularly when associated with the risk of neonatal airway compromise. Data on prenatal indications, pharmacokinetics, and outcomes remain limited. We aimed to describe patient selection, treatment management, maternal and fetal safety, and early outcomes. Between December 2023 and May 2025, 15 fetuses with extensive cystic or mixed vascular anomalies suggestive of lymphatic malformations were referred to a national reference center.

Read the full abstract on PubMed →

At a glance

Evidence type	H Human study Marked H because it is direct evidence from a human clinical trial or human cohort; the code names the kind of study, not its quality -- a small, well-run trial is still H.
Study type	3 - Human Observational
Model system	Humans, observational case series (pregnant women and fetuses, n=6)
Journal	Prenatal Diagnosis
Year	2026
Peer reviewed	Yes
Record last updated	2026-10-01
Source	DOI 10.1002/pd.70230 · PMID 42607031

Extracted findings

These fields were extracted from the paper with AI assistance and have not been checked against the source. Read them as a pointer into the paper, not as curated claims; where they disagree with the curated summary above, the curated summary stands.

Intervention	Maternal sirolimus (rapamycin) during pregnancy, dose-adjusted to maternal trough levels
Target	mTOR
Model	Human (pregnant women and fetuses, n=6)
Effect	Partial or marked regression of extensive fetal lymphatic malformation occurred in five of six newborns, with transplacental drug transfer and mild maternal side effects.

In the Atlas

Cite this paper

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Guibaud, L., Bordas-Fournel, M., Cabet, S., Le Vaillant, C., Bruel, A., Lefrançois, T., Megier, C., Benachi, A., Sohier, M., Gallois, Y., Simon, T., Jouannic, J. M., Canaud, G., Guilhem, A., Atallah, A., & Fraissenon, A. (2026). Maternal Sirolimus Therapy for Fetal Extensive Lymphatic Cystic Malformation: Selection Criteria, Management, and Outcomes in a Series of Six Cases. Prenatal Diagnosis. https://doi.org/10.1002/pd.70230

@article{GUIB2026,
  author       = {Guibaud, L. and Bordas-Fournel, M. and Cabet, S. and Le Vaillant, C. and Bruel, A. and Lefrançois, T. and Megier, C. and Benachi, A. and Sohier, M. and Gallois, Y. and Simon, T. and Jouannic, J. M. and Canaud, G. and Guilhem, A. and Atallah, A. and Fraissenon, A.},
  title        = {{Maternal Sirolimus Therapy for Fetal Extensive Lymphatic Cystic Malformation: Selection Criteria, Management, and Outcomes in a Series of Six Cases}},
  journal      = {Prenatal Diagnosis},
  year         = {2026},
  doi          = {10.1002/pd.70230},
  note         = {PMID: 42607031},
}

Cite this Atlas record

The record is the Atlas's own work — the evidence label, the extracted findings and the links. It is cited as part of the dataset, not as the paper.

Barton, O. (2026). Oliver's mTOR Atlas (record GUIB2026) [Data set]. https://mtor-atlas.org/study/GUIB2026/ · Dataset DOI 10.5281/zenodo.22059963

@misc{atlas_GUIB2026,
  author       = {Barton, Oliver},
  title        = {{Oliver's mTOR Atlas}, record GUIB2026},
  howpublished = {Data set},
  year         = {2026},
  url          = {https://mtor-atlas.org/study/GUIB2026/},
  doi          = {10.5281/zenodo.22059963}
}

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