Evidence map›Paper›PMID 42607031›Full record

ArticlePrenatal diagnosis2026

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

L Guibaud, M Bordas-Fournel, S Cabet, C Le Vaillant, A Bruel, T Lefrançois, C Megier, A Benachi, M Sohier, Y Gallois and 6 more

Abstract readCase Reports
In one paragraph

Article in Prenatal diagnosis, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

2 citing papers in PubMed.

  1. Article
  2. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

16 authors.

L GuibaudPediatric and Fetal Imaging, Reference Center for Superficial Vascular Anomalies, Hôpital Femme Mère Enfant, Université Claude Bernard Lyon 1, Lyon-Bron, France.ORCID https://orcid.org/0000-0002-5747-6654
M Bordas-FournelDepartment of Obstetrics and Gynecology, CHU Grenoble Alpes, Nantes, France.ORCID https://orcid.org/0009-0001-1697-3777
S CabetPediatric and Fetal Imaging, Reference Center for Superficial Vascular Anomalies, Hôpital Femme Mère Enfant, Université Claude Bernard Lyon 1, Lyon-Bron, France.ORCID https://orcid.org/0000-0003-0115-2586
C Le VaillantMultidisciplinary Center for Prenatal Diagnosis, CHU de Nantes, Nantes, France.
A BruelService de Néphro-Pédiatrie, CHU de Nantes, Nantes, France.
T LefrançoisService d'Imagerie Pédiatrique, CHU de Nantes, Nantes, France.
C MegierMultidisciplinary Center for Prenatal Diagnosis, Hôpital Antoine-Béclère, AP-HP, Paris, France.
A BenachiMultidisciplinary Center for Prenatal Diagnosis, Hôpital Antoine-Béclère, AP-HP, Paris, France.ORCID https://orcid.org/0000-0001-6045-0765
M SohierMultidisciplinary Center for Prenatal Diagnosis, Maternité Paule de Viguier, CHU de Toulous, Toulouse, France.
Y GalloisORL Pédiatrique, Hopital Pierre Paul Riquet, CHU de Toulous, Toulouse, France.ORCID https://orcid.org/0000-0003-3197-8290
T SimonService de Néphrologie Pédiatrique, Hôpital des Enfants-Purpan, CHU de Toulous, Toulouse, France.
J-M JouannicMultidisciplinary Center for Prenatal Diagnosis, Hôpital Trousseau, AP-HP, Paris, France.
G CanaudINSERM U1151, Necker Enfants Malades Institute, Paris, France.
A GuilhemPediatric and Fetal Imaging, Reference Center for Superficial Vascular Anomalies, Hôpital Femme Mère Enfant, Université Claude Bernard Lyon 1, Lyon-Bron, France.
A AtallahMultidisciplinary Center for Prenatal Diagnosis, Hôpital Femme Mère Enfant, Lyon-Bron, France.ORCID https://orcid.org/0000-0002-4805-2083
A FraissenonPediatric and Fetal Imaging, Reference Center for Superficial Vascular Anomalies, Hôpital Femme Mère Enfant, Université Claude Bernard Lyon 1, Lyon-Bron, France.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

objectiveMaternal 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.

methodsBetween 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. Prenatal ultrasound and fetal magnetic resonance imaging were performed. After maternal evaluation and informed consent, sirolimus was administered with weekly dose adjustments based on maternal trough levels. Tolerance, drug concentrations at delivery, obstetric outcomes, and neonatal evolution were assessed. Decrease in lesion extent was assessed using a semi-quantitative 0-4 imaging scale.

resultsSix fetuses received therapy after the exclusion of nine cases. Treatment began between 24 and over 30 weeks' gestation. Maternal adverse effects were mild. Transplacental drug transfer was observed with variable maternal-fetal ratios. Mean gestational age at birth was 38.1 weeks. Partial or marked lesion regression occurred in five newborns.

conclusionsMaternal sirolimus therapy appears feasible and well tolerated for extensive fetal lymphatic malformations, demonstrating transplacental transfer and encouraging early perinatal outcomes in carefully selected pregnancies overall.

Indexed as

Fetal DiseasesLymphatic AbnormalitiesSirolimusAdultFemaleHumansImmunosuppressive AgentsInfant, NewbornPregnancyPregnancy OutcomeUltrasonography, PrenatalImmunosuppressive AgentsSirolimus

Identifiers

PMID42607031
PMCPMC13571794

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.