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Klippel-Trénaunay syndrome (KTS) is a rare congenital disorder defined by a triad of capillary malformation, venous malformation, and soft tissue or bone hypertrophy most commonly affecting unilateral lower limbs. Due to the rarity of KTS, evidence-based guidelines for the management of pregnancy in people with KTS are still lacking. A 34-year-old woman (gravidity 1; parity 0) presented at 25 weeks of gestation with malformations of the right side of her body. The extent of the KTS affecting the vulva, pelvis, and right leg was remarkable. As the prenatal MRI showed massive vascular malformations of the pelvis and vulva, we performed an elective cesarean section to avoid severe perinatal hemorrhage during a vaginal delivery. Intraoperatively, we observed varices on the parietal peritoneum within the vesico-uterine pouch and the isthmocervical transition of the uterus, which were not identifiable in the preoperative MRI. Although KTS patients have been discouraged from pregnancy in the past because of a high risk for complications, successful and uncomplicated pregnancies are possible. For this purpose, we believe a multidisciplinary strategy that is crucial.

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Effektivere hämatoonkologische Therapien sowie Verbesserungen von Langzeitüberleben rücken Fertilität und Familienplanung in den Fokus. Insbesondere bei hohem Therapiedruck ist kurzfristige interdisziplinäre Zusammenarbeit bedeutend. Der Artikel zeigt den Versorgungsstand und -bedarf im Rhein-Main-Gebiet exemplarisch anhand der Universitätsmedizin Mainz (UM) sowie einer online durchgeführten Umfrage im regionalen hämatoonkologischen Zirkel Anfang 2022 auf.

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