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Prenatal diagnosis of Klippel–Trenaunay–Weber syndrome with Kasabach–Merritt syndrome in utero

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Abstract

Klippel-Trenaunay-Weber syndrome (KTWS), a congenital disease characterized by cutaneous hemangiomas, soft tissue and bone hypertrophy, and occasionally arteriovenous malformations, is extremely rare and its natural history in utero is unknown. We present a prenatally diagnosed case of KTWS complicated with Kasabach-Merritt syndrome in utero and fetal hydrops from acute anemia. The fetus was diagnosed with KTWS at 24 weeks of gestation based on the ultrasound findings of hemangiomas and unilateral hypertrophy of the lower extremity. Acute enlargement of the hemangiomas and the appearance of new retroperitoneal hemangiomas were detected at 27 weeks, along with skin edema and cardiomegaly. Doppler examination showed elevated peak systolic velocity in the middle cerebral artery, indicating acute fetal anemia. We believe the fetus’s condition was complicated with Kasabach-Merritt syndrome in utero, which caused acute hemolytic anemia leading to high-output cardiac failure and fetal hydrops.

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Conflict of interest

The authors, Kei Tanaka, Noriko Miyazaki, Miho Matsushima, Reiko Yagishita, Tomoko Izawa, Shinji Tanigaki, Keiji Sakai, and Mitsutoshi Iwashita, have no conflicts of interest, sources of financial support, corporate involvement, or patent holdings to disclose.

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All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the Helsinki Declaration of 1975, as revised in 2008 (5). Informed consent was obtained from all patients for being included in the study.

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Correspondence to Kei Tanaka.

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Tanaka, K., Miyazaki, N., Matsushima, M. et al. Prenatal diagnosis of Klippel–Trenaunay–Weber syndrome with Kasabach–Merritt syndrome in utero. J Med Ultrasonics 42, 109–112 (2015). https://doi.org/10.1007/s10396-014-0557-5

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  • DOI: https://doi.org/10.1007/s10396-014-0557-5

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