What is it about?

Klippel–Trenaunay syndrome (KTS) is a rare disease with a classic triad of port wine stains, varicose veins, and bony and soft tissue hypertrophy of an extremity. In this paper we present four prenatally (in utero) diagnosed cases of KTS and review of the literature for similar cases. The issues of prenatal diagnosis, complications, treatment and postnatal outcomes of this disease are also discussed.

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Why is it important?

The prognosis of KTS is usually favorable, but the quality of life is significantly affected. This makes the early diagnosis of this syndrome during the fetal life extremely impotant. Our cases series makes a valuable contribution to the global experience in ultrasound diagnosis of this anomaly. It also contains a unique case – the ectrodactyly of the hand ("claw-like", split hand) in fetus with KTS. Such a combination is very rare and has not been previously reported prenatally.

Perspectives

In all cases presented in the article, the referral diagnosis to our department did not include KTS. I hope that our article will help introduce fetal medicine specialists to this rare pathology and will be useful in their routine clinical practice.

Olga Ivanitskaya
Moscow Regional Research Institute of Obstetrics and Gynecology

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This page is a summary of: Prenatal diagnosis of Klippel–Trenaunay syndrome: Series of four cases and review of the literature, Ultrasound, October 2019, SAGE Publications,
DOI: 10.1177/1742271x19880327.
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