A 7-day-old baby with an AV malformation

This challenging case explores the management of a rare and aggressive vascular tumor in a newborn.

A 7-day-old baby presents with a rapidly enlarging kaposiform hemangioendothelioma (KHE), complicated by significant vascular malformation and a high risk of life-threatening bleeding.

What treatment strategy would you choose to control the tumor and its severe coagulopathy while minimising long-term harm?

Giovanni F. Torsello

University Medical Center Göttingen, Germany

Giovanni B. Torsello

University Medical Center Göttingen, Germany

Part I - Case presentation

A 7-day-old baby.
The skin surface is dark red. The local skin temperature is increased.
Lab: decline in platelet count followed by ecchymoses.

7-days old baby with AV malformation: The skin surface is dark red in color. The local skin temperature is increased.

MR-angiography

AV malformation with multiple feeding arteries and irregular capillary blush with non-uniform distribution.

Normal arteries are embedded in the tumor blush.

7-days old baby with AV malformation: AV malformation with multiple feeding arteries

MR: the tumor rapidly increased in size and spread to surrounding areas.
Diagnosis: kaposiform hemangioendothelioma (KHE)

This is a locally aggressive vascular tumor with high morbidity and mortality.

The tumor usually causes systemic disseminated intravascular coagulation, and young patients die because of severe bleeding.