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.

Part II - Final strategy

Echo-guided puncture of the common femoral artery

Selective angiography of posterior tibial artery

Preparation of iodinated oil emulsion + polyvinyl alcohol + 350-500 micron particles

Selective injection of emulsion consisted of 2 ml bleomycin as sclerosant, and 2 ml iodinated oil (Lipiodol) as embolisation agent and particles

Perfusion reduction of the tumor by angiography and duplex scan

Normal perfusion of the foot and of posterior tibial artery and vein. Pedal arteries palpable.

7-days old baby with AV malformation: Normal perfusion of the foot and of posterior tibial artery and vein