Work

PHACE syndrome in a preterm infant

Year: 2020

Type: article

Abstract: In a preterm infant (28 weeks), postnatal cranial ultrasound showed unilateral cerebellar hypoplasia. On day 5, a facial erythematous lesion developed, progressing to a segmental hemangioma during the next 2 weeks (figure 1, A and B). PHACE syndrome (posterior fossa anomalies, most commonly located in the mid brain or hindbrain, such as the Dandy-Walker complex and focal dysplasia and/or hypoplasia of the cerebellum, hemangioma, arterial lesions, cardiac abnormalities or coarctation of the aorta, eye or endocrine abnormalities)1 was suspected. MRI confirmed cerebellar hypoplasia and intracranial hemangioma (figure 2). Magnetic resonance angiography and echocardiogram were normal. Because of obstruction of the visual axis, low-dose atenolol was started (0.5–1.0 mg/kg/d), and continued for 2 years.

Source: Neurology

DOI: 10.1212/wnl.0000000000010800

Authors: Sylke J. Steggerda, Ratna N.G.B. Tan, Peter C.J. de Laat

Institutions: Leiden University, Leiden University Medical Center, Erasmus MC

Language: English

Topic: Vascular Malformations and Hemangiomas

Subfield: Surgery

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: bronze

OpenAlex ID: W3086806510