A case of phace syndrome and acquired hypopituitarism?

Background:
PHACE is a neurocutaneous syndrome associated with: Posterior fossa brain malformations,large "segmental" facial hemangiomas, arterial cerebrovascular-, cardiovascular-, and eyeanomalies.Case vignetteWe are reporting a girl with PHACE syndrome. The patient had a congenital right-sidedfacial hemangioma with plaque-morphology. At age 11 years and 2 months she presentedwith short stature, markedly decreased growth velocity and signs and symptoms suggestive ofhypothyroidism. Magnetic Resonance Imaging (MRI) of the brain revealed complexstructural and cerebrovascular arterial anomalies, including an empty sella. Testing ofpituitary function revealed multiple pituitary dysfunctions, including absolute growthhormone deficiency, hypogonadotropic hypogonadism, central hypothyroidism, andsecondary adrenal insufficiency.
Conclusions:
This case suggests the necessity to screen all patients with PHACE syndrome and intracranialmalformations for pituitary dysfunction at regular intervals.

Source: http://www.ijpeonline.com/content/2012/1/20

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