Phace syndrome in children: an association of segmental facial hemangiomas and sternal cleft
DOI:
https://doi.org/10.11603/2414-4533.2017.4.8374Keywords:
PHACE syndrome, infantile hemangiomas, sternal cleft.Abstract
The aim of the work: assessing the prevalence of PHACE syndrome in children with infantile hemangiomas (IHs), document the outcome of treatment with propranolol and necessary of addition surgical interventions.
Materials and Methods. The study included 27 children with IHs in the head and neck region that underwent inpatient treatment at the Children’s Hospital “OKHMATDYT” from December 2010 to March 2017. For the diagnosis, ultrasound, MRI, CT with contrast, echocardiography, and ophthalmologic examination were used.
Results and Discussion. Diagnosis of PHACE syndrome was established in 4 (14.81 %) patients of 27 children with infantile hemangiomas (IHs) of the face and neck region with a diameter of more than 5 cm. The PHACE syndrome was diagnosed according to the following criteria: presence of segmental facial IHs (n=4), including reticular hemangioma (n=1), sternal cleft (n=3), pathology of the posterior cranial fossa (n=1), aortic coarctation (n=1), aortic right arch (n=1), supraumbilical raphe (n=1). All patients were female. Involving of the upper airway with IHs with a violation of their patency was diagnosed in 3 (75 %) patients with PHACE syndrome. Children with IHs were treated conservatively using propranolol – a non-selective beta-blocker – at a dose of 2 mg/kg/day. Conservative therapy of IHs in children with PHACE syndrome is effective and safe, but is characterized by a relatively longer duration. Operative treatment of sternal cleft was performed in 3 patients; in two children primary early correction of abnormalities performed at the age of 2 and 4 months. All patients have achieved a good functional and cosmetic outcome after surgical treatment.
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