Successful treatment of multifocal intracerebral and spinal hemangiomas with propranolol
Authors: Juliette Miquel, Bertrand Bruneau, Alain Dupuy
Journal: Journal of the American Academy of Dermatology (2014)

To the Editor: Multifocal infantile hemangioma (MIHs) is defined by the presence of at least 5 cutaneous infantile hemangiomas (IHs). Extracutaneous involvement may occur, with an estimated mortality rate of 5%.1Glick Z.R. Frieden I.J. Garzon M.C. Mully T.W. Drolet B.A. Diffuse neonatal hemangiomatosis: an evidence-based review of case reports in the literature.J Am Acad Dermatol. 2012; 67: 898-903Abstract Full Text Full Text PDF PubMed Scopus (60) Google Scholar Central nervous system (CNS) disease is exceptional. A full-term male newborn was hospitalized with 50 small superficial and deep subcutaneous IHs, without heart failure or hepatomegaly (Fig 1). At day 2 of age, ultrasound imaging showed a focal liver IH measuring 14 mm with arteriovenous shunting, a testicular IH, and multiple hyperechogenic lesions visualized by transfontanellar imaging. Cerebral and spinal magnetic resonance imaging (MRI) confirmed the presence of MIH in the brain and in 3 spinal locations, with multiple gadolinium enhancements seen on T1-weighted images, sometimes surrounded by slight edema (Fig 2, A). No other visceral IHs were detected. Thyroid function and echocardiography were normal.Fig 2Magnetic resonance imaging of central nervous system infantile hemangiomas (IHs) before (A) and after (B) initiation of propranolol treatment. A, One spinal and numerous intracerebral IHs with multiple gadolinium enhancements are seen on T1-weighted images (red arrows), some with surrounding edema (red circle). B, Edema and contrast enhancement are no longer visible on T1-weighted images at 6 months.View Large Image Figure ViewerDownload Hi-res image Download (PPT) Treatment with propranolol 1 mg/kg daily was started on day 8 of life. Heart rate, blood pressure, blood glucose level, and electrocardiogram results were normal before and after initiating propranolol therapy. The dose was increased to 3 mg/kg daily by day 18. There were no adverse effects and he was discharged home on day 23. At day 29, clinical regression of subcutaneous lesions was observed and the liver lesion had decreased by a few millimeters on ultrasound imaging. At day 45, MRI showed a decrease in number and size of the CNS IHs. At day 60, all subcutaneous IHs had disappeared. At day 100, the liver and testicular IHs were undetectable. At day 180, edema and contrast enhancement of the brain and spinal cord lesions were no longer visible on T1-weighted MR images (Fig 2, B). The child's growth and psychomotor development were normal. One year after starting treatment, only a few superficial cutaneous IHs remained and the MRI findings were unchanged. It was planned to continue propranolol 3 mg/kg daily until 18 months of age and then progressively reduce the dose, with repeat imaging studies to monitor for rebound IH growth. CNS disease in MIH is exceptional. Two cases with extensive CNS involvement treated with systemic corticosteroids had neurologic sequelae and side effects.2Al-Kaabi A. Yanofsky R. Bunge M. Hyman J. Rafay M.F. Diffuse hemangiomatosis with predominant central nervous system involvement.Pediatr Neurol. 2009; 40: 54-57Abstract Full Text Full Text PDF PubMed Scopus (11) Google Scholar, 3Balaci E. Sumner T.E. Auringer S.T. Cox T.D. Diffuse neonatal hemangiomatosis with extensive involvement of the brain and cervical spinal cord.Pediatr Radiol. 1999; 29: 441-443Crossref PubMed Scopus (24) Google Scholar Three cases of cerebellar IH are reported4Dotan M. Lorber A. Congestive heart failure with diffuse neonatal hemangiomatosis—case report and literature review.Acta Paediatr. 2013; 102: e232-e238Crossref PubMed Scopus (23) Google Scholar; 2 were treated with surgical excision. Recently, an isolated cerebellar IH associated with MIH was treated effectively with propranolol.4Dotan M. Lorber A. Congestive heart failure with diffuse neonatal hemangiomatosis—case report and literature review.Acta Paediatr. 2013; 102: e232-e238Crossref PubMed Scopus (23) Google Scholar Our case of MIH with multiple cerebral and spinal lesions was also successfully treated with propranolol, with rapid improvement by day 45 and disappearance of lesions on neuroimaging at 6 months.