1. Unidade de Neurocirurgia Pediátrica, Hospital de Dona Estefânia. Centro Hospitalar de Lisboa Central EPE (CHLC)
2. Unidade de Cuidados Intensivos Neonatais, Hospital Dona Estefânia, CHLC
3. Unidade de Neurorradiologia Pediátrica, Hospital Dona Estefânia, CHLC
4. Serviço de Neuropediatria, Hospital Dona Estefânia, CHLC
5. Serviço de Anatomia Patológica, Hospital São José, CHLC
- Poster apresentado no congresso “2017 AANS Annual Scientific Meeting”, 22 a 26 de abril, 2017, Los Angeles
Introduction: Intracranial infantile hemangiomas are extremely rare and have barely been reported in association with concurrent multiple cutaneous or visceral lesions.
Clinical Case: We report a case of a 27-day-old full term neonate who presents with a seven-day history of acute facial assymetry with progressive worsening. She showed right peripheral facial palsy and no cutaneous lesions on physical examination. Cranial magnetic resonance imaging (MRI) revealed a contrast enhancing right cerebellopontine angle (CPA) mass, with extension to the internal auditory canal. No further lesions were found on the neuroaxis exam. Right auditory evokedpotentials were absent. She was started on corticosteroids and repeated cranial MRI a few days later, showing enlargement of the lesion. A retrosigmoid approach was performed with total removal of the cisternal component of a largely vascularized lesion encasing both vestibular nerves. The postoperative course was uneventful. Histological examination demonstrated hemangioma with intralesional nerve bundles. Endothelial cells were immunopositive for GLUT1. She was started on oral propranolol and initiated a rehabilitation program. At six-month follow-up, she recovered partially and shows no radiological signs of recurrence.
Conclusion: This case encloses unique features, namely the presence of an intracranial nerve hemangioma in a neonate without associated hemangiomatosis, causing progressive neurological deficit. Despite their rarity, hemangiomas should be considered in the differential diagnosis of CPA lesions in children.
Palavras-chave: hemangioma, ângulo ponto-cerebeloso, tumores infantis do SNC