Spontaneous Regression Of Multiple Intracranial Capillary Hemangiomas In A Newborn-long-term Follow-up And Literature Review
Noureldine, M. H. A., Rasras, S., Safari, H., Sabahi, M., Jallo, G. I., & Arjipour, M. (2021). Spontaneous regression of multiple intracranial capillary hemangiomas in a newborn-long-term follow-up and literature review. Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery, 37(10), 3225–3234. https://doi.org/10.1007/s00381-021-05053-7
View Original Source →Abstract
BACKGROUND: Intracranial capillary hemangiomas (ICHs) have a natural history and behavior that is very different from intracranial cavernous malformations. The literature is not consistent as to the best management strategy for ICHs. CASE DESCRIPTION: Our patient is a 40-day-old male infant who presented with progressive increase in head circumference and multiple cutaneous capillary and ICHs. Obstructive hydrocephalus necessitated urgent cerebrospinal fluid (CSF) diversion, but no other surgical intervention was pursued due to the high risk-to-benefit ratio. All intracranial lesions spontaneously regressed by 11 years of age, albeit at a slower speed than the cutaneous lesions, with no functional or cognitive sequelae. We conducted a comprehensive literature review and provided a summary of all reported ICH cases. CONCLUSION: Asymptomatic patients with ICHs are best approached with close follow-up and serial imaging studies as the potential for spontaneous regression is relatively high. Patients with isolated lesions or unclear diagnoses may benefit from a stereotactic biopsy, and surgical resection should be reserved for symptomatic lesions only.
Case Details
Disease Location
Brain, skin
Personal Characteristics
40-day-old male infant
Clinical Characteristics
Presenting with a progressive increase in head circumference and multiple cutaneous, 2-10 mm red-to-purple, strawberry-like lesions on the face, chest, lower back, palm, elbow, and legs; swelling of he mesial part of his left orbit was also noted. He was referred following an episode of afebrile seizure. Biopsy was obtained and histopathology demonstrated meager-walled vascular malformations with fragile vessels a 2-week trial of prednisolone (2 mg per kg) was completed CT scan showed obstructive hydrocephalus with an intraventricular hematoma and multiple lesions. Urgent shunting was performed. MRI showed two large (20 mm) extra-axial lesions were noted in the right frontal and parietal lobes. Multiple smaller lesions were also found at the left frontal lobe, left atrial paraventricular region, pons, and midbrain, as well as an intraventricular lesion in the fourth ventricle. A large extraconal lesion with similar characteristics was found in the mesial wall of the left orbit, compressing the globe. A retro-cerebellar arachnoid cyst was also noted.
Remission Characteristics
Over the first year of follow-up, the cutaneous, orbital, and intracranial lesions spontaneously regressed. At 3 years of age, the intracranial lesions did regress
Treatment & Mechanisms
Proposed Remission Mechanisms
None reported
Clinical Treatment
Prednisolone ventriculostomy
Non-Clinical Treatment
None reported