A case report of hemorrhagic presentation of diffuse neonatal hemangiomatosis (DNH) associated to obstructive hydrocephalus: Medical and neurosurgical considerations

TL;DR

This case report describes a rare neonatal condition involving brain hemorrhages and hydrocephalus, successfully treated with surgery and medical therapy.

Contribution

The paper presents a rare case of DNH with CNS complications and highlights the successful use of ETV as a treatment.

Findings

DNH can cause CNS hemorrhages and obstructive hydrocephalus in neonates.

Endoscopic ventriculocisternostomy (ETV) was effective in treating triventricular hydrocephalus.

Medical therapy led to complete resolution of cerebral and skin lesions.

Abstract

Diffuse neonatal hemangiomatosis (DNH) is a rare disorder typically recognized at birth or during the neonatal period. DNH involves three or more organ systems, including the central nervous system (CNS). In these cases, serious complications such as hemorrhages and obstructive hydrocephalus can develop.

We present a case of DNH with intracranial hypertension and CNS hemorrhagic lesions on the mesencephalic aqueduct, resulting in triventricular hydrocephalus, treated with endoscopic ventriculocisternostomy (ETV) and medical therapy.

DNH is a rare condition that can involve the CNS with serious complications. From a review of the literature, we highlighted only two cases of DNH with brain involvement treated surgically. We report the successful outcome of ETV, along with surgical considerations, imaging findings, and the complete resolution of cerebral and skin lesions following medical therapy.

Medical therapy is not standardized and must be individualized. In patients who develop severe neurological symptoms such as obstructive hydrocephalus, surgery may be considered to avoid neurological sequelae.

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