Delayed Massive Cystic Transformation Following Germinal Matrix Hemorrhage Presenting as Hydrocephalus in a Preterm Infant: A Case Report

Abstract

Germinal matrix hemorrhage/intraventricular hemorrhage (GMH-IVH) is a significant neurological complication of prematurity, particularly in infants born before 32 weeks of gestation. Severe GMH-IVH may lead to long-term sequelae including post-hemorrhagic ventricular dilatation, white matter injury, cystic degeneration, and neurodevelopmental impairment. Delayed large cystic transformation with progressive hydrocephalus remains an uncommon and underrecognized complication. We report the case of a 10-month-old premature female infant born at 32 weeks of gestation with a birth weight of approximately 1700 g from a twin pregnancy. Neonatal cranial ultrasonography demonstrated right-sided Grade III germinal matrix/intraventricular hemorrhage. After an initial period of relatively acceptable growth and neurodevelopment, the patient progressively developed macrocephaly, developmental delay, poor feeding,
and lethargy between 6 and 10 months of age. Brain magnetic resonance imaging (MRI) revealed a large right parieto-occipital intra-axial cystic cavitary lesion with cerebrospinal fluid signal intensity, peripheral hemosiderin staining on susceptibility-weighted imaging, marked thinning of the adjacent cortical mantle, ipsilateral white matter loss, and severe supratentorial hydrocephalus. Imaging findings were most consistent with delayed posthemorrhagic cystic transformation, likely representing porencephalic evolution secondary
to prior periventricular hemorrhagic injury. The patient subsequently underwent ventriculoperitoneal shunt placement with early postoperative reduction in ventricular size. However, during follow-up, the patient developed a severe systemic infection complicated by septic shock. No definite evidence of shunt malfunction, recurrent intracranial hemorrhage, or progression of the intracranial lesion was identified. Despite intensive
medical management, the patient died approximately two months after surgery. This case highlights a rare delayed complication of severe neonatal GMH-IVH with progressive cystic cavitary transformation and hydrocephalus presenting months after the initial hemorrhagic insult. The report emphasizes the importance of long-term neuroimaging surveillance and careful neurodevelopmental follow-up in premature infants
with severe GMH-IVH, particularly those at risk for periventricular hemorrhagic injury and post-hemorrhagic ventricular dilatation.