Abstract

Background
Hydrocephalus is the abnormal accumulation of cerebrospinal fluid (CSF) within the cerebral ventricles. Neglected hydrocephalus refers to untreated or significantly delayed management of this condition and is an extremely rare phenomenon with few cases reported in the literature. In advanced stages, prolonged intracranial pressure causes progressive thinning of the scalp and skull, predisposing patients to spontaneous external rupture. This condition may result in serious complications, including meningitis, ventriculitis, decubitus ulcers, malnutrition, and dehydration. Neglected hydrocephalus is commonly associated with poverty, illiteracy, and limited access to healthcare services.
Case Summaries
A 2-month-old male infant with progressive head enlargement and lumbosacral swelling presented with scalp ulceration over the anterior fontanelle and cerebrospinal fluid leakage measuring approximately 1 cm. His occipitofrontal circumference was 68 cm (>99.6th percentile). Despite resuscitation and antibiotic therapy, the infant died from sepsis and ventriculitis within 24 hours of admission.
A 4-year-old male child presented with progressive head enlargement for four years, multiple scalp ulcerations with exposed skull, and intermittent CSF leakage. A ventriculoperitoneal shunt was inserted, but postoperative shunt infection occurred, necessitating shunt removal. The child subsequently died from ventriculitis and sepsis two weeks later.
Conclusion
Neglected hydrocephalus may lead to irreversible neurological damage, spontaneous rupture, severe infection, and death. Early diagnosis, improved public health awareness, better access to neurosurgical care, and strengthened healthcare financing systems are essential for prevention.

Key words: Neglected hydrocephalus, spontaneous rupture, myelomeningocele, ventriculoperitoneal shunt