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Late-onset obstructive hydrocephalus associated with occipital encephalocele with large skull defect successfully
Yuki Munekata1, Taku Sugiyama2, Yuki Ueda3
1Department of Neurosurgery, Hokkaido University Graduate School of Medicine, North 15 West 7, Kita-ku, Sapporo, 060-8638, Japan.
Insights
This case report details a rare instance of late-onset obstructive hydrocephalus in a child with an occipital encephalocele and a large skull defect. Endoscopic third ventriculostomy effectively treated the hydrocephalus, highlighting a unique neurological presentation.
Area of Science:
- Neurology
- Pediatric Neurosurgery
- Developmental Biology
Background:
- Occipital encephaloceles commonly present with hydrocephalus in infancy.
- This report focuses on a rare case of late-onset obstructive hydrocephalus linked to an occipital encephalocele with a significant cranial defect.
Observation:
- A newborn girl with occipital hydroencephalomeningocele and a large occipital defect underwent successful epithelialization without infection.
- Despite an obstructed cerebral aqueduct, the patient remained asymptomatic for hydrocephalus until age 7.
- At age 8, worsening gait and imaging revealed enlarged lateral and third ventricles, indicating obstructive hydrocephalus.
Findings:
- The patient underwent successful endoscopic third ventriculostomy, relieving symptoms and improving hydrocephalus.
- This is the first reported case of late-onset obstructive hydrocephalus associated with an occipital encephalocele and extensive cranial bony defects.
Implications:
- This rare presentation of late-onset hydrocephalus in occipital encephalocele warrants attention in neurological and radiological follow-ups.
- Further research is needed to understand the specific mechanisms driving hydrocephalus in such cases.
- The findings suggest that endoscopic third ventriculostomy can be an effective treatment for late-onset obstructive hydrocephalus in this context.
Background:
Hydrocephalus is one of the most common presentations of occipital encephaloceles and usually develops within the first year of life. This case report presents a rare case of late-onset obstructive hydrocephalus associated with occipital encephalocele with an extraordinarily large occipital skull defect.
Case Report:
At birth, a newborn girl presented with an absence of a vast amount of occipital cranium and skin and was diagnosed with occipital hydroencephalomeningocele. Under meticulous sterile management, the affected area was successfully epithelialized, and the patient was discharged without infectious complication. Despite an obstructed cerebral aqueduct, she grew without any signs of hydrocephalus until the age of 7 years. Her gait gradually worsened, and imaging tests at the age of 8 years revealed markedly enlarged lateral and third ventricles but not the fourth ventricle. Endoscopic third ventriculostomy successfully relieved her symptoms with improvement of hydrocephalus.
Conclusion:
This is the first case of late-onset obstructive hydrocephalus associated with an occipital encephalocele characterized by large-scale cranial bony defects. Although further investigation is required to elucidate the mechanism of hydrocephalus, this rare phenomenon should be noted during neurological and radiological follow-up.
