Huge interparietal posterior fontanel meningohydroencephalocele

Area of Science:

• Pediatric Neurosurgery
• Developmental Neuroscience
• Congenital Malformations

Background:

• Congenital encephalocele is a neural tube defect resulting from embryonic developmental abnormalities.
• It involves protrusion of brain and meninges through a skull defect, commonly at the occipital bone.
• Incidence varies globally, with rare presentations occurring at the interparietal-posterior fontanel.

Observation:

• A case report of a 1-month-old male infant with a huge interparietal-posterior fontanel meningohydroencephalocele is presented.
• Diagnosis was confirmed via physical examination and volumetric computed tomography.
• The rare condition involved herniation of meninges, brain, and large blood vessels through a significant bone defect.

Findings:

• Surgical resection of the meningohydroencephalocele was performed using intradural and extradural approaches.
• The bone defect was not primarily closed, and the patient developed hydrocephaly post-surgery, necessitating ventriculoperitoneal shunting.
• The presence of large blood vessels communicating with the superior sagittal sinus and ventricular system posed surgical challenges.

Implications:

• Surgical management of large interparietal-posterior fontanel meningohydroencephaloceles requires careful strategic planning due to potential technical difficulties and complications.
• Accurate assessment of individual lesion features, including vascular involvement, is crucial for achieving favorable surgical outcomes.
• This case highlights the importance of tailored surgical approaches for rare and complex congenital neural tube defects.