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[A case of transsphenoidal meningoencephalocele]
S Kobayashi1, M Miyazaki, O Miyagi
1Department of Neurosurgery, Maebashi Red-Cross Hospital.
Insights
This case report details a rare basal meningoencephalocele in a 6-year-old boy, presenting with multiple congenital anomalies and hypopituitarism. Surgical intervention was cautiously approached due to potential hypothalamic-pituitary dysfunction.
Area of Science:
- Neuroscience
- Pediatric Neurology
- Medical Imaging
Background:
- Basal meningoencephaloceles are rare congenital malformations involving protrusion of meninges and brain tissue through a defect at the skull base.
- Associated conditions can include various craniofacial abnormalities and endocrine deficiencies, impacting diagnosis and management.
- Early detection and comprehensive evaluation are crucial for affected children.
Observation:
- A 6-year-old boy presented with a basal meningoencephalocele, diagnosed via MRI, with a history of strabismus, morning glory syndrome, and hypopituitary dwarfism.
- Physical examination revealed hypertelorism and left coloboma. Nasopharyngeal examination identified a midline mass.
- Imaging studies including CT and MRI demonstrated the meningoencephalocele protruding into the nasopharynx, associated with a stalk from the hypothalamus and evidence of Moyamoya-like vascular changes.
Findings:
- The meningoencephalocele contained brain tissue, with a stalk extending from the hypothalamus.
- Growth hormone deficiency was confirmed, consistent with hypopituitarism.
- Vascular abnormalities, including internal carotid artery stenosis and Moyamoya-like networks, were noted on angiography.
Implications:
- This case highlights the complex presentation of basal meningoencephalocele, emphasizing the need for multidisciplinary assessment.
- Surgical management requires careful consideration of potential risks, particularly hypothalamic-pituitary dysfunction.
- The rarity of transsphenoidal encephalocele underscores the importance of reporting such cases for advancing clinical understanding and treatment strategies.
Abstract:
A 6-year-old boy was sent to us due to basal meningoencephalocele detected by MRI. He had had a past history of strabismus and morning glory syndrome since 6 months of age, as well as hypopituitary dwarfism since he was 3 years old. On admission, physical examination revealed hypertelorism and left coloboma. Laryngoscopic examination revealed a round mass covered by normal nasal mucose in the midline of the tegmen of the choana. X-ray tomogram and CT scan demonstrated an isodense mass protruding into the nasopharyngeal cavity through a bony defect of the sella turcica. Right carotid angiogram showed stenosis of the right internal carotid artery and abnormal fine vessels in the basal ganglia similar to basal Moyamoya network. There was an irregular filling of the right anterior cerebral artery. Left carotid angiogram showed an irregular filling of the left middle cerebral artery. MRI showed an anterior part of the third ventricle descending into, and the meningocele protruding into the nasopharyngeal cavity. It also showed a stalk extending from the hypothalamus into the meningocele, but the pituitary gland was not recognized. The meningocele was of the same signal intensity as CSF in both T1 and T2 weighted images. Growth hormone deficiency was confirmed by radioimmunoassay of the peripheral blood. An operation was scheduled transcranial to prevent snoring during sleep at age 9. The stalk was observed behind the chiasm during the operation, but a radical operation was not performed because of a possibility of postoperative hypothalamic-pituitary dysfunction. Transsphenoidal encephalocele is rare. So far as we could see, only 30 cases have been reported.(ABSTRACT TRUNCATED AT 250 WORDS)