Swelling at bridge of nose

Nasoethmoidal encephalocele. A cranial meningocele consists of a cerebrospinal fluid {CSF} filled meningeal sac only, and a cranial encephalocele contains the sac plus cerebral cortex, cerebellum, or portions of the brainstem. Infants with a cranial encephalocele are at increased risk for developing hydrocephalus due to aqueductal stenosis, Chiari malformation, or the Dandy-Walker syndrome. Examination might show a small sac with a pedunculated stalk or a large cyst like structure that can exceed the size of the cranium. The lesion may be completely covered with skin, but areas of denuded lesion can occur and require urgent surgical management. Ultrasonography is most helpful in determining the contents of the sac. MRI or CT further helps define the spectrum of the lesion. Children with a cranial meningocele generally have a good prognosis, whereas patients with an encephalocele are at risk for vision problems, microcephaly, mental retardation, and seizures. Generally, children with neural tissue within the sac and associated hydrocephalus have the poorest prognosis.

E-published: July 2012 Vol 9 Issue 7 Art # 41

References :

Waldo E, Nelson MD. Encephalocele. Nelson Textbook of Pediatrics 15th ed Saunders 1997; 184-. 185.