Surgical management of congenital median perisellar transsphenoidal encephaloceles with an extracranial approach: a series of 6 cases.
OBJECTIVE
Median perisellar congenital transsphenoidal encephalocele (CTE) is a rare entity associated with multiple endocrine, visual, and respiratory deficits. The most likely causative factor for these pathological alterations is distension of neural structures (hypothalamic-pituitary system, optic pathway), resulting in protrusion of the dural sac through a sphenoid bone defect into the pharynx. The continuity with the extracranial space can be associated with an increased risk of tearing of the sac, with consequent cerebrospinal fluid leakage and subsequent increase in the risk of infection. We retrospectively reviewed the surgical treatment of CTE in our hospital using either an extracranial transoral or transnasal approach.


METHODS
We retrospectively reviewed our database. Between July 1994 and June 2005, CTE we identified 6 patients. Five of them were treated by a surgical intervention. The first patient was treated via a transcranial approach but had a relapse of the prolapse 11 years later. The relapse was treated with an extracranial transpalatal approach. Four patients were treated with an extracranial surgical approach: an extracranial transoral approach was performed in 2 cases, and an extracranial transnasal approach was used in the other 2 cases. Surgery was not performed in 1 patient because the parents refused to consent to the procedure.


RESULTS
Preoperative symptoms remained stable or improved in all of the patients after the surgical procedure and worsened in the patient who did not have a surgical intervention. Two patients experienced a palatal dehiscence. No mortality was recorded in this series of patients.


CONCLUSIONS
The surgical treatment of CTE is indicated to stop the progression or improve symptoms related to this disease entity. If approached correctly, the extracranial approach is a safe procedure with subsequent low morbidity.
Spacca
Barbara
B
Department of Pediatric Neurosurgery, Anna Meyer Children's Hospital, Florence, Italy
Amasio
Maria Enrica
ME
Giordano
Flavio
F
Mussa
Federico
F
Busca
Gianpaolo
G
Donati
Pierarturo
P
Genitori
Lorenzo
L
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003399:Craniotomy / Q000379:methods*; D004677:Encephalocele / Q000473:pathology* / Q000601:surgery*; D005260:Female; D006801:Humans; D007223:Infant; D008137:Longitudinal Studies; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D019635:Neurosurgical Procedures / Q000379:methods*; D012658:Sella Turcica / Q000601:surgery*; D015898:Tomography Scanners, X-Ray Computed; D016896:Treatment Outcome