Transsphenoidal Cephaloceles in Adults. A Report of Two Cases and Review of the Literature

Abstract

Transsphenoidal cephalocele rarely occurs in adults. We describe two adult cases with transsphenoidal cephaloceles. The first case was a 53-year-old female who presented with spontaneous cerebrospinal fluid (CSF) rhinorrhea. Magnetic resonance (MR) imaging demonstrated a transsphenoidal meningocele. Surgical repair was attempted via the transsphenoidal route. Rhinorrhea recurred at one month and also two years later. Transsphenoidal surgical repairs were repeated. There has been no evidence of CSF leakage in the four years of follow-up after the last surgery. Transsphenoidal repair seems to be the most suitable approach in this case. The second case was a 26-year-old female with secondary amenorrhea, diabetes insipidus, bitemporal hemianopsia, and see-saw nystagmus. MR imaging demonstrated a transsphenoidal encephalocele with the optic chiasma and infundibular recesses descending into the meningocele and an agenesis of the corpus callosum. The first operation was performed via the transnasal route and the second by sublabial transmaxillary transsphenoidal approach six months later. Postoperative MR imaging revealed reduction of the encephalocele. There was neither worsening nor noticeable improvement in the neurological or endocrinological function. However, in this type, complete repair is often impossible, and non-radical surgery such as transsphenoidal repair may be indicated as most suitable. They should be selected with careful assessment referring to MR findings and clinical symptoms and signs.

Hypopituitarism in a patient with transsphenoidal cephalocele: longitudinal changes in endocrinological abnormalities

“…noidal encephalocele was recently reported, the number of treatable cases will probably increase in the near future [10][11][12].…”

Endoscopic repair of transsellar transsphenoidal meningoencephalocele; case report and review of approaches

“…Although most cases were managed conservatively, the transcranial approach was used in the others [1]. Due to higher morbidity, mortality and more post-operative pituitary dysfunction, the transcranial approach is not well accepted in transsphenoidal meningoencephalocele [4,7].…”

“…Transsphenoidal route was chosen by Abe et al [7] for a 26-year-old woman presenting with secondary amenorrhea, diabetes insipidus, bitemporal hemianopia, nystagmus, and morning glory syndrome. The sac was reduced and the defect was repaired by a bony graft, muscle, and fibrin glue.…”

Middle Fossa Approach to a Temporosphenoidal Encephalocele -Technical Note-

“…2) Few cases of intrasphenoidal encephalocele have been reported. 1,4,5) Therefore, diagnosis and treatment is a challenge for the rhinologist and skull base neurosurgeon. The more common trans-sphenoidal encephalocele can occur through a larger defect in the sellar floor, planum sphenoidale or posterior ethmoid.…”