Temporobasal, Transsphenoidal Meningoencephalocele Becoming Symptomatic with Spontaneous Cerebrospinal Fluid Rhinorrhea: Diagnostic Work-up and Microsurgical Strategy

Abstract

Background We report the rare case of an adult transsphenoidal meningoencephalocele and outline the microneurosurgical strategy. Clinical history, the findings of computerized tomography (CT) scans and magnetic resonance imaging (MRI), the microsurgical procedure, and histopathology are reported.

Case Report A 54-year-old female patient complained about cerebrospinal fluid (CSF) rhinorrhea; a transnasal biopsy of a mass in the maxillar sinus prior to diagnostic work-up was performed elsewhere. Persisting CSF leakage prompted CT and MRI, which showed brain tissue extending from the left middle cranial fossa into the left sphenoid sinus through several bony defects. The diagnosis of a transsphenoidal meningoencephalocele was made, and the lesion was targeted via a subtemporal intradural approach with resection of the herniated brain tissue and closure of the bony as well as of dural defects. The postoperative course was uneventful without recurrence of the CSF fistula.

Conclusion The transsphenoidal subtype of basal meningoencephaloceles is exceedingly rare. Nonetheless, it has to be considered as a differential diagnosis if a nasal or intrasphenoidal mass is diagnosed. Otherwise, unjustified biopsy or unsuccessful management of CSF leakage could not be avoided. The intradural subtemporal approach is effective to treat the transsphenoidal type of basal meningoencephaloceles.

Keywords

temporobasal - transsphenoidal meningoencephalocele - rhinorrhea - skull base surgery

• References

• 1 McLaurin RL. Encephalocele and related anomalies. In: Hoffmann HJ, , ed. Disorders of the Developing Nervous System: Diagnosis and Treatment. St Louis, MO: Blackwell Scientific; 1986: 153-171
• 2 Abe T, Lüdecke DK, Wada A, Matsumoto K. Transsphenoidal cephaloceles in adults. A report of two cases and review of the literature. Acta Neurochir (Wien) 2000; 142: 397-400
• 3 French BN. Midline fusion defects and defects of formation. In: Youmans JR, , ed. Neurological Surgery. Vol. 3. Philadelphia: Saunders; 1982: 1236-1380
• 4 David DJ, Proudman TW. Cephaloceles: classification, pathology, and management. World J Surg 1989; 13: 349-357
• 5 Matson DD. Neurosurgery of Infancy and Childhood. 2nd ed. Springfield, IL: Charles C Thomas; 1969
• 6 Batsakis JG. Tumors of the Head and Neck: Clinical and Pathological Considerations. 2nd ed. Baltimore: Williams and Wilkins; 1979: 514-530
• 7 Suwanwela C, Suwanwela N. A morphologic classification of sincipital encephalomeningoceles. J Neurosurg 1972; 30: 201-211
• 8 Mood GF. Congenital anterior herniations of brain. Ann Otol Rhinol Laryngol 1938; 47: 392-406
• 9 DeBeer GR. The Development of the Vertebrate Skull. Oxford, England: Clarendon; 1937: 1-123
• 10 Schlosser RJ, Wilensky EM, Grady MS, Bolger WE. Elevated intracranial pressures in spontaneous cerebrospinal fluid leaks. Am J Rhinol 2003; 17: 191-195
• 11 Schlosser RJ, Woodworth BA, Wilensky EM, Grady MS, Bolger WE. Spontaneous cerebrospinal fluid leaks: a variant of benign intracranial hypertension. Ann Otol Rhinol Laryngol 2006; 115: 495-500
• 12 Samii M, Draf W. Surgery of the Skull Base. Berlin: Springer-Verlag; 1989: 114-126
• 13 Pollock JA, Newton TH, Hoyt WF. Transsphenoidal and transethmoidal encephaloceles. A review of clinical and roentgen features in 8 cases. Radiology 1968; 90: 442-453
• 14 Abiko S, Aoki H, Fudaba H. Intrasphenoidal encephalocele: report of a case. Neurosurgery 1988; 22: 933-936
• 15 Yokota A, Matsukado Y, Fuwa I, Moroki K, Nagahiro S. Anterior basal encephalocele of the neonatal and infantile period. Neurosurgery 1986; 19: 468-478
• 16 Jabre A, Tabaddor R, Samaraweera R. Transsphenoidal meningoencephalocele in adults. Surg Neurol 2000; 54: 183-187 , discussion 187–188
• 17 DeMyer W. The median cleft face syndrome. Differential diagnosis of cranium bifidum occultum, hypertelorism, and median cleft nose, lip, and palate. Neurology 1967; 17: 961-971
• 18 Diebler C, Dulac O. Cephaloceles: clinical and neuroradiological appearance. Associated cerebral malformations. Neuroradiology 1983; 25: 199-216
• 19 Kenna MA. Transsphenoidal encephalocele. Ann Otol Rhinol Laryngol 1985; 94 (5 Pt 1) 520-522
• 20 Kennedy EM, Gruber DP, Billmire DA, Crone KR. Transpalatal approach for the extracranial surgical repair of transsphenoidal cephaloceles in children. J Neurosurg 1997; 87: 677-681
• 21 Mylanus EAM, Marres HAM, Vlietman J , et al. Transalar sphenoidal encephalocele and respiratory distress in a neonate: a case report. Pediatrics 1999; 103: E12
• 22 Pollock JA, Newton TH, Hoyt WF. Transsphenoidal and transethmoidal encephaloceles. A review of clinical and roentgen features in 8 cases. Radiology 1968; 90: 442-453
• 23 Shimizu T, Kitamura S, Kinouchi K, Fukumitsu K. A rare case of upper airway obstruction in an infant caused by basal encephalocele complicating facial midline deformity. Paediatr Anaesth 1999; 9: 73-76
• 24 Smith DE, Murphy MJ, Hitchon PW, Babin RW, Abu-Yousef MM. Transsphenoidal encephaloceles. Surg Neurol 1983; 20: 471-480
• 25 Tsutsumi K, Asano T, Shigeno T, Matsui T, Ito S, Kaizu H. Transcranial approach for transsphenoidal encephalocele: report of two cases. Surg Neurol 1999; 51: 252-257
• 26 Yokota A, Matsukado Y, Fuwa I, Moroki K, Nagahiro S. Anterior basal encephalocele of the neonatal and infantile period. Neurosurgery 1986; 19: 468-478
• 27 Lai SY, Kennedy DW, Bolger WE. Sphenoid encephaloceles: disease management and identification of lesions within the lateral recess of the sphenoid sinus. Laryngoscope 2002; 112: 1800-1805
• 28 Landreneau FE, Mickey B, Coimbra C. Surgical treatment of cerebrospinal fluid fistulae involving lateral extension of the sphenoid sinus. Neurosurgery 1998; 42: 1101-1104 , discussion 1104–1105
• 29 Komotar RJ, Starke RM, Raper DMS, Anand VK, Schwartz TH. Endoscopic endonasal versus open repair of anterior skull base CSF leak, meningocele, and encephalocele: a systematic review of outcomes. J Neurol Surg A 2012; ; October 1 (Epub ahead of print)
• 30 Rohde V, Spangenberg P, Mayfrank L, Reinges M, Gilsbach JM, Coenen VA. Advanced neuronavigation in skull base tumors and vascular lesions. Minim Invasive Neurosurg 2005; 48: 13-18
• 31 Taniguchi M, Perneczky A. Subtemporal keyhole approach to the suprasellar and petroclival region: microanatomic considerations and clinical application. Neurosurgery 1997; 41: 592-601