Surgical management and reconstruction of sincipital encephalocele presenting in adulthood

  • A. Krajewski
  • S. J. Borch
  • A. Khan
  • M. Bhaya
  • R. Y. Chandawarkar
Case Report


Sincipital encephaloceles presenting in adulthood are misdiagnosed until they present with persistent cerebrospinal fluid (CSF) rhinorrhea or, in extreme cases, meningeal infection. The basic approach to their repair is a frontal craniotomy and bandeau-type removal of the orbital framework, and reconstruction using dural patch for watertight dural closure, split calvarial bone graft fixation for rigid repair of the bony framework, and a vascularized pericranial flap to provide the soft tissue interposition. We emphasize the pitfall of biopsy when encephalocele is included in the differential diagnosis of a nasal mass and include a step-by-step operative technique that ensures safe and effective reconstruction of the skull base defect.


Nasoethmoidal encephalocele Sincipital Craniofacial Vascularized pericranial flaps Reconstruction skull base 


  1. 1.
    Agthong S, Wiwanitkit V (2002) Encephalomeningocele cases over 10 years in Thailand: a case series. BMC Neurol 2:3 doi: 10.1186/1471-2377-2-3 PubMedCrossRefGoogle Scholar
  2. 2.
    Czech T, Reinprecht A, Matula C et al (1995) Cephaloceles—experience with 42 patients. Acta Neurochir (Wien) 134:125–129 doi: 10.1007/BF01417678 CrossRefGoogle Scholar
  3. 3.
    Rojvachiranonda N, David DJ, Moore MH et al (2003) Frontoethmoidal encephalomeningocele: new morphological findings and a new classification. J Craniofac Surg 14:847–858 doi: 10.1097/00001665-200311000-00006 PubMedCrossRefGoogle Scholar
  4. 4.
    Simpson DA, David DJ, White J (1984) Cephaloceles: treatment, outcome, and antenatal diagnosis. Neurosurgery 15:14–21 doi: 10.1097/00006123-198407000-00004 PubMedCrossRefGoogle Scholar
  5. 5.
    Suwanwela C, Suwanwela N (1972) A morphological classification of sincipital encephalomeningoceles. J Neurosurg 36:201–211PubMedCrossRefGoogle Scholar
  6. 6.
    Woodworth BA, Schlosser RJ, Faust RA et al (2004) Evolutions in the management of congenital intranasal skull base defects. Arch Otolaryngol Head Neck Surg 130:1283–1288 doi: 10.1001/archotol.130.11.1283 PubMedCrossRefGoogle Scholar
  7. 7.
    Schlosser RJ, Faust RA, Phillips CD et al (2002) Three-dimensional computed tomography of congenital nasal anomalies. Int J Pediatr Otorhinolaryngol 65:125–131 doi: 10.1016/S0165-5876(02)00147-7 PubMedCrossRefGoogle Scholar
  8. 8.
    Macfarlane R, Rutka JT, Armstrong D et al (1995) Encephaloceles of the anterior cranial fossa. Pediatr Neurosurg 23:148–158 doi: 10.1159/000120952 PubMedCrossRefGoogle Scholar
  9. 9.
    Hoving EW, Vermeij-Keers C (1997) Frontoethmoidal encephaloceles, a study of their pathogenesis. Pediatr Neurosurg 27:246–256 doi: 10.1159/000121262 PubMedCrossRefGoogle Scholar
  10. 10.
    Hoving EW (2000) Nasal encephaloceles. Childs Nerv Syst 16:702–706 doi: 10.1007/s003810000339 PubMedCrossRefGoogle Scholar
  11. 11.
    Narang R, Pathania OP, Tomar S (1989) An unusual sincipital meningoencephalocele: a case report. J Pediatr Surg 24:208–209 doi: 10.1016/S0022-3468(89)80251-9 PubMedCrossRefGoogle Scholar
  12. 12.
    Schlosser RJ, Gross CW, Kountakis S (2002) Skull base erosion by sphenoid fungus balls: diagnosis and endoscopic treatment. Am J Rhinol 16:161–164PubMedGoogle Scholar
  13. 13.
    Schlosser RJ, Bolger WE (2002) Management of multiple spontaneous nasal meningoencephaloceles. Laryngoscope 112:980–985 doi: 10.1097/00005537-200206000-00008 PubMedCrossRefGoogle Scholar
  14. 14.
    David DJ, Simpson DA (1987) Frontoethmoidal meningoencephaloceles. Clin Plast Surg 14:83–89PubMedGoogle Scholar
  15. 15.
    David DJ, Proudman TW (1989) Cephaloceles: classification, pathology, and management. World J Surg 13:349–357 doi: 10.1007/BF01660747 PubMedCrossRefGoogle Scholar
  16. 16.
    David DJ (1993) Cephaloceles: classification, pathology, and management—a review. J Craniofac Surg 4:192–202PubMedGoogle Scholar
  17. 17.
    Schlosser RJ, Bolger WE (2002) Nasal cerebrospinal fluid leaks. J Otolaryngol 31(Suppl 1):S28–S37 doi: 10.2310/7070.2002.21316 PubMedCrossRefGoogle Scholar
  18. 18.
    Rahman NU (1979) Nasal encephalocoele. Treatment by trans-cranial operation. J Neurol Sci 42:73–85 doi: 10.1016/0022–510X(79)90153–9 PubMedCrossRefGoogle Scholar
  19. 19.
    Lund VJ (2002) Endoscopic management of cerebrospinal fluid leaks. Am J Rhinol 16:17–23PubMedGoogle Scholar
  20. 20.
    Koopmann CF Jr, Reynolds AF (1981) Combined neurosurgical-otolaryngologic operative approach to a massive nasoethmoidal encephalomeningocele. Otolaryngol Head Neck Surg 89:545–549PubMedGoogle Scholar
  21. 21.
    Woodworth B, Schlosser RJ (2005) Endoscopic repair of a congenital intranasal encephalocele in a 23 months old infant. Int J Pediatr Otorhinolaryngol 69:1007–1009PubMedGoogle Scholar
  22. 22.
    Brodie HA (1997) Prophylactic antibiotics for posttraumatic cerebrospinal fluid fistulae. A meta-analysis. Arch Otolaryngol Head Neck Surg 123:749–752PubMedGoogle Scholar
  23. 23.
    Bernal-Sprekelsen M, Bleda-Vazquez C, Carrau RL (2000) Ascending meningitis secondary to traumatic cerebrospinal fluid leaks. Am J Rhinol 14:257–259 doi: 10.2500/105065800779954473 PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 2008

Authors and Affiliations

  • A. Krajewski
    • 1
  • S. J. Borch
    • 1
  • A. Khan
    • 2
  • M. Bhaya
    • 3
  • R. Y. Chandawarkar
    • 1
    • 4
  1. 1.Division of Plastic Surgery, Department of SurgeryUniversity of Connecticut School of MedicineFarmingtonUSA
  2. 2.Division of Neurosurgery, Department of SurgeryUniversity of Connecticut School of MedicineFarmingtonUSA
  3. 3.Private Practice, ENTWaterburyUSA
  4. 4.University of Connecticut Health Center, MC 1601FarmingtonUSA

Personalised recommendations