• Muhammad Umar Qayyum Govt of Pakistan
  • Ehtesham ul Haq Ehtesham
  • Syed Atif Mahmood Kazmi
  • Rubbab Zahra




Neurofibromatosis, Sphenoid Bone, Craniofacial Fibrous Dysplasia, Craniotomy


Sphenoidal Dysplasia is the absence of complete or a part of sphenoid bone, most commonly the greater wing of sphenoid. It can occur as an isolated deformity or in Neurofibromatosis-1 (NF1). Features of NF1 include café au lait spots, inguinal or axillary freckling, neurofibromas, optic gliomas, scoliosis and tibial deformity. Our study is retrospective case series of 3 cases of Sphenoid wing dysplasia. There was 1 case of isolated bone defect, 1 case of NF-1 and 1 case of operated Craniofacial Fibrous Dysplasia involving the sphenoid wing. There were 2 primary operated cases while 1 was operated secondarily. There was resolution of pulsatile exophthalmos in patient with sphenoid and temporal bone defect. Patient with facial deformity NF1 was debulked to the satisfaction of the patient, the patient however declined surgery to correct the sphenoid bone deformity. The 3rd patient was a re-do surgery patient in which the previous implant material was removed and the CSF rhinorrhoea, the patient did not consent to the correction of vertical orbital dystopia. Sphenoid wing dysplasia is a complex deformity requiring multi-speciality care and treatment planning. With meticulous planning and surgery, good results can be achieved as shown in our case series.


Huson SM, Compston DA, Clark P, Harper PS. A genetic study of von Recklinghausen neurofibromatosis in south east Wales. I. Prevalence, fitness, mutation rate, and effect of parental transmission on severity. J Med Genet 1989;26(11):704–11.

Huson SM, Compston DA, Harper PS. A genetic study of von Recklinghausen neurofibromatosis in south east Wales. II. Guidelines for genetic counselling. J Med Genet 1989;26(11):712–21.

Havlik R J, Thorne CH, Chung KC, Gosain AK, Gurtner GC, Mehrara BJ, et al. Miscellaneous Craniofacial Conditions. In: eds. Grabb and Smith’s Plastic Surgery. 7th ed. Philadelphia: Lippincott Williams & Wilkins 2014; p.306–8.

Jackson IT, Carbonnel A, Potparic Z, Shaw K. Orbitotemporal neurofibromatosis: classification and treatment. Plast Reconstr Surg 1993;92(1):1–11.

Harkens K, Dolan KD. Correlative imaging of sphenoid dysplasia accompanying neurofibromatosis. Ann Otol Rhinol Laryngol 1990;99(2 Pt 1):137–41.

Macfarlane R, Levin AV, Weksberg R, Blaser S, Rutka JT. Absence of the greater sphenoid wing in neurofibromatosis type I: congenital or acquired: case report. Neurosurgery 1995;37(1):129–33.

Dandy WE. An operative treatment for certain cases of meningocele (or encephalocele) into the orbit. Arch Ophthalmol 1929;2:123–32.

Binet EF, Kieffer SA, Martin SH, Peterson HO. Orbital dysplasia in neurofibromatosis. Radiology 1969;93(4):829–33.

LeWald L. Congenital absence of the superior orbital wall associated with pul- sating exophthalmos: report of four cases. Am J Roentgenol 1933;93:756–64.

Mortada A. Pulsating exophthalmos with orbital neurofibromatosis. Am J Ophthalmol 1967;64(3):462–464.

Mukherji MM. Giant neurofibroma of the head and neck. Plast Reconstr Surg 1974;53(2):184–9.

Robertson EG. Pulsating exophthalmos due to defective development of the sphenoid bone. Am J Roentgenol Radium Ther 1949;62(1):44–51.

Rovit RL, Sosman MC. Hemicranial aplasia with pulsating exophthalmos. An unusual manifestation of von Recklinghausen's disease. J Neurosurg 1960;17:104–21.

Sivaramasubrahmany AM P. Pulsating exophthalmos due to neurofibromatosis. Br J Ophthalmol 1965;49(2):106–8.

Havlik RJ, Boaz J. Cranio-orbital-temporal neurofibromatosis: are we treating the whole problem? J Craniofac Surg 1998;9(6):529–35.

Snyder BJ, Hanieh A, Trott JA, David DJ. Transcranial correction of orbital neurofibromatosis. Plast Reconstr Surg 1998;102(3):633–42.

Wu CT, Lee ST, Chen JF, Lin KL, Yen SH. Computer-aided design for three-dimensional titanium mesh used for repairing skull base bone defect in pediatric neurofibromatosis type 1. A novel approach combining biomodeling and neuronavigation. Pediatr Neurosurg 2008;44(2):133–9.

Friedman JM. Epidemiology of neurofibromatosis type 1. Am J Med Genet 1999;89(1):1–6.

Martin MP, Olson S. Post-operative complications with titanium mesh. J Clin Neurosci 2009;16(8):1080–1.

Probst C. Multiple frontobasal meningoencephaloceles in neurofibromatosis. Neurofibromatosis 1989;2(4):233–7.

Hunt JC, Pugh DG. Skeletal lesions in neurofibromatosis. Radiology 1961;76:1–20.

Fadda MT, Giustini SS, Verdino GG, Bartoli DD, Mustazza MC, Iannetti GG, et al. Role of maxillofacial surgery in patients with neurofibromatosis type I. J Craniofac Surg 2007;18(3):489–96.

Serletis D, Parkin P, Bouffet E, Shroff M, Drake JM, Rutka JT. Massive plexiform neurofibromas in childhood: natural history and management issues. J Neurosurg 2007;106(5 Supl):363–7.

Prada CE, Rangwala FA, Martin LJ, Lovell AM, Saal HM, Schorry EK, et al. Pediatric plexiform neurofibromas: impact on morbidity and mortality in neurofibromatosis type 1. J Pediatr 2012;160(3):461–7.

Niddam J, Bosc R, Suffee TM, Le Guerinel C, Wolkenstein P, Meningaud JP. Treatment of sphenoid dysplasia with a titanium-reinforced porous polyethylene implant in orbitofrontal neurofibroma: report of three cases. J Craniomaxillofac Surg 2014;42(8):1937–41.

Gaillard S, Pellerin P, Dhellemmes P, Pertuzon B, Lejeune J, Christiaens JL. Strategy of craniofacial reconstruction after resection of spheno-orbital “en plaque” meningiomas. Plast Reconstr Surg 1997;100(5):1113–20.

Kosaka M, Matsuzawa Y, Mori H, Matsunaga K, Kamishii H. Orbital wall recon- struction with bone grafts from the outer cortex of the mandible. J Craniomaxillofac Surg 2004;32(6):374–80.

Metzger MC, Schön R, Schmelzeisen R. Preformed titanium meshes: a new standard? Skull Base 2007;17(4):269–72.

Bikmaz K, Mrak R, Al-Mefty O. Management of bone-invasive, hyperostotic sphenoid wing meningiomas. J Neurosurg 2007;107(5):905–12.

Gear AJ, Lokeh A, Aldridge JH, Migliori MR, Benjamin CI, Schubert W. Safety of titanium mesh for orbital reconstruction. Ann Plast Surg 2002;48(1):1–9.

Schubert W, Gear AJ, Lee C, Hilger PA, Haus E, Migliori MR, et al. Incorporation of titanium mesh in orbital and midface reconstruction. Plast Reconstr Surg 2002;110(4):1022–32.