title: Management of trigeminal neuralgia in sclerosteosis.
abstract: BACKGROUND
  Sclerosteosis is a rare bone disorder characterized by a progressive craniotubular hyperostosis. The diagnosis of sclerosteosis is based on characteristic clinical and radiographic features and a family history consistent with autosomal recessive inheritance. The skull overgrowth may lead to lethal elevation of intracranial pressure, distortion of the face, and entrapment of cranial nerves, resulting in recurrent facial palsy or secondary trigeminal neuralgia.
  
  
  METHODS
  The authors reported cases of two siblings who were diagnosed with familial sclerosteosis and presented with secondary trigeminal neuralgia. The patients were 28 and 40-year-old and presented with pain in the right V2-V3 and V3 distributions, respectively. The facial pain was resistant to medications and was treated with percutaneous techniques. The foramen ovale puncture was complicated initially and the difficulty increased over the years due to stenosis of the foramen.
  
  
  CONCLUSIONS
  The treatment of the trigeminal neuralgia secondary to hyperostosis and resistant to medications presents a dilemma. The narrowing of the foramen oval and difficulty in the identifying and approaching of the foramen makes the percutaneous technique a challenge for the neurosurgeon in patients harboring sclerosteosis. Microvascular decompression should not be considered since the primary cause of the trigeminal neuralgia is the nerve entrapment by the narrowing of neurovascular foramina and not the neurovascular conflict related to essential trigeminal neuralgia. Stereotactic radiosurgery may be a good treatment option, but there is a lack of published data supporting the use of this method in cranial hyperostosis.
authors.affiliation: Division of Functional Neurosurgery - Department of Neurosurgery, University of São Paulo Medical School (FMUSP); São Paulo, Brazil.
authors.forename: Emerson Magno
authors.initials: EM
authors.lastname: de Andrade
authors.affiliation: Division of Functional Neurosurgery - Department of Neurosurgery, University of São Paulo Medical School (FMUSP); São Paulo, Brazil.
authors.forename: André
authors.initials: A
authors.lastname: Beer-Furlan
authors.affiliation: Division of Functional Neurosurgery - Department of Neurosurgery, University of São Paulo Medical School (FMUSP); São Paulo, Brazil.
authors.forename: Kleber Paiva
authors.initials: KP
authors.lastname: Duarte
authors.affiliation: Division of Functional Neurosurgery - Department of Neurosurgery, University of São Paulo Medical School (FMUSP); São Paulo, Brazil.
authors.forename: Erich Talamoni
authors.initials: ET
authors.lastname: Fonoff
authors.affiliation: Division of Functional Neurosurgery - Department of Neurosurgery, University of São Paulo Medical School (FMUSP); São Paulo, Brazil.
authors.forename: Manoel Jacobsen
authors.initials: MJ
authors.lastname: Teixeira
keywords: Percutaneous balloon compression; pain control; sclerostesosis; trigeminal neuralgia