- Division of Functional Neurosurgery - Department of Neurosurgery, University of São Paulo Medical School (FMUSP); São Paulo, Brazil
Emerson Magno de Andrade
Division of Functional Neurosurgery - Department of Neurosurgery, University of São Paulo Medical School (FMUSP); São Paulo, Brazil
DOI:10.4103/2152-7806.121644Copyright: © 2013 de Andrade EM. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: de Andrade EM, André Beer-Furlan, Duarte KP, Fonoff ET, Teixeira MJ. Management of trigeminal neuralgia in sclerosteosis. Surg Neurol Int 20-Nov-2013;4:
How to cite this URL: de Andrade EM, André Beer-Furlan, Duarte KP, Fonoff ET, Teixeira MJ. Management of trigeminal neuralgia in sclerosteosis. Surg Neurol Int 20-Nov-2013;4:. Available from: http://sni.wpengine.com/surgicalint_articles/management-of-trigeminal-neuralgia-in-sclerosteosis/
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.
Cases Description: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.
Conclusion: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.
Keywords: Percutaneous balloon compression, pain control, sclerostesosis, trigeminal neuralgia
Sclerosteosis is a rare bone disorder characterized by a progressive craniotubular hyperostosis. It is caused by mutations in SOST gene encoding sclerostin protein, an osteocyte-specific secreted protein that is likely involved in the suppression of bone formation.[
The diagnosis of sclerosteosis is based on clinical characteristics, radiographic features, and a family history consistent with autosomal recessive inheritance. The main features are a significant sclerosis of the long bones, ribs, pelvis, and skull.
The skull overgrowth may lead to lethal elevation of intracranial pressure (ICP), distortion of the face, and entrapment of cranial nerves. The clinical result is conductive hearing loss in childhood followed by additional entrapment of the eighth cranial nerve and closure of the oval and round windows, leading to sensorineural hearing loss in adulthood.[
The authors present here the management of TN in two siblings with diagnosed familial sclerosteosis and discuss the possibilities and potential complication of treatment modalities.[
A 40-year-old male patient started his symptoms at the age of 10. At the beginning, he presented intermittent right side facial muscle paralysis and jaw pain. Over the years the symptoms progressed to bilateral facial muscle paralysis, bilateral hypoacusia, headaches, and right side facial pain (jaw, teeth, and tongue).
In 2002, he underwent a dental procedure in another institution because of his jaw pain. In 2004, at the age of 30, the patient moved to São Paulo where he was diagnosed with sclerosteosis and TN affecting the right mandibular branch - V3 [
The patient had a 2-year period with no facial pain and another 2 years with the pain under control with the use of gabapentin. The control of the TN became worse, despite the increasing doses, and combination of medications. Another percutaneous RFR was performed to treat his right V3 pain in 2012 [
A 28-year-old woman started her symptoms at the age of 1. Initially she presented an episode of right facial palsy that evolved with spontaneous remission after 3 months. After 1 year, she had new-onset facial palsy that affected her left facial side, with complete resolution after 3 months. Over the next years, she experienced recurrent facial palsies and suffered from several headaches. A generous bilateral bifrontotemporal decompressive craniectomy was then performed to treat the symptoms of raised ICP that improved after the surgical procedure [
At the age of 21, the patient was diagnosed with sclerosteosis and TN in the right V2 and V3 distributions. The patient was primarily treated with carbamazepine and gabapentin until presented with an adverse reaction, before being considered for surgery. She underwent a PBC for treatment of the right facial pain in 2007. After surgery, the patient had an immediate and complete resolution of her V2 and V3 pain but experienced right facial numbness in these trigeminal distributions. After 3 years, the pain recurred with the same intensity and a new PBC was performed. She was again placed on medical therapy with carbamazepine and the pain improved in the immediate postoperative period.
After 2 years, in 2012 the pain recurred with the same initial characteristics and another PBC was performed [
TN may be classified as idiopathic or symptomatic according to the etiology. In the idiopathic or essential TN the etiology is unknown. Patients describe a short, intermittent, and sharp or electric shock like one-sided pain affecting one or more division of the trigeminal nerve. In the symptomatic TN, also called secondary, an etiology for the symptoms is identified. Multiple sclerosis and posterior fossa tumors are the most frequent cause of symptomatic TN.[
The molecular genetic studies of patients with the sclerosing bone dysplasias revealed that the loss of function of SOST gene results in an osteoblast hyperactivity and is involved in the pathogenesis of this condition, as well as in the van Buchem disease. Kim et al.[
Tacconi et al.[
Hofmeyr et al.[
Facial nerve palsy usually presents as an acute and recurrent paralysis, and many patients are frequently diagnosed erroneously as suffering from Bell Palsy. Dort et al.[
TN in the related cases may be attributed to the bony growth that occurs in the superior orbital fissure and in foramen ovale and rotundum. The slow compression of the trigeminal branches can induce a progressive degeneration of the nerve.[
Percutaneous techniques for treatment of TN include RFR, PBC, and percutaneous retrogasserian glycerol rhizolysis (PRGR). According to a systematic review,[
In patients harboring sclerosteosis the percutaneous techniques are hindered due to the narrowing and difficulty in identifying the foramen ovale. In such cases, the use of CT-guided percutaneous rhizotomy with three dimensional imaging reconstructions, or a neuronavigation system can facilitate the needle placement and reduce the risk of complications.[
Microvascular decompression (MVD) leads to the highest rate of pain control and is the most durable surgical treatment, however, is more invasive than other methods and requires general anesthesia and a craniotomy. Several authors recommend that MVD must be the method of choice even in elderly patients, since they present with good health conditions.[
Stereotactic radiosurgery (SRS) can be an effective alternative in the treatment of TN.[
Compared with open surgery and percutaneous techniques, the SRS is less invasive, more precise and has a reduced amount of postoperative complications. The mean disadvantages of radiosurgery are the high rate of recurrence and the time latency to provide pain relief after radiation treatment.[
The treatment of the TN secondary to hyperostosis and resistant to medications presents a dilemma. The percutaneous technique is a reasonable treatment option in sclerosteosis patients, but the narrowing of the foramen oval and difficulty in the identifying and approaching of the foramen makes this modality of approach a challenge for the neurosurgeon. Although MVD is recognized as the “gold standard” treatment for patients with TN, there is no role for this surgical approach in patients harboring sclerosteosis since the etiology of pain is diverse and not related to the neurovascular conflict. SRS provide a minimally invasive option for management of TN in patients who are not able or reluctant to undergo surgery, but no evidence has been published about the results of this technique in patients with cranial hyperostosis.
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