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.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.
Highlights
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
Sclerosteosis is a rare bone disorder characterized by a progressive craniotubular hyperostosis
We presume that SRS to be a good alternative in patients harboring sclerosteosis and presenting with no acute pain, since there is no need for foramen oval puncture or craniotomy, but no evidence has been published about the results of this technique in patients with cranial hyperostosis
Summary
The treatment of the trigeminal neuralgia secondary to hyperostosis and resistant to medications presents a dilemma. 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. 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 [Figure 2]. It should be noted that the difficulty in the percutaneous procedures increased over the years due to stenosis of the foramen ovale Today his neuralgia is controlled with no medications and he presents with hypoestesia in the right V3 territory. 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. It is important to note, similar do case 1, the increasing difficulty of the foramen ovale puncture over the years due to its stenosis
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