Elsevier

World Neurosurgery

Volume 115, July 2018, Pages 24-28
World Neurosurgery

Case Report
Gamma Knife Radiosurgery for Trigeminal Neuralgia Associated with Osteogenesis Imperfecta

https://doi.org/10.1016/j.wneu.2018.03.192Get rights and content

Highlights

  • Trigeminal neuralgia is a rare condition of basilar impression associated osteogenesis imperfecta.

  • Trigeminal neuralgia associated with osteogenesis imperfecta is difficult for surgical treatment due to complexity of skull base.

  • Gamma knife radiosurgery is a novel approach for trigeminal neuralgia associated with osteogenesis imperfecta.

Background

Trigeminal neuralgia is a rare feature of basilar impression, a complication of osteochondrodysplasic disorders. Microvascular decompression is difficult in medically refractory cases. Gamma knife radiosurgery (GKS) is effective for classical trigeminal neuralgia, and we first applied this GKS for a patient suffering from trigeminal neuralgia with basilar impression complicated by osteogenesis imperfecta.

Case Description

An 18-year-old man with type I osteogenesis imperfecta presented with a 2-year history of typical left trigeminal neuralgia affecting the ophthalmic, maxillary, and mandibular nerves. His condition was poorly controlled by carbamazepine and pregabalin. Magnetic resonance imaging confirmed severe basilar impression and an elongated cisternal portion of the left trigeminal nerve. GKS was performed under local anesthesia. The middle part of the cisternal portion of the left trigeminal nerve was chosen as the radiosurgical target, despite the lack of neurovascular compression. The maximum radiosurgical dose was 85 Gy, using a single 4-mm collimator. The patient's trigeminal neuralgia improved by 1 month after GKS, and at 21 months after GKS he remained free of pain and medications.

Conclusions

We report a case of trigeminal neuralgia resulting from severe basilar impression. Conventional microvascular decompression would not have been an appropriate treatment for this patient, so GKS was used. GKS is a simple and effective option even in cases with severe cranial deformity.

Introduction

Osteogenesis imperfecta, a rare inherited bone disorder marked by fractures from trivial bony injuries, was first linked to basilar impression in the 1940s.1 Osteogenesis imperfecta is a clinically and genetically heterogeneous group of heritable disorders of connective tissue characterized by reduced bone mass (osteopenia) with associated bone fragility.2

Basilar impression, the most common malformation of the craniocervical region, is the upward displacement of basilar and condylar portions of the occipital bone, causing an infolding of the foramen magnum and leading to translocation of the upper cervical spine into the brainstem.2 In such cases, the brainstem and cranial nerves are distorted by the rostrally displaced clivus-atlas-odontoid complex within the foramen magnum, leading to potentially fatal neurologic dysfunction.

Neurologic symptoms resulting from basilar impression include headache (typically occipital and precipitated by cough and laughter), trigeminal neuralgia, vertigo or imbalance precipitated by head movement, weakness in legs and arms, sleep apnea, and seizures.2

Successful treatment of trigeminal neuralgia is rarely reported. Moreover, microvascular decompression (MVD) has been unsuccessful because of difficulty exploring the posterior fossa. A recent report described a patient successfully treated by MVD.3

Gasselian ganglion block is difficult due to anatomic variation.4 Thus, we explored the use of gamma knife radiosurgery (GKS) to treat the trigeminal neuralgia associated with osteogenesis imperfecta. Here we report a novel GKS technique for treating osteogenesis imperfecta–associated trigeminal neuralgia.

Section snippets

Case Presentation

An 18-year-old man diagnosed with severe type I osteogenesis imperfecta (heterozygous mutant gene of the COL1A1 gene) had experienced left trigeminal neuralgia at the maxillary and mandibular divisions starting at age 16. Chewing and talking seemed to exacerbate the episodes, but there were no trigger points on the face. The pain was often severe enough to awaken him.

At first, the patient was treated with carbamazepine and pregabalin, and the neuralgia was controlled. However, by age 18, the

Osteogenesis Imperfecta and Basilar Impression

Osteogenesis imperfecta is a heterogeneous group of disorders characterized by abnormal formation of type 1 collagen, resulting in extreme bone fragility and inclination to fracture.5 In most cases, the disease is mild, and adults have minimal deformity.

Basilar impression is a rarely reported but potentially devastating complication that may also occur in Paget disease, rickets, Hadju–Cheney syndrome, and other osteochondrodysplasias.6 The weight of the cranium may progressively deform the

Conclusions

We report a rare case of trigeminal neuralgia resulting from severe basilar impression without vascular compression in which conventional MVD would not have been appropriate and so GKS was used. GKS is a simple and effective procedure, even in cases of severe cranial deformity.

Acknowledgment

We thank Dr. Masahiro Nonaka (Department of Neurosurgery, Kansai Medical College, Hirakata, Osaka) and Motohiro Kitano (Department of Orthopedics, National Hospital Organization, Osaka National Hospital, Osaka) for referring the patient and for productive discussions.

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    Citation Excerpt :

    Unexpectedly, acetabular protrusion, the displacement of the femoral head medial from the ilio-ischial line (Ajlouni et al., 2020; Violas et al., 2002), has been suggested to contribute to recurrent abdominal pain in OI (Lee et al., 1995). While pain in OI is mainly nociceptive, trigeminal neuralgia is a frequent source of neuropathic pain (Iwai et al., 2018; Ruschel et al., 2017) and arises due to basilar skull displacement (Hayes et al., 1999; Ray, 1942). In OI, higher pain scores are associated with worse quality of life (Nghiem et al., 2017).

Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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