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A lternative option for osteogenesis imperfecta and trigeminal neuralgia
Rev Assoc Med B ras 2017; 63(4):307-310 307
IMAGE IN MEDICINE
Alternative option for osteogenesis imperfecta and
trigeminal neuralgia
Leonardo Gilmone Ruschel1, Guilherme José A gnoletto1, Sonival Cândido Hunhevicz2*, Daniel Benzecry de A lmeida2,
Walter Oleschko A rruda3
1MD, Neurosurgery Department, Instituto de Neurologia de Curitiba (INC), Curitiba, PR, Brazil
2Neurosurgeon, Neurosurgery Department, INC, Curitiba, PR, Brazil
3Neurologist, Neurology Department, INC, Curitiba, PR, Brazil
Summary
Study conducted at Instituto
de Neurologia de Curitiba (INC),
Curitiba, PR, Brazil
Article received: 10/10/2016
Accepted for publication: 11/7/2016
*Correspondence:
Departamento de Neurocirurgia,
Instituto de Neurologia de Curitiba
Address: Rua Jeremias Maciel
Perreto, 300
Curitiba, PR – Brazil
Postal code: 81210-310
Osteogenesis imperfecta (OI) is a bone disorder that can lead to skull base defor-
mities such as basilar invagination, which can cause compression of cranial nerves,
including the trigeminal nerve. Trigeminal neuralgia in such cases remains a
challenge, given distorted anatomy and deformities. We present an alternative
option, consisting in cannulation of the foramen ovale and classical percutaneous
treatment. Percutaneous balloon microcompression was performed in a 28 year-
-old woman with OI and severe trigeminal neuralgia using computed tomography
(CT) and radiographic-guided cannulation of the Gasserian ganglion without
neuronavigation or stereotactic devices. The patient developed hypoesthesia on
the left V1, V2 and V3 segments with good pain control. This alternative technique
with a CT-guided puncture, using angiosuite without the need of any Mayfield
clamp, neuronavigation systems, frame or frameless stereotactic devices can be a
useful, safe and efficient alternative for patients with trigeminal neuralgia with
other bone deforming diseases that severely affect the skull base.
Keywords: trigeminal neuralgia, pain, osteogenesis imperfect, percutaneous
balloon compression.
Introduction
Osteogenesis imperfecta (OI), an inherited bone disorder,
may produce severe disability and altered bone develop-
ment, leading to multiple fractures after minimal or no
trauma, thus inducing deformity.1-3
Often called “brittle bone disease,” OI leads to various
phenotypes. Mild forms can be premature or postmeno-
pausal osteoporosis, and severe forms can lead to death
in the perinatal period.1,4
Basilar invagination, a clinical manifestation, may cause
cranial nerve compression due to odontoid process protru-
sion through foramenmagnum into the intracranial cavity.5,6
The trigeminal nerve may be involved, resulting in
neuralgic pain, often refractory to drug treatment. Other
possible pathogeneses include arachnoid adhesions and
increased vascularization in the foramen magnum area.7-9
Interventional treatment in such cases is difficult,
mostly due to difficult access and bone fragility, besides
cranial base anatomy distortion.10
In the past, foramen ovale cannulation was considered
impossible for Gasser ganglion microcompression with
conventional radiographic or tomographic-guided per-
cutaneous approach without stereotactic or neuronaviga-
tion devices.11
Case report
Female patient, 28 years old, presenting OI type III, com-
plaining of progressively worsening shooting pain with
onset three years before, affecting her lower left jaw (V3
segment). Pain usually worsened while chewing, swallow-
ing or talking and was refractory to multiple drug treat-
ments, including carbamazepine at maximum dosage
(1,200 mg daily) and pregabalin.
On physical examination, she showed multiple bone
deformities, typical blue sclera, low height, marked tho-
racolumbar kyphoscoliosis and bilateral hearing loss,
marked frontal bossing, no cranial nerve deficits, normal
facial cutaneous sensation, and intact corneal reflexes.