Alternative option for osteogenesis imperfecta and trigeminal neuralgia

Abstract

Osteogenesis imperfecta (OI) is a bone disorder that can lead to skull base deformities 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.

Clinical manifestations of osteogenesis imperfecta in adulthood: An integrative review of quantitative studies and case reports

“…3.12 | Other clinical manifestations: Presence of basilar invagination, gastrointestinal issues, and genitourinary problems A paucity of research has been observed in the literature concerning basilar invagination, gastrointestinal issues, and genitourinary problems. Briefly, three case reports addressed the issue of trigeminal neuralgia, which was due to basilar invagination (Hajioff, Dorward, Wadley, Crockard, & Palmer, 2000;Iwai, Ishibashi & Yamanaka, 2018;Ruschel, Agnoletto, Hunhevicz, Almeida, & Arruda, 2017). Augustin, Jelincic, Majerovic, and Stefancic (2007) examined the obstructed colon of a woman with OI Type III caused by a pelvic stenosis.…”

Desafios enfrentados pelos enfermeiros frente à criança com osteogênese imperfeita

“…Osteogenesis imperfecta (OI) is known to be a disease characterized by bone fragility triggered by a qualitative or quantitative defect of collagen type one synthesized by osteoblasts. 1 The frequency of 6-7 / 100,000 individuals has been shown in studies, and in Brazil it is estimated that there are around 12,000 individuals with this diagnosis. 2 OI is defined as a rare genetic disease that primarily affects tissue rich in collagen type one present in bone tissue, ligaments, tendons, dentin, sclera and skin.…”

“…6 It is understood that OI, as a rare disease, does not have wide wisdom and management by health professionals, however, individuals affected by such pathology need specialized assistance for their clinical condition. 1,4 For the definition of the research scenario, a survey was conducted from the institute's databases and it was found that 164 children were hospitalized from 2009 to 2019 with diagnosis of OI in order to undergo some surgical treatment due to injuries caused by the underlying pathology. Among the children who underwent hospitalization and surgery, 7.4% were infants, 27% were preschoolers, 45% were school age and 20.6% were adolescents.…”

“…It is also a laboratory diagnosis in the evaluation of calcium metabolism, and is useful for ruling out pre-existing hypokalemia and hyperparathyroidism. 1 In a relatively recent past, OI treatment was conducted by conservative measures, which stimulated minimal physical activity and, when the fracture was installed, was treated with surgical correction. Currently, treatment is associated with surgical-orthopedic procedures for the treatment of fractures or deformities together with drugs that reduce bone fragility.…”

“…It is recommended to use it under the following conditions: more than three fractures per year, the last two years without significant trauma; need for surgery due to vertebral fractures or deformities and radiographs showing signs of OI. 1 Patients with osteogenesis imperfecta are known to need care from their support network and all family support in an attempt to avoid fractures. This requires both home and recreational environments to have a different architecture to minimize the risk of fractures.…”