RESUMO
Osteopetrosis is a genetic illness defined by defective osteoclasts that are incapable of absorbing adequate amounts of bone. This exceedingly rare disorder has been linked to multiple genetic mutations that have a direct impact on osteoclast function. Osteopetrosis causes bones to become brittle with large amounts of cortical bone formation making patients susceptible to pathologic fractures, pancytopenia, and cranial neuropathies among other sequelae. Known as the "marble bone disease," this condition can range from as severe as causing death in newborn infants to as mild as an incidental finding of increased cortical thickening in a trauma patient. This case demonstrates an incidental finding of osteopetrosis in a trauma patient who suffered from significant injuries as a result of a high-velocity trauma. The patient was the pedestrian in a car vs pedestrian accident and suffered from a central cord syndrome in his cervical spine, a right humerus fracture, a left subtrochanteric femur fracture, a right tibia fracture, and a right fibula fracture. This case report illustrates the complexity of dealing with a polytrauma patient with osteopetrosis and reviews the literature on the approach to fracture fixation in osteopetrotic individuals. This paper will also discuss current medication recommendations and the current standard of care for optimizing patients with osteopetrosis as well as genetic counseling.
RESUMO
BACKGROUND: Traditional manual retraction to access deep-seated brain lesions has been associated with complications related to vascular compromise of cerebral tissue. Various techniques have been developed over time to minimize injury, such as self-sustaining retractors, neuronavigation, and endoscopic approaches. Recently, tubular retractors, such as the ViewSite Brain Access System (VBAS), have been developed to reduce mechanical damage from retraction by dispersing the force of the retractor radially over the parenchyma. Therefore, we sought to review the current literature to accurately assess the indications, benefits, and complications associated with use of VBAS retractors. METHODS: A literature search for English articles published between 2005 and 2019 was performed using the MEDLINE database archive with the search terminology "Vycor OR ViewSite OR Brain-Access-System NOT glass." The VBAS website was also examined. Only articles detailing neurosurgical procedures using the VBAS tubular retractor system alone, or in combination with other retractors, were included. Postoperative morbidity and mortality were analyzed to estimate complications linked to using the retractor. RESULTS: Twelve publications (106 patients) met the inclusion criteria. The VBAS retractor was used for tumor resections, hematoma evacuations, cyst removal, foreign body extractions, and lesion resection in toxoplasmosis and multiple sclerosis. These cases were subdivided into groups based on lesion location, size, and resection volume for further analysis. Gross total resection was achieved in 63% of tumor excisions, and subtotal resection was achieved in 37%. Hematoma evacuation was successful in all cases. There were 3 short-term postoperative complications linked to the retractor, with an overall complication rate of 2.8%. CONCLUSIONS: This report is the first formal assessment of the VBAS, highlighting technical considerations of the retractor from the surgeon's perspective, patient outcomes, and complications. The retractor is a safe and efficacious tubular retraction system that can be used for tumor biopsy and resection, colloid cyst removal, hematoma evacuation, and removal of foreign bodies. However, further randomized controlled trials are indicated to accurately assess complication rates and outcomes.
