Background: The etiology of multifocal osteonecrosis is not definitively known; however, hypercoagulable state is a very plausible cause. Methods: We present an unusual case of a 12-year-old boy with a history of Legg-Calve-Perthes disease presenting with right wrist pain who was subsequently diagnosed with Kienbock's disease. The finding of multifocal osteonecrosis prompted testing for a hypercoagulable state that was positive for Factor V Leiden thrombophilia. A thorough literature review using Medline database was conducted to investigate associations between inherited hypercoagulable states and multifocal osteonecrosis. Results: Our literature review identified 2 similar cases of multifocal osteonecrosis associated with a hypercoagulable disorder in adult patients. There were no reports among the pediatric patient population. Meta-analysis has demonstrated a potential link between Legg-Calve-Perthes disease and Factor V Leiden thrombophilia. Conclusions: This study offers further evidence to support the theory that multifocal osteonecrosis may be linked to a hypercoagulable state. Patients presenting with multifocal osteonecrosis should undergo screening for hypercoagulable states. Further investigation is needed to ascertain the potential benefit of prophylactic anticoagulation in patients with a known hypercoagulable state and multifocal osteonecrosis.
Background: The etiology of multifocal osteonecrosis is not definitively known; however, hypercoagulable state is a very plausible cause. Methods: We present an unusual case of a 12-year-old boy with a history of Legg-Calve-Perthes disease presenting with right wrist pain who was subsequently diagnosed with Kienbock's disease. The finding of multifocal osteonecrosis prompted testing for a hypercoagulable state that was positive for Factor V Leiden thrombophilia. A thorough literature review using Medline database was conducted to investigate associations between inherited hypercoagulable states and multifocal osteonecrosis. Results: Our literature review identified 2 similar cases of multifocal osteonecrosis associated with a hypercoagulable disorder in adult patients. There were no reports among the pediatric patient population. Meta-analysis has demonstrated a potential link between Legg-Calve-Perthes disease and Factor V Leiden thrombophilia. Conclusions: This study offers further evidence to support the theory that multifocal osteonecrosis may be linked to a hypercoagulable state. Patients presenting with multifocal osteonecrosis should undergo screening for hypercoagulable states. Further investigation is needed to ascertain the potential benefit of prophylactic anticoagulation in patients with a known hypercoagulable state and multifocal osteonecrosis.
Entities:
Keywords:
Factor V Leiden; Kienbock’s; Legg-Calve-Perthes disease; avascular necrosis; lunate
Authors: Jose A Gómez-Puerta; Pilar Peris; Joan Carles Reverter; Gerard Espinosa; Angeles Martinez-Ferrer; Ana Monegal; Juan Monteagudo; Dolors Tàssies; Nuria Guañabens Journal: Medicine (Baltimore) Date: 2013-11 Impact factor: 1.889