Bone pain management with opioid medication in a patient with Camurati-Engelmann disease: a case report

Resumen

Introduction: Camurati-Engelman Disease is a rare genetic sclerosing bone dysplasia with periosteal and endosteal thickening of the cortical of the long bones. It generates pain secondary to the reduction of the medullary canal that is usually controlled with corticosteroids and, in severe cases, with surgical decompression. Case history: We present the case of a woman with a genetic diagnosis of Camurati-Engelman Disease with poor pain control with corticosteroid management and surgical procedures throughout her childhood and early adulthood. In whom optimal pain control was achieved with pain regimen with hydrocodone analgesic management. This is the first case described in the literature for adequate pain control using an opioid drug. Discussion: CE disease is an extremely rare genetic entity with little more than 300 cases reported in the world. It is generated by an alteration in the gene for growth factor-beta 1 (TGF-B1); it has a varied clinical presentation that can begin with bone alterations accompanied by muscle weakness, joint angular alterations, headache, and nerve compressions. It has a differential diagnosis with some genetic entities that may present clinical similarity, but its morphological and radiological characteristics are distinctive. The usual management of bone pain generated by this entity is based on corticosteroids, in addition to losartan or surgical intervention aimed at reducing cortical changes. The intervention with opioid analgesics accompanied by a rehabilitation plan is not a frequent report, this being a case of success due to the refractoriness of the symptoms in a patient with chronic pain, with a positive impact on her functionality and quality of life. Conclusion: It is considered that analgesic management with opioids may be a treatment option in patients with Camurati-Engelman disease refractory to corticosteroid management and surgical interventions.