Treatment of refractory bone pain with intravenous lidocaine, ketamine and magnesium sulfate in a patient with Camurati-Engelmann Disease. Case report

ABSTRACT

BACKGROUND AND OBJECTIVES  Camurati-Engelmann Disease (CED) or Progressive Diaphyseal Dysplasia is a rare genetic syndrome, characterized by progressive periosteal and endostal hyperostosis mainly in bone diaphysis. CED can presents intense pain that is difficult to control with conventional treatment using corticosteroids and non-steroidal anti-inflammatory drugs (NSAIDs), which may be secondary to diaphyseal hyperostosis and narrowed medullary canal, and in most severe cases, there is a need surgical decompression. The aim of this study was to report the case of a patient diagnosed with CED, in whom sympathetic venous blockade (SVB) was performed by intravenous infusion of lidocaine, ketamine and magnesium sulfate associated with opioids to treat severe chronic pain refractory to several usual treatment methods, bringing to light new therapeutic proposals for pain in this rare disease.

CASE REPORT  A female patient, 38 years old, diagnosed with CED at 16 years old, with intense chronic widespread pain, functional limitation and previous unsuccessful treatments. She underwent a serial intravenous infusion of lidocaine, ketamine, and magnesium sulfate associated with opioid use, resulting in a significant reduction in pain intensity, improvement in physical limitation and increasing the interval between pain attacks. This report is unprecedented, with no previous description of this therapeutic approach to control pain in this disease.

CONCLUSION  Treatment with intravenous infusion of lidocaine, ketamine and magnesium sulfate associated with opioid can be a safe option for treating refractory pain related to CED.

Keywords:
Carmurati-Engelmann syndrome; Chronic pain; Lidocaine; Opioid analgesics; Rare diseases

HIGHLIGHTS

Patient with Camurati-Engelmann disease, a rare syndrome with severe pain in the upper limbs, lower limbs, spine, face and headache, with no response to conventional treatment with corticosteroids and non-steroidal anti-inflammatory drugs

Pain relief with weekly sympathetic venous blockade associated with opioid

A new perspective on pain relief