gms | German Medical Science

Objectives/Interrogation: Melorheostosis is a rare mesenchymal dysplasia. It develops with a sclerotomal distribution, usually affecting one limb. On plain radiographs the irregular cortical hyperostosis occurring on one side of the affected bone is seen as the characteristic melting "candle wax" sign. We present two rare cases of melorheostosis of the hand.

Methods: The first case involves a 55 years old Greek woman presenting with melorheostosis of her left hand. The disease involved the lunate, the capitate, the metacarpal and the phalanges of the middle and index fingers. The patient complained for pain and dyskinesia due to bony spurs between the metacarpals, as well as the proximal and middle phalanges. At presentation flexion at the metacarpophalangeal (MCP) joint of the index finger was at 20o and at the proximal interphalangeal (PIP) joint was 0o.

Although the difficulties of any surgical intervention were presented to the patient, she insisted on being operated. Under general anesthesia and tourniquet a straight incision was performed between the second and third metacarpal, while as a second straight incision at the radial site of the middle finger. Although extensive tenolysis of the extensor tendons successful, during the procedure to remove the marble - like bony spurs two saw blades were destroyed and any effort to continue stopped. A plaster cast was used for protection and rest and the patient was discharged from hospital the next day.

The second case involves a 36 years old Albanian woman who reported pain and mild stiffness at her left hand. The radiological studies revealed melorheostosis affecting the lunate, the capitate, the metacarpal bones of the middle and index finger, and the proximal and middle phalanx of the middle finger.

Results: At three months postoperatively the first patient showed no improvement in relation to her previous condition. Also she denied any suggestion of ray amputation of any or both of the affected fingers. The second patient continues her professional as a hair stylist, without the need for any surgical intervention.

Conclusion: Literature lacks evidence considering effective surgical intervention for the cortical hyperplasia in cases of melorheostosis of the hand. Ray amputation seems as the only alternative in cases of extensive hand dysfunction, only after taking into consideration the special needs of the patient.