Neuropediatrics 2005; 36 - P99
DOI: 10.1055/s-2005-868084

Neuropathic osteoarthropathy in Meningomyelocele (MMC): A case-report

M Walther 1, J Rössler 2, R Korinthenberg 1, V Mall 1
  • 1Zentrum für Kinderheilkunde und Jugendmedizin, Klinik II: Abteilung für Neuropädiatrie und Muskelerkrankungen, Freiburg
  • 2Zentrum für Kinderheilkunde und Jugendmedizin, Klinik IV: Abteilung für Onkologie und Hämatologie, Freiburg

Introduction: Neuropathic osteoarthropathy is a typical destructive process, based on a neurosensoric disease. It has been primarily characterized by Charcot. Currently there are two theories, elucidating the appearance of neuropathic osteoarthropathy: On the one hand the neurotraumatic theory by Eloesser, postulating the lack of protective sensory feedback after traumatic lesions and on the other hand the neurovascular theory based on vascular changes caused by the failure of sympathetic tone, thereby vasodilatation and hyperemia activate vascular changes.

In addition to acquired neuropathic diseases, spinal tumors and congenital neuropathic deseases like syringomyelia and meningomyelocele (MMC) provoke neuropathic Osteoarthropathy.

Case: Patient history:

A 10 8/12 years old boy with MMC (palsy from L4) suffered from progressive swelling and elevated temperature at the left thigh since 9 month. There was no history of trauma. In October 2000 he received an intertrochantic osteotomy at the left hip which been revised in January 2001. A period of immobilisation followed each orthopaedic surgery.

Diagnostic findings: According to age, we detected normal results for leukocytes, CRP and ESR. A radiograph of the left thigh depicted a exuberant bone formation over the entire length of the femur. The bioptic findings showed a distinctive reactive fibro-histiocytic proliferation of the skeleton-muscles socialized with a mild, chronical inflammatory concomitant reaction. Neither in smear test, nor in bioptic material germs were detected.

Discussion: The neuropathic osteoarthropathy is a known destructive process in patients with MMC. Frequently this process is triggered by a subacute painless fracture after immobilisation. Often the fracture is not recognized as traumatic incidence. Swelling, redness and local hyperthermy are usually first symptoms. This symptoms can be often misattributed and mistreated like a osteomyelitis, a metaphyseal dysplasia or a osteosarcoma.

Normally radiographic features point at the proper diagnosis. A biopsy can usually be avoided.