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C1-C2 INSTABILITY IN PEDIATRIC PATIENTS WITH SPASTIC CEREBRAL PALSY



Abstract

Objective: To report the development of acquired atlan-toaxial instability in pediatric patients with spastic cerebral palsy and no previous history of traumatic incident.

Study Design-Material: We present three patients with severe spastic quadriplegia who developed C1–C2 instability and cervical myelopathy at mean age 12.6 years.

Results: These patients demonstrated similar clinical picture with symptoms attributed to cervical myelopathy in varied severity including apneic episodes, opisthotonus, alteration in muscle tone, torticollis, respiratory problems, hypereflexia and bradycardia. Patient 1 was scheduled for surgery but died due to an apneic episode. Patient 2 denied surgery and has been followed for 3 years while his neurological condition remains unchanged. Patient 3 underwent decompression through a C1 laminectomy and occiput-C4 posterior spinal fusion, recovered neurologically and resumed his previous functional skills.

Conclusions: The cases included in our study serve to illustrate the numerous difficulties related to the diagnosis and treatment of severely involved children with spastic cerebral palsy complicated with acquired atlan-toaxial instability and myelopathy. A high index of suspicion in patients who demonstrate a significant functional deterioration or a sudden change in their previous neurological status may lead to the diagnosis of cervical myelopathy at an early stage, when the possibility of recovery is higher. The management of spastic patients with documented cervical instability and myelopathy remains controversial, mainly due to the complexity of the medical problems that affect the general condition of these patients. We believe that even in severely involved pediatric patients appropriate management, including spinal cord decompression and fusion, may alter the natural history of myelopathy if the treatment is performed when the neurologic damage is not long standing. If the etiology of myelopathy is not addressed it may result to fatal complications, although clinical progression is not documented in all patients.

The abstracts were prepared by Eleni Koutsoukou. Correspondence should be addressed to him at the Hellenic Association of Orthopaedic Surgery and Traumatology (HAOST), 20, A. Fleming str, 15123 Marousi, Athens, Greece.