Radek Kaiser1, Esin Rothenfluh2, Dominique Rothenfluh2, Eyal Behrbalk2, Ana Belen Perez Romera2, Oliver M Stokes2, Hossein Mehdian2. 1. The Centre for Spinal Studies and Surgery, Queen's Medical Centre, Nottingham University Hospitals NHS Trust, Derby Rd, Nottingham, UK. Electronic address: rkaiser@hotmail.cz. 2. The Centre for Spinal Studies and Surgery, Queen's Medical Centre, Nottingham University Hospitals NHS Trust, Derby Rd, Nottingham, UK.
Abstract
BACKGROUND CONTEXT: Proteus syndrome (PS) is an extremely rare congenital disorder causing asymmetric overgrowth of different tissues. The etiology remains unclear. Limb deformities are common and often necessitate amputations. Only a few cases associated with spinal deformities have been described. PURPOSE: The aim was to report a rare case of PS associated with spinal deformity and its surgical management. STUDY DESIGN: A case of young boy with PS causing vertebral hypertrophy and kyphoscoliotic deformity, which was surgically corrected, is presented. METHODS: The patient was assessed clinically and with whole spine plain radiographs, computed tomography, and magnetic resonance imaging. Surgical correction was performed. RESULTS: Satisfactory correction of the deformity was achieved by posterior spinal fusion with instrumentation from T4-L5, five Ponte osteotomies T8-L1, and an L2 pedicle subtraction osteotomy. The kyphosis was corrected from 87° to 55°; there was improvement in all spinopelvic parameters. One year after surgery, there was maintenance of the deformity correction with no deterioration of the sagittal balance, and the patient was free of pain and had no loss of neurologic function. CONCLUSIONS: Proteus syndrome can be associated with spinal stenosis and deformity. Although the syndrome can be progressive in nature, the symptomatic spinal pathology should be treated appropriately.
BACKGROUND CONTEXT: Proteus syndrome (PS) is an extremely rare congenital disorder causing asymmetric overgrowth of different tissues. The etiology remains unclear. Limb deformities are common and often necessitate amputations. Only a few cases associated with spinal deformities have been described. PURPOSE: The aim was to report a rare case of PS associated with spinal deformity and its surgical management. STUDY DESIGN: A case of young boy with PS causing vertebral hypertrophy and kyphoscoliotic deformity, which was surgically corrected, is presented. METHODS: The patient was assessed clinically and with whole spine plain radiographs, computed tomography, and magnetic resonance imaging. Surgical correction was performed. RESULTS: Satisfactory correction of the deformity was achieved by posterior spinal fusion with instrumentation from T4-L5, five Ponte osteotomies T8-L1, and an L2 pedicle subtraction osteotomy. The kyphosis was corrected from 87° to 55°; there was improvement in all spinopelvic parameters. One year after surgery, there was maintenance of the deformity correction with no deterioration of the sagittal balance, and the patient was free of pain and had no loss of neurologic function. CONCLUSIONS:Proteus syndrome can be associated with spinal stenosis and deformity. Although the syndrome can be progressive in nature, the symptomatic spinal pathology should be treated appropriately.