title: Surgical correction of kyphotic deformity in a patient with Proteus syndrome.
abstract: BACKGROUND
  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.
  
  
  OBJECTIVE
  The aim was to report a rare case of PS associated with spinal deformity and its surgical management.
  
  
  METHODS
  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.
authors: 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.
authors: Radek
authors: R
authors: Kaiser
authors: The Centre for Spinal Studies and Surgery, Queen's Medical Centre, Nottingham University Hospitals NHS Trust, Derby Rd, Nottingham, UK.
authors: Esin
authors: E
authors: Rothenfluh
authors: The Centre for Spinal Studies and Surgery, Queen's Medical Centre, Nottingham University Hospitals NHS Trust, Derby Rd, Nottingham, UK.
authors: Dominique
authors: D
authors: Rothenfluh
authors: The Centre for Spinal Studies and Surgery, Queen's Medical Centre, Nottingham University Hospitals NHS Trust, Derby Rd, Nottingham, UK.
authors: Eyal
authors: E
authors: Behrbalk
authors: The Centre for Spinal Studies and Surgery, Queen's Medical Centre, Nottingham University Hospitals NHS Trust, Derby Rd, Nottingham, UK.
authors: Ana Belen
authors: AB
authors: Perez Romera
authors: The Centre for Spinal Studies and Surgery, Queen's Medical Centre, Nottingham University Hospitals NHS Trust, Derby Rd, Nottingham, UK.
authors: Oliver M
authors: OM
authors: Stokes
authors: The Centre for Spinal Studies and Surgery, Queen's Medical Centre, Nottingham University Hospitals NHS Trust, Derby Rd, Nottingham, UK.
authors: Hossein
authors: H
authors: Mehdian
mesh_terms: D000293:Adolescent; D002648:Child; D006801:Humans; D007738:Kyphosis / Q000000981:diagnostic imaging / Q000601:surgery*; D008297:Male; D010027:Osteotomy / Q000379:methods*; D016715:Proteus Syndrome / Q000000981:diagnostic imaging / Q000601:surgery*; D011859:Radiography; D013123:Spinal Fusion / Q000379:methods*; D016896:Treatment Outcome
keywords: Kyphosis; Overgrowth; Proteus syndrome; Spinal deformity; Spinal instrumentation; Spinal osteotomy