title: Lower limbs deformities in patients with McCune-Albright syndrome: Tomography and treatment.
abstract: BACKGROUND
  The skeletal changes in McCune-Albright disease are usually severe because of the polyostotic form of the disease. Trendelenberg gait and limited mobility are the most common presenting features. The constellation of Café-au lait spots and polyostotic bone involvement is commonly referred to as McCune-Albright's syndrome (MAS).
  
  
  METHODS
  One boy and 4 girls (7-16 years) were sought in our departments from 1998 to 2012. Limb length discrepancy was the main clinical presentation. Repetitive micro-fractures caused the development of 'Shepherd crook' deformity with pain were the main burden.
  
  
  RESULTS
  Because of the repetitive micro-fractures and the significant deformity that distorted the integrity of the long bones which were associated with pain. We referred to re-alignment valgus osteotomy with internal fixation to preserve proper alignment. Moreover, guided growth technique with 8-plates was performed in 1 case.
  
  
  CONCLUSIONS
  Tendency to progressive unilateral lower limb deformity in patients with MAS is usually associated with thinning and expansion of the cortex and distortion of the normal lower limb integrity secondary to repetitive micro-fractures. The latter is a situation which warrants surgical treatment to re-align the deformity and to preserve function. Prophylactic intramedullary nailing via the application of locking nails to ensure stabilisation of the femoral neck was found to be effective. However, nevertheless, the mosaic nature of MAS means any cell, tissue and organ in any site of the body could be affected to varying degrees. The clinical manifestations are a diversity of the disorder ranging from mild clinical signs to severe life-threatening disease.
authors: Department of First Medical, Ludwig Boltzmann Institute of Osteology, Hanusch Hospital of WGKK and AUVA Trauma Centre Meidling, Hanusch Hospital; Paediatric Department, Orthopaedic Hospital of Speising, Vienna, Austria.
authors: Ali
authors: A
authors: Al Kaissi
authors: Department of Foot and Ankle Surgery, Neuroorthopaedics and Systemic Disorders, Pediatric Orthopedic Institute n.a. H. Turner, 64-68, Pushkin, Saint-Petersburg, Russia.
authors: Vladimir
authors: V
authors: Kenis
authors: Radiology and Imaging Studies, Ibn Zohr Institute of Radiology and Imaging, Tunis, Tunisia.
authors: Farid Ben
authors: FB
authors: Chehida
authors: Department of First Medical, Ludwig Boltzmann Institute of Osteology, Hanusch Hospital of WGKK and AUVA Trauma Centre Meidling, Hanusch Hospital, Vienna, Austria.
authors: Jochen
authors: J
authors: Hofstaetter
authors: Paediatric Department, Orthopaedic Hospital of Speising, Vienna, Austria.
authors: Franz
authors: F
authors: Grill
authors: Paediatric Department, Orthopaedic Hospital of Speising, Vienna, Austria.
authors: Rudolf
authors: R
authors: Ganger
mesh_terms: D000293:Adolescent; D002648:Child; D005260:Female; D005359:Fibrous Dysplasia, Polyostotic / Q000000981:diagnostic imaging* / Q000473:pathology / Q000601:surgery*; D005593:Fracture Fixation, Internal; D006801:Humans; D035002:Lower Extremity / Q000000981:diagnostic imaging* / Q000473:pathology*; D008297:Male; D010027:Osteotomy; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed