title: Management of Scoliosis in Patients With Loeys-Dietz Syndrome.
abstract: BACKGROUND
  Loeys-Dietz syndrome (LDS) is a genetic connective tissue disorder. We sought to determine the incidence of scoliosis in patients with LDS, characterize the spectrum of spinal deformity, determine the results of bracing and surgery, and define surgical complications.
  
  
  METHODS
  Patients were selected from our institution's database of 183 patients with LDS. Imaging measurements were performed for 141 patients whose records permitted spinal evaluation. Deformity changes and complications after intervention were recorded for patients who underwent bracing or surgery, and associations were tested using Student t tests (significance, P<0.05).
  
  
  RESULTS
  Eighty-eight of 141 (62%) patients with LDS had scoliosis, with main thoracic and thoracolumbar curves being most common. Fifteen patients were braced (mean age, 9±3 y) for a mean of 2.3 years. They had a mean postbracing curve progression of 12±21 degrees (5±9 deg./y). There were no significant differences in age, sex, curve type, or prebracing curve magnitude between successfully braced (n=4) and unsuccessfully braced (n=11) patients (P>0.05). Nine patients, (mean age, 12±3 y), underwent 24 surgical procedures (16 growing rod procedures, 8 fusions). Mean curve corrections were 61% for growing rods and 73% for fusions. Associated blood loss for these procedures was 400 mL and 1293 mL, respectively, and normalized blood loss for fusion was 2.34 mL/kg/level. Fifteen of 24 surgical procedures involved complications (63%), including cerebrospinal fluid leaks (n=7) and blood loss >20% of estimated total blood volume (n=11).
  
  
  CONCLUSIONS
  Scoliosis was present in 62% of our sample of LDS patients. Bracing did not halt curves in 11 of 15 patients, whose curves progressed >5 degrees or to >50 degrees by completion of bracing. At latest follow-up, 47% of the braced patients had undergone surgery after prior bracing attempts. The high blood loss associated with these operations is believed to be related to vascular fragility in patients with LDS.
  
  
  METHODS
  Level IV-retrospective cohort study.
authors.affiliation: *The Johns Hopkins University School of Medicine †The Institute of Genetic Medicine Departments of ‡Pediatrics §Pediatric Orthopaedic Surgery, The Johns Hopkins University, Baltimore, MD.
authors.forename: Jarred A
authors.initials: JA
authors.lastname: Bressner
authors.forename: Gretchen L
authors.initials: GL
authors.lastname: MacCarrick
authors.forename: Harry C
authors.initials: HC
authors.lastname: Dietz
authors.forename: Paul D
authors.initials: PD
authors.lastname: Sponseller
mesh_terms: D000293:Adolescent; D000328:Adult; D001915:Braces; D002648:Child; D018450:Disease Progression; D005260:Female; D006801:Humans; D015994:Incidence; D007738:Kyphosis / Q000178:diet therapy / Q000601:surgery*; D055947:Loeys-Dietz Syndrome / Q000150:complications*; D008297:Male; D012189:Retrospective Studies; D012600:Scoliosis / Q000453:epidemiology / Q000209:etiology* / Q000628:therapy*; D013123:Spinal Fusion; D016896:Treatment Outcome