title: Lymphangioleiomyomatosis screening in women with tuberous sclerosis.
abstract: BACKGROUND
  Lymphangioleiomyomatosis (LAM) occurs in at least 40% of women with tuberous sclerosis complex (TSC), as diagnosed based on chest CT scan findings. Early identification may inform lifestyle choices and treatment decisions. Here we report LAM prevalence in a large TSC clinic and propose an approach to CT scan screening for LAM in women with TSC.
  
  
  METHODS
  We retrospectively reviewed initial chest CT scans of all female patients with TSC aged ≥ 15 years seen at our center over a 12-year period. Each CT image slice was manually scored for the presence or absence of characteristic thin-walled cysts, and the diagnosis of LAM was made if the sum of the cysts on all slices exceeded three cysts.
  
  
  RESULTS
  Of 133 female patients with TSC, 101 had chest CT scans available for review. Forty-eight (47.5%) met criteria for TSC-LAM on the initial CT scan. The risk of LAM was age dependent, rising by about 8% per year. The prevalence of LAM was 27% in subjects &lt; 21 years of age and 81% in subjects &gt; 40 years of age. Among asymptomatic subjects with LAM, 84% had cysts present in the single image at the level of the carina. Most subjects with LAM eventually developed pulmonary symptoms (63%), and 12.5% died from LAM.
  
  
  CONCLUSIONS
  These results suggest that most women with TSC ultimately develop cystic changes consistent with LAM and that most cases can be identified from a single CT imaging slice at the level of the carina. TSC-LAM was associated with appreciable morbidity and mortality in this referral population. An age-based approach using limited CT scanning methods may facilitate screening and subsequent treatment decisions with decreased radiation exposure in this at-risk population.
authors.affiliation: Division of Pulmonary, Critical Care, and Sleep Medicine, University of Cincinnati Medical Center, Cincinnati, OH.
authors.forename: Corey J
authors.initials: CJ
authors.lastname: Cudzilo
authors.affiliation: Division of Biostatistics and Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH; Division of Pulmonary Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
authors.forename: Rhonda D
authors.initials: RD
authors.lastname: Szczesniak
authors.affiliation: Department of Radiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
authors.forename: Alan S
authors.initials: AS
authors.lastname: Brody
authors.affiliation: Department of Radiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
authors.forename: Mantosh S
authors.initials: MS
authors.lastname: Rattan
authors.affiliation: Division of Neurology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
authors.forename: Darcy A
authors.initials: DA
authors.lastname: Krueger
authors.affiliation: Division of Nephrology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
authors.forename: John J
authors.initials: JJ
authors.lastname: Bissler
authors.affiliation: Division of Neurology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
authors.forename: David N
authors.initials: DN
authors.lastname: Franz
authors.affiliation: Division of Pulmonary, Critical Care, and Sleep Medicine, University of Cincinnati Medical Center, Cincinnati, OH.
authors.forename: Francis X
authors.initials: FX
authors.lastname: McCormack
authors.affiliation: Division of Pediatric Pulmonary Medicine, Vanderbilt University School of Medicine, Nashville, TN; Division of Allergy, Pulmonary, and Critical Care Medicine, Vanderbilt University School of Medicine, Nashville, TN. Electronic address: Lisa.Young@Vanderbilt.edu.
authors.forename: Lisa R
authors.initials: LR
authors.lastname: Young
mesh_terms: D000293:Adolescent; D000328:Adult; D005260:Female; D006801:Humans; D018192:Lymphangioleiomyomatosis / Q000000981:diagnostic imaging* / Q000453:epidemiology / Q000209:etiology*; D008875:Middle Aged; D009820:Ohio / Q000453:epidemiology; D015995:Prevalence; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D014402:Tuberous Sclerosis / Q000150:complications* / Q000000981:diagnostic imaging* / Q000453:epidemiology