Orthopaedic diagnoses in patients with klippel-trenaunay syndrome

J. J. Schoch, H. Nguyen, B. S. Schoch, K. R. Anderson, A. A. Stans, D. Driscoll, M. Tollefson

Research output: Contribution to journalArticle

Abstract

Purpose Klippel-Trenaunay syndrome (KTS) is a rare combined vascular malformation composed of capillary malformation, lymphatic and/or venous malformation and limb overgrowth, which commonly affects the extremities. Due to limb involvement, it is not uncommon for these patients to require referral to an orthopaedic surgeon. Herein we reviewed the prevalence of orthopaedic diagnoses in a large cohort of KTS patients and described the associated surgical interventions. Methods Between 1976 and 2012, 410 patients fulfilling strict criteria for KTS were evaluated at a single institution. Patient charts were reviewed for demographic information, details of the clinical evaluation, orthopaedic consultation and surgical interventions. Results A total of 264 of 410 patients (64%) with confirmed KTS required orthopaedic evaluation. Of these 264 patients, 84% had documented limb-length discrepancy. Other common diagnoses included: angular deformities (10%), scoliosis (9%), osteopenia/osteoporosis (7%), pathological fractures (6%), joint contracture (5%), degenerative joint disease (4%) and limb/joint pain (4%). Of the 264 patients evaluated by orthopaedic surgery, 133 patients (50.4%) underwent 169 surgeries. Surgery was most commonly performed for limb-length discrepancy (62%). Multivariable analysis confirmed an orthopaedic condition was more likely in patients with lymphatic malformation (odds ratio (OR) 3.78; p < 0.001), as well as in those with bone and/or soft-tissue hypertrophy of the lower extremity (OR 7.51; p < 0.001) or foot (OR 3.23; p < 0.001). Conclusion Orthopaedic conditions are common in patients with KTS and approximately 50% require surgical intervention. Those with a lymphatic malformation and/or soft-tissue hypertrophy of the lower extremity are more likely to need surgery. Level of Evidence: Level IV, Descriptive.

Original languageEnglish (US)
Pages (from-to)457-462
Number of pages6
JournalJournal of Children's Orthopaedics
Volume13
Issue number5
DOIs
StatePublished - Oct 2019

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Klippel-Trenaunay-Weber Syndrome
Orthopedics
Extremities
Odds Ratio
Hypertrophy
Lower Extremity
Referral and Consultation
Spontaneous Fractures
Vascular Malformations
Metabolic Bone Diseases
Arthralgia
Scoliosis
Contracture
Osteoarthritis
Osteoporosis
Foot
Joints
Demography

Keywords

  • Klippel-trenaunay syndrome
  • Limb hypertrophy
  • Limb-length discrepancy
  • Overgrowth
  • Surgical intervention
  • Vascular malformation

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Orthopedics and Sports Medicine

Cite this

Schoch, J. J., Nguyen, H., Schoch, B. S., Anderson, K. R., Stans, A. A., Driscoll, D., & Tollefson, M. (2019). Orthopaedic diagnoses in patients with klippel-trenaunay syndrome. Journal of Children's Orthopaedics, 13(5), 457-462. https://doi.org/10.1302/1863-2548.13.190065

Orthopaedic diagnoses in patients with klippel-trenaunay syndrome. / Schoch, J. J.; Nguyen, H.; Schoch, B. S.; Anderson, K. R.; Stans, A. A.; Driscoll, D.; Tollefson, M.

In: Journal of Children's Orthopaedics, Vol. 13, No. 5, 10.2019, p. 457-462.

Research output: Contribution to journalArticle

Schoch, JJ, Nguyen, H, Schoch, BS, Anderson, KR, Stans, AA, Driscoll, D & Tollefson, M 2019, 'Orthopaedic diagnoses in patients with klippel-trenaunay syndrome', Journal of Children's Orthopaedics, vol. 13, no. 5, pp. 457-462. https://doi.org/10.1302/1863-2548.13.190065
Schoch JJ, Nguyen H, Schoch BS, Anderson KR, Stans AA, Driscoll D et al. Orthopaedic diagnoses in patients with klippel-trenaunay syndrome. Journal of Children's Orthopaedics. 2019 Oct;13(5):457-462. https://doi.org/10.1302/1863-2548.13.190065
Schoch, J. J. ; Nguyen, H. ; Schoch, B. S. ; Anderson, K. R. ; Stans, A. A. ; Driscoll, D. ; Tollefson, M. / Orthopaedic diagnoses in patients with klippel-trenaunay syndrome. In: Journal of Children's Orthopaedics. 2019 ; Vol. 13, No. 5. pp. 457-462.
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abstract = "Purpose Klippel-Trenaunay syndrome (KTS) is a rare combined vascular malformation composed of capillary malformation, lymphatic and/or venous malformation and limb overgrowth, which commonly affects the extremities. Due to limb involvement, it is not uncommon for these patients to require referral to an orthopaedic surgeon. Herein we reviewed the prevalence of orthopaedic diagnoses in a large cohort of KTS patients and described the associated surgical interventions. Methods Between 1976 and 2012, 410 patients fulfilling strict criteria for KTS were evaluated at a single institution. Patient charts were reviewed for demographic information, details of the clinical evaluation, orthopaedic consultation and surgical interventions. Results A total of 264 of 410 patients (64{\%}) with confirmed KTS required orthopaedic evaluation. Of these 264 patients, 84{\%} had documented limb-length discrepancy. Other common diagnoses included: angular deformities (10{\%}), scoliosis (9{\%}), osteopenia/osteoporosis (7{\%}), pathological fractures (6{\%}), joint contracture (5{\%}), degenerative joint disease (4{\%}) and limb/joint pain (4{\%}). Of the 264 patients evaluated by orthopaedic surgery, 133 patients (50.4{\%}) underwent 169 surgeries. Surgery was most commonly performed for limb-length discrepancy (62{\%}). Multivariable analysis confirmed an orthopaedic condition was more likely in patients with lymphatic malformation (odds ratio (OR) 3.78; p < 0.001), as well as in those with bone and/or soft-tissue hypertrophy of the lower extremity (OR 7.51; p < 0.001) or foot (OR 3.23; p < 0.001). Conclusion Orthopaedic conditions are common in patients with KTS and approximately 50{\%} require surgical intervention. Those with a lymphatic malformation and/or soft-tissue hypertrophy of the lower extremity are more likely to need surgery. Level of Evidence: Level IV, Descriptive.",
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AU - Schoch, J. J.

AU - Nguyen, H.

AU - Schoch, B. S.

AU - Anderson, K. R.

AU - Stans, A. A.

AU - Driscoll, D.

AU - Tollefson, M.

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N2 - Purpose Klippel-Trenaunay syndrome (KTS) is a rare combined vascular malformation composed of capillary malformation, lymphatic and/or venous malformation and limb overgrowth, which commonly affects the extremities. Due to limb involvement, it is not uncommon for these patients to require referral to an orthopaedic surgeon. Herein we reviewed the prevalence of orthopaedic diagnoses in a large cohort of KTS patients and described the associated surgical interventions. Methods Between 1976 and 2012, 410 patients fulfilling strict criteria for KTS were evaluated at a single institution. Patient charts were reviewed for demographic information, details of the clinical evaluation, orthopaedic consultation and surgical interventions. Results A total of 264 of 410 patients (64%) with confirmed KTS required orthopaedic evaluation. Of these 264 patients, 84% had documented limb-length discrepancy. Other common diagnoses included: angular deformities (10%), scoliosis (9%), osteopenia/osteoporosis (7%), pathological fractures (6%), joint contracture (5%), degenerative joint disease (4%) and limb/joint pain (4%). Of the 264 patients evaluated by orthopaedic surgery, 133 patients (50.4%) underwent 169 surgeries. Surgery was most commonly performed for limb-length discrepancy (62%). Multivariable analysis confirmed an orthopaedic condition was more likely in patients with lymphatic malformation (odds ratio (OR) 3.78; p < 0.001), as well as in those with bone and/or soft-tissue hypertrophy of the lower extremity (OR 7.51; p < 0.001) or foot (OR 3.23; p < 0.001). Conclusion Orthopaedic conditions are common in patients with KTS and approximately 50% require surgical intervention. Those with a lymphatic malformation and/or soft-tissue hypertrophy of the lower extremity are more likely to need surgery. Level of Evidence: Level IV, Descriptive.

AB - Purpose Klippel-Trenaunay syndrome (KTS) is a rare combined vascular malformation composed of capillary malformation, lymphatic and/or venous malformation and limb overgrowth, which commonly affects the extremities. Due to limb involvement, it is not uncommon for these patients to require referral to an orthopaedic surgeon. Herein we reviewed the prevalence of orthopaedic diagnoses in a large cohort of KTS patients and described the associated surgical interventions. Methods Between 1976 and 2012, 410 patients fulfilling strict criteria for KTS were evaluated at a single institution. Patient charts were reviewed for demographic information, details of the clinical evaluation, orthopaedic consultation and surgical interventions. Results A total of 264 of 410 patients (64%) with confirmed KTS required orthopaedic evaluation. Of these 264 patients, 84% had documented limb-length discrepancy. Other common diagnoses included: angular deformities (10%), scoliosis (9%), osteopenia/osteoporosis (7%), pathological fractures (6%), joint contracture (5%), degenerative joint disease (4%) and limb/joint pain (4%). Of the 264 patients evaluated by orthopaedic surgery, 133 patients (50.4%) underwent 169 surgeries. Surgery was most commonly performed for limb-length discrepancy (62%). Multivariable analysis confirmed an orthopaedic condition was more likely in patients with lymphatic malformation (odds ratio (OR) 3.78; p < 0.001), as well as in those with bone and/or soft-tissue hypertrophy of the lower extremity (OR 7.51; p < 0.001) or foot (OR 3.23; p < 0.001). Conclusion Orthopaedic conditions are common in patients with KTS and approximately 50% require surgical intervention. Those with a lymphatic malformation and/or soft-tissue hypertrophy of the lower extremity are more likely to need surgery. Level of Evidence: Level IV, Descriptive.

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KW - Overgrowth

KW - Surgical intervention

KW - Vascular malformation

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