Management of thoracic insufficiency syndrome in patients with jeune syndrome using the 70mm radius vertical expandable prosthetic titanium rib

Aaron O'Brien, Megan K. Roth, Hariharan Athreya, Kent Reinker, William Koeck, Vishwas Patil, Hope Trevino, James Simmons, Ajeya P. Joshi, Melvin D. Smith, Robert M. Campbell

Research output: Contribution to journalArticle

Abstract

Background: Jeune syndrome (JS) often results in lethal thoracic insufficiency syndrome. Since 1991, vertical expandable prosthetic titanium rib Dynamic PosteroLateral Expansion Thoracoplasty was used at our institution for treatment of JS. This study assesses the safety and efficacy of this procedure. Methods: Twenty-four JS patients were treated, 2 lost to followup, 17 with a minimum of 2-year follow-up retrospectively reviewed for clinical course: Assisted Ventilation Rate, respiratory rate, capillary blood gases, pulmonary function testings, and complications. Upright anteroposterior/lateral radiographs were measured for Cobb angle, kyphosis, lordosis, thoracic width, and thoracic/lumbar spinal height. Computed tomography scan lung volumes were obtained in 12 patients. Results: Mean age at initial implant was 23 months (7 to 62 mo) with an average 8.4 years (2.3 to 15.6 y) of follow-up. Average chest width increased from 121 to 168mm at follow-up (P

Original languageEnglish (US)
Pages (from-to)783-797
Number of pages15
JournalJournal of Pediatric Orthopaedics
Volume35
Issue number8
StatePublished - 2015
Externally publishedYes

Fingerprint

Ribs
Titanium
Thorax
Thoracoplasty
Lordosis
Lung
Kyphosis
Respiratory Rate
Ventilation
Gases
Tomography
Safety
Jeune syndrome
Therapeutics

Keywords

  • Jeune syndrome
  • Thoracic insufficiency syndrome
  • TIS
  • VEPTR
  • Vertical expandable prosthetic titanium rib

ASJC Scopus subject areas

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

Cite this

O'Brien, A., Roth, M. K., Athreya, H., Reinker, K., Koeck, W., Patil, V., ... Campbell, R. M. (2015). Management of thoracic insufficiency syndrome in patients with jeune syndrome using the 70mm radius vertical expandable prosthetic titanium rib. Journal of Pediatric Orthopaedics, 35(8), 783-797.

Management of thoracic insufficiency syndrome in patients with jeune syndrome using the 70mm radius vertical expandable prosthetic titanium rib. / O'Brien, Aaron; Roth, Megan K.; Athreya, Hariharan; Reinker, Kent; Koeck, William; Patil, Vishwas; Trevino, Hope; Simmons, James; Joshi, Ajeya P.; Smith, Melvin D.; Campbell, Robert M.

In: Journal of Pediatric Orthopaedics, Vol. 35, No. 8, 2015, p. 783-797.

Research output: Contribution to journalArticle

O'Brien, A, Roth, MK, Athreya, H, Reinker, K, Koeck, W, Patil, V, Trevino, H, Simmons, J, Joshi, AP, Smith, MD & Campbell, RM 2015, 'Management of thoracic insufficiency syndrome in patients with jeune syndrome using the 70mm radius vertical expandable prosthetic titanium rib', Journal of Pediatric Orthopaedics, vol. 35, no. 8, pp. 783-797.
O'Brien, Aaron ; Roth, Megan K. ; Athreya, Hariharan ; Reinker, Kent ; Koeck, William ; Patil, Vishwas ; Trevino, Hope ; Simmons, James ; Joshi, Ajeya P. ; Smith, Melvin D. ; Campbell, Robert M. / Management of thoracic insufficiency syndrome in patients with jeune syndrome using the 70mm radius vertical expandable prosthetic titanium rib. In: Journal of Pediatric Orthopaedics. 2015 ; Vol. 35, No. 8. pp. 783-797.
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AU - Patil, Vishwas

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