Management of thoracic insufficiency syndrome in patients with Jarcho-Levin syndrome using VEPTRs (vertical expandable prosthetic titanium ribs).
BACKGROUND
Jarcho-Levin syndrome represents a spectrum of clinical and radiographic irregularities including abnormal vertebral segmentation or formation defects, rib deformities, and short-trunk dwarfism. These abnormalities cause reduced thoracic capacity for lung development, resulting in thoracic insufficiency syndrome. In the present study, we reviewed outcome measures related to scoliotic curve correction, thoracic growth, and respiratory function following VEPTR treatment in patients with Jarcho-Levin syndrome.


METHODS
Twenty-nine patients with Jarcho-Levin syndrome, subclassified as spondylocostal dysostosis (SCD) or spondylothoracic dysplasia (STD), were treated with VEPTR expansion thoracoplasty and followed for at least two years since the initial implantation. Spinal and respiratory measures were collected prior to the initial VEPTR implantation, immediately after implantation, and at the most recent follow-up.


RESULTS
VEPTR treatment was associated with improved clinical respiratory function and with increases in thoracic height (by 50% in the SCD group and 42% in the STD group) and thoracic width (by 37% in the SCD group and 28% in the STD group). VEPTR treatment resulted in scoliosis curve correction (improvement in the Cobb angle of 41% [22°] in the SCD group and 26% [3.7°] in the STD group) and in improved thoracic symmetry in patients with SCD. Patients with SCD displayed increased lumbar lordosis, and both groups of patients developed increased thoracic kyphosis approaching normal.


CONCLUSIONS
VEPTR treatment improved thoracic symmetry, controlled spinal deformity, and was associated with improved clinical respiratory function.
Department of Orthopaedics (V.P. and D.C.), College of Medicine (J.G.K.), University of Texas Health Science Center at San Antonio (UTHSCSA), 7703 Floyd Curl Drive, San Antonio, TX 78229.
Joshua G
JG
Karlin
Spinal & Thoracic Treatment and Research (STTAR) Center, Children's Hospital of San Antonio, 333 North Santa Rosa Street, San Antonio, TX 78207. E-mail address for M.K. Roth: megan.roth@christushealth.org.
Megan K
MK
Roth
Department of Orthopaedics (V.P. and D.C.), College of Medicine (J.G.K.), University of Texas Health Science Center at San Antonio (UTHSCSA), 7703 Floyd Curl Drive, San Antonio, TX 78229.
Vishwas
V
Patil
Department of Orthopaedics (V.P. and D.C.), College of Medicine (J.G.K.), University of Texas Health Science Center at San Antonio (UTHSCSA), 7703 Floyd Curl Drive, San Antonio, TX 78229.
Davin
D
Cordell
Spinal & Thoracic Treatment and Research (STTAR) Center, Children's Hospital of San Antonio, 333 North Santa Rosa Street, San Antonio, TX 78207. E-mail address for M.K. Roth: megan.roth@christushealth.org.
Hope
H
Trevino
Spinal & Thoracic Treatment and Research (STTAR) Center, Children's Hospital of San Antonio, 333 North Santa Rosa Street, San Antonio, TX 78207. E-mail address for M.K. Roth: megan.roth@christushealth.org.
James
J
Simmons
Children's Hospital of Philadelphia, 324 South 34th Street #10, Philadelphia, PA 19104.
Robert M
RM
Campbell
Spinal & Thoracic Treatment and Research (STTAR) Center, Children's Hospital of San Antonio, 333 North Santa Rosa Street, San Antonio, TX 78207. E-mail address for M.K. Roth: megan.roth@christushealth.org.
Ajeya P
AP
Joshi
D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging / Q000601:surgery; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery; D006548:Hernia, Diaphragmatic / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery; D006801:Humans; D008297:Male; D019736:Prostheses and Implants; D011474:Prosthesis Design; D011859:Radiography; D012131:Respiratory Insufficiency / Q000209:etiology / Q000601:surgery*; D012189:Retrospective Studies; D012272:Ribs / Q000002:abnormalities*; D012600:Scoliosis / Q000209:etiology / Q000601:surgery*; D013577:Syndrome; D013905:Thoracoplasty / Q000379:methods*; D014025:Titanium; D016896:Treatment Outcome
D014025:Titanium