TY - JOUR
T1 - Management of thoracic insufficiency syndrome in patients with jeune syndrome using the 70mm radius vertical expandable prosthetic titanium rib
AU - O'Brien, Aaron
AU - Roth, Megan K.
AU - Athreya, Hariharan
AU - Reinker, Kent
AU - Koeck, William
AU - Patil, Vishwas
AU - Trevino, Hope
AU - Simmons, James
AU - Joshi, Ajeya P.
AU - Smith, Melvin D.
AU - Campbe, Robert M.
N1 - Publisher Copyright:
Copyright ©2015 Wolters Kluwer Health, Inc. All rights reserved.
Copyright:
Copyright 2018 Elsevier B.V., All rights reserved.
PY - 2015/12
Y1 - 2015/12
N2 - 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>0.001). Preoperatively, 7/17 (41%) patients had scoliosis. The remainder developed scoliosis during treatment, 8 requiring additional implants. Thoracic and lumbar spinal height was normal preoperatively and stayed normal during treatment. Thoracic kyphosis/lumbar lordosis was stable. Average computed tomography scan total lung volumes increased 484 to 740mm3 (P>0.001), and Assisted Ventilation Rate status tended to improve (P=0.07). Average forced vital capacity was 34% predicted at first test and 27% predicted at last follow-up. Early demise after surgery was common with multisystem disease. Mean respiratory rate decreased from 35 to 24 bpm at last follow-up (P>0.05). Survival rate of the 22 patients was 68%. Migration of the rib cradles/titanium slings occurred in 12 patients, superficial infections in 5 patients, deep infections in 4 patients, and wound dehiscence in 5 patients. Infection rate was 4.6% per procedure. Conclusions: The survival rate in JS with surgery was nearly 70% (compared with 70% to 80% mortality without treatment) with less ventilator dependence. Both C1 stenosis and scoliosis are common in JS. Spinal height in JS is normal. Complications are frequent, but tolerable in view of the clinical gains and increase in survival. Level of Evidence: Level IV-retrospective therapeutic case series.
AB - 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>0.001). Preoperatively, 7/17 (41%) patients had scoliosis. The remainder developed scoliosis during treatment, 8 requiring additional implants. Thoracic and lumbar spinal height was normal preoperatively and stayed normal during treatment. Thoracic kyphosis/lumbar lordosis was stable. Average computed tomography scan total lung volumes increased 484 to 740mm3 (P>0.001), and Assisted Ventilation Rate status tended to improve (P=0.07). Average forced vital capacity was 34% predicted at first test and 27% predicted at last follow-up. Early demise after surgery was common with multisystem disease. Mean respiratory rate decreased from 35 to 24 bpm at last follow-up (P>0.05). Survival rate of the 22 patients was 68%. Migration of the rib cradles/titanium slings occurred in 12 patients, superficial infections in 5 patients, deep infections in 4 patients, and wound dehiscence in 5 patients. Infection rate was 4.6% per procedure. Conclusions: The survival rate in JS with surgery was nearly 70% (compared with 70% to 80% mortality without treatment) with less ventilator dependence. Both C1 stenosis and scoliosis are common in JS. Spinal height in JS is normal. Complications are frequent, but tolerable in view of the clinical gains and increase in survival. Level of Evidence: Level IV-retrospective therapeutic case series.
KW - Jeune syndrome
KW - TIS
KW - Thoracic insufficiency syndrome
KW - VEPTR
KW - Vertical expandable prosthetic titanium rib
UR - http://www.scopus.com/inward/record.url?scp=84922237756&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84922237756&partnerID=8YFLogxK
U2 - 10.1097/BPO.0000000000000383
DO - 10.1097/BPO.0000000000000383
M3 - Article
C2 - 25575358
AN - SCOPUS:84922237756
VL - 35
SP - 783
EP - 797
JO - Journal of Pediatric Orthopaedics
JF - Journal of Pediatric Orthopaedics
SN - 0271-6798
IS - 8
ER -