Management of Thoracic Insufficiency Syndrome in Patients With Jeune Syndrome Using the 70 mm Radius Vertical Expandable Prosthetic Titanium Rib

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

doi:10.1097/bpo.0000000000000383

Cited by 28 publications

(19 citation statements)

References 37 publications

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“…They reported the usefulness of VEPTR to improve scoliosis, however, they also reported the difficulty to increase lung function and volume. O’Brien et al [ 7 ] revealed that VEPTR improved survival rate with less ventilator dependence in Jeune syndrome. Further long-term results for VEPTER is needed in the future.…”

Section: Discussionmentioning

confidence: 99%

“…However, in our two cases, cervical magnetic resonance imaging, which was performed before surgery, did not show any abnormality. Recently, O’Brien et al [ 7 ] studied 24 Jeune syndrome patients and reported that scoliosis was present in 41 %, several patients required surgery. However, to our knowledge, there was hitherto no information about causes or characteristics of scoliosis in Jeune syndrome who survived their early stage.…”

Section: Discussionmentioning

confidence: 99%

“…There are a few reports or comments in the literature regarding the association between Jeune syndrome and spinal disorders [ 3 , 5 – 7 ]. However, to our knowledge, there are no detailed reports of the treatment of scoliosis in this syndrome.…”

Section: Introductionmentioning

confidence: 99%

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Spinal correction of scoliosis in Jeune syndrome: a report of two cases

et al. 2016

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BackgroundJeune syndrome (asphyxiating thoracic dystrophy) is an autosomal recessive disorder with constriction and narrowing of the thorax. To our knowledge, there are no reports regarding spinal deformity and correction in Jeune syndrome. Herein, we report two cases of spinal correction in patients with Jeune syndrome, and review the literature.Case PresentationWe experienced cases of spinal scoliosis in an adolescent boy and a young adult woman, both with Jeune syndrome. Their spinal deformities had progressed by the time they came to our hospital for surgical correction. After preoperative evaluation of their general condition, including respiratory function in detail to confirm that they could undergo surgery, we treated both cases with posterior spinal correction and fusion. Spinal correction was performed safely and there were no severe complications, including respiratory depression, associated with surgery, and relatively satisfactory correction was obtained in both cases. In case 1, coronal deformity was corrected from 70° to 36° and from 82° to 42°, respectively. In case 2, Cobb angle was corrected from 52° to 20° and from 55° to 21°.ConclusionsPosterior spinal correction can be performed safely in young patients with Jeune syndrome who have survived their infant stage and matured without a severe general condition.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Spinal correction of scoliosis in Jeune syndrome: a report of two cases

et al. 2016

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“…Tres meses más tarde se expande el hemitórax izquierdo de la misma manera. Ambos VEPTR se tienen que expandir quirúrgicamente dos veces al año 12,17 . O'Brein y cols reportaron un aumento de sobrevida de los pacientes de un 70% y una menor dependencia de ventilador con este tratamiento en comparación con los pacientes sin tratamiento 17 .…”

Section: Tratamientounclassified

Deformidad de la caja torácica

Ramirez-Lluch

Acevedo-Echevarría

2018

Rev Chil Cir

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Chest wall deformities are divided as an abnormal development during the growth or those secondary to a congenital malformation. The developmental type is the most common: pectus excavatum or pectus carinatum. The less common are the congenital types of chest wall abnormalities: Poland's syndrome, Jeune's syndrome, espondylothoracic dysplasia, espondylocostal dysplasia and defects of the ribs or sternum. The congenital type usually affects the relationship between the spine, rib cage and the lungs. Therefore, many of these patients will develop a progressive respiratory disturbance of restrictive type known as Thoracic Insufficiency Syndrome. Thoracic insufficiency syndrome is defining as a deficiency of the rib cage to maintain a normal respiration and to sustain the physiological growth of the lungs. In this article will discuss several conditions that will affect the development and function of the chest wall.

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“…The favorable control of complex spinal deformities stimulated surgeons to expand the VEPTR's indications to EOS of idiopathic, neuromuscular, and syndromic etiologies. 2,10,11,15,22 However, it is known that the major difference between VEPTR and GRI is that VEPTR selects ribs as proximal anchor sites, while GRI has anchors only at the spine either proximally or distally. Thus, although VEPTR and GRI encourage spinal growth via regular lengthening, they can create different results because of different fixation characteristics and mechanisms in correcting scoliosis.…”

mentioning

confidence: 99%

Evolution of the postoperative sagittal spinal profile in early-onset scoliosis: is there a difference between rib-based and spine-based growth-friendly instrumentation?

Chen¹,

Song²,

Qiu³

et al. 2017

Journal of Neurosurgery: Pediatrics

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OBJECTIVEAlthough the vertical expandable prosthetic titanium rib (VEPTR) and growing rod instrumentation (GRI) encourage spinal growth via regular lengthening, they can create different results because of their different fixation patterns and mechanisms in correcting scoliosis. Previous studies have focused comparisons on coronal plane deformity with minimal attention to the sagittal profile. In this retrospective study, the authors aimed to compare the evolution of the sagittal spinal profile in early-onset scoliosis (EOS) treated with VEPTR versus GRI.METHODSThe data for 11 patients with VEPTR and 22 with GRI were reviewed. All patients had more than 2 years’ follow-up with more than 2 lengthening procedures. Radiographic measurements were performed before and after the index surgery and at the latest follow-up. The complications in both groups were recorded.RESULTSPatients in both groups had similar diagnoses, age at the index surgery, and number of lengthening procedures. The changes in the major coronal Cobb angle and T1–S1 spinal height were not significantly different between the 2 groups. Compared with the GRI group, the VEPTR group had less correction in thoracic kyphosis (23% ± 12% vs 44% ± 16%, p < 0.001) after the index surgery and experienced a greater correction loss in thoracic kyphosis (46% ± 18% vs 11% ± 8%, p < 0.001) at the latest follow-up. Although the increase in the proximal junctional angle was not significantly different (VEPTR: 7° ± 4° vs GRI: 8° ± 5°, p = 0.569), the incidence of proximal junctional kyphosis was relatively lower in the VEPTR group (VEPTR: 18.2% vs GRI: 22.7%). No significant changes in the spinopelvic parameters were observed, while the sagittal vertical axis showed a tendency toward a neutral position in both groups. The overall complication rate was higher in the VEPTR group than in the GRI group (72.7% vs 54.5%).CONCLUSIONSThe VEPTR had coronal correction and spinal growth results similar to those with GRI. In the sagittal plane, however, the VEPTR was not comparable to the GRI in controlling thoracic kyphosis. Thus, for hyperkyphotic EOS patients, GRI is recommended over VEPTR.

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Management of Thoracic Insufficiency Syndrome in Patients With Jeune Syndrome Using the 70 mm Radius Vertical Expandable Prosthetic Titanium Rib

Cited by 28 publications

References 37 publications

Spinal correction of scoliosis in Jeune syndrome: a report of two cases

Spinal correction of scoliosis in Jeune syndrome: a report of two cases

Deformidad de la caja torácica

Evolution of the postoperative sagittal spinal profile in early-onset scoliosis: is there a difference between rib-based and spine-based growth-friendly instrumentation?

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