Effects on Lung Function of Multiple Expansion Thoracoplasty in Children With Thoracic Insufficiency Syndrome: A Longitudinal Study

Motoyama, Etsuro K.; Deeney, Vincent F.; Fine, Gavin F.; Yang, Charles I.; Mutich, Rebecca L.; Walczak, Stephen A.; Moreland, Morey S.

doi:10.1097/01.brs.0000197203.76653.d0

Cited by 94 publications

(74 citation statements)

References 10 publications

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“…These findings support the utility of clinical and radiographic evaluations when assessing curve severity [14]. When evaluating the type of construct selected by each surgeon, we found poor intraobserver and fair interobserver agreement.…”

Section: Discussionsupporting

confidence: 73%

Variability of Expert Opinion in Treatment of Early-onset Scoliosis

Vitale

Gómez

Matsumoto

et al. 2011

Clinical Orthopaedics &Amp; Related Research

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Background In contrast with treatment recommendations for adolescent idiopathic scoliosis, there are no clear algorithms for treating patients with early-onset scoliosis. There has been rapid expansion of treatment options for children with early-onset scoliosis, including casting, growth rods, the vertical expandable prosthetic titanium rib, and anterior vertebral stapling.Questions/purposes Given the range of treatment options, we assessed variability in decision making regarding treatment of patients with early-onset scoliosis. Methods We presented 12 clinical and radiographic vignettes about patients with early-onset scoliosis to 13 experienced spine surgeons who are members of the Chest Wall and Spine Deformity Study Group. The reviewers were asked to choose type of treatment, type of construct, construct location, and whether a thoracotomy should be performed. Results All 13 surgeons agreed regarding the need for surgery in eight of the 12 cases. When the reviewers chose surgery, 76% (40%-100%) selected the vertical expandable prosthetic titanium rib; of those selecting that approach, 61% (0%-100%) coincided on using it bilaterally. Agreement was 20% (0%-60%) for growing rods and 4% (0%-25%) for fusions. Among all cases, agreement regarding whether instrumentation should extend to the pelvis was 71% (50%-100%). In all but two cases, at least 85% of surgeons recommended against a thoracotomy. Conclusions Although most surgeons agreed about the indication for surgery, we found wide variability in choice of construct type, number of constructs, and level of instrumentation.

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Section: Discussionsupporting

confidence: 73%

Variability of Expert Opinion in Treatment of Early-onset Scoliosis

Vitale

Gómez

Matsumoto

et al. 2011

Clinical Orthopaedics &Amp; Related Research

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“…3, 4, 7, 18, 23, 29) progressed more than 10°until the last follow-up, 3 (Nos. 8,12,17) improved 10°or more (10°, 16°, 17°) with subsequent lengthenings and 14 patients remained within 0°-9°of the post-IP value. Between distractions, the curve deteriorated on an av of 9.7°(7°-13°) and improved with subsequent distractions: av 8.6°(6°-13°).…”

Section: Effect Of Expansion Procedures On Main Deformitymentioning

confidence: 82%

“…Ten patients (Nos. 2,8,9,12,16,17,18,20,22,23) showed a thoracic hyperkyphosis (68°-128°). Six patients were corrected and four stabilised.…”

Section: Sagittal Plane (?Kyphosis -Lordosis) Deformitymentioning

confidence: 98%

“…Routine interventions included 110 lengthenings and 39 changes to longer components (25), changes of type of constructs (8) and repositioning of upper cradles (8), laminar hooks (1) and pelvic hooks (5). Eighteen implantrelated events required seven unplanned interventions due to pain, skin slough or apparent loss of correction.…”

Section: Surgeriesmentioning

confidence: 99%

“…The concomitant control of complex spinal deformities by force transmission from the ribs to the spine and pelvis [2][3][4] stimulates surgeons to expand the indications beyond the primary scope to early onset deformities of idiopathic, neuromuscular and syndromic origin [5,6]. The growth-promoting lengthening strategy and the polyaxial anchor points may overcome post-fusion issues such as short and stiff trunk, small thorax, poor pulmonary function and crankshaft phenomenon [4,7,8]. However, VEPTR's safety and efficacy remains still to be shown for non-congenital deformities.…”

Section: Introductionmentioning

confidence: 99%

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Efficacy and safety of VEPTR instrumentation for progressive spine deformities in young children without rib fusions

2009

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This retrospective study analyses 23 children treated with vertical expandable prosthetic titanium rib (VEPTR) for correction of non-congenital early onset spine deformities. After the index procedure (IP), the device was lengthened at 6-month intervals. The average (av) age at the time of IP was 6.5 years (1.11-10.5). The av follow-up time was 3.6 years (2-5.8). Diagnosis included 1 early onset idiopathic scoliosis, 11 neuromuscular, 2 post-thoracotomy scoliosis, 1 Sprengel deformity, 2 hyperkyphosis, 1 myopathy and 5 syndromic. Surgeries (187) included 23 IPs, av 6.5 (4-10) device expansions per patient (149) and 15 unplanned surgeries. 23 complications (0.13 per surgery) included 10 skin sloughs, 5 implant dislocations, 2 rod breakages and 6 infections. Coronal Cobb angle was av 68°(11°-111°), at follow-up av 54°(0°-105°). Pelvic obliquity was av 33°( 13°-60°), at follow-up av 16°(0°-42°). T1 tilt was av 29°( 5°-84°), two remained unchanged, the remainder improved 10°-68°. Sagittal plane: All but two had stable profiles, two hyperkyphosis of 110°/124°improved to 56°/86°. Space available for lung ratio was less than 90% in ten before the IP, improved in nine and deteriorated in one. Originally designed for thoracic insufficiency syndromes related to rib and vertebral anomalies, VEPTR proved to be a valuable alternative to dual growing rods for non-congenital early onset spine deformities. The complication rate was lower, the control of the sagittal plane and the pelvic obliquity was as good, but the correction of the coronal plane deformity was less than growing rods. However, VEPTR's spine-sparing approach might provoke less spontaneous spinal fusion and ease the final correction at maturity.

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Respiratory motile cilia dysfunction in a patient with cranioectodermal dysplasia

Garrod

Madan‐Khetarpal

et al. 2015

American J of Med Genetics Pt A

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Ciliopathies such as cranioectodermal dysplasia, Sensenbrenner syndrome, short-rib polydactyly, and Jeune syndrome are associated with respiratory complications arising from rib cage dysplasia. While such ciliopathies have been demonstrated to involve primary cilia defects, we show motile cilia dysfunction in the airway of a patient diagnosed with cranioectodermal dysplasia. While this patient had mild thoracic dystrophy not requiring surgical treatment, there was nevertheless newborn respiratory distress, restrictive airway disease with possible obstructive airway involvement, repeated respiratory infections, and atelectasis. High-resolution videomicroscopy of nasal epithelial biopsy showed immotile/dyskinetic cilia and nasal nitric oxide was reduced, both of which are characteristics of primary ciliary dyskinesia, a sinopulmonary disease associated with mucociliary clearance defects due to motile cilia dysfunction in the airway. Exome sequencing analysis of this patient identified compound heterozygous mutations in WDR35, but no mutations in any of the 30 known primary ciliary dyskinesia genes or other cilia-related genes. Given that WDR35 is only known to be required for primary cilia function, we carried out WDR35 siRNA knockdown in human respiratory epithelia to assess the role of WDR35 in motile cilia function. This showed WDR35 deficiency disrupted ciliogenesis in the airway, indicating WDR35 is also required for formation of motile cilia. Together, these findings suggest patients with WDR35 mutations have an airway mucociliary clearance defect masked by their restrictive airway disease.

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Effects on Lung Function of Multiple Expansion Thoracoplasty in Children With Thoracic Insufficiency Syndrome: A Longitudinal Study

Cited by 94 publications

References 10 publications

Variability of Expert Opinion in Treatment of Early-onset Scoliosis

Variability of Expert Opinion in Treatment of Early-onset Scoliosis

Efficacy and safety of VEPTR instrumentation for progressive spine deformities in young children without rib fusions

Respiratory motile cilia dysfunction in a patient with cranioectodermal dysplasia

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