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Thoracic Insufficiency Syndrome QuickTime™ and a decompressor are needed to see this picture. Laura Stoll November 2010 Normal thorax • Spine + rib cage + sternum + diaphragm • Lung development depends on thoracic growth – Increase in T-spine height – Symmetrical enlargement of rib cage through rib growth – Correct orientation of ribs Thoracic spine growth • Birth-5 years: 1.4 cm/yr • 6-10 years: 0.6 cm/yr • 11-15 years: 1.2 cm/yr • Marked loss of T-spine height = severe reduction in thoracic volume for lungs • Jarcho-Levin syndrome (spondylothoracic dysplasia) – Single, short, block vertebra (1/4th normal height) – High mortality due to respiratory complications Campbell RM, et al 2007; Ramirez N, et al Rib contribution • Birth - 2 years: – Ribs horizontal = growth of rib in length – Increase in diameter of rib cage = square-shaped thoracic cross-section • 2 years-10 years: – Rib course downward = oval-shaped thoracic cross-section • 10 years - skeletal maturity: – Ribs grow rapidly = final rectangular shaped thoracic cross-section • Rib cage contributes to volume increase by expanding anteriolaterally “buckethandle” biomechanism • Diaphragm “piston” increases thoracic volume by downward excursion Thoracic insufficiency syndrome • Inability of the thorax to support normal respiration or lung growth • Thoracic insufficiency syndrome does not equal respiratory insufficiency • Dx based on H&P, imaging studies, V-P lung scans, PFTs, labs Campbell RM, et al 2003 Disorders of normal, stable thorax volume • • • • • Causes extrinsic restrictive lung disease Scoliosis with absent or fused ribs Scoliosis with windswept deformity Jarcho Levine syndrome Jeune syndrome QuickTime™ and a decompressor are needed to see this picture. QuickTime™ and a decompressor are needed to see this picture. Disorders of thorax’s ability to change volume • CDH - decreased contraction of diaphragm • Unilateral hemidiaphragm paralysis • Anomalous insertion of the diaphragm in congenital scoliosis and rib anomalies • Secondary thoracic insufficiency syndrome - torso collapsed on pelvis Campbell RM, et al 2004 QuickTime™ and a decompressor are needed to see this picture. Campbell RM, et al 2007 • Standard scoliosis: – Thorax usually spacious, no rib cage deformities, near normal vital capacity – Characterized by degree of curve on AP radiographs – Tx: bracing or definitive spinal fusion Exotic scoliosis • Early onset • Thorax distorted by spinal lordosis and curve rotation • Often associated with rib-cage deformities: absent or fused ribs • Volume depletion deformity and thoracic growth inhibition = adverse effects on lung growth • Can’t do conventional treatments (fusion): – Bone stock may be insufficient – Patient too small for standard implants for fusion – Lung function may be too poor – Will not address the 3D thoracic deformity – Loss of growth of T-spine from early fusion would impact thoracic volume VEPTR • Vertical expandable prosthetic titanium rib • Dr. Robert Campbell and Dr. Melvin Smith of Christus Santa Rosa Children’s Hospital in San Antonio, TX • Received FDA approval in August 2004 QuickTime™ and a decompressor are needed to see this picture. Surgical Indications • Rib fusion and progressive scoliosis • Hypoplastic thorax syndrome – – – – Jeune’s syndrome Achondroplasia Jarcho-Levin syndrome VACTERL syndrome • Flail chest syndrome – Rib agenesis – Acquired causes Type I: Absent ribs and scoliosis • Stabilization expansion thoracoplasty – Stabilizes the flail chest-wall segment – Laterally expands and lengthens the collapsed hemithorax section • 2-3 VEPTR devices used QuickTime™ and a decompressor are needed to see this picture. Campbell RM, et al 2007 Type II - fused ribs and scoliosis • Opening wedge thoracostomy • Fused hemithorax is osteotomized transversely, lengthened until the thorax is equilibrated • Those under 2 years old with no history spine surgery do best Campbell RM, et al 2007 QuickTime™ and a decompressor are needed to see this picture. Campbell RM, et al 2007 Type IIIa - Jarcho-Levin Syndrome • Staged bilateral opening-wedge thoracostomies • Rib-to-rib VEPTR devices • Lengthen sequentially constricted hemithorax while correcting any associated scoliosis Campbell RM, et al 2007 QuickTime™ and a decompressor are needed to see this picture. Campbell RM, et al 2007 Type IIIb - Jeune syndrome or infantile scoliosis with windswept deformity • Circumferential thoracic constriction • Staged bilateral dynamic segmental curved VEPTR • In windswept deformity scoliosis, concave chest is lengthened with opening-wedge thoracostomy, lengthening of hemithorax, stabilization with unilateral rib to spine or rib to pelvis VEPTR Campbell RM, et al 2007 QuickTime™ and a decompressor are needed to see this picture. VEPTR complications • Prone to complications due to repetitive nature of maintenance surgeries – Wound infection - 3.3% per procedure – Skin slough – Scarring • Migration of fixation - 0.09 migrations/yr/pt • Device breakage • Brachial plexus injuries Campbell RM, et al 2007 Outcomes • Improvement of percentile weight • Decreased in ventilator dependence, increase in thoracic volume • +/- increase in lung volume (and decrease in FVC) - benefit may lie more in stabilizing thorax and improving respiratory mechanics measured in other ways • 10/21 patients had increased activity levels postoperatively Skaggs, et al; Campbell RM, et al 2007; Yazici, et al; Mayer, et al; Waldhausen, et al • Further defining thoracic insufficiency syndrome • VEPTR new device. Long term followup studies still needed References • • • • • • • • • Campbell RM, et al. JBJS Am. 2003;85:399 Campbell RM, et al. JBJS Am. 2004;86:1659 Campbell RM, et al. JBJS Am. 2004;86Suppl1:51 Campbell RM, et al. JBJS Am. 2007;89-A:108 Mayer, et al. J Pediatr Orthop. 2009;29:35 Rameriz N, et al. JBJS Am. 2007;89:2663 Skaggs, et al. Spine. 2009;34:2530 Waldhausen JHT, et al. J Pediatr Surg. 2007;42:76 Yazici, et al. Spine. 2009;34:1800