P EDIATRIC S COLIOSIS AND T HORACIC P ATHOLOGY By: Amanda Roof, M.D. Pediatric General Surgery Lecture June 2,

C LASSIFICATION OF S COLIOSIS : Nonstructural Postural and compensatory – both correct when pt leans toward convexity of curve (lateral bending films) Structural Do not correct completely Idiopathic Congenital Neuromuscular Neurofibromatosis Mesenchymal disorders Trauma sections/msk/teaching-materials/online-musculoskeletal- radiology-book/scoliosis

S TRUCTURAL S COLIOSIS : Idiopathic scoliosis – spinal deformity characterized by lateral bending and fixed rotation of the spine in absence of known cause Coronal plane spinal curve (Cobb angle) >10 degrees (<10 = spinal asymmetry) Subtypes: Infantile (0-3 yr) – approx 15-50% progress Assoc with intrauterine molding (plagiocephaly), rib molding, hip contracture. Juvenile (3-10 yr) – approx 70% progress Adolescent (>10 yr) – most common scoliosis in children

O THER S TRUCTURAL C AUSES : Congenital sections/msk/teaching-materials/online-musculoskeletal- radiology-book/scoliosis

I NITIAL WORKUP : PMHx & Family Hx PE Adams forward bend test with scoliometer to measure rotation Neuro exam - esp if suspect cause Standing full spine PA & lateral xrays Treatment of idiopathic scoliosis Observation if curve <20 degrees POSNA: scoliosis (idio/neuromusc) undergoes change in behavior/rapid progression in adolescent growth spurt = 2 degrees/mo Orthoses, serial casting Operation >50-60 degrees and pt desiring correction Must pick procedure based on age of pt and growth of spine remaining

I NTERNAL F IXATION : Posterior Harrington rod – pure distraction Segmental – rods and wires combined to allow translation and rotational correction Growing Rod or VEPTR (vertical expandable prosthetic titanium rib) Anterior Disc excision Segmental anterior instrumentation New thorascopic approach Risks: paralysis, pseudoarthrosis, infection Currently changing field of surg correction per POSNA

E XTRAOSSEOUS I NFLUENCES : P ULMONARY NO significant amount of data on other influences of idiopathic scoliosis Pulmonary: usually only seen in thoracic curves >60 degrees Strong inverse relationship between restrictive ventilatory impairment and scoliotic angle Strongest predictors of resp failure = large scoliotic angle (<110) and low spirometric results (VC<45% predicted) in childhood as only observed age related lung changes

P ULMONARY : Newman et al 2007: Preop pulmonary fxn most impt predictor of post op pulmonary function: but anterior thoracotomy or thoracoplasty = modest decline in PFT 2 yrs after AIS surgical correction. Gitleman et al 2011: Intraoperative chest wall violation for open anterior spinal fusion/instrumentation via open thoracotomy and rib-resection thoracoplasty Decrease in percent-predicted FEVC (85 to79%) and FEV1 (80-76%) values at 10 years

R EVERSE Q UESTION : T HORAX SURGERY CAUSING S COLIOSIS ? = T HORACOGENIC S COLIOSIS West felt and Nordwall Review and Retrospective study: 121 children with lat thoracotomy Incidence of >30% scoliosis over 10 degrees (normal pop 2%) EA – concave toward operated side – thought due to pleural scaring Cardiac malformation correction – convex toward operated side – thought due to musclar imbalance Only 2 >20%

C ASE REPORT : Dunlay et al (Iowa Orthopaedic Journal): 22 yo F with severe structural upper thoracic scoli PMHx: R postlat thoracotomy for repair TE fisula in infancy

Dunlay, Jone, & Weinstein. Scoliosis caused by rib fusion following thoracotomy for tracheoesophageal fistula: Case Report. The Iowa Orthopaedic Journal. 2007: 27. p95-88

Q UESTSIONS / DISCUSSION ? ent=scoliosis.jpg&newest=1

R EFERENCES Univeristy of Washington Departmen of Radiology: Musculoskeletal Radiology: Scoliosis on WWW at materials/online-musculoskeletal-radiology-book/scoliosis materials/online-musculoskeletal-radiology-book/scoliosis Scoliosis under 40 degrees from POSNA.org 2011 on WWW at: media=print Pehrsson et al. Lung function in adult idiopathic scoliosis: a 20 yr follow up. Thorax 1991; 46; Pehrsson Et al. Long term follow up of Patients with Untreated scoliosis. Spine Vol 17(9) Gitelman et al. Pulmonary Function in Adoescent Idiopathic Scoliosis Relative to the Surgical procedure: A 10-year follow-up Analysis Spine publish ahead of print Newton et al. Predictors of Change in Post op Pulmonary Function in Adolescent Idopathic Scoliosis: A prospective Study o f254 Pt. Spine 2007, 32(17) p Westfelt and Norwall. Thoracotomy and Scoliosis. Spine Sep; 16: p Dunlay, Jone, & Weinstein. Scoliosis caused by rib fusion following thoracotomy for tracheoesophageal fistula: Case Report. The Iowa Orthopaedic Journal. 2007: 27. p95-88 Pictures – as referenced on slide

ADDITIONAL SLIDES

O RTHOSES Milwaukee brace = based on stable pelvic girdle and 3 pressure points at ribs, flank, pelvis Wilmington and Boston = underarm Charleston and Providence = night only braces Spine-Cor