Myelodysplasia 

Background  Common group of congenital disorders by various chromosomal anomalies that lead to failurre of closure of the spinal cord  Anatomic anomalies  Neurologic impairment  Chromosomal abnormalities  10% T13, T18, triploidy, various single gene  AFP 2nd trimester  Risk Factors  Folate deficiency  Maternal hyperthermia  Maternal diabetes  Valproic acid  Associated Conditions  Ortho  Path fractures (osteopenia)  Spine  Scoliosis –  – PSIF toContinue reading “Myelodysplasia “

Atlantoaxial Rotatory Dis 

Background  C12 subluxation or facet dislocation  Presents with kid with what looks like torticollis  Congenital muscular torticollis –   The spasm itself turns the head (so opposite side of chin)  AARD  The spasm is protective to prevent FURTHER rotation (so same side as chin)  Pathophysiology  Ligamentous laxity  TL intact  TL ruptured  Causes  Infection (25%)  Grisel’s diseaseContinue reading “Atlantoaxial Rotatory Dis “

EOS (Idiopathic) 

Background  Infantile <4  Juvenile 4-10  Natural history:  Infantile spontaneous resolution in many (esepcially infantile)  Juvenile high requirement of surgery  M>F  Left thoracic  Risk of Progression  Radiographic  Rib – Vert overlap at apical – high risk  RVAD – rib – vert angle difference – >20 high risk  Investigation  Higher incidence of neural axis anomaly  Syringomyelia (cyst inContinue reading “EOS (Idiopathic) “

Syndromic Scoliosis

Clinical Features of Syndromic Dysplastic Curves  Atypical shape  Long C  Short angular curve  High rotation  Bony dysplasia  Atypical progression  Variable rates  Progress after fusion  Systemic Features  Types  Neurofibromatosis  Dystrophic  Non dystrophic  Dural ectasia  Marfan’s  Dural ectasia  Rett  Ehlers Danlos  Prader Willi  OI  Down’s  Dural ectasia findings (NF and Marfan)  Vertebral scalloping (posterior and lateral)  VertebralContinue reading “Syndromic Scoliosis”

Neuromuscular Scoliosis

Types  Neurologic  Central   Scoli  Cavus feet  SMA (ANTERIOR HORN)  SMN 2  Ttype 1 worse  Hip dislocaiotn  Scoliosis 100%  Contracture release before Scoli  For WC sitting  Nucenecin  CP  Bracing does not prevent scolio  Periop mortality 0.5-1%  Surgery increases life expectancy  Scoli development directly correlataed to GMFCS score  100% with GMFCS5  Main indication:  Improves caregiver QOL  CMT  Myelomeningocele  Polio  Syringomyelia  Myopathic  Becker’s/ Duchenne MD  95%Continue reading “Neuromuscular Scoliosis”

Congenital Scoliosis

Background  Epi 1-4%  Failure of neural axis 4-6w of development (mesenchymal anlage)  Progresses most rapidly in early years  Etiology  Maternal  DM  Alcohol  Valproic acid  Hyperthermia  Associated Conditions  60% with associated  Cardiac, genitourinary, spinal cord  VACTERRL  38-55%  Vertebral Malformation  Anal atresia  Cardiac  Tracheal- Esophageal fistula  Renal  Radial anomaly  Limb defects  Goldenhar  Jarco Levin  Trunkal dwarfism  Thoracic Insufficiency Syndrome Continue reading “Congenital Scoliosis”

AIS 

Background  Children 10 – 18 idiopathic  3% have minor, .3% serious (>30)  10:1 female  R thoracic more common (wraps around the heart)  Presentation  Shoulder height  Truncal shift  Pelvis symmetrry  Adams bend test – axial plane deformity (rib hump) means structural curve  Midline skin defects (spinal dysraphism)  Café au lait (NF)  Foot deformity (cavovarus) – neural axial,Continue reading “AIS “

Scoliosis Overview

Types of Scoliosis  Vertebral defects (formation or segmentation)  VACTERL  Neuromuscular  CP, DMD  myelodysplasia  Syndromic  NF1, Marfan’s, Rett’s, OI, Klippel Feil, Ehler’s Danlos  Idiopathic  Infantile < 3 years  Juvenile 4-9  Adolescent >10 years  Alternative  Early onset <10  Congenital   Structural   Chest wall abnormalities, fused ribs, congenital diaphragmatic hernia  Neuromuscular  Syndromic  Idiopathic EOS  Infantile <4  Juvenile 4-10  LateContinue reading “Scoliosis Overview”