Objective Congenital & Developmental Malformation Overview of Musculoskeletal dev. Abnormal pattern of dev. Common upper & lower ext. abnormalities READ : SPINE and more information in text book Definition Development of Musculoskeletal System Congenital Abnormality of organ, found before / after labor Developmental Abnormality of organ, gradually occur during growing period Embryonic or Dev. period ( 8 wks) Organogenesis & Morphogenesis Fetal period Four weeks of Intrauterine life Six weeks 1
Eight weeks Approach to Infant with deformity Intrinsic factors Chromosomal or genetic program abnormalities abortion or abnormality Extrinsic factors Biomech. Force Irradiation Infection Drug & Chemical Normal,Malformation,Disruption, Deformation,Dysplasia Genetic Drug,Chemical Infection Malformation Failure or formation Failure of separation Duplication Overgrowth Undergrowth Failure of formation Failure of formation 2
Failure of separation Duplication Biomech. Force Disruption Duplication Biomech. Force Deformation : * FOREFOOT DEFORMITY * TYPE - MILD METATARSUS ADDUCTUS - MODERATE - SEVERE 3
Acetabular dysplasia Common Cong.& Dev. Abnormality of Upper extremity Syndactyly : The MOST common Polydactyly Most common between MF-RF Metatarsus adductus 4
Deficientcy & Absence of the radius Problem? Failure of formation of the radial side of upper limb bud 1. Cosmetic 2. Function Treatment : Centralization Carpal bones on one bone ( ULNA ) Stretching Splinting Surgery Don t forget associated abnormality VATER VACTERLS Treatment : Centralization Carpal bones on one bone ( ULNA) Treatment : Centralization Carpal bones on one bone (ULNA) 5
Common Cong.& Dev. Abnormality of Lower extremity CLUB FOOT or TALIPES EQUINOVARUS Clubfoot : Talipes equinovarus * COMBINATION OF DEFORMITY * TYPE - POSITIONAL - FLEXIBLE - RIGID Prevalence of 1.5 per 1,000 live births. Bilateral in approximately half of cases. Male are most affected. 2-6 % chance of next sibling if one child has a clubfoot. 25 % chance of next offspring if the parent has a clubfoot. 6
Clinical features of clubfoot CLUB FOOT : TREATMENT Ankle equinus. Heel varus. Forefoot adduction. * Stimulation * Manipulation * Stretching * Serial casting * Tendon lengthening * Surgical Rx(rare) Developmental Dysplasia & Dislocation of the Hip ( DDH ) Dev.dysplasia of the Hip DDH /// SPECTRUM Teratologic hip Unstable hip Subluxated hip Dislocated hip Acetabular dysplasia Teratologic hip DDH /// ETIOLOGY PHYSIOLOGIC FACTORS MECHANICAL FACTORS ENVIRONMENTAL FACTORS GENETIC FACTORS 7
DDH /// HIGH RISK INFANTS High risk breech presentation first born, female familial history High-risk factor for DDH Breech presentation Female gender Positive family history of ethnic background Lower limb deformity Associated torticollis, metatarsus adductus?? Oligohydramnios Significant persistent hip asymmetry (eg. adducted hip on one side, adducted hip on the other side) Other significant musculoskeletal abnormalities DDH // PHYSICAL EXAMINATION VARY WITH AGE!!! 1. 0-3 m. old : laxity stage based on Barlow & Ortolani test 2. > 3 m. old : surrounding pelvic - femoral muscle adaptively shorten in response to the position of the hip BARLOW TEST : 1st. Adduction Pressure on the knee downward along the axis of the femur Provoking subluxation, dislocation BARLOW TEST : 2nd ~ ORTOLANI TEST + Barlow test + Ortolani test Reduction Abduction Gently lifting the femur socket A hip that is reduced but dislocatable A hip that is dislocated But reducible 8
Barlow test Ortolani test DDH // CLINICAL FINDINGS after 3 mo. old Trendelenberg gait tight adductors shortening telescoping abn.skin fold Allis(Galleazzi ) Trendelenberg Waddling gait Allis ( Galleazzi ) DDH /// IMAGING // X- Ray Unreliable before 2nd ossification presented ( normal at 4-6m) 9
DDH : INVESTIGATION ULTRASOUND (0-3 m) PLAIN X-ray MRI, CT SCAN DDH : MANAGEMENT Acetabular dysplasia Early diagnosis & imm. Treatment Pavlik s Harness F/U with US, X-ray Open reduction in unreducible case, at any age ( >1yr) 10