Legg-Perthes disease in a Down syndrome patient Jong Sup Shim Samsung Medical Canter Sungkyunkwan University School of Medicine

An 11 year-old female patient Painful limping for 2 months Can’t walk without assistance Has underlying Down syndrome (Diagnosed at age 11 mo) LCP disease in Down syndrome

Past Medical History She was diagnosed as Legg-Perthes disease at other university hospital. Operation was performed for LCP disease and hip subluxation by open reduction, varization, Salter operation and trochanteric apophysiodesis (at age 10yr 8 mo). After 2 months of Spica cast immobilization, then persistent hip dislocation was found. LCP disease in Down syndrome

Preop -Xray LCP disease in Down syndrome

Postop Postop 2 mo

Postop 4 mo LCP disease in Down syndrome

What can we do for this girl? LCP disease in Down syndrome

Re-Do open reduction (with tight capsular plication) Chiari osteotomy Postop cast immobilization for 6 weeks

LCP disease in Down syndrome Postop 3 mo

LCP disease in Down syndrome Left genu valgum was detected

Hemiepiphyseal stapling (at age 11 yr 6 mo) Preop Postop 2 yr LCP disease in Down syndrome

Postop 1 yr Postop 2 yr

Postop 1 yr LCP disease in Down syndrome

Postop 2 yr

LCP disease in Down syndrome Postop 2yr

Hip Joint in Down syndrome Shaw ED, Beals RK, CORR,1992 LCP disease in Down syndrome 218 hips in 114 patients with Down syndrome ( years old) 11 hips(4.8%) in 9 Patients(7.9%) had some hip abnormality Dysplasia (5) Dislocation (4) Avascular necrosis (1) – 15 yr old male Slipped capital femoral epiphysis (1)

Hip Joint in Down syndrome Shaw ED, Beals RK, CORR,1992 LCP disease in Down syndrome 218 hips in 114 patients with Down syndrome ( years old) 11 hips(4.8%) in 9 Patients(7.9%) had some hip abnormality Dysplasia (5) Dislocation (4) Avascular necrosis (1) – 15 yr old male Slipped capital femoral epiphysis (1)

Perthes disease/avascular necrosis in Down syndrome LCP disease in Down syndrome Lawhon et al, Contemp Orthop,1990 4/162 Makley and Sligar, Orthop Tran,1979 3/100

Treatment of severe late onset Perthes disease with soft tissue release and articulated hip distraction: early result Segev et al,JPO(B),2004 “In Down syndrome, results significantly inferior to those having only Perthes’ disease.” LCP disease in Down syndrome

Thank you for your kind attention