Legg-Perthes disease in a Down syndrome patient

Jong Sup ShimSamsung 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

LCP disease in Down syndrome

LCP disease in Down syndrome

LCP disease in Down syndrome

Postop

Postop 2 mo

Postop 4 mo

LCP disease in Down syndrome

LCP disease in Down syndrome

• What can we do for this girl?

LCP disease in Down syndrome

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

LCP disease in Down syndrome

Postop 1 yr Postop 2 yr

Postop 1 yr

LCP disease in Down syndrome

LCP disease in Down syndrome

Postop 2 yr

LCP disease in Down syndrome

Postop 2yr

Hip Joint in Down syndromeShaw ED, Beals RK, CORR,1992

LCP disease in Down syndrome

218 hips in 114 patients with Down syndrome(10- 43 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 maleSlipped capital femoral epiphysis (1)

Hip Joint in Down syndromeShaw ED, Beals RK, CORR,1992

LCP disease in Down syndrome

218 hips in 114 patients with Down syndrome(10- 43 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 maleSlipped 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

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