Osteogenesis imperfecta mumbai

MANAGEMENT OF OSTEOGENESIS IMPERFECTA

Dr Atul BhaskarFRCS(Orth),FRCS, M.S., MCh

Paediatric Orthopaedic Surgeon

Fellowship Training in Paediatric Orthopaedics (Hospital for Sick Children – Toronto)

Texas Scottish Rite HospitalGillette’s Children Hospital

Fractures in Osteogenesis Imperfecta

• Three Types1) Multiple fractures in Severe OI: acute /stress fractures2) Acute fracture with trivial Injury: Classical OI – usually involves

lower limb, humerus3) Fractures akin to normal children: mild OI (tarda form)

“fracture pattern not typical of any OI classification”

Multiple pathological fractures in a 2 year old girl.

Acute / Stress Fracture Chronic Stress Fracture

Sites

• Femur• Tibia• Humerus• Forearm• Olecranon( Sleeve Fracture) Multiple Fractures leading to coxa vara, angular and bowing

deformities and LLD 90% fractures before skeletal maturity

Treatment

• Conservative

• Intrameduallry Rodding Non-Telescoping: Rush/Williams Telescoping: Sheffield / F –D

• Bisphosphonates

Any role of conservative Treatment in OI Fractures

Waiting for Surgery !!!Children < 18 months

RODDING OPTIONS

RUSH RODS AND DUAL RUSH RODS

WILLIAMS ROD

TELESCOPING ROD

Never use plate in OI

Plating

MUST BE AVOIDED AS HIGH RISK OF STRESS FRACTURE.

Revised to F – D Telescoping Rod

7 year old child with OI

Plating done for femur fracture

Deformity appears above and below the plate due to stress shielding of bone

20 month old girl, repeated femur fractures, unable to stand ?delayed milesones

Four months post op

Nine Months post-op

Standing with support

FRACTURE --------OSTEOPOROSIS---------REFRACTURE

Early Surgery and Preparatory Pamidronate

Bilateral Femur Deformity in a 12 year old girl with Osteogenesis Imperfecta

Operated 3x

Plating

Cross Wires

Intra-medullary rodding and post deformity correction.

Post IM rodding of femur: one year FU

TIBIA FRACTURE: TREATED WITH TELESCOPING ROD

SUPPLEMENTARY FIXATION MAY BE REQUIRED FOR DISTAL FRACTURES AND TO CONTROL ROTATION

OI

• 10 year old child• OI ? Type III, progressive deformity• 5 cycles of Pamidronate received• No surgery done

How early to rod ?

Rodding : before or after bisphosphonate treatment

Choice of Rod

Classical Shish Kabab Surgery

Very Narrow IM Canal: 2mm Rod inserted

Spica for 6 weeks and followed by splints