[Limb lengthening in children with osteogenesis imperfecta]

Area of Science:

• Orthopedic Surgery
• Pediatric Orthopedics
• Genetics and Bone Diseases

Background:

• Osteogenesis imperfecta (OI) type I presents with brittle bones and can cause significant lower limb length discrepancies in children.
• Managing leg length discrepancies in pediatric OI patients requires specialized surgical techniques.

Purpose of the Study:

• To evaluate the feasibility and outcomes of lower limb lengthening in children with osteogenesis imperfecta type I.
• To assess the effectiveness of Ilizarov and Wagner techniques in correcting limb length discrepancies.

Main Methods:

• Seven children (5 girls, 2 boys) with OI type I underwent lower limb lengthening procedures.
• Ilizarov and Wagner techniques were employed for femur and tibia elongations, with intramedullary Rush rods used in most cases.
• Osteotomies were performed in metaphyseal or distal regions, with controlled daily elongation of 1 mm.

Main Results:

• A mean bone lengthening of 5.5 cm was achieved, resulting in a mean limb lengthening of 7.9 cm.
• The mean elongation period was 2.8 months, with an elongation index of 26 days/cm.
• Complications were observed in all patients, highlighting the inherent risks of the procedure.

Conclusions:

• Lower limb lengthening is a viable option for children with OI type I, enabling equalization or reduction of leg length discrepancies.
• Despite a high incidence of complications, the benefits of limb equalization justify the surgical intervention.
• Careful surgical planning and execution are crucial for managing this challenging patient population.