Publication: Clinical audit on osteogenesis imperfecta patients treated in Hospital Universiti Sains Malaysia for past 17 years
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Date
2022
Authors
Mohamed, Nik Abdullah Afif Nik
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Abstract
Osteogenesis imperfecta is an uncommon disease that presented with different severities (Sillence type I,II, III, and IV) and condition. Many surgeons,even without good experience in treating osteogenesis imperfecta patients, need to treat these patients due to limited number of pediatric orthopedic surgeons. Recurrent fracture, deformity, problem related to intramedullary rod, malrotation, nonunion and scoliosis were among the problem faced by those patients. We reviewed all the cases treated in our institution to know the pattern of presentation and treatment with the outcome. Methodology We have reviewed all osteogenesis imperfecta patients treated in our hospital from 2003 -2020. Patients were traced from record office based on International Classification of Disease, 10th Revision (ICD -10). The required clinical information was obtained from patients record and General Healthcare Centricitytm picture archiving communication system (PACS). The information was scrutinized and presented with descriptive manner. Results We have treated 27 patients for the past 17 years, however 4 of them lost from follow up. Most of patients with Sillence type III and 4 presented with deformity or fracture shaft of long bones. Among the presentation of Sillence type I patient were developmental dislocated hip (2 patients), olecranon fracture (3 patients), flexion type supracondylar humerus fracture (2 patients). Scoliosis were found in 4 Sillence type III patients at adolescent age. Most common fixation for femur was single rush rod fixation. All femurs of Sillence type III and Sillence type IV patients treated with rush rod united with malrotation. Single rush rod in the femur required revision within 2 to 3 years due to problem related to growth. Double rush rod and expandable nail of femur did not require revision with 5 years. Rush rod through the piriformis fossa did cause radiological changes of avascular necrosis of femoral head. Modified Finidori’s technique using rush rod and wires through the neck of femur was able to correct Coxa Vara but not able to control rotation. Autogenous bone graft from iliac crest of four patient to treat nonunion and acute lengthening were successfully done without causing morbidity to the patients. Malrotation and short limb were derotated and acutely lengthened with interlocking nail at maturity. Ambulation of patient at maturity depend on the type of severity based on Sillence classification. Conclusion Treatment of osteogenesis imperfecta patients in our institution was comparable with available evidences in the literature.
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Keywords
Bone graft , Double rod