Congenital Knee Dislocation at Birth - An Extraordinary Case of Spontaneous Reduction.

Congenital knee dislocation (CKD) is a rare malformation characterized by hyperextension deformity of the knee with anterior tibia displacement, present at birth. Rarely reported, CKD might occur as an isolated deformity or commonly associated with musculoskeletal abnormalities, with the most common ones being developmental dysplasia of the hip (DDH) and clubfoot. The etiology is unknown, but CKD has been associated with certain intrinsic and extrinsic factors. Treatment with conservative methods at an early stage is most likely to yield successful results. We report here a rare case of successful spontaneous reduction of CKD in an infant within 24 hours of life.

Congenital Knee Dislocation at Birth - An Extraordinary Case of Spontaneous Reduction / Luxação congênita do joelho no nascimento - Um caso extraordinário de redução espontânea

Abstract Congenital knee dislocation (CKD) is a rare malformation characterized by hyperextension deformity of the knee with anterior tibia displacement, present at birth. Rarely reported, CKD might occur as an isolated deformity or commonly associated with musculoskeletal abnormalities, with the most common ones being developmental dysplasia of the hip (DDH) and clubfoot. The etiology is unknown, but CKD has been associated with certain intrinsic and extrinsic factors. Treatment with conservative methods at an early stage is most likely to yield successful results. We report here a rare case of successful spontaneous reduction of CKD in an infant within 24 hours of life.

Resumo A luxação congênita do joelho (LCJ) é uma malformação rara caracterizada por deformidade de hiperextensão do joelho com deslocamento anterior da tíbia, presente ao nascimento. Raramente relatada, a LCJ pode ocorrer como uma deformidade isolada ou comumente associada a anormalidades musculoesqueléticas, sendo as mais comuns a displasia do desenvolvimento do quadril (DDQ) e o pé torto congênito (PTC). A etiologia é desconhecida, mas a LCJ foi associada a certos fatores intrínsecos e extrínsecos. O tratamento com métodos conservadores em um estágio inicial tem maior probabilidade de produzir resultados bem-sucedidos. Relatamos aqui um caso raro de redução espontânea bem-sucedida de LCJ em um bebê nas suas primeiras 24 horas de vida.

Cross-cultural Adaptation and Validity of the Patient Rated Michigan Hand Outcome Questionnaire in Bahasa Melayu for Malaysian Patients.

Background: The Michigan Hand Outcomes Questionnaire (MHQ) was designed in 1998 by Chung and colleagues at the University of Michigan in 1998. It is a hand-specific patient-rated outcome (PRO) instrument used to measure patients' outcome with conditions of, or injury to, the hand and/or wrist. This study is aimed at translating and cross-culturally adapting the MHQ into Bahasa Melayu (BM), examining the validity and reliability of the MHQ-BM version for our local population to be used as a patient-rated outcome measurement in Malaysian patients with hand injuries. Methods: This study was performed in two phases. Phase 1 involved the translation of the MHQ into BM using Beaton Protocol. In Phase 2, we administered the final version of the MHQ-BM in patients with hand and/or wrist injuries to test its stability (n = 50) and validity (n = 185). Results: The MHQ-BM showed good content and face validity. Cronbach α totalled 0.821 for the MHQ-BM. The Intra-class Correlation Coefficient (ICC) of the test-retest reliability for the six domains ranged from 0.960 to 0.984 with excellent correlation (ICC = 0.984) for the total score. The BM version of MHQ-BM showed excellent test-retest reliability. Conclusions: The MHQ-BM is well-translated and culturally well-adapted to maintain the reliability and content validity of the instrument (MHQ) at a conceptual level across Malaysia. We found the application and evaluation of the instrument to be feasible and understandable among patients with hand and/or wrist injuries in Malaysia.

Severe presentation of non-ossifying fibroma of the femur in osteoglophonic dysplasia.

Osteoglophonic dwarfism, also known as osteoglophonic dysplasia (OD), is an uncommon skeletal dysplasia with an autosomal dominant mode of inheritance, which equally affects boys and girls. OD is saliently featured by craniosynostosis, dysmorphic facial features, impacted mandibular teeth, rhizomelic limb shortening and non-ossifying fibromas habitually at the metaphyseal regions, which usually disappear after skeletal maturity. The long bones in OD are portrayed by this distinguishable 'hollowed-out' appearance with metaphyseal cystic defects that have a natural history of spontaneous resolution. We report a case of a rare and unusual presentation of OD in a 23-year-old woman whom has been diagnosed with OD during her early childhood. She presented with a progressively enlarging right thigh swelling associated with pain for the past 1 year. Her right femur plain radiograph revealed diffuse lysis of the whole femur with cortical thinning. MRI revealed multiple bilateral femur benign cystic lesion synonymous with a severe spectrum of OD. She was started on a trial of oral bisphosphonates, which led to a significant improvement in pain.