Bruck syndrome: A rare case presentation with isolated lower extremity contractures and multiple postoperative peri-implant fractures

Bruck syndrome, characterized by congenital brittle bones and multiple joint contractures, is a rare variant of osteogenesis imperfecta. Here, we report the case of a 7-year-old male patient who presented with a fractured shaft of the femur following trivial trauma. He was diagnosed case of arthrogryposis multiplex congenital with deformities involving both knees and ankle. He had a history of bilateral femoral fractures during birth. Due to the knee contracture and a narrow canal, we fixed the fracture by plating. However, the patient developed peri-implant fractures proximal to the plate. Due to the presence of multiple peri-implant fractures and joint contractures, we diagnosed the patient with Bruck’s syndrome and initiated intravenous bisphosphonate therapy. Subsequently, the patient developed one more fracture in the contralateral femur. This case signifies the importance of screening all patients with multiple congenital contractures and recurrent fractures for Bruck syndrome.

Ilizarov method in treatment of neurological foot deformities

Introduction Diseases related to the lesions of upper or lower motor neurons, such as spina bifida, myopathy, and residual polio, often cause severe deformities of the foot and ankle. The treatment goal is to convert a deformed and rigid foot into a plantigrade foot. Matherial and method We treated 51 neurological feet from 2001 to 2018 with the Ilizarov method. They were polio outcomes in 27 cases, 17 feet were affected by spina bifida, 6 other patients had Charcot-Marie-Tooth disease. Results Results were assessed by comparing appearance and function before and after surgery described by Dimeglio, and modified by Dong Y.L. et al. Before surgery, there were 33 moderate and 28 severe deformities (average preoperative scores, 6.3). The mean time required to correct a deformity was 37.1 days (range, 22–58 days) and the mean time for stabilization was 67.2 days (range, 45–98 days). At latest follow-up (mean 7.42 years, from 13 month to 16 years), clinical outcomes and patient satisfaction were considered acceptable. 28 patients achieved an excellent outcome and 21 patients had a good outcome, two feet were classified as having a poor result. Nearly 86 % of patients (44 patients) were satisfied with their outcomes and replied they would repeat their procedure. Complications include infection in 15 % of K-wire tracts, Dysesthesia in 5 cases, superficial necrosis in 3. One patient developed knee contracture. 2 patients were re-operated with V osteotomy. Conclusions The Ilizarov method represents a great resource in the treatment of severe neurological deformities of the foot.

Knee Spine Syndrome Mechanism in Knee Osteoarthritis: Review Article

Introduction: Osteoarthritis (OA) is the most common arthritis and one of the main causes of disability. This injury can occur in any joint, but the most common OA occurrence globally, especially in Indonesia, is Knee OA (KOA). Damage caused by KOA's degenerative process affects the knee and affects the surrounding structures, such as the lumbar vertebra, which increases symptoms of low back pain (LBP). The occurrence of symptoms of LBP is thought to be caused by knee spine syndrome, which is a condition reduced lumbar lordosis angle due to the degenerative phase of KOA. the mechanism of this syndrome has not yet been discussed in detail, so further discussion is needed in the future. Methods: We searched articles using a list of keywords, including "knee osteoarthritis and low back pain", "knee osteoarthritis and knee contracture", "knee contracture and muscle weakness", "knee contracture and spinal alignments", "knee osteoarthritis and spinal alignment", "knee osteoarthritis and sagittal spinal alignment", and "muscle activation and spinal imbalance" on the data sources of PubMed, ScienceDirect, and Google Scholar. The argumentative, descriptive analysis was used for the data analysis technique. Results: Based on the literature search, the author found four related articles. This study provides evidence of the effect of KOA on lumbar proprioception. These articles explained that an impact on knee posture could have long-term effects both in the spine and pelvis, such as lumbar lordosis. Conclusion: Knee spine syndrome results from a reduced angle of lumbar lordosis, increasing pressure between discs, which might increase LBP symptoms. In the future, it is hoped that there will be studies that raise the relationship between KOA and symptom of LBP.

Fourteen-year follow-up of a child with acroscyphodysplasia with emphasis on the need for multidisciplinary management: a case report

Background Acroscyphodysplasia has been described as a phenotypic variant of acrodysostosis type 2 and pseudohypoparathyroidism. In acrodysostosis, skeletal features can include brachydactyly, facial hypoplasia, cone-shaped epiphyses, short stature, and advanced bone age. To date, reports on this disorder have focused on phenotypic findings, endocrine changes, and genetic variation. We present a 14-year overview of a patient, from birth to skeletal maturity, with acroscyphodysplasia, noting the significant orthopaedic challenges and the need for a multidisciplinary team, including specialists in genetics, orthopaedics, endocrinology, and otolaryngology, to optimize long-term outcomes. Case presentation The patient presented as a newborn with dysmorphic facial features, including severe midface hypoplasia, malar flattening, nasal stenosis, and feeding difficulties. Radiologic findings were initially subtle, and a skeletal survey performed at age 7 months was initially considered normal. Genetic evaluation revealed a variant in PDE4D and subsequent pseudohypoparathyroidism. The patient presented to the department of orthopaedics, at age 2 years 9 months with a leg length discrepancy, right knee contracture, and severely crouched gait. Radiographs demonstrated cone-shaped epiphyses of the right distal femur and proximal tibia, but no evidence of growth plate changes in the left leg. The child developed early posterior epiphyseal arrest on the right side and required multiple surgical interventions to achieve neutral extension. Her left distal femur developed late posterior physeal arrest and secondary contracture without evidence of schypho deformity, which improved with anterior screw epiphysiodesis. The child required numerous orthopaedic surgical interventions to achieve full knee extension bilaterally. At age 13 years 11 months, she was an independent ambulator with erect posture. The child underwent numerous otolaryngology procedures and will require significant ongoing care. She has moderate intellectual disability. Discussion and conclusions Key challenges in the management of this case included the subtle changes on initial skeletal survey and the marked asymmetry of her deformity. While cone-shaped epiphyses are a hallmark of acrodysostosis, posterior tethering/growth arrest of the posterior distal femur has not been previously reported. Correction of the secondary knee contracture was essential to improve ambulation. Children with acroscyphodysplasia require a multidisciplinary approach, including radiology, genetics, orthopaedics, otolaryngology, and endocrinology specialties.

The judet quadricepsplasty for elderly traumatic knee extension contracture: a case report and review of the literature

Traumatic injuries to the knee are frequently complicated by extension contractures. The Judet Quadricepsplasty allows for controlled, sequential release of extrinsic and intrinsic knee contracture components while reducing the potential for iatrogenic quadriceps rupture. We document our institutions experience with this procedure and a systematic review of the current literature. We followed up on an elderly patient with posttraumatic flexion contracture that failed conservative management and underwent Judet Quadricepsplasty. Her knee range of motion improve dramatically from 20 degrees of flexion to 100 degrees of flexion. There was a residual extension lag of 5 degrees which did not impede on the patients daily activities. A review of the literature was performed and relevant data from 12 articles was extracted. The procedure was mainly performed in young adult males in most previous studies and the range of motion improvement ranged from 51° to 110°. Wound infections were the most common complication but otherwise other complications and severe extension lag were rare. The Judet Quadricepsplasty is a useful procedure for severe extension knee contractures that has failed conservative management in all age groups of patients. It is associated with significant increases in range of motion with low rates of complication or extension lag. Diagnostic IV