Bilateral hip arthroplasty for ochronotic arthropathy.

Ochronosis is a musculoskeletal manifestation of alkaptonuria, a rare hereditary metabolic disorder characterised by the absence of the enzyme homogentisic acid oxidase and associated with various systemic abnormalities related to the deposition of homogentisic acid pigment (ochronotic pigment). In this report, we describe a 53-year-old, HLA-B27(+) woman with ochronotic arthropathy. In addition to the typical clinical features of the disorder, she had bilateral hip involvement, which was improved by cementless total hip prosthesis.

Detection of an unbalanced t(4;15) by FISH in a child with multiple congenital anomalies.

Detection of an unbalanced t(4;15) by FISH in a child with multiple congenital anomalies: In this report, we present the clinical history and findings in a 6-month-old male with multiple congenital anomalies, developmental delay, and an initial male karyotype with 4q+. The origin of the additional segment on 4q was unequivocally established by fluorescence in situ hybridization (FISH). Whole chromosome probe for chromosome 4 and chromosome 15-specific a-satellite probe were used. The karyotype was demonstrated to be 46,XY,der(4), t(4;15)(q35;?),inv(9)(p13q13). To the best of our knowledge the above cytogenetic abnormalities with these clinical findings have not been described previously. This case further demonstrates the advantage of FISH in the identification of anomalous chromosome regions and breakpoints.

Fuhrmann syndrome associated with cortical dysplasia.

We describe a male newborn with bilateral angle bowing of femora, absent fibulae, aplasia of the fingernails, hypoplastic toenails, malformed thumbs, hypospadias, inguinal hernia and cortical dysplasia in a consanguineous Turkish Family. The MCA syndrome in the present patient is similar to these reported in 3 affected sibling by Fuhrmann et al.

Proteus syndrome associated with liver involvement: case report.

We report a patient with proteus syndrome who has epidermal nevus, right-sided asymmetric growth of extremities, pelvis, vertebrae and hemimegalencephaly. This patient also had enlargement of the liver which is not reported before in the proteus syndrome.

Proximal focal femoral deficiency, contralateral hip dysplasia in association with contralateral ulnar hypoplasia and clefthand: a case report and review of literatures of PFFD and/or FFU.

A 14 year old boy with proximal focal femoral deficiency (PFFD) on left side, contralateral hip dysplasia in association with ulnar hypoplasia and cleft hand was seen our clinic one year ago. From our research, despite it is atypical presentation, this case in a broad context conforms with the femoral-fibula-ulna complex (FFU). This boy was born to healthy and nonconsanguineous parents. Pregnancy and delivery were uncomplicated, and no history of prenatal teratogen exposure, any drug ingestion, infection, diabetes mellitus or other conditions that could be associated with malformation. The patient refused any sort of surgical treatment.

Malignant mixed tumor of salivary gland with a solitary metastasis to the tibia.

A 23-year-old, male patient previously operated on four times for a recurring, benign, paranasal mass was admitted to our clinic with pain and swelling under the knee. Physical and radiological examination revealed a malignant process. The histopathological examination of the fine-needle biopsy specimen of the tibia was a malignant mixed tumor. Then. en-bloc resection, free fibular grafting, and fixation with blade plate was performed for one-third of the tibia, conserving the knee joint. Recurrences and metastasis were not observed after surgery and radiotherapy.

Three-headed reversed palmaris longus muscle: a case report and review of the literature.

The palmaris longus muscle (PLM) is extremely variable both in number and form. We present the first reported case of three-headed reversed PLM in a 36-year old woman. The relevant literature is reviewed. Variations of the muscles of the forearm and specifically the PLM are not uncommon. However, different rates are given for the types and agenesis of PLM. In one study, the incidence of agenesis was 12.8% and other anomalies were 9%. 15 cases of reversed PLM have been reported in the literature. In our case, the three-headed PLM was resected. The patient's symptoms resolved and at 1-year follow-up she remains symptom-free.

A new interference nail fixation for anterior cruciate ligament soft tissue grafts.

We present a new device for femoral and tibial fixation in anterior cruciate ligament reconstruction using soft tissue. We evaluated the method biomechanically in pullout tests using human cadaver knee joints. Fixation strengths ranged from 300 +/- 60 to 600 +/- 50 N (mean 400 +/- 50 N). This corresponds to loads in the graft during aggressive rehabilitation although cadaveric specimens were used. With respect to primary fixation strength this implant is a reasonable alternative for anterior cruciate ligament graft fixation.

Synovial hemangioma in Hoffa's fat pad (case report).

A 15-year-old girl presented with anterolateral knee pain for 6 months. There were tenderness and pain at the anterolateral triangulation of the knee joint. Magnetic resonance imaging revealed a tumor in the anterolateral corner of the knee joint. Arthroscopy did not show the tumor because it was located in fat pad. The tumor was found during fat pad shaving and was resected arthroscopically. Synovial hemangioma was demonstrated histologically. The patient is asymptomatic postoperatively.

An accessory ossification centre in the calcaneus with talonavicular and second metatarsocuneiform coalitions.

We present the case of an 11-year-old girl with an accessory ossification centre of the calcaneus with talonavicular and second metatarsocuneiform coalitions, and bilateral os tibiale externum.

Treatment of osteochondral defects with tendon autografts in a dog knee model.

Tendon autograft was used for the treatment of osteochondral defects induced experimentally in dogs' knees. Osteochondral defects were created on the femoral condyles in both knees of five dogs. The tendon was harvested from the anterior cruris, and a knot was tied in the middle. Then two tunnels were bored in the medial condyle. The free ends of the tendon were individually inserted into the tunnels and pulled from the openings on the medial recessus side so that the knot itself sat within the osteochondral defect. The lateral condyle was left untreated for comparison. At 24 weeks postoperatively, the dogs were killed and macroscopical and histological examinations were performed. A congruous articular surface had formed, and the grafted tendon was bonded into the defect. The tendon was not degraded, and was covered with reparative tissue as seen microscopically. Despite weight-bearing being allowed in the early stages after the operation, no sign of degradation was noted in the tendon.