Cases reported "Arthrogryposis"

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1/8. misoprostol embryotoxicity: clinical evaluation of fifteen patients with arthrogryposis.

    We report on clinical evaluations of Brazilian patients with misoprostol-induced arthrogryposis. All 15 patients had growth retardation, underdeveloped bones, short feet with equinovarus, rigidity of several joints with skin dimples and webs, decreased movement of legs stemming from neurologic impairment, bilateral symmetrical hypoplasia or atrophy of limb muscles, and absent tendon reflexes. Of the 15 patients, five had upper limb deformities in addition to lower limb involvement, and one had spinal cord disruption leading secondarily to segmental sensory loss and neurogenic bladder and bowel. Electroneuromyography of five patients indicated that the abnormalities were of neurogenic origin and suggestive of anterior horn cell defects. All of their mothers took 400-4,800 mcg of misoprostol orally or vaginally at 8 to 12 weeks of pregnancy. Our observations support a previously stated caution with regard to the embryotoxicity of misoprostol.
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ranking = 1
keywords = equinovarus
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2/8. Bruck syndrome.

    The combination of arthrogryposis multiplex congenita and osteogenesis imperfecta is extremely rare. This combination is named Bruck syndrome. A 34 week male baby weighing 1.7 kg at birth was noted to have multiple flexion contractures and pterygia at elbows, wrists and knees, in addition to right foot talipes equinovarus deformity. Postnatally the child developed multiple swellings involving both the upper and lower limbs. A plain radiograph revealed the presence of fractures involving the long bones of the upper and lower limbs. A diagnosis of osteogenesis imperfecta with arthrogryposis multiplex congenita was made, and the patient was labeled as a case of Bruck syndrome. The aim of this report is to make the readers aware regarding this rare entity and to specifically look for presence of features suggestive of osteogenesis imperfecta when encountered with a neonate born with arthrogryposis multiplex congenita.
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ranking = 1.9626767539487
keywords = equinovarus, talipes equinovarus, talipes
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3/8. arthrogryposis multiplex congenita--a rare congenital anomaly.

    A 4-year-old female child, known case of arthrogryposis multiplex congenita was posted for bilateral congenital talipes equinovarus (CTEV) correction with unilateral right sided Jess-external fixator. Patient was induced, intubation was possible and maintained with oxygen, nitrous oxide and halothane with non-depolarising muscle relaxant pancuronium bromide. At the end of the surgery patient was reversed and extubated. Recovery was uneventful.
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ranking = 1.9626767539487
keywords = equinovarus, talipes equinovarus, talipes
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4/8. Pelvic dysplasia associated with arthrogrypotic changes in the lower extremities. A new syndrome.

    Two sisters exhibit arthrogrypotic changes in the lower extremities associated with a previously undescribed pelvic dysplasia. The pelvic dysplasia is characterized by: severe abnormalities of ossification with markedly widened triradiate cartilages; irregular, notched acetabulae; marked hypoplasia of the ilia; notching of the iliac wings; and delayed ossification of the capital femoral ossific nucleus. The elder girl has bilateral clubfeet, as well as flexion contractures of both knees. The younger girl has a right talipes equinovarus and left hip dislocation. Autosomal recessive inheritance is suggested. This distinctive syndrome should be considered in the differential diagnosis of the patient with arthrogryposis.
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ranking = 1.9626767539487
keywords = equinovarus, talipes equinovarus, talipes
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5/8. arthrogryposis multiplex congenita occurring with maternal multiple sclerosis.

    All children of a mother with multiple sclerosis (MS) had increasing grades of congenital joint contractures without demonstrable neuromuscular disease. Two had talipes equinovarus, one had congenital hip subluxation, and the youngest had arthrogryposis multiplex congenita. Maternal MS may be causally related to the development of congenital joint contractures.
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ranking = 1.9626767539487
keywords = equinovarus, talipes equinovarus, talipes
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6/8. Separating Pena-Shokeir I syndrome from the "arthrogryposis basket".

    The Pena-Shokeir I syndrome is characterized by prenatal onset of growth deficiency, a specific constellation of facial features, multiple ankyloses, camptodactyly, and talipes equinovarus and is almost invariably fatal. A common and perhaps specific radiographic sign is that of subluxations at interphalangeal joints of the fingers, present in four of six patients described here. This autosomal recessive syndrome should be differentiated from arthrogryposis multiplex congenita on the basis of the unusual facial configuration and early lethality, even when interphalangeal subluxations are absent.
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ranking = 1.9626767539487
keywords = equinovarus, talipes equinovarus, talipes
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7/8. Soft-tissue expander failure in severe equinovarus foot deformity.

    Soft-tissue expanders have recently been reported to be useful in clubfoot surgery. We report our experience with this procedure in five patients with seven severe equinovarus foot deformities. The primary diagnoses of these patients included distal arthrogryposis, cerebro-oculo-facial syndrome, ischemic contracture and spina bifida. Our patients' major complications included ischemia, infection, and sepsis, which resulted in premature removal of the soft-tissue expanders in five of seven clubfeet. Minor complications, such as partial wound dehiscence, occurred in the two successful expansions. Predisposing factors may include soft-tissue expanders with self-contained ports, poor compliance, and extremely tight tissues that limit the subcutaneous space available for expansion, with resultant diminution of the vascular supply to the overlying skin.
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ranking = 5
keywords = equinovarus
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8/8. arthrogryposis multiplex congenita: report of a case of amyoplasia.

    arthrogryposis multiplex congenita is a congenital syndrome characterized by multiple congenital joint contractures; and refers to a large heterogeneous group of disorders. We present a 40 days old male baby who has had multiple fixed contractures of joints since birth. Midline capillary hemangioma, internal rotation of bilateral shoulders, extension contracture of elbows, flexed wrists with pronation deformity, flexion and internal rotation of hips, equinovarus of feet were noted. A series of examinations for differential diagnosis including brain sonogram, echocardiogram, muscle echogram, muscle biopsy, chromosome study were performed and all showed normal findings. Amyoplasia was impressed. Early rehabilitation was arranged. Normal intelligence and a normal life span are expected.
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ranking = 1
keywords = equinovarus
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