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Treatment of spastic diplegia in patients with cerebral palsy.

Appropriate treatment, which includes orthopaedic surgery, physical and occupational therapy, recreational therapy, orthotics, and utilization of assistive devices, will improve the functional outcomes of children with cerebral palsy. Medical modalities such as intramuscular injections of botulinum toxin, and constant intrathecal administration of Baclofen via an implanted pump may also be of benefit. There is a defined set of orthopaedic surgical procedures that can enhance function, and the challenge for the surgeon is to identify which combination of procedures is appropriate for each individual patient and at what point during development to implement them. Some surgeons prefer to wait until patients are older (8-10 years) and perform all of their surgical interventions in one sitting. We, however, favor a different approach wherein surgical procedures are done as indicated during childhood development to enhance function and allow further improvement of motor skills. We refer to this approach as 'Staged Multilevel Interventions in the Lower Extremity' or 'SMILE'. This paper will discuss the rationale for this approach and our recommendations regarding the indications and timing of surgical interventions, as well as techniques and outcomes as reported in the literature.

Cerebral Palsy↗

Spondylolisthesis in myelomeningocele.

Radiographic spinal evaluations were done on 305 patients with myelodysplasia. Asymptomatic spondylolisthesis, which averaged 37.1% translation, was seen in 5.9% of the patients. The prevalence of spondylolisthesis increased to 15.6% of patients with L5-S1 motor levels. Patients with myelodysplasia do develop spondylolisthesis. Increased lumbar lordosis and concomitant diminished thoracic kyphosis were commonly seen in the patients with spondylolisthesis.

Adolescent↗

Crohn's disease penetrating into the spinal canal.

An 11-year-old boy who had suffered from Crohn's disease of the large bowel for 4 years developed high fever, severe back pain and flexion-contracture of the right hip joint with inability to walk. After several weeks radicular irritation also became apparent. CT-scan demonstrated an abscess of the right psoas muscle that had also infiltrated the spinal canal, leading to a spinal extradural abscess extending from L2-S4. Therapy comprised surgical drainage of the psoas abscess and conservative therapy (mainly steroids and hypercaloric diet) and resulted in complete cure of the psoas and the spinal abscess. Spinal infiltration is a rare and serious complication of Crohn's disease and must be considered in every case of significant back pain with or without obvious neurological signs.

Abscess↗

Neonatal Marfan syndrome caused by an exon 25 mutation of the fibrillin-1 gene.

Neonatal Marfan syndrome caused by an exon 25 mutation of the Fibrillin-1 gene: We describe a male infant with severe arachnodactyly, hypermobility of the fingers, flexion contractures of elbows, wrists, hips, and knees, microretrognathia, crumpled ears, rockerbottom feet, loose redundant skin, and lens dislocations. Cardiac valve insufficiency and aortic dilatation resulted in cardiac failure, decompensated with digitalisation and death occurred at the age of 4 months. This case represents the severe end of the clinical spectrum of Marfan syndrome, namely neonatal Marfan syndrome. Molecular diagnostic analyses confirmed a de novo exon 25 mutation in the FBN1 gene.

Abnormalities, Multiple↗

Juvenile chronic arthritis.

Juvenile chronic arthritis is a heterogenous group of diseases in which the common denominator is a persistent arthritis in one or more joints commencing before the age of 16 and lasting three months or more. The care of these patients by a pediatric rheumatologist, an orthopedic surgeon, and a full rehabilitation team must be combined to afford the best treatment. Medication, splintage, and physiotherapy are useful in helping to suppress and reduce the effects of the disease, but in some patients arthritis attacks in acute episodes, while in others arthritis chronically and relentlessly pursues its destructive course. Surgical treatment, particularly of the soft parts, has a considerable part to play in correcting deformity, maintaining movement, and relieving pain. Timely surgical treatment may also serve to prevent deformity and to maintain function as well as muscle strength so that by the time the child becomes an adult he can lead a normal life. The plan of treatment is thus long range.

Arthritis, Juvenile↗