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PubMedDate not supplied

[Duchenne muscular dystrophy--contracture preventive operations of the lower extremities with special reference to anesthesiologic aspects].

So far there is no causal treatment for Duchenne muscular dystrophy up to now, it has been proven, however, that its course can be considerably improved by an early contracture-prophylactic operation of both lower limbs--mostly between age 4 and 6 years--as well as by a surgical stabilization of the spine before any progressive scoliosis appears, that is at the very beginning of the wheel-chair stage: Walking and standing ability can be prolonged for several years and a significant scoliosis can be avoided. A decisive prolongation of life can be achieved by treating the fatal respiratory insufficiency with timely started mechanical ventilation. Our first experiences have shown, that there is no proven justification for a reserved attitude against early lower limb surgery in view of modern anaesthesia. Malignant hyperthermia-reaction and hyperkalaemia are the known anaesthetic rise factors of operations in Duchenne muscular dystrophy. However, we could exclude virtually these rises by choosing the right anaesthesia and by a comprehensive monitoring routine. If need be, malignant hyperthermia can be treated effectively by using the obligatory antidote (Dantrolene). In view of an expected causal treatment in future, early detection of Duchenne muscular dystrophy by newborn screening ("CK-Test") as well as a comprehensive stage-depending treatment programme (early surgery/mechanical ventilation) are nowadays of outstanding importance.

Anesthesia, General↗
PubMedDate not supplied

[Elbow contracture caused by increased tonus of the flexor muscles].

Increased muscle tone leads occasionally to fixed flexion and supination deformities of the elbow in tetraplegics and subsequently to additional restriction of function. The incidence, course, remaining functions and therapeutic measures were studied retrospectively in 22 patients (37 arms). Muscle hypertonus was found to start during the first 12 weeks after spinal cord lesion and to provoke a flexion contracture of 35 to 95 degrees in 40% of the cases despite therapy. Due to the failure of physical therapy and operative measures, prophylaxis through the prevention of shoulder-pain, early use of adequate splinting of the upper extremity and electrical stimulation of the triceps brachii should be carried out.

Adult↗
PubMed1993

Hypnotherapy and contractures of the hand.

Contractures of the hand are rather uncommon conversion phenomena. As yet, very little is known about treatment. After a brief overview of the literature on the various treatment strategies, we describe the treatment of four patients. There are indications that a complaint-centered approach, which attempts to influence the symptom directly, produces positive results. Hypnotherapeutic techniques were used in the four patients described here. Treatment resulted in improvement in three of the four.

Adult↗
PubMed2002

The vegetative and minimally conscious states in children: spasticity, muscle contracture and issues for physiotherapy treatment.

The neuropathology of the vegetative (VS) and minimally conscious (MCS) states and the pathophysiology of spasticity are reviewed. Current treatment options available in the physical management of children in a low-level state and factors influencing the physiotherapy treatment of children in a low-level state will be discussed. The complex neuropathology of VS and MCS helps to explain the varied clinical presentations of children in VS and MCS. Spasticity and muscle contracture are common motor sequelae of VS and MCS. Loss of inhibition by descending motor pathways is thought to result in increased muscle tone or spasticity. However, secondary changes in muscle fibre structure and periarticular connective tissue may be an additional component to increased muscle tone. A multimodal approach combining physical, pharmacological and surgical interventions is likely to be the most effective. Knowledge of the likelihood of recovery from VS and MCS can be helpful in determining the frequency and intensity of physiotherapy. Ethical issues in the management of children in a low-level state include a consideration of the benefits to the child and the child's family and the costs to the health care team and the medical institution.

Adolescent↗
PubMed2000

Results after surgery for severe Dupuytren's contracture: does a dynamic extension splint influence outcome?

Seventy-six consecutive patients were operated on for advanced Dupuytren's contracture and the results evaluated after nine months with special reference to the use of a dynamic extension splint. The patients were separated into three groups: those in whom the splint was used according to our guidelines (n = 15); those in whom the splint was used, but inadequately (n = 15); and those who did not require splinting (n = 24). Our results nine months postoperatively were similar to those of other studies in showing that the fifth proximal interphalangeal joint constituted the greatest problem. Comparison of the three groups indicated that splinting the way we used it did not influence the natural course of the disease after operation.

Dupuytren Contracture↗
PubMed2003

Activation markers of connective tissue in Dupuytren's contracture: relation to postoperative outcome.

We investigated 103 consecutive patients operated on for Dupuytren's contracture (DC) to find out the relation between the expression of activation markers of connective tissue in surgical specimens obtained prospectively and recurrence of disease. The history of the disease and present state of the operated hand were obtained a mean of 4 years (range 2.5-6) after the latest operation. Immunohistochemical staining for anticollagen type IV, integrin alpha5, laminin, smooth muscle beta-actin, procollagen type I, and desmin was evaluated. Almost half of the patients noticed recurrences during the study period, one fifth within six months of operation. No differences in the expression of any of the markers investigated were found, either earlier or later than six months postoperatively, in patients with or without recurrent bending. Furthermore, there were no associations between sex, age at onset, number of operations, heredity, diabetes mellitus, or drugs taken for cardiovascular disease, and the expression of any of the immunohistochemical markers. The individual characteristics that place a person at high risk are not obviously related to ongoing production of connective tissue at the time of operation or to connective tissue activity in its conventionally-used sense.

Adult↗
PubMed1998

Assignment of the disease locus for lethal congenital contracture syndrome to a restricted region of chromosome 9q34, by genome scan using five affected individuals.

Lethal congenital contracture syndrome (LCCS) is an autosomal recessive disease leading to death before the 32d gestational week. It is characterized by the fetal akinesia phenotype, with highly focused degeneration of motoneurons in the spinal cord as the main neuropathological finding. We report here the assignment of the LCCS locus to a defined region of chromosome 9q34, between markers D9S1825 and D9S1830. The initial genome scan was performed with the DNA samples of only five affected individuals from two unrelated LCCS families. The conventional linkage analysis performed with 20 affected individuals and their families was focused on those chromosomal regions in which the affected siblings were identical by descent in the initial scan. One core haplotype of 3 cM was observed in LCCS alleles, supporting the assumption of one major mutation underlying LCCS, and linkage disequilibrium analysis restricted the critical chromosomal region to <100 kb in the vicinity of marker D9S61. Two genes, NGAL (neutrophil gelatinase-associated lipocalin and NOTCH 1, were excluded as causative genes for LCCS

Chromosome Mapping↗
PubMed1997

A rare branch-point mutation is associated with missplicing of fibrillin-2 in a large family with congenital contractural arachnodactyly.

Congenital contractural arachnodactyly (CCA) is an autosomal dominant disorder that is phenotypically similar to but genetically distinct from Marfan syndrome. Genetic-linkage analysis has implicated the fibrillin-2 gene (FBN2) as the CCA locus. Mutation analysis of two isolated CCA patients revealed missense mutations, indicating that defects in FBN2 may be responsible for this disorder. However, cosegregation of a mutant allele with the disease phenotype has not yet been established. We have investigated the primary cause of CCA in a large well-characterized kindred with five generations comprising 18 affected individuals. Previous studies demonstrated linkage of this family's CCA phenotype to FBN2. Mutation analysis of cDNA derived from the proband and her affected brother, using a nonisotopic RNase cleavage assay, revealed the partial skipping of exon 31. Approximately 25% mutant transcript is produced, which is apparently sufficient to cause a CCA phenotype. Sequence analysis of genomic DNA revealed an unusual base composition for intron 30 and identified the mutation, a g-26t transversion, in the vicinity of the splicing branch-point site in intron 30. Genomic DNA from 30 additional family members, both affected and unaffected, then was analyzed for the mutation. The results clearly demonstrate cosegregation of the branch-point mutation with the CCA phenotype. This is the first report of a CCA mutation in a multiplex family, unequivocally establishing that mutation in FBN2 are responsible for the CCA phenotype. In addition, branch-point mutations only very rarely have been associated with human disease, suggesting that the unusual composition of this intron influences splicing stability.

Adult↗
PubMed1989

Toe-walking in children with cerebral palsy: contributions of contracture and excessive contraction of triceps surae muscle.

The study was designed to provide a quantitative analysis of toe-walking in children with cerebral palsy (CP). The total internal moment developed about the ankle joint during locomotion and the passive component of this internal moment were measured. The contributions of the active and passive components were expressed as the ratio (R) between the passive moment and the total internal moment. Measurements were compared for 13 children with CP and 5 healthy children. For the data analysis, the children with CP, exhibiting apparently similar toe-walking, were divided into two groups: 1) Group CPI and 2) Group CPII. Group CPI was characterized by a small ratio R value, which indicated the presence of excessive contractions of the triceps surae muscle during locomotion. In Group CPII, the ratio R value was abnormally high, which indicated that a contracture (ie, structural change of the muscle or the tendon) was entirely or at least partly responsible for toe-walking. Each group requires a different therapeutic strategy.

Adolescent↗
PubMed1993

Knee flexion contractures in institutionalized elderly: prevalence, severity, stability, and related variables.

BACKGROUND AND PURPOSE: The purpose of this study was to document the prevalence, severity, and progression of knee flexion contractures (KFCs) in a population of institutionalized elderly and to identify relationships between knee extension and other variables. SUBJECTS: The subjects were 112 nursing home residents who exhibited a broad range of ambulation and cognitive function abilities. METHODS: Data were collected initially (T1) and after a 10-month period (T2). RESULTS: Only 25% of the population had extension in the 0- to 5-degree (lacking full extension) range bilaterally at T1 and T2, leaving the majority of subjects with some degree of unilateral KFC. Most of the subjects with a KFC greater than 20 degrees were nonambulatory and had a significantly higher occurrence of resistance to motion than did nonambulators with a KFC of less than 20 degrees. Knee extension measurements did not change in most subjects between T1 and T2. The knees that did show a change in KFC (either an increase or a decrease) had a significantly higher occurrence of resistance to passive motion than did other knees. Compared with the subjects who gained extension, the subjects who lost extension over the study period more frequently had minimal KFC at T1, were ambulatory at T1, showed a regression in ambulation at T2, and developed resistance to motion at T2. CONCLUSION AND DISCUSSION: The data confirmed significant positive correlations between degree of KFC and presence of resistance to passive knee motion, cognitive impairment, impaired ambulation, and presence of knee pain. Physical therapy assessment and intervention may be appropriate in nonambulatory nursing home residents with resistance to passive motion, residents with KFC approaching 20 degrees, and ambulatory residents with minimal KFC who develop resistance and begin to regress in ambulation. Several areas for future study are suggested.

Aged↗
PubMed1996

Treatment of a knee contracture using a knee orthosis incorporating stress-relaxation techniques.

The subject of this case report was a 67-year-old Caucasian woman who had undergone total knee arthroplasty of the right knee. Postoperative physical therapy and a new orthosis that utilizes principles of stress relaxation (constant displacement) and static progressive stretching were used to reestablish range of motion. The total treatment time (cumulative orthosis wear time) was 32.5 hours over a period of 29 days, and the patient obtained a 17-degree increase in active range of motion. Six months later there was no measurable loss in range of motion. The results of the treatment may be beneficial for some patients. Researchers and clinicians need to conduct studies to further evaluate this approach to knee contracture management.

Aged↗
PubMed1981

The treatment of Dupuytren's contracture with flexor tendon sheath involvement-the sliding volar flap.

When the flexor tendon sheath must be opened to release a Dupuytren's contracture of the finger, one is sometimes left with an exposed flexor tendon. If the initial incisions in the finger are made to create a proximally based flap, adequate skin is left to cover the defect in the flexor tendon sheath and a split-thickness skin graft can be placed distally where the sheath has not been violated.

Dupuytren Contracture↗
PubMed1983

The release of flexion contractures as a prerequisite for the treatment of pressure sores in multiple sclerosis: a report of ten cases.

Ten patients with multiple sclerosis who had severe flexion contractures of the lower extremity ranging from 120 to 180 degrees were treated for large pressure sores. Each patient had at least one pressure sore in the sacral, greater trochanteric, or ischial areas. It was necessary to perform a tendon release procedure of the knee joint prior to addressing the problem of the pressure sores. Prior to treatment all patients were unable to utilize a wheelchair owing to their condition. Subsequent treatment, including development of skin flaps and skin grafts, postoperative casting, and immobilization in a special lamb's wool sling, allowed all 10 patients to improve to the point where they could utilize a wheelchair, with the result that their self-images improved.

Contracture↗
PubMed1992

Plantar fibromatosis and bilateral flexion contractures: a review of the literature.

Plantar fibromatosis is a benign multinodular lesion occurring in the plantar fascia. It is generally an asymptomatic lesion or may occasionally cause discomfort or pain in the foot. We present a man who has bilateral flexion contractures of his great toes as a result of his plantar fibromatosis. A review of the literature indicates that this is a very rare presentation of plantar fibromatosis.

Aged↗
PubMed1993

The triceps muscle flap for axillary contracture release.

Previously overlooked, the concept of a triceps muscle flap has been recently proven valid. By using only the long head portion, necessary function preservation may be achieved. A distinct proximal neurovascular source to this component alone allows an axis of rotation at the level of the teres major muscle. This permits transposition either in an anterior or posterior direction as a local muscle flap that has usefulness, albeit in a secondary role, for axillary reconstruction such as commonly required as a sequela to burn contracture.

Adolescent↗
PubMedDate not supplied

Uncommon causes of anterior knee pain: a case report of infrapatellar contracture syndrome.

The uncommon causes of anterior knee pain should always be considered in the differential diagnosis of a painful knee when treatment of common origins become ineffective. A case is presented in which the revised diagnosis of infrapatellar contracture syndrome was made after noting delayed progress in the rehabilitation of an active female patient with a presumed anterior horn medial meniscus tear and a contracted patellar tendon. The patient improved after the treatment program was augmented with closed manipulation under arthroscopy and infrapatellar injection of both corticosteroids and a local anesthetic. Infrapatellar contraction syndrome and other uncommon sources of anterior knee pain, including arthrofibrosis, Hoffa's syndrome, tibial collateral ligament bursitis, saphenous nerve palsy, isolated ganglions of the anterior cruciate ligament, slipped capital femoral epiphysis, and knee tumors, are subsequently discussed. Delayed functional advancement in a rehabilitation program requires full reassessment of the patient's diagnosis and treatment plan. Alternative diagnoses of knee pain are not always of common origins. Ample knowledge of uncommon causes of anterior knee pain is necessary to form a full differential diagnosis in patients with challenging presentations.

Adult↗
PubMedDate not supplied

Gluteal pouching: a complication of perineal burn scar contracture. A case report.

Burns to the buttocks are rare. Gluteal pouching, a complication of burn scar contractures of the buttocks, with total effacement of the gluteal folds and hooding of the rectum is described. A barium enema is helpful for preoperative evaluation. Recreation of the gluteal folds and unhooding of the rectum can be performed effectively without skin grafting.

Burns↗