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Scarred muscle excision for the treatment of established ischemic contracture of the lower extremity.

Nine patients with rigid equinocavovarus contractures of the foot and ankle after ischemic episodes of the leg were treated from 1986 to 1989. The ischemic contractures occurred after tibial and fibular fractures in six cases, and after a trimalleolar ankle fracture, an electrical burn, and cardiac bypass surgery in one case each. Previous tendon and nerve releases had failed. The scarred portion of the various involved muscles of the deep posterior compartment were widely excised along with the respective tendons. A variety of additional foot procedures were generally necessary to obtain a plantigrade foot. Night splinting to maintain a plantigrade foot was prescribed for all patients.

Adolescent↗

Treatment of severe rigid contractures of cerebral palsied upper limbs.

Flexion contractures caused by cerebral palsy in adult patients can become so rigid and so severe that skin breakdown and infection on the flexor surfaces of the palm and elbow can result. In 11 such patients we have utilized tendon resections, ray amputations, elbow resection arthroplasties and other techniques to correct deformities. Because improvement of hygiene and not functional rehabilitation was the goal in these patients, these aggressive measures could be utilized more freely than usual without fear of jeopardizing the patient's ability to use the hand post-operatively. Treatment of combined intrinsic and extrinsic contracture usually required resection of the sublimi and metacarpal head resection. Thumb-in-palm deformities were difficult to correct and required osteotomies of the first metacarpal or greater multangular resection. Elbow resection was useful in correcting flexion deformities of that joint but deformity recurred unless the biceps tendon was released as part of the procedure.

Cerebral Palsy↗

Familial occurrence of typical and severe lethal congenital contractural arachnodactyly caused by missplicing of exon 34 of fibrillin-2.

Genetic linkage studies have linked congenital contractural arachnodactyly (CCA), a usually mild heritable connective-tissue disorder, to FBN2, the fibrillin gene on chromosome 5. Recently, FBN2 mutations in two patients with CCA have been described. Here we report an A-->T transversion at the -2 position of the consensus acceptor splice site, resulting in the missplicing of exon 34, a calcium-binding epidermal growth factor-like repeat in fibrillin-2 in a mother and daughter with CCA. Significantly, the mother exhibited a classic CCA phenotype with arachnodactyly, joint contractures, and abnormal pinnae, whereas her daughter exhibited a markedly more severe CCA phenotype, which included cardiovascular and gastrointestinal anomalies that led to death in infancy. Analysis of cloned fibroblasts showed that the mother is a somatic mosaic for the exon 34 missplicing mutation, whereas all the daughter's cells harbored the mutation.

Adult↗

Postoperative flexion contracture of the knee: a simple technique for its treatment.

Flexion contracture is not a common problem following knee surgery, but it can be severe enough to jeopardize the success of the operation. With the Beath technique a stove-pipe cast is windowed anteriorly and the cast is padded behind the leg, which is then gently wedged into extension. In this way many flexion contractures can be gently and easily overcome. In addition, the need for serial casting or forcible manipulation is obviated and the overall rehabilitation program is unimpeded. In this paper the authors report two illustrative cases, and discuss the advantages and applications of the Beath technique.

Adolescent↗

[The efficacy of vibration and traction in correcting contractures of the joints (an experimental study)].

The experiments on rabbits with modelled articular contracture established a marked corrective effect of simultaneous exposure of the rabbits to vibration and traction. Clinical trials in patients with traumas of the peripheral nerves complicated by articular contractures provided evidence for high efficacy of combined rehabilitation including vibration and traction methods.

Animals↗

Joshi External Stabilising System (JESS) in proximal interphalangeal joint (PIP) contractures in leprosy.

The authors present their experience in the use of the JESS (Joshi External Stabilising System) for correction of proximal interphalangeal joint (PIP joint) contracture deformity in 68 fingers. The use of the JESS has made this surgery easier, faster in releasing contractures and it has given better correction than the methods so far used by the same authors, like capsulotomy, local flaps and free skin grafting. The procedure is simple and has no serious side effects; it can be repeated if need be. The JESS is easy to apply, economical, reliable, reusable, well accepted by the patient. Compared to the other distractors made in USA and Europe, the JESS has an added advantage in that it costs so very much less (US $5-10), that our leprosy hospitals can afford it. In our patients, we have achieved full extension in 75% and good extension in 10.3% of the cases. These figures are much better than what was possible, in the past, with local flaps and free skin grafting. With those procedures we had excellent results in only 53% of the cases and poor result in 28%.

Adolescent↗

Prenatal diagnosis of a constitutional interstitial deletion of chromosome 5 (q15q31.1) presenting with features of congenital contractural arachnodactyly.

Prenatal diagnosis of a constitutional interstitial deletion of chromosome 5 (q15q31.1) in a 30-year-old woman is reported. At 21 weeks of pregnancy, routine fetal ultrasounds showed the presence of apparently isolated bilateral club feet. Fetal karyotyping documented an interstitial deletion of the long arm of chromosome 5: 46,XX,del(5) (q15q31) in all 50 analyzed metaphases. Because such deletion is associated with severe psychomotor retardation, the pregnancy was terminated. Postmortem karyotyping of skin fibroblasts confirmed the presence of this interstitial de novo deletion in all mitoses. The breakpoints on 5q were analyzed by fluorescent in situ hybridization and were localized at 5q15 and q31.1. This case illustrates the importance of fetal karyotyping in cases of isolated club feet. At autopsy, the fetus presented had minor anomalies and contractures of knee and hip joints. These clinical findings could fit the diagnosis of congenital contractural arachnodactyly (CCA) or Beals syndrome. CCA is caused by a defect in the fibrillin-2 (FBN2) gene. This gene was previously mapped on 5q23-31. Our molecular studies of both parents and the fetus, using an intragenic polymorphic GT repeat, showed that the FBN2 gene was deleted in the fetus and that the de novo interstitial deletion occurred on the paternally inherited chromosome 5. Thus, CCA may be caused by a loss of function of the FBN2 gene. Clinical findings in this fetus and those of other described cases with interstitial 5q deletions are reviewed, and similarities with CCA are stressed.

Abnormalities, Multiple↗

Fibrous contracture of the elbow following cannulation of the antecubital fossa vein.

A case of flexion contracture of the elbow resulting from thrombophlebitis is described. Thrombophlebitis is a recognised complication following cannulation of the antecubital fossa vein. In severe cases a tender fibrous band is palpable. There are no previous reports of a contracture deformity occurring from such a fibrous band. This case therefore presents an unusual clinical manifestation of thrombophlebitis not previously described.

Adult↗

Outcome of surgery for superior oblique palsy with contracture of ipsilateral superior rectus treated by superior rectus recession.

PURPOSE: To report our experience with this special subgroup of patients with superior oblique palsy. SUBJECTS AND METHODS: All six patients seen since 1990 which the senior author treated who had a unilateral superior oblique palsy accompanied by ipsilateral superior rectus muscle contracture. Surgical management included superior rectus recession along with treatment of the superior oblique palsy by one of several appropriate procedures. RESULTS: Five out of six had an "excellent" surgical outcome defined as no diplopia in primary and reading position, elimination of their abnormal head posture and normalization of versions postoperatively. The sixth patient continued to have a small intermittent vertical deviation, but was functionally satisfactory. CONCLUSIONS: This subset of individuals with superior oblique palsy and ipsilateral rectus contracture can be improved with weakening of the ipsilateral superior rectus as part of the surgical plan.

Adult↗

Congenital contracture of the superficial flexor of the hand.

The authors describe a case of an inborn contracture of the superficial flexors of the three-phalangeal fingers in a 15-year-old girl and treatment of the condition. For clinical purposes a classification on congenital deformities in the proximal part of the flexor complex was proposed in which the described case is included. The classification was derived from the ontogenetic development of flexor tendons. There are three types of deformities: S1--the muscular belly of the superficial flexor of the fingers is only in the palm; S2--the muscle is attached to the area between the wrist and medial epicondyle; S3--the palmaris longus of the fingers is on the medial epicondyle, however the m. palmaris longus is lacking; Pl-1 the superficial flexor exists only in the form of a proximal muscular belly of varying length; Pl-2--the palmaris longus is on the wrist and the insertion tendon may be thinner than on the contralateral side. The described contracture deformity was classified as type S2.

Adolescent↗

Ectopia lentis and aortic root dilatation in congenital contractural arachnodactyly.

Congenital contractural arachnodactyly (CCA) was described by Beals and Hecht as an autosomal dominant disorder distinct from Marfan syndrome and comprising joint contractures, arachnodactyly, scoliosis, and a distinct "crumpled ear" deformity. While the disorder is similar to Marfan syndrome, it was split from it due to the distinct physical appearance of the patients and, more importantly, the lack of heart and eye findings. Since the original report, several CCA patients have been found to have mitral valve prolapse, structural cardiac anomalies, and occasionally aortic root dilatations similar to those seen in Marfan syndrome. We report on a patient with CCA with bilateral ectopia lentis and aortic root dilatation. Our review of the literature of CCA showed that serial echocardiography and careful eye examinations have not become a standard of medical practice in this condition. Partly this may be due to a lack of documented cases of CCA having severe ectopia lentis and cardiac complications. This patient underscores the need for periodic eye and echocardiographic evaluations of all CCA patients.

Aortic Aneurysm↗

The temperature dependence of halothane-induced contractures of skeletal muscle.

Frog muscles bathed in solutions equilibrated with 0.5%-2.0% halothane develop large contractures when cooled to 2-4 degrees C. These halothane-cooling contractures (HCC) are dose dependent and reproducible; they are potentiated by lidocaine (5 mM), abolished by procaine (5 mM), and partially inhibited by procainamide (5 mM) or sodium salicylate (2 mM). The HCC provides a simple model system for investigating the effects of low halothane concentrations on Ca homeostasis and contractility in skeletal muscle.

Anesthetics, Local↗

Teratogens associated with congenital contractures in humans and in animals.

An evaluation of over 350 patients in a study of congenital contractures of the joints (arthrogryposis) included a review of family, pregnancy, and delivery histories for teratogenic exposures. Fifteen out of the total 350 patients studied had a possible teratogenic exposure: an infectious agent (viral or bacterial), maternal drug or toxin ingestion, chronic maternal neurologic or muscular illness, or a direct physical insult such as a structural uterine anomaly. Literature was reviewed for all human and animal cases reported with congenital contractures of the joints with an associated teratogenic insult. Those findings are discussed here.

Animals↗

Procaine enhancement of the rapid cooling contracture and inhibition of the decay of potentiated state in rabbit papillary muscle.

We tested the possibility that inhibition of Ca2+ release from the sarcoplasmic reticulum decreases the transsarcolemmal Ca2+ efflux and results in an increased sarcoplasmic reticulum Ca2+ content. We used procaine to inhibit sarcoplasmic reticulum Ca2+ release in rabbit papillary muscles. Rapid cooling-induced contractures were used as an index of sarcoplasmic reticulum Ca2+ content. Decay of potentiated state was measured to study whether the transsarcolemmal Ca2+ efflux is decreased in the presence of procaine. Procaine (0.5, 1.0 mM) inhibited contractions elicited by electrical stimulation and enhanced the rapid cooling-induced contracture. The initial decay of potentiated state that occurs when the muscle was stimulated at a low frequency was slowed in the presence of procaine. These results suggest that procaine inhibition of Ca(2+)-induced release of sarcoplasmic reticulum Ca2+ decreases the transsarcolemmal Ca2+ efflux relative to the influx, resulting in an increased sarcoplasmic reticulum Ca2+ content.

Animals↗

Hypoxia, calcium, and contracture as mediators of myocardial enzyme release.

During calcium-free perfusion, anoxic contracture of myocardial cells causes cells to separate at intercalated disks and leads to an energy-independent enzyme release in the absence of active transmembrane calcium fluxes. It is proposed that contracture mediates membrane damage and enzyme release in cells sensitized to the calcium paradox.

Animals↗

Ischaemic contracture and myocardial perfusion in isolated rat heart.

The development of left ventricular contracture and myocardial perfusion defect was studied in isolated rat hearts during global ischaemia of 90 min duration. The left ventricular pressure was measured by a balloon catheter inserted into the ventricle and filled with water. The pressure reached the maximum at 16 min of ischaemia. The left ventricular volume and compliance (passive distensibility) were measured by the same balloon, the former by connecting the balloon to an open catheter and the latter by applying a constant additional volume (0.020 ml) into the balloon. the left ventricular volume and compliance both decreased progressively for 20 min of ischaemia after which they remained low for the rest of the observation period (90 min). The myocardial perfusability was tested by infusing 0.1 per cent sodium fluorescein in isotonic saline into the cannulated aortic root of the isolated heart preparation. The percentage perfused with the fluorescent tracer in horizontal frozen myocardial sections was estimated by point counting from colour photogragraphs taken under ultraviolet light. The proportion of the perfused area decreased gradually from 100% at 0 min of ischaemia to 93, 67, 43 and 37% at 15, 30, 60 and 90 min of ischaemia, respectively. It was concluded that ischaemic contracture of the left ventricle is followed by the development of a myocardial perfusion defect in isolated ischaemic rat heart.

Animals↗

Neonatal nemaline myopathy presenting with multiple joint contractures.

A sporadic case of the rare and most severe neonatal form of nemaline myopathy is reported. Intrauterine manifestation included reduced fetal movements and breech position with a normal amount of amniotic fluid. After delivery by Caesarian section at 34 weeks of gestation the infant boy, who was not asphyctic, failed to establish spontaneous breathing and required immediate intubation and ventilation. Marked muscular hypotonia and weakness persisted and reflexes remained absent. Hip dislocation, joint contractures, absent palmar creases, prominent lateral palatal ridges and cryptorchidism were interpreted as consequent to prenatal paralysis. The boy died after 5 h due to hyaline membrane disease and meconium aspiration. At autopsy the skeletal muscles were found to be hypoplastic. The muscle fibres contained numerous rods, a typical finding of nemaline myopathy. Nemaline myopathy should be considered in fetuses and newborns with multiple joint contractures, severe muscular weakness and respiratory insufficiency.

Arthrogryposis↗