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Since 1973 we have increasingly performed Dupuytren's contracture as an outpatient procedure. The results are the same as after hopital care. No prolongation of the out of work time.
We report our experience in the treatment of 39 patients with Dupuytrens' contracture using the "open-palm" technique (McCASH). In this method the palmar fascia is excised through a transverse incision in the distal plamer crease. The wound is left open. The advantages of this method are: no necrosis of the skin, absence of haematomas, closure of other incisions without tension, early motion with little postoperative discomfort. Based on our experience we recommend this method for a selected group of patients.
We describe a family in which 2 sisters born to consanguineous parents developed childhood onset fibrosing pleuritis in association with constrictive pericarditis and bilateral deforming arthropathy of large and small joints of upper and lower extremities including flexion contractures of several fingers (camptodactyly) and toes. One patient also had mitral value prolapse. Histopathological examination of the synovium and pericardium revealed fibrosis, and ultrastructural study of synovium showed abundant inter and intracellular mesh of 9 nm microfibrils. We describe this distinct clinicopathological entity with pleiotropic manifestations, the common features of which appears to be the fibrosis of serous membranes. Therefore, the term "familial fibrosing serositis" is proposed for this entity.
An operation was performed in an 8-years-old girl suffering from a recessive dystrophic epidermolysis bullosa from birth complicated by a developed contracture and syndactyly of the both bones in order to eliminate these defects without using an autotransplant. The postoperative course was without complications, epithelialization developed by the end of the 3rd week. No recurrences were noted during 2 years.
Arthritic knee conditions leading to deformities of the anterior and posterior aspects of the knee joint require careful preoperative planning. Constrained condylar implants should be available in the operating room if asymmetrical soft-tissue laxity is produced as the deformities are corrected. The operative approach must be aggressive, and soft-tissue releases often have to be quite extensive to correct severe extension and flexion contractures. Soft tissues should never be released transversely but always in a subperiosteal fashion from the underlying bone. In this fashion, the implant will block open these soft-tissue cavities symmetrically and knee stability will be restored. Asymmetric soft-tissue imbalance from varus or valgus deformity must be corrected in combination with anterior and posterior soft-tissue management. Rehabilitation of these complex knees requires individualization and careful progression of motion and unsupported ambulation. Care must be taken to prevent wound problems, and rapid intervention is necessary should they occur. If the technical and rehabilitative aspects of these severe knee deformities are carried out successfully, excellent function is possible in this disabled group of patients.
We describe a patient who was born with flexion contractures and pterygia at the elbows, clubfeet, torticollis, and several rib fractures. During infancy and childhood, multiple fractures of the lower limbs occurred with minimal trauma and led to disabling deformities. When evaluated at age 19 years, he was normally intelligent, but extremely short, with severe kyphoscoliosis compromising his pulmonary function. Pterygia limited elbow extension to 90 degrees, and severe lower limb deformities prevented ambulation. He did not have blue sclerae, dentinogenesis imperfecta, or hearing loss. X-ray studies showed demineralized bones, severe deformity and cystic change at old fracture sites, and vertebral wedging. Collagen studies on skin fibroblasts were normal.
A 32-year-old male with progressive systemic sclerosis developed shoulder abduction contractures secondary to fibrous replacement of the central portions of his deltoid muscles. Surgical release of the fibrous bands resulted in prompt restoration of nearly full shoulder motion. One month later, however, a similar band developed in the triceps muscle. Although the association in this cases may have been coincidental, the neraly simultaneous onset of muscle fibrosis and cutaneous manifestations of progressive systemic sclerosis suggests a causal relationship.
It has been reported that the skin of the palm is supplied by arteries receiving their blood from a radial, thenar, ulnar, or hypothenar arterial system. Interestingly, the middle of the palm has been described as hypovascularized, but detailed informations on number and distribution of blood vessels are not available. By surgical treatment of the Dupuytrens contracture we could show that preservation of the blood vessels and nerves supplying the skin leads to excellent postoperative results. However, our intraoperative observations do not correspond to the picture of a poorly vascularized region of skin in the middle of the hand. During surgical treatment we found between six and nine bundles consisting of one artery, at least one vene, and one nerve in each case. The bundles perforate the palmar aponeurosis through channels, and subaponeurotically a lot of them originate from further proximal than expected. This information should be taken into consideration during the exstirpation of the palmar aponeurosis.
A strain injury to the infraspinatus muscle was a potential cause of forelimb lameness in a dog. Ultrasonography was used for evaluation of infraspinatus muscle injury. The ultrasonographic appearance of a typical infraspinatus muscle, contracture of an infraspinatus muscle, acute strain of an infraspinatus muscle, and progression of healing of the infraspinatus muscle are described.
Bruck syndrome manifests with combined features of arthrogryposis and osteogenesis imperfecta. It is a distinct autosomal recessive disorder with normal collagen I. The main features are osteoporosis, bowing of the long bones, scoliosis due to vertebral deformities, and congenital joint contractures. The presence of arthrogryposis differentiates this syndrome from "classical" osteogenesis imperfecta. A family with three affected children is presented with a review of the literature.
The clinical appearance of hamstring shortening in 10 patients referred with diagnosis of asymmetry of the trunk, scoliosis, postural defect, strange gait, and back pain is reported. Additionally, an assessment of knee extension deficit (KED) angle in 575 healthy children (4-15 years of age) was performed. The mean KED angle in the group of healthy children was 35 degrees (SD = 14 degrees). The border between norm and disease was defined as a mean + 2 SD and equaled 63 degrees. A KED angle greater than 60 degrees occurred in 10% of normal children. There was no correlation between age and a value of KED angle. In the study group, KED angle greater than 40 degrees was more frequent in boys. A correlation between disorders within musculoskeletal system and greater KED angle was found. These disorders were more frequent in children with asymmetrical KED angle. In our opinion, the border value of the KED angle is not a good differentiating factor between hamstring shortening and contracture because it does not explain the cause of the disorder.
Congenital contractural arachnodactyly (CCA) is an autosomal dominant condition phenotypically related to Marfan syndrome (MFS). CCA is caused by mutations in FBN2, whereas MFS results from mutations in FBN1. FBN2 mRNA extracted from 12 unrelated CCA patient cell strains was screened for mutations, and FBN2 mutations were identified in six of these samples. All of the identified FBN2 mutations cluster in a limited region of the gene, a region where mutations in FBN1 produce the severe, congenital form of MFS (so-called neonatal MFS). Furthermore, three of the identified mutations occur in the FBN2 locations exactly corresponding to FBN1 mutations that have been reported in cases of neonatal MFS. These mutations indicate that this central region of both of the fibrillins plays a critical role in human embryogenesis. The limited region of FBN2 that can be mutated to cause CCA may also help to explain the rarity of CCA compared to MFS.
In the operative treatment of Dupuytren's contracture stage II to IV according to Iselin and Dieckmann (1951), we often prefer the open-palm technique by McCash (1964). In the past ten years, we have treated 1461 cases of Dupuytren's disease. With the assistance of a health insurance company, we followed up 214 cases. When compared to those cases treated with primary wound closure, there was no difference in regard to return to work time.
BACKGROUND AND OBJECTIVE: Reports in the literature indicate that low energy laser irradiation has a biostimulatory effect on wound healing; however, no mechanism of this effect has been elucidated. STUDY DESIGN/MATERIALS AND METHODS: We attempted to establish a model from which to study the mechanism of biostimulation. The effects of low energy helium-neon irradiation on wound healing were observed in two rat models. In the first model, 1.5 cm diameter full thickness excisional skin defects were created in the dorsal midline of rats (n = 32). All animals were anesthetized and all eschars were debrided daily. Wound area was determined by caliper measurements for 2 weeks postoperatively. Rats that received a treatment of 1 J/cm2 had two defects in the dorsal skin. One wound was treated and the second was used as its own control. These measurements were not blinded. Rats that received 2 J/cm2, 4 J/cm2, or anesthesia alone had one defect on the dorsal skin. Caliper measurements of these wounds were blinded. We were unable to demonstrate any difference in the rate of wound contracture in rats that received a daily dose of 1 J/cm2, 2 J/cm2, 4 J/cm2, or anesthesia alone (P > 0.8 by student's t-test). In the second model, a single 2 cm longitudinal full thickness skin incision was created in the dorsal midline of each rat (n = 24). No difference was found between rats that received anesthesia alone and those treated daily with 2 J/cm2 as assessed by tensile strength measurements on postoperative days 7 and 14 (P > 0.8 by student's t-test between groups at both time points). These determinations were blinded. RESULTS: Despite our intentions of studying the mechanism of low energy HeNe biostimulation, we were unable to demonstrate a beneficial effect. CONCLUSION: In this study, helium-neon laser irradiation produced no measurable benefit on wound healing.
Three generations of a kindred had a history and physical findings consistent with congenital contractural arachnodactyly (CCA) segregating in an autosomal-dominant manner. Six of the seven affected patients we examined had mitral valve prolapse (MVP) diagnosed clinically or by echocardiography. The family members without CCA did not have MVP. This association of cardiac involvement with CCA further lessens the distinction between CCA and the Marfan syndrome. The indication for ophthamologic and echocardiographic follow-up of patients carrying the diagnosis of CCA is stressed.
We describe two sibs with postnatal-onset growth deficiency, microcephaly, cataract, prominent supraorbital ridge, large joint contractures, severe osteoporosis, cortical dysplasia, cerebellar atrophy, and mental retardation. The combination appears to constitute a previously undescribed syndrome inherited in an autosomal recessive pattern.
OBJECTIVE: To investigate muscle function in patients with severe myalgia resulting from fluoroquinolone (FQ) treatment. We used histology, in vitro contracture tests (IVCTs), and (31)P magnetic resonance spectroscopy ((31)P MRS) to explore muscle contraction and metabolism. METHODS: We studied 3 patients with myalgia, hyperalgia tendinopathy, and arthralgia following FQ treatment and 3 normal subjects after taking FQs. Results were compared with those of a control group of 9 subjects free of any muscle disease and not taking FQs. Muscle biopsies were performed on the left biceps, and IVCTs were performed in accordance with the protocol recommended by the European Malignant Hyperthermia Group. (31)P MR spectra of forearm flexor muscles were recorded at 4.7T throughout a rest-exercise-recovery protocol. RESULTS: (31)P MRS showed a significant reduction of pH changes measured at the end of exercise and a faster rate of proton efflux measured during recovery in all patients. IVCTs diagnosed 1 patient as being susceptible to malignant hyperthermia. No specific histologic anomalies were observed in muscle biopsy samples, which showed normal mitochondria. CONCLUSION: The adverse effects recorded in the 3 patients are related to a preexisting muscular anomaly revealed by FQ treatment.
Congenital contractural arachnodactyly (CCA) is an autosomal dominant condition that shares skeletal features with Marfan syndrome (MFS), but does not have the ocular and cardiovascular complications that characterize MFS. CCA and MFS result from mutations in highly similar genes, FBN2 and FBN1, respectively. All the identified CCA mutations in FBN2 cluster in a limited region similar to where severe MFS mutations cluster in FBN1, specifically between exons 23 and 34. We screened exons 22 through 36 of FBN2 for mutations in 13 patients with classic CCA by single stranded conformational polymorphism analysis (SSCP) and then by direct sequencing. We successfully identified 10 novel mutations in this critical region of FBN2 in these patients, indicating a mutation detection rate of 75% in this limited region. Interestingly, none of these identified FBN2 mutations alter amino acids in the calcium binding consensus sequence in the EGF-like domains, whereas many of the FBN1 mutations alter the consensus sequence. Furthermore, analysis of the clinical data of the CCA patients with characterized FBN2 mutation indicate that CCA patients have aortic root dilatation and the vast majority lack evidence of congenital heart disease. These studies have implications for our understanding of the molecular basis of CCA, along with the diagnosis and genetic counseling of CCA patients.