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[Hauptmann-Thannhauser muscular dystrophy and differential diagnosis of myopathies associated with contractures].

Hauptmann-Thannhauser muscular dystrophy is characterized by the clinical triad of early-onset contractures of elbow, Achilles tendons, and cervical spine, slowly progressive humeroperoneal muscle wasting and weakness, and life-threatening cardiac involvement with conduction blocks manifesting in the third decade. Hauptmann-Thannhauser muscular dystrophy is due to mutations in the LMNA gene affecting the nuclear envelope proteins lamin A and C. We present a 16-year-old German boy with typical muscular involvement and contractures and typical course of Hauptmann-Thannhauser muscular dystrophy due to the novel missense mutation R401C. The data of this family suggest a lower penetrance of muscular and especially cardiac symptoms than expected. Autosomal-dominant Hauptmann-Thannhauser muscular dystrophy and X-chromosomal Emery-Dreifuss muscular dystrophy are not clearly distinguishable by phenotypic criteria. Other muscular diseases associated with contractures and congenital or childhood onset are reviewed.

Adolescent↗

External ultrasonic treatment of capsular contractures in breast implants.

The authors report their experience on the nonsurgical treatment of capsular contractures due to breast implant augmentation mammaplasty. External ultrasonic repeated applications have been applied to 24 patients after closed capsulotomy procedures in order to reduce the recurrency rate. The new ultrasonic device used was based on a 2-MHz generator with a timing adjustable power emission connected to eight transducers designed for breast anatomy. The authors report significant improvement of the closed capsulotomy technique demonstrating a persistent stability of the achieved results in 82% of the treated contractures, even in severe cases (Baker's IV), after a minimum follow-up period of 12 months. Methods of application, technical features of the ultrasonic device, experimental charts, and results obtained on 34 breast implant capsular contractures are reported and discussed.

Adult↗

Eosinophilic fasciitis leading to painless contractures.

UNLABELLED: We report on an 11-year-old girl who developed multiple joint contractures over a period of 3 months. The disease presented with progressive involvement of the fingers, elbows, shoulders, knees and feet and was not accompanied by other symptoms. Laboratory investigations showed eosinophilia and hypergammaglobulinaemia. Muscle ultrasound and magnetic resonance imaging of the right forearm revealed thickened fascia and a full thickness biopsy confirmed the diagnosis of eosinophilic fasciitis. Following treatment with pulsed steroids, the contractures resolved. CONCLUSION: our case shows that eosinophilic fasciitis can present without skin involvement and arthritis and therefore has to be regarded as a differential diagnosis of contractures in childhood. Pulsed steroid treatment was effective and without side-effects.

Biopsy↗

Prevention and management of contractures in patients with burns of the neck.

Two hundred patients with neck burns were analyzed to determine the incidence of contractures. It was found that only 8 per cent of patients with second degree burns had contractures, all of which were mild. Both the overall incidence of cervical contractures in patients with third degree neck burns and their severity can be decreased by the use of a custom-formed isoprene splint. Splinting should begin as soon as possible after the burn and continue until scar maturation is complete.

Burns↗

Irreversible muscle contracture after functioning free muscle transplantation using the ipsilateral facial nerve for reinnervation.

Four patients who underwent functioning free muscle transplantation (FFMT) for facial reconstruction developed a progressive disfiguring muscle contracture. This complication has not been previously reported. Three of the patients had longstanding facial paralysis and were reanimated by FFMT. The fourth patient had left hemifacial atrophy but without facial paralysis. She also underwent FFMT for augmentation. All four FFMTs were innervated by the ipsilateral facial nerve. Initially, they all had a normal facial appearance at rest during the first few months after FFMT. However, they all developed a progressive severe muscle contracture from 6 to 12 months after FFMT. Continuous spontaneous electrical impulse activity, which stimulated the transferred muscle day and night, may be responsible for the progressive muscle contracture. From the patients' reported sensations and from clinical evaluation, which included a local xylocaine injection test, over-reinnervation with a synkinesis effect of the transferred muscle is hypothetically the main cause, not over-tension of the muscle itself. This complication may possibly be avoided by limiting or decreasing the number of fascicles from the ipsilateral facial nerve or better by using a cross-facial nerve graft instead of the ipsilateral facial nerve as the innervating motor nerve. The outcome with a cross-facial nerve is likely to be more predictable and reliable.

Adolescent↗

Pharmacologic inhibition of scar contracture of intracardiac prosthetic patches.

Significant fibrosis and contracture often cause complications of pericardial and Dacron grafts within the heart. Both continue to be utilized because more suitable materials are unavailable. Newer prosthetic materials and the administration of drugs that might minimize intracardiac fibrosis and contracture after implantation were studied. Measured patches of three prosthetic materials were implanted into the right atrium of 18 dogs: 6 with expanded polytetrafluoroethylene (PTFE or Gore-Tex--W. L. Gore & Associates, Elkton, Md.), 6 with glutaraldehyde-preserved bovine pericardium, and 6 with Dacron patches. The animals were sacrificed after 8 weeks and gross and microscopic pathologic examinations were performed. The patch materials did not intrinsically shrink but all demonstrated marked distortion from the growth of surrounding scar tissue. In a fourth group of eight dogs with intraatrial Dacron patches, four were treated for 8 weeks with oral 3-aminopropionitrile fumarate, and four were not treated. A blinded observed evaluated the adhesions on a scale of 0 = no adhesions or patch distortion, to 4 = dense adhesions with marked distortion of the patches. The untreated dogs had dense adhesions of the lung and pericardium and the patches were encased in scar tissue with marked distortion. The average score was 3.5. The treated dogs had only minimal pleural adhesions and only a thin neointimal covering of the patch with virtually no distortion. The average score was 1.0 (P = 0.0032). The tensile strength of the healed atriotomies was evaluated with stress testing. The intact atrial wall ruptured at a force of 2.60 +/- 0.37 kg/m/sec2, the untreated atriotomy wound at 2.38 +/- 0.18 kg/m/sec2, and the treated atriotomy wound at 2.60 +/- 0.17 kg/m/sec2. There was no statistically significant difference among these groups. No other side effects of the 3-aminopropionitrile fumarate were noted, with only a single superficial wound infection caused by early postoperative trauma. Pharmacologic inhibition of scar formation minimized distortion and contracture of intracardiac prosthetic patches and decreased adhesions. Though further experimental evaluations are necessary, prolonged low-dose oral administration of beta-aminoproprionitrile (BAPN) may be beneficial in preventing complications of prosthetic implants and reoperative cardiac surgery in children.

Aminopropionitrile↗

Fibrous contracture of bladder neck: cause, prevention, and treatment.

Through a retrospective study of bladder neck contracture it was found that bladder neck resection and incision were equally effective for treatment of postoperative bladder neck contractures. It was also found that incising the bladder neck at the end of transurethral resection of the prostate (TURP) did not cause vesicoureteral reflux and did not improve the incidence of postoperative bladder neck contracture.

Contracture↗

Neodymium:YAG laser treatment of postoperative bladder neck contractures.

Conventional therapy for postoperative bladder neck contracture (BNC) has been associated with a high recurrence rate. Fifty bladder neck contractures were treated with neodymium (Nd):YAG laser using a contact technique. There were eight recurrences, seven of which were managed successfully with a second treatment. Nd:YAG laser disintegration is a safe and effective therapy for postoperative bladder neck contracture.

Adult↗

Release of burn scar contractures of the neck in paediatric patients.

Burn scar contractures of the neck represent a continuing problem for plastic surgeons. A review of 143 neck-release procedures performed at the Cincinnati Shriners Burns Institute documented a high rate of contracture recurrence. This was especially true in those patients who had previously suffered burns to the entire anterior neck. Treatment of this condition with Z-plasties, or releasing incisions with split thickness skin grafts, had a recurrence rate of 81 per cent and 62 per cent, respectively. The use of a neck hyper-extension brace for over 1 year following skin grafting decreased the recurrence rate to 17 per cent. In patients who were not compliant in wearing a brace, the best result was obtained by using a full thickness skin graft in the release site. Contractures resulting from smaller burns were successfully handled by a variety of techniques.

Braces↗

Surgical correction of burn scar contractures of the foot in children.

The results of reconstructive procedures for the treatment of burn scar contractures of the feet in 55 children undergoing 90 operations were reviewed. The patients were treated in all but one case by release of the contracture band with placement of a skin graft in the resulting defect. There was an overall recurrence rate of 15 per cent that was not affected by the use of split-thickness versus full-thickness grafts. The time delay from the thermal injury to the reconstructive procedure was also found not to affect the outcome. Postoperative immobilization by the use of either a dynamic or an adynamic splint was found to be important both for preventing graft loss and for decreasing the rate of contracture recurrence.

Burns↗

X-plasty for repair of burn contractures.

Release of skin contractures across a joint surface by X-incision is presented. The method is particularly suitable for severe and moderate contractures. Resultant long lateral flaps lead to good coverage of the flexor surface of the joint. This prevents recurrence of contracture even in growing children. The method is suitable for any joint.

Adult↗

Running Y-V-plasty for burn scar contracture.

Seventeen patients with 24 regions received running Y-V-plasties to release burn scar contractures over a 3-year period. The scar band can be completely interrupted and lengthened without the need for undermining and transposition of the skin flaps, circulatory embarrassment of the flaps can be avoided. The wound morbidity was extremely low, and no recurrence of the contracture was noted during follow-up periods. The running Y-V-plasty has unique advantages for the treatment of cord-like or linear burn scar contractures. This approach resulted in shorter hospital stay and allows early mobilization of the involved extremities.

Adolescent↗

Reconstruction of anterior neck contractures with sensate expanded radial forearm free flap.

The surgical management of anterior neck contractures, related to burn injuries, is a challenging problem for reconstructive surgeons. The use of sensate expanded radial forearm fasciocutaneous free flaps in two patients suffering from anterior burn contractures of the neck is presented as an alternative method of surgical management. The advantages of employing sensate expanded radial forearm free flaps to reconstruct anterior neck contractures are also discussed in this paper.

Adult↗

The effect of prior transurethral resection of the prostate on post radiation urethral strictures and bladder neck contractures.

Patients treated for prostate cancer from 1975-1982 were reviewed to assess if pre-irradiation transurethral resection of the prostate (TURP) predisposed to the formation of post treatment urethral strictures or bladder neck contractures. A total of 368 patients were treated with external beam irradiation delivered by a linear accelerator or 125I interstitial implants. Of the 253 patients treated by external beam, 138 patients had a history of at least one TURP before treatment. Implants were performed in 115 patients and 57 had a history of TURP before treatment. A total of 40 patients from both groups developed post treatment complications of urethral strictures or bladder neck contractures. Three patients developed both complications. An analysis of the 40 patients revealed that 29 (72.5%) patients had a TURP performed within a median time of 33 days prior to their initiation of radiation therapy. Of 195 patients in the prior TURP group, 29 (15%) developed one or both complications. Only 11 (6%) of the 173 patients in the non-TURP group developed one or both complications. This comparison was statistically significant with a p value of .025. Various factors analyzed including the treatment regimen, histologic grade, stage of disease, and volume of prostatic tissue removed at surgery did not show any positive correlation. Multiple TURP's were evaluated for greater distribution to the incidence of post treatment complications. The prior TURP group of 195 patients contained 60 with a history of at least two TURPs before radiation therapy. Eleven (18.3%) developed one or both complications. Of the 135 patients in the single TURP group, 18 (13.3%) developed complications. This was not statistically significant. Therefore, we concluded that the initial TURP was its mechanical description of mucosa and resultant scarring is a predisposing risk for development of urethral strictures or bladder neck contractures. Review of the literature concerning time sequence for healing of the urethra shows 4-6 weeks to be appropriate interval between surgery and radiation.

Combined Modality Therapy↗

Elbow flexion contractures treated with serial casts and conservative therapy.

We report on the technique of serial casts to successfully reverse elbow flexion contractures in three patients. Patients considered for this technique had a traumatic injury to the elbow that resulted in residual soft tissue restrictions; their mean flexion deformity was 44 degrees, which had been present for an average of 6 months. Treatment consisted of traditional therapy methods to obtain initial stretch and elongation of tissue followed by application of a cylindrical fiberglass cast in the position of maximal elbow extension. The cast was worn for 3 to 5 days, then removed, and the entire process was repeated. After the use of serial casts, elbow flexion contractures were reduced to a mean of 11 degrees. These results have been maintained over an average of 26 months. The use of casts in the treatment of elbow flexion contractures appears to be a viable technique for increasing range of motion when traditional methods of treatment alone are unsuccessful and surgical intervention may be the only alternative.

Adolescent↗

First web space contracture and hand function.

We studied 125 normal volunteers to determine the normal first web space angle and to evaluate the influence of thumb-index finger web space contracture on hand function. One hundred ninety-five hands were measured and found to have a mean web space angle of 100 degrees. There was no significant difference in the mean angle in relation to sex or hand dominance. The mean angle was significantly smaller for persons 50 to 79 years of age than for the two younger age groups. Twenty-five normal volunteers (50 hands) took the Jebsen-Taylor test three times: first, with no restrictions; second, with splints simulating a 60-degree web space contracture; and third, with splints simulating a 30-degree contracture. More than half of the volunteers had abnormal small-object subtests. Other abnormal subtests included card turning, feeding, and stacking checkers. Grasping large light and heavy objects was less difficult because of compensatory techniques used by the volunteers.

Adolescent↗

Central slip attenuation in Dupuytren's contracture: a cause of persistent flexion of the proximal interphalangeal joint.

This paper stresses the importance of central slip attenuation in the management of Dupuytren's contracture. Such attenuation occurs in patients who have had prolonged proximal interphalangeal joint contractures and is a common problem when the flexion contracture exceeds 60 degrees. The diagnosis can be made at the time of surgical correction by a tenodesis test. If central slip attenuation is confirmed, the postoperative regime described here will give a better correction of what at first might appear to be an intractable proximal interphalangeal joint flexion deformity.

Dupuytren Contracture↗

Passive stretching does not enhance outcomes in patients with plantarflexion contracture after cast immobilization for ankle fracture: a randomized controlled trial.

OBJECTIVE: To compare the efficacy of short- and long-duration passive stretches with a control treatment for the management of plantarflexion contracture after cast immobilization for ankle fracture. DESIGN: Assessor-blinded, randomized controlled trial. SETTING: Hospital physical therapy outpatient departments. PARTICIPANTS: Adults with plantarflexion contracture (N=150) after cast immobilization for ankle fracture. All subjects were weight bearing or partial weight bearing. INTERVENTIONS: Exercise only, exercise plus short-duration passive stretch, and exercise plus long-duration passive stretch. All subjects had a 4-week course of exercises. In addition, subjects in the short-duration stretch plus exercise group completed 6 minutes of stretching per day, and subjects in the long-duration stretch plus exercise group completed 30 minutes of stretching per day. MAIN OUTCOME MEASURES: Lower Extremity Functional Scale and passive dorsiflexion range of motion with the knee bent and straight at baseline, and at 4 weeks and 3 months postintervention. RESULTS: One hundred thirty-nine (93%) subjects completed the 4-week assessment and 134 (89%) subjects completed the 3-month assessment. There were no statistically significant or clinically important between-group differences for the primary outcomes. CONCLUSIONS: The addition of passive stretching confers no benefit over exercise alone for the treatment of plantarflexion contracture after cast immobilization for ankle fracture.

Adult↗