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PubMed2006

Long-term survival in a child with severe congenital contractural arachnodactyly, autism and severe intellectual disability.

The severe form of congenital contractural arachnodactyly is usually associated with early mortality due to multisystem complications. Here, we report a 9-year-old male child with severe skeletal manifestations of congenital contractural arachnodactyly. He had none of the cardiovascular or gastrointestinal features that have been described in severe congenital contractural arachnodactyly. He had profound intellectual disability with autism. All exons of FBN2, the gene associated with congenital contractural arachnodactyly, were sequenced and no disease-causing mutation was found. When severe congenital contractural arachnodactyly is diagnosed in the newborn period, parents need to be aware that long-term survival is possible, particularly if no significant extraskeletal complications are present, and that significant neurodevelopmental delay may occur.

Arachnodactyly↗
PubMed2004

The surgical treatment of Dupuytren's contracture: a synthesis of techniques.

Dupuytren's disease is an affliction of the palmar fascia. Selective fasciectomy is recommended once contracture has occurred. Alternatives for wound closure include tissue rearrangement, the open palm technique, and full-thickness skin grafting. In this prospective study, a new "synthesis" technique was used to treat a cohort of patients with advanced Dupuytren's disease. The results were then compared with those of a second cohort of patients who underwent the open palm technique. Thirty consecutive patients were selected. Ten patients (nine men and one woman; average age, 67 years) underwent the open palm technique, and 20 patients (18 men and two women; average age, 70 years) underwent the synthesis method. Follow-up was 3.5 years for the open palm group and 2.7 years for the synthesis group. All patients in both groups improved with respect to motion, function, appearance, and satisfaction. Objectively, for the open palm technique, metacarpophalangeal joint contracture decreased from 50 degrees to 0 degrees, and proximal interphalangeal joint contracture decreased from 40 degrees to 6 degrees. Using the synthesis method, metacarpophalangeal joint contracture decreased from 57 degrees to 0 degrees, and proximal interphalangeal joint contracture decreased from 58 degrees to 10 degrees. The Disabilities of the Arm, Shoulder, and Hand Test scores decreased from 37 to 30 in both groups. There were no significant differences between groups in these parameters. The two significant intergroup differences were healing time (40 days for the open palm technique versus 28 days for the synthesis method) and recurrence rate (50 percent for open palm versus 0 percent for synthesis). The synthesis technique combines with success the best features of current methods for the surgical treatment of advanced Dupuytren's disease.

Aged↗
PubMed2003

Usefulness of palatal mucoperiosteal grafts for artificial eye socket contracture.

The authors performed palatal mucoperiosteal grafting for contracture of the artificial eye socket in 4 patients. Mucoperiosteal grafts were collected from the paramedian area of the hard palate. After release of contracture, the grafts were sutured with absorbable thread to the defective areas on the conjunctival side of the artificial eye socket after release of contracture. All patients showed mucoperiosteal graft survival without problems, no recurrence of contracture, and good courses of artificial eye wear. The mucoperiosteal donor areas showed closed healing after 3 to 4 weeks. Palatal mucoperiosteal grafts can be collected en bloc and are relatively rigid, which allows the simultaneous reconstruction of the conjunctival side and supportive tissue of the eyelid. Although the size of graft collection is limited, grafts with adequate size for partial reconstruction can be collected. Mucoperiosteal grafts are a good reconstruction material for contracture of the artificial eye socket.

Aged↗
PubMed2005

Surgical intervention and capsular contracture after breast augmentation: a prospective study of risk factors.

Epidemiologic data on local complications after breast augmentation are scarce. In particular, few prospectively collected data are available on modern breast implants on this issue. Using data from the Danish Registry for Plastic Surgery of the Breast, the authors examined determinants of surgery-requiring complications and capsular contracture grades III to IV among 2277 women who underwent cosmetic breast implantation from June 1999 through April 2003. During an average follow-up period of 1.6 years after implantation, 4.3% of these women (3% of implants) required secondary surgery as a result of short-term complications. The most frequent clinical indications for surgery were displacement of the implant (38%), capsular contracture grades III to IV (16%), ptosis (13%), and hematoma (11%). Overall, the authors found that inframammary incision and subglandular placement were associated with decreased risks of developing complications requiring surgical intervention, whereas implants larger than 350 mL increased the risk of such complications (relative risk [RR], 2.3; 95% confidence interval [CI], 1.3-4.0). Thirty-nine Baker III to IV capsular contractures were identified, of which 22 were treated surgically within the study period. Submuscular placement of the implant decreased the risk of capsular contracture grades III to IV (RR, 0.3; 95% CI, 0.2-0.8), whereas surgical routes other than inframammary and drainage of implant cavity were associated with increased risk of capsular contracture. Current surgical practices and modern implants used for breast augmentation produce fewer short-term complications than procedures and devices of the past. This prospective study indicates that surgical procedures are more important predictors for local (short-term) complications than implant or patient characteristics.

Adult↗
PubMed2005

Prevention of severe contractures might replace multilevel surgery in cerebral palsy: results of a population-based health care programme and new techniques to reduce spasticity.

During the 1990s three new techniques to reduce spasticity and dystonia in children with cerebral palsy (CP) were introduced in southern Sweden: selective dorsal rhizotomy, continuous intrathecal baclofen infusion and botulinum toxin treatment. In 1994 a CP register and a health care programme, aimed to prevent hip dislocation and severe contractures, were initiated in the area. The total population of children with CP born 1990-1991, 1992-1993 and 1994-1995 was evaluated and compared at 8 years of age. In non-ambulant children the passive range of motion in hip, knee and ankle improved significantly from the first to the later age groups. Ambulant children had similar range of motion in the three age groups, with almost no severe contractures. The proportion of children treated with orthopaedic surgery for contracture or skeletal torsion deformity decreased from 40 to 15% (P = 0.0019). One-fifth of the children with spastic diplegia had been treated with selective dorsal rhizotomy. One-third of the children born 1994-1995 had been treated with botulinum toxin before 8 years of age. With early treatment of spasticity, early non-operative treatment of contracture and prevention of hip dislocation, the need for orthopaedic surgery for contracture or torsion deformity is reduced, and the need for multilevel procedures seems to be eliminated.

Anti-Dyskinesia Agents↗
PubMed1982

Surgical management of knee contractures in myelomeningocele.

Contractures of the knee joint can interfere with orthotic fitting and prevent the child from being upright and ambulatory. Two types of knee contractures are seen: flexion and extension. A flexion deformity is more common in the thoracolumbar level and, when beyond 20 degrees, will require surgical treatment. The author reviewed his surgical experience with 23 knees undergoing a radical flexor release. With an average follow-up of 38 months, 10 knees showed no contractures, 11 knees 5 to 10 degrees of flexion deformity, and 1 knee a 15 degrees deformity. Three knees had a simple tendon release with poor results. Fifteen knees with an extension contracture were treated surgically (VY quadriceps lengthening). With a follow-up of 43 months, eight knees had 120 degrees of flexion, five 90 degrees, and two only 45 degrees. Three knees showed full recovery of quadriceps strength. It is concluded that a knee flexion deformity will respond well to the radical flexor release. Prolonged splinting is important in order to avoid recurrence. An extension contracture can be successfully treated by the VY quadriceps plasty with improvement in the child's gait and sitting.

Child↗
PubMed1985

Congenital contractural arachnodactyly. Report of four additional families and review of literature.

We report here four families with congenital contractural arachnodactyly (CCA) in which a wide range of phenotypic expression is observed. In one family with a large number of affected individuals the condition is mildly expressed. These individuals usually have crumpled ears, camptodactyly with ulnar deviation of the fingers, adducted thumbs, limited elbow and/or knee extension, and hypoplasia of the calf muscles. Arachnodactyly is not a constant feature. No spinal deformities are present and only the proband has clubfoot deformities. With time, affecteds have experienced spontaneous improvement of their contractures and their condition in adulthood has not interfered with a normal lifestyle. Within this family there is little phenotypic variation between affected individuals. Those affected within each of the other families have had varying degrees of severity of the condition. A review of 29 other kindreds described in the literature with congenital contractural arachnodactyly shows that in this condition the most common features are abnormally formed ears, camptodactyly, arachnodactyly, adducted thumbs, limited movement of the elbows and knees, and underdevelopment of the calf muscles. Spontaneous improvement of the contractures with age is reported in 94% of cases. Kyphosis, scoliosis or kyphoscoliosis occurred in 50% and these defects were present in those who where more severely affected with CCA. No ocular problems have been reported in this syndrome, but congenital heart defects have occurred in 14.7%. Marfan syndrome is the most important condition to differentiate from congenital contractural arachnodactyly since these two conditions are similar phenotypically. However, in the former there are frequently serious ocular and cardiovascular problems which lead to significant morbidity and/or early death.

Adolescent↗
PubMed1988

Efficacy of soft splints in reducing severe knee-flexion contractures.

This study evaluated the effectiveness of soft splints made from polyurethane foam in reducing severe knee-flexion contractures of patients with cerebral palsy. The splints were applied nightly over a period of 10 months. Knee-flexion contractures were reduced by an average of 24 degrees in all patients. Younger patients generally presented with less severe initial contractures and had the greatest percentage of improvement. When used on a single limb when bilateral contractures were present, sympathetic reduction of the contracture occurred to varying degrees in the unsplinted limb. It is recommended that the splints be replaced regularly to maximize their effectiveness. The benefit-to-cost ratio is extremely high and their low cost makes soft splints feasible for use in developing countries and by low-income families.

Adolescent↗
PubMedDate not supplied

Infrapatellar contracture syndrome. Diagnosis, treatment, and long-term followup.

Infrapatellar contracture syndrome is an uncommon but recalcitrant cause of reduced range of motion after knee surgery or injury. The results and conclusions presented here are based on a retrospective clinical study evaluating the long-term outcome in 75 patients who developed infrapatellar contracture syndrome. These 75 patients (76 knees) were evaluated at an average followup of 53 months after the index (inciting) procedure or injury. Comparing subgroups within the study population, factors that correlated with poorer results or more severe infrapatellar contracture syndrome were found to be acute anterior cruciate ligament repair or reconstruction, the use of patellar tendon autograft for anterior cruciate ligament reconstruction, nonisometric graft placement, multiple surgical procedures, use of closed manipulation, and the development of patella infera. We concluded that appropriate procedures can substantially increase the range of motion in patients with infrapatellar contracture syndrome. However, residual functional morbidity persists in many patients, and the outcome, as determined by subjective knee function scores, is only fair. The natural history of an anterior cruciate ligament-deficient knee appears to be more benign than the natural history of a knee that develops infrapatellar contracture syndrome.

Adolescent↗
PubMed2006

Congenital contractural arachnodactyly (Beals syndrome).

Congenital contractural arachnodactyly (Beals syndrome) is an autosomal dominantly inherited connective tissue disorder characterized by multiple flexion contractures, arachnodactyly, severe kyphoscoliosis, abnormal pinnae and muscular hypoplasia. It is caused by a mutation in FBN2 gene on chromosome 5q23. Although the clinical features can be similar to Marfan syndrome (MFS), multiple joint contractures (especially elbow, knee and finger joints), and crumpled ears in the absence of significant aortic root dilatation are characteristic of Beals syndrome and rarely found in Marfan syndrome. The incidence of CCA is unknown and its prevalence is difficult to estimate considering the overlap in phenotype with MFS; the number of patients reported has increased following the identification of FBN2 mutation. Molecular prenatal diagnosis is possible. Ultrasound imaging may be used to demonstrate joint contractures and hypokinesia in suspected cases. Management of children with CCA is symptomatic. Spontaneous improvement in camptodactyly and contractures is observed but residual camptodactyly always remains. Early intervention for scoliosis can prevent morbidity later in life. Cardiac evaluation and ophthalmologic evaluations are recommended.

Abnormalities, Multiple↗
PubMed2001

Do associated reactions in the upper limb after stroke contribute to contracture formation?

OBJECTIVE: To establish (1) whether associated reactions could contribute to contracture formation and (2) whether the presence of spasticity was essential for their expression, after stroke. SUBJECTS: Subjects were 24 hemiparetics within 13 months of a stroke, unselected for contracture or spasticity. MAIN OUTCOME MEASURES: Associated reactions were identified by the presence of muscle activity in the affected biceps brachii and quantified as the amount of affected elbow flexor torque produced during a moderate contraction of either the contralateral biceps brachii or the contralateral quadriceps muscles. Contracture was measured as loss of elbow joint range of motion and spasticity as the presence of abnormal reflex activity. RESULTS: Associated reactions were present in at least one testing condition in seven subjects. During contractions of the contralateral biceps brachii, the median amount of elbow flexor torque produced was 0.39 (interquartile range, IQR 2.5) Nm while during contractions of the contralateral quadriceps muscle it was 0.19 (IQR 1.6) Nm. Associated reactions were not associated with contracture (p = 0.39) which was present in over half of the subjects. The incidence of associated reactions was about the same as that of spasticity, but the two were not related (p = 0.61). CONCLUSIONS: Even though associated reactions were present in 29% of the subjects during moderate contraction of the contralateral muscles, they were not large, nor were they associated with contracture or spasticity, suggesting that this phenomenon is not usually a major problem for everyday function after stroke.

Adult↗
PubMed2006

The Tardieu Scale differentiates contracture from spasticity whereas the Ashworth Scale is confounded by it.

OBJECTIVE: To compare the Tardieu Scale as a clinical measure of spasticity after stroke with the Ashworth Scale. DESIGN: Cross-sectional study. PARTICIPANTS: Sixteen people, living in the community three years after their stroke. MAIN MEASURES: The Ashworth Scale and Tardieu Scale as well as laboratory measures of spasticity (stretch-induced electromyographic (EMG) activity) and contracture (maximum passive joint excursion) were collected from the affected elbow flexors and extensors and ankle plantarflexors and dorsiflexors by three examiners who were blinded to the results of the other measures. RESULTS: The percentage exact agreement (PEA) between the Tardieu Scale and a laboratory measure of spasticity was 100% for both the elbow flexors and ankle plantarflexors. This was significantly (P= 0.02) greater than the PEA of 63% for both muscles between the Ashworth Scale and the same laboratory measure of spasticity. For contracture, the PEA between the Tardieu Scale and a laboratory measure was 94% for both the elbow flexors and the ankle plantarflexors. Pearson correlation coefficients between the Tardieu Scale and laboratory measures of spasticity were 0.86 for the elbow flexors and 0.62 for the ankle plantarflexors and between the Tardieu Scale and laboratory measures of contracture were 0.89 for the elbow flexors and 0.84 for the ankle plantarflexors. CONCLUSION: In all cases that spasticity was overestimated by the Ashworth Scale, participants had a contracture. These findings suggest that the Tardieu Scale differentiates spasticity from contracture whereas the Ashworth Scale is confounded by it.

Aged↗
PubMed2002

Operative treatment of elbow contracture in patients twenty-one years of age or younger.

BACKGROUND: Elbow contracture is a recognized sequela of traumatic and developmental elbow disorders, but little information is available regarding the surgical treatment of elbow stiffness in the pediatric population. METHODS: Thirty-seven patients who had had open surgical release of an elbow contracture at a mean age of sixteen years (range, ten to twenty years) were retrospectively studied after a mean duration of follow-up of fifteen months (range, six to forty-four months). The elbow contracture was posttraumatic in twenty-eight patients. The operation consisted of a capsular release with removal of osseous impediments to motion as necessary. No patient had muscle or tendon-lengthening. RESULTS: The total arc of motion improved from a mean of 66 preoperatively to a mean of 94 postoperatively; however, only twenty-eight patients (76%) had an improvement of 10 and only seventeen (46%) achieved a functional arc of motion of 100 (from 30 to 130 ). Two patients lost motion after surgery. These results are less favorable than the results of previous studies of both pediatric and adult patients. Patients in whom the contracture had been caused by a simple dislocation of the elbow or an extra-articular fracture tended to have better results than those in whom the contracture was due to other causes. CONCLUSIONS: The results of surgical treatment of elbow stiffness in pediatric patients are less favorable and less predictable than those in adult patients.

Adolescent↗
PubMed1998

Contracture of the deltoid muscle: imaging findings in 17 patients.

OBJECTIVE: A retrospective review of the MR images and radiographs of 26 shoulders in 17 patients suffering from contracture of the deltoid muscle was used to establish the characteristic imaging findings. MATERIALS AND METHODS: Deltoid muscle contractures in 26 shoulders in 17 patients encountered over a 4-year period are reported. The history of intramuscular injection and clinical symptoms and signs are detailed. Imaging studies including routine radiographs (24 shoulders), MR images (25 shoulders), and computed arthrotomograms (two shoulders) were reviewed. On MR images, the winging angle of the scapula (angle between the axis of the scapular body and the coronal plane of the chest) and the diameter of the lesion were measured and compared with data derived from 24 age-matched control subjects. RESULTS: Diagnostic MR features of deltoid muscle contracture include fibrotic cord in the deltoid muscle, especially its middle portion, extending from the superior acromial surface to the deltoid tuberosity, and winging of the scapula (increased winging angle of the scapula). Characteristic radiographic features include abduction contracture, winging of the scapula, lateral down-sloping of the acromial process, and a superior acromial enthesophyte. CONCLUSION: MR images are sensitive and accurate in the diagnosis of contracture of the deltoid muscles. Characteristic features also allow accurate routine diagnosis on radiographs.

Arm↗
PubMed1996

Soft tissue contractures of the knee or ankle treated by the Ilizarov technique. High recurrence rate in 26 patients followed for 3-6 years.

We used Ilizarov frames in 26 patients for correction of severe contractures in 10 knees and 19 ankles. All patients had good initial correction. 2 patients developed posterior subluxation of the knee and 2 patients developed anterior subluxation of the ankle. At a minimum of 3 years' follow-up, 11 contractures had recurred. All patients except 1 had persistent restriction of motion. Considering the final position, improvement of motion and complications, we obtained an excellent result in 1 patient, good in 13, fair in 11, and a poor result in 1. Better results were obtained in patients with posttraumatic contractures than with other etiologies. There were also better results in correcting equinus contracture of the ankle than in knee flexion contracture.

Adolescent↗
PubMedDate not supplied

Slow, gradual external fixator distraction in acquired ankle and foot contracture.

BACKGROUND: External fixators have been noted to have a place in the orthopaedic management of problems involving the ankle and foot. We here report a case of ankle and foot contracture managed by soft tissue release and slow, gradual external fixator distraction. METHOD: A case report of a patient with acquired ankle and foot contracture and discussion of relevant literature. RESULTS: A 9-year old female presented to our out-patient clinic with features of right ankle and foot contracture following treatment by traditional bone setters 6 years earlier. The contracture was fixed at 30 degrees. She had soft tissue release and slow, gradual external fixator distraction which corrected the foot to a plantigrade position. Subsequently she had skin grafting for the skin defect. After removal of the external fixator she was placed on a below knee cast and commenced weight bearing. The cast was removed after three weeks and the patient has continued to bear weight on a plantigrade foot. CONCLUSION: External fixators have a definite place in contracture release and should be widely utilized.

Ankle Injuries↗
PubMed2001

Surgical treatment of extension contractures caused by war injuries to the knee.

Immobilization with external fixation bridging the knee joint in extension is frequently used after sustaining a war injury to the region of the knee joint with femoral and tibial bone fractures. Immobilization of the knee with plaster splints is performed in the same position. This usually prolonged treatment results in extension contractures of the knee joint. From June 1991 until March 1994, 54 patients with extension contractures of the knee caused by war wounds were treated at the Department of Orthopedics, Zagreb University Hospital Center, in Zagreb, Croatia. Results of surgical treatment of 44 patients are presented. The operative procedure consisted of extensive intra- and extra-articular adhesiolysis of the knee. The control group included 30 patients with knee contractures caused by injuries sustained in car crashes or secondary to previous operative procedures. The mean duration of immobilization was 5 months and 6 days. The mean preoperative knee motion amplitude ranged from 5 degrees in extension to 38 degrees in flexion. The mean postoperative knee motion was 98 degrees (range, 2 to 110 degrees). Treatment results did not depend on either duration of preoperative immobilization of the knee or previous infection in the region of injury. Previous knee joint bridging with an external fixator had no impact on the results of extension contracture treatment. Adequate intra- and extra-articular adhesiolysis with appropriate long-term postoperative rehabilitation is essential for the success of the operative treatment for knee joint contracture.

Adolescent↗
PubMed2000

[Experiment research on the mechanism of skin contracture after expansion].

OBJECTIVE: The aim of this study is to observe the repairing process of skin chronic injury due to expansion and explore the mechanism of skin contracture after expansion. METHODS: 20 dogs used in this study were divided randomly into four groups. Skin samples were collected from the back of dogs when expansion finished and 3 months, 6 months, 1 year after expansion. Normal skim from the other side on the back of dogs was used as control group. The dermal ultrastructure was observed by electriscope. The expression of alpha-SMA in dermis at different stage has been analyzed by immunohistochemistry, and the concentration of intracellular free Ca2+ in the fibroblast has been measured by Fura-2 fluorescen indicator. Meanwhile we constructed a 3-dimension system in vitro to analyze the afgfect of different cell density and collagen concentration to fibroblast contracture. RESULTS: (1) The microfilment in fibroblast was fragile, and the features of myofibroblast was absent, the collagen fibers were returned to reticular arrangement along with time. One after expansion few of the collagen remained irregular in arrangement and aparse. (2) After expansion the concentration of Ca2+ increased significantly. (3) In 3-D culture system the density of fibroblasts positively corresponded to fibroblast contracture and the collagen concentration negatively corresponded to it. CONCLUSION: After expansion the dog skin has a structure between scar tissue and thoroughly regeneration skin. Dermal fibroblast is the dynamic source of the skin contracture, meanwhile the density of fibroblast and collagen concentration are two essential factors during skin contracture.

Actins↗