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At least 91 records · Page 5Linked to original sources
PubMed2003

FGFR2 mutation in a patient with Apert syndrome associated with humeroradial synostosis.

Most cases of Apert syndrome are due to S252W or P253R mutations in the fibroblast growth factor receptor 2 (FGFR2) gene. Differences in the effects of S252W and P253R mutations on the clinical features of Apert syndrome have been studied, but little is known about the type of FGFR2 mutation in Apert syndrome with humeroradial synostosis. To study a correlation between the FGFR2 mutations and the clinical complications, we examined the FGFR2 gene in a patient with Apert syndrome associated with humeroradial synostosis, and found that the mutation was S252W. This report suggested that S252W mutation in FGFR2 may cause humeroradial synostosis in Apert syndrome.

Acrocephalosyndactylia↗
PubMed1997

Surgical management of sagittal synostosis: a comparative analysis of strip craniectomy and calvarial vault remodeling.

Although surgical treatment of sagittal synostosis has become normative, the procedure of choice is still debated. This retrospective analysis, during a 10-year period (1986-1995) at Children's Hospital Los Angeles, compares the more conservative strip craniectomy (SC) procedure with a more extensive occipital reduction-biparietal widening calvarial vault remodeling (CVR) technique for the management of sagittal synostosis. SC was performed in 72 patients (61%) and CVR in 46 (39%). Patients undergoing CVR were older (mean 5.3 vs. 3.0 months, p < 0.01), the procedure time longer (mean 126 min vs. 72 min, p < 0.01), intraoperative blood loss greater (mean 243 vs. 54 cm3, p < 0.01), and the frequency of intraoperative blood transfusion increased (100 vs. 38%, p < 0.01; mean volume 357 vs. 51 cm3, p < 0.01) compared to patients undergoing SC. No significant intraoperative complications occurred in either group. The total time of hospitalization was similar (mean 4.4 vs. 3.8 days, p = 0.02). No neurological, hematological, or transfusion-related complications or deaths were encountered in either group. Cosmetic outcomes were significantly better in the CVR group with 79% rated as excellent compared to 41% in the SC group (p < 0.01). Fifty-nine percent of the SC patients manifested bony defects at last follow-up visit. Two SC patients required reoperation for poor cosmetic outcomes; CVR was successfully employed in both cases. These findings demonstrate that CVR is superior to SC in providing immediate and lasting correction of calvarial deformities secondary to sagittal synostosis. The optimal age at time of surgery is <6 months; however, CVR can be utilized throughout infancy, while SC becomes progressively less effective after 6 months of age. No significant complications were observed in either group, despite the increased operative manipulation and greater incidence of blood transfusion in the CVR patients.

Craniotomy↗
PubMedDate not supplied

Tibiofibular synostosis and recurrent ankle sprains in high performance athletes.

Recent evidence points toward a weight bearing and dynamic stabilizing function of the distal fibula in ankle joint mechanics. When fibular rotation and translation are restricted, ankle pain during weight bearing and push off often (but not always) results. The case histories of six professional athletes with distal tibial synostosis resulting from internal rotation-inversion injury confirm recent reports of ankle disability resulting from restriction of fibular motion, but suggest that there may be many patients with this lesion who are not disabled. Two patients with incomplete synostosis were asymptomatic, and one with complete synostosis had only occasional pain after vigorous exercise.

Adult↗
PubMedDate not supplied

Tibiofibular synostosis: an unusual cause of shin splint-like pain.

Tibiofibular synostosis may be an underlying cause of tibial pain with activity or ankle pain of uncertain origin. The diagnosis may be suspected when symptoms begin and progress months after an injury, when symptoms occur with vigorous activity, and when symptoms are most pronounced during midstance and preswing phases of the gait cycle. Confirmation of the diagnosis is easily made through radiographic examination, which should include the entire leg. Bone scan may show increased tracer uptake. Interference with normal dynamic function of the fibula subjects the synostosis to repetitive stress and ultimate fatigue fracture with resultant pain. Conservative treatment is recommended initially. Surgical excision should be reserved for the athletically active patient whose symptoms are gradually progressing and disabling, and whose synostosis has taken on the appearance of mature cortical bone on radiographs. The principles of tumor biopsy should be adhered to, lest the diagnosis be in error. Finally, meticulous hemostasis and use of bone wax may lessen the possibility of recurrence.

Adolescent↗
PubMed1993

Distal tibiofibular synostosis after malleolar fractures treated using absorbable implants.

Three patients (3.8%) of 80 with displaced malleolar fractures treated by open reduction and internal fixation using absorbable polyglycolide rods were found to have developed distal tibiofibular synostosis. To assess the general incidence of this complication, the serial radiographs of 150 patients with an identical distribution of the initial fracture severity treated with AO-ASIF implants were examined for comparison. Among these, no cases of tibiofibular synostosis were found. The difference (P = .04) in incidence was postulated to be due to an osteogenic potential of the absorbable polymeric material, suggested previously in experimental studies. This study included fractures of the lateral malleolus and bimalleolar fractures, but patients with a disruption of the inferior tibiofibular syndesmosis causing tibiofibular diastasis on admission were excluded. The synostoses occurred in middle-aged, sedentary people, and no active measures were necessary because of the mild subjective symptoms of these patients. Previously, posttraumatic distal tibiofibular synostosis has been recorded as an uncommon late complication of severe ankle fractures with tibiofibular diastasis requiring transsyndesmotic fixation, but it has not been reported to occur after malleolar fractures of the kind surveyed in this study.

Adolescent↗
PubMed1998

Mobilization of a congenital proximal radioulnar synostosis with use of a free vascularized fascio-fat graft.

We present the results of a new mobilization procedure for the treatment of a congenital proximal radioulnar synostosis in seven patients. The operative procedure included separation of the synostosis and placement of a free vascularized fascio-fat graft to prevent recurrent ankylosis. The average age at the time of the operation was eight years and two months (range, six years and four months to eleven years and ten months). All of the patients were boys who had no other congenital anomalies. The radial head was dislocated in all seven patients (anteriorly in two and posteriorly in five). The final four index operations included an osteotomy of the radius in order to reduce the dislocated radial head. The average duration of follow-up was three years and eight months (range, two years and four months to four years and five months). Preoperatively, the patients had had difficulty with holding a bowl of soup and accepting objects, such as coins, into the palm. Postoperatively, they were able to perform these activities. None of the patients had recurrent ankylosis or loss of the flap. The average supination was 26 degrees (range, 10 to 45 degrees), and the average pronation was 45 degrees (range, 10 to 80 degrees). The four patients who had had an osteotomy of the radius in addition to the index procedure did not have a dislocation of the radial head and had an average arc of motion of 83 degrees of pronation and supination. The three patients who had not had an osteotomy had a dislocation of the radial head and an average arc of motion of 40 degrees after the index procedure. These findings demonstrate that separation of a congenital radioulnar synostosis with a vascularized fascio-fat graft and osteotomy of the radius can achieve pronation and supination of the forearm.

Adipose Tissue↗
PubMed2000

Herrmann multiple synostosis syndrome with neurological complications caused by spinal canal stenosis.

A young man was found to have multiple synostosis syndrome type I after presenting with a neck injury causing a cervical spinal cord contusion. Neurological symptoms and signs suggested spinal cord compression. Magnetic resonance (MR) and computerized tomography (CT) imaging of the spine showed spinal canal stenosis with cord compression at C3-C6, a deformed spinal canal flattened in the anteroposterior dimension, vertebral fusions and deformed lateral processes of the vertebrae. He had a long broad nose with hypoplasia of the alae nasi, conductive hearing loss requiring hearing aids, muscular build, stiff spine, prominent acromia, pectus excavatum, ischial prominences, short fifth fingers, fusion at the proximal interphalangeal joints of the fifth fingers with indistinct overlying creases, and toe syndactyly. Spinal cord stenosis is a serious complication of multiple synostosis syndrome, that should be kept in mind in considering the risk of neck or back injury associated with certain sports or other activities. In both the multiple synostosis syndrome and the less severe proximal symphalangism deafness syndrome, mutations have been detected in the human homologue of the noggin gene on chromosome 17q21-q22.

Abnormalities, Multiple↗
PubMed2002

[Rotational osteotomy technique for congenital radio-ulnar synostosis with central medullary nailing and external fixation].

PURPOSE OF THE STUDY: Congenital radio-ulnar synostosis is a very uncommon condition. The functional impairment resulting from the complete pronation posture justifies rotational osteotomy. We present a series of six rotational osteotomies using the Hernigou-Goutallier technique. MATERIAL AND METHODS: The Hernigou-Goutallier osteosynthesis procedure is based on a centromedullary nail and two external fixation pins positioned in the ulna at an angle corresponding to the desired rotation. The nail is partially withdrawn to allow subperiosteal osteotomy in the synostosis via a horizontal incision between the two pins. After rotation, the osteotomy is stabilized with clamps holding the external fixation pins in parallel. RESULTS: Bone healing was achieved at two months in all six cases with no loss of correction. DISCUSSION: Among the different treatments proposed for radio-ulnar synostosis, the risk of vessel or nerve injury is high with screw-plate fixation despite shortening osteotomy. Palm-tree osteotomy raises the same risks and is more invasive and difficult to achieve. The rotational osteotomy technique presented here has the advantage of allowing local care more easily than with cast fixation. Another important advantage is that an overcorrection leading to vessel or nerve injury can be reversed without losing the axis, allowing progressive correction if necessary.

Bone Nails↗
PubMed1990

Proximal radioulnar synostosis after repair of distal biceps brachii rupture by the two-incision technique. Report of four cases.

In four cases, proximal radioulnar synostosis developed as a complication of distal biceps tendon reattachment through a two-incision technique. The synostosis was excised in all four cases but recurred in two. In each case, the initial reattachment exposed the bicipital tuberosity of the radius anteriorly and exposed the ulna extraperiosteally through a second incision along its subcutaneous border. This two-incision approach may place the patient at risk for synostosis. If two incisions are to be used for distal biceps tendon reattachment, then a limited muscle-splitting approach through the extensor muscle mass, which avoids exposure of the ulna, may be preferable.

Adult↗
PubMed1989

Post-traumatic proximal radio-ulnar synostosis. Results of surgical treatment.

During a period of forty-two years, twenty patients who had a post-traumatic proximal radio-ulnar synostosis were treated by excision of the area of synostosis and various concurrent procedures in an attempt to restore rotation of the forearm. The time from injury to operation averaged eighteen months, and the postoperative follow-up averaged forty months. Results were graded on the basis of improvement in the arc of rotation of the forearm. After excision of the area of synostosis in the fifteen patients for whom data were available, the intraoperative arc of passive rotation averaged 121 degrees. At the most recent follow-up, the range of active rotation averaged 55 degrees for all twenty patients. Four patients had an excellent result; three, good; four, fair; and nine, poor. The outcome of this type of surgical treatment varies, but roughly half of the patients can be benefited.

Adolescent↗
PubMed1985

Congenital proximal radio-ulnar synostosis. Natural history and functional assessment.

We evaluated the cases of twenty-three patients with thirty-six congenital proximal radio-ulnar synostoses who had been followed between 1938 and 1984. None of the patients had had any attempt at surgical correction or any ipsilateral congenital anomalies. Emphasis was placed on analyzing the natural history of the lesion in these patients who had not been operated on, and on determining the effect of a fixed position of the forearm on function. Ten patients had unilateral and thirteen, bilateral synostosis. Their mean age when last examined was twenty-two years (range, three to fifty years). Eight patients were female and fifteen, male. The forearms were fixed in an average of 30 degrees of pronation. The position of the forearm was not found to be related to subjective functional limitations, employment status, or the results of the hand-function test of Jebsen et al. Most patients had few or no functional limitations, and were employed in jobs that demanded extensive use of the forearm. Contrary to the findings of other studies, we observed four distinct radiographic patterns based on the presence of an osseous synostosis and the position of the radial head. However, we noted no relationship between any of these patterns and function. We concluded that operative treatment of congenital radio-ulnar synostosis is rarely indicated, that less emphasis should be placed on the single factor of the position of the forearm, and that objective functional tests should be included in the assessment of these patients.

Activities of Daily Living↗
PubMed1979

Congenital radio-ulnar synostosis: surgical treatment.

The results of an operative approach to the problem of radio-ulnar synostosis were assessed in thirteen patients, ten to twenty-five and one-half years after the procedure was performed. We concluded that in a patient with bilateral synostosis one hand, the one not used in writing, should be shifted to a position of 20 to 35 degrees of supination. With one hand in this position, the other may be left in considerable pronation. Often after such a shift it is not necessary to rotate the second arm. However, if the pronation is marked in the second forearm, and if function is impaired unduly by this position, surgical correction is indicated. The arm should be placed in a position of 30 to 45 degrees of pronation. In unilateral radio-unlar synostosis, the ordinarily ideal position of the radius is between 10 and 20 degrees of supination. In an adult, the patient's occupation should be considered in deciding on the rotatory positions of the forearms. We usually prefer a method of transverse osteotomy through the conjoined mass of the radius and ulna. Careful observation of the effect on the vascular status of the limb during and immediately after surgery is important.

Adolescent↗
PubMedDate not supplied

Congenital radio-humeral synostosis. A case report.

In a 7-month-old male infant with congenital radiohumeral synostosis and associated absent first metacarpal, floating thumb, and hypoplasia of the humerus, the synostosis was resected. Elbow motion was obtained and one year postoperatively there was no recurrence of the synostosis.

Abnormalities, Multiple↗
PubMed1978

Proximal tibiofibular synostosis.

The occurrence of a proximal tibiofibular synostosis is indeed a rare condition with only 2 cases unassociated with other diseases reported to our knowledge to date. Two skeletally immature patients presented with a synostosis of the proximal tibiofibular region associated with shortening of the limb in the affected segments. Although the shortening and the synostosis seem interrelated no explanation of their relationship is evident from these 2 cases.

Child↗
PubMedDate not supplied

[Congenital radio-ulnar synostosis of children (author's transl)].

29 congenital radio-ulnar synostosis have been observed in 16 childrens. The authors review the clinical patterns of this affection: most often bilateral, it results in impossibility of pronosupination of the wrist which has but little functional consequence, if the hand is in an intermediary position. On the X-ray its almost always a superior radio-ulnar synostosis but the inferior radio-ulnar joint is abnormal and non functional. Only the children severely handicapped by a hand fixed in pronation should be operated upon. No good result can be hoped from a surgery that tries to restore pro-supination. The best surgical technique seems to be a simple horizontal osteotomy through the synostosis itself which allows a derotation of the forearm into the functional intermediary position. Severe complications can occur. Indications and technique must be very careful since this congenital abnormality is very well tolerated.

Child↗
PubMed1993

Pin-site radioulnar synostosis after external fixation of a distal radial fracture: two case reports.

Complications of distal radial fracture and external fixation are common, but the authors report, in two patients, a previously unrecognized complication, that of radioulnar synostosis. In both cases the proximally placed pins extended across the interosseous region, and the distal ends were adjacent to the ulna. Hematoma and osseous debris were introduced into the interosseous region. Several weeks after the external fixator was removed, the patients were noted to have a restricted range of pronation and supination. A radioulnar synostosis had formed across the interosseous region. Resection of the synostosis restored pronation and supination. With careful pin placement this complication will be avoided.

Adult↗
PubMed1998

[Bilateral congenital radio-ulnar synostosis with hyperpronation--findings and surgical therapy].

Congenital proximal synostoses of the ulna and radius do not require surgery in most of the cases, since the proximal synostosis can be sufficiently compensated by the wrist joint. In cases of considerable functional deficit, the synostosis has to be separated to enable re-ossification in a more advantageous position. We report on a patient with bilateral synostosis and a significant functional deficit due to the grotesque hyperpronation of the forearms. Following operative longitudinal separation and redressive casts, a good correction was obtained. X-ray imaging in lateral view documented the improved forearm position.

Child, Preschool↗
PubMed1997

Preoperative anthropometric dysmorphology in metopic synostosis.

Anthropometric identification of dysmorphology in craniofacial anomalies, including the craniosynostoses, provides invaluable assistance in clinical diagnosis as well as offering a technique for interpreting possible deformities in skeletal remains. Premature closure of the metopic suture is a rare form of craniosynostosis, representing about 4% of clinically diagnosed synostoses. Accompanying this closure are defects of the head and face, particularly the upper face and orbits. To identify quantitatively the craniofacial dysmorphology associated with metopic synostosis, 50 patients with a diagnosis of primary (nonsyndromal) metopic synostosis were examined using a battery of 24 anthropometric measurements from which 11 proportion indices were calculated. The data were compared to sex- and age-matched normal students and converted to standard (Z) scores before being analyzed using Student's t-test. The data indicate a complex pattern of dysmorphology arising from the synostosis which affects the upper face and orbits as well as the cranial vault. The entire fronto-orbito-zygomatic complex is narrowed, and vertex is reduced. There is compensatory sagittal and transverse growth of the posterior neurocranium and compensatory vertical and sagittal growth of the upper face. There are statistically significant differences in the pattern of dysmorphology between patients presenting prior to 6 months of age and those older but not significant differences between sexes.

Age Factors↗