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At least 271 records · Page 15Linked to original sources

Free adipofascial flap interposition for pediatric posttraumatic forearm synostosis with closed head injury.

A case report is presented of a 12-year-old male after a motor vehicle accident: head injury and multiple fractures, including bilateral both bone forearm fractures. The patient developed bilateral extensive forearm synostosis that required release and interpositional free flap at 6 months postinjury. At 3-year follow-up, the patient has maintained full forearm rotation and reports unrestricted sports and other recreational activities.

Child↗

Assessment and neurologic involvement of patients with cervical spine congenital synostosis as in Klippel-Feil syndrome: study of 19 cases.

We reviewed 19 children and adolescents with cervical spine congenital synostosis as in Klippel-Feil syndrome (KFS), with an average follow-up of 12.5 years. We paid particular attention to neurologic complications associated with cervical spine abnormalities. Five patients were affected by neurologic complications; four underwent a surgical procedure; and 14 had no neurologic finding. Two had hypermobility at one level, and one had hypermobility at two levels. We found that the more numerous the occipito-C1 abnormalities, the more significant the neurologic risk. In contrast, this risk was not related to the number of "mobile blocks" or to age. Various mechanisms of neural complications have been studied in the literature: medullary abnormality, spinal instability, narrowing of the cervical canal, and vascular dysfunction. Surgery is usually thought to be required in cases with neurologic complications. The indication for surgery is, however, less clear in cases of pure instability without neurologic involvement because surgery is likely to increase the future risks at mobile disks either above or below the fuse level. Careful clinical and radiologic observation is necessary in such patients. Magnetic resonance imaging (MRI) with lateral views in flexion and extension seem to be the best method for detecting impingement of the spine on the cord.

Adolescent↗

Proximal tibiofibular synostosis.

A case of proximal tibiofibular synostosis with a 10-year follow-up is presented. The lesion was documented roentgenographically when the patient was 3 years of age and when she became symptomatic at 13 years of age after vigorous running. The symptoms were successfully treated with custom-molded shoe orthotics.

Child, Preschool↗

Ulnar nerve palsy following rotational osteotomy of congenital radioulnar synostosis.

Two successive cases of ulnar nerve palsy following rotational osteotomy through a congenital radioulnar synostosis are described. Entrapment of the ulnar nerve by the fascia connecting the two heads of the flexor carpi ulnaris was documented in one patient. Recommendations to avoid this complication include shortening of the forearm at the osteotomy site and the release of unyielding soft tissue restraints. Rotational osteotomy at the distal radial metaphysis may provide a safe, alternative approach in the surgical management of this difficult problem.

Adolescent↗

The genetics of and associated clinical findings in humero-radial synostosis.

This paper compares the manifestations of sporadic, dominantly inherited and recessively inherited humero-radial synostosis with the aim of determining ways of separating these forms on clinical grounds. The genetic forms are characterized by bilateral involvement and by lack of the distal ulnar malformations and the absence of digits that are common in the sporadic cases. The majority of patients with the dominantly inherited form have a characteristic pattern of anomalies, including brachymesophalangy, and the recessive cases have a high frequency of malformations in addition to those of the limbs. Consanguinity is frequent in the families of recessive cases. Four additional patients are presented; two of them illustrate many of the features of the phocomelic syndrome reported by Herrmann et al. (1969). A possible teratogenic cause of these cases is discussed.

Abnormalities, Multiple↗

Management of antebrachial deformity and shortening secondary to a synostosis in a dog.

A nine-week-old labrador was presented with a deformed left forelimb causing obvious lameness. Radiographs suggested that the dog had suffered a fracture involving the radius and ulna which had healed resulting in a synostosis. There was a concurrent radial physeal injury. The dog was treated initially by osteotomy of the radius and ulna stabilised using a static external skeletal fixator. At a subsequent surgery lengthening of the limb was achieved by using an Ilizarov apparatus. Worsening carpal hyperflexion was managed by bandaging in forced extension. A successful outcome was achieved with a final result of a 24 per cent discrepancy in length between the two radii at 17 months of age. An increase in length of the ipsilateral humerus was seen in association with the shortened forelimb.

Animals↗

A locus for spondylocarpotarsal synostosis syndrome at chromosome 3p14.

Spondylocarpotarsal synostosis syndrome is a rare autosomal recessive disorder characterised by vertebral fusions, frequently manifesting as an unsegmented vertebral bar, as well as fusions of the carpal and tarsal bones. In a study of three consanguineous families and one non-consanguineous family, linkage analysis was used to establish the chromosomal location of the disease gene. Linkage analysis localised the disease gene to chromosome 3p14. A maximum lod score of 6.49 (q = 0) was obtained for the marker at locus D3S3532 on chromosome 3p. Recombination mapping narrowed the linked region to the 5.7 cM genetic interval between the markers at loci D3S3724 and D3S1300. A common region of homozygosity was found between the markers at loci D3S3724 and D3S1300, defining a physical interval of approximately 4 million base pairs likely to contain the disease gene. Identification of the gene responsible for this disorder will provide insight into the genes that play a role in the formation of the vertebral column and joints.

Carpal Bones↗

The clinical significance of bilateral synostosis of the lambdoid suture and the usefulness of its treatment.

Extended craniectomy has been employed in 148 +/- 4 month old infants with bilateral synostosis of lambdoid suture. Intraoperatively the ICP was less than 180 mm H2O in 6, 180-200 in 1, greater than 200 in 7. 18 +/- 10 months following surgery clinical signs as psychomotor retardation and so forth were present in 4 compared to 11 patients preoperatively. With 1 exception there was a distinct remodelling of neurocranium and an almost complete ossification of the operative site.

Cephalometry↗

Rotational osteotomies for congenital radioulnar synostosis.

In five children, six forearms with a fixed pronation deformity secondary to congenital radioulnar synostosis were treated by a derotation osteotomy of the distal radius and the midshaft of the ulna. There were three boys and two girls with a mean age of 4.9 years (3.5 to 8.25) who were followed up for a mean of 29 months (18 to 43). The position of the forearm was improved from a mean pronation deformity of 68 degrees (40 degrees to 80 degrees ) to a pre-planned position of 10 degrees of supination in all cases. Bony union was achieved by 6.3 weeks with no loss of correction. There was one major complication involving a distal radial osteotomy which required exploration for a possible compartment syndrome.

Child↗

Immediate correction of sagittal synostosis.

A technique for correction of sagittal synostosis with achievement of an immediately pleasing cosmetic result is presented. Even with replacement of bone and no attempt to inhibit bone union, premature reclosure does not occur. Moss' theory of dural tensions is discussed to explain the effect.

Humans↗

Humeroulnar synostosis: case report.

This is a report of a case of humeroulnar synostosis at the right elbow of a native Nigerian girl in whose family there is no history of genetic/hereditary diseases. This defect was not associated with any other deformity. This is a rare defect that has never been mentioned in literature. By excluding any causes, this case has been proved to be a failure of differentiation that is one of the major categories in the classification of congenital limb defects by Alfred B. Swanson.

Child, Preschool↗

Congenital talonavicular synostosis. A review of the literature and a case report.

The diagnosis of a congenital talonavicular coalition is one that often may be little more than an incidental radiographic finding. However, the acute onset of vague midfoot symptomatology secondary to a minor traumatic event may reveal this rarely reported condition on further clinical examination. The authors present a description of the characteristic historical and clinical features of talonavicular coalitions, current diagnostic and treatment modalities available, and a case presentation of an acute painful talonavicular synostosis in an adolescent male athlete.

Adolescent↗

Unilateral ectrodactyly, metatarsal synostosis, and hypoplasia in an Eskimo.

A unilateral foot deformity in an archaeological specimen from Point Hope, Alaska, is presented. Radiographic and gross examination of the bones of the malformed left foot suggests that this adult female suffered from a rare and possibly unique limb deficiency presenting as unilateral congenital absence of the phalanges, synostosis and hypoplasia of the metatarsals, and mild hypoplasia of the calcaneus.

Abnormalities, Multiple↗

Hereditary symphalangism with associated tarsal synostosis and hypophalangism.

Symphalangism is a rare genetic condition that may represent the earliest documentation of mendelian inheritance in man. The disorder results in interphalangeal joint fusion in the hands and feet. The authors review this rare condition and present a case study consisting of four generations with 15 affected family members. The association of multiple tarsal synostosis and the previously unreported associated occurrence of pedal hypophalangism in this pedigree is presented.

Adult↗