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A case of post-traumatic proximal radio-ulnar synostosis in a young adult is described. Treatment was by excision of 1 cm of the shaft just below the synostosis and interposition of a radial head silicone implant. The functional result was excellent after 10 years.
One hundred sixty-seven forearm fractures treated with plate osteosynthesis were analyzed for synostosis occurring between the radius and the ulna. Eleven cases (6.6%) were found, in 7 of which both bones of the forearm had been fractured, while the other 4 were cases of isolated radial or ulnar fracture. Overall there were 89 cases of fracture of both bones; of the 65 cases treated utilizing the two-incision approach, cross-union was seen in only 1, while in a comparable group (12 cases) in which the fractures were stabilized using the Boyd approach, there were 5 synostoses. In 38 cases of isolated ulna fracture, only 2 synostoses were seen one associated with severe soft-tissue trauma and the other arising after treatment with a modified Boyd approach. Among 40 isolated radial fractures, 2 radioulnar synostoses occurred, one in an open fracture and the other after fixation via the Boyd approach. These results emphasize the importance of using two approaches for fractures of both forearm bones. The highest risk of post-osteosynthetic synostosis appears to be in fractures of the proximal third of both radius and ulna.
This report describes a newborn boy with Antley-Bixler (AB) syndrome. AB syndrome is a rare disorder characterized mainly by craniosynostosis and multiple joint contractures including radiohumeral synostosis. Our patient differs from the usual presentation by the presence of radioulnar rather than a radiohumeral synostosis. In addition, the child had a FGFR1 1300T mutation, which has not previously been associated with AB syndrome. Thus, our patient presents unique features and represents another argument in favour of an AB-like spectrum rather than a single syndrome.
Proximal tibiofibular synostosis without multiple hereditary exostosis is extremely rare and only 7 cases have been reported in the literature. All of the previously reported cases accompanied deformities such as distal positioning of the proximal tibiofibular joint, leg length discrepancy, bowing of the fibula, and valgus deformity of the knee. The present case of a 24-year-old man had neither a history of trauma nor deformity around the knee. Therefore, it was suggested that this type of synostosis occurred after epiphyseal plate closure.
Congenital synostosis between the fourth and fifth metacarpals is a rare congenital anomaly. This article reports the clinical features, hand pattern profiles, and dermatoglyphics of 14 patients with congenital synostosis between their fourth and fifth metacarpals. Surgical procedures are discussed.
Two cases of posttraumatic radioulnar synostosis treated with excision and low-dose radiation are presented. Routine postoperative hand therapy was employed. Nearly full range of motion was restored in both cases. No wound healing problems were encountered. We suggest that low-dose radiation may be a useful method of prophylaxis against recurrence after excision of radioulnar synostosis.
PURPOSE OF THE STUDY: Congenital radio-ulnar synostosis blocks the wrist in a position of more or less pronounced pronation. The degree of pronation and possible bilateral involvement can compromise upper limb function. We propose percutaneous osteoclasis of the antebrachial skeleton to remedy this situation PATIENTS AND METHODS: We performed 12 percutaneous rotation osteoclasis procedures. The objective was to weaken the metaphysodiaphyseal cortical periosteum of one or both of the forearm bones by intermittent perforations. This enabled derotation of the wrist which was maintained for six to eight weeks in a brachiopalmar cast. RESULTS: Mean correction of pronation was 51 degrees. There were no cases of vascular or nervous complications and healing was uneventful. The esthetic outcome was very satisfactory. DISCUSSION: Surgery is not indicated for all cases of synostosis. We retain for surgery patients presenting pronation at 60 degrees or more. At this degree of pronation, function is greatly compromised in adolescence, particularly if there is a bilateral involvement. Compared with other techniques, percutaneous osteoclasis is a simple safe and reliable technique. Reoperation is not required to remove material. The procedure is easier in young children, preferably at the age of 3 to 7 years, before the development of a functional handicap.
Congenital radioulnar synostosis can be severely disabling, especially if it is bilateral or if severe hyperpronation exists. Functionally, patients with severe deformity have trouble getting a cup to the mouth, using eating utensils, or accepting objects in an open palm. Of 33 patients (17 bilateral and 7 unilateral) underwent derotational osteotomy, with the majority being performed through the synostosis held with, an intramedullary wire and secondary transfixing device. There were eight complications, four involving neurovascular compromise. In bilateral cases, the best end position appears to be 10% to 15% of pronation in the dominant extremity and neutral in the other. Eighty-two percent of the patients had good or excellent results.
The authors have treated a case of bilateral fracture of the scaphoid bone in a young man who had complete and bilateral lunato-triquetral synostosis, without any other associated congenital condition. Consolidation was obtained by orthopedic treatment on one side, but a Matti-Russe operation was necessary on the other. The authors emphasize but then rule out the hypothesis of a congenital bipartite scaphoid associated with the synostosis. A review of the literature on lunato-triquetral synostoses sums up the main points. The condition is rare in whites and occurs more frequently in blacks. It is sometimes associated with other congenital conditions. Four anatomic and radiologic types have been described. The pathogenic hypotheses, based on embryology, are summarized.
The Saethre-Chotzen syndrome (SCS) is an autosomal dominant craniosynostosis syndrome with uni- or bilateral coronal synostosis and mild limb deformities. It is caused by loss-of-function mutations of the TWIST 1 gene. In an attempt to delineate functional features separating SCS from Muenke's syndrome, we screened patients presenting with coronal suture synostosis for mutations in the TWIST 1 gene, and for the Pro250Arg mutation in FGFR3. Within a total of 124 independent pedigrees, 39 (71 patients) were identified to carry 25 different mutations of TWIST 1 including 14 novel mutations, to which six whole gene deletions were added. The 71 patients were compared with 42 subjects from 24 pedigrees carrying the Pro250Arg mutation in FGFR3 and 65 subjects from 61 pedigrees without a detectable mutation. Classical SCS associated with a TWIST 1 mutation could be separated phenotypically from the Muenke phenotype on the basis of the following features: low-set frontal hairline, gross ptosis of eyelids, subnormal ear length, dilated parietal foramina, interdigital webbing, and hallux valgus or broad great toe with bifid distal phalanx. Functional differences were even more important: intracranial hypertension as a consequence of early progressive multisutural fusion was a significant problem in SCS only, while mental delay and sensorineural hearing loss were associated with the Muenke's syndrome. Contrary to previous reports, SCS patients with complete loss of one TWIST allele showed normal mental development.
Bone marrow failure syndromes can be associated with abnormalities of the forearms. We observed a neonate with congenital thrombocytopenia who had bilateral radio-ulnar synostosis and fifth finger clinodactly. We performed an evaluation of the mechanism causing the thrombocytopenia using a combination of direct and indirect measures of thrombopoiesis. These tests indicated decreased platelet production. This entity of congenital hyporegenerative thrombocytopenia with bilateral radio-ulnar synostosis and fifth-finger clinodactly is an uncommon but easily recognizable form of congenital amegakaryocytic thrombocytopenia (CAMT). This entity can be distinguished from the TAR syndrome (thrombocytopenia and absent radii) by the distinctive orthopedic issues, different underlying genetic mutations, and a more worrisome prognosis for CAMT than for TAR.
PURPOSE: The purpose of this study was to describe the results of a newly developed method of correction osteotomy for congenital radioulnar synostosis. With this method the osteotomy is performed at the distal one third of the radius and proximal one third of the ulna. After K-wires are inserted intramedullarly into both bones the forearm is derotated manually to the position planned before surgery followed by cast immobilization. METHODS: Four patients with an average age of 3.9 years underwent surgery with this method and were followed-up for 45.8 months. All of their forearms were fixed before surgery at over 70 degrees of pronation. RESULTS: The average correction after surgery was 65 degrees and bone union occurred at 8 weeks after surgery without any complications. The patients' ability to perform daily activities showed a marked improvement after surgery, but there was a 20 degrees loss of correction during cast immobilization in one case. CONCLUSION: This method is a simple and safe technique to derotate the forearms of the patients with congenital radioulnar synostosis that are fixed in pronation.
The radiological criteria of juvenile, rheumatic, cervical synostosis discovered in adult life are described and illustrated. These include: involvement of few or many segments, a tendency to bony ankylosis of the diseased intervertebral joints, dysplasias or hypoplasia of the vertebral body and intervertebral disc and dysplasias of the neural arches and hypoplasia of the transverse processes. Pathological ossification may involve the ligamentum flavum, the annulus, or the entire disc. The differential diagnosis of juvenile, rheumatic, cervical synostosis includes congenital block vertebrae, Klippel-Feil syndrome, acquired block vertebrae, juvenile ankylosing spondylitis, synostosing, intervertebral osteochondrosis and myositis ossificans progressiva.
Radio-ulnar synostosis is a congenital bony union between radius and ulna, usually in the vicinity of the radial tuberosity. A family is described on whom this anomaly was inherited over three generations. The rare cases described in the literature were usually bilateral, in our family it was unilateral. The following aspects are discussed: the frequency of this condition, the anatomical localisation of the synostosis, their types, local soft tissue and other abnormalities, embrylogy and the hereditory pattern. Finally, methods of treatment are discussed briefly.
Posttraumatic bone formation between radius and ulna can limit forearm rotation considerably. Recurrence after resection of synostoses is likely to develop if the bony surfaces are not covered by gliding soft tissue that is well vascularised. The interposition of a fascial forearm flap pedicled on the septal vessels of the posterior interosseous artery is suitable for this particular purpose. We report on the case of a spontaneous radioulnar synostosis by cartilaginous exostoses and its treatment by resection and interposition of a vascularised fascial flap. Forearm rotation was restored to normal. Until two years postoperatively there has been no recurrence of the synostosis.
Among 13 patients with congenital proximal radioulnar synostosis the chromosomal analysis revealed a 47, XXY-constellation in an 8 years old boy and a 47, XXX-syndrome in a 12-year-old girl. The investigations show, that the congenital radio-ulnar synostosis may be combined with the chromosome aberration more often than it was commonly thought.
Why do the craniofacial bones grow asymmetrically in patients with plagiocephaly (unilateral coronal synostosis)? We obtained three-dimensional skeletal replicas of two patients with the condition and analysed the deformities of the facial bones. From this analysis we deduced that the asymmetric deformation of the facial bones in these patients was caused by a combination of three rotations: rotation of the calvaria toward the affected side because of premature synostosis of the coronal and sphenofrontal sutures; rotation of the facial bones on the horizontal plane toward the unaffected side caused by anterior displacement of the TM (temporomandibular)-joint on the affected side; and downward rotation of the facial bones toward the unaffected side caused by inferior displacement of the TM-joint on the affected side.
Two cases of posttraumatic radioulnar synostosis are presented. The patients were treated with excision of the cross-union and interposition of a free vascularized fat transplant. A newly devised pronation-supination dynamic splint was employed for 3 months postoperatively in both patients. After a 1-year postoperative follow-up, an increased range of motion was restored in both cases, and there was no evidence of recurrent synostosis formation in subsequent radiographs. We suggest that an interposed vascularized fat graft may be an ideal biologic barrier to fill the space created by cross-union excision.