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Five years after an untreated ulnar fracture, a young man seen with no active or passive forearm rotation was found to have a radio-ulnar synostosis, which was surgically excised with interposition of a silicone membrane and soft tissue to decrease any tendency for new formation of the synostosis.
Radioulnar synostosis in a boy with XYY syndrome is discussed. Only four other cases of radioulnar synostosis with XYY syndrome have been reported in the literature.
Four cases of congenital radioulnar synostosis in three patients were treated by rotational osteotomy of the distal radius. The diaphysis of the radius was osteotomized transversely, and then the forearm was supinated manually. Following surgery, the palm was immobilized in a fully supinated position by a long-arm cast. The average age at surgery was 4 years 5 months old (range 3 years 11 months to 4 years 11 months), and the average follow-up was 21 months (range 12-36 months). Bone union was obtained in all patients without any complications or correction loss. Functional improvements were achieved in all patients. This procedure is simple and safe in the treatment of congenital radioulnar synostosis.
The management of three cases of traumatic radio-ulnar synostosis involved surgical excision of the synostotic bone followed by radiotherapy. Irradiation was commenced on the first postoperative day and was continued daily. The first patient received 20 Gy midline in 10 fractions and the second and third patients 10 Gy in five fractions. No acute side effects were observed. All three patients regained a good, functional range of forearm rotation with no evidence of recurrence of the synostosis after 2 years. This method of treatment is recommended as an alternative to other adjunctive therapies including interposition of material such as silicone sheet between the bones or peri-operative pharmacological suppression.
A 9-month-old neutered male cat was referred because of left forelimb lameness. Physical examination, laboratory analysis, and radiographic examination of the entire skeleton were performed. The radiographic diagnosis was bilateral radio-ulnar synostosis with secondary elbow malformation. A comparison between clinical and radiographic findings of the cat and those described in children affected by radio-ulnar synostosis is reported. Correlations and differences are discussed.
Syndactyly type I is an autosomal dominant condition with complete or partial webbing between the third and fourth fingers or the second and third toes or both. We report here a previously undescribed phenotype of severe mesoaxial syndactyly and synostosis in patients born to affected parents. The characteristic features of these severe cases are (1) complete syndactyly and synostosis of the third and fourth fingers; (2) severe bone reduction in the proximal phalanges of the same fingers; (3) hypoplasia of the thumbs and halluces; (4) aplasia/hypoplasia of the middle phalanges of the second and fifth fingers; and (5) complete or partial soft tissue syndactyly of the toes. We report on three offspring with this phenotype from two different branches of a syndactyly type I family, suggesting that they may be homozygous for this condition. SSCP and linkage analysis indicated that neither HOXD13 nor other relevant genes in the chromosome 2q31 region was responsible for this phenotype.
Congenital synostosis between the fourth and fifth metacarpals is a rare congenital anomaly. We present a case of type IIIB metacarpal synostosis according to the Buck Gramcko's classification with the camptodactyly of the little finger. It was treated successfully with the phalangeal angulation osteotomy and soft tissue release procedures.
We evaluated 27 children who had been operated on 5-10 years previously for sagittal synostosis. The mean age at operation was 0.55 years. Twenty-two children were treated by the senior author's technique, 4 by the Pi craniectomy technique and 1 by parasagittal sagittal strip craniectomies. The mean age at follow-up was 9.6 years. The cosmetic appearance of the head was graded independently by 3 examiners. Skull radiographs were graded by 2 examiners according to the appearance and presence of beaten copper markings. The cephalic index was measured. Frequent headaches were reported in 7 of 27 children and were migrainous in 3 of the 7. No child had papilledema. The children's appearance was considered to be normal or mildly abnormally by the parents in every case and by the medical observers in 25 of 27 (93%). The only 2 children with an unacceptable appearance were either operated late (3.3 years) or had a craniofacial syndrome. Skull radiographs revealed a normal or mildly abnormal appearance in 18-19 children, depending on the observer. Beaten copper markings were observed to some extent in 14 of 27 cases and did not correlate with the presence of headaches. The cranial index ranged from 62 to 78 with a mean of 70 (normal 81). Sagittal reconstructions resulted in an acceptable appearance 5-10 years postoperatively in all cases operated on at less than 1 year of age who had no syndrome. The need for long-term follow-up of children after sagittal synostosis operations cannot be determined from this sample size.
In craniosynostosis, as in all congenital diseases, the interaction of hereditary and neonatal environmental factors can be separated only in rare cases. Four siblings with an asymmetry of the posterior right portion of the skull are presented. The youngest of the four suffered from severe cyanotic congenital heart diseases and secondarily suffered from ischemic/hypoxic cerebral injury. This child had complete synostosis of the right lambdoid and posterior sagittal sutures by the age of 5 1/2 months. The cranial asymmetry was less pronounced in each of the older children and was nearly undetectable in the oldest, a 7 1/2-year-old girl. True synostosis was not present in any of the older siblings. It is suggested that an autosomal-dominant hereditary condition caused a hypotrophic mesenchymal relationship of occipitotemporal ontogenesis. This condition was compensated for through childhood by the molding effect of the brain in the older three siblings. The systemic disease and delayed cerebral growth in the youngest probably led to the complete picture of craniosynostosis.
The operative technique and result of treatment of traumatic radio-ulnar synostosis in two patients are described. In both, the treatment was excision of the cross-union and interposition of a free non-vascularised fat transplant. The functional result was excellent, and there was no evidence of regrowth of the synostosis at two and three years respectively.
A wide spectrum of anomalies can be associated with Pierre Robin sequence. This report presents a 3-day-old infant with micrognathia, U-shaped cleft palate, low-set right ear with microtia, glossoptosis, esophageal atresia, and right congenital radioulnar synostosis. The association of congenital radioulnar synostosis and esophageal atresia with Pierre Robin sequence has not been previously described.
A transmetatarsal pin was used as part of an Ilizarov apparatus to correct equinovarus deformity of a foot in a 7-year-old patient. The pin created synostosis between the metatarsal bones. The synostosis led to persistent flattening of the transverse arch of the foot, producing a painful gait. To avoid this problem, we recommend that the metatarsal wire be placed only through the first and fifth metatarsals rather than through the neighboring metatarsals.
Congenital radioulnar synostosis, which is caused by fusion between the proximal end of the radius and ulna, is an uncommon deformity of the upper extremity. It is characterized by the forearm being fixed in some degree of pronation and patients present with a variety of functional limitations of the involved limb. This condition may lead to difficulties in daily activities, such as writing, eating, and accepting objects in an open palm. Many operative procedures have been developed to mobilize the fixed forearm but the long-term results are disappointing. Herein, we reported a 12-year-old female patient with congenital radioulnar synostosis of the bilateral forearm who was treated with separation of the bony bridge between the left radius and ulna combined with a free groin flap transfer into the separated space. The functional results are good and no recurrent ankylosis occurred during a period of 28 months postoperatively. The flap size and volume of the subcutaneous fat are the key factors for satisfactory results.
Complex tibia nonunions and defects caused by tumor, trauma, and congenital abnormality are difficult to treat. The tibiofibular synostosis procedure is a treatment option. Fifteen patients with complex diaphyseal tibia fractures (4 segmental, 11 comminuted; 10 open, 5 closed) were managed with this procedure. Mean time between injury and procedure was 20.3 weeks. Mean healing time was 16 weeks (acceptable); 14 of 15 cases showed healing by final follow-up (36 months). At final follow-up, each patient's knee range of motion (ROM) and ankle ROM were acceptable. Knee ROM varied from full extension to 150 degrees of flexion for 13 patients and from full extension to 120 degrees of flexion for 2 patients. Ankle dorsiflexion ranged from 0 degrees to 40 degrees for 10 patients, from 0 degrees to 30 degrees for 3 patients, and from 0 degrees to 20 degrees for 2 patients. There were no radiographic arthritic changes in any case. The tibiofibular synostosis procedure is effective in treating complex nonunions and defects.
A case of proximal tibiofibular synostosis is presented along with a review of the literature. The variety of presenting complaints is discussed and the syndrome is compared with that of radio-ulnar synostosis. Possible modes of treatment are explained.
The late sequelae of an ankle sprain is described in the form of an acquired tibiofibular synostosis. A synostosis can result in loss of dynamic motion between the tibia and fibula, which may create decreased and painful ankle motion. A case report and surgical procedure is presented.
Hyperpronation deformity due to congenital radioulnar synostosis has previously been managed by derotational osteotomy through the synostosis, while stabilization has been achieved with K-wires and a long arm cast. The technique presented here uses a small external fixation device that allows precise rotational correction and affords adequate stabilization yet avoids cast immobilization.
Two members of a family affected with autosomal dominant multiple synostosis syndrome and malformations of various joints are presented, the most prominent signs being humeroradial synostosis and symphalangism. Moreover, dysplastic hip joints and familial conductive deafness as well as craniofacial abnormalities are observed. The present report may serve as an introduction to the complex radiological pattern in patients with hereditary brachydactyly syndrome.