Proximal congenital radial-ulnar synostosis and synchondrosis; pathogenic concept and a new therapeutic method
نویسندگان
چکیده
BACKGROUND CONTEXT Proximal congenital radial-ulnar synostosis (PCRUS) is defined by the development before birth of a bony bridge between the radius and ulna, usually at the proximal level, which blocks forearm rotation. This anomaly is rarely reported in the medical literature, because of its low prevalence, and treatment usually yields unsatisfactory results. The most commonly used surgical interventions are: forearm repositioning osteotomies with derotation of the radius and ulna, segmental resections of the middle third of the radius with muscular interposition, resection of the synostosis with the interposition of fatty tissue, tendons or fascia lata and resection of the proximal radius along with the transfer of the distal extensor carpi ulnaris tendon on the lateral edge of the radius. PURPOSE To describe a new treatment method for PCRUS, which we based on a new pathogenic concept, and to present our preliminary results. MATERIALS AND METHOD Between 2011 and 2013 our team performed two myo-osteo-arthroplastic reconstructions of the elbow and forearm for PCRUS. The intervention involves the extraperiosteal stripping of the origins of the ventral forearm musculature, release of the interosseous membrane, resection of the proximal two thirds of the radius, reshaping of the synostosis, a double osteotomy of the ulna and the transfer and fixation of a proximal fibular graft, including the head with its articular cartilage, in place of the resected segment of the radius. RESULTS Our preliminary study reveals favorable postoperative results, in comparison with other published methods. At the latest follow-up, one case had -10 degrees of pronation and 68 degrees of supination, and the other had 10 degrees of pronation and 66 degrees of supination. CONCLUSIONS Compared with other techniques, myo-osteo-arthroplastic reconstruction may seem overly invasive. However, the extent of this intervention is mandated by the pathogenic concepts of helical distortion, muscular retraction and anomalous configuration of the interosseous membrane. Benign cases do not require surgery. When there is no helical distortion, the intervention may be limited to the transfer of the proximal extremity of the fibula for the infant and small child.
منابع مشابه
گزارش یک مورد نادر از نقصان مادرزادی استخوان اولنا
Introduction: Ulnar bone deficiency is one of rarest congenital anomalies of upper limb which is classified under longitudinal deficiencies of upper limb. Ulnar bone deficiency is classified to several subgroups and musculoskeletal deformities are very common in this abnormality. Hand always has abnormality in the involved limb. The presenting case has ulnar deficiency with humero-radial sy...
متن کاملDevelopmental anterior dislocation of the radial head resulting from a congenital solitary osteochondroma of the proximal ulna in an infant.
A 4-month-old female infant was brought to our office by her parents, who had noticed a lump on the child's right elbow. Examination revealed a hard, painless lump in the antero-external region that was not reducible in flexion-extension or in pronation-supination. Neither palpation nor passive motion produced pain. Preoperative radiographs revealed a bony mass in the anteroexternal region of t...
متن کاملBilateral Radial Ulnar Synostosis and Vertebral Anomalies in a Child with a De Novo 16p13.3 Interstitial Deletion
We describe an 8-year-old boy with developmental delay, clinical bilateral radial ulnar synostosis, Klippel-Feil anomaly, and other vertebral deformities who was found to have a de novo deletion of 114.5kb at 16p13.3. The deletion contains five genes and three miRNAs. The genes are E4F1, DNASE1L2, ECI1, RNPS1, and ABCA3; miRNAs are MIR3677, MIR940, and MIR4717. The specific deletion has never b...
متن کاملSurgical outcome of delayed presentation of congenital proximal radioulnar synostosis.
BACKGROUND Presentation of proximal radioulnar synostosis varies from cosmetic concerns with no functional limitations to significant pronation deformity which hampers activities of daily living. Surgical management must be considered based on the position of the forearm and functional limitations. We describe the surgical technique, results, and complications of excision of the radial head alo...
متن کاملSynostosis in Feet and Hands with Dysplasia of Elbows
In 1944, after a legal case of disputed paternity in Switzerland, Nievergelt reported three children with deformities like those ofthe alleged father. They had talipes with tarsal synostosis, dysplasia of the elbows with radio-ulnar synostosis and subluxation of the radial head, and dysplasia of the tibia and fibula with exostoses. So far as had been ascertained neither of the parents of the fa...
متن کامل