Congenital Bilateral Proximal Radio-Ulnar Synostosis in a Nigerian Child

Congenital radio-ulnar synostosis (CRUS) is a rare skeletal malformation of the upper limb and the most common congenital functional disorder of the elbow joint, causing limitation in forearm rotational movements, which may lead to difficulties with some activities of daily living. We reported a 4-year-old girl with congenital bilateral proximal radio-ulnar synostosis who presented with functional discomfort due to limitation of the prono-supination movements of the forearms and abnormal gestures when handling objects. She has clinical and radiological features of congenital radio-ulnar synostosis (CRUS). However, the parents declined corrective surgery because of wrong perception of the condition to be normal. This case highlighted the poor health seeking behavior and wrong illness perception prevalent in the developing countries.

Unusual presentation of congenital radioulnar synostosis with osteoporosis,fragility fracture and nonunion:A case report and review of literature

BACKGROUND Congenital radioulnar synostosis(CRUS)is a rare deformity of the upper extremity.It is characterized by loss of rotation of the involved forearm and functional limitations in daily activities.No studies on CRUS with osteoporosis have been reported to date,and osteoporosis is usually recognized as an important dimension of genetic disorder in children.We discuss the possible relationship among this disorder,osteoporosis and fracture nonunion,investigate the strict surgical indications and recommended treatments.CASE SUMMARY A 14-year-old male patient with bilateral CRUS with osteoporosis,fragility fracture and nonunion of fractures in ulna and radius presented our institution for further treatment,complaining of limitation in rotation.The bone mineral density of the hip and lumbar spine was 0.687 g/cm2 and 0.705 g/cm2,respectively,and the Z-score for both was-2.1,which revealed osteoporosis and a high risk of fracture.Tow serum bone turnover markers indicated an imbalance of bone metabolism.Reoperation for ulna fracture with autogenous bone grafting and a postoperative physiotherapy program were adopted rather than the separation of pathological synostosis.Radiological examination,observational posture assessment and limb function scale were evaluated before and 1 year after surgery.At 1 year,the fracture nonunion had almost recovered,forearm movement function on the fracture side was restored,and function on the healthy side was significantly improved compared with that before rehabilitation.CONCLUSION Surgical indications for CRUS vary from person to person.Surgery should not be the first choice of treatment,and physiotherapy is not inferior to surgical treatment.

Influence of distal tibiofibular synostosis on ankle function

Objective： To study the influence of distal tibiofibular synostosis on ankle function. Methods： Prom October 1998 to October 2004, a total of 281 consecutive patients underwent operations because of ankle fractures or distal fractures of the tibia and fibula. Distal tibiofibular synostosis occurred after operation in 8 patients. The duration of follow-up averaged 20.6 months （14-44 months）. The ankle function was assessed on the basis of functional rating system described by Mazur. Results： According to Mazur＇s ankle evaluation system, 4 patients achieved an excellent result, 2 a good result and 2 a fair result. The dorsiflexion of the synostosis ankle reduced by 8.26 degrees as compared with that of the contralateral ankle, and there was little influence on the plantar flexion. All the patients had a normal gait. Conclusion： The distal tibiofibular synostosis after the operation of ankle fractures or distal fractures of the tibia and fibula usually gives rise to few symptoms and needs no specific treatment.

An iatrogenic proximal radioulnar synostosis： a case report and review of literature

The most common cause of proximal radioulnar synostosis in adults is traumatic, usually after forearm fractures. Disabling complications are mainly loss of rotatory movements of the forearm. Various surgical procedures have been described in the literature to end up in forearm synostosis as a complication. We here presented a rare case of proximal forearm synostosis following a common but improper surgical technique for an olecranon fracture complicated by implant infection. The synostosis was treated by resection and fascia lata interposition graft.

Methods to shorten the duration of an external fixator in the management of tibial infections

Massive segmental bone loss due to chronic osteomyelitis represents a considerable challenge to orthopedic surgeons and is a limb threatening condition.The only option available in such a clinical situation is segment transport using the Ilizarov technique of distraction osteogenesis;yet the most common problem in cases of bone transport with the Ilizarov technique in massive bone loss,is the long duration of the fixator.In addition to autologous bone grafting,several mechanical,biologic,and external physical treatment modalities may be employed to promote bone formation and maturation during segment transport in osteomyelitis patients.Mechanical approaches include compressive loading of the distraction regenerate,increased frequency of small increments of distraction,and compression-distraction.Intramedullary nailing and hemicorticotomy can reduce the time in external fixation;however,these techniques are associated with technical difficulties and complications.Exogenous application of low-intensity pulsed ultrasound or pulsed electromagnetic fields may shorten the duration of external fixation.Other promising modalities include diphosphonates,physician-directed use(off-label use)of bone morphogenetic proteins,and local injection of bone marrow aspirate and platelet gel at the osteotomy site.Well-designed clinical studies are needed to establish safe and effective guidelines for various modalities to enhance new bone formation during distraction osteogenesis after segment transfer.

Diagnosis,treatment and complications of radial head and neck fractures in the pediatric patient

Radial head and neck fractures represent up to 14%of all pediatric elbow fractures and can be a difficult challenge in the pediatric patient.In up to 39%of proximal radius fractures,there is a concomitant fracture,which can easily be overlooked on the initial standard radiographs.The treatment options for proximal radius fractures in children range from non-surgical treatment,such as immobilization alone and closed reduction followed by immobilization,to more invasive options,including closed reduction with percutaneous pinning and open reduction with internal fixation.The choice of treatment depends on the degree of angulation and displacement of the fracture and the age of the patient;an angulation of less than 30 degrees and translation of less than 50%is generally accepted,whereas a higher degree of displacement is considered an indication for surgical intervention.Fractures with limited displacement and non-surgical treatment generally result in superior outcomes in terms of patient-reported outcome measures,range of motion and complications compared to severely displaced fractures requiring surgical intervention.With proper management,good to excellent results are achieved in most cases,and long-term sequelae are rare.However,severe complications do occur,including radio-ulnar synostosis,osteonecrosis,rotational impairment,and premature physeal closure with a malformation of the radial head as a result,especially after more invasive procedures.Adequate follow-up is therefore warranted.

Carpal Coalitions;Failures of Differentiation of the Carpus: A Description of Cases

Synostosis of carpal bones originates from lack of cavitation at the site of the future joint space with subsequent chondrification and ossification during the 4th to 8th weeks of intrauterine life. It is mostly seen as a chance finding discovered on radiographs. These coalitions are often asymptomatic, but can give complaints after trauma. We report six patients with different congenital carpal coalitions. These cases include synostoses of: scaphoid and trapezium, scaphoid and trapezium with a large scapholunate distance, lunate and triquetrum, capitate and trapezoid, capitate and hamate, triquetrum and pisiform. We also give a review of the literature and treatment proposal.

Filamin B:The next hotspot in skeletal research?

Filamin B （FLNB） is a large dimeric actin-binding protein which crosslinks actin cytoskeleton filaments into a dynamic structure. Lip to present, pathogenic mutations in FLNB are solely found to cause skeletal deformities, indicating the important role of FLNB in skeletal development. FLNB-related disorders are classified as spondylocarpotarsal synostosis （SCT）, Larsen syndrome （LS）, atelosteogenesis （AO）, boomerang dysplasia （BD）, and isolated congenital talipes equinovarus, presenting with scoliosis, short- limbed dwarfism, clubfoot, joint dislocation and other unique skeletal abnormalities. Several mecha- nisms of FLNB mutations causing skeletal malformations have been proposed, including delay of ossi- fication in long bone growth plate, reduction of bone mineral density （BMD）, dysregulation of muscle differentiation, ossification of intervertebral disc （IVD）, disturbance of proliferation, differentiation and apoptosis in chondrocytes, impairment of angiogenesis, and hypomotility of osteoblast, chondrocyte and fibroblast. Interventions on FLNB-related diseases require prenatal surveillance by sonography, gene testing in high-risk carriers, and proper orthosis or orthopedic surgeries to correct malformations including scoliosis, cervical spine instability, large joint dislocation, and clubfoot. Gene and cell therapies for FLNB-related diseases are also promising but require further studies.