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, whi...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.展开更多
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 ...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.展开更多
文摘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.
文摘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.
