The incidence of orthopedic related problems in Lesch Nyhan syndrome patients and their management

Objective: to find out the incidence of orthopaedic related problems in lesch nyhan syndrome patients and their management place and duration of study: the study was conducted at john hopkins university from 1985 to 1993 during my post graduate training under supervision of dr. paul. d. sponsceller baltimore - usa patients & methods: seven were included in this study whose diagnosis was confirmed by bio mechanical assay showing complete absence of hgprt. five out of seven patients had hips surgery along with balancing of muscle forces by adductor and psoase lengthening. open reduction was performed in those hips where proper reduction was not possible by repositioning method. femoral osteotomy was only performed in patients older than five years and was fixed with blade plates. the orthopedic problems and there complication were studied. results: neuromuscular hip dysplasia was present in majority of patients it was unilateral in two patients and bilateral in three patients. the mean age for diagnosis of hip dysplasia was eleven years. one patient had posterior subluxation of knee joint after septic arthritis of knee joint and was treated by antibiotic. two patients had bilateral equinovarus deformity. mild degree of scoliosis was noted in two patients and both were flexible and no surgical intervention was done. conclusion: the results of surgical interventions are satisfactory in our opinion and every effort should be taken to make the life of lns patients comfortable,the caretaker should be educated properly as having pivot role and must be aware of incidence of re-fracture,postural adjustments,use of padding and prevention of bed sores.