بررسی تظاهرات بالینی و علائم رادیولوژیک سندرم لارسن

  Background : Larsen syndrome has complex clinical manifestations accompanying different congenital joint dislocations. Therefore, early diagnosis, correct treatment and prevention of complications are essential. This study was performed on 10 cases of Larsen syndrome and their clinical / radiological aspects were assessed.   Case report: In our review, there were 6 male (cases 1 to 6) and 4 female patients. The youngest and oldest patients at the time of referral were 3 and 51 years old, respectively. An interesting aspect is that hypertelorism and nose widening were detected in all cases. In spine, six patients had Spina bifida, whereas hip dislocation was also detected in eight cases. The significant points were high riding DDH in a 46- year old female patient and bilateral femoral head subluxation in a 51- year old male patient. Knee dislocation was seen in 9 patients. The above two cases had neglected knee dislocation, but acceptable knee function was reported. In upper extremities of eight patients, radial head posterior dislocation was seen. A 5- year old male patient had distal humerus physical deformity. All patients had 2nd metacarpal base deformity (double epiphysis in 2nd metacarpal bone).   Conclusion: Concerning multiple joints involvement in the syndrome, accurate physical examination is essential. Prioritization and staged management of the involvements is recommended.