One Stage Bilateral Total Hip Arthroplasty in Siblings with Larsen Syndrome

Abstract

Background:Larsen syndrome is a rare genetic disorder caused by congenital weakness of the connective tissues. It can present with a variety of musculoskeletal and cardiovascular abnormalities. The current report describes two siblings with Larsen Syndrome who presented with severe bilateral hip arthritis and underwent one stage bilateral total hip arthroplasty (THA). The aim was to report on the clinical features of Larsen Syndrome and their implications for total hip replacement surgery.Methods:Two siblings, a 32 year-old female and a 30 year-old male, presented with severe bilateral hip arthritis and a history of Larsen Syndrome. Both patients underwent a detailed, multidisciplinary preoperative medical work up and radiological imaging including computer tomography. All four hips were operated using a cementless primary press-fit cup (Pinnacle, DePuy, Warsaw, IN) and a cementless modular stem system (S-ROM, DePuy, Warsaw, IN) utilizing a posterior approach. Prophylactic cerclages wires were placed proximal to the lesser trochanter before stem preparation and bone grafting of bone cysts in the greater trochanter was performed in all four hips.Results:After 2 years both patients reported significant improvements of function, pain and quality of life. The Oxford hip score improved from preoperative 21 (range 12-24) points up to 39 (range 38-41) points at 2-year follow up. Radiographic follow-up showed good graft incorporation and no signs of implant loosening.Conclusion:The current case report suggests that one stage bilateral THA is a feasible treatment option for young adults with Larsen syndrome and secondary arthritis of the hip.

Staged Bilateral Total Hip Arthroplasty in a Patient With Larsen Syndrome

“…Hip-specific problems include subluxation and dislocation due to capsuloligamental hyperlaxity [ 4 ]. A recent case series described bilateral one-stage total hip arthroplasties (THAs) performed in 2 siblings with severe end-stage bilateral arthritis secondary to Larsen syndrome [ 14 ]. Two-year follow-up revealed improvement in function and quality of life, suggesting THA may be a viable option for patients with Larsen syndrome and resultant hip arthritis.…”

Histological features of articular cartilage and bone marrow reparative potential under conditions of coxarthritis in patients with radiographic signs of epiphyseal dysplasia

“…According to published information [1,3,4,[10][11][12], 5-29 % of the population have phenotypic signs of various types of collagenopathies with clinically expressed lesions of the musculoskeletal system, and osteoarthritis is steadily progressing on their basis. There is currently no etiological therapy for this impairment, which reduces the treatment of patients to slowing down the progression of osteoarthritis through symptomatic therapy with the timely application of radical treatment methods in order to preserve the social adaptation of patients [5,6,9,10,[13][14][15]. But the presence of signs of epiphyseal dysplasia in patients with osteoarthritis of the joints of the limbs is quite often overlooked by practicing doctors, which disorients the prospects for the use of conservative treatment methods and leads to unjustified postponement of effective surgical intervention [12,[15][16][17][18][19][20].…”