One Stage Bilateral Total Hip Arthroplasty in Siblings with Larsen Syndrome
Christian Merle1, Wenzel Waldstein2, Joseph D Lipman3, Maximilian F Kasparek4, Friedrich Boettner4, *
Identifiers and Pagination:Year: 2016
First Page: 569
Last Page: 576
Publisher ID: TOORTHJ-10-569
Article History:Received Date: 13/07/2016
Revision Received Date: 26/07/2016
Acceptance Date: 29/10/2016
Electronic publication date: 30/11/2016
Collection year: 2016
open-access license: This is an open access article licensed under the terms of the Creative Commons Attribution-Non-Commercial 4.0 International Public License (CC BY-NC 4.0) (https://creativecommons.org/licenses/by-nc/4.0/legalcode), which permits unrestricted, non-commercial use, distribution and reproduction in any medium, provided the work is properly cited.
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.
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.
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.
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.