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Case Reports
. 2016 Nov 30:10:569-576.
doi: 10.2174/1874325001610010569. eCollection 2016.

One Stage Bilateral Total Hip Arthroplasty in Siblings with Larsen Syndrome

Christian Merle  1 , Wenzel Waldstein  2 , Joseph D Lipman  3 , Maximilian F Kasparek  4 , Friedrich Boettner  4
Affiliations
Case Reports

One Stage Bilateral Total Hip Arthroplasty in Siblings with Larsen Syndrome

Christian Merle et al. Open Orthop J. .

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.

Keywords: Computer tomography; Hip; Larsen syndrome; Osteoarthritis; Skeletal malformation total hip arthroplasty.

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Figures

Fig. (1)
Fig. (1)
Preoperative (A) postoperative (B) and 2-year postoperative follow-up radiographs (C) of a female patient with Larsen Syndrome.
Fig. (2)
Fig. (2)
Preoperative (A) and postoperative (B) lateral radiographs of both hips of the female patient.
Fig. (3)
Fig. (3)
Preoperative (A) postoperative (B) and 2-year postoperative follow-up radiographs (C) after one stage simultaneous total hip replacement of a male patient with Larsen Syndrome.
Fig. (4)
Fig. (4)
Preoperative (A) and postoperative (B) lateral radiographs of both hips of the male patient.
Fig. (5)
Fig. (5)
Computer tomography planning of reconstruction demonstrating a size 6 mm stem in the distal diaphysis (A) and a 11mm sleeve in the proximal femur (B). The final planned femoral reconstruction is shown in a three dimensional image (C).
See this image and copyright information in PMC

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References

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