scholarly journals Total Hip Arthroplasty in a Patient with Mucopolysaccharidosis Type IVB

2021 ◽  
Vol 2021 ◽  
pp. 1-8
Author(s):  
Yannick N. T. van den Eeden ◽  
Niklas Unter Ecker ◽  
Holger Kleinertz ◽  
Thorsten Gehrke ◽  
Tobias M. Ballhause

Introduction. Morquio syndrome or mucopolysaccharidosis (MPS) type IV is a rare autosomal recessive lysosomal storage disease, characterized by abnormal metabolism of glycosaminoglycans associated with specific skeletal deformities, also known as dysostosis multiplex. Case Presentation. We present the case of a 23-year-old patient with advanced osteonecrosis of the femoral head (ONFH) on both sides due to Morquio syndrome. A diagnosis of mucopolysaccharidosis type IVB was made after extensive genetic profiling. The patient had the condition for a long time. At 7 years old, the patient was treated with bilateral pelvic Salter’s osteotomy. Afterward, the patient was able to walk freely but could never take part in sports. At 22 years old, pain in the hip increased, and magnetic resonance imaging showed a bilateral femur head necrosis. Hence, the patient underwent cementless total hip arthroplasty (THA). Intraoperatively, a periprosthetic fracture occurred. Therefore, revision surgery with internal fixation was performed on the next day. Postoperatively, a weight-bearing restriction of 20 kg on the left leg was imposed for 6 weeks. The patient made a full recovery and was able to move without residual complaints. Annual orthopedic evaluation in patients treated with surgical intervention is recommended. Discussion. Orthopedic challenges for mucopolysaccharidoses and corresponding bone alterations, known as dysostosis multiplex, involving trunk and limbs with typical radiological findings have been well described. The hip is invariably involved, with dysplasia affecting the femoral neck (coxa valga), femoral epiphysis (loss of sphericity, osteonecrosis), and a flared hypoplastic iliac wing. Symptomatic therapy consists, on the one hand, of a surgical procedure and, on the other hand, a variety of supportive measures. However, the management of joint replacement in lysosomal storage diseases has not been well reported. All patients with MPS should be considered at high risk for surgical intervention requiring anesthesia because of airway and cardiac disease manifestations. In the case of a need for THA, we recommend cemented stem fixation because of the overall poor bone quality in patients with Morquio syndrome.

2011 ◽  
Vol 2011 ◽  
pp. 1-2 ◽  
Author(s):  
S. O'hEireamhoin ◽  
T. Bayer ◽  
K. J. Mulhall

Children affected by mucopolysaccharidosis (MPS) type IH (Hurler Syndrome), an autosomal recessive metabolic disorder, are known to experience a range of musculoskeletal manifestations including spinal abnormalities, hand abnormalities, generalised joint stiffness, genu valgum, and hip dysplasia and avascular necrosis. Enzyme therapy, in the form of bone marrow transplantation, significantly increases life expectancy but does not prevent the development of the associated musculoskeletal disorders. We present the case of a 23-year-old woman with a diagnosis of Hurler syndrome with a satisfactory result following uncemented total hip arthroplasty.


2020 ◽  
Vol 106 (3) ◽  
pp. 52-62
Author(s):  
O.M. Kosiakov ◽  
P.V. Bulych ◽  
K.O. Hrebennikov ◽  
A.V. Myloserdov ◽  
Ye. V. Tuz ◽  
...  

Summary. Relevance. Total hip arthroplasty remains one of the most successful and cost-effective surgical interventions in modern medicine. This is proven by the ever-growing number of total arthroplasties worldwide, which reached 650,000 implantations in Europe only in 2018. In 2024, this number is expected to be 730,000 [1]. According to experts, from 15 to 25 percent of patients need a bilateral procedure [2, 8]. The experience of our Center allows us to recommend a successful one-stage replacement of both hip joints for patients with a number of medical and social comorbidities. Objective. The study is set out to highlight the possibilities of performing simultaneous bilateral arthroplasty of the hip joint in a group of patients with bilateral joint damage, as well as to substantiate the economic, medical and social benefits of this surgical intervention. Materials and Methods. We have analyzed the data from publications of our foreign colleagues. We also present our own observations – more than 100 cases over the past 11 years (2008-2019). Results. Based on data from foreign publications and our own observations, the undoubted advantages, features and limitations of this type of surgical intervention are shown. Complex cases of bilateral joint damage and the results of one-stage surgical treatment have been demonstrated. Conclusions. One-stage bilateral hip arthroplasty is the method of choice for symmetric lesions and should take its rightful place in the arsenal of modern orthopedics clinics. We continue to advocate simultaneous bilateral hip replacement for a selected group of healthy patients ≤65 years old. Thoughtful preoperative planning, surgical intervention lasting up to 90 minutes on each joint, careful postoperative monitoring, strictly controlled prevention of thromboembolic complications and active postoperative rehabilitation provide our patients with quick and safe functional recovery.


2015 ◽  
Vol 6 ◽  
pp. CMTIM.S12265 ◽  
Author(s):  
Joshua L. Gary

As the population ages, the incidence of osteoporotic fractures, including those of the pelvis and acetabulum, continues to rise. Treatment of the elder patients with an acetabular fracture is much more controversial than the treatment of younger patients with similar injuries, where prevention of posttraumatic arthritis and total hip replacement remains optimal to limit need for revision arthroplasty. Arthroplasty for fractures of the proximal femur is commonplace in an older population and is a mainstay of treatment to promote early mobilization and weight-bearing. However, even with acute total hip arthroplasty for a geriatric acetabular fracture, most surgeons do not permit immediate weight-bearing postoperatively. Therefore, controversy regarding optimal treatment of these challenging fractures persists. Four treatment options have emerged: nonoperative treatment with early mobilization, open reduction and internal fixation (ORIF), limited open reduction and percutaneous screw fixation, and acute total hip arthroplasty. The exact indications and benefits of each treatment remain unknown. This article serves as a review of these four treatments and the data existing to support them.


2019 ◽  
Vol 158 (02) ◽  
pp. 214-220
Author(s):  
Karl Philipp Kutzner ◽  
Alexander Meyer ◽  
Marie Bausch ◽  
Michael Schneider ◽  
Philipp Rehbein ◽  
...  

Abstract Background An inpatient hospital stay of up to 10 days after total hip arthroplasty (THA) is still common in Germany, mostly followed by inpatient rehabilitation. Internationally already widespread concepts for enhanced recovery are increasingly gaining popularity in Germany. Objectives The presentation of content and results of a newly implemented enhanced recovery concept in THA. Materials and Methods In this single-center, prospective observational study of a consecutive patient collective of a single surgeon, between January 2016 and July 2016, 103 short-stem THA patients were enrolled and treated using a newly introduced enhanced recovery concept. After 6 weeks and 6 months clinical examination was performed regarding function, pain, satisfaction and possible complications. Results The goal of discharge on day 4 after operation was reached in 61.2% of the patients with a mean postoperative inpatient stay of 4.9 days. After 6 weeks and 6 months respectively, excellent clinical results were achieved with high patient satisfaction. The complication rate was found to be low. Mean hemoglobin concentration decreased by 2.1 g/dl. A fissure of the femur below the implant healed conservatively applying no weight bearing for a total of 6 weeks. A pulmonary embolism that occurred during rehabilitation was also successfully treated. After 6 months one case showed a bursitis trochanterica. Conclusions Inpatient length of stay can be reduced by enhanced recovery concepts without increasing the risks to patients. Thus, in Germany these concepts will be applied increasingly.


2013 ◽  
Vol 2013 ◽  
pp. 1-5 ◽  
Author(s):  
Mitsuyoshi Yamamura ◽  
Nobuo Nakamura ◽  
Hidenobu Miki ◽  
Takashi Nishii ◽  
Nobuhiko Sugano

Introduction. The perforation and fracture of the femur during the removal of bone cement in revision total hip arthroplasty (THA) are serious complications. The ROBODOC system has been designed to selectively remove bone cement from the femoral canal, but results have not been reported yet. The purpose of our study was to evaluate the clinical and radiographic results of revision THA using the ROBODOC system for cement removal.Materials and Methods. The subjects comprised 19 patients who underwent revision THA using the ROBODOC system. The minimum duration of follow-up was 76 months (median, 109 months; range, 76–150 months). The extent of remaining bone cement on postoperative radiography, timing of weight bearing, and the complications were evaluated.Results. The mean Merle d’Aubigne and Postel score increased from 10 points preoperatively to 14 points by final follow-up. Bone cement was completely removed in all cases. Full weight bearing was possible within 1 week after surgery in 9 of the 19 cases and within 2 months in all remaining cases. No instances of perforation or fracture of the femur were encountered.Conclusions. Bone cement could be safely removed using the ROBODOC system, and no serious complications occurred. Full weight bearing was achieved early in the postoperative course because of circumferential preservation of the femoral cortex.


2014 ◽  
Vol 135 (2) ◽  
pp. 265-269 ◽  
Author(s):  
Anja Schaefer ◽  
Thilo Hotfiel ◽  
Johannes Pauser ◽  
Bernd Swoboda ◽  
Hans-Dieter Carl

10.29007/3lbz ◽  
2019 ◽  
Author(s):  
Morteza Meftah ◽  
Vinnay Siddappa ◽  
Jeffery Muir ◽  
Peter White

Computer-assisted navigation has the potential to improve the accuracy of cup positioning during total hip arthroplasty (THA) and prevent leg length discrepancy (LLD). The purpose of this study was to compare acetabular cup position and post- operative LLD after primary THA using posterolateral approach. Between August 2016 to December 2017, 57 THAs using imageless navigation were matched with 57 THA without navigation, based on age, gender and BMI. Post-operative weight-bearing radiographs were assessed using for anteversion, inclination and LLD. Goal for functional cup placement was 40° inclination and 20° anteversion based on preoperative weight bearing pelvic images. Functional LLD was measured as compared to pre- operative radiographs and contralateral side. Proportion of cups within Lewinnek’s safe zone, proximity to a pre-operative target of and the LLD >5 mm was assessed. The mean age was 54.9 ± 9.6 years (30 – 72) and 57.6 ± 12.5 years (20 – 85) in control and navigated groups, respectively. Mean cup orientation in the navigated group was 20.6°± 3.3° (17 - 25) of anteversion and 41.9°± 4.8° (30 - 51) of inclination, vs. 25.0°± 11.1° (10 - 31) and 45.7°± 8.7° (29 – 55) in control group, where were statistically significant (p=0.005 and p=0.0001), respectively. In the navigated group, significantly more acetabular cups were placed within Lewinnek’s safe zone (anteversion: 78% vs. 47%, p=0.005; inclination: 92% vs. 67%, p=0.002). There was no significant difference in mean LLD in navigation and control groups (3.1 ± 1.5 mm vs. 4.6 ± 3.4 mm, p=0.36), although fewer LLDs >5 mm were reported in the navigated group (7.1%) than in controls (31.4%, p=0.007). The use of this image-less computer-assisted navigation improved the accuracy with which acetabular cup components were placed and may represent an important method for limiting post-operative complications related to cup malpositioning and LLD.


2021 ◽  
Vol 59 (3) ◽  
pp. 344-350
Author(s):  
D. V. Volchenko ◽  
I. F. Akhtiamov ◽  
A. Yu. Terskov ◽  
O. A. Sozonov ◽  
M. N. Velichko ◽  
...  

Aim of the research – to evaluate the effectiveness, to analyze the complications and long-term results of total hip arthroplasty (THA) in patients with active psoriatic arthritis (PsA).Materials and methods. The results of THA were studied in 26 patients with PsA (19 men, 7 women; average age 54.8±3.1 years, from 38 to 56 years) up to 2 years after surgery. The diagnosis of PsA was established according to CASPAR criteria.Results. The visual analogue scale assessment showed a significant decrease in the intensity of pain in the hip joint in the postoperative period. The average DAPSA activity index before surgery was 25.9 (11–34), after 2 months – 31.3 (16–38), after 6 months – 30.5 (12–37), with a further decrease to 26.1 (12–35) by 24th month. The results of clinical and functional assessment according to Harris in 19 (73%) patients corresponded to an excellent level, in 7 (27%) – to a good level. No infectious, thromboembolic complications were detected. In 1 (4%) patient periprosthetic intraoperative fracture of the femur was diagnosed No new skin psoriatic elements in the field of surgical intervention was fixed.Conclusion. Our data indicate the effectiveness of THA in active PsA, provided that the requirements for careful planning of the intervention, prevention of possible complications and an individualized approach to rehabilitation are met.


2020 ◽  
pp. 112070002096700
Author(s):  
Suleman Qurashi ◽  
Matthew H Pelletier ◽  
Tian Wang ◽  
Nicholas Bramich ◽  
Jason Chinnappa ◽  
...  

Background: The aim of this study was to investigate total hip arthroplasty (THA) Morse taper pull-off strengths after impaction prior to cyclical loading compared to cyclical loading alone. The practical relevance of the experiment is to provide a perspective on what may be clinically satisfactory taper assembly given the spectrum of head tapping patterns used by surgeons, as well as compare traditional impaction performed in standard THA with alternate methods of taper engagement such as ‘ in situ assembly’ used in micro-invasive techniques. Methods: 36 taper constructs utilising a combination of cobalt-chrome alloy and ceramic-titanium alloy junctions were investigated in vitro in wet and dry conditions with cyclical loading of the constructs. Taper disengagement strengths with and without impaction were compared. Secondary investigation of the surface roughness of the heads and tapers was also assessed. Results: An impaction to a wet taper resulted in a greater pull off force compared to a dry taper with a CoCr head and taper combination. Impacting the head and dryness of the taper did not affect pull off forces of a ceramic femoral head on titanium taper. Pulling a head off a taper significantly alters the head surface roughness. Conclusion: Impaction of a taper does not provide any benefit over cyclical loading of a taper assembly alone for pull-off strength.


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