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Year : 2020  |  Volume : 23  |  Issue : 10  |  Page : 1426-1430

Total hip arthroplasty in sickle cell disease patients in a developing country

1 Arthroplasty Division, Department of Orthopaedics and Trauma Surgery, National Orthopaedic Hospital, Igbobi, Lagos, Nigeria
2 Department of Orthopaedics and Traumatology, Faculty of Clinical Sciences, University of Uyo/University of Uyo Teaching Hospital, Uyo, Nigeria

Date of Submission31-Jul-2019
Date of Acceptance09-May-2020
Date of Web Publication12-Oct-2020

Correspondence Address:
Dr. M E Ugbeye
Arthroplasty Division, Department of Orthopaedics and Trauma Surgery, National Orthopaedic Hospital, Igbobi, Lagos
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/njcp.njcp_402_19

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Background: Osteoarthritis secondary to osteonecrosis of the femoral head is a common presentation in patients with sickle cell disease. Functional limitations with or without deformities from these complications of sickle cell disease often require Total Hip Arthroplasty (THA) to improve outcome. Aim: The aim of this study was to evaluate the postoperative outcome of THA for secondary osteoarthritis from osteonecrosis of the head of the femur in patients with sickle cell disease. Methodology: A retrospective study of outcome of THA in sickle cell disease patients between January 2010 and December 2015 is presented. Primary outcome was measured using the rates of complications and the Harris Hip scores at 6 weeks, 12 weeks, 6 months, and yearly. Results: A total of 68 THA in 56 patients with sickle cell disease was reviewed. The age range was 13–68 years, with a mean of 30.9 years. There were 15 males and 41 females (M: F ratio = 1:3). Approaches to the hip were direct lateral (64.3%), anterolateral (1.8%), and posterior (33.9%). 12 (21.4%) patients had bilateral total hip arthroplasty, and 44 (78.6%) had unilateral total hip arthroplasty. No patient required adductor tenotomy. Superficial surgical site infection was recorded in 7 (12.5%) patients. The postoperative functional outcome, measured by the Harris Hip score, was good or excellent in all patients, except 2 (2.9%) who required revision surgery following unacceptable results after primary hip arthroplasty. Conclusion: THA is a viable and effective treatment modality for sickle cell disease patients with osteonecrosis and secondary hip osteoarthritis. The choice of surgical approach does not affect the outcome.

Keywords: Osteoarthritis of the hip, osteonecrosis femoral head, post-operative outcome, sickle cell disease, total hip arthroplasty

How to cite this article:
Ugbeye M E, Lawal W, Ayodabo O, Dim E M, Adegoke S. Total hip arthroplasty in sickle cell disease patients in a developing country. Niger J Clin Pract 2020;23:1426-30

How to cite this URL:
Ugbeye M E, Lawal W, Ayodabo O, Dim E M, Adegoke S. Total hip arthroplasty in sickle cell disease patients in a developing country. Niger J Clin Pract [serial online] 2020 [cited 2022 Dec 10];23:1426-30. Available from:

   Introduction Top

Sickle cell disease is an autosomal-recessive disorder characterized by presence of hemoglobin S. In homozygous states, the result is hemolytic anemia on account of sickle shaped red blood cells in an environment of low oxygen tension.[1] This is prevalent among black people in Africa. The heterozygous variant is the sickle cell trait (Hemoglobin AS). Other forms of the disease include the Hemoglobin SC and the sickle cell thalassemia (S-Thal). Homozygous hemoglobin SS individuals have a high risk of bone osteonecrosis resulting from microvascular occlusion. The incidence of osteonecrosis is also high in patients with heterozygous hemoglobin SC, and in the various types of sickle-beta-thalassemia (S-Thal).[1],[2],[3]

Osteonecrosis of the femoral head is a common outcome of microvascular occlusion following vaso-occlusive crisis. Hip symptoms are commonly seen in the second and third decades.[2],[4] These symptoms frequently present as disabling hip pain, associated with limb length discrepancy, joint fusion, and other abnormalities. THA remains a gratifying procedure in these patients, because it controls pain and improves functional status. Substantial improvement in health related quality of life and hip functionality[4],[5],[6] has been shown in patients who had THA. Postoperative hip function can be assessed using the Harris Hip Score,[4],[7] which has been validated against a number of other outcome measures, and found to be useful in assessing functional outcome.[8] A multidisciplinary approach to implementing effective preoperative treatment strategies increases the likelihood of a successful surgical outcome.[1],[9],[10] Intra-operatively, consideration of bone stock, bone quality, and method of component fixation may help minimize the risk of eccentric reaming, perforation, or fracture of either the acetabulum or the femur, and loosening.[2],[10]

The aim of this study was to determine the postoperative outcome of THA for secondary osteoarthritis from osteonecrosis of the head of the femur in patients with sickle cell disease.

   Patients and Methods Top

This is a 6-year retrospective study of post-operative outcome of THA in sickle cell disease patients between January 2010 and December 2015. We retrospectively reviewed 56 patients in whom 68 THAs were done for osteonecrosis of the femoral head from sickle cell hemoglobinopathy. All patients had Ficat and Arlet stage IV osteonecrosis of the femoral head with secondary osteoarthritis of the hip. Informed consent was obtained from all patients, after obtaining ethical clearance from the ethical review board of the institution. Case folders and radiographs were retrieved from the records department of the hospital, and required data obtained from them (Approval for the study was obtained from the ethical review board of the institution in July 2017.).

Patients' biodata, symptomatology and duration, functional status, and examination findings were recorded. The preoperative Harris Hip score, preoperative surgical preparation, and intraoperative findings were all retrieved. All patients received a dose of antibiotic prophylaxis at induction of anesthesia. The surgeries were done by four independent surgeons, using three different approaches (direct lateral, anterolateral, and posterior approach). Intraoperative details, including blood loss, duration of surgery, and implant size were documented. Postoperative treatment plan and protocols were noted, and complications recorded. Postoperative management included parenteral antibiotics, analgesics, and thromboprophylaxis using low molecular weight heparin (0.5–1 mg/kg/day) while on admission, and Rivaroxaban 10 mg/day after discharge for at least 6 weeks.

Patient mobilization was commenced on the second postoperative day, and guided weight bearing allowed with a walking frame. All patients underwent a standardized physiotherapy program while on admission and after discharge. Pain, hip function, and range of motion of the involved hips were evaluated at 6 weeks, 12 weeks, 6 months, and yearly using the Harris hip rating (Harris 1969).

The retrieved data was analyzed using the statistical package for social sciences software, version 24 (SPSS 24). Statistical significance of tested variables was assessed using the Chi-squared test. A P value <0.05 was considered statistically significant. Postoperative complications were evaluated, and overall outcome documented. Failure of Arthroplasty was defined as clinical or radiological loosening.

   Results Top

Sixty eight (68) primary THA were reviewed in 56 patients with sickle cell disease-related osteonecrosis of the femoral head and secondary osteoarthritis of the hip. There were 15 (26.8%) males and 41 (73.2%) females, giving an approximate male to female ratio of 1:3 (M: F = 1:3). The age range was 13–68 years with a mean of 30.9 years. 40 (71.0%) patients were homologous HBSS, while 16 (29.0%) were heterozygous HBSC.

Bilateral total hip arthroplasty was done in 12 (21.4%) patients. Out of this, 9 (75.0%) were females and 3 (25.0%) were males. 44 (78.6%) patients had unilateral total hip arthroplasty. Out of this, 28 (63.6%) was on the right hip and 16 (36.4%) on the left. 12 (27.3%) out of the unilateral total hip arthroplasty were males and 32 (72.7%) were females.

Presenting symptoms were varied, but all patients complained of hip pain associated with functional limitation. Other observed features included limping in 42 (75.0%) patients, limb length discrepancy less than or equal to 2 cm in 22 (39.3%) patients, limb length discrepancy more than 2 cm (3–8 cm) in 10 (17.9%) patients, positive Trendelenburg test in 11 (19.6%) patients and waddling gait in 1 (1.8%) patient. There was no limb length discrepancy in 24 (42.9%) patients. Ankylosis was seen in 3 (5.3%) patients, fixed flexion deformity of less than 5 degrees in 16 (28.6%), fixed flexion deformity of more than 5 degrees in 9 (16.1%), and hip abduction of less than 10 degrees in 41 (73%) patients. 42 (75.0%) patients did not use walking aid. 12 (21.4%) used walking sticks, while 2 (3.6%) were on wheelchair. The average duration of symptoms at presentation was 8 years (range 1–18 years).

All patients had radiographic features of osteonecrosis with secondary osteoarthritis of the hip. Additional radiographic findings were acetabular protrusio in 4 (7.1%) patients, joint fusion in 2 (3.6%), subluxation in 2 (3.6%), coxa vara in 2 (3.6%), and wide acetabulum in 1 (1.8%). 45 (80.4%) patients had no additional X-ray findings. The preoperative Harris hip score was 49 [Figure 1].
Figure 1: Preoperative Harris hip score

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Surgical approaches for the patients were direct lateral in 36 (64.3%), posterior in 19 (33.9%), and antero-lateral in 1 (1.8%). The prosthesis was cementless in 54 (96.4%) and cemented in 2 (3.6%) patients, respectively. 23 (41.1%) patients required acetabular augmentation with one or two screws. The sizes of the femoral stem components ranged from 6 mm to 12 mm, and the acetabular components from 46 mm to 54 mm. 52 (92.9%) patients were done under spinal anesthesia and remaining 4 (7.1%) under general anesthesia. The mean duration of surgery was 2.15 hours (range 1.5–3.2 h). The average blood loss was 1150 mls, with a range of 800–1500 mls. The average blood transfusion requirement was 4 units.

Different complications were recorded in 23 (41.1%) patients [Table 1]. The mean postoperative Harris hip scores at 6 weeks, 12 weeks, and 6 months were 101, 107, and 126.5, respectively. There was no statistically significant relationship between the surgical approaches and the postoperative Harris hip scores [Figure 2] and [Figure 3].
Table 1: Complications in 23 (41.1%) patients

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Figure 2: Bar chart depiction of relationship between surgical approach and postoperative Harris hip score at 12 weeks (Chi-squared = 1.234; P value = 0.507)

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Figure 3: Bar chart depiction of relationship between surgical approach and postoperative Harris hip score at 6 months (Chi-squared = 1.134; P value = 0.540)

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   Discussion Top

Sickle cell disease is a problem of the black race estimated to occur in over 30 million people world over, with the highest prevalence found among Africans, Arabs, and Indians.[9] Patients with sickle cell disease complicated by osteonecrosis of the femoral heads can have significant functional limitations. These limitations of activities of daily living are often sources of physical, social, financial, and emotional distress for the patient. As a result, patients are quite motivated to treat the source of this limitation, often a disabling hip pain. THA engenders improved functional outcome, and thus remains the appropriate surgical option for these patients, given the severe degenerative changes that occur after collapse. Most patients present late in our environment, with stage IV Ficat and Arlet, and occasional features to suggest joint fusion, acetabular protusio, and subluxation as depicted in this study.

The typical presentation is that of a young adult with homologous HBSS genotype, with disabling hip pain often associated with limb length discrepancy and a limp. Bilateral hip involvement is not uncommon at presentation, and accounted for 12 (21.4%) patients in this study. However, there is no uniform trend of bilateral hip involvement by sickle cell disease as can be deduced from other authors who have reported varying degrees of bilateral involvement. Whereas Katchy et al.[4] reported 8 (38.1%) bilateral hip affectations in 21 patients, Farook et al.[11] reported 4 (13.3%) bilateral total hip arthroplasty in 30 patients with sickle cell disease. Notwithstanding the bilateral involvement of the hips by osteonecrosis in sickle cell disease, clinical symptoms are usually more severe in one hip than the other.

The mean age at presentation in our study (30.9 years) was similar to the findings by Al-Mousawi et al.,[5] Farook et al.[11] and Nikose et al.[3] However, Katchy et al.[4] reported a younger mean age of 23.8 years in their series. The implication of this includes a tendency for more revision surgeries among the patient population of Katchy et al.[4] Our results of pre and postoperative hip scores, average operation time and blood loss were also similar to findings by these authors.[3],[4],[5],[11] Adequate preoperative preparation and planning is essential for good outcome. Preoperative templating was done for all patients in this study to determine appropriate prosthesis size and component position. This is a very important aspect of preoperative work-up aimed at achieving adequate planning of the surgery and ensuring optimal restoration of anatomic biomechanics in order to achieve lasting reconstruction.[12]

Notwithstanding the surgical approach, technical difficulties can still be encountered due to long-standing deformities, poor bone quality, and presence of marrow sclerosis.[2],[4],[11],[12] Marrow sclerosis has been shown to obliterate the femoral canal making its preparation quite difficult and hazardous, with a risk of perforating the medial femoral cortex.[2],[4],[11],[12] In our experience, we address proximal femoral canal narrowing by sequential reaming over guide wire, after initially opening the canal with 6 mm drill. Where large osteophytes and fibrous adhesions between the femoral head and acetabulum prevented dislocation of the femoral head, the femoral head was divided at the neck with an oscillating saw to prevent an iatrogenic proximal femoral fracture. Otherwise, fracture may occur following application of torsional force in an attempt to dislocate the hip at the early phase of the operation, or while preparing the narrow medullary canal.[13]

Also, average blood loss can be significant as shown in this study, in which we recorded an average blood loss of 1150 ml, with a range of 800–1500 ml. This is comparable to the results reported by Al-Mousawi,[13] but higher than that by Gulati et al.[2] Bleeding arises mainly from femoral canal preparation and dissection of fibrous tissues off the acetabular floor. Bleeding from femoral canal preparation is because of the chronic marrow hyperplasia found in sickle cell disease patients.

The revision rate in our study was low (2.9%), when compared with 21.4% in 35 THAs in 28 patients reported by Al-Mousawi et al.[5] and 25.0% in 29 THAs in 21 patients reported by Katchy et al.[4] Also, the infection rate in this study (12.5%) is lower than 36.4% reported by Bishop et al.,[14] and 16.0% by Hanker et al.[15] Hanker et al.[15] encountered a complication rate of 100% in these patients, making them to warn of the considerable risk of postoperative morbidity, with a recommendation that the risk should be carefully weighed against the benefit for each individual patient. Evidence in literature[3] indicate that previous reports[14],[15] showed high failure rate of THA in patients with sickle cell disease ranging from 31% to 66%, compared with more recent reports, which showed more promising results especially for cementless total hip arthroplasties.[3] Loosening rate of 94% was been reported in cemented hips, compared with 39% in uncemented hips of patients with sickle cell disease.[3] The revision rate of 2.9% in our study may be traceable to the use of cementless fixation in 54 (96.4%) out of our 56 patients with 68 primary hip arthroplasties. The use of cement is thought to cause thermal necrosis of the already infarcted bone, leading to a higher incidence of infection and loosening.[3]

Like in any other surgery for sickle cell disease, optimal result following THA for osteonecrosis secondary to hemoglobinopathy requires that adequate perioperative precautions be undertaken, including optimization of preoperative hemoglobin, use of appropriate prophylactic antibiotics, ensuring adequate preoperative and intraoperative hydration to avoid sickling, intraoperative oxygenation and caution in the use of bone cement. Intra- and postoperative management should focus on minimizing hypoxia, hypothermia, acidosis, and intravascular volume depletion.[9],[16]

The postoperative Harris hip scores in our patients were gratifying, showing most patients as having complete relief of pain, and marked improvement of functional status.

   Conclusion Top

THA is a viable and effective treatment modality for sickle cell disease patients with osteonecrosis and secondary hip osteoarthritis. Careful patient selection, adequate preoperative planning, discipline in intraoperative techniques, and post-operative management are important for good outcome.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of Interest

There are no conflicts of interest.

   References Top

Manzary M. Total hip arthroplasty in sickle cell disease. Reconstr Rev 2016;6:37-42.  Back to cited text no. 1
Gulati Y, Sharma M, Bharti B, Bahl V, Bohra I, Goswani A. Short term results of cementless total hip arthroplasty in sicklers. Indian J Orthop 2015;49:447-51.  Back to cited text no. 2
[PUBMED]  [Full text]  
Nikose SS, Nikose D, Saoji K, Shrivastava S, Dudhekar U, Pisulkar G. Total hip arthroplasty in sickle-cell disease: Comparing cemented and non-cemented options. J Health Sci Res 2019;4:11-6.  Back to cited text no. 3
Katchy AU, Anyaehie UE, Nwadinigwe CU, Eyichukwu GO. Total hip replacement in sickle cell disorder: A preliminary report of challenges and early outcome of 21 consecutive patients. Nigerian J Clin Pract 2018;21:492-5.  Back to cited text no. 4
Al-Mousawi F, Malki A, Al-Aradi A, Al-Bagali M, Al-Sadadi M, Booz MMY. Total hip replacement in sickle cell disease. Int Orthop 2002;26:157-61.  Back to cited text no. 5
Ilyas I, Alrumaih HA, Rabbani S. Non-cemented total hip arthroplasty in sickle-cell disease: Long-term results. J Arthroplasty 2018;33:477-81.  Back to cited text no. 6
Marchetti P, Binazzi R, Vaccari V, Girolami M, Morici F, Impallomeni C, et al. Long term results with cementless Fitek (of Fitmore) cups. J Arthroplasty 2005;20:730-7.  Back to cited text no. 7
Soderman P, Malchau H. Is the Harris Hip Score system useful to study outcome of total hip replacement? Clin Orthop Relat Res 2001;384:189-97.  Back to cited text no. 8
Adjepong KO, Otegbeye F, Adjepong YA. Perioperative management of sickle cell disease. Mediterr J Hematol Infect Dis 2018;10:e2018032.  Back to cited text no. 9
Jeong GK, Ruchelsman DE, Jazrawi LM, Jaffe WL. Total hip arthroplasty in sickle cell hemoglobinopathies. J Am Acad Orthop Surg 2005;13:208-17.  Back to cited text no. 10
Farook MZ, Awogbade M, Somasundaram K, Reichert ILH, Li PLS. Total hip arthroplasty in osteonecrosis secondary to sickle cell disease. Int Orthop 2019;43:293-8.  Back to cited text no. 11
Ayekoloye C, Oladiran AB, Omololu AB. Complex primary total hip replacement in a patient with sickle cell disease and contralateral poliomyelitis: A case report and review of literature. Nigerian J Orthop Trauma 2019;18:69-73.  Back to cited text no. 12
Al-Mousawi F. Complications and failures of hip replacement in sickle cell disease. Bahrain Med Bull 2004;26:1-8.  Back to cited text no. 13
Bishop AR, Roberson JR, Eckman JR, Fleming LL. Total hip arthroplasty in patients who have sickle cell heamoglobinopathy. J Bone Joint Surg 1988;70:853-5.  Back to cited text no. 14
Hanker GJ, Amstutz HC. Osteonecrosis of the hip in the sickle-cell disease: Treatment and complications. J Bone Joint Surg 1988;70:499-506.  Back to cited text no. 15
Carden MA, Fay M, Sakurai Y, McFarland B, Blanche S, DiPrete C, et al. Normal saline is associated with increased sickle red cell stiffness and prolonged transit times in a microfluidic model of the capillary system. Microcirculation 2017;24. doi: 10.1111/micc.12353.  Back to cited text no. 16


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