Total Hip Arthroplasty in Sickle Cell Hemoglobinopathies Gerard K. Jeong, MD, David E. Ruchelsman, MD, Laith M. Jazrawi, MD, and William L. Jaffe, MD Abstract Advances in medical therapy have led to improved life expectancy in patients with sickle cell hemoglobin- opathies. These patients present a unique set of preoperative, intra- operative, and postoperative chal- lenges. Early series reported a high failure rate of total hip arthroplasty (THA); 1-3 however, few other effec- tive treatment options are available. As techniques for THA evolve, a substantial number of patients with sickle cell hemoglobinopathies will become candidates for the proce- dure. Effective treatment requires understanding the disease patho- physiology, knowledge of the po- tential medical and surgical com- plications associated with the disease, and multidisciplinary man- agement. Pathophysiology Sickle cell syndromes have variable clinical manifestations. Patients range from asymptomatic, heter ozygous in- dividuals to severely debilitated, re- currently symptomatic homozygous patients with chronic end-organ ne- crosis and infarction. The three main features of the sickle cell syndromes are anemia, recurrent painful crises, and chronic organ dysfunction sec- ondary to infarction and necrosis. Pa- tients suffer chronic anemia not only from the intravascular hemolysis of rigid, sickled red blood cells but also because of the decreased hematopoie- sis caused by splenic infar ction. Pain- ful crises are precipitated by sickling of red blood cells under conditions of low oxygen tension and/or stress. End-organ infarction is the result of repetitive ischemic episodes. Musculoskeletal Manifestations Pyogenic Infections The three principle orthopaedic manifestations of the sickle cell hemo- globinopathies are pyogenic infec- tions, marrow hyperplasia, and os- teonecrosis. Pyogenic infections occur secondary to splenic autoinfarction. The r esulting functional asplenia leads to an increased susceptibility to Dr. Jeong is Resident, Department of Orthopaedic Surgery, NYU–Hospital for Joint Diseases Ortho- paedic Institute, New York, NY. Dr. Ruchelsman is Resident, Department of Orthopaedic Surgery, NYU–Hospital for Joint Diseases Orthopaedic In- stitute. Dr. Jazrawi is Assistant Professor, Depart- ment of Orthopaedic Surgery, NYU–Hospital for Joint Diseases Orthopaedic Institute. Dr. Jaffe is Vice Chairman and Clinical Professor, Department of Orthopaedic Surgery, NYU–Hospital for Joint Diseases Orthopaedic Institute. None of the following authors or the department with which they are affiliated hasreceived anything of value from or owns stock in a commercial com- pany or institution related directly or indirectly to the subject of this article: Dr. Jeong, Dr. Ruch- elsman, Dr. Jazrawi, and Dr. Jaffe. Reprint requests: Dr. Jeong, Hospital forJoint Dis- eases Orthopaedic Institute, 14th Floor, 301 East 17th Street, New York, NY 10003. Copyright 2005 by the American Academy of Orthopaedic Surgeons. Advances in medical treatment have led to improved life expectancy in patients with sickle cell hemoglobinopathies. Improved life expectancy has, in turn, led to an in- creasing number of patients with osteonecrosis requiring total hip arthroplasty. Pa- tient evaluation begins with understanding the extent of the disease process and the patient’s musculoskeletal manifestations (ie, pyogenic infection, marrow hyperpla- sia, osteonecrosis). A multidisciplinary approach to implementing effective preop- erative treatment strategies increases the likelihood of a successful surgical outcome. Intraoperatively, consideration of bone stock, bone quality, and method of compo- nent fixation may help minimize the risk of eccentric reaming, perforation or frac- ture of either the acetabulum or the femur, and loosening. The optimal mode of ac- etabular and femoral fixation in these patients has not been conclusively determined, but recent results of cementless total hip arthroplasty have been en- couraging. Although patients with sickle cell disease are at increased risk of medical and surgical complications, total hip arthroplasty in the appropriately selected pa- tient can provide significant pain relief, restoration of function, and patient satisfaction. J Am Acad Orthop Surg 2005;13:208-217 208 Journal of the American Academy of Orthopaedic Surgeons polysaccharide-encapsulated organ- isms, such as Streptococcus pneumoniae, Salmonella typhi, and Klebsiella pneu- moniae. 4-7 This immunodeficiency makes patients susceptible to bac- teremia and hematogenous seeding in their bones and joints. Although S typhi is the most characteristic organ- ism, Staphylococcus aureus is still the most common cause of osteomyeli- tis in the sickle cell population. 6 Patient age appears to play a sig- nificant role in the particular organ- ism that causes osteomyelitis. Chil- dren younger than 12 years are more susceptible to osteomyelitis fr om Sal- monella species, whereas adults are more commonly infected with staph- ylococcal or streptococcal osteomye- litis. 7 There are no reported cases of salmonella osteomyelitis complicat- ing a THA. Marrow Hyperplasia In patients with splenic insufficien- cy, chronic anemia stimulates in- creased erythropoiesis in the bone marrow. Marrow hyperplasia leads to widening of the medullary canal and thinning of the trabeculae and corti- ces, which are especially prominent in the metaphyseal area of long bones, such as the femur. 8 Marrow hyperpla- sia warrants substantial intraopera- tive technical considerations during THA in patients with sickle cell dis- ease. The changes in metaphyseal ge- ometry create suboptimal conditions for intramedullary reaming and for prosthesis fitting, insertion, and fix- ation. 6,9,10 Osteonecrosis Proximal Femur Osteonecrosis secondary to vascu- lar thr ombosis and infarction is a com- mon complication in patients with sickle cell syndrome. Microvascular occlusion from hypoxia-induced erythrocyte sickling, along with ex- travascular compression of the in- traosseous blood supply caused by medullary hyperplasia and hyperten- sion, results in ischemia and culmi- nates in bone infarction. 8 After re- peated episodes of marrow infarction, focal and patchy areas of sclerosis with canal obliteration may be found in the same femur adjacent to areas of medullary widening (Figs. 1 and 2). Recognizing these osseous changes is important when considering recon- structive procedures. Hip The incidence of osteonecrosis of the femoral head depends on the sickle cell genotype; the prevalence is 3% to 41% in patients with sickle cell disease, 8,11,12 20% to 50% in those with hemoglobin SC disease, 8 and 24% to 50% in patients with sickle β-thalassemia. 12 In some early se- ries, 1,13,14 hip involvement was re- ported to be more common in patients with mixed states, such as hemoglo- bin SC disease, than in those with sickle cell disease. This finding may reflect the fact that severe sickle cell disease pr eviously caused premature death before osseous changes could be noted. In a more recent series, Mil- ner et al 12 reported that osteonecro- sis of the femoral head is most likely to be seen in patients with sickle cell disease. Patient Evaluation and Natural History In patients with sickle cell hemo- globinopathies, osteonecrosis of the femoral head presents with signs and symptoms similar to those in patients with osteonecrosis resulting from other etiologies. Patients report groin pain and difficulty with ambulation. Physical examination reveals painful, limited range of motion of the hip. As a result of the systemic nature of the vaso-occlusion responsible for os- teonecrosis, hip involvement is often bilateral. 2,10,15 Hernigou et al 16 re- ported radiographic evidence of os- teonecrosis in the contralateral asymptomatic hip in 14 of 36 patients with unilateral symptomatic hip pain (39%). Nonsurgical Management The nonsurgical options for man- aging osteonecrosis caused by sickle cell disease consist of pain manage- ment with nonsteroidal anti-inflam- matory agents and/or opiates and the use of assistive devices. Without sur- gical intervention, symptomatic sick- le cell–induced osteonecrosis of the hip typically progresses, leading to femoral head collapse, advanced de- generative changes and, finally, to marked disability. 16 Hernigou et al 16 reviewed the natural history of symp- tomatic osteonecr osis of the hip treat- Figure 1 Anteroposterior radiograph of the femoral diaphysis in a patient with sickle cell disease demonstrating intramedullary sclero- sis, marrow hyperplasia, and thinning of the cortices. The focal patchy areas of sclerosis with canal obliteration are the result of repeat- ed episodes of marrow infarction. Gerard K. Jeong, MD, et al Vol 13, No 3, May/June 2005 209 ed nonsur gically in patients with sick- le cell hemoglobinopathies. They found that femoral head collapse (Steinberg stage IV) developed in all 43 symptomatic, radiographically in- volved hips (Steinberg stage II) at a mean of 30 months; in the 32 symp- tomatic hips with evidence of osteone- crosis on MRI only (Steinberg stage I), femoral head collapse occurred at a mean of 42 months. Clinical failure (ie, surgical intervention for intracta- ble pain) occurred in 96% of all hips at a mean of 48 months after the onset of symptoms for stage I hips, at 43 months for stage II hips, and at 10 months for stage III and IV hips. Re- sults of nonsurgical management have been poor because of the underlying nature of the disease, with persistent and progressive bony infarcts. Surgical Management The primary indication for surgi- cal management is intractable, dis- abling hip pain refractory to nonsur- gical treatment in the patient with a severely damaged hip joint. Surgical intervention may be contraindicated in patients with sickle cell disease who are not medically fit for surgery. Surgical options other than THA in- clude core decompression, femoral osteotomy, arthrodesis, hemiarthro- plasty, and resection arthroplasty. The utility of core decompression in the sickle cell patient remains controver- sial. Femoral osteotomy, arthrodesis, and hemiarthroplasty are not recom- mended, except in unusual circum- stances. Resection arthroplasty is rarely indicated in the primary set- ting and usually is reserved as a sal- vage procedure for failed THAs. No large, prospective studies with long-term follow-up have been pub- lished directly examining the effica- cy of core decompression for early- stage osteonecrosis (Steinberg stages I and II) of the hip in patients with sickle cell hemoglobinopathies. Styles and Vichinsky 17 reported symptom- atic improvement in 11 of 13 hips in 10 patients with sickle cell disease af- ter core decompression for precol- lapse stages of osteonecrosis of the femoral head (mean follow-up, 3.7 years), despite radiographic progres- sion in 5 hips. In two early small se- ries, failure occurred in all three hips that underwent core decompression for early osteonecrosis secondary to sickle cell disease. 10,18 Moran 19 sug- gested three reasons that cor e decom- pression in patients with sickle cell syndrome is likely to yield clinical and radiographic results inferior to those in patients with other nontrau- matic etiologies. First, although core decompression may alleviate elevat- ed intraosseous pressures secondary to marrow hyperplasia, it does not address the primary underlying pathophysiology of vaso-occlusion. Second, femoral head involvement usually is diffuse and too large to de- compress. Hernigou et al 16 found that >40% of the femoral head was in- volved at presentation in all stages of sickle cell–induced osteonecrosis. Fi- nally, the chronic nature o f the disease makes future infarcts inevitable de- spite decompression and angiogen- esis. 19 Femoral osteotomy is a poor op- tion for patients with sickle cell dis- ease because it does not stop the rap- id progression of hip disease and can relieve only focal areas of an infarcted femoral head. Long-term failure rates with this procedure increase with ex- tensive preoperative osteonecrosis, 20 and most patients with sickle cell dis- ease have >40% involvement irr espec- tive of the stage 16 (Fig. 3). Arthrodesis is rarely a viable op- tion for most patients with sickle cell syndrome because of the frequency of bilateral hip involvement and re- sultant shortening after débriding the avascular femoral bone down to vi- able areas required for fusion. Uni- formly poor results with hemiarthro- plasty attributed to both sepsis and protrusio have been reported in the sickle cell population. 10,21 Hemiarthroplasty often fails be- cause of the s oft acetabular bone stock Figure 2 Same patient as in Fig. 1. Gross specimen of the femoral diaphysis demon- strating the focal areas of intramedullary scle- rosis after repeated episodes of infarction. In patients with sickle cell disease, osteocyte in- farction can extend distal to the femoral head into the metaphysis and diaphysis, which may complicate reaming during femoral ca- nal preparation. Figure 3 Gross specimenofthefemoral head of a patient with sickle cell disease demon- strating diffuse sclerosis in the femoral head resulting from repeated infarctions. Total Hip Arthroplasty in Sickle Cell Hemoglobinopathies 210 Journal of the American Academy of Orthopaedic Surgeons caused by chronic marrow hyperpla- sia and the subsequent increase in mi- gration and protrusion of the prosthe- sis into the pelvis. 6,10 Resection arthroplasty is rarely in- dicated as a primary procedure be- cause it provides less potential ben- efit than THA and because of the frequent bilateral hip involvement associated with the disease. How- ever, when reserved as a salvage pr o- cedure after failed THA secondary to infection or extensive bone loss, re- section arthroplasty has yielded ad- equate results. 2 Total Hip Arthroplasty The primary indication for THA in patients with sickle cell disease who are medically fit to undergo elective surgery is persistent, intractable, dis- abling pain, referable to the hip joint, that is refractory to nonsurgical man- agement. Preoperative Considerations Because patients with sickle cell hemoglobinopathies usually have multisystem involvement, they often present a unique set of preoperative challenges. A multidisciplinary ap- proach involving specialists in hema- tology, infectious disease, cardiology, pulmonary and critical car e, anesthe- siology, and nutrition may help op- timize the patient’s medical condition preoperatively and minimize postop- erative complications. Preoperative concerns include (1) preventing sickle cell crises through avoidance of acidosis, appropriate fluid resuscitation, and adequate ox- ygenation; (2) paying careful atten- tion to cardiac status and fluid bal- ance (cardiomegaly and underlying congestive heart failure may be present in patients with chronic ane- mia); (3) assessing infection in other body parts that could spread to the prosthetic implant; (4) using preop- erative transfusion or plasmaphere- sis to improve oxygen-carrying ca- pacity; and (5) considering anesthetic modalities and pain management techniques for patients whomay have long-standing narcotic tolerance. A thorough preoperative evalua- tion of possible sources of infection is important because patients with sickle cell hemoglobinopathies suffer from an unusually high infection rate related to functional asplenia. A THA infection rate between 16% and 20% has been reported in patients with sickle cell disease. 2,3,9,13 Chronic sta- sis ulcers on the lower extremities, which are common in many patients with sickle cell syndrome, are a po- tential source of hematogenous spread to the THA. 1,21 For inactive and noninfected chronic stasis ulcers, Gunderson et al 1 recommended a pre- operative, prophylactic antibiotic r eg- imen consisting of a first-generation cephalosporin (cephalothin) for 24 hours before and 72 hours after THA. Any ulcer or lesion suspicious for in- fection should be managed effective- ly before surgery, even if doing so de- lays the operation. Because patients with sickle cell syndrome often har- bor latent Staphylococcus and Salmo- nella organisms in infarcted bone mar - row , 22 Ilyas and Moreau 23 recommend routine intraoperative bone cultures and histopathologic analyses to rule out osteomyelitis before implantation of the prosthesis. Ilyas and Moreau utilized an antibiotic regimen consist- ing of cefazolin and gentamicin, be- gun intraoperatively and continued postoperatively until intraoperative culture data were available. When culture results were negative, the an- tibiotics were discontinued; when positive, the regimen was adjusted per the sensitivity profile of the iso- lated organism(s). 23 Preoperative transfusion or plas- mapheresis should achieve a hemo- globin level >11 g/dL and a hemat- ocrit level >30%, with circulating levels of hemoglobin A >30% and of hemo- globin S <30%. 9 With deoxygenation, the rate at which hemoglobin S poly- mers form depends on the hemoglo- bin S concentration; thus, reduction in hemoglobin S levels may reduce sickle cell–r elated complications. How- ever, i n a series of 74 patients with sick- le cell syndrome undergoing prima- ry or revision THA, hemiarthroplasty, or core decompression, the rates of overall and sickle cell–related compli- cations did not differ significantly be- tween patients randomized to conser- vative (hemoglobin level >10 g/dL) and aggr essive (hemoglobin level >10 g/dL and hemoglobin S level <30%) transfusion regimens. 24 A more con- servative pr eoperative transfusion r eg- imen has been associated with fewer transfusion-related complications. 25 Because alloimmunization occurs in 20% to 30% of patients with sickle cell disease, phenotypically matched blood typically is used. Leukocyte-depleted blood usually is used in patients with a history of febrile reactions. Preop- erative autologous donation in patients with sickle cell syndrome who are un- dergoing orthopaedic procedur es may be useful in the alloimmunized pa- tient. Folate and recombinant eryth- ropoietin also may be administered. Epidural, spinal, and general an- esthesia are all routinely used for pa- tients undergoing THA. However, epidural anesthesia in this population may help prevent sickling and sub- sequent crisis via a temporary sym- pathectomy. 10 The peripheral vasodi- latation and decreased peripheral vascular resistance produced in the lower extremities necessitates main- taining arterial pressure by increas- ing the intravascular volume. The in- creased volume may result in greater perfusion to the distal extremities at a given arterial pressure and may pre- vent postoperative painful crises. Intraoperative Considerations Sickle cell hemoglobinopathies are characterized by widening of the medullary canal. Typical features in- clude thinning of the trabeculae and cortices (a result of marrow hyperpla- sia), patchy areas of sclerosis, and ca- nal obliteration (caused by osteone- Gerard K. Jeong, MD, et al Vol 13, No 3, May/June 2005 211 crosis from repeated episodes of marrow infarction) (Figs. 1 and 2). These aspects complicate the prepa- ration, insertion, and fixation of both acetabular and femoral components. Bony Preparation and Component Insertion Acetabular bone quality and pro- trusio are two important consider- ations in bone preparation. Acetabu- lar bone quality may be poor because of chronic marrow hyperplasia, which may make the acetabulum more susceptible to fracture during implantation of the cementless com- ponent. Additionally, patches of densely sclerotic bone, a result of os- teonecrosis or secondary degenera- tive changes, can require eccentric reaming. There also is a high inci- dence of acetabular protrusio, which limits the extent of medial reaming and may require medial bone graft- ing or structural acetabular support (Fig. 4). Dislocation of the hip also be- comes more difficult in the presence of the acetabular protrusio, and a pre- liminary osteotomy of the femoral head and/or neck may be required to facilitate dislocation. Femoral medullary widening from chronic marr ow hyperplasia adjacent to patchy areas of dense sclerosis can make preparation of the canal diffi- cult. Areas of sclerosis may be so dif- fuse that the canal becomes complete- ly obliterated (Fig. 5). As a result of these changes, rates of femoral shaft perforation and fracture in the sickle cell population are high, ranging from 4.9% to 18.2%. 1,9 Intraoperative fem- oral perforation and fractur e may oc- cur during reaming and/or compo- nent insertion. Reaming is more difficult in femurs with hyperplastic medullary canals, thin cortices, and scattered foci of sclerotic bone. Some authors recommend introducing a drill bit under image intensifier un- til it is possible to insert a guidewire for flexible reamers. 10,26 In patients with extensive femoral canal obliter- ation, radiographic confirmation of proper guidewire placement is help- ful before flexible reaming. Insertion of a large prosthesis in a patient with thinned cortices of the proximal metaphysis of the femur may cause perforation and/or frac- ture. It can be difficult to seat a ce- mentless prosthesis into a femur con- taining areas of sclerotic bone. 2,13 Cemented Versus Cementless Fixation The choice of cemented or cement- less fixation is an important consid- eration in treating the sickle cell pop- ulation. Polymethylmethacrylate has been implicated as a source of high infection rates and septic loosen- ing. 6,13 The use of cement may cause thermal necrosis of already infarcted bone, thereby predisposing the pa- tient to a higher incidence of infection and loosening. 23 However, the abil- ity to achieve biologic fixation with a cementless prosthesis is compro- mised when the bone quality in pa- tients with sickle cell disease is avas- cular and necrotic. Although there are no prospective studies comparing cementless and ce- mented acetabular components in pa- tients with sickle cell hemoglobinop- athies, the results of cementless THA appear to be promising. 13,15,23 The out- come of cemented cups in the sickle cell population has been generally poor, with a high rate of loosening. Moran et al 6 reported that the most common reason for failure in prima- ry THA was a 33% aseptic loosening rate with cemented acetabular cups. In contrast, early results of cement- less cups have been more promis- ing. 5,10,15,23 Hickman and Lachiewicz 15 reported no evidence of acetabular component loosening in 13 cement- less arthroplasties (mean follow-up, 6 years). Ilyas and Moreau 23 report- ed only one case of acetabular cup loosening in 18 consecutive patients with sickle cell disease who under- went bilateral cementless THA(mean follow-up, 5.7 years). When a cement- less acetabular component is used, supplemental scr ew fixation may pr o- vide additional initial fixation; a press fit may be difficult to achieve and maintain because of the poor quality of the host bone. The selection of cemented versus cementless femoral fixation in patients with sickle cell syndr ome remains con- troversial. Potential advantages of ce- mented femoral components include Figure 4 Anteroposterior radiograph of the left hip of a patient with sickle cell syndrome demonstrating advanced degenerative changes. The changes—flattening of the femoral head, subchondral collapse, reactive sclerosis in the proximal femur, and acetabular protrusio— are characteristic of a sickle cell patient with long-standing disease. Total Hip Arthroplasty in Sickle Cell Hemoglobinopathies 212 Journal of the American Academy of Orthopaedic Surgeons additional hemostasis, decreased risk of femoral perforation with implan- tation, and avoidance of biologic fix- ation in compromised bone. Mean in- traoperative blood loss during THA in patients with sickle cell disease of- ten is greater than the blood loss re- ported in other patients. 24 Cement may provide additional hemostasis via a tamponade effect on intramedullary bleeding, which may decrease the number of required blood products as well as the incidence of wound drainage, hematoma, and infection. Hickman and Lachiewicz 15 reported that 33% of patients with a cement- less femoral prosthesis (5/15 patients) experienced intraoperative blood loss >2,000 mL. In contrast, Clarke et al 10 reported average intraoperative blood loss of 1,390 mL in 17 primary cement- ed arthr oplasties. That amount is high- er than the blood loss most common- ly experienced in THA in non–sickle cell patients (range, 180 to 1,400 mL). 24 Others have reported difficulties with hemostasis and intraoperative blood loss. 3,13 Cementation provides initial rig- id fixation and likely decreases the in- traoperative risk of femoral perfora- tion and femoral fracture because less aggressive preparation of the femo- ral canal is needed. Cement fixation obviates the need both for adequate canal fit with the cementless compo- nents in a widened canal and for bi- ologic fixation in pathologic avascu- lar bone in which ingrowth may be impaired. 6,10 Biologic fixation has potential ad- vantages in patients with sickle cell disease. Three reports demonstrated a low rate of loosening with cement- less components, which is particularly important in this young patient pop- ulation. 13,15,23 Hickman and Lachie- wicz 15 reported no deep infections and no aseptic loosening in 13 cementless THAs (7 primary, 6 revision) at a mean follow-up of 5 years. A variety of proximally porous-coated femoral com- ponents (cir cumfer entially versus non- circumferentially por ous-coated, and straight versus anatomic stem designs) were used in that series. Acurio and Friedman 13 reported a statistically sig- nificant (P < 0.05) difference in the re- vision rates between 17 cemented and 18 cementless arthroplasties in 25 pa- tients with sickle cell syndrome. The type of cementless component was not specified. Although 10 of the 17 ce- mented arthroplasties required revi- sion (59%), only 4 of the 18 cement- less arthr oplasties were r evised (22%). Sixteen of the seventeen cemented hips revised were either loose clinically and/or radiographically (94%), in con- trast with only 7 of the 18 cementless hips (39%). The authors noted a 20% overall infection rate; >70% of the in- fected cases were found in the ce- mented THAs. 13 More recently, Ilyas and Moreau 23 reported only one deep infection and no evidence of femoral stem loosen- ing in their series of 18 consecutive patients with sickle cell disease who underwent simultaneous bilateral ce- mentless THA (mean follow-up, 5.7 years). Proximally porous-coated ce- mentless femoral stems were used in that series. The limited current data do not allow definitive statements concerning the best type (design) of cementless femoral components in these patients. Results Direct comparison of different series reporting the results of THA in pa- tients with sickle cell hemoglobin- opathies is difficult. Series with small patient cohorts often report combined outcomes of primary THAs, bipolar arthroplasties, hemiarthroplasties, and revision arthroplasties in a single Figure 5 Anteroposterior radiograph of the pelvis in a sickle cell patient with osteonecrosis demonstrating advanced disease on the left side (patient’s right hip) and early disease on the right side. Also, there are patchy areas of intramedullary sclerosis at the level of the meta- diaphysis near the lesser trochanter on the left side. Marrow hyperplasia, thinning of the cortices and trabeculae, and acetabular protrusio are evident on the left side. Gerard K. Jeong, MD, et al Vol 13, No 3, May/June 2005 213 study. Also, these series often include a heter ogeneous group of patients with different sickle cell syndromes (eg, sickle β-thalassemia, hemoglobin SS disease, hemoglobin SC disease). The results of THA have been mixed, with good to excellent results ranging from 34% to 100% (Table 1). Reported complication rates vary, from 18% to 80%. 1-3,6,9,10,13,15,26 The sur- vivorship of THA is sever ely compr o- mised compared with that of age- matched control subjects. Although the complication rate has been report- ed to be as high as 80% at 6-year follow-up 15 and the revision rate as high as 63% at 6.5 years postopera- tively, 3 several series of cemented and cementless THA have demonstrated that THA can afford patients with sickle cell hemoglobinopathies an im- proved quality of life and significant pain relief. 2,6,9,10,23,26 In their follow-up of 17 cases of pre- dominantly cemented THAs, Bishop et al 2 reported that, at a mean of 8.9 years, 73% of patients were pain free, ambulated without an assistive device, and performed activities of daily liv- ing without restrictions. The remain- ing patients, who eventually under- went r esection arthroplasty, ultimately experienced pain relief and reported that they would choose to have the procedure again. In the series of Epps and Castro, 9 all patients experienced pain r elief despite a high incidence of postoperative complications. In a series of 35 cemented THAs in 28 patients with sickle cell disease, Al-Mousawi et al 26 reported substan- tial improvements in the Harris hip score (HHS), from a preoperative mean of 36 to a postoperative mean of 86 (mean follow-up, 9.5 years). In a series of 20 cemented THAs (13 pri- mary, 7 revision) and 2 cementless primary THAs, Moran et al 6 report- ed improvements in HHS following THA(mean follow-up, 5 years). In the 15 cases of primary THA, the mean HHS improved from 47 preoperative- ly to 88 postoperatively; in the 7 cas- es of revision arthroplasty, the mean HHS improved from 43 preoperative- ly to 78 postoperatively. Hickman and Lachiewicz 15 reported 87% good to excellent results in their study of 16 cementless THAs in 10 patients with sickle cell disease (mean follow-up, 6 years). At 2-year follow-up, the mean HHS improved from 36 preop- eratively to 94 postoperatively in the primary THAgroup, and fr om 58 pre- operatively to 80 postoperatively in the revision THA group. In a series of 15 patients who un- derwent 27 THAs (13 cemented, 14 cementless), mean pain relief im- proved from 1.7 of 10 preoperatively to 6 of 10 at 5.5-year follow-up, us- ing the UCLA modification of the Merle d’Aubigne and Postel hip scale. 10 Similarly, in a series of 9 pa- tients (9 primary THAs, 2 revision THAs, 19 total operations), Hanker and Amstutz 3 reported a mean im- provement in pain relief from 1.6 of 10 preoperatively on the UCLA hip scale to 7.1 of 10 at 6.5-year follow- up. Mor e recently, Ilyas and Moreau 23 reported substantial improvement in pain, function, and range of motion after simultaneous bilateral cement- less THAin 18 patients (mean follow- up, 5.7 years). Despite the high rate of perioper- ative complications and revision sur - gery, most series have demonstrated that both cemented and cementless THA provide marked relief of severe, disabling hip pain secondary to os- teonecrosis in the patient with sickle cell disease. Patient satisfaction, ev- ident in improvements in hip pain scores, has been demonstrated in multiple studies. 2,3,6,9,10,15,23,26 Resec- tion arthroplasty for a failed primary THA provides a clinical result that of- ten is preferable to the patient’s con- dition before the original THA. 2,9 Medical and Surgical Complications Medical complications during THA in the patient with sickle cell syn- drome include excessive bleeding, transfusion reactions, pulmonary complications, sickle cell crises, car- diac complications, and mental sta- tus changes. Careful intraoperative and postoperative attention to hemo- dynamic balance, blood loss, and ox- ygenation may help reduce such com- plications. Intraoperative blood loss during THA in patients with sickle cell dis- ease often is greater than the blood loss during THAin non–sickle cell pa- tients. 24 Technical difficulties, such as hip dislocation in the presence of os- teophytes, acetabular protrusio, and intra-articular adhesions, as well as difficulties preparing the femoral ca- nal and acetabulum, increase surgi- cal time and blood loss. Aggressive replacement of blood products may reduce cardiopulmonary and central nervous system complications. The currently recommended indications for postoperative transfusion in pa- tients with sickle cell syndrome are a hemoglobin level <10 mg/dL as well as signs and symptoms of ane- mia, including tachycardia, syncope, angina, and high output failure; acute central nervous system complications from hypoxia or anemia; sequestra- tion crisis; acute chest syndrome with hypoxia; and acute hemorrhage. 27 Alloimmunization, which is seen in more than 20% of patients with sickle cell disease, accounts for the el- evated frequency of major transfusion reactions; the incidence of such reac- tions is as high as 4% in patients un- dergoing THA. 6,13 Other potential medical complications in the imme- diate postoperative period include congestive heart failure (4% inci- dence) 6 and sickle-cell related events (17% incidence), 24 such as vaso- occlusive crises and acute chest syn- drome. (Acute chest syndrome is de- fined as a new pulmonary infiltrate involving at least one full lung seg- ment, excluding atelectasis.) Patients with sickle cell syndrome are more susceptible tobleeding complications; thus, there is an increased risk of Total Hip Arthroplasty in Sickle Cell Hemoglobinopathies 214 Journal of the American Academy of Orthopaedic Surgeons Table 1 Results of Total Hip Arthroplasty in Patients with Sickle Cell Hemoglobinopathies Study THAs Hip Score Preoperative / Postoperative Score (mean) Mean Follow-up (yr) Compli- cation Rate (%) Revision or Failure Rate (%) Cause of Failure Results Acurio and Fried- man 13 35 (17 cemented, 18 cementless) Harris 66 / NA 7.5 49 40 (59% cemented revised, 22% ce- mentless revised [P < 0.05]) — 34% good to excellent Bishop et al 2 17 (15 cemented, 2 cementless) Harris NA / 72 (13 primary THAs) 8.9 29 24 Septic loosening All patients either pain-free or had significant pain relief after resec- tion arthroplasty Clarke et al 10 27 (13 cemented, 14 cementless) UCLA 11.5 / 24.6 (out of 40) 5.5 >59 (femoral shaft fracture/ perforation rate, 15%) 59 Aseptic loosening Pain relief improved from 1.7/10 preop to 6/10 postop Epps and Castro 9 45 NA NA / NA 3.3 63 (sickle cell crisis most common) 14 — All patients had significant pain relief Gunder- son et al 1 11 NA NA / NA NA 18 (femoral shaft fracture/ perforation) 0— — Hanker and Amstutz 3 11 (9 primary, 2 revision) UCLA 10 * /25 * (out of 40) 6.5 63 63 Aseptic loosening and infection Pain relief improved from 1.6/10 preop to 7.1/10 postop * Hickman and Lachie- wicz 15 16 (7/8 cementless primary; 6/8 cementless revision) Harris Primary, 36; revision, 58 / Primary, 94; revision, 80 Primary, 2; Revision, 6 80 (femoral osteolysis most common postop) 33 — 87% good to excellent Moran et al 6 22 (13/15 cemented primary; 7/7 cemented revision) Harris Primary, 47; revision, 43 / Primary, 88; revision, 78 Primary, 4.8; Revision, 5.3 40 (wound drainage and hematoma most common) 38 (primary failure), 43 (revision failure) Aseptic loosening — Ilyas and Moreau 23 36 (18 cementless bilateral) Merle d’Aubigne Postel 5.8 / 14.3 (out of 18) 5.7 50 6 — No femoral com- ponent loosening; 11% rate of wound infection Al- Mousawi et al 26 35 (cemented) Harris 36 / 86 9.5 40 20 Aseptic (acetab- ular) loosening All patients had improved pain, function, and motion after THA; 17% rate of aseptic loosening * = mean for entire series, including 11 THAs, 2 surface replacements, 5 excision arthroplasties, and 1 primary arthrodesis; NA = Not available Gerard K. Jeong, MD, et al Vol 13, No 3, May/June 2005 215 wound drainage and hematoma for- mation, which have been reported to be as high as 18% and 14%, respec- tively. 6 Postoperative wound infection has occurred in 16% to 25% of THAs in some series. 3,9,27 Recommended anti- biotic prophylaxis in patients not al- lergic to penicillin is a first-generation cephalosporin, which provides gram- positive coverage. There are no re- ported cases of Salmonella osteomy- elitis complicating a THA; therefore, prophylactic administration of anti- biotics that provide coverage for this gram-negative microorganism is not recommended. 6 Hip dislocation has been reported as a complication in as many as 26% of patients with sickle cell hemoglo- binapathy. 15 The high rate of hip dis- location may be related to changes in bony anatomy that predispose pa- tients with sickle cell disease to this complication. The risk of late infection is as high as 17% to 27% 2,3,13 and may result in the need for resection arthr oplasty. In- fection was the indication for six of nine resection arthroplasties done by Acurio and Friedman. 13 Deep infec- tion was the indication for 100% of the r esection arthroplasties performed by Bishop et al. 2 Late THA infection likely develops as a r esult of hematog- enous seeding of the arthr oplasty dur- ing recurrent episodes of bacteremia, which is commonly seen in patients with sickle cell syndrome. Summary THA in patients with sickle cell he- moglobinopathies pr esents both med- ical and surgical challenges. The in- creased risk of complications after THA results in part from the under- lying natur e of sickle cell disease. Mus- culoskeletal manifestations of sickle cell hemoglobinopathies include os- teonecrosis, pyogenic infection, and marrow hyperplasia; the latter two complications may compromise a suc- cessful result after THA. Intraopera- tive considerations of bone stock, bone quality, and method of component fix- ation in patients with sickle cell dis- ease are important to recognize in or- der to minimize the risks of eccentric reaming, perforation or fracture o f the acetabulum or femur, and loosening. The superior technique for achiev- ing optimal acetabular and femoral fixation has not been conclusively de- termined. There are no randomized, prospective, long-term studies that directly compare cementless, hybrid, and cemented THA in patients with sickle cell hemoglobinopathies. How- ever, recent series of cementless THA in patients with sickle cell syndrome report promising results. Despite in- creased risks for both medical and surgical complications, THAprovides pain relief, improved hip function, and satisfaction to appropriately se- lected patients. References 1. Gunderson C, D’Ambrosia RD, Shoji H: Total hip replacement in patients with sickle-cell disease. 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Vichinsky EP, Haberkern CM, Neu- mayr L, et al: A comparison of con- servative and aggressive transfusion regimens in the perioperative manage- ment of sickle cell disease. The Preop- erative Transfusion in Sickle Cell Dis- ease Study Group. N Engl J Med 1995; 333:206-213. 26. Al-Mousawi F, Malki A, Al-Aradi A, Al-Bagali M, Al-Sadadi A, Booz MM: Total hip replacement in sickle cell dis- ease. Int Orthop 2002;26:157-161. 27. Garden MS, Grant RE, Jebraili S: Perioperative complications in patients with sickle cell disease: An orthopedic perspective. Am J Orthop 1996;25: 353-356. Gerard K. Jeong, MD, et al Vol 13, No 3, May/June 2005 217