Open access peer-reviewed chapter - ONLINE FIRST

A Vascular Necrosis of Femoral Head in Sickle Cell Anemia

Written By

Mohammed Lafi Al-Otaibi

Submitted: December 20th, 2021 Reviewed: January 24th, 2022 Published: February 25th, 2022

DOI: 10.5772/intechopen.102837

IntechOpen
Sickle Cell Disease Edited by Osaro Erhabor

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Sickle Cell Disease [Working Title]

Dr. Osaro Erhabor

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Abstract

Sickle cell disease is a well-known disease with evolving changes in medical as well as surgical management. Recent developments in medical management and the well-adjusted screening protocols for the disease complications toward its anticipation and prevention and all these recent changes have led to more work toward understanding and managing disease orthopedics complications. Many researchers considered the major ones affecting patients’ daily living activity, with the improvement in patients living expectancy. Thanks to the evidence-based medical management and the development of new agents such as L-Glutamate that are recently implemented and help space the vaso-occlusive crises. This phenomenon plays the cornerstone effects on the disease pathology and leads to its harmful effects on many systems, including the muscles and bones. The infarct does occur almost everywhere through the muscle-skeletal system, with predilected site happening to be the hip joints. A vascular necrosis of the femoral head does occur in other conditions, and dealing with the one happening in sickle cell anemia must take into account all issues concerning this disease. There is growing evidence that surgical intervention with the total hip is best when there is a loss of congruency of the femoral head with head subchondral collapse and not in pure infarct with femoral head maintaining its sphere shape.

Keywords

  • bone infarct
  • hip pain
  • hip replacement
  • hip score
  • secondary arthritis
  • sickle cell anemia
  • osteonecrosis

1. Introduction

The presence of a heterozygous glutamic acid to valine substitution on chromosome 11 in the β-globin gene is known as sickle cell trait, which is found in around 300 million individuals across the globe [1]. In comparison to the homozygous genotype (HbSS) in individuals who have sickle cell disease, individuals with sickle cell trait possess heterozygous genotype (HbAS).

Over the years, few bone complications have been reported in sickle cell trait that is back in the 1970s era [2]. It is pretty uncommon that people with sickle cell trait suffer from a vascular necrosis (AVN) of bone and femoral head as the most common destruction site [3].

Multifactorial etiology is investigated for nontraumatic a vascular necrosis of the femoral head. In the majority of the cases (30–70%), both the hips are affected by the lesion [4]. The cause of AVN of the head of the femur is yet unknown. However, several risk factors have been linked to the development of this disease.

Sickle cell anemia, alcohol consumption/abuse, hemoglobinopathies, myeloproliferative disorders, chemotherapy, pregnancy, genetic susceptibility are chronic steroid usage, smoking, coagulopathies, leukemia, ionizing irradiation, and HIV infection are considered as the main risk factors [4]. The fundamental pathophysiologic cause of osteonecrosis in sickle cell disease is the obstruction of micro vessels, resulting in ischemia and oxygen and nutrition deprivation of the afflicted bone [5].

Sickle cell disease is the most common cause of osteonecrosis in children, with a prevalence incidence of 3% before 15 years. Sickle cell trait is thought to be primarily protective, particularly in the case of malaria. Despite this therapeutic benefit, afflicted carriers might have various problems and clinical sequelae, including exercise-related injury, venous thromboembolism, and renal difficulties [1, 6].

The possibility of AVN of the femoral head in sickle cell trait was reported by Perumal and Corbett [7]; however, there is a need for additional research. This index case describes and raises awareness of a vascular necrosis of the femoral head and its relationship to sickle cell trait.

The autosomal recessive genetic disorder sickle cell disease is marked by abnormal sickle hemoglobin synthesis and reduced pliability of red blood cells. It causes blood arteries to clog, resulting in ischemia and infarction of the afflicted tissue [8]. Despite being indigenous to tropical Africa and the Middle East, it has become a worldwide disease due to population movement [9].

One of the most prevalent sickle cell disease clinical signs is bone involvement, which can vary from an acute severe vaso-occlusive crisis to a persistent impairment such as a vascular necrosis. Pathological fractures, septic arthritis, osteonecrosis, and osteomyelitis are orthopedic consequences of sickle cell disease [10].

The most common location for a vascular necrosis in sickle cell disease is the femoral head, followed by the shoulder, knee, and other minor joints [11]. A vascular necrosis of the femoral head causes hip osteoarthritis and reduces its functional capability. To avoid morbidity and mortality associated with late diagnosis, osteonecrosis must be diagnosed early and treated promptly. The majority of orthopedic surgeons advocate complete hip replacement to increase functional capacity [12].

In sickle cell disease patients, aberrant metaphyseal femoral morphology with thin cortices and trabeculae, low bone density, and medullary hyperplasia are only a few skeletal abnormalities that affect the hip joint. The femoral canal can also be obliterated by irregular bone sclerotic regions, resulting in hip joint congruency loss. Thinned femoral cortical lining inside the outer cortex might sometimes seem like a femur inside a femur [13].

The disturbance in the proximal femur’s blood supply causes a vascular necrosis of the femoral head, known as osteonecrosis. Each year, between 10,000 and 20,000 new cases are recorded in the United States alone [14]. It can be caused by a multitude of factors, both traumatic and nontraumatic. Fractures, dislocations, chronic steroid usage, prolonged alcohol use, coagulopathy, and congenital reasons are only a few of the causes.

A vascular necrosis of the femoral head is a devastating illness requiring healthcare providers to look for its symptoms. This exercise will provide you with a summary of the etiology and treatment options, as well as some clinical pearls.

The lateral and medial circumflex branches of the profunda femoris, which is a branch of the femoral artery, give the bulk of the blood flow to the head of the femur. It is known that the profunda femoris is the deep penetrating branch present in the upper thigh region. A ring around the femur’s neck is created by joining medial and lateral circumflex femoral arteries. This is the place from which many tiny arteries branch for perfusing the femoral head.

The foveal artery, also known as the ligamentum teres artery, is another direct route of blood flow. The femur head is linked to the acetabulum through the ligamentum teres. The foveal artery flows through the ligament, but its contribution is only noticeable in children [15].

Two significant anastomoses provide collateral blood flow for supporting femoral; although, the flow is limited;

  • Cruciate anastomosis—it maintains flow between medial circumflex femoral and inferior gluteal artery.

  • trochanteric anastomosis—it maintains flow between medial/lateral circumflex femoral and superior gluteal artery.

The internal iliac artery, also known as the hypogastric artery, is the major artery of the pelvis and nourishes a portion of the buttock and posterior thigh [15]. The common iliac artery is derived from the aorta, and the internal iliac artery is derived from it.

The socket of the hip, the acetabulum, is the place where the femoral head is connected. The acetabular branch of the obturator artery provides blood supply to the acetabulum, along with the deep branches of the superior gluteal artery and pubic branches of the obturator artery. Disruption of the blood flow to the head of the femur might produce ischemia and necrosis due to restricted collateral circulation. The osteocytes will die, the articular surface will crumble, and degenerative arthritis will develop if the blood supply is not restored quickly [16].

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2. Etiology

The etiology of AVN of the femur head is not well established. But several conditions have been regarded as risk factors for its development.

The dislocation and fracture involving femoral neck fracture and the acetabulum are among the most common traumatic causes. A vascular necrosis can result during sorts due to damage of the blood supply to the head of the femur, which is easily disturbed in some traumas associated with sport activities. In total, 15–50% of femur neck fractures and 10–25% of hip dislocations are caused by osteonecrosis [17].

Most nontraumatic etiologies are represented through increased alcohol use and chronic use of steroids, accounting for >80% of the cases. After trauma, the second most common cause of osteonecrosis is steroid-associated osteonecrosis. The actual pathogenesis is unknown and most likely complex despite indications of an association between steroids and osteonecrosis. Fat emboli, fat cell hypertrophy leading to increased endothelial dysfunction, bone marrow stem cell pool abnormalities, intraosseous pressure, and hyperlipidemia are all possible contributors to ischemia and necrosis [18].

There is a lack of understanding about alcohol-induced osteonecrosis. However, proliferation, bone marrow fat cell hypertrophy, blood vessel occlusion, subsequent lack of perfusion, changes in serum lipid levels, and increased intraosseous pressure are known to cause osteonecrosis [19].

Osteonecrosis is frequently triggered by sickle cell disease. Ischemia and bone infarction result from the malformed inflexible red blood cells, with the femoral head being the most prevalent location of osteonecrosis in these patients [20]. Autoimmune and chronic inflammatory illnesses, such as systemic lupus erythematosus, have long been linked to femoral head osteonecrosis. Long-term steroid therapy is frequently related to developing the illness in these people, while there have been instances among steroid naïve.

Perthes, which affects the pediatric population due to idiopathic a vascular necrosis of the femoral head. There is an increased risk of osteoarthritis and losing range of motion due to lack of blood supply resulting in necrosis of the femoral head leading to deformity. The progression of disease takes place in four steps as follows [21];

  • Necrosis—it is when necrosis begins due to disruption of blood supply.

  • Fragmentation—resorption of necrotic bone takes place that is replaced with woven bone, with increased susceptibility of either collapsing or breaking.

  • Re-ossification—development of stronger bone.

  • Bone remodeling—completion of bone regrowth and finalized shape, considering the damage caused during the second phase of fragmentation.

The development of femoral head osteonecrosis has also been linked to vascular disease caused by diabetes and direct injury by cytotoxic chemicals [22].

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3. Epidemiology

A vascular necrosis of the femoral head is expected to develop at a rate of 20,000–30,000 new cases each year in the United States, accounting for 10% of the approximately 250,000 total hip arthroplasties performed each year [23]. This number is comparable to the 0.01% incidence observed in German-speaking nations and the 1.9 per 100,000 incidences reported in Japan.

There is no link between race and sickness, except in cases of sickle cell disease, which is more common among people of African heritage. Overall, men are more likely than women to have this illness, with studies predicting ratios ranging from 3 to 5 to 1 [24]. The average age of the patients at the time of therapy is between 33 and 38 years old [14].

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4. Pathophysiology

The actual pathophysiologic processes behind a vascular necrosis of the femoral head are not always evident, and the condition is commonly thought to be complex [4]. The consequence is the death of osteocytes and bone marrow due to inadequate blood supply to the subchondral bone of the proximal femur, regardless of the causative event [17]. If not treated adequately in the early stages, this cell loss will collapse the femoral head and subsequent osteoarthritis. It is also clear in literature that the AVN is inevitable end result in some conditions.

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5. History of the disease

Early on in the illness phase, patients may be asymptomatic. However, when they become symptomatic, the most common complaint is hip discomfort that might extend to the groin and thigh. Activities such as climbing stairs and walking worsen the discomfort, which is relieved by relaxation. Even when there is no movement, the pain will often persist [4]. Restricted range of motion, soreness on palpation, and discomfort during abduction and internal rotation of the hip area are some physical exam findings suggestive of femoral head osteonecrosis.

Total hip replacement is one of the most satisfying procedures and has been dubbed the “century surgery” due to its nearly 50-year track record of success [25]. Total hip replacement became popular in the 1960s to restore hip function and, as a result, everyday activities. Since then, surgical technique and surgeon training advancements have led to today’s condition of high-quality prosthetic implants, well-proven surgical indications and contraindications, and evidence-based pre- and postoperative treatment.

On average, 85–95% of hip replacement patients survive [26]. Total hip replacement for sickle anemia is a complicated treatment with medical, intraoperative, and postoperative problems. The success necessary to medically optimize patients and improvements in surgical procedures and prosthetic implant manufacturing has yielded equivalent outcomes for sickle cell anemia patients.

Adherence to proper perioperative measures such as hydration, body temperature management, oxygenation, and a hemoglobin level of over 10 mg/dl contributes favorably to the outcome and is relatively simple to implement. Intraoperative complications such as soft tissue contractures, bone fractures, and perforations are challenging to manage and are a primary cause of poor results [27].

There is a need for further improvement for a hip replacement among the affected individuals, considering the impact of bony involvement such as scoliosis or soft tissue conditions. One such condition is Periarticular Contracture on hip replacement such as degenerative hip and spine for sickle cell anemia elderly patients.

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6. Evaluating sickle cell disease

Early detection can have a significant impact on the result. The clinical presentation is combined with adequate imaging to make a diagnosis. X-rays, radionuclide bone scanning, and magnetic resonance imaging (MRI) are all examples of imaging. Imaging in the context of a patient’s symptoms can aid in determining the best course of treatment.

Usually, the plain-film radiography is performed in two planes, using both frog-leg lateral and anterior–posterior films. Subchondral radiolucency, often known as the “crescent sign,” is a pathognomonic indication of subchondral collapse. The “donut sign,” which is a ring of enhanced uptake around a chilly core, might appear when Technetium-99 m is absorbed. At the demarcation, where reactive bone meets dead bone, this indication signifies increased bone turnover [23].

The gold standard for diagnosing osteonecrosis is an MRI. X-rays and radionuclide scans can also help diagnose, but none is as sensitive or accurate as MRI to detect radiographic evidence early in disease development. When determining a patient’s prognosis and devising a treatment plan, MRI can identify bone marrow alterations, the size/location of the necrotic region, the influence on acetabular cartilage, the depth of collapse, and so on [4].

The amount of necrosis can be classified if appropriate imaging has been obtained. While there are other staging systems available, the Steinberg staging system is the most often utilized. It specifies the seven stages illustrated in Table 1.

StageFeatures
0Normal radiograph, bone scan, and MRI
INormal radiograph, abnormal bone scan, and or magnetic resonance imaging
IA Mild (involves less than 15% of the femoral head).
IB Moderate (involves 15–30% of the femoral head)
IC Severe (affects over 30% of the femoral head)
IICystic and sclerotic change of the femoral head
IIA Mild (involves less than 15% of the femoral head)
IIB Moderate (affects 15–30% of the femoral head)
IIC Severe (affects more than 30% of the femoral head)
IIISubchondral collapse (crescent sign) without flattening of the femoral head
IIIA Mild (involves under 15% of the femoral head)
IIIB Moderate (affects 15–30% of the femoral head)
IIIC Severe (affects over 30% of the femoral head)
IVFlattening of the femoral head/femoral head collapse
IVA Mild (involves under 15% of the femoral head)
IVB Moderate (involves 15–30% of the femoral head)
IVC Severe (affects more significant than 30% of the femoral head)
VJoint space narrowing and acetabular changes
VA Mild
VB Moderate
VC Severe
VIAdvanced degenerative joint disease

Table 1.

Steinberg staging system [23].

In patients with suspected osteonecrosis, a laboratory workup should be performed to help rule out alternative causes of hip pain and check for concomitant conditions. The lipid panel, C-reactive protein (CRP), anti-nuclear antibody (ANA), hemoglobin electrophoresis, a complete blood count (CBC), erythrocyte sedimentation rate (ESR), rheumatoid factor (RF), and anti-cyclic citrullinated peptide (anti-CCP) are some of the tests that may be used in a workup.

Elevated ANA and RF are nonspecific indicators of active autoimmune disease. Inflammatory processes raise both ESR and CRP. However, they are nonspecific. Rheumatoid arthritis is associated with elevated anti-CCP antibodies, whereas sickle cell disease is associated with HbS and a low quantity of HbF on hemoglobin electrophoresis [28].

A CBC with indications of normocytic or microcytic anemia and a high reticulocyte count is also constant with a sickle cell disease diagnosis. Sickle cell disease and rheumatoid arthritis are two disorders that can lead to femoral head osteonecrosis and produce hip discomfort even if there is no osteonecrosis.

A biopsy is rarely required because the diagnosis can frequently be determined precisely based on imaging and clinical presentation. If a biopsy is taken, the typical histological results will be trabecular necrosis (more than 50% empty osteocytic lacunae) and necrotic hematopoietic marrow, with no signs of inflammation, tumor cells, or sepsis [29]. Similarly, angiography tests are rarely regularly conducted, despite their excellent vasculature imaging, and may aid in disease pathology research [28, 30].

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7. Disease management

A vascular necrosis of the femoral head can be treated in different ways, such as they can be treated through conservative to invasive methods. The particular therapy used is determined on the basis of the patient’s condition individually along with a variety of circumstances for optimal results. All considered are the patient’s age, location and extent of necrosis, comorbidities, amount of pain/discomfort, and whether the articular surface has collapsed. Treatments, which include both operational and non-operative approaches, are best applied during the pre-collapse period. Femoral head necrosis, if left untreated, can develop subchondral fractures in as little as 2–3 years [31].

Treatment options should be depending on the severity of the lesions, but primarily on whether or not they have collapsed. In asymptomatic and symptomatic small to medium-sized pre-collapse lesions, non-operative therapies or core decompression can be beneficial. Bone grafts (non-vascularized or vascularized) or osteotomies can be used to treat medium-to-large lesions. Arthroplasty is recommended if there is femoral collapse or acetabular involvement.

The term “conservative management” refers to a group of non-operative therapies. Physical therapy, reduced weight-bearing, alcohol abstinence, steroid discontinuance, pain management medication, and focused pharmacologic treatment are only a few examples. Because the time it takes for a vascular necrosis of the femoral head to develop and collapse varies widely, and data are poor, there is no consensus on conservative therapies. Despite the fact that minor asymptomatic lesions can heal independently, the majority develop and require treatment.

Off-label usage of statins, anticoagulants, vasodilators, and bisphosphonates has been attempted to revascularize the femoral head. Vasodilators, such as iloprost (PGI2), work by lowering intraosseous pressure and increasing blood flow [32]. Statins inhibit the development of stem cells into fat cells, lowering intraosseous pressure and improving perfusion [33]. Anticoagulants such as enoxaparin are used to prevent osteonecrosis from progressing due to thromboembolic events and hypercoagulability [34]. Bisphosphonates, such as alendronate, block osteoclasts’ ability to reduce bone resorption [35].

There is still no significant consensus on the efficacy of any of these drugs over another, despite the availability of a multitude of pharmacologic treatment options. In general, intra-articular steroid injections are not advised. The benefits of steroid injections are generally fleeting, and their usage might exacerbate a vascular necrosis; although, they can give pain relief.

For individuals who require more intrusive therapy, there are many surgical choices. They are classified as either joint preservation techniques or joint reconstruction procedures. Bone grafts, osteotomy, core decompression, biologics, and cellular treatments are examples of joint preservation operations, whereas joint replacement is an example of reconstructive interventions.

The lateral approach was used for all surgeries. The incision was made around 8 cm down the line of the femur, 5 cm proximal to the tip of the greater trochanter longitudinal incision centered over, the greater trochanter’s tip. The fascia lata was split and retracted anteriorly during the superficial dissection, exposing the gluteus medius tendon.

The gluteus medius fibers that were connected to the fascia lata were removed using sharp dissection. Deep dissection separated the gluteus medius fibers longitudinally, commencing in the center of the greater trochanter and extending the incision inferiorly via the vastus lateralis fibers to avoid harm to the superior gluteal nerve [10]. The anterior greater trochanter and its underlying gluteus minimus formed an anterior flap in the anterior gluteus medius.

To expose the anterior joint capsule, the anterior region of the vastus lateralis needed forceful dissection of the muscles from the bone or lifting specks of bone where possible. Following anterior dissection down the greater trochanter and onto the femoral neck, the gluteus minimus capsule was freed from the anterior greater trochanter, allowing for simple hip dislocation.

To avoid problems or fractures, enough exposure and attentive soft-tissue manipulation were used. In patients with a sclerotic and constricted femoral canal, extra measures were used during femoral stem preparation [10]. To avoid femoral stem perforation, sequential reaming over guidewire was performed.

The patients are likely to get routine physical therapy for total hip replacement after surgery, including patient education, pain management, range of motion, and muscle-strengthening activities. Partially weight-bearing was allowed for the first 6 weeks, followed by full weight-bearing. Ten to twenty-one days after surgery, the patients were discharged from the hospital.

The most common intervention during pre-collapse phases is core decompression, which involves the surgical removal (by drilling) of damaged tissue from the interior of the femoral head to reduce pressure and enhance perfusion. Cell treatments have been utilized as adjuvants to core decompression. They have been found to be safe, with better clinical results and a lower rate of disease progression than core decompression alone [36, 37].

However, core decompression shows good results, and it is considered a good option for symptomatic small to medium-sized pre-collapse lesions. It is not recommended in case of a collapsed femoral head.

Bone grafting is a therapy option for more extensive lesions that do not collapse prematurely. The bone graft is likely to be obtained as a vascularized bone graft from another part of the patient’s body along with intact vasculature, an autograph from another part of the patient’s body, or an allograft from another person via a bone bank. The vascularized bone transplant has the extra benefit of transporting a fresh blood supply, which can help with revascularization and perhaps revitalize the necrotic zone.

The removal of segments of bone to change the weight distribution of the joint to the healthy, uninvolved bone is known as osteotomy. Angular intertrochanteric or rotational trans-trochanteric operations, in which the necrotic portion of the femoral head is shifted away from weight-bearing regions, supposedly enabling healing or slowing development [38], are common examples.

If the injury is severe, the hip has collapsed, and/or there is acetabular involvement, arthroplasty (removal of the ball-and-socket joint and replacement with a prosthesis) may be necessary [23]. While there have been improvements in hardware such as low-wear surface bearings that have significantly improved the outcome of hip replacement in the setting of osteonecrosis in the last 20 years [4], the outcome of hip replacement in the setting of osteonecrosis has previously shown mixed success rates.

The patients who underwent congruous hip joint radiologically were diagnosed to have infarct area > 30% through magnetic resonance imaging. These patients are likely to have previous joint preservation treatments (core decompression); however, pain alleviation did not seem to benefit from them. The patients undergoing an incongruent hip joint with arthritic alterations are likely to use an uncemented complete hip replacement (Figures 13).

Figure 1.

(a) Preoperative hip joint with subchondral collapse and loss of acetabular congruency; (b) postoperative hip joint with a prosthesis.

Figure 2.

(a) Preoperative hip joint with preserved acetabular congruency; (b) postoperative hip joint with a prosthesis.

Figure 3.

Postsurgical head of the femurs; (a) Femur’s round head; (b) Femur’s triangular head.

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8. Musculoskeletal changes

Surgical treatment, such as total hip replacement, is the last resort to address the changes occurring in the hip joint due to a vascular necrosis and secondary osteoarthritis in sickle cell anemia, even though there are less invasive procedures such as core decompression and trap door procedure that can and should be offered earlier to alter the situation.

It is important to remember that the presence of soft tissue changes such as contracture or infection, as well as skeletal findings such as the loss of the medullary canal, variation in bone quality, bone load resistance, and spine vertebral segmental collapse with a vascular necrosis, all affect the outcome of total hip replacement. Patients with just hip joint alterations are likely to have a better prognosis, but those with multiple musculoskeletal changes are expected to have a range of outcomes.

There is the proportional correlation of medullary obliteration acetabular periarticular infarct (Figure 4a and b), spine vertebral column collapse with kyphoscoliosis (Figure 5), and soft tissue hip and knee contractures (Figure 6) with the severity of preoperative symptoms, postoperative Harris Hip Scores (HHSs), and intraoperative difficulties. Other characteristics contributing to a lower hip score include severe leg length disparity, obliteration of the medullary cavities, soft tissue contractures, and poor bone quality [39].

Figure 4.

(a) Advanced bilateral a vascular necrosis away from hips; (b) unilateral a vascular necrosis restricted to the left hip.

Figure 5.

Scoliosis of the spine secondary to a vascular necrosis.

Figure 6.

Soft tissue hip and knee contractures.

Sickle cell disease is linked to a variety of orthopedic conditions, including femoral head osteonecrosis. Leg length difference is caused by lumbar spine involvement with osteonecrosis and collapse with subsequent sclerosis. Intraoperative blood loss should be avoided as feasible in patients with a high difficulty score, and blood transfusions should be administered as needed.

Sickle cell anemia is a kind of anemia that affects vascular necrosis, which affects the hip joint early in a patient’s life. As sickle cell anemia patients’ survival rates improve, it is more likely that hip replacement and revision procedures will be performed sooner than expected [40]. Infarcts cause most instances with boney infarct without loss of congruency with intraosseous compartmental pressure of the femoral head, which may benefit from core decompression [39]. Hips become complicated since the disease has deteriorated owing to weakness, stiffness, and a lack of desire by the time they present.

The time necessary for surgery is equivalent to the time required for a problematic main hip, and our findings are consistent with those of other writers. Technical challenges observed during surgery included the severity of localized disease alterations such as stiffness, femoral canal obliteration, and acetabular bone stock variation due to cysts, sclerosis, and protrusio in rare cases.

Intraoperative blood loss varies according to these technological challenges, necessitating more blood transfusions. Our study’s duration is longer than predicted compared with previous studies on primary total hip replacement (THR), which had mean operational times of 89 minutes and 12,328 minutes. The mean intraoperative blood loss was 1600 ml, more significant than the 1090 ml, 984 ml, and about 371 ml reported in primary THR.

The acetabulum of the femur can both be perforated and fractured. The individuals who had femoral perforation did not require any additional treatment. The channel was ultimately discovered, and the stem was able to avoid the hole. A sickler and a young guy with steroid-induced AVN both had acetabular perforation. Due to severe irregularity and a poor acetabular floor, the hole developed during reaming. In sicklers, Al-Mousawi [41] found acetabular perforation, femoral perforation, and fractures identical to our findings.

At birth, the hip joint lacks sphericity and congruency. It is also prone to subluxation and dislocations due to its lack of rigidity. A deeper spherical acetabular cavity occurs due to stress and musculoskeletal alterations, enhancing joint stability [42].

Osteoarthritis of the hip can be caused by various developmental disorders defined by a lack of hip joint congruency. However, femoroacetabular impingements might be a secondary cause of osteoarthritis [43]. Patients with a lack of congruency were thought to have more significant clinical problems, and complete hip replacement in these patients would result in substantial functional benefits.

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9. Postoperative care

After surgery, the patients got routine physical therapy for total hip replacement, including pain management, muscle-strengthening activities, patient education, and range of motion. Partially weight-bearing was allowed for the first 6 weeks, followed by full weight-bearing. Ten to twenty-one days after surgery, the patients were discharged from the hospital.

The functional results were assessed using the Harris Hip Score (HHS) [44]. To evaluate functional results clinically and radiographically, all patients were followed up at 6-week intervals and subsequently at 6-month intervals. Loosening, dislocation, and heterotopic ossification were all looked for on radiographs.

Surgical problems, infection, loosening, and dislocation were all considered failures, necessitating revision replacement surgery. Patients were examined by a hematologist and the research author at each follow-up, and medical and surgical problems were recorded.

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10. Differential diagnosis

A vascular necrosis of the femoral head symptoms coincides with other etiologies that should be considered in a differential diagnosis. The first is bone marrow edema syndrome (BMES), also known as temporary osteoporosis, which develops due to an accident, increased physical activity, or osteoarthritis. This syndrome manifests as sudden, atraumatic hip discomfort that is self-limited and usually fades within a year. Table 2.

Complex regional pain syndrome
Inflammatory synovitis
bone marrow edema syndrome (BMES)
Osteomyelitis
Osteoarthritis
Osteoporosis
Soft tissue trauma

Table 2.

Problems faced by patients in osteonecrosis.

Magnetic resonance imaging or extensive bone marrow edema gives the condition its name; imaging is frequently essential [45]. A subchondral fracture, which commonly arises as a fracture following minor trauma in the elderly, in the setting of osteoporosis leading to subchondral insufficiency, is another disorder that can be mistaken with osteonecrosis of the proximal head of the femur.

11. Prognosis

Many variables influence the prognosis of femoral head osteonecrosis. The point at when it is diagnosed is one of them. The sooner an illness is diagnosed, the more successful the preventative measures are and the better the prognosis.

Moreover, there are markers of a poor prognosis such as lateral head involvement, age > 50 years, and involvement of greater than one-third of the weight-bearing portion of the femoral head, along with the disease advancement when the diagnosis is being made [46]. A patient’s prognosis might differ and should be decided by a skilled physician following an adequate examination; although, one or more of these characteristics are present [47].

12. Complications

Joint discomfort worsens with time, decreased range of motion, and osteoarthritis are all complications [48]. Patients may have a considerable handicap as a result of these consequences.

13. Consultations

A trained orthopedic surgeon should be consulted for an expert examination when a practitioner believes a patient has femoral head osteonecrosis.

14. Deterrence and patient education

Patients should seek medical attention if patients are suffering discomfort in their hips, thighs, or buttocks. Specific patient populations should be informed of the risk factors and tested as appropriate because early a vascular necrosis of the femoral head might be asymptomatic.

Patients on long-term steroid treatment, long-term bisphosphonates, heavy alcohol usage, hemoglobinopathies, chemotherapy or radiation patients, and those who sustain damage to the hip and surrounding region are all examples of higher-risk patients [23].

15. Enhancing healthcare team outcomes

Many inter-professional individuals’ makeup healthcare teams, including (but not limited to) a physician, nurse practitioner or physician assistant, pharmacist, and nurse. All members of this team who treat patients with a vascular necrosis of the femoral head must recognize the problem, engage in a qualified care plan, and seek expert advice when necessary. The pharmacist can assist in evaluating drug regimens, both in the lead-up to AVN and in attempts to treat AVN pharmaceutically, and can report any concerns to the physician.

If surgery is required, an orthopedic specialist will lead the treatment, and nursing will assist in preparing the patient for surgery, monitoring the patient during and after surgery, and checking on treatment efficacy as well as administering postoperative medications, all while keeping an eye out for adverse effects that should be reported as soon as possible.

Patients should be educated about the dangers of drinking by primary care providers, especially nurses. In addition, doctors should use the lowest effective dosage of corticosteroids when prescribing them. Patients taking long-term corticosteroids should be queried about hip discomfort at every clinic visit by nurses and doctors. The pharmacist should educate patients about the importance of quitting smoking and collaborate with physicians to develop pharmacological aids to help smokers quit. Patients should be educated on the signs and symptoms of AVN to seek treatment as soon as possible.

Each inter-professional healthcare team member’s awareness and knowledge of a vascular necrosis of the femoral head will enhance patient treatment and prognosis outcomes.

16. Conclusion

In sickle cell anemia patients, the primary total hip should be adequately prepared; we believe lumping all sickle cell hip a vascular necrosis together is unjust to patients and surgeons. While some of these individuals may be treated with ease, others have a greater degree of difficulty, as evidenced by the existence of musculoskeletal alterations that are not limited to the hip joint.

A thorough history, physical, and radiological examination focusing on these predictors of poor prognosis are required to distinguish between the two groups. Strict preoperative optimization is needed, as well as a well-stocked implant arsenal for hip replacement. Technically, the hips of sickle cell anemia patients with a high difficulty index are complex; challenges should be anticipated and addressed to minimize a high rate of intraoperative problems, increased operation duration, and blood loss. Primary hip replacement in sickle cell disease patients is prevalent and might be challenging to do.

Acknowledgments

The author is thankful to all the associated personnel who contributed for this study by any means.

Conflict of interest

There is no conflict of interest.

Notes/thanks/other declarations

None.

References

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Written By

Mohammed Lafi Al-Otaibi

Submitted: December 20th, 2021 Reviewed: January 24th, 2022 Published: February 25th, 2022