This patient, born in the Congo, was diagnosed with sickle cell disease aged 9 months. An MRI showed avascular necrosis (AVN) of the left hip. We present a series of plain radiographs and an MRI demonstrating the development and treatment of AVN of the hip due to sickle cell disease.
The patient presented complaining of increasing left hip pain for several weeks. He had a background history of sickle cell disease. There is no family history of hip pathology. Examination of the painful hip revealed a decrease in internal rotation and spasms at the extremes of all planes of motion. Plain radiographs suggested a diagnosis of an osteochondral fracture with epiphyseal involvement. The patient was admitted for treatment with skin traction pending a bone scan and MRI scan. The MRI and bone scan diagnosed AVN of the left hip and splenomegaly. The patient was discharged home partial weight bearing on crutches. Eight months later he was readmitted via outpatient clinic with a two week long history of worsening limp and increasing left hip pain. Examination again demonstrated decreased hip movements: flexion =120 degrees, internal rotation = 0 degrees, external rotation = 30 degrees and abduction = 40 degrees. He was treated with skin traction effectively and discharged 24 hours later nonweight bearing, with a planned admission date for an arthrogram. This was performed as a day case and showed significant hinge abduction of the left hip. This was treated six weeks later with a proximal femoral valgus osteotomy, psoas and adductor release. Post operatively the patient was managed with traction and a single hip spica. He was discharged nonweight bearing. We plan to remove the screws at a subsequent date.
Sickle cell disease is an autosomal recessive chronic haemoglobinopathy. In sickle cell disease, aseptic skeletal conditions have two causes: chronic haemolytic anaemia leading to marrow hyperplasia and hypoxia leading to red cell sickling ischamia and infarction. 1 In adolescence and adulthood, a common complication of sickle cell disease is AVN typically in areas supplied by end arteries such as the femoral or humeral heads. Patients often have pain associated with functional limitation of the affected joint. Chronic pain is often associated with later stages of AVN necrosis, particularly in the femoral head. Weight bearing on the joint exacerbates the pain due to AVN. Infarction of bone and bone marrow in patients with sickle cell disease can lead to the following radiological changes: • osteolysis (in acute infarction) • AVN • articular disintegration • myelosclerosis • periosteal reaction (unusual in the adult) • H vertebrae (step like depression in the end plates)4 Areas of AVN become radiographically visible as sclerosis of bone with secondary repair reaction and eventually result in degenerative bone and joint destruction. In a UK study in 1991 a series of 41 patients with sickle cell disease eleven (41%) were affected by AVN of the femoral head. Seven (26%) had bilateral disease.2MRI is the best method for detecting early signs of AVN in patients with sickle cell disease and for identifying episodes of osteomyelitis. Nuclear scanning can also be used to detect early AVN. Plain radiography is useful in evaluating subacute and chronic infarction but is of little value in the acute phase of AVN where MRI has little advantage.3Perthes’ disease normally affects younger children. In older children and adolescents a Perthes ‘lesion’ probably is equivalent to idiopathic AVN.
Left hip AVN, secondary to sickle cell disease.
Based on the provided anteroposterior (AP) radiograph of the hip joint and MRI images, abnormal structural changes can be observed in the left femoral head. The main observations include:
Overall imaging characteristics are consistent with avascular necrosis (AVN) of the femoral head, especially in the setting of a previously confirmed diagnosis of sickle cell disease (SCD).
Based on the patient’s clinical information (13-year-old male with a lifelong history of sickle cell disease) and the imaging findings described above, the following potential diagnoses are considered:
Considering the patient’s history, sickle cell disease–induced aseptic necrosis of the femoral head remains the most likely diagnosis.
Taking into account the patient’s age, medical history (sickle cell disease diagnosed in early childhood), clinical symptoms (hip pain, restricted mobility), and imaging findings (femoral head collapse, sclerosis, and MRI signal changes), the most probable diagnosis is:
Left Femoral Head Avascular Necrosis (AVN) Secondary to Sickle Cell Disease.
If any doubts remain or if further evaluation of lesion activity and extent of subchondral fractures is needed, higher-resolution MRI sequences or additional imaging and laboratory tests should be considered to rule out infection or other complications.
The following treatment strategies may be selected in consultation with the medical team, based on the patient’s specific situation:
Throughout the rehabilitation process, exercise prescriptions should be tailored according to the patient’s skeletal condition, level of pain, and cardiopulmonary tolerance. Close collaboration with specialist physicians and rehabilitation therapists is crucial to maximize recovery and minimize the risk of complications.
Disclaimer: This report is a reference-based medical analysis and does not replace an in-person consultation or professional medical advice. Patients should undergo further evaluation and treatment under the guidance of specialist physicians and a dedicated medical team.
Left hip AVN, secondary to sickle cell disease.
