Management of Bilateral Humeral Head Avascular Necrosis in Sickle Cell Disease Using Surgical Intervention With Adjuvant Hyperbaric Oxygen Therapy: A Case Report

Introduction

Avascular necrosis (AVN) is a debilitating complication most commonly affecting weight-bearing joints; however, the humeral head can also be involved, particularly among sickle cell disease (SCD) patients. In SCD, recurrent vaso-occlusive episodes lead to microvascular obstruction, impaired perfusion, and repeated ischemia-reperfusion injury, ultimately leading to bone infarction and structural collapse [1]. While femoral head involvement is more frequently reported, humeral head AVN is a clinically significant but less commonly recognized manifestation that can result in substantial pain, functional limitation, and reduced quality of life.

The reported prevalence of humeral or femoral head AVN in SCD patients reaches up to one-third by adulthood, with bilateral involvement not uncommon due to the systemic nature of the disease [2,3]. Management of AVN in this patient population is particularly challenging, as ongoing microvascular compromise limits the efficacy of conventional treatment options. Pharmacological therapies offer limited disease-modifying benefit, while surgical interventions such as core decompression or joint arthroplasty are often associated with variable outcomes and higher complication rates in SCD patients [4]. Consequently, interest has grown in adjunctive therapies that can enhance bone healing and improve surgical outcomes.

HBOT has recently emerged as a potential adjunct in the management of AVN. By increasing dissolved oxygen delivery to hypoxic tissues, HBOT can promote angiogenesis, reduce bone marrow edema, and support osteogenesis through the upregulation of hypoxia-related growth factors [5]. These mechanisms suggest a complementary role alongside surgical decompression in early-stage disease. Although HBOT has been increasingly investigated in femoral head AVN, evidence regarding its application in non-weight-bearing joints such as the humeral head remains limited, particularly in patients with sickle cell disease. Given the unique pathophysiology of SCD-related osteonecrosis, adjunctive therapies targeting tissue hypoxia may be of particular relevance. This case report describes the clinical course and multimodal management of bilateral stage II humeral head AVN in a patient with SCD and explores the potential adjunctive role of HBOT in joint-preserving surgical strategies.

Case Report

A 32-year-old Bahraini woman with a known history of sickle cell disease (HbSS genotype) presented to the orthopedic clinic with a 2-month history of progressive bilateral shoulder pain and stiffness. The pain was described as deep and activity-related, with increasing limitation of shoulder range of motion and difficulty performing overhead activities and activities of daily living. There was no history of trauma, swelling, or neurological symptoms reported. She reported having 3 or 4 vaso-occlusive crises in the past year, all managed conservatively with analgesics and transfusion support. She denied tobacco, alcohol, or substance use. Her regular medications included hydroxyurea 500 mg daily and folic acid 5 mg daily. Baseline laboratory evaluation demonstrated chronic anemia consistent with homozygous sickle cell disease. Hemoglobin was 8.6 g/dL, hematocrit 26%, and reticulocyte count 6.8%. White blood cell count was 9.8×109/L and platelet count was 410×109/L, both within expected ranges for stable SCD without acute crisis. Erythrocyte sedimentation rate was 18 mm/h and C-reactive protein was 4 mg/L, with no laboratory evidence of acute infection or inflammatory exacerbation. Pre-exchange hemoglobin S (HbS) fraction was 78%. Baseline values are shown in Table 1.

Physical examination revealed tenderness over both proximal humeri and decreased range of motion in abduction and external rotation. There was no neurovascular deficit. Magnetic resonance imaging (MRI) of both shoulders demonstrated stage II pre-collapse AVN of the humeral heads, showing subchondral sclerosis and signal loss without articular surface collapse (Figure 1A, 1B).

In patients with SCD, differential diagnoses for shoulder pain include vaso-occlusive pain crisis, rotator cuff pathology and subacromial impingement, adhesive capsulitis, fracture following minor trauma, and infection such as osteomyelitis or septic arthritis. In this case, the chronic progressive course with functional limitation without clinical evidence suggestive of acute infection and the characteristic imaging findings were most consistent with AVN.

Following multidisciplinary consultation, the patient underwent arthroscopic-assisted core decompression with ipsilateral fibular strut autograft under general anesthesia. Intraoperative findings confirmed intact articular cartilage with underlying necrotic bone. The procedure was completed without complications. An identical procedure was performed on the contralateral shoulder 10 weeks later.

Postoperatively, she was started on HBOT, completing 40 sessions at 2.5 atmospheres absolute (ATA) for 60 min each. Additionally, she received oral alendronate 70 mg once weekly for 6 months. The postoperative course was uneventful, with progressive improvement in pain and mobility. At 6-month clinical follow-up after the second procedure, the patient reported complete resolution of shoulder pain. On examination, active forward flexion was 170°, abduction 165°, and external rotation 60° bilaterally, without pain or mechanical symptoms. Internal rotation reached the level of the T8 vertebra. Functional recovery allowed return to normal daily activities without limitation. The 6-month follow-up MRI performed during postoperative surveillance demonstrated preserved joint architecture with improved marrow signal intensity (Figure 2A, 2B).

Discussion

Avascular necrosis of the humeral head is the second most common site of osteonecrosis in SCD, after the femoral head [6]. The pathogenesis involves microvascular occlusion and impaired bone perfusion, leading to subchondral collapse if untreated [7]. Early diagnosis is critical, as intervention before structural failure can preserve joint integrity

The literature on management of AVN in SCD has largely focused on conventional approaches such as pharmacological therapy, core decompression, and joint arthroplasty, with reported outcomes remaining variable due to persistent microvascular compromise. Surgical decompression, while commonly used, has not been shown to improve outcomes when added to physical therapy compared with conservative management alone in patients with sickle cell disease [8]. HBOT has gained interest as an adjunctive option in early-stage AVN. By increasing plasma oxygen tension and stimulating angiogenic and osteogenic signaling, HBOT can improve reparative potential and pain relief [9]. Several studies have demonstrated improved radiological and functional outcomes when HBOT is used for pre-collapse AVN, particularly in combination with decompressive surgery [10]. The decision to incorporate HBOT as an adjunct in this patient was guided by several clinical considerations. She presented with early-stage (stage II) bilateral humeral head AVN, a disease phase in which joint-preserving strategies are prioritized. In the context of SCD, ongoing microvascular compromise limits the effectiveness of pharmacological therapies and can reduce the durability of surgical decompression alone. HBOT was therefore selected to address the underlying hypoxia and ischemia. Alternative treatment options were less favorable due to the patient’s young age, bilateral involvement, and the desire to delay joint replacement. Additionally, the absence of contraindications to HBOT in this patient made it a suitable adjunctive option.

In our patient, the integration of HBOT with core decompression and fibular strut grafting resulted in substantial functional recovery and pain resolution. This may be explained by the complementary mechanisms of HBOT in the setting of AVN. By significantly increasing tissue oxygen tension, HBOT can counteract the chronic hypoxia associated with vaso-occlusive disease, improving osteocyte viability, and promoting angiogenesis within the necrotic humeral head. Additionally, HBOT has been shown to reduce bone marrow edema and modulate inflammatory pathways, which can contribute to pain reduction and functional improvement. When used alongside core decompression and structural support with fibular strut grafting, these effects can enhance revascularization and bone healing, particularly in early-stage disease. In patients with SCD, in which persistent microvascular compromise limits intrinsic reparative capacity, HBOT can play a valuable adjunctive role in optimizing surgical outcomes. This aligns with findings of other studies demonstrating HBOT-induced osteogenic differentiation and improved bone healing in AVN models [11,12].

A distinctive aspect of this case lies in the application of hyperbaric oxygen therapy to avascular necrosis affecting a non-weight-bearing joint. The literature on AVN in sickle cell disease has predominantly focused on weight-bearing joints, particularly the femoral head, where mechanical load is a major contributor to disease progression and structural collapse. In contrast, humeral head involvement is less common and is driven more prominently by microvascular insufficiency and repeated ischemic injury rather than mechanical stress alone. This pathophysiological distinction provides a compelling rationale for the use of HBOT, as enhanced oxygen delivery and angiogenesis can directly target the underlying ischemic mechanisms in non-weight-bearing joints. By improving local tissue oxygenation and supporting bone remodeling in an environment less influenced by mechanical load, HBOT can offer particular benefit in such anatomical sites. This case report therefore expands the potential clinical scope of adjunctive HBOT in avascular necrosis and suggests a role for its consideration beyond traditional weight-bearing joint pathology in selected patients with sickle cell disease.

It is important to acknowledge that our patient received multiple concurrent interventions, including surgical decompression, structural grafting, perioperative transfusion optimization, bisphosphonate therapy, and hyperbaric oxygen therapy. As such, the specific independent contribution of HBOT to the observed clinical and radiological improvement cannot be determined from this single case. The findings should therefore be interpreted as hypothesis-generating rather than confirmatory.

Limitations of this case report include the lack of long-term follow-up data including assessment of sustained functional and radiological outcomes and the inherent constraints of a single-patient design. However, the outcome highlights the importance of considering HBOT as a feasible, noninvasive adjunct in the early stages of AVN among SCD patients.

Conclusions

This case report illustrates that a multimodal approach combining surgical decompression with adjunctive hyperbaric oxygen therapy can provide meaningful symptomatic and functional improvement in stage II humeral head AVN secondary to sickle cell disease. Early recognition and intervention before articular collapse remain essential for preserving joint function in young patients with SCD. The application of HBOT in a non-weight-bearing joint is a noteworthy aspect of this report and highlights its potential relevance in targeting the hypoxic and microvascular mechanisms underlying osteonecrosis in this population.

In early-stage disease, HBOT can serve as a promising adjunct to joint-preserving surgical strategies by enhancing tissue oxygenation, promoting angiogenesis, and supporting bone remodeling. However, because multiple concurrent interventions were employed, including surgical decompression, structural grafting, perioperative transfusion optimization, and bisphosphonate therapy, the independent contribution of HBOT cannot be determined from this single case. These findings should therefore be interpreted as hypothesis-generating.

Further prospective and controlled studies are needed to clarify the role of HBOT as an adjunct in sickle cell-related AVN, define standardized treatment protocols, and evaluate long-term clinical and radiological outcomes in both weight-bearing and non-weight-bearing joints.