Sickle Cell Crisis-Induced Painless Bilateral Orbital Bone Infarction: A Case Report

Abstract

Introduction

Sickle cell disease is associated with painful vaso-occlusive crises, bone infarction, avascular necrosis, and osteomyelitis. Sickle cell orbitopathy is a rare manifestation with signs similar to periorbital cellulitis and has subtle radiologic features.

Case Presentation

We report a case of a five-year-old girl with sickle cell who presented with bilateral orbital infarctions and subperiosteal hematomas, notably without periorbital pain, and was treated with antibiotics and steroids.

Conclusion

Physicians should be aware of this rare manifestation, especially in the pediatric population, as it can affect subsequent management.

Introduction

Sickle cell disease (SCD) is a group of inherited disorders caused by genetic mutations in the hemoglobin β-globin gene that results in the production of hemoglobin S instead of normal adult hemoglobin [1]. The tetramer of hemoglobin S is poorly soluble and polymerizes when deoxygenated, which leads to distortion of red blood cells and vaso-occlusion [1, 2]. Bony infarctions may result from recurrent vaso-occlusive episodes and hypoxia, leading to severe pain, osteonecrosis, and potential collapse of long weight-bearing bones [3]. Orbital bone infarction in SCD is rare as facial bones have a small amount of marrow [4]. When orbital infarctions do occur, they are typically seen in children due to the higher amount of active bone marrow [5]. Orbital infarction syndrome typically presents with pain, erythema, and periocular edema. Conjunctival hyperemia, chemosis, proptosis, ptosis, exposure keratitis, and ophthalmoplegia have been described [6]. While there are limited case reports documenting orbital bone infarctions in patients with SCD [4, 6], instances of painless SCD orbitopathy are exceedingly rare, with only a handful of cases reported in the literature [5, 7–9]. We report a rare case presentation of painless bilateral orbital infarctions with associated bilateral orbital hematomas that highlights the importance of recognizing orbital infarction as a potential non-painful complication in sickle cell patients, which may be misdiagnosed as infection or trauma if not carefully evaluated.

Case Presentation

A 5-year-old African American girl with a past medical history of SCD (HbSS) on hydroxyurea and penicillin prophylaxis presented in the emergency department with 1 week of bilateral periorbital edema, abdominal pain, low-grade fever, and diarrhea. There was no discharge or orbital pain. On examination, she had bilateral periorbital edema, worse on the right than the left, without overlying erythema or warmth (Fig. 1). Extraocular movements were full, and visual acuity was at least counting fingers, though it was difficult to assess due to limited patient cooperation. Anterior and posterior ophthalmic examinations were unremarkable except for a larger cup-to-disc ratio of 0.7 bilaterally.

Fig. 1.

External photograph of the patient demonstrating bilateral periorbital fullness and right sided ptosis without inflammatory signs.

Magnetic resonance T1-weighted post-contrast fat-saturated imaging revealed well-defined subperiosteal oval-shaped collections with surrounding extraconal enhancement and no significant orbital fat enhancement, which was more clearly visualized on T2-weighted sequences (Fig. 2a). Magnetic resonance T1 post-contrast coronal imaging also showed hyperintense subperiosteal extraconal lesions in the lateral aspects of the bilateral orbits with resulting displacement of the intra-orbital contents and proptosis of the right globe (Fig. 2b). Moreover, fat-suppressed T2-weighted axial MRI showed no inflammatory changes in the orbital fat with focal areas of hyperintensity of the subperiosteal lesions bilaterally (Fig. 2c). Magnetic resonance angiography and venography were normal. These exam and imaging findings were most consistent with bilateral orbital infarctions with accompanying bilateral subperiosteal orbital hematomas.

Fig. 2.

a Axial T1-weighted post contrast fat-saturated MRI showing oval shaped well-defined collections with surrounding extraconal enhancement and no significant enhancement of the orbital fat, abnormal heterogenous enhancement of the sphenoid bones (arrows). b Coronal T1-weighted MRI showing hyperintense subperiosteal extraconal fluid collections in the lateral aspects of the bilateral orbits with resulting displacement of the intra-orbital contents and proptosis of the right globe. c Fat suppressed T2-weighted axial MRI showing no inflammatory changes in the orbital fat with focal areas of hyperintensity of the lesions and abnormal signals in bilateral sphenoid bones (arrows).

Following admission, urinalysis revealed a urinary tract infection, and the patient was started on broad-spectrum antibiotics. Additionally, she received blood transfusions for low hemoglobin and was given one dose of intravenous methylprednisolone at 1 mg/kg with marked improvement of her periorbital edema the following day. The patient was discharged with trimethoprim-sulfamethoxazole for outpatient treatment of her urinary tract infection, and she was continued on her prophylactic penicillin and hydroxyurea. At the 6-month follow-up, the patient’s mother reported mild residual periorbital swelling without eye pain, discharge, extraocular movement abnormalities, or vision changes.

Discussion

This case demonstrates a rare presentation of sickle-cell disease featuring painless bilateral orbital infarctions and bilateral subperiosteal orbital hematomas. Four cases of painless SCD orbitopathy have been previously described (Table 1) [5, 7–9]. The typical pain that is associated with bone infarction is believed to arise from the inflammatory reaction to the infarct, resulting in edema and pressure on the adjacent structures [2–4]. In our patient, the absence of erythema and warmth upon physical examination is suggestive of a gradual development of the hematomas, which may explain the lack of pain in the setting of a limited inflammatory response.

Table 1.

Summary of cases involving SCD painless orbital infarction

​	Case 1 (Janssens et al. [5])	Case 2 (Alsaggaf et al. [7])	Case 3 (McBride et al. [8])	Case 4 (Hanna et al. [9])
Patient history	17-year-old boy	4-year-old girl	5-year-old boy	19-year-old man
Presenting complaint	Generalized pain	BL lower limb pain, painless L orbital swelling	Swelling of R upper and lower eyelids	Sickle crisis
Presence of systemic pain	Yes	Yes	No	Yes
BCVA	No visual disturbances	20/40 OU	20/30 OD, 20/25 OS	20/20 OU
EOM	Not mentioned	Limited supraduction and abduction of OS	Decreased upgaze	Normal
Periorbital pain	No	No	No	No
Periorbital edema	BL, OS>OD	OD only	OD only	OD only
Periorbital erythema	No	Yes	No	No
Conjunctiva/sclera	Normal	OS mild injection, mild subconjunctival hemorrhage	OD injection, chemosis	Normal
Imaging	MRI: periorbital edema of OS and bone anomalies of L orbital wall extending intra-orbitally	CT: L superior lateral orbital subperiosteal fluid collection; R inferolateral orbital subperiosteal collection. MRI: BL subacute hematomas of the sphenoid	CT: superolateral fluid collection of R orbit	CT: extraconal mass on R superolateral orbit. MRI: subperiosteal orbital hematoma of R orbit
Mass effect	On L lateral rectus	On L lateral and superior recti muscles	On R lacrimal gland and orbital contents	None
Treatment	IV antibiotics	Systemic antibiotics, transfusion	IV antibiotics	IV antibiotics, corticosteroids

Sickle cell orbital infarction is a diagnostic challenge as it closely resembles several other conditions, such as osteomyelitis and orbital cellulitis. Computed tomography and MRI can help distinguish between the diagnoses and identify the presence of orbital hematoma associated with orbital infarction [4]. In this case, fat-saturated T2 MRI (Fig. 2c) indicates an absence of inflammatory changes in the orbital fat, with all findings confined to the bone and surrounding subperiosteal collections. The lack of diffuse enhancement and inflammatory signs of the orbital fat and soft tissues around the orbit support the absence of cellulitis or orbital abscess and align more with a hematoma secondary to infarction rather than an infectious process. Given this patient initially received antibiotics for a UTI, it may be impossible to definitively rule out infection. Surgical or histological proof may provide the ultimate diagnosis, though in our case, the patient did not undergo these procedures. Nevertheless, in a patient with SCD who presents with acute periorbital pain and swelling, with or without proptosis and ophthalmoplegia, the differential diagnosis should consistently consider preseptal and orbital cellulitis, orbital abscess, malignancy, osteomyelitis, and orbital bone infarction.

Orbital infarction is a rare manifestation of SCD that can lead to significant complications if treatment is delayed, one of which is orbital hematoma formation. Orbital hematomas arising from bone infarction are hypothesized to originate from the extravasation of blood cells caused by damaged and necrosed blood vessels [4]. Inflammation from the infarction and pressure from orbital hematomas could lead to the visually threatening orbital compression syndrome [9]. Management of SCD-related orbital hematomas may be limited to treatment of the sickle cell crisis with intravenous fluids and analgesia while monitoring orbital involvement [6]. Supportive corticosteroids have also been utilized [9]. In more severe cases with evidence of hematoma-induced optic nerve dysfunction, surgical drainage might be indicated [6].

In all the reported cases of painless orbital infarctions, antibiotics were administered given high concern for infection such as osteomyelitis [5, 7–9]. Outside of the management of concomitant infections, antibiotics may not be beneficial. Of the four previously published cases of painless SCD orbital infarctions, only one utilized steroids [5, 7–9]. Our patient was treated with one dose of intravenous methylprednisolone. While she overall had a limited inflammatory component to her presentation, the steroids were given later during her hospitalization course to hasten the resolution of edema, thought to be associated with a component of intramedullary inflammation, albeit mild. Furthermore, some studies have suggested that steroids reduce the transfusion demand and the duration of hospitalization during SCD crises [10].

The occurrence of bilateral involvement in patients presenting with orbital hematomas secondary to SCD is interesting. In a series reported by Sokol in 2008, the bilateral form represented approximately one-third of the documented cases, indicating that although uncommon, bilateral presentation is a documented manifestation. Notably, within the past 6 years, only five additional cases have been reported in the literature, emphasizing the rarity of this finding [11–13].

This case emphasizes SCD can cause a rare presentation of orbital infarctions and hematomas that present without typical signs of inflammation, namely pain. Treatment involves management of the sickle cell crisis and possibly adjunct steroids or surgical evacuation of the hematomas. Cases with a concern for infection should be treated with antibiotics. Although uncommon, clinicians should remain vigilant regarding this rare manifestation of painless orbital infarction accompanied by bilateral subperiosteal hematomas in the context of SCD, as well as its potential management options.

Statement of Ethics

Collection, evaluation, and storage of protected patient health information and the writing of this case report were compliant with the US Health Insurance Portability and Accountability Act of 1996 and the Declaration of Helsinki. Per the Committee for the Protection of Human Subjects at UTHealth Houston, no Institutional Review Board approval was needed for this case report. Written informed consent was obtained from the patient’s parents for publication of the details of their medical case and any accompanying images. The authors have completed the CARE Checklist for this case report and attached it as online supplementary material (for all online suppl. material, see https://doi.org/10.1159/000550138) [14].

Conflict of Interest Statement

The authors have no conflicts of interest to declare.

Funding Sources

This study was not supported by any sponsor or funder.

Author Contributions

Writing – original draft: L.F. and M.S. Writing – review and editing: L.F., M.S., A.Kak., A.Kas, and C.S. Visualization: L.F., M.S., and A.Kak. Conceptualization: T.M. and Y.C. Supervision: C.S., T.M., and Y.C.

Funding Statement

This study was not supported by any sponsor or funder.

Data Availability Statement

All data generated or analyzed during this study are included in this article and its online supplementary material files. Further enquiries can be directed to the corresponding author.