External Compressive Ischemic Orbitopathy following cocaine use

Abstract

Purpose

To report a rare case of External Compressive Ischemic Orbitopathy (ECIO), otherwise known as “Saturday Night Retinopathy”, following cocaine use, characterized by its distinctive presentation, diagnostic challenges, and management approach.

Observations

A 48-year-old female with a history of polysubstance abuse and schizoaffective disorder presented with sudden, painless vision loss in one eye and limitations in extraocular motility. Imaging studies demonstrated diffuse swelling of the extraocular muscles without sinus disease, and toxicology screening confirmed recent cocaine use, supporting a diagnosis of ECIO.

Conclusions and importance

The patient was diagnosed with ECIO following cocaine use and left against medical advice prior to completing intravenous steroid therapy. Subsequent clinic visits showed improved ophthalmoplegia and a small improvement in visual acuity from light perception to hand motion. This case showcases the importance of considering ECIO in patients presenting with vision loss, ophthalmoplegia, and proptosis with a history of substance abuse.

1. Introduction

External Compressive Ischemic Orbitopathy (ECIO), also known as “Saturday Night Retinopathy", is a rare condition characterized by ischemic damage to the orbit and retina following prolonged external pressure. In the 1950s, these cases were first described to be iatrogenic in nature, described in patients who underwent neurosurgical procedures requiring prolonged prone positioning under general anesthesia, with recent cases still reported in the literature.^1,2 ECIO was later reported in patients outside the hospital setting after episodes of prolonged unconsciousness or immobilization, leading to venous compression and subsequent retinal and choroidal ischemia associated with substance use, often with worse visual outcomes than iatrogenic cases.³

Due to the rarity of reported cases and its status as a diagnosis of exclusion, ECIO can be difficult to recognize. As substance use continues to rise, awareness of this condition becomes increasingly important. In this report, we present a rare case of ECIO following cocaine use, characterized by its unique clinical presentation, diagnostic challenges and clinical outcome after management with IV steroids.

2. Case report

A 48-year-old female patient with a past medical history of polysubstance abuse and schizoaffective disorder presented with painless vision loss and inability to move her left eye. She reports having fallen to the ground the night prior. After regaining consciousness, she returned to bed. Upon waking in the morning, she noticed her eye was swollen and blind and went to the emergency department. Due to her substance use during the event and psychiatric disorder, the patient did not provide further details regarding the chronology of the events or specifics. Her visual acuity was 20/20 in the right eye and light perception (LP) in the left. Intraocular pressure (IOP) was 12 and 13. Pupil examination was normal in the right eye (OD) and showed a 4-mm fixed pupil in the left eye (OS) with a relative afferent pupillary defect (rAPD) by reverse. Motility was full in the right eye and frozen in the left with −4 in all gaze positions (Fig. 1A). The patient exhibited periorbital edema, chemosis and proptosis.

Fig. 1.

A) External photograph demonstrating left proptosis, chemosis, and periorbital edema with severe limitations in extraocular movements in all directions. B) Coronal T1-weighted fat-suppressed MRI demonstrating left-sided diffuse enlargement and enhancement of all extraocular muscles.

Fundoscopy was significant for normal optic nerves without disc edema and a grossly ischemic macula with a cherry red spot in the left eye. Computed Tomography Angiography (CTA) of the head and neck showed no evidence of arteriovenous fistula or ophthalmic artery or vein occlusion. Magnetic resonance imaging (MRI) revealed diffuse enlargement of the extraocular muscles without any evidence of sinus disease (Fig. 1B). MRI Venogram was negative for venous sinus thrombosis. Vital signs were within normal limits and initial labs were significant for a normal white blood cell count. A urine toxicology screen was positive for cocaine. Empiric orbital-cellulitis coverage was initiated with IV Vancomycin and IV ampicillin-sulbactam. Orbital inflammatory and hypercoagulability labs were ordered which were all within normal limits. Workup for infectious causes was unremarkable. ENT performed a nasal endoscopy and mucosal biopsy which was negative for invasive fungal pathogens. Given the lack of sinus disease on imaging and a negative mucosal biopsy for fungal elements, the patient was initiated on a 5-day course of IV methylprednisolone 1 g daily. However, the patient left against medical advice after completing one dose.

Four weeks later, the patient returned to the clinic and was found to have improvement in her vision from LP to hand motion and improvement in her motility to −2 in all gazes. Optical Coherence Tomography (OCT) of her macula (Fig. 2A–B) showed retinal atrophy affecting all layers. Her fundus exam (Fig. 2C–D) was significant for optic nerve pallor and cupping, as well as arteriolar narrowing. Fundus autofluorescence (Fig. 2E–F) showed an area of localized retinal pigment epithelium changes. Ultimately, due to the patient's clinical presentation, substance use, and negative workup, she was diagnosed with ECIO which was suspected to have occurred after lying unconscious and prone on the ground for a considerable duration of time.

Fig. 2.

Images obtained 1 month after initial presentation. OCT macula of the right eye (A) with normal foveal contour and of the left eye (B), demonstrating significant retinal atrophy affecting all layers. Fundus photography of the right eye (C) and the left eye (D) showing blunted foveal reflex, nerve pallor and cupping, and arteriolar narrowing. Fundus autofluorescence of the normal right eye (E) and the affected left eye (F) demonstrating localized retinal pigment epithelium changes.

3. Discussion

Given the fact that ECIO is a diagnosis of exclusion, other orbital pathologies had to be considered and ruled out before arriving at the final diagnosis. Posterior ischemic optic neuropathy (PION) was an unlikely diagnosis because the presentation featured marked proptosis, chemosis, frozen extraocular motility, and diffuse enlargement of every extraocular muscle on MRI. These findings indicate a compartment-wide orbital process rather than an isolated ischemia to the optic nerve. Granulomatous or infiltrative inflammation (sarcoidosis, granulomatosis with polyangiitis) was a less likely cause given the normal inflammatory lab values, the absence of sinus disease or focal orbital mass on imaging, and a negative nasal-mucosal biopsy for granulomatous or fungal pathology. Infectious orbital cellulitis was ruled out because the patient was afebrile with a normal white-blood-cell count, had no sinus opacification or sub-periosteal abscess on imaging, and an infectious work-up remained unremarkable. Excluding these other potential causes supports the diagnosis of External Compressive Ischemic Orbitopathy.

Multiple mechanisms have been proposed in the literature to explain the pathogenesis of External Compressive Ischemic Orbitopathy (ECIO). The prevailing theory suggests prolonged external pressure on the orbit, typically due to substance use or altered consciousness, leads to arteriovenous compression, decreased perfusion pressure, and subsequent retinal and choroidal ischemia.1, 2, 3, 4, 5, 6, 7 Upon release of pressure, these compressed ischemic blood vessels dilate, leading to orbital edema, proptosis, ophthalmoplegia, and massive retinal edema.³ In ECIO, reduction and/or complete absence of signal on electroretinogram (ERG) are seen suggesting compression of the ophthalmic artery as opposed to the central retinal artery where ERG results would be normal.³

Recent literature highlights that cocaine can injure the optic nerve through at least three distinct mechanisms—direct toxic injury, chronic granulomatous inflammation, and acute orbital compression and ischemia.¹⁰ Burggraaf-Sánchez et al. described a 36-year-old man who developed bilateral, painless vision loss with striking peripapillary hemorrhages only three days after intranasal cocaine and heavy ethanol use; multimodal imaging supported a toxic optic neuropathy without orbital congestion, and vision recovered to 20/20 after nutritional repletion and cessation of use.¹¹ In contrast, Siemerink et al. reported two long-term intranasal cocaine users who presented with unilateral optic neuropathy, an orbital mass contiguous with a destroyed nasal septum, and biopsy-proven chronic inflammation. Despite corticosteroids, visual acuity failed to improve, presumably from prolonged compression or infiltrative damage. Our patient differs from both scenarios: she experienced acute external compressive ischemic orbitopathy (ECIO) marked by diffuse extraocular muscle enlargement and rapid visual decline following cocaine use. These case reports represent the potentially wide continuum of cocaine-related orbital pathology which includes primary vascular toxicity, chronic granulomatous inflammation, and fulminant orbital ischemia. This wide variance in clinical and pathophysiological outcomes highlights the importance of tailoring medical management.

Although ECIO is rare, the case presented here adds to the growing spectrum of its clinical manifestations, particularly in the context of substance use and highlights the importance of considering this diagnosis in such presentations. The coexistence of polysubstance abuse and a complex psychiatric history makes history-taking difficult. This case highlights the unique diagnostic challenges faced in this situation.

Due to the rarity of ECIO, there are few studies on the management of this disease. There were few cases reported in the literature where IV steroids were utilized, but due to rarity of this disease, there is no clear data on how this affects clinical outcome.^8,9 In this case, the patient was initially managed with intravenous dexamethasone to reduce inflammation and mitigate the compressive effects on the optic nerve and surrounding structures. A key limitation of this case is that the patient left against medical advice (AMA) after receiving only a single 1-g dose of IV methylprednisolone, precluding completion of the planned 5-day course. Despite this, the patient showed gradual improvement in visual acuity and eye movement. Cases in the literature often report improvement in orbital edema and ocular motility, but vision often remains poor with minimal or no improvement even in cases without reports of steroid use.^4,5,8 Our patient's disease course is consistent with these findings. Whether steroids may have played a role in improving the patient's outcome or expediting her recovery is uncertain.

As the literature on ECIO remains sparse, additional studies would be ideal to further clarify its pathogenesis, optimal management, and the effects of factors such as duration of compression on long-term prognosis. It may also be beneficial to assess whether use of IV steroids may play a role in improving visual and functional outcomes. However, the patient population in which these cases typically arise often has a history of substance use, leading to poor follow-up. Additionally, the scarcity of this disease makes comprehensive study of ECIO difficult.

4. Conclusion

In summary, this case report contributes to the understanding of External Compressive Ischemic Orbitopathy (ECIO) and presents a unique instance of the condition following cocaine use. The specific clinical presentation observed in this patient, including sudden vision loss and the diagnostic challenges, adds to the known spectrum of ECIO manifestations. Due to the rarity of this condition and the limited data available, further studies, particularly longitudinal follow-ups and the role of IV steroids in improving visual and ocular motility outcomes, are necessary to better understand the natural course and potentially develop management strategies for ECIO cases.

CRediT authorship contribution statement

Ethan Abizadeh: Writing – review & editing, Writing – original draft, Methodology. Wenyu Deng: Writing – review & editing, Writing – original draft, Validation, Data curation. Kevin Babapour Digaleh: Writing – review & editing, Writing – original draft. John Petrie: Writing – review & editing, Writing – original draft, Data curation. Nickisa Hodgson: Writing – review & editing, Writing – original draft, Validation, Supervision, Data curation, Conceptualization.

Patient consent

Written consent to publish this case has not been obtained. This report does not contain any personal identifying information.

Disclosures

None.

Acknowledgements and disclosures

No funding or grant support

Financial support

None.

Declaration of competing interest

The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.