An Unusual Case of Bilateral Subperiosteal Orbital Hemorrhage

Abstract

Subperiosteal orbital hemorrhage usually occurs in the setting of facial or orbital trauma. Non-traumatic subperiosteal orbital hemorrhage (NTSOH) has rarely been reported in literature. The proposed mechanism of NTSOH is the transmission of sudden increase in cranial venous pressure to the orbital veins, which are valveless. We present a case of a 37 year old right-handed woman with a past medical history significant for type 1 diabetes, end-stage renal disease, peripheral artery disease and hypertension who developed NTSOH following an elective revision of a clotted right upper extremity arteriovenous fistula. During this procedure, she had acute eye pain, bilateral complete vision loss and emesis. CT of the orbits revealed large heterogeneously hyperdense lesions in the bilateral orbital apex extending anteriorly along the roof of the orbit, concerning for hemorrhage. Cultures obtained through nasal endoscopy were negative for a bacterial or fungal infection involving the sinuses. Ophthalmology was consulted and she underwent bilateral canthotomy and lateral cantholysis. Postoperatively, she was started on systemic and topical ocular antihypertensives, as well as prophylactic antibiotics. Visual acuity remained poor with finger counting on the right eye and lack of consistent response to light on the left eye. This case highlights periprocedural increase in systemic venous pressure secondary to a fistula repair procedure as a potential cause of NTSOH.

Introduction

Subperiosteal orbital hemorrhage usually occurs in the setting of facial or orbital trauma. ¹ Non-traumatic subperiosteal orbital hemorrhage (NTSOH) occurs less commonly, in association with bleeding disorders, sinusitis, vascular malformations and orbital lesions.^2,3,4 NTSOH usually happens in females and almost always involves the superior orbit. ²

Patients with NTSOH usually present with acute onset eye pain, diplopia, decreased vision, chemosis, proptosis or ophthalmoplegia. ⁵ CT head and orbits is the initial imaging modality of choice, revealing a well-demarcated, biconvex, non-enhancing mass with homogenous density slightly higher than that of the brain. ⁶ Inferior displacement of the adjacent orbital soft tissues is usually seen. MRI may be useful to increase the diagnostic yield by confirming the presence of blood. The signal intensity varies depending on the age of the hemorrhage, with acute lesions appearing isointense or hyperintense on T1 and hypointense on T2-weighted MRI sequences. ⁶

NTSOH is commonly associated with vascular lesions, bleeding diatheses, infection, inflammation and neoplasm. Increased venous pressure is a rare non-traumatic etiology that can lead to subperiosteal orbital hemorrhage. A sudden increase in intracranial venous pressure can trigger a rupture of a vein in the subperiosteal space.^5,7 Several cases of NTSOH have been reported associated with vomiting, straining, parturition, weight lifting, scuba diving, coughing, strangulation.^3,8,9,10,11 Here we present a unique case of NTSOH following a dialysis fistula declotting procedure.

Case Presentation

A 37 year old right-handed woman with a history of type 1 diabetes, end-stage renal disease on intermittent hemodialysis complicated by recurrent thrombosis of arteriovenous fistula, peripheral arterial disease status post femoro-popliteal bypass requiring anticoagulation with Eliquis, right foot amputation secondary to osteomyelitis, and hypertension initially presented to an outside hospital for an elective revision of a clotted right upper extremity arteriovenous fistula. Her last dose of Eliquis 5 mg BID was the night prior.

She underwent right upper extremity fistulogram, declotting and venoplasty with outflow stenosis. During the procedure, she felt flushing and pain behind her right eye which extended to the left eye. Two hours later, she developed sudden complete vision loss bilaterally, associated with epistaxis and emesis. Stroke code was activated and she was transferred to the emergency department. She was found to have systolic blood pressures elevated to 230 mm Hg. While CT head was negative for acute hemorrhage or ischemia in the brain parenchyma, there were large heterogeneously hyperdense lesions in the bilateral orbital apices extending anteriorly along the roof of the orbit concerning for hemorrhage, along with intraorbital mass effect and bilateral proptosis (Figure 1A and 1B). CT-Angiography was deferred given kidney function and plans for further assessment with Magnetic Resonance Imaging (MRI) with venography. She was transferred to the neurologic intensive care unit at our institution for further management.

Figure 1.

(A) Coronal and (B) Axial CT of the brain and orbits show the well-demarcated bilateral biconvex hemorrhages (red arrows) in the superior orbit. The medial aspect of the lesions run along the superior rectus muscles. (C) T1-weighted axial magnetic resonance imaging (MRI) of the orbits demonstrate biconvex lesions of mixed hyper/isointensity (red arrows) in the orbital roof bilaterally, representing NTSOH. (D) T2-weighted axial MRI of the orbits shows low signal lesions (red arrows), which along with the T1 sequence findings, reflect acute to early subacute hemorrhages.

Her neuro-ophthalmologic exam was notable for significant periorbital swelling, chemosis and proptosis bilaterally. Ophthalmologic examination depicted pinpoint, nonreactive pupils bilaterally confirmed on automated pupilometer with absence of light perception in either eye. Bilateral optic disc pallor was noted on funduscopic exam. Intraocular pressure was measured as 46 mm Hg in OD and OS. Bilateral orbital hemorrhages were demonstrated on the contrasted MRI brain and orbits which was completed on admission day 2 (Figure 1C and 1D). No acute intraparenchymal abnormalities, including mass or abnormal enhancement were found. MR venography was negative for cavernous sinus thrombosis or superior ophthalmic vein thrombosis. DVT ultrasound of the upper and lower extremities demonstrated an acute right internal jugular (RIJ) thrombus. Cultures obtained through nasal endoscopy did not show evidence of an acute bacterial or fungal sinus infection.

She was evaluated by ophthalmology and underwent bilateral canthotomy and lateral cantholysis. Post procedure, intraocular pressure decreased to 17 mm Hg OD and 21 mm Hg OS. She continued to be treated for ocular hypertension with timolol-dorzolamide, latanoprost, brimonidine and prednisolone ophthalmic drops. Visual acuity in the left eye remained but the right eye had an improvement to finger counting.

She was started on IV steroids to decrease inflammation. She continued to receive veno-venous hemodialysis and repeat CT venography head and neck showed no evidence of venous sinus thrombosis. Her condition stabilized and she was transferred to the regular nursing floor on admission day 9. Her blood pressure and blood glucose remained well-controlled. She was cleared for re-initiation of anticoagulation by ophthalmology and started on heparin drip with bridge to warfarin for the treatment of the RIJ DVT. On admission day 14, she reported transient distal right arm numbness. Repeat CT head and CT orbit which were negative for bleed. Anticoagulation was resumed after confirming coagulation markers were within the normal range.

Later that evening, she complained of nausea and sweating. She was found down and pulseless overnight. Cardiopulmonary resuscitation was performed but despite return of spontaneous circulation, the patient ultimately expired. An autopsy was performed, which revealed acute ischemia of the left ventricle with an organized thrombus in the right atrium and occlusive thrombi in the mesenteric arteries. Her cardiac arrest was felt to be likely secondary to a thrombogenicity process such as disseminated intravascular coagulation, though the underlying precipitant remains unclear.

Discussion

While several reports of NTSOH associated with cardiac, vascular procedures or endoscopy have been described in literature, there are no reported cases describing NTSOH occurring in the setting of declotting and venoplasty of a dialysis fistula. ^2,5,7,12 The proposed mechanism is sudden increase in intracranial venous pressure, similar to the previous descriptions with vomiting, labor or strangulation.^2,10,11 The absence of valves in the orbital veins allows for the transmission of increased intrathoracic pressure to the orbital veins. The superior aspect of the orbit is the most common location of NTSOH, followed by the medial orbit. It has been suggested that loose adhesions between the periosteum and orbital bones allow for the development of orbital subperiosteal hemorrhage, originating from the small veins crossing the subperiosteal space between the bone of the orbital roof and periorbita.^9,12

There are 33 cases of NTSOH reported in the literature that are associated with a rise in venous pressure, the majority of which (29/33 patients) had unilateral NTSOH. Similarly, perioperative general anesthesia use which has also been linked to NTSOH due to a proposed mechanism of increase in venous pressure, was also associated with unilateral vision loss in the majority of cases. ¹³ The cause of unilaterality in the majority of these cases remains unclear, however can be due to anatomical differences between the two orbits rendering one side more susceptible to systemic pressure changes. Our patient developed bilateral NTSOH, which is a less common presentation in reported NTSOH cases associated with increased venous pressure.

Conservative management is the preferred method of treatment in cases with small asymptomatic NTSOH with normal optic nerve function.^2,13 Some authors support the use of systemic or topical ocular antihypertensives, especially for patients with moderately elevated intraocular pressure. ² Most cases of NTSOH are expected to resolve gradually with the correction of underlying conditions such as infection, inflammation or coagulopathy. As illustrated in our case, if vision is compromised, as evidenced by reduced visual acuity or optic nerve involvement, surgical evacuation of the hematoma is indicated. The prognosis of reported cases in literature are favorable, with restoration or improvement of vision over several weeks.^13,14

In conclusion, acute presentation of NTSOH warrants a thorough investigation for underlying vascular abnormality, venous congestion, infection and coagulopathy. Given the temporal relationship with the AV fistula repair procedure, our case highlights the importance of consideration of venous pressure changes secondary to a fistula repair procedure, as a potential cause of acute subperiosteal orbital hemorrhage.

ORCID IDs

Sanem P. Uysal, MD https://orcid.org/0000-0002-5633-4961

Catherine E. Hassett, DO https://orcid.org/0000-0003-1949-5181