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Abbreviations

aPTT

activated partial thromboplastin time

CLD

chronic liver disease

CT

computed tomography

EGD

esophagogastroduodenoscopy

ERCP

endoscopic retrograde cholangiopancreatography

ETOH

alcohol

HCV

hepatitis C virus

IOP

intraocular pressure

NR

not reported

PT

prothrombin time

ROTEM

rotational thromboelastometry

SLKT

simultaneous liver‐kidney transplantation

SOH

spontaneous orbital hemorrhage

Case Presentation

A 60‐year‐old man with decompensated alcoholic cirrhosis—refractory ascites and hepatic hydrothorax, intermittent encephalopathy and esophageal variceal hemorrhage, and progressive chronic kidney disease secondary to hepatorenal syndrome—was being treated in our intensive care unit while wait‐listed for a simultaneous liver‐kidney transplantation (SLKT) at a Model for End‐Stage Liver Disease score of 35. His additional comorbidities included hypertension and paroxysmal atrial fibrillation. His current medications included lactulose, rifaximin, and midodrine. He admitted to heavy alcohol consumption for years but had quit 10 years ago, and he never used or abused tobacco or illicit substances. In the absence of similar prior symptomatology, the patient complained of sudden‐onset right‐sided eye pain (aggravated with cough), blurry vision and diplopia, without left eye symptoms. He was afebrile, normotensive (135/55 mm Hg), and had a normal heart rate (85 beats/min). An ophthalmological examination revealed right periorbital edema, ecchymosis, proptosis, and conjunctival injection (Fig. 1); visual acuity of 20/80 bilaterally, and right and left intraocular pressures (IOPs) of 19 and 14 mm Hg, respectively; and normal pupil, confrontational field, extraocular movements, cornea, iris, lens, optic nerve, and macula bilaterally.

FIG 1.

Comparative photographs of the patient’s eyes, open (A) and closed (B), demonstrate right‐sided periorbital edema, ecchymosis, proptosis, and conjunctival injection.

1. Which, if any, laboratory and/or diagnostic studies would be required to make the correct diagnosis and direct the treatment of this patient?

1. Coagulation panel only

2. Coagulation panel and computed tomography (CT) scan of head and orbits

3. Coagulation panel, CT scan of head and orbits, and IOPs

As expected in patients with cirrhosis, a reduced platelet count (44,000/μL) and fibrinogen level (121 mg/dL) and prolonged prothrombin time (PT; 21.7 seconds) and activated partial thromboplastin time (aPTT; 37 seconds) were noted on biochemical laboratory tests. A noncontrast CT scan of the head and orbits showed a lenticular‐shaped high‐density lesion, suggestive of a focal hematoma (average attenuation value of 70 Hounsfield units), measuring 2.9 × 1.5 × 3.0 cm just superior to the right orbit in the retroconal fat causing downward displacement of ipsilateral orbit and exophthalmos (Fig. 2).

FIG 2.

CT scan of the head and orbits demonstrates a lenticular‐shaped hematoma superior to and causing downward displacement of the orbit (solid arrow in [A] coronal and [B] sagittal views) and resultant ipsilateral exophthalmos (dashed arrow in C, axial view).

2. What is your leading diagnosis at this time?

1. Central retinal artery occlusion

2. Spontaneous orbital hemorrhage (SOH)

3. Orbital compartment syndrome

4. Acute stroke

3. What is the appropriate management of this patient?

1. Observation only

2. Orbitotomy and hematoma evacuation

3. Rotational thromboelastometry (ROTEM)‐directed correction of coagulopathy

4. Lateral canthotomy/cantholysis

Presumably, transient elevation of intracranial venous pressure (during cough) in the face of cirrhosis‐related coagulopathy caused an SOH in our patient. Normal IOP and unchanged visual acuity warranted no surgical intervention. ROTEM‐directed transfusion of fresh frozen plasma and platelets corrected the coagulopathy. On follow‐up, an orbital noncontrast CT scan on post‐SOH day 7 showed reduction in the size of the hematoma without sequelae, and on day 24 the patient underwent a successful SLKT and was discharged to rehabilitation 3 weeks later.

Discussion

SOH, sometimes referred to as nontraumatic orbital hemorrhage, is a rare entity. SOHs are seldom idiopathic and usually occur due to underlying factors, such as vascular malformations, increased intracranial venous pressure, bleeding disorders, infection, inflammation, or neoplasms. ¹ Bleeding disorders can arise in isolation or as a result of various disease processes, including chronic liver disease (CLD). Coagulopathy of CLD predisposes patients to spontaneous hemorrhage. ² However, SOH in the setting of CLD is exceptionally rare and has been reported in only 11 patients to date ³ , ⁴ , ⁵ , ⁶ , ⁷ , ⁸ , ⁹ , ¹⁰ , ¹¹ , ¹² (see Table 1). Among these patients, the most common cause of liver disease was alcoholic cirrhosis (50%), and 25% of the patients had never been diagnosed with any type of liver disease until being worked up for SOH, which stresses the importance of a broad differential when evaluating patients with SOH from a presumably “idiopathic” cause. ³ , ⁴ , ⁵ , ⁶ Symptoms, usually sudden‐onset, include orbital pain, periorbital edema, and ecchymosis, and many (75%) experience variable visual disturbances. ¹ The majority of the SOHs in patients with CLD were located in the superior orbit (58% superior, 33% superomedial), and the only case reported in the inferior orbit was in a 13‐year‐old boy with autoimmune hepatitis. ³

TABLE 1.

Clinical Features of the Reported Cases of SOH in Patients With CLD

Case Report	Age (years)	Sex	Cause of Liver Disease	Coagulopathy*	Symptoms	Sudden onset	Vison Affected	Inciting Event	Laterality	Location of Hemorrhage	Management	Outcome
Griffeth et al. (1997) 7	33	Woman	ETOH	Yes	Diplopia, edema, bilateral progressive orbital pain and decrease in vision	Yes	Yes	NR	Bilateral	Superomedial	Nonsurgical	Improvement of symptoms
Meltzer et al. (2019) 11	45	Man	HCV	NR	Orbital pain, periorbital edema	Yes	No	Emesis	Right	Superior	Nonsurgical	Initial improvement of symptoms, lost to follow‐up
Modi et al. (2012) 6	52	Woman	ETOH	Yes	Orbital pain, difficulty opening eye	No (24 hours)	Yes	Cataract surgery 12 days prior	Right	Superior	Orbitotomy	Improvement of symptoms
Moorthy et al. (1992) 4	35	Woman	Hepatitis B virus	Yes	Vertical diplopia	Yes	Yes	Emesis	Left	Superior	Nonsurgical	Improvement of symptoms
	49	Woman	ETOH	Yes	Periorbital edema, subconjunctival hemorrhage, proptosis	Yes	Yes	NR	Left	Superomedial	Orbitotomy	Initial improvement of symptoms, lost to follow‐up
Nemiroff et al. (2014) 5	33	Woman	ETOH	Yes	Periorbital ecchymosis	No (24 hours)	Yes	NR	Left	Superior	Lateral canthotomy /cantholysis	No improvement of symptoms, succumbed to complications of CLD
Parakh et al. (2009) 12	59	Man	HCV	NR	Periorbital edema and ecchymosis	Yes	NR	EGD	Bilateral	Superior	Nonsurgical	Improvement of symptoms
Russell et al. (2011) 8	42	Man	Intrahepatic cholangiocarcinoma	Yes	Bilateral orbital pain, periorbital edema, diplopia, headache, right‐sided ptosis	Yes	No	ERCP	Bilateral	Superomedial	Nonsurgical	Improvement of symptoms
Saeed et al. (2014) 9	51	Woman	Unspecified cirrhosis	Yes	Orbital pain, periorbital edema, diplopia	Yes	Yes	EGD	Right	Superomedial	Orbitotomy	Improvement of symptoms
Sibille et al. (2012) 10	35	Man	ETOH	Yes	Blindness	Yes	Yes	Emesis	Bilateral	Superior	NR	NR
Sires et al. (1993) 3	13	Man	Autoimmune hepatitis	Yes	Orbital pain, periorbital edema and ecchymosis, vertical diplopia	No (18 hours)	Yes	NR	Left	Inferior	Nonsurgical	Improvement of symptoms
Current case	60	Man	ETOH	Yes	Orbital pain, diplopia, decreased visual acuity	Yes	Yes	Cough	Right	Superior	Nonsurgical	Improvement of symptoms

*Defined as prolonged PT/aPTT and/or thrombocytopenia.

Trauma is the most common cause of orbital hemorrhages ⁴ ; however, several factors may predispose patients to the risk for SOH. ¹ Specifically, in patients with CLD, the tendency to spontaneously bleed exists secondary to multifactorial coagulopathy from portal hypertension, endothelial dysfunction, sepsis, concomitant renal failure, and derangements in coagulation hemostasis (reduced procoagulant and/or augmented anticoagulant factors). ² Most of the cases (83%) listed in Table 1 had evident coagulopathy on biochemical laboratory tests (prolonged PT, aPTT, and/or thrombocytopenia). ³ , ⁴ , ⁵ , ⁶ , ⁷ , ⁸ , ⁹ , ¹⁰ That said, coagulopathy may not always be the only cause for SOH. ⁴ , ⁸ , ⁹ , ¹⁰ , ¹¹ , ¹² Events such as retching and/or emesis, related ⁸ , ⁹ , ¹² or unrelated ⁴ , ¹⁰ , ¹¹ to upper endoscopic procedures, or coughing, as in our patient, in the presence of coexisting coagulopathy may provoke SOH. The postulation for such an occurrence is that increased intrathoracic/abdominal pressures result in increased central venous pressure, which ultimately on intracranial transmission to the orbital vasculature via valveless veins leads to congestion, decompensation, and rupture. ¹²

Most patients with SOH do not necessitate surgical intervention and may need only correction of coagulopathy, especially in those with CLD, if necessary. ¹ Viscoelastic assays, like thromboelastography and ROTEM, can help direct the coagulopathy correction in CLD because they provide a dynamic means of monitoring hemostasis, giving real‐time information on the kinetics of clot formation and growth, as well as the strength and stability of the formed clot. This information allows the provider to quickly and accurately transfuse the appropriate blood product (plasma, platelets, or cryoprecipitate) to correct the underlying coagulopathy all while avoiding overtransfusion. ¹³ As long as the coagulopathy is corrected, the hematoma usually resorbs gradually without sequelae. However, 4 of the previously reported 11 cases of SOH in patients with CLD required an operation for worsening visual acuity with ⁵ , ⁹ or without ⁴ , ⁶ elevated IOP. Modi et al. ⁶ and Saeed et al. ⁹ performed an orbitotomy and hematoma evacuation to release retinal compression and alleviate elevated IOP establishing unrestricted extraocular muscle motility, respectively, with favorable outcomes. Nemiroff et al. ⁵ successfully treated elevated IOP (61 mm Hg) secondary to SOH by performing lateral canthotomy/cantholysis of the left lower eyelid; however, the patient’s eye symptoms never improved, and she eventually succumbed to the complications of decompensated CLD: disseminated intravascular coagulation and acute respiratory distress syndrome. Lastly, Moorthy et al. ⁴ performed an orbitotomy and hematoma evacuation to address compressive optic neuropathy from SOH.

Key Points

• SOH in the setting of CLD is uncommon. This is either secondary to it being a rare occurrence or an underreported occurrence. Nevertheless, it is important to be cognizant of SOH in CLD, especially in those with overt coagulopathy.

• Presenting symptoms of SOH are usually sudden onset and include orbital pain, periorbital edema, and ecchymosis, and many (75%) experience variable visual disturbances.

• Surgical intervention is warranted in patients with SOH with worsening visual acuity and/or increasing IOP; however, most patients require only conservative measures with correction of underlying coagulopathy if necessary, because hematomas usually resorb gradually without sequela.