Hemolacria due to possible idiopathic intracranial hypertension: A case report

Abstract

Background

Hemolacria, the excretion of blood-tinged tears, merges biological oddity with mysticism. This report discusses a unique case of hemolacria in a patient with possible idiopathic intracranial hypertension without papilledema (IIHWOP), resolving after acetazolamide treatment, exploring a potential pathogenic link.

Case Presentation

A 50-year-old male experienced daily hemolacria for three months along with occasional epistaxis and otorrhagia. He also suffered from chronic migraine, often accompanied with blurred vision and vertigo, and primary stabbing headache. Systemic and neurological assessments, along with computed tomography (CT) brain scans, ophthalmological and hematological tests revealed no underlying diseases. Brain magnetic resonance imaging (MRI) along with magnetic resonance venography, indicated left transverse sinus aplasia, bilateral ocular bulb flattening and enlargement of optic nerve sheaths, suggesting increased intracranial pressure. The patient began acetazolamide treatment. In the next three months, he experienced only four bleeding episodes and a dramatic reduction of monthly migraine days.

Discussion

Our report first associates hemolacria with possible IIHWOP, proposing a new pathogenic pathway. A derangement in the glymphatic system, implicated in idiopathic intracranial hypertension pathophysiology, could explain its ocular symptoms, including hemolacria. Our findings may contribute to understanding IIHWOP and the glympathic system's role in ocular physiology.

Introduction

Hemolacria, the excretion of blood or blood-tinged tears ¹ is a medical anomaly that intertwines the threads of biological rarity with the narratives of mysticism and mythology. Historically, accounts of weeping blood have been recorded in various cultural and religious texts, imbuing the condition with a mystical aura. The 6th-century compendiums of Aëtius of Amida are among the earliest to describe such phenomena scientifically, and tales from the Middle Ages chronicle the life of a nun who, instead of experiencing the normal process of menstruation, exhibited tears mixed with blood. ²

Stripping away the veil of folklore, the modern understanding of hemolacria remains limited due to the rarity of its occurrence. The true epidemiology of this condition is obfuscated by the scarcity of comprehensive data and the heterogeneity of reported cases. Most case reports situated within the medical literature treat hemolacria as a secondary symptom, a manifestation of a range of underlying ophthalmic or hematological disorders. Conditions such as conjunctival infections, vascular diseases and systemic disorders have all been noted as potential contributors to this enigmatic presentation. ²

Nonetheless, despite the insights garnered from these associations, a significant portion of hemolacria cases are categorized as idiopathic where no discernible cause is ascertained despite exhaustive investigative efforts.

In the shadow of such intriguing instances, we present a peculiar case of a patient with possible idiopathic intracranial hypertension without papilledema (IIHWOP), whose recurrent hemolacria episodes resolved after acetazolamide treatment. Hence, a pathogenic linking mechanism between the two conditions is discussed.

Case presentation

A 50-year-old male presented with a 3-month history of daily episodes of hemolacria (Figure 1). Each was self-limiting, usually lasting a few minutes. Bleeding was bilateral or more rarely unilateral, accompanied by tinnitus and dizziness in almost half of the episodes. Hemolacria, when unilateral, often presented in conjunction with ipsilateral otorrhagia or epistaxis, although these phenomena could also manifest independently from hemolacria. The patient also suffered from migraines, which began episodically when he was 22 years old and gradually worsened into chronicity with approximately 20 days of migraine-like headaches per month over the last year. Notably, during migraine attacks, he also reported brief but frequent episodes of blurred vision, diplopia, and vertigo. The patient also suffered from primary stabbing headache, with daily stabs almost invariably preceding within a few minutes the onset of migraine attacks and, sometimes, of hemolacria episodes. Because of hemolacria, the patient was admitted to the emergency room multiple times over the last months. Each time he was tested extensively for ophthalmological or hematological disorders and each time he tested negative. Additionally, no abrasions of external acoustic meatus could ever be found, repeatedly supporting the hypothesis of a psychiatric condition. He underwent 5 computed tomography (CT) brain scans over the last three months, with no evidence of any underlying disease.

Figure 1.

Hemolacria and otorrhagia.

When the patient came to our attention a complete systemic and neurological examination was performed, entirely normal. A detailed ophthalmologic evaluation, including slit-lamp examination and fundoscopy, performed by an expert ophthalmologist, was unremarkable. Similarly, a complete visit with an otolaryngologist revealed no underlying diseases. Complete blood count, coagulation profile, and blood pressure measurements were within normal limits, with no evidence of hematological abnormalities or systemic hypertension.

Magnetic resonance venography (MRV) was performed, showing left transverse sinus aplasia. Brain magnetic resonance imaging (MRI) showed enlargement and tortuosity of optic nerves and posterior flattening of the globe (Figure 2(a)–(c)). Based on neuroradiological and clinical features a possible IIHWP diagnosis was suggested. ³

Figure 2.

Mr Venography shows aplasia of the left transverse sinus (a). Brain MRI shows bilateral optic nerve tortuosity with associated ocular bulb posterior flattening (b) and distension of optic nerve sheats (c).

The patient refused to undergo lumbar puncture. He was therefore commenced on acetazolamide 250 mg three times per day. After one month of treatment, the patient reported only one bleeding episode, and he suffered from just 5 migraine days. Notably, he also only reported two “stabs” over the month. In the next three months, the patient reported only two episodes of hemolacria and one episode of otorrhagia. Migraine days stabilized around 4–5 per month and, remarkably, primary stabbing headache and tinnitus remitted completely.

Discussion

In this report, we delineate an unprecedented case of hemolacria which resolved following acetazolamide treatment.

The pathophysiology underlying idiopathic intracranial hypertension remains a matter of debate. However, there is accumulating evidence that dural sinus stenosis, as observed in our patient, serves as a salient and common underlying mechanism. ⁴ In particular, proposed as central to idiopathic intracranial hypertension pathogenesis is the excessive collapsibility of dural sinuses, often exacerbated by anatomical anomalies like sinus hypoplasia or giant granulations. ⁵ This condition triggers a self-limiting venous collapse feedback loop where elevated cerebrospinal fluid (CSF) pressure constricts the sinuses, which in turn hampers CSF drainage, increasing its volume and pressure further and perpetuating sinus compression. This cycle stabilizes only when sinus compression peaks, resulting in a new, elevated equilibrium of the coupled CSF and sinus pressures.

Attention has recently been directed toward the glymphatic system, a CSF clearance pathway that facilitates the drainage of interstitial fluid and solutes from the central nervous system. Dysfunction within this system has been extensively implicated in the pathophysiology of idiopathic intracranial hypertension (IIH) ⁶ and, in clinical practice, the glymphatic pathway likely accounts for symptoms such as rhinorrhea and otorrhea in patients with altered intracranial pressure. ⁷

The existence of an ocular glymphatic system ⁸ introduces a potential explanation for the ocular manifestations of IIH, which extend beyond papilledema to include subretinal fluid accumulation, central retinal vein occlusion, or retinal hemorrhages. ⁹ It has been proposed that a glymphatic system overflow may occur in conditions of elevated intracranial pressure. ¹⁰ The hypothesis is that CSF may transude through the eye via the ocular glymphatic system in conditions of elevated intracranial pressure, akin to the noted phenomena in the nasal and ear cavities.

The selective presentation of hemolacria in our patient may point to the presence of an underlying unknown and rare predisposing factor possibly involving connective tissue or vascular wall disorders, which could promote the addition of some blood to the CSF/ISF leakage in conditions of oculo-glymphatic system overflow. Notably, this would align with similar observations of epistaxis resolution following acetazolamide treatment in IIH. ¹¹

Lastly, although somewhat obfuscated by the striking visual impact of hemolacria, it is worth noting that following acetazolamide treatment we observed a complete remission of primary stabbing headache in our patient. The correlation between these two conditions has been recently proposed ¹² and in our view stabs could be reflective of peaks in dural venous pressure, leading to abrupt opening of anastomosis, possibly with diploic veins, contributing to cranial venous outflow. Further research could determine the actual prevalence of primary stabbing headache in IIH patients to investigate a definitive link.

Although the MRI showed signs of altered intracranial pressure, consistent with the patient's symptoms characterized by chronic headaches, diplopia, and blurred vision, all of them almost completely regressed following treatment with acetazolamide, it is important to recognize as a limitation of this study the absence of an opening pressure value demonstrated by a lumbar puncture, due to the patient's refusal to undergo it. Future research is therefore necessary to more clearly demonstrate the causality between hemolacria and altered intracranial pressure.

Conclusions

Our report is the first to link hemolacria with possible IIHWOP, suggesting a novel pathogenic pathway. Despite its plausibility, this pathogenic model raises unanswered questions, needing further validation. We aim with this case to contribute to the broader spectrum of IIH/IIHWOP presentations and to the understanding into the complex interplay between the glymphatic system and ocular physiology.

Clinical implications

• Hemolacria can be a rare manifestation of increased intracranial pressure.

• Acetazolamide is effective in treating hemolacria due to increased intracranial pressure.

• Primary stabbing headache may present in the context of increased intracranial pressure, and responds well to acetazolamide.