Hemolacria

Etiology

The source of blood in tears may be:

• Bleeding from the conjunctiva - Conjunctiva is a vascular tissue with limbal, bulbar, forniceal, and palpebral parts. The conjunctival vessels lie at the ocular surface and may bleed spontaneously or after eye-rubbing, usually causing subconjunctival hemorrhage. Subconjunctival hemorrhage without a breach in the conjunctiva is often not associated with frank blood in tears, though red blood corpuscles may be seen on microscopic examination of tears.[1] Causes of haemolacria due to the involvement of the conjunctiva  include
  • Trauma - Conjunctival laceration or rupture of conjunctival vessels may cause hemolacria. Surgery involving incision or excision of the conjunctiva may also cause hemolacria. The surgeries include small incision cataract surgery, pterygium surgery, squint surgery, scleral buckling, trabeculectomy, pars plana vitrectomy, and other ocular surgeries. After manipulation of the conjunctival follicle in trachoma, there may be haemolacria.
  • Inflammation - Severe conjunctivitis including hemorrhagic conjunctivitis, membranous or pseudomembranous conjunctivitis, follicular conjunctivitis with congested semilunar fold and caruncle, severe viral or bacterial conjunctivitis cause blood-stained tears. Conjunctival ulcers or granulation due to various reasons may cause haemolacria. Postoperative sclera buckle infection can also cause bloody tears.
  • Vascular lesions - Hemangioma, lymphangioma, inflammatory papilloma of the conjunctival sac, telangiectasia of conjunctival vessels, pyogenic granuloma,[2] conjunctival varies, pathological vasodilation of the conjunctival vessels, severely vascular papillae, giant papillary conjunctivitis may bleed leading to bloody tears. Malignant melanoma of the conjunctiva may be associated with haemolacria.
  • Vicarious menstruation - The conjunctiva may periodically/cyclically bleed (vicarious menstruation) during menstruation or hormonal disturbances. This phenomenon is usually seen around menarche or rarely around the menopause. Sir Duke-Elder proposed that 'instability of the nervous system' and not hormonal disturbance is the cause of vicarious menstruation from the conjunctiva.[3] The source of blood in hemolacria in vicarious menstruation has been thought to be the lacrimal gland, lower forniceal conjunctiva, episclera, dilation of forniceal, and bulbar conjunctival vessels, and hemorrhages in upper tarsal conjunctiva. The ophthalmic examination may be normal with normal-appearing conjunctiva in some cases. Vicarious menstruation may cause a subconjunctival hemorrhage. The vicarious menstruation is usually painless, may last for seconds to minutes, and may involve one or both eyes. Islands of ectopic endometrial tissue may be present outside the uterus. This is called endometriosis, and such aberrant endometrial tissue in conjunctiva may cause vicarious menstruation. Primary localized conjunctival amyloidosis may cause recurrent subconjunctival bleed due to vicarious menstruation.[4] Other possible explanations of vicarious menstruation include[1]:
    • Normal conjunctiva stimulated by hormonal or other factors.
    • Estrogenic premenstrual light blood hypertension.
  • Foreign body - Foreign body at the upper fornix can cause chronic irritation, erosion of the conjunctiva, and hemolacria. Subconjunctival metallic splinter after trauma may cause bleeding into the tear.
  • Chemical injury - Application of silver nitrate over the conjunctiva is another cause of bloody tears.
• Bleeding from the lid margin - Inflammed lid margin due to blepharitis may cause erosion of the surface of the eyelid and lead to bleeding. Trauma causing laceration of the lid margin may lead to haemolacria. An infestation of the lid with Phthirus pubis may also cause mild staining of blood in the tear.[5]
• Bleeding from the lacrimal puncta - The source of the bleeding in tears may be the puncta. There is one lacrimal punctum, each at the medial side of the upper and lower lid, respectively. These are situated at the inner margin of the lid. Punctum connects medially to a lacrimal canaliculus on both the upper and lower lid. Both of these canaliculi may meet to form a common canaliculus and then connect to the lacrimal sac, or they may open into the lacrimal sac separately. The lacrimal sac connects to the lower nasal cavity below the inferior nasal concha via the nasolacrimal duct. Thus, the lacrimal puncta drain tear to the nasal cavity normally. If the nasal cavity fills with any fluid under pressure, that fluid may get released through the puncta; this happens when profuse epistaxis is managed with nasal packing. Sometimes, bleeding through the puncta may occur in epistaxis without nasal packing also. When the pressure within the nasal cavity increases, the blood can pass retrogradely through the nasolacrimal duct, lacrimal sac, and canaliculi to the puncta. Rarely, the blood may come to the puncta from connected body cavities, including the upper digestive tract or trachea.[1] The bleeding from the lacrimal puncta may be evident on pinching or blowing the nose in case of epistaxis.[6] Fracture of the nasal bone or walls of the sinuses, and Le Fort fracture type I can cause epistaxis, which may retrogradely flow to the puncta. Haemolacria has been reported after orbital floor fracture due to displaced implant eroding the nasolacrimal drainage system in a patient on warfarin and aspirin.[7] The bleeding through the lacrimal puncta due to epistaxis or a local pathology of the lacrimal sac or nasolacrimal duct usually occurs through the lower punctum, sometimes from both puncta, and rarely through the upper punctum alone.[1]  Other causes of bleeding from the lacrimal punctum include: 
  • Trauma
  • Infection
  • Tumor- angioma, meningioma of the lacrimal sac
  • Vascular lesions including varices[8] and dacryolith, rupture of dilated/distended vessels within the lacrimal sac
• Tear glands - In some rare cases, the source of bleeding remains undetermined. In such cases, some authors have hypothesized that the hemorrhage may come from the lacrimal gland or accessory lacrimal gland. Other hypothesized origins of hemolacria include meibomian glands or conjunctival goblet cells.[1] Dacryoadenitis or inflammation of the lacrimal gland may cause bloody tears.
• Orbit - orbital varix at the junction of the angular vein and superior ophthalmic has been reported to be associated with bloody tears due to bleeding from the medial conjunctiva and overlying skin after jugular compression.[9] Rarely, fracture of the orbital roof, orbital hemangioma, and epibulbar dermoid may cause haemolacria if the conjunctiva is open.
• Hematologic diseases that cause increased bleeding diathesis may lead to hemorrhages at multiple organs, including haemolacria. The disorders include hemophilia, thrombocytopenic purpura, deficiency of clotting factors, including factor VII deficiency. 
• Other systemic diseases that have correlations with bloody tears include anemia, jaundice, exanthematous fever (may also cause epistaxis, and bleeding through the mouth), and severe disturbances of the autonomic nervous system.
• Vascular disorders may play an important role in the pathogenesis of hemolacria. Hypertension is an important factor that has been reported to cause epistaxis and retrograde haemolacria through the leak of hemorrhage via the lacrimal puncta. Causes of haemolacria include Hereditary hemorrhagic telangiectasia or Osler Weber Rendu disease, Henoch-Schönlein purpura, Gardner Diamond syndrome (autoerythrocyte sensitization or psychogenic purpura, or painful bruising syndrome), and conjunctival varicose vessels. Osler Weber Rendu disease or hereditary hemorrhagic telangiectasia is an autosomal dominant disease characterized by mucocutaneous telangiectasia and visceral involvement leading to hemorrhage, which may be life-threatening.[10] A patient with occlusion of the posterior inferior cerebellar artery causing unilateral Horner's syndrome and vasodilation has been reported as well. [Ziegler LH. Bloody tears. JAMA 1938;110 (17):1387]. 
• Medications:
  • The mecholyl test uses subcutaneous methacholine to evaluate the autonomic nervous system in psychiatric patients. This test has also been used for the diagnosis of achalasia cardia. Mecholyl tests may cause haemolacria.[11] 
  • Systemic drugs like aspirin, clopidogrel, heparin, and warfarin can rarely cause haemolacria.
  • Acetylcholine may cause bloody tears.
• Psychiatric disorders - Attention seeking behavior or other psychiatric disorders may lead to bloody tears. A case report of an 11-year-old girl with recurrent hemolacria has been reported. Her ocular exam was otherwise normal.[12] On further evaluation, she was noted to have multiple needle pricks over the fingertips, which could explain her symptoms. She was diagnosed with Munchausen syndrome (factitious disorder imposed on self). In this syndrome, the affected person repeatedly feign disease/trauma/illness to draw attention, sympathy, or to fulfill other psychological needs. Other psychiatric risk factors for haemolacria include stress, emotion, anger, fright, anxiety, and hysteria. Some patients may malinger as haemolacria by putting red colored liquid over the conjunctival sac.
• Bloody sweats or hemathydrosis - A prepubertal 9-year-old female has been reported to have bloody sweats at the skin between the lacrimal sac and medial canthus, and haemolacria. The symptoms resolved eight days after the application of iron perchloride over the affected skin {Sous G. [Observation of bloody sweat]. Crón Oftalmol Cádiz 1871;1:86-9. Cited by Murube}.[1]
• Other causes of haemolacria include[3][13][14]
  • Cranial trauma
  • Post-traumatic epilepsy
  • In otherwise normal individuals after stooping, or muscular effort
  • In children after 'copious weeping.'
  • Coughing
  • Hypertensive crisis
  • Acute hemorrhagic edema of infancy
• Unknown cause/idiopathic - In some cases, despite a thorough search for ocular, systemic, or psychiatric causes, no obvious etiology or source is found.

Epidemiology

Haemolacria is an exceedingly rare condition. Due to low incidence and limited literature, the prevalence and predilection of haemolacria for a specific gender, race, or age is not known.

Pathophysiology

The pathophysiology of haemolacria depends on the case-specific cause of this condition, as explained above in the etiology section.

History and Physical

Hemolacria is typically a benign and self-limited process. However due to associations with severe systemic and ocular diseases a thorough evaluation is warranted for all patients presenting with hemolacria.

All patients presenting with haemolacria should undergo a comprehensive ocular examination as well as a systemic examination to look for organic causes and sources of the hemorrhage. Specifically, the history of systemic diseases (hematological disorders, coagulopathies, hypertension) and medications like warfarin, aspirin or clopidogrel is necessary.

When no ocular or systemic causes are present, psychiatric diseases and vicarious menstruation must be ruled out before labeling the etiology as 'unknown' or 'idiopathic.'

Evaluation

Thorough ocular and physical examinations are warranted on presentation. In patients with suspected ocular causes, referral to an ophthalmologist is recommended for a thorough evaluation. Radiographic imaging, usually with computed tomography of the head and orbits, is also recommended.

A unique approach involving the placement of punctal plugs has been described in the literature as well. This involves the placement of punctal plugs in the superior and inferior puncta. This helps identify retrograde flow as the cause of hemolacria. If hemolacria resolves with the placement of punctal plugs with continued or initiation of epistaxis, the anatomical source can be identified. 

Dacryoendoscopy promises to be an important tool to rule out bleeding lesions within the tear drainage system, which may be undetected without this modality.[8]

Treatment / Management

The treatment of haemolacria depends on the cause. Close observation is needed when no apparent cause of haemolacria is detected to rule out malingering.

Differential Diagnosis

Differential diagnosis of haemolacria includes:

• Red-colored tears may be noted in patients on therapy with rifampicin.
• Occult haemolacria is the term used for apparently clear tears if microscopic or chemical evidence of red blood corpuscles is present in tears. Microscopy showed erythrocytes in the tear of 10% of the normal population, and chemically blood was detected by the chemical stix method in 3% of normal persons.[15] Occult blood was found commonly in acute infectious conjunctivitis and was also noted in chronic and subacute infectious conjunctivitis though it was less frequent.[15]
• False bloody tears - Malingerers may feign red-colored liquids and may claim that tear is bloodstained. Animal studies have shown that rats treated with muscarinic agents may secrete reddish-brown tears (chromodacryorrhea) due to protoporphyrin and coproporphyrin secreted from Harderian glands. Melanodacryorrhea or black tears may be a feature in conjunctival argyrosis or necrotic uveal melanoma.

Prognosis

Severe bleeding through tears may even be fatal in some cases especially in a patient with coagulopathy. Identifying hemolacria from systemic causes is of utmost importance. 

Complications

Haemolacria as such will not lead to complications. But it can be a complication of a multitude of conditions, as explained in the etiology section. Identifying the cause and targetting the treatment towards it, is the most important step in the management of hemolacria.