Diagnostic Dilemma of Painful Red Eye With Severe HeadacheCorrespondence Address :
Dr Sunil Kumar, FRCS, MS, Mohamad Dossary Hospital, Department of Ophthalmology. PO BOX 335, Al Khobar 31952, Saudi Arabia.
Traditionally, the differential diagnosis of non traumatic painful red eye is limited to microbial keratitis, acute congestive glaucoma & acute iritis. However, if painful red eye is associated with headache, then migraine, sinusitis, orbital infection and inflammation are the usual differential diagnoses. Cluster headache as a cause of painful red eye with severe headache may be missed both by internists as well as by ophthalmologists.
I report two cases initially diagnosed, investigated and treated variously as glaucoma, partial third nerve paresis due to diabetes and meningitis. The correct diagnosis became apparent when all investigations were normal and the patient did not respond to treatment appropriate for the initial, presumed diagnoses. These cases emphasize the importance of a high index of suspicion for cluster headache, and the dramatic response to corticosteroid therapy in this usually overlooked malady.
Severe headache, Painful red eye, Ptosis
Case Number 1
A 48-year-old female presented with the complaints of recurrent episodes of severe pain in the left eye for last three days. She reported that the pain started suddenly along left supra-orbital margin, within minutes the pain spread in and around eye and became unbearable. Redness of the eye closely followed the onset of pain. The lid became ptotic but the vision remained unaffected.
Injections of Diclofenac sodium were given to relieve the pain. Her past medical and ophthalmic history was unremarkable. She denied any head injury in the past. Investigations revealed that she was an overt diabetic, although her systemic examination was within normal limits ENT examination and X-ray Para-nasal sinus view excluded the sinusitis. A provisional diagnosis of partial third nerve paresis due to diabetes mellitus was made and ophthalmic consultation was advised. Complete blood count, Erythrocyte sedimentation rate, renal function test were within normal limit. VDRL was non reactive and anti nuclear antibodies were not detected. During pain free period the anterior segment was unremarkable except conjunctival congestion. Her intraocular pressure was 32 and 30 mm of Hg. Well defined pink optic disc with CD ratios of 0.7 and 0.6 in the right and left eye respectively excluded the clinical diagnosis of benign intracranial hypertension. Field changes on automated perimetry were consistent with the diagnosis of glaucoma.(Table/Fig 1). Timolol maleate (0.5%) eye drops, twice a day were prescribed. The intraocular pressure dropped down to 20 mm of Hg but episode of pain recurred and injection Diclofenac was given. Patient was re-evaluated again. Her vision was 6/6 in each eye. There was no proptosis. Although lid edema and mild ptosis was apparent but ocular movements were within normal limit. Conjunctiva was congested and chemosed(Table/Fig 2). Possibility of orbital inflammatory disease or orbital myositis was omitted due to normal ocular movements and absence of proptosis. There was no clinical evidence of scleritis or iritis. Pupil were reactive without an afferent pupillary defect. Gonioscopy revealed grade three open angle in all quadrants. Absence of blood in schlemmâ€™s canal excluded the possibility of low-flow carotid-cavernous fistula. Contrast enhanced computerized tomographic (CT) scan of the brain and orbit excluded any structural or space occupying lesion and orbital inflammatory diseases.
Considering the episodic nature of severe periocular pain, conjunctival congestion with mild ptosis, and the normal CT scan findings of the orbit and brain, a presumptive diagnosis of cluster headache was made and treatment was commenced with tablet prednisolone 60 mg per day for three days, and tapered by 10 mg every third day (Campbell protocol). After initiation of this treatment, there was no recurrence of the episodes of pain.
The ptosis as well as conjunctival congestion resolved completely.
Case Number 2:
A 35-year old male patient was brought to the triage with severe headache and pain in the right eye, associated with vomiting, of 30 minutes duration. Although he reported having suffered episodes of headache in the past, but the nature of the current headache was entirely different. Injection Diclofenac sodium was given for a presumed diagnosis of migrainous attack and he was advised to consult the internist in the out patient department. The patient returned in the morning with severe headache, vomiting, painful red right eye and watery rhinorrhea. He was admitted for further evaluation. Systemic examination did not reveal abnormal findings. Results of the complete blood count, erythrocyte sedimentation rate, renal and liver function tests and serum electrolyte levels were found to be within normal limits. The ENT examination was
Cluster headache, an uncommon, most painful primary headache, heralds episodes of very severe unilateral orbital, supra-orbital and/or temporal pain, which may extend to the face, neck, ear or hemicranium, with signs and symptoms of autonomic dysfunction. The pain starts without any warning, intensifies rapidly and becomes agonizing in a short time. The designation came from the cluster pattern of attacks, both in terms of the number of episodes per day and the number of days in a bout (cluster pattern)(1). The frequency of attack may vary from six per 24 hour period to once in a week. If the patient experiences more than three attacks within 24 hours, it may seem that the pain recurs when the effects of analgesics are wearing off. This gives a false impression of continuous pain, and masks the episodic nature of the illness.
Misdiagnosis of cluster headache is common in clinical practice and can lead to significant morbidity.(2) An interesting non-clinic, population based, internet survey in US observed an average delay of 6.6 years in the diagnosis of cluster headache. The average number of incorrect diagnoses was 3.9 and an average 4.3 physicians were consulted before the correct diagnosis was made, 4% of patients had unnecessary sinus or deviated septum surgery. Many inappropriate medications such as propanolol, amitriptyline, and antibiotics were commonly prescribed while sumatriptan and oxygen were often denied due to a failure to understand the nature of this disorder.(3)
International Headache Society has laid down the criteria for diagnosis of cluster headache.((Table/Fig 3): Criteria of international headache society for cluster headache). At times the clinical boundaries between migraine and cluster headache are ill defined, none meet the strict international society criteria and patient may present with headache sharing the characteristic of both headache type. These occasions are diagnostic challenge.
Change in the pattern or intensity of headache in a patient with history of cluster headache needs neuro-imaging to rule out a structural lesion masquerading as cluster headache. Granulomatous pituitary lesion and intracranial aneurysm may masquerade as cluster headache(4),(5),(6). Overlap in symptoms between recurrent orbital myositis and cluster headache may delay the diagnosis of orbital myositis. Orbital myositis should be considered in patients with atypical cluster headache characterized by proptosis, painful eye movements, and pain that does not completely resolve within 3 hours.(7) Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT syndrome), a newly described headache, characterized by short lasting, unilateral attacks of a neuralgiform pain, accompanied by conjunctival injection and tearing on the symptomatic side, should be considered in the differential diagnosis of cluster headache.
The exact etio-pathophysiological mechanism of cluster headache remains to be elucidated. Recent studies have demonstrated the important role of the ipsilateral ventral hypothalamus in the pathogenesis of cluster headache(8),(9). The diagnosis of cluster headache is clinical, based on a history of recurrent episodes, the description of the pain, the temporal profile and the accompanying autonomic manifestations. Oxygen inhalation is one of the most effective treatments for cluster headache.(10),(11) Subcutaneous administration of 6 mg of Sumatriptan has been shown to abort the attack of headache in 75 percent of patients(12) but it is contraindicated in patients with hypertens
To conclude, in a patient presenting with excruciating headache of sudden onset, associated with painful red eye and other autonomic dysfunctions, consider the diagnosis of cluster headache. Dramatic relief of the symptom complex by administration of 60 mg of tablet prednisone can be used as a therapeutic test.
I wish to acknowledge the precious suggestions and comments of Dr. Philip Thomas in preparation of this manuscript
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