Blind Painful Eye
Caring for patients with a blind, painful eye presents unique challenges, as it is often the final stage of a variety of ocular disease processes. Patients may have exhausted vision-restoring treatment and now require counseling regarding goals of care. The blind, painful eye is defined as a terminal condition where vision is unsalvageable and there is poorly responsive ocular discomfort . The focus of any management plan is to address the debilitating effects on quality of life. Clinicians must initially broach the difficult conversation with compassion and seek to understand the patient’s values regarding medical versus surgical treatment.
Etiology and Workup
Patients may present with blind, painful eye due to a wide spectrum of etiologies . Common causes include end-stage glaucoma, bullous keratopathy, uveitis, chronic retinal detachment, penetrating trauma, chronic hypotony, and post-operative complications such as endophthalmitis. Unlike other causes of severe vision loss, such as acute angle closure glaucoma, blind painful eye is characterized by its chronic, gradual progression of vision loss and ocular pain. In a study conducted by Shah-Desai et al., patients with blind painful eye ranged in duration of ocular pain from 1-210 months, with an average duration spanning 59 months (4.91 years) .
The source of pain may be multifactorial itself – corneal bullae, chronic hypotony, chronic elevated intraocular pressure, and neuropathic  . The patient’s description of their pain should be noted. Patients with blind painful eye may characterize their pain as excruciating, aching, occurring with blink, or photophobic. A detailed history and physical exam are usually able to elucidate the root cause. Typically, the etiology is readily available. If adequate view of the posterior segment is not available then posterior segment malignancy must be ruled out with B-scan ultrasonography.  Computed tomography scan or magnetic resonance imaging are rarely indicated.
Primary goal of management aims to reduce pain and make the patient physically and emotionally comfortable. Medical therapy is typically the first-line treatment for patients with a blind painful eye, especially if the patient is not concerned with cosmesis and/or psychologically ready to discuss surgical removal of the eye. However, failure to minimize pain with medical therapy should warrant the discussion for minimally invasive therapy.
In a retrospective review of 99 blind painful eyes, 39% of eyes were pain-controlled with topical therapy alone (cycloplegics, steroids, antihypertensives), 75% of eyes were pain-controlled with “minimally invasive interventions” (laser cyclophotocoagulation, retrobulbar injection, or corneal electrocautery), and 100% of eyes were pain-controlled with enucleation or evisceration . The authors found that patients with higher initial pain scores were at risk for non-surgical therapy failure and may require an earlier discussion of surgical intervention. Recently, calcitonin gene-related peptide (CGRP) has been found to be overexpressed in the choroid of enucleated blind, painful eyes . Modulation of this factor in otherwise treatment-resistant patients may be explored as a potential therapeutic target.
Medical therapy with intraocular pressure-lowering medications
In patients with blind, painful eye secondary to chronically increased intraocular pressure, maximum glaucoma medication provides an excellent starting point. The pain can generally be controlled for years on this regimen, although it does put patients at increased risk for developing ocular surface breakdown. Agents are typically administered at standard frequency, and include:
- prostaglandin analogues
- carbonic anhydrase inhibitors
In patients who are resistant to maximum glaucoma medication, cyclophotocoagulation (CPC) should be explored. CPC uses the principle of cyclodestruction, which has been used for refractory glaucoma since the 1930s . modern CPC treatment involves a diode laser (810 nm wavelength) that is either transslceral or with an endoscopic probe. The diode laser is preferred over other wavelengths since the melanin in the ciliary epithelium better absorbs this wavelength than others and therefore causes more targeted destruction with less inflammation. CPC is advantageous because it avoids incisional surgery and can be performed in the office setting with local anesthesia. However, in eyes with no light perception, CPC may confer risk of sympathetic ophthalmia in the fellow eye.
EDTA for Band Keratopathy
Patients with a blind painful eye due to band keratopathy (BK) may benefit from EDTA-assisted removal. Chelation of calcium deposits with topical EDTA is a safe and effective treatment for BK . EDTA use has been shown to minimize damage to Bowman’s layer and reduce the risk of an irregular refractive surface. However, despite the benefits, EDTA only addresses the calcium precipitated on the cornea, and not the underlying pathology. Therefore, recurrence of calcium is a common problem. Furthermore, EDTA is toxic to the ocular surface, and inadequate removal during or after the procedure will cause a chemical burn.
Retrobulbar Injection (ethanol/chlorpromazine)
Retrobulbar alcohol has long been used to relieve ocular pain, but the popularity of this therapy has declined among ophthalmologists in recent times. Recent case reports highlight its continued effectiveness at long term ocular pain management [11, 12]. Retrobulbar alcohol works via the analgesic destruction of nerve cells. This procedure may be preferred in patients with cosmetically acceptable blind painful eye, or in patients where globe preservation is a secondary consideration. The effects of retrobulbar alcohol injections typically last for 3 to 6 months [13-15] As an alternative to alcohol, retrobulbar injection with chlorpromazine has been reported to be very successful at achieving pain management for a year or more time . A single retrobulbar injection of chlorpromazine provided 80% of blind eyes pain relief for more than one year in duration. However, the treatment is associated with swelling of the eyelid, restrictive strabismus, and postoperative ptosis, and is generally recommended only in rare occasions.
Atropine and topical steroids
For patients with ocular pain due to inflammation, uveitis, chronically elevated intraocular pressure, or ischemia, topical anti-inflammatory drugs provide viable adjunct therapy. Patients may be started on prednisolone acetate 1% QID and Atropine 1% BID .
Other notable treatments
The hypothesis that blind, glaucomatous, eye pain might be explained by structural damage responsible for neuropathic pain has led to the exploration of various therapeutic strategies. One strategy involves antiepileptics as an adjuvant to standard IOP-lowering medication. On case report published a trial of gabapentin, where the dose was gradually increased to reach a total daily dose of of 2,400 mg. There was successful decrease in patient-reported pain scores at 6 months .
Another approach employs a stellate ganglion nerve block, which has been successful in one case report of six patients with blind, painful eye . This type of block has historical significance in treating glaucoma , however its role in treating blind, painful eye is new and evolving.
A last resort for patients with a blind painful eye involves incisional surgery. The two modern procedures are evisceration and enucleation, and the choice of surgical procedure is dependent on ocular disease and patient and surgeon preference. During an evisceration, only the cornea and the contents of the eye are removed. The scleral shell and the eye muscles are left intact, and the implant is then placed into the scleral shell to which the muscles are still attached. In contrast, enucleation involves removal of the entire globe with resewing of the extraocular muscles to the spherical implant.
Evisceration with prosthesis can result in excellent cosmetic outcomes and is favored by many oculoplastic specialists. However, enucleation is recommended when an intraocular tumor is suspected, and a definitive histopathologic diagnosis is required .
Blind painful eye was the most common indication for surgical eye removal in one multi-center study, representing ~37% of enucleations . While enucleation is generally effective in resolving pain, persistent postsurgical pain (PPSP) is well documented and was present in 47% of patients. This can be further characterized by phantom limb pain, which was found in approximately 23% of all eye amputation patients. Visual hallucinations following eye removal surgery were also a common ophthalmic complaint. Ophthalmologists should inform their patients about these phenomena, as they do not indicate a mental disorder or feigning of illness. Aside from pain, enucleation also influences patient self-perception and their overall quality of life. Questionnaire-based studies suggest that half of surgery-treated patients are concerned about their facial appearance following enucleation, causing them distress or diminished self-concept [22, 23]. Similarly, other studies report that eye-amputated patients have poorer health-related quality of life, poorer self-rated health, and more perceived stress than the general population . Notably, the largest differences in quality of life between these patients and the general population were related to role limitations. About 25% of patients retired or changed to part-time jobs due to eye disease and 39.5% stopped participating in leisure activities. 41% of patients stated that their eye-removal surgery changed their life significantly. Anxiety and depression were present in nearly one-third of this cohort.
Blindness involves a great emotional burden for the patient and ophthalmologist. It is important to address the patient’s feelings towards their diagnosis and refer them to the appropriate nonmedical resources when unmet needs are identified. Clinicians should first seek to provide emotional support as the patient comes to terms with their diagnosis. Though the visual loss associated with the blind painful eye is gradual, patients may experience a sudden shock upon realizing there is little hope for recovery. It is not uncommon for patients to deny their diagnosis, question their prognosis with available online information, or seek another opinion. Patient concerns and doubts should be addressed with the most up-to-date medical information so that the patient can make an educated decision for course of treatment. The physician may broach the topic of evisceration or enucleation after the patient has adequately adjusted to the diagnosis. Apart from medical and pain management considerations, ophthalmologists must consider the whole patient in treatment. Sight is crucial for activities of daily living, and therefore it should be ensured that the patient has adequate support in these areas, through a family member or hospice care. Furthermore, if in-office medical therapy is indicated, patient transportation should be coordinated with a social worker. Peer-support groups offer another way of making life easier for these patients and provide the opportunity for the patient to share their experiences with others who are able to sympathize.
The blind painful eye present an opportunity to practice holistic care and redirect goals of treatment towards pain alleviation. Physicians must practice excellent communication skills to best understand the patient’s values with medical management. When surgery is indicated, the ophthalmologist must be adept in broaching the topic of evisceration/enucleation as the patient may be psychologically hesitant. Lastly, clinicians must coordinate care with the interdisciplinary medical team to address unmet needs in patient’s life that come with life-altering vision loss.
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