Factitious Keratoconjunctivitis

Key Concepts

Factitious keratoconjunctivitis is an uncommon and difficult diagnosis to make. It can often be missed if not included in the differential diagnosis of any case in which the trauma sustained arises under suspicious or poorly explained circumstances.
Injury from factitious keratoconjunctivitis should be distinguished from other similar, though distinct, entities of self-induced injuries such as malingering, topical anesthetic abuse, and other forms of self-induced ocular trauma. Psychopathology is a common associated factor in factitious disease.
Factitious disease most commonly presents in patients in the second or third decade of life and more often among medical or allied health professionals.
Factitious ocular disease is nearly always found in the inferior or nasal quadrants—rarely in the superior quadrant.
Topical anesthetic abuse represents one of the most common and destructive forms of self-induced ocular injury.
Successful treatment of these self-inflicted injuries largely rests on identification and recognition of the underlying etiology. Making the diagnosis of this uncommon disease is of the utmost importance.

Introduction

The eye is a sounding board for the travails of the whole persona (Trevor-Roper). As Trevor-Roper suggests, when the psyche is troubled, it can express itself in many difficult to understand ways such as self-induced injury. This is often expressed as self-induced eye injury or factitious keratoconjunctivitis. The medical literature is replete with examples of self-induced eye injuries. This may range from simple mucous fishing syndrome to incomprehensible self-enucleation. The motivations behind these injuries are as varied and fascinating as the types of injuries themselves. Voutilainen and Tuppurainen felt that self-injuring patients were expressing a strong cry for help but at the same time were incapable of describing what kind of help they needed. Factitious behavior may be difficult to comprehend at times, but it must be exposed. Failure to do so not only prevents proper intervention but may also result in enormous unnecessary costs to the medical system. While factitious disorders are the focus of this chapter, other types of self-induced injury will also be discussed.

Factitious Disorders Defined

The term “factitious,” when used correctly, describes only disorders where symptoms or physical findings are intentionally produced by the patient in order to assume the sick role. Unfortunately, outside the psychiatric literature, the term factitious is used more broadly to describe any self-induced injury. Factitious disorders must be distinguished from malingering and somatoform disorders. While malingering also results in intentionally produced symptoms or physical findings, in contrast to factitious disorders, there is always evidence of external incentive. Malingering can be adaptive in certain situations (e.g., with prisoners of war) and does not necessarily imply psychopathology. Somatoform disorders include hysteria, conversion disorder, and hypochondriasis. These disorders also produce physical or mental findings that are not fully explained; however, in contrast to factitious disorders and malingering, these symptoms are not voluntarily produced and are not under conscious control. Somatoform disorders rarely, if ever, result in ocular disease and will not be discussed further.

Factitious disorders, by definition, imply psychopathology. The patient possesses a pathologic need to assume the sick role and be involved in the medical system. This drive is so strong that all sorts of self-induced injury, in any degree, may result. Achieving the sick role is the sole motivation for the behavior. External incentives, such as financial gain or avoiding responsibility, are characteristically absent. Patients repeatedly victimize themselves, often with disastrous results, while masking the true nature of their illness. The true source of the pathology is often overlooked for several reasons. First, the patients often emphatically deny any trauma—self-induced or accidental. Second, the motivation for the self-destructive behavior is deeply seated in the patient’s psyche and is difficult for the physician to understand. Third, the physician is often reluctant to consider the possibility of self-induced disease because of an inherent desire to trust the patient’s history as factual. Manifestations of factitious disorders are wide and varied. Some examples in the literature are recurrent skin ulceration by autoinjection of bacteria, subcutaneous emphysema, simulated herpes zoster, hematuria, feigned sickle cell crisis, iron deficiency anemia, ^, epilepsy, acquired immunodeficiency syndrome, hypercalcemia, cancer, fatal asthma, fatal water intoxication, fatal hypoglycemia, ^, and a whole host of other manifestations. Understanding that fatalities occur from factitious disease puts in perspective some of the bizarre ophthalmic injuries that result from this disorder.

Factitious Keratoconjunctivitis

In 1990, a classic case of bilateral factitious crystalline keratopathy was reported. An 18-year-old male presented with bilateral corneal ulcers involving the deep stroma associated with bilateral hypopyon ( Fig. 89.1 ). Peculiar blue, refractile, crystalline stromal deposits surrounding both ulcers were noted. The patient emphatically denied any traumatic injury. After extensive diagnostic and therapeutic intervention, no etiology could be discovered. After insulin syringes and a liquid mixture of the eye shadow were found, self-induced trauma was suspected. On confrontation, the patient admitted injecting his corneas with the mixture.

$Fig. 89.1, An 18-year-old with bilateral self-induced corneal ulcers produced by injecting each cornea with a solution mixed from eye shadow. Peculiar blue refractile crystals were visible in the corneal stroma.$

In 1982, six cases of self-inflicted eye injury were reported. Sharply delineated borders in the inferior and nasal quadrants were characteristic. Most were between 18 and 32 years old. Characteristically, they all had medical training and showed an attitude of serene indifference along with associated psychopathology. Pathologic examination of conjunctival epithelium showed no inflammation.

In 1963, 22 cases of self-inflicted corneal and conjunctival injuries were reported. A total of 166 injuries were described including chemical, mechanical, and thermal injuries. Ten eyes suffered complete blindness. A lack of interest in seeking medical care was characteristic and contributed significantly to a poor outcome.

In 1990, an 18-year-old female was discovered to be injecting air into the face, chest, and around the eye, resulting in subcutaneous, orbital, and subconjunctival emphysema. Anterior scleritis and cicatricial conjunctivitis have also been reported as factitious disorders. ^,

A peculiar type of factitious keratoconjunctivitis was reported in 1970. A 25-year-old man undertook a deliberate, systematic effort to eliminate vitamin A and β-carotene from all aspects of his diet for 5.5 years. Predictably, corneal xerosis, and corneal perforation occurred. Nonrecordable electroretinogram and electro-oculogram tracings were found. Interestingly, in the absence of generalized poor nutrition, Bitot spots were not found. The patient continually refused to receive vitamin A therapy.

Munchausen Syndrome (Chronic Factitious Disorder)

Munchausen syndrome, first described by Asher in 1951, is a chronic form of factitious disease characterized by frequent hospitalizations, self-inflicted injuries, dramatic medical histories, and multiple unnecessary invasive procedures resulting in massive, often unpaid, medical bills. The patient’s entire life may consist of trying to gain admission to a hospital. The famous case of McIlroy, the “champion sufferer,” illustrates this. McIlroy gained admission to more than 80 different hospitals in Great Britain with more than 200 separate admissions, receiving thousands of unnecessary tests and surgeries—all for factitious reasons.

Ocular Munchausen syndrome was best described by Rosenberg in 1986. A case series was presented of ocular Munchausen involving four female patients, ranging from 21 to 27 years, three of whom worked in the medical field. Self-induced trauma leading to corneal transplants and eventual enucleation, as well as self-inflicted alkali burns leading to blindness, were found. Voutilainen and Tuppurainen reported a case in 1989 of an 18-year-old woman who repeatedly inflicted damage to both eyes with a safety pin, causing perforation of the corneas. Winans et al. described a 23-year-old woman in 1983 who repeatedly injected air into her right orbit and periocular tissues. Eventual enucleation was required, after which she resumed the behavior on the opposite side. In 2000, Tahir et al. reported a case of a 30-year-old woman who had injected a mixture of saliva and tap water into both eyes, resulting in endophthalmitis.

Munchausen syndrome by proxy occurs when a parent is unable to distinguish the child’s needs from his or her own and submits the child to multiple unnecessary procedures and dangerous treatments while feigning laboratory results and concocting medical history to perpetuate the deception. Baskin et al. reported a case of this in 2003 in which an infant presented to the emergency department multiple times with recurrent keratoconjunctivitis. This is an important syndrome that all ophthalmologists should be aware of in perplexing cases of childhood ocular trauma.

Diagnosis of Factitious Keratoconjunctivitis

Many physicians have been first victims and then students of factitious disease. To make the diagnosis of factitious disease, the clinician must first discard any preconceived notions of the type or severity of self-induced injury a patient is capable of inflicting. Nothing is too bizarre. One must also discard the requirement to understand the reason behind the self-destructive behavior. It is the absolute senselessness of the act that makes this disorder so difficult to diagnose. An unusual presentation of a more common condition should first be eliminated. Second, somatoform disorders and malingering should be separated. Once these related disorders have been ruled out, any trauma that arises under suspicious or poorly explained circumstances should be suspect for factitious disease. Bilateral presentation of suspicious corneal lesions should raise a red flag. Any patient exhibiting poor healing or recurrent breakdown in which the clinical context does not support such occurrences should also be suspect. Epithelial defects that have nonbiologic edges (straight or square) should be considered as possibly factitious. As illustrated above, patients with factitious injuries often have associated psychologic stresses or other internal conflicts occurring simultaneously that may increase the risk of self-destructive behavior.

In general, factitious disease most commonly presents in patients in the second or third decade of life and more often among health allied professionals. Frequently, these patients will display less concern for the presenting problem than would normally be appropriate. Serene indifference is a hallmark in suggesting the diagnosis. Finally, factitious disease is nearly always found in the inferior or nasal quadrants and very rarely in the superior quadrant.

Patients with chronic factitious disease typically either emphatically deny any history of trauma or have an explanation that incompletely explains the severity or true nature of the injury. A history of multiple recurrent episodes of poorly explained disease is typical. The patients often improve dramatically when a marked eye patch is placed. Patients often refuse psychiatric care and seem inappropriately willing to accept the devastating consequences of their abusive behavior. The diagnosis is only made by the alert physician who is willing to confront the patient with conflicting evidence. Otherwise, these patients are all too willing to maintain their clever charades.

Treatment of Factitious Disease

Once the diagnosis has been made, success can occasionally be realized by using sympathetic authority. A diagnosis of unexplained injury is made. The physician then presents the patient with the conflicting evidence in a nonthreatening manner, which, in some cases, may result in an admission of involvement. If the patient’s confidence can be preserved, the patient’s need to depend on the physician may be maintained while, at the same time, gradually decreasing the frequency of visits. Most patients, however, remain resistant to treatment and refuse to cooperate with efforts to decrease the incidence of injury. A complete discussion of the treatment of these patients is beyond the scope of this text. ^, Psychiatric referral should be considered in all cases. Despite appropriate therapy, a large percentage of patients are destined for prolonged physical and psychologic morbidity.

Treatment of Munchausen syndrome is much less hopeful than that of isolated factitious disease.

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