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Many terms are useful ( Table 63.1 ) but none perfectly encapsulates what we refer to as visual conversion disorder (VCD). Conversion disorder implies a particular unconscious process wherein vision is apparently and inexplicably lost, outside of the patient’s awareness. In this chapter we will cover a variety of types of unexplained vision loss, including conversion disorder and unexplained vision loss within the patient’s conscious awareness.
Terminology | Advantages/disadvantages |
---|---|
Conversion disorder (visual conversion disorder, VCD) | The preferred term of the American Psychiatric Association but it implies certain knowledge of the underlying mechanism |
Psychogenic blindness | Terms that are still used and can be helpful |
Hysterical blindness/amblyopia | Incorrectly implies instability or “madness” but it is still commonly used |
Stress-related visual disorder | Many cases are not apparently stressed by any definition |
Conversion neurosis | Implies a certain knowledge of the underlying mechanism and instability |
Medically unexplained visual loss | Obscures the meaning from parents and patients and poses a question that must be answered |
Amblyopic schoolgirl syndrome | A sexist and pejorative term |
Functional visual loss | Meaningless term designed to obscure the meaning from parents and patients |
Malingering | A term correctly used for someone who deliberately feigns a symptom for some form of gain – compensation, avoidance of work, military service, etc. This is usually seen in adults |
Factitious disorder | A term correctly used for a person acting as if they have an illness by deliberately producing, feigning, or exaggerating symptoms to gain attention or sympathy. Used to be known as Munchausen syndrome |
Factitious disorder by proxy | Used to be known as Munchausen syndrome by proxy. Symptoms or signs produced in a child by a carer to elicit sympathy, etc., for the carer |
Non-organic visual loss | Many parents do not understand what “organic” means in this context but it can be useful when explained |
These fabricated disorders will be also be termed VCD. In fact, considerable overlap exists between deliberate fabrication of vision loss and unconscious mechanisms of vision loss. Tests for either cause are the same, and the ophthalmologist’s charge remains the same, to accurately diagnose a problem and its treatment.
Children commonly present to ophthalmologists not only with symptoms that do not fit in with known ophthalmic diseases but that also must be proved to have characteristics that definitely cannot be caused by organic disease . VCD is not just a diagnosis of exclusion but one that is made by positive identification of signs that cannot be due to disease. It is safer to assign those without positive diagnosis to an “unknown cause – to be reviewed” category than to assign them an incorrect diagnosis.
Many symptoms that children describe are not understandable but they are not necessarily fabricated. A child's naïve description of a normal phenomenon is easily, incorrectly interpreted to be an abnormal or fabricated one. The child's interpretation is not usually a conscious deception.
The major characteristics of VCD are as follows (modified from the Diagnostic and Statistical Manual, Fourth Edition [DSM-IV] of the American Psychiatric Association ):
One or more symptoms or deficits are present that affect voluntary motor or sensory function, suggesting a neurologic or other medical condition.
Psychologic factors are associated with the symptom or deficit because conflicts or other stressing events precede the initiation or exacerbation of the symptom or deficit by a variable time. It is often difficult or impossible to find any clearly abnormal stressing event.
The symptom or deficit is not intentionally produced or feigned (as it is in factitious disorder or malingering ):
After appropriate investigation, it cannot be explained fully by a medical condition, the effects of a substance, or as a culturally sanctioned behavior.
It causes significant distress or impairment in social, educational, or other important areas of functioning or warrants medical evaluation.
It is not limited to pain or, in older children, to sexual dysfunction and is not better accounted for by another mental disorder.
Conversion disorder was described as a loss or distortion of neurological function not fully explained by organic disease. The patient has an internal conflict, of which they are unaware, which becomes converted into a symptom as a means of expression after dissociation, a mental mechanism whereby underlying feelings and the symptoms are separated. Conversion disorder can be distinguished from other psychiatric disorders mimicking organic loss by its absence of conscious or intentional desire to trick the doctor (or parent). The child with the ocular manifestations of a VCD develops visual loss due to unconscious problems or mental disturbances outside their awareness. Often such children have a history of previous conversion reactions, not necessarily involving the visual system.
The child with VCD is typically between 6 and 16 years of age, with a preponderance of girls. There may be a family history of illness or of eye disease. The symptoms come on gradually in most cases, often following a marginal failure at an eye test. Subsequent examinations reveal varying degrees of acuity and visual field loss, often worsening as time goes on but rarely to the extent that the child becomes bilaterally blind. Most children are little inconvenienced even by an apparently marked visual loss. Repeated objective examinations, neurophysiology, and radiology, are all normal. The condition is usually bilateral, with the most common complaints being “just not seeing,” blurred vision, or distorted or small images. Occasionally visual field defects are described, commonly “tunnel vision”; hemianopias are occasionally encountered. Central scotomas are rare and should make one think of associated organic disease. Non-visual defects occasionally also occur, including spasm of the near reflex, headaches, voluntary nystagmus, and eye movement tics, contraversive eye deviation, and accommodation paralysis.
The symptoms of VCD have some of the following characteristics:
They conform to the child's concept of a symptom or a disorder.
They are definable, if somatic, in terms of positive evidence and, if psychologic, by techniques of clinical examination.
They are related to emotional conflict.
Despite being profound, the symptoms often cause little concern to the affected child (formerly known as “la belle indifference.”
There is usually only one symptom. It is unusual for this to present with multiple symptoms, especially in more than one organ system.
Conversion disorders are rare under 6 years old, and the gender ratio is equal up to 10 years, then females outnumber males by 3:1.
In children, both visual fields and acuity are affected; adults tend to be monosymptomatic.
Family disharmony is common and abusive or incestuous relationships should be borne in mind as an underlying cause.
Patients tend to have equal difficulty with both large and small letters and read down the letter chart very slowly from the top to the lowest they can achieve, often getting further down the chart if cajoled or if the test is done competitively. They read the near vision test excruciatingly slowly, often only to a much worse level than that achieved at distance.
A few children have a history of previous psychiatric or psychologic disease, but it is often difficult to elicit the history. Most are normal children.
Some children develop somatic complaints in response to depression. Therefore, questions that can elicit signs of depression should be raised when depression is suspected. Depressed children may have sleep and eating disturbances, and may have suicidal thoughts. Their peer relationships may have suffered in the preceding months, and they may appear irritable. These need to be addressed, with help from a pediatrician or psychiatrist.
The occasional child in an abusive environment will use vision symptoms as a proxy-request for help from a health provider. Abuse can be hard to recognize and its history difficult to elicit, especially if the child is in the company of abusive caregivers. Suspicion should be aroused when a child has multiple visits to the doctor with unexplained symptoms, physical signs of abuse, recurrent trips to the emergency department, or, rarely, an actual complaint about abuse. Healthcare providers are mandated reporters (to social services) in most areas. Abuse only need be suspected to report it. It does not require confirmation by the eyecare provider. Child maltreatment is discussed further in Chapter 71 .
Non-organic symptoms are common among referrals to a pediatric ophthalmology service. Prompt, correct diagnosis with appropriate management saves the doctor, the child, and the parents much heartache and time and saves the discomfort and risk of unnecessary investigations.
The fear of missing organic disease makes doctors more cautious about diagnosing non-organic disorders. In VCD, misdiagnoses occur particularly with macular disease and hereditary optic neuropathy in children, but no disease is exempt!
The most common misinterpretation of unexplained vision loss occurs in Stargardt disease, where visual acuity may be diminished in the absence of physical findings. Retinal autofluorescence, fluorescein angiography or electrophysiology testing can elucidate the diagnosis, but many children with this disorder are seen repeatedly before the diagnosis becomes apparent.
Organic disease may be associated with non-organic disease; the classic situation is the occurrence of pseudoseizures in epileptics. The same may happen with VCD but, in childhood , the symptoms usually occur free of both organic and psychiatric disease.
Inquiry into the background, looking for the underlying stress that produces the symptoms, should center on two main areas:
The home and family . Conflict between children, sibling rivalry, the child who needs more attention, an unhappy marriage, overcrowding, sexual abuse, harassment or bullying by relatives or others, or conflict with neighbors and their children may be predisposing factors.
The school . In the school setting, it is often the slow child who is being “overstretched” or the bright child who is being “understretched” who may produce visual symptoms. Unsympathetic or aggressive teachers, teasing or bullying, sexual or other harassment, or abuse all predispose to VCD.
Enlist the help of the parents; tell them explicitly of the possible underlying problems so that they can best help their own child, because relieving underlying factors is the best treatment.
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