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Prevalence of psychosocial disturbances in children with nonorganic visual loss
Prevalence of Psychosocial Disturbances in Children With Nonorganic Visual Loss Alexander Taich, MD,a Sue Crowe, COT.,b Gregory S. Kosmorsky, DO,b and Elias I. Traboulsi, MDb Purpose: To report on the prevalence of psychiatric disease and psychosocial stress in children with nonorganic visual loss. Materials and methods: Case series of 71 consecutive pediatric patients with a variety of nonorganic visual signs and symptoms. Chart review of ophthalmologic findings and details of medical and social history with main outcome measures of prevalence of psychiatric disease and psychosocial stress. Results: Psychological/ psychiatric disturbances in the form of anxiety, depression, and attention deficit hyperactivity disorder had been previously diagnosed in 19 (26.7%) patients. We had a very high index of suspicion of psychiatric illness in another four patients. Furthermore, we uncovered significant home and school stress in 22 (31%) patients. Sixteen (22.5%) patients wanted glasses and in 10 (14.1%) patients no cause for the behavior could be determined. Of those patients who wanted glasses, girls outnumbered boys 3:1. Conclusions: An underlying psychiatric or psychosocial disturbance should be ruled out in children who present with nonorganic visual loss. (J AAPOS 2004;8:457-461) isual dysfunction without an organic cause in children has been given a number of different names such as psychogenic visual disturbance,1 hysterical blindness,2 hysterical amblyopia,3 ocular conversion reaction,4 visual conversion reaction,5 psychogenic amblyopia,6 and amblyopic school-girl syndrome.7 Psychogenic visual loss in children has been recognized as being significantly different from malingering, in which there is willful, deliberate, and fraudulent feigning or exaggeration of symptoms of illness or injury done for the purpose of a consciously desired end.8 Finally, the term pithiatism has been used by some to refer to psychogenic visual loss. This term was originally coined by Babinski to describe the fact that isolated “hysterical” symptoms can be the product of the patient’s suggestibility and resolve when the doctor persuades the patient that they should go away.9 Indeed most of the signs and symptoms in children with nonorganic visual loss can be cured by suggestion. Thompson10 studied nonorganic visual loss in children and adults. He did not believe that such patients, as a rule, have psychiatric disease and hence do not need to see a psychiatrist. He recommended that the ophthalmologist prove that the patient has better visual fields and visual acuity than admitted to and perform a careful, dispassionate examination to establish that no organic disease is
V
From the Case Western Reserve University School of Medicine, Cleveland, OH and the Department of Pediatric Ophthalmology, Cole Eye Institute, Cleveland Clinic Foundation, Cleveland, OH. Revision accepted June 22, 2004. Reprint requests: Elias I. Traboulsi, MD, i32, 9500 Euclid Avenue, Cleveland, OH 44195. Copyright © 2004 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/2004/$35.00 ⫹ 0 doi:10.1016/j.jaapos.2004.06.006
Journal of AAPOS
present. Such an examination, he states, should make it possible to offer believable reassurance and effective therapy to the patient. However, nonorganic visual loss in adults is entirely different from children and the incidence of true malingering is much higher in adults. Several authors have demonstrated that significant psychosocial and psychiatric disorders are prevalent among children with nonorganic visual loss.3,6,7,11-13 For example, Yasuna3 studied 26 children who were mistakenly diagnosed with amblyopia. He concluded that many of these children had a pathologic pattern of school and home adjustment difficulties; however, he did not give details of his observations. In another study, a conversion type of psychoneurotic disorder was found in 11 of 20 children.11 A psychiatrist felt that in nine patients the reactions were secondary, and in two patients the reactions were primary. The remainder of the 20 children all showed anxiety, depressive anxiety, or psychoneurotic disorders, and 7 were felt to have a hysterical personality. Van Balen and Slijper6 used psychological testing and interviews with parents of children with psychogenic amblyopia and found no evidence of hysteria. They concluded that these children had a neurotic conflict; they wished to express feelings of hostility towards their parents, and yet did not want to lose their love. They found patients to be of aboveaverage intelligence and to react to emotional problems with physical complaints. In another study of 52 children, Mantyjarvi7 found a shared feature of pre-pubertal stress among children with nonorganic visual loss and a psychologic reason for the visual loss in 8% of patients. Catalano and co-workers12 discovered evidence of school difficulties in 39% of their patients and family problems in 35%. Clarke et al.,14 however, felt that such behavior is benign and did not suspect an underlying psychiatric disturbance October 2004
457
Journal of AAPOS Volume 8 Number 5 October 2004
458
MATERIALS AND METHODS We reviewed the charts of 71 consecutive pediatric patients with nonorganic visual loss who were examined from 1/1998 to 6/2003 at the Cole Eye Institute of the Cleveland Clinic Foundation. Patients were identified and classified at the time of diagnosis. The study was exempted from the need for informed consent by the IRB because it was an anonymous review of medical record data. One of the authors (E.I.T.) questioned all patients and accompanying adults about the medical and psychosocial history of the children. This author was sensitive to the possible role of adverse social or psychiatric factors in the pathogenesis of nonorganic visual loss and asked pertinent yet nondirective questions to elicit the necessary information. These questions were part of a routine review of patients with presentations consistent with nonorganic visual loss. Some questions were open-ended like “How are things at school/home?” and “Do you like school?” Others were more specific like “Are you very upset at anything at school or at home?” We diagnosed nonorganic visual loss when a rigorous examination failed to produce any evidence of ophthalmic pathology and when vision parameters significantly improved by the end of the examination compared to presenting values. No children were excluded on the basis of age. Patients were assigned to the following categories: (1) Previously diagnosed psychiatric disorder, including patients with Attention Deficit Hyperactivity Disorder (ADHD), and patients who presented with overwhelming evidence of psychiatric disease in the opinion of one of the authors (E.I.T.); (2) Easily identified stressors in home or school life such as significant academic difficulties, difficulty adjusting to new environments, separation from parents, physical abuse, and others. These stressors had to be evident from interview with patients and their guardians and sufficiently significant in the judgment of one of the authors (E.I.T.) to have potentially contributed to the nonorganic visual loss. Sexual abuse was included as a home stressor. However, guardians were not routinely or specifically asked about sexual abuse, and in all cases in which it was reported, the information was volunteered by
14
Females
12
Number of patients
in a group of 70 Canadian children with nonorganic visual loss. Other functional disorders in children may be caused by stressful events. For example, Vrabec and associates15 evaluated children 18 months to 10 years of age presenting with functional blinking and found that the parents of 41% of these children could identify a temporally related stressful event that coincided with onset of blinking. Because of conflicting conclusions about the etiology of nonorganic visual loss in the pediatric population in recent studies, we reviewed our experience to estimate the prevalence of psychosocial and psychiatric pathology in these children.
Males
10 8 6 4 2 0 4
5
6
7
8
9
10
11
12
13
14
15
16
17
18
Age
FIG 1. Age distribution in 71 boys and girls diagnosed with nonorganic visual loss.
the guardian in response to a more general inquiry about home environment; (3) Patients who wanted glasses, usually with history of a sibling or close friend recently receiving glasses, and who did not meet criteria for groups 1 or 2; (4) Patients in whom the etiology of the behavior was unclear and who did not meet criteria for above categories. All patients underwent detailed ocular examinations including visual acuity testing, evaluation of ocular motility, Ishihara color vision (Kanehara & Co., Ltd., Tokyo, Japan), and Titmus stereo acuity (Stereo Optical Co., Inc., Chicago, IL, USA) assessment, and Goldmann (Haag-Streit, Bern, Switzerland) or automated visual field testing as indicated. The only “trick” used to confirm nonorganic visual loss was the use of a plano lens. This was used only in patients in whom encouragement was insufficient to improve performance. The suspicion that anxiety derived from significant home or school stress might be the cause of the children’s behavior was communicated to the parent or guardian. Only the guardians of children with a psychiatric disorder or children in the psychosocial stress group were encouraged to seek professional help for their children in case they were not already receiving such help. A report of the eye examination findings as well as recommendations for psychosocial evaluation were sent to the primary care providers of patients who were not already under the care of a psychiatrist, psychologist, or social worker.
RESULTS There were 24 (33.8%) boys and 47 (66.2%) girls who ranged in age from 5 to 16 years. Mean age for girls was 9.5 years; mean age for boys was 8.1 years, and for all patients mean age was 9.0 years (Figure 1). The diagnosis of nonorganic visual loss was originally included in the differential for 76 patients. However, five patients were later excluded from the study. One patient was lost to follow-up before an accurate diagnosis was made; another was diagnosed with migraine headache; two were eventually diagnosed with Stargardt disease after fluorescein an-
Journal of AAPOS Volume 8 Number 5 October 2004
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TABLE 1 Presenting complaints and reasons for referral of 71 children with nonorganic visual loss (Some children had more than one complaint/reason for referral) Number of Patients (Percent Symptom of total)
TABLE 2 Classification of nonorganic visual loss in categories of “wants glasses,” psychosocial stress, psychiatric illness, undetermined, and their sub-categories in a group of 71 children by gender
Poor Vision Failed School/Pediatrician Screen Headache Other Symptoms Abnormal Visual Field Double Vision Poor Vision (monocular) Eye Pain Change in School Performance Squint Light Sensitivity Red Eye Routine Exam Poor Night Vision Tired Eye Eye Rubbing Loss of Color Vision
Wants Glasses Psychosocial Stress School Home Psychiatric illness ADHD alone Depression Other Undetermined
33 (46.5%) 26 (36.6%) 12 (16.9%) 7 (9.9%) 4 (5.6%) 4 (5.6%) 4 (5.6%) 4 (5.6%) 3 (4.2%) 3 (4.2%) 2 (2.8%) 2 (2.8%) 2 (2.8%) 1 (1.4%) 1 (1.4%) 1 (1.4%) 1 (1.4%)
giography was obtained due to consistently poor vision on further follow-up; and, finally, the fifth patient’s oscillopsia was determined to be of organic etiology. Complaints of poor vision, headache, and the failure of school or pediatrician’s visual acuity screening were the most common reasons for presentation or referral (Table 1). Eye pain was present in four patients. Two of these patients had suffered sexual abuse; another had an alcoholic mother and lived in a severely disrupted home environment, and the fourth patient did not have identifiable psychosocial pathology. Unilateral visual loss was present in four patients: two were sexually abused; one suffered from depression; and the fourth patient (who was the only patient with both monocular visual loss and eye pain) did not have identifiable psychosocial pathology. Objective components of comprehensive ocular examinations did not reveal significant findings in any patients. Of the 23 patients characterized as having a psychiatric illness, 19 carried a prior diagnosis of such. Four more patients had given a history sufficiently consistent with a psychiatric diagnosis that we were comfortable classifying them within this group. In this group the age range was 5 to 16 years; mean age was 10.2 years, and seven patients were 13 or older. While eight patients had a prior diagnosis of ADHD, only four carried the diagnosis of ADHD alone; the other four patients had ADHD with other psychiatric diagnoses. Seven patients carried a diagnosis of depression; of these, six were girls. Sixty-one percent of patients in the psychiatric illness group were already on medications for their disorders but it was not possible for us to assess how well their illnesses were controlled. Twenty-two patients were felt to be under significant home or school stress, based on history given by guardians.
Category
Total
Boys (Percent of all boys)
Girls (Percent of all girls)
16 22 8 14 23 4 7 12 10
4 (16.7%) 6 (25.0%) 1 (4.2%) 5 (20.8%) 9 (37.5%) 3 (12.5%) 1 (4.2%) 5 (20.8%) 5 (20.8%)
12 (25.5%) 16 (34.0%) 7 (14.9%) 9 (19.1%) 14 (29.8%) 1 (2.1%) 6 (12.8%) 7 (14.9%) 5 (10.6%)
Sixteen patients evidently sought the prescription of glasses; among these no apparent home or school stressors or psychiatric disease were identified. Finally, it was not possible to assign 10 patients to a specific group (Table 2). Of the children who presented with decreased visual acuity, at presentation visual acuity ranged from 20/40 to Hand Movement (HM) in the worst eye, with 50% of the patients 20/100 or worse. While all children had to manifest significant improvement in vision parameters to be included in the study, there were several in whom we were not able to document vision 20/30 OU or better. Overall, 57 (80.2%) patients had documented vision 20/30 OU or better at the end of the initial visit. A further six (8.5%) children demonstrated normal vision on follow-up. Eight children did not demonstrate vision 20/30 OU or better at the initial visit (most often due to lack of cooperation) and did not follow up. Among patients in whom we were not able to document normal visual acuity, three were in the home stress group; three were in the psychiatric illness group; two were in the “wants glasses” group, and one was in the unclear etiology group.
SELECTED CASE REPORTS Cases 1 and 2 Twin eight-year-old girls were first examined by an optometrist who recorded visual field defects without any apparent ocular abnormalities. He suspected a retinal dystrophy and referred them for further evaluation. Findings were identical in both. Vision was 20/40 OU in both children. Neither child had any stereo perception. With encouragement, vision improved to 20/20 in each eye, and they both had 40 seconds of arc of stereo acuity. Their sister was recently prescribed glasses. A diagnosis of benign childhood nonorganic visual loss was given, and the patients were included in the “wants glasses” category. The patients and their parents were reassured. Case 3 A nine-year-old girl failed her vision screening at her pediatrician’s office. She had 20/200 vision in the right eye
460 and 20/30 in the left eye. Stereo acuity was 80 seconds of arc. A plano lens improved vision to 20/20 in both eyes. On questioning, the patient was found to be under psychiatric care for depression and was receiving two different medications. She was included in the previously diagnosed psychiatric illness group. Case 4 A nine-year-old girl presented with left eye pain and would not open her eye. She also claimed HM vision in the same eye. She was examined on several occasions in the emergency room, claiming to have eye discharge and pain but no pathology could be found. After her first visit with us and on questioning her grandmother, we were told that she had been sexually abused. She was recently permanently separated from her mother and was already under a social worker’s care. She was included in the group of patients with significant home stress. Case 5 A 10-year-old girl was referred to rule out Stargardt disease. Her vision was recorded at 20/200 OU on several occasions. Nonorganic visual loss was not suspected initially. A number of tests were performed including electroretinography and fluorescein angiography, both of which were normal. She received a low vision evaluation and arrangements were made at school for preferential seating and print enlargement. The patient complied with these interventions without volunteering that she could indeed see well. Finally, after several visits, as our index of suspicion was rising, we were able to diagnose her with nonorganic visual loss. Her final visual acuity was 20/20 OU. Her father was unknown to her, and she lived with her grandmother while her mother lived in another state. She had significant school problems and had a difficult time adjusting both at home and at school. She was also included in the category of patients with significant home stress.
DISCUSSION Nonorganic or so-called functional visual loss is not uncommon in the pediatric population. Eames16 found that 9% of 193 unselected school children had tubular visual fields. In a population study in Finland, Mantyjarvi7 found that 1.75% of school children had possible nonorganic visual disorders, corresponding to 1.4 cases/1000 children per year in a population of 14,500 children in Finland between the ages of 7 and 18. Signs and symptoms of nonorganic visual loss include monocular or binocular reduced visual acuity, visual field loss, color vision anomalies, loss of stereo acuity, accommodation paralysis, and accommodation spasms, with all of these findings having been reported in different age groups. We found binocular visual loss (present in 67 patients) to be much more common than monocular visual loss (4 patients) in children. Vision was often reduced for
Journal of AAPOS Volume 8 Number 5 October 2004
distance and for near and children often claimed concomitant poor stereo and color vision. In our series, monocular visual loss was associated with underlying psychosocial pathology in three of four cases. Eye pain was also associated with psychosocial pathology in three of four cases and was the presenting complaint in one sexually abused girl (Case 4). The present series of 71 consecutive patients was collected over a period of four and a half years and gives an estimate of the number of patients that may be encountered in a busy tertiary care pediatric ophthalmology practice. Females outnumbered males 2:1. The average age of girls was 9.5 years as opposed to an average age of 8.1 years for boys. This is comparable to other studies.3,7,11 There was a variety of presenting complaints, the most common of which was poor or blurry vision. Some children were referred because they failed vision screening. Other complaints included headaches, abnormal visual fields, double vision, poor vision in one eye, change in school performance, eye pain, light sensitivity, and one patient with loss of color vision. One patient had a spiraling type of visual field typical of nonorganic disease; another had a tubular field and one had a hemianopic defect. Nineteen children were already under psychiatric care. This information was volunteered by the accompanying adult or was elicited when they were questioned about medical history or the use of medications. Specific inquiries revealed probable significant psychiatric problems in another four patients. Psychosocial pathology in the form of home/school stress was uncovered in an additional 22 patients. Consequently, additional psychosocial evaluation was suggested to the families and to primary care physicians of most of these patients but the results of any such evaluations, or whether they were performed, were not communicated to the authors. We recognize that formal evaluations may not have agreed with our impressions in some of these children. However, we feel strongly that the conditions that possibly made these children react with their ocular complaints were flagrant and quite significant. Overall, girls predominated in previous series of patients with nonorganic visual loss and constituted 77,3 55,11 77,6 and 92%7 of cases. Our results are consistent with these data, with girls constituting 66.2% of all patients. However, when broken down by group, the ratio of boys to girls changed. There were three girls for each boy in the group of patients who wanted to have glasses prescribed, as opposed to a ratio of 1.5:1 in the group with diagnosed or strongly suspected psychiatric disorders. The ophthalmologist must therefore suspect an underlying psychological abnormality more commonly in boys than in girls given a child presenting with nonorganic visual loss, but larger series of patients and formal psychosocial evaluations are needed to confirm this observation. Our youngest patient was five years old. Nonorganic visual loss can occur in children as young as three years of age. One such girl claimed blindness after being repri-
Journal of AAPOS Volume 8 Number 5 October 2004
manded by her mother.17 In another report, a four-andone-half-year-old girl claimed blindness at the age of two. She was on a rigid feeding and toilet training program and was cured after seven months of relaxing her schedule.18 The children in our “wants glasses” group span the range from “deliberate malingerer” to “suggestible innocent.”10 A malingerer will feign illness in order to achieve secondary gain. For the children in our “wants glasses” group this secondary gain may be, quite innocently, glasses. It is not illogical to suppose that some of these children are malingerers who, for whatever reason, want glasses. Yet others are “suggestible innocents” who, when someone close to them begins wearing glasses, ask themselves whether their vision, too, might not be impaired. These thoughts progress, perhaps, to a complaint to an adult, at which point they are likely reinforced by the concerns expressed by the adults. On the other hand, 63.4% of the children in our study do not seem to have readily apparent secondary gain; thus most of them are unlikely to be consciously feigning these symptoms. We believe that their ocular complaints are manifestations of the underlying disturbances. The use of ancillary ophthalmologic or neuroimaging tests may reinforce the abnormal behavior, as in Case 5 of this report. Unnecessary psychiatric testing may also induce undue stress and should only be obtained if a nonbenign cause of the behavior is suspected. A high index of suspicion of real ocular pathology should be maintained and a detailed social and psychiatric history should be obtained, trying to avoid unnecessary tests. The ophthalmologist must recognize that children presenting with nonorganic visual loss often have psychiatric or psychosocial pathology and must alert the parents and the primary care physician to this possibility. Reassurance, explanation of the problem to the family, and an offer to return should the symptoms recur are sufficient treatment. While this study is a retrospective case series review, we feel that ascertainment bias was minimized due to identification and classifying of all patients at the time of diag-
461 nosis. A prospective approach, however, might be preferable to standardize evaluation of psychiatric disease and psychosocial abnormalities. Additional studies that include formal psychological testing are warranted to clearly outline the spectrum of psychiatric and psychosocial abnormalities in children with nonorganic visual loss. References 1. Freud S. Psychogenic visual disturbance according to psychoanalytical conceptions. Collected Papers. 2 vol. 1st ed.; 1959. pp. 105-12. 2. Wolff E, Lachman GS. Hysterical blindness in children: report of two cases. Am J Dis Child 1938;55:743-9. 3. Yasuna E. Hysterical amblyopia in children. Am J Dis Child 1963; 106:68-73. 4. Krill AE, Newell FW. The diagnosis of ocular conversion reaction involving visual function. Arch Ophthalmol 1968;79:254-61. 5. Rada RT, Meyer GG, Kellner R. Visual conversion reaction in children and adults. J Nerv Ment Dis 1978;166:580-7. 6. van Balen AT, Slijper FE. Psychogenic amblyopia in children. J Pediatr Ophthalmol Strabismus 1978;15:164-7. 7. Mantyjarvi MI. The amblyopic schoolgirl syndrome. J Pediatr Ophthalmol Strabismus 1981;18:30-3. 8. Kramer KK, La Piana FG, Appleton B. Ocular malingering and hysteria: diagnosis and management. Surv Ophthalmol 1979;24:89-96. 9. Kathol RG. Pithiatism—lost but not forgotten. Psychiatr Med 1988; 6:17-21. 10. Thompson HS. Functional visual loss. Am J Ophthalmol 1985;100: 209-13. 11. Rada RT, Meyer GG, Krill AE. Visual conversion reaction in children. I. Diagnosis. Psychosomatics 1969;10:23-8. 12. Catalano RA, Simon JW, Krohel GB, Rosenberg PN. Functional visual loss in children. Ophthalmology 1986;93:385-90. 13. Barnard NA. Visual conversion reaction in children. Ophthalmic Physiol Opt 1989;9:372-8. 14. Clarke WN, Noel LP, Bariciak M. Functional visual loss in children: a common problem with an easy solution. Can J Ophthalmol 1996; 31:311-3. 15. Vrabec TR, Levin AV, Nelson LB. Functional blinking in childhood. Pediatrics 1989;83:967-70. 16. Eames TH. A study of tubular and spiral field in hysteria. Am J Ophthalmol 1947;30:610-1. 17. Schlaegel TF. Psychosomatic Ophthalmology. Baltimore: Williams and Wilkins; 1957. 18. Wolpe Z. Psychogenic visual disturbance in a four year old child. Nervous Child: Childhood Hysteria 1953;10:314-25.
An Eye on the Arts – The Arts on the Eye
“I dug this out of our library when the exhibition first came through. That’s Captain Barker. The New England whaling skippers were a tough lot. Many became captains in their twenties. Mutinies, destructive storms, hostile natives—all in a day’s work to them. The adversity made some men ogres, others humanitarians.” Austin examined the grainy black-and-white photograph in the book. Barker was dressed in native garb, and it was hard to make out his features. A fur parka framed his face, and some goggles with horizontal slits in them covered his eyes. White stubble adorned his chin. “Interesting eyewear,” Austin said. Those are sunglasses. The Inuit were very aware of the dangers from snow blindness. They would have been particularly important to Barker, whose eyes were probably sensitive to light. There was albinism in Barker’s family. They say that’s why he spent so many winters in the frozen north to avoid the direct sunlight. —Clive Cussler (from White Death)
V
From the Case Western Reserve University School of Medicine, Cleveland, OH and the Department of Pediatric Ophthalmology, Cole Eye Institute, Cleveland Clinic Foundation, Cleveland, OH. Revision accepted June 22, 2004. Reprint requests: Elias I. Traboulsi, MD, i32, 9500 Euclid Avenue, Cleveland, OH 44195. Copyright © 2004 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/2004/$35.00 ⫹ 0 doi:10.1016/j.jaapos.2004.06.006
Journal of AAPOS
present. Such an examination, he states, should make it possible to offer believable reassurance and effective therapy to the patient. However, nonorganic visual loss in adults is entirely different from children and the incidence of true malingering is much higher in adults. Several authors have demonstrated that significant psychosocial and psychiatric disorders are prevalent among children with nonorganic visual loss.3,6,7,11-13 For example, Yasuna3 studied 26 children who were mistakenly diagnosed with amblyopia. He concluded that many of these children had a pathologic pattern of school and home adjustment difficulties; however, he did not give details of his observations. In another study, a conversion type of psychoneurotic disorder was found in 11 of 20 children.11 A psychiatrist felt that in nine patients the reactions were secondary, and in two patients the reactions were primary. The remainder of the 20 children all showed anxiety, depressive anxiety, or psychoneurotic disorders, and 7 were felt to have a hysterical personality. Van Balen and Slijper6 used psychological testing and interviews with parents of children with psychogenic amblyopia and found no evidence of hysteria. They concluded that these children had a neurotic conflict; they wished to express feelings of hostility towards their parents, and yet did not want to lose their love. They found patients to be of aboveaverage intelligence and to react to emotional problems with physical complaints. In another study of 52 children, Mantyjarvi7 found a shared feature of pre-pubertal stress among children with nonorganic visual loss and a psychologic reason for the visual loss in 8% of patients. Catalano and co-workers12 discovered evidence of school difficulties in 39% of their patients and family problems in 35%. Clarke et al.,14 however, felt that such behavior is benign and did not suspect an underlying psychiatric disturbance October 2004
457
Journal of AAPOS Volume 8 Number 5 October 2004
458
MATERIALS AND METHODS We reviewed the charts of 71 consecutive pediatric patients with nonorganic visual loss who were examined from 1/1998 to 6/2003 at the Cole Eye Institute of the Cleveland Clinic Foundation. Patients were identified and classified at the time of diagnosis. The study was exempted from the need for informed consent by the IRB because it was an anonymous review of medical record data. One of the authors (E.I.T.) questioned all patients and accompanying adults about the medical and psychosocial history of the children. This author was sensitive to the possible role of adverse social or psychiatric factors in the pathogenesis of nonorganic visual loss and asked pertinent yet nondirective questions to elicit the necessary information. These questions were part of a routine review of patients with presentations consistent with nonorganic visual loss. Some questions were open-ended like “How are things at school/home?” and “Do you like school?” Others were more specific like “Are you very upset at anything at school or at home?” We diagnosed nonorganic visual loss when a rigorous examination failed to produce any evidence of ophthalmic pathology and when vision parameters significantly improved by the end of the examination compared to presenting values. No children were excluded on the basis of age. Patients were assigned to the following categories: (1) Previously diagnosed psychiatric disorder, including patients with Attention Deficit Hyperactivity Disorder (ADHD), and patients who presented with overwhelming evidence of psychiatric disease in the opinion of one of the authors (E.I.T.); (2) Easily identified stressors in home or school life such as significant academic difficulties, difficulty adjusting to new environments, separation from parents, physical abuse, and others. These stressors had to be evident from interview with patients and their guardians and sufficiently significant in the judgment of one of the authors (E.I.T.) to have potentially contributed to the nonorganic visual loss. Sexual abuse was included as a home stressor. However, guardians were not routinely or specifically asked about sexual abuse, and in all cases in which it was reported, the information was volunteered by
14
Females
12
Number of patients
in a group of 70 Canadian children with nonorganic visual loss. Other functional disorders in children may be caused by stressful events. For example, Vrabec and associates15 evaluated children 18 months to 10 years of age presenting with functional blinking and found that the parents of 41% of these children could identify a temporally related stressful event that coincided with onset of blinking. Because of conflicting conclusions about the etiology of nonorganic visual loss in the pediatric population in recent studies, we reviewed our experience to estimate the prevalence of psychosocial and psychiatric pathology in these children.
Males
10 8 6 4 2 0 4
5
6
7
8
9
10
11
12
13
14
15
16
17
18
Age
FIG 1. Age distribution in 71 boys and girls diagnosed with nonorganic visual loss.
the guardian in response to a more general inquiry about home environment; (3) Patients who wanted glasses, usually with history of a sibling or close friend recently receiving glasses, and who did not meet criteria for groups 1 or 2; (4) Patients in whom the etiology of the behavior was unclear and who did not meet criteria for above categories. All patients underwent detailed ocular examinations including visual acuity testing, evaluation of ocular motility, Ishihara color vision (Kanehara & Co., Ltd., Tokyo, Japan), and Titmus stereo acuity (Stereo Optical Co., Inc., Chicago, IL, USA) assessment, and Goldmann (Haag-Streit, Bern, Switzerland) or automated visual field testing as indicated. The only “trick” used to confirm nonorganic visual loss was the use of a plano lens. This was used only in patients in whom encouragement was insufficient to improve performance. The suspicion that anxiety derived from significant home or school stress might be the cause of the children’s behavior was communicated to the parent or guardian. Only the guardians of children with a psychiatric disorder or children in the psychosocial stress group were encouraged to seek professional help for their children in case they were not already receiving such help. A report of the eye examination findings as well as recommendations for psychosocial evaluation were sent to the primary care providers of patients who were not already under the care of a psychiatrist, psychologist, or social worker.
RESULTS There were 24 (33.8%) boys and 47 (66.2%) girls who ranged in age from 5 to 16 years. Mean age for girls was 9.5 years; mean age for boys was 8.1 years, and for all patients mean age was 9.0 years (Figure 1). The diagnosis of nonorganic visual loss was originally included in the differential for 76 patients. However, five patients were later excluded from the study. One patient was lost to follow-up before an accurate diagnosis was made; another was diagnosed with migraine headache; two were eventually diagnosed with Stargardt disease after fluorescein an-
Journal of AAPOS Volume 8 Number 5 October 2004
459
TABLE 1 Presenting complaints and reasons for referral of 71 children with nonorganic visual loss (Some children had more than one complaint/reason for referral) Number of Patients (Percent Symptom of total)
TABLE 2 Classification of nonorganic visual loss in categories of “wants glasses,” psychosocial stress, psychiatric illness, undetermined, and their sub-categories in a group of 71 children by gender
Poor Vision Failed School/Pediatrician Screen Headache Other Symptoms Abnormal Visual Field Double Vision Poor Vision (monocular) Eye Pain Change in School Performance Squint Light Sensitivity Red Eye Routine Exam Poor Night Vision Tired Eye Eye Rubbing Loss of Color Vision
Wants Glasses Psychosocial Stress School Home Psychiatric illness ADHD alone Depression Other Undetermined
33 (46.5%) 26 (36.6%) 12 (16.9%) 7 (9.9%) 4 (5.6%) 4 (5.6%) 4 (5.6%) 4 (5.6%) 3 (4.2%) 3 (4.2%) 2 (2.8%) 2 (2.8%) 2 (2.8%) 1 (1.4%) 1 (1.4%) 1 (1.4%) 1 (1.4%)
giography was obtained due to consistently poor vision on further follow-up; and, finally, the fifth patient’s oscillopsia was determined to be of organic etiology. Complaints of poor vision, headache, and the failure of school or pediatrician’s visual acuity screening were the most common reasons for presentation or referral (Table 1). Eye pain was present in four patients. Two of these patients had suffered sexual abuse; another had an alcoholic mother and lived in a severely disrupted home environment, and the fourth patient did not have identifiable psychosocial pathology. Unilateral visual loss was present in four patients: two were sexually abused; one suffered from depression; and the fourth patient (who was the only patient with both monocular visual loss and eye pain) did not have identifiable psychosocial pathology. Objective components of comprehensive ocular examinations did not reveal significant findings in any patients. Of the 23 patients characterized as having a psychiatric illness, 19 carried a prior diagnosis of such. Four more patients had given a history sufficiently consistent with a psychiatric diagnosis that we were comfortable classifying them within this group. In this group the age range was 5 to 16 years; mean age was 10.2 years, and seven patients were 13 or older. While eight patients had a prior diagnosis of ADHD, only four carried the diagnosis of ADHD alone; the other four patients had ADHD with other psychiatric diagnoses. Seven patients carried a diagnosis of depression; of these, six were girls. Sixty-one percent of patients in the psychiatric illness group were already on medications for their disorders but it was not possible for us to assess how well their illnesses were controlled. Twenty-two patients were felt to be under significant home or school stress, based on history given by guardians.
Category
Total
Boys (Percent of all boys)
Girls (Percent of all girls)
16 22 8 14 23 4 7 12 10
4 (16.7%) 6 (25.0%) 1 (4.2%) 5 (20.8%) 9 (37.5%) 3 (12.5%) 1 (4.2%) 5 (20.8%) 5 (20.8%)
12 (25.5%) 16 (34.0%) 7 (14.9%) 9 (19.1%) 14 (29.8%) 1 (2.1%) 6 (12.8%) 7 (14.9%) 5 (10.6%)
Sixteen patients evidently sought the prescription of glasses; among these no apparent home or school stressors or psychiatric disease were identified. Finally, it was not possible to assign 10 patients to a specific group (Table 2). Of the children who presented with decreased visual acuity, at presentation visual acuity ranged from 20/40 to Hand Movement (HM) in the worst eye, with 50% of the patients 20/100 or worse. While all children had to manifest significant improvement in vision parameters to be included in the study, there were several in whom we were not able to document vision 20/30 OU or better. Overall, 57 (80.2%) patients had documented vision 20/30 OU or better at the end of the initial visit. A further six (8.5%) children demonstrated normal vision on follow-up. Eight children did not demonstrate vision 20/30 OU or better at the initial visit (most often due to lack of cooperation) and did not follow up. Among patients in whom we were not able to document normal visual acuity, three were in the home stress group; three were in the psychiatric illness group; two were in the “wants glasses” group, and one was in the unclear etiology group.
SELECTED CASE REPORTS Cases 1 and 2 Twin eight-year-old girls were first examined by an optometrist who recorded visual field defects without any apparent ocular abnormalities. He suspected a retinal dystrophy and referred them for further evaluation. Findings were identical in both. Vision was 20/40 OU in both children. Neither child had any stereo perception. With encouragement, vision improved to 20/20 in each eye, and they both had 40 seconds of arc of stereo acuity. Their sister was recently prescribed glasses. A diagnosis of benign childhood nonorganic visual loss was given, and the patients were included in the “wants glasses” category. The patients and their parents were reassured. Case 3 A nine-year-old girl failed her vision screening at her pediatrician’s office. She had 20/200 vision in the right eye
460 and 20/30 in the left eye. Stereo acuity was 80 seconds of arc. A plano lens improved vision to 20/20 in both eyes. On questioning, the patient was found to be under psychiatric care for depression and was receiving two different medications. She was included in the previously diagnosed psychiatric illness group. Case 4 A nine-year-old girl presented with left eye pain and would not open her eye. She also claimed HM vision in the same eye. She was examined on several occasions in the emergency room, claiming to have eye discharge and pain but no pathology could be found. After her first visit with us and on questioning her grandmother, we were told that she had been sexually abused. She was recently permanently separated from her mother and was already under a social worker’s care. She was included in the group of patients with significant home stress. Case 5 A 10-year-old girl was referred to rule out Stargardt disease. Her vision was recorded at 20/200 OU on several occasions. Nonorganic visual loss was not suspected initially. A number of tests were performed including electroretinography and fluorescein angiography, both of which were normal. She received a low vision evaluation and arrangements were made at school for preferential seating and print enlargement. The patient complied with these interventions without volunteering that she could indeed see well. Finally, after several visits, as our index of suspicion was rising, we were able to diagnose her with nonorganic visual loss. Her final visual acuity was 20/20 OU. Her father was unknown to her, and she lived with her grandmother while her mother lived in another state. She had significant school problems and had a difficult time adjusting both at home and at school. She was also included in the category of patients with significant home stress.
DISCUSSION Nonorganic or so-called functional visual loss is not uncommon in the pediatric population. Eames16 found that 9% of 193 unselected school children had tubular visual fields. In a population study in Finland, Mantyjarvi7 found that 1.75% of school children had possible nonorganic visual disorders, corresponding to 1.4 cases/1000 children per year in a population of 14,500 children in Finland between the ages of 7 and 18. Signs and symptoms of nonorganic visual loss include monocular or binocular reduced visual acuity, visual field loss, color vision anomalies, loss of stereo acuity, accommodation paralysis, and accommodation spasms, with all of these findings having been reported in different age groups. We found binocular visual loss (present in 67 patients) to be much more common than monocular visual loss (4 patients) in children. Vision was often reduced for
Journal of AAPOS Volume 8 Number 5 October 2004
distance and for near and children often claimed concomitant poor stereo and color vision. In our series, monocular visual loss was associated with underlying psychosocial pathology in three of four cases. Eye pain was also associated with psychosocial pathology in three of four cases and was the presenting complaint in one sexually abused girl (Case 4). The present series of 71 consecutive patients was collected over a period of four and a half years and gives an estimate of the number of patients that may be encountered in a busy tertiary care pediatric ophthalmology practice. Females outnumbered males 2:1. The average age of girls was 9.5 years as opposed to an average age of 8.1 years for boys. This is comparable to other studies.3,7,11 There was a variety of presenting complaints, the most common of which was poor or blurry vision. Some children were referred because they failed vision screening. Other complaints included headaches, abnormal visual fields, double vision, poor vision in one eye, change in school performance, eye pain, light sensitivity, and one patient with loss of color vision. One patient had a spiraling type of visual field typical of nonorganic disease; another had a tubular field and one had a hemianopic defect. Nineteen children were already under psychiatric care. This information was volunteered by the accompanying adult or was elicited when they were questioned about medical history or the use of medications. Specific inquiries revealed probable significant psychiatric problems in another four patients. Psychosocial pathology in the form of home/school stress was uncovered in an additional 22 patients. Consequently, additional psychosocial evaluation was suggested to the families and to primary care physicians of most of these patients but the results of any such evaluations, or whether they were performed, were not communicated to the authors. We recognize that formal evaluations may not have agreed with our impressions in some of these children. However, we feel strongly that the conditions that possibly made these children react with their ocular complaints were flagrant and quite significant. Overall, girls predominated in previous series of patients with nonorganic visual loss and constituted 77,3 55,11 77,6 and 92%7 of cases. Our results are consistent with these data, with girls constituting 66.2% of all patients. However, when broken down by group, the ratio of boys to girls changed. There were three girls for each boy in the group of patients who wanted to have glasses prescribed, as opposed to a ratio of 1.5:1 in the group with diagnosed or strongly suspected psychiatric disorders. The ophthalmologist must therefore suspect an underlying psychological abnormality more commonly in boys than in girls given a child presenting with nonorganic visual loss, but larger series of patients and formal psychosocial evaluations are needed to confirm this observation. Our youngest patient was five years old. Nonorganic visual loss can occur in children as young as three years of age. One such girl claimed blindness after being repri-
Journal of AAPOS Volume 8 Number 5 October 2004
manded by her mother.17 In another report, a four-andone-half-year-old girl claimed blindness at the age of two. She was on a rigid feeding and toilet training program and was cured after seven months of relaxing her schedule.18 The children in our “wants glasses” group span the range from “deliberate malingerer” to “suggestible innocent.”10 A malingerer will feign illness in order to achieve secondary gain. For the children in our “wants glasses” group this secondary gain may be, quite innocently, glasses. It is not illogical to suppose that some of these children are malingerers who, for whatever reason, want glasses. Yet others are “suggestible innocents” who, when someone close to them begins wearing glasses, ask themselves whether their vision, too, might not be impaired. These thoughts progress, perhaps, to a complaint to an adult, at which point they are likely reinforced by the concerns expressed by the adults. On the other hand, 63.4% of the children in our study do not seem to have readily apparent secondary gain; thus most of them are unlikely to be consciously feigning these symptoms. We believe that their ocular complaints are manifestations of the underlying disturbances. The use of ancillary ophthalmologic or neuroimaging tests may reinforce the abnormal behavior, as in Case 5 of this report. Unnecessary psychiatric testing may also induce undue stress and should only be obtained if a nonbenign cause of the behavior is suspected. A high index of suspicion of real ocular pathology should be maintained and a detailed social and psychiatric history should be obtained, trying to avoid unnecessary tests. The ophthalmologist must recognize that children presenting with nonorganic visual loss often have psychiatric or psychosocial pathology and must alert the parents and the primary care physician to this possibility. Reassurance, explanation of the problem to the family, and an offer to return should the symptoms recur are sufficient treatment. While this study is a retrospective case series review, we feel that ascertainment bias was minimized due to identification and classifying of all patients at the time of diag-
461 nosis. A prospective approach, however, might be preferable to standardize evaluation of psychiatric disease and psychosocial abnormalities. Additional studies that include formal psychological testing are warranted to clearly outline the spectrum of psychiatric and psychosocial abnormalities in children with nonorganic visual loss. References 1. Freud S. Psychogenic visual disturbance according to psychoanalytical conceptions. Collected Papers. 2 vol. 1st ed.; 1959. pp. 105-12. 2. Wolff E, Lachman GS. Hysterical blindness in children: report of two cases. Am J Dis Child 1938;55:743-9. 3. Yasuna E. Hysterical amblyopia in children. Am J Dis Child 1963; 106:68-73. 4. Krill AE, Newell FW. The diagnosis of ocular conversion reaction involving visual function. Arch Ophthalmol 1968;79:254-61. 5. Rada RT, Meyer GG, Kellner R. Visual conversion reaction in children and adults. J Nerv Ment Dis 1978;166:580-7. 6. van Balen AT, Slijper FE. Psychogenic amblyopia in children. J Pediatr Ophthalmol Strabismus 1978;15:164-7. 7. Mantyjarvi MI. The amblyopic schoolgirl syndrome. J Pediatr Ophthalmol Strabismus 1981;18:30-3. 8. Kramer KK, La Piana FG, Appleton B. Ocular malingering and hysteria: diagnosis and management. Surv Ophthalmol 1979;24:89-96. 9. Kathol RG. Pithiatism—lost but not forgotten. Psychiatr Med 1988; 6:17-21. 10. Thompson HS. Functional visual loss. Am J Ophthalmol 1985;100: 209-13. 11. Rada RT, Meyer GG, Krill AE. Visual conversion reaction in children. I. Diagnosis. Psychosomatics 1969;10:23-8. 12. Catalano RA, Simon JW, Krohel GB, Rosenberg PN. Functional visual loss in children. Ophthalmology 1986;93:385-90. 13. Barnard NA. Visual conversion reaction in children. Ophthalmic Physiol Opt 1989;9:372-8. 14. Clarke WN, Noel LP, Bariciak M. Functional visual loss in children: a common problem with an easy solution. Can J Ophthalmol 1996; 31:311-3. 15. Vrabec TR, Levin AV, Nelson LB. Functional blinking in childhood. Pediatrics 1989;83:967-70. 16. Eames TH. A study of tubular and spiral field in hysteria. Am J Ophthalmol 1947;30:610-1. 17. Schlaegel TF. Psychosomatic Ophthalmology. Baltimore: Williams and Wilkins; 1957. 18. Wolpe Z. Psychogenic visual disturbance in a four year old child. Nervous Child: Childhood Hysteria 1953;10:314-25.
An Eye on the Arts – The Arts on the Eye
“I dug this out of our library when the exhibition first came through. That’s Captain Barker. The New England whaling skippers were a tough lot. Many became captains in their twenties. Mutinies, destructive storms, hostile natives—all in a day’s work to them. The adversity made some men ogres, others humanitarians.” Austin examined the grainy black-and-white photograph in the book. Barker was dressed in native garb, and it was hard to make out his features. A fur parka framed his face, and some goggles with horizontal slits in them covered his eyes. White stubble adorned his chin. “Interesting eyewear,” Austin said. Those are sunglasses. The Inuit were very aware of the dangers from snow blindness. They would have been particularly important to Barker, whose eyes were probably sensitive to light. There was albinism in Barker’s family. They say that’s why he spent so many winters in the frozen north to avoid the direct sunlight. —Clive Cussler (from White Death)