SURVEY OF OPHTHALMOLOGY

MAJOR

VOLUME 37.

NUMBER 3. NOVEMBER-DECEMBER

1992

REVIEW

Pediatric Pseudotumor Cerebri (Idiopathic Intracranial Hypertension) SIMMONS

LESSELL,

M.D.

Neuro-ophthalmology Unit, Department of Ophthalmology, Massachusetts Eye and Ear Infrmary and Harvard Medical School, Boston, Massachusetts

Abstract. This review focuses on the features of pseudotumor cerebri in the pediatric age group. There is no sex predilection in children, and obesity does not appear to be an important factor. Infants and young children may present with irritability, apathy, or somnolence, rather than headache. Dizziness and ataxia may also occur. Papilledema is infrequently noted in pediatric patients if the fontanelles are open or the sutures are split. Pre-adolescents a@ear more likely than adults or adolescents to have manifestations of their pseudotumor cerebri other than headache and papilledema, including lateral rectus pareses, vertical strabismus, facial paresis, back and neck pain. Among the etiologies that are particularly pertinent to children are tetracycline therapy, malnutrition or renutrition, and the correction of hypothyroidism. Children with pseudotumor cerebri are at risk for visual loss and their visual function must be closely monitored. Surgical intervention is imperative when vision is threatened. (Surv Ophthalmol 37:155-166, 1992)

Key words. corticosteroids drug toxicity optic nerve sheath fenestration papilledema thyroid replacement pseudotumor cerebri ??

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intracranial hypertension pediatric pseudotumor vitamin deficiency

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motility cerebri

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I. Nomenclature and Definitions

Many ophthalmologists are surprised to learn that pseudotumor cerebri occurs in the pediatric age group. In fact, a sizable proportion of the patients in early series28*37were between 5 an’d 15 years of age, and the medical literature is replete with examples of the disorder in infants. This review will concentrate on those features of pseudotumor that are important in the pediatric age group, with emphasis on those aspects with which the educated and interested ophthalmologist should be familiar - semiology, etiology, and evaluation. Treatment will be dealt with only briefly, since the literature, unfortunately, provides a scant foundation for constructing a rational treatment plan for children with pseudotumor cerebri.

Experts disagree der consideration

about how the disorder should be designated,

unlet

alone defined. No single definition or designation will satisfy all critics. I choose to call it pseudotumor cerebri (PTC), despite the increasing preference for “idiopathic intracranial hypertension,” because patients in whom the etiology of the intracranial hypertension is known should be included. Pseudotumor cerebri also seems to be the name by which most physicians recognize the disease. This is a review ofpediatric pseudotumor cerebri and, to be complete, the term pediatric should also be defined. Unfortunately, it is more 155

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difficult to define pediatric than to define pseudotumor cerebri. Everyone would agree that pre-pubertal patients are pediatric, but when, if ever, an adolescent can be considered an adult must be determined on an individual basis. Pseudotumor cerebri is traditionally defined as a disease that satisfies the following criteria: raised intracranial pressure, normal cerebrospinal fluid contents, and a normal brain with normal or small ventricles on imaging studies. Papilledema is assumed to be present in one or both eyes. Abducens palsies are allowed, but the remainder of the elementary neurological examination should be unremarkable. These criteria seem satisfactory. However, a recent neurophilosophical contribution5’ has rekindled controversy concerning the concept of pseudotumor cerebri by offering “. . . a proposed classification of the pseudotumor syndrome aimed at broadening the diagnostic criteria applied currently to PTC.” I find myself only partially in sympathy with the catholic views of those authors. One should include cases of known and unknown etiology, since all instances of pseudotumor cerebri have an etiology; etiology simply has yet to be identified in the idiopathic group. Some “splitters” argue for the exclusion of patients with clinical or neuroimaging evidence of impaired cerebral venous drainage. “Lumpers” argue that they should be included since they satisfy -- the classical criteria for the diagnosis of pseudotumor cerebri. The lumpers have a more convincing position. However, I stand with splitters in favoring the exclusion of patients whose intracranial pressure is not elevated, the exclusion of patients with ventriculomegaly, and the exclusion of patients with abnormalities in the contents of their cerebrospinal fluid. Especially relevant to the topic ofpediatric pseudotumor cerebri is the category, “infantile pseudotumor syndrome,” identified by Johnston et al. 61This refers to neurologitally normal, macrocephalic infants with an expanded subarachnoid space and some degree of ventricular enlargement. I would exclude these cases, if only because of the ventricular enlargement. The issue of whether or not to include patients who have normal optic discs is probably moot in the special case of childhood pseudotumor cerebri, since for one reason or another papilledema is absent or unrecognized in many young children who almost certainly have the disease (see below). In the sections that follow I have tried to be liberal by including, for the purposes of this re-

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view, cases of pseudotumor that depart somewhat from the strict definition. These include some cases in which seizures or focal neurological signs other than papilledema or lateral rectus palsies were noted. Some day, when the pathophysiology of pseudotumor cerebri is known and the diagnosis can be validated by extrasemiological means, nosologists can decide if these cases deserve inclusion. II.

Clinical Characteristics

A. SEX RATIO Most adult patients with pseudotumor cerebri are female 94,g6,‘20but this sex predilection is not found in children. Among Grant’s 79 patients up to the age of 15 years, 53% were male and 47% fernalee4’j In Rose and Matson’s series of patients up to age 11 years, there were 14 males and nine females.g4 In Weisberg and Chutorian’s series, in the group 12 years and younger, 16 of the 30 patients were male. lz2 There were eight males and six females in the patients under age 13 years reported by Hagberg and Sillanpaa.48 B. INITIAL

SYMPTOMS

Older children and adolescents typically complain of headache, sometimes accompanied by nausea and vomiting. However, as Baker et al* observed in their cases of “idiopathic intracranial hypertension,” young children may present with irritability rather than headache. This has also children been noted by others. 33,38,71,74,79&3 SOme are asymptomatic, their papilledema discovered at a routine school eye examination.46 Pseudotumor cerebri can also present in infants as somnolence” or apathy. 50*g’Ataxia and dizziness are early symptoms in some childhood cases.z’26,4’*50 The ataxia is intermittent. Neck, shoulder, or There is a re_ back pain may occur. 2,5,*6,*9.35,41,80 port of seizures7’ and another of (possibly) ictal twitching of one hand.38 Paresthesias,26 facial numbness,50 tinnitus,50 and limb numbness*’ have also been described. C. PAPILLEDEMA Papilledema is nearly always found in older children and adolescents, but is infrequently reported in infants with pseudotumor cerebri who have a bulging fontanelle or split sutures. In many of these cases, one cannot determine if papilledema was absent or merely unrecognized. Two series provide exceptions. Information about the fontanelles was not included, but apparently papilledema was present in Grant’s cases of pseudotumor cerebri even in infants

157 with split sutures.46 Rose and Matsong included six cases of papilledema in childhood pseudotumor patients with suture diastasis. There is reason to conclude that, contrary to the prevalent belief, infants with open fontanelles who have elevated intracranial pressure may manifest papilledema. This is based upon observations in children with brain tumors.“’ D. OTHER SIGNS The incidence of paralytic esotropias seems high in the pediatric pseudotumor cerebri population. Twenty-nine of 68 in the series by Baker et al* had sixth nerve palsies. Sixth nerve palsies were found in 6 of 9, 7 of 10, and 10 of 23 in three other series,47*g4*‘08but in only 5 of 37 patients in another.‘(j Other disorders of ocular motility have also been observed, including 3rd nerve palsies, ” 4th nerve palsies, 40,5’and hypertropias” (concomitant and nonconcomitant) that were not further characterized. In the Baker et al series, there were two patients with signs of upward herniation of the cerebellum.* Both had light-near dissociation, one had limited upward gaze, and the other had a bilateral internuclear ophthalmoplegia! In 1977 Chutorian et al” reported three cases of unilateral facial paralysis in children with pseudotumor cerebri. The subsequent literature contains additional cases,2’26,50*7’ including six examples among 68 cases of pseudotumor cerebri in the Baker et al series.’ Other reported signs include Babinski reflexes,2g~45~67 bruit,26,74,8s hypoglossal nerve palsy,’ and torticollis.2,5 Pseudotumor cerebri in infants is usually diagnosed without measuring the cerebrospinal fluid pressure and often without analyzing the fluid. In one publication the authors commented that they were not certain if the pressure they measured in a recumbent 3-month-old represented elevated intracranial pressure!” Considering the difficulty in accurately measuring the intracranial pressure in small children, this is not surprising. Nevertheless, I have been assured by Dr. Charles Barlow, Professor of Pediatric Neurology at Harvard Medical School (personal telephone communication, September 13, 1991) that a bulging fontanelle in a calm, seated infant indicates elevated intracranial pressure. A method has even been published for estimating the intracranial pressure from the bulging fontanelle to within + one centimeter of water.‘25,‘26 According to the principle of applanation, the fontanelle will be flat when the intracranial pres-

sure equals the atmospheric pressure. If the fontanelle is bulging in a quiet recumbent infant, the head is raised until the fontanelle is flat and the intracranial pressure is estimated by measuring the height of the fontanelle above the midclavicular line. The mean pressure in a group of infants seven months of age and younger was found to be 45 mm water with a standard deviation of f 12 mm. Before CT scanning was available, neuroimaging was often omitted in cases of infantile pseudotumor cerebri. Therefore, if one applies the traditional diagnostic criteria, the diagnosis of pseudotumor cerebri was not established in these pediatric patients.

III. Specific Etiologies A. INTRODUCTION Several investigators have studied groups of predominantly adult pseudotumor cerebri patients in attempts to identify causes of the disorder. Ireland et a15’performed a case-controlled, retrospective study of 40 women who were 16 years or older at diagnosis. Obesity and recent weight gain were the only factors that were found significantly more often in the pseudotumor cerebri patients than in the controls. Giusefli et a144conducted a case-controlled, prospective study of 50 pseudotumor cerebri patients including a few pediatric patients as young as 11 years. They also found that obesity and recent weight gain were the only factors studied that were significantly associated with pseudotumor cerebri. The authors of these reports are careful to mention possible shortcomings of their own work, such as small sample size and low response rate, and insufficient statistical power in the case of agents to which few individuals are exposed. Such studies might also fail to identify an etiologic agent if the disorder was an idiosyncratic reaction to a commonly used drug. For example, if one used such a case-controlled study to identify etiologies of anaphylaxis, one would be apt to miss penicillin, because the rates of exposure would be similar in the anaphylaxis and control patients. Since there are no case-controlled studies of etiology in pediatric pseudotumor cerebri patients, we must rely on anecdotal data. Nevertheless, detailed case-reports can provide valuable information about etiology. As Gittinger has notshould not share ed, “neuro-ophthalmologists the contempt many academics have for case reports, since much of what we know is the cumulative experience contained in many single

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cases.“43 What follows are discussions of some of the etiologies and associations of childhood pseudotumor cerebri. In a few, a cause-and-effect relationship seems to be valid. In certain others, to borrow a term from Scottish jurisprudence, the verdict is “not proved.” B. MASTOIDITIS

AND LATERAL SINUS

THROMBOSIS

The importance of otitis. media, mastoiditis, and lateral sinus thrombosis in childhood pseudotumor cerebri has long been recognized. Venous sinus thrombosis from mastoiditis accounted for fully 14 of the 48 pediatric pseudotumor cerebri cases in Greer’s series.4s The entity is so well known that it warrants no further discussion here. The incidence of mastoiditis has lessened in recent decades and cases of “otitic hydrocephalus” have declined accordingly. Cerebral venous sinus thrombosis likely plays a role in the pathogenesis of increased intracranial pressure in some of the other etiologies of pseudotumor cerebri that are discussed elsewhere in this review. C. ENDOCRINE

CONDITIONS

1. Hypoparathyroidism

Several cases of pseudotumor cerebri have been reported in children with hypoparathyroidism.67,87,‘05 One was in a g-week-old with idiopathic hypoparathyroidism. Another was in an adolescent who developed papilledema within three days of a thyroidectomy for carcinoma. In that case the pseudotumor cerebri may have resulted from surgical interruption of venous drainage in the neck, rather than from the metabolic “insult.” Hypocalcemia may also have been a factor in these cases. 2. Menarche In 1964 Greer reported ten instances of pseudotumor cerebri occurring around menarche.47 Several other reports followed.50*“o There is otherwise little about these cases that appears to be special. Menarche is, to say the least, a common event. Literally millions of girls reach this stage of sexual maturation each month and yet fewer than 20 cases of alleged menarchial or perimenarchial pseudotumor have been published. It is notable that none of the 20 female pseudotumor cerebri patients up to age 14 years, reported by Couch et al from Halifax, Nova Scotia, were perimenarchial. 26 I can accept the p ossibility that menarche might be a cofactor, but not that it is by

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itself capable of causing pseudotumor

cerebri.

3. Adrenal Corticosteroids Pediatric pseudotumor cerebri has been associated with both corticosteroid treatment and the cessation or reduction of treatment.3~4~20.3s.46’50’.55, 72~82~11g~122~128~14g Even topical (cutaneous) therapy has been implicated. However, there are far more cases consequent to cessation of treatment than there are from treatment (see below). It should be noted that some authorities are unconvinced by the available evidence that corticosteroid treatment causes pseudotumor cerebri. Dr. Michael Wall (written communication, October 26, 199 1) has been unable to find a satisfactorily documented case of pseudotumor cerebri in which corticosteroid treatment could have been the only etiology. In this regard, there are three pertinent pediatric reports.‘4*2g,“3 One described a 4-year-old with nephrotic syndrome (itself a possible cause or association of pseudotumor cerebri) who developed the intracranial hypertension six weeks after triamcinolone was substituted for an unstated dose of prednisolone.‘13 Wall invalidates that case because of an abnormal electroencephalogram, but it is also possible that the change in medications actually represented a reduction in the corticosteroid dose. He invalidates another case in a 2 10/l 2year-old, nephrotic girl on longterm, low-dose corticosteroid therapy who developed vomiting, irritability, papilledema and bilateral sixth nerve palsies, because the cerebrospinal fluid pressure and contents were not evaluated.14 Her symptoms and signs all cleared when the corticosteroids were withdrawn. Dees and McKayZg reported the case of a 2 9/12-year-old asthmatic boy who developed headache, vomiting, stiff neck, right sixth nerve palsy, Babinski reflexes, and a widened coronal suture after being on corticosteroids for 15 months. Papilledema and a third nerve palsy later developed. A ventriculogram gave normal results and the cerebrospinal fluid pressure was elevated, but had normal contents. He recovered completely. I find it difficult to consider this an example of anything other than pseudotumor cerebri consequent to corticosteroid therapy. The occurrence of pseudotumor cerebri does not seem to be a function of the disease for which the corticosteroid had been prescribed. Among these diseases have been pancytopenia, ichthyosis, adrenogenital syndrome, systemic lupus erythematosus, Hirschsprung’s disease, ulcerative colitis, eczema, infantile spasms, acute

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PEDIATRIC PSEUDOTUMOR CEREBRI hemiplegia, asthma, nephrosis, and rheumatoid arthritis. When pseudotumor cerebri supervenes during corticosteroid therapy, it is after a prolonged course of treatment (average 2% years in one early seriesllg); however, when it results from a reduction or cessation of corticosteroid therapy it can appear within days. In a report of three asthmatic children who developed increased intracranial pressure when steroids were reduced, all had meningismus, and two had Babinski reflexes.” One had 8 white cells/mm3 in his cerebrospinal fluid, which purists would consider incompatible with the diagnosis of pseudotumor cerebri. Pseudotumor cerebri has also been reported with congenital adrenal hyperplasia45 and after trans-sphenoidal adenomectomy for Cushing’s syndrome.iz4 4. Thyroid Replacement Sufficient examples of pseudotumor cerebri developing in children after initiation of thyroid replacement therapy have now been documented that no one should doubt the validity of the association.54~56*75*go~‘03~115~116 Pseudotumor cerebri has occurred in cases of hypothyroidism from end-organ failure and (in one case56) from hypothalamic hypothyroidism. The exact etiology of the hypothyroidism otherwise appears to be irrelevant. Intracranial pressure becomes elevated within weeks to months. In one case,75 a child was treated for hypothyroidism twice at an interval of three years. Each time that he was treated he developed pseudotumor cerebri! D. OBESITY Obesity is associated with, and probably contributes to the occurrence of or actually causes, pseudotumor cerebri in adults and adolescents.44’5g How obesity would raise the intracranial pressure remains to be satisfactorily explained. There have been so few instances of obesity-related pseudotumor cerebri in preadolescents that I doubt it is an important factor in this age group. E. DRUGS 1. Nalidixic Acid One of the drugs that has been implicated in childhood pseudotumor cerebri is the urinary tract antiseptic nalidixic acid (NegGram). 10~1g*2’,40, 66*7g*g1,‘12 Nalidixic acid is bactericidal to gramnegative microorganisms such as E. coli and Proteus that cause most urinary tract infections. Considering that this is not a “front-line” anti-

bacterial, there are a surprisingly large number of case-reports of pseudotumor cerebri ascribed to nalidixic acid in children. Cases have occurred with low doses for short periods, as well as with prolonged administration at high doses. The available anecdotal evidence would not indicate that there is a dose-response relationship. In one instance a 6-month-old had three separate attacks of pseudotumor cerebri, each within 24 hours of initiating therapy!” There is also an instance of pseudotumor cerebri in a 14-year-old consequent to treatment of cystic fibrosis with ciprofloxacin,‘*’ a drug whose chemical structure resembles that of nalidixic acid. Nalidixic acid is also used for bacillary dysentery. A report from India” described 12 cases of pseudotumor cerebri that developed in infants under the age of six months within 24-48 hours of starting loo-150 mg/kg/day (a rather high dose) for bacillary dysentery. In two of those cases, as well as in one Italian infant who developed pseudotumor cerebri while being treated for Salmonellosis,1’2 there were seizures. 2. Tetracycline Tetracyclines are primarily bacteriostatic antibiotics that came into wide use in the 1950s because of their efficacy against many bacteria, rickettsia, chlamydia, mycoplasma, and some spirochetes. With the passage of time, the use of these drugs has declined somewhat, partially because tetracyclines are deposited in developing bones and teeth which interdicts their use in children under the age of eight years. Minocycline represents an exception to the decline in tetracycline use. This semisynthetic drug is used by many adolescents to suppress acne. Minocycline may be more likely than other tetracyclines to influence intracranial pressure, because it has greater lipid solubility and more easily crosses the blood-brain barrier. Tetracycline is purported to cause pseudotumor cerebri in children.6,s,36,41,48,68,77,84,85,89.99 Nev_ ertheless, there have been few cases, considering the wide use of the drug. Even before 1969, when tetracyclines were more widely employed, especially in children, they were implicated in only 1/79,46 1/4S4* and 1/1S5’ in three series of pediatric pseudotumor cerebri cases. It is also possible that the intracranial hypertension in some of the cases ascribed to tetracyclines was the result of the infection for which the tetracycline was prescribed. However, most of the antibiotics that are used much more than the tetracyclines have never even been indicted.

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In one instance of alleged tetracycline-induced pseudotumor cerebri, a 5-month-old developed a bulging fontanelle five hours after a single 75 mg dose. I84 This is plausible from the point of view of cerebrospinal fluid rheology, since the cerebrospinal fluid volume is renewed every 5-7 hours. A report from India mentioned the cases of three infants 7 months of age or younger who developed pseudotumor cerebri 48-96 hours after starting tetracycline therapy.” As with nalidixic acid, pseudotumor cerebri can supervene immediately after the initiation of tetracycline therapy or only after the patient has been on the antibiotic for months. The occurrence of elevated intracranial pressure with tetracycline does not appear to be a dose-related phenomenon. In one instancea an infant developed intracranial hypertension twice, each time after treatment Unfortunately, there is no with tetracyclines. other clinical information about that case. 3. Vitamin A Vitamin A intoxication seems an established cause of pseudotumor cerebri in children.35,42*48, 70~74~78~80~88~100 Infants can allegedly develop signs of increased intracranial pressure within 12 hours of a single dose of vitamin A!76 This entity is particularly important in adolescents who may take vitamin A for acne. In many instances this represents self-m&cation. The intoxication in these individuals results from longterm, low-dose intake.s5 There are sometimes other signs of vitamin A intoxication, including nausea, fatigue, muscle aches, stiff neck, anorexia, lip fissuring and even hepatosplenomegaly. The serum glutamic oxaloacetic transaminase, as well as the vitamin A level, may be elevated. There are also reports of pseudotumor cerebri in adolescent girls who received the synthetic vitamin A derivative isoretinoin for acne.g5,104 F. NUTRITIONAL 1. Vitamin D Deficiency Fewer than 20 cases of pseudotumor cerebri associated with nutritional rickets in infants have been reported.30*52*53,67The child at special risk is the exclusively breast-fed child of a strict vegan mother, but nutritional rickets also occurs with generalized malnutrition. This form of pseudotumor cerebri resolves slowly. Since pseudotumor cerebri may occur from malnutrition or from hypocalcemia alone, it isn’t clear that the deficiency of vitamin D is responsible in those patients with nutritional rickets.

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2. Vitamin A Deficiency Deficiency of Vitamin A is also said to cause There are 11 repseudotumor cerebri. 25,63*65,g3 ported cases in infants between 3 and 8 months old and one in a 12-year-old girl who also had hyperthyroidism. The etiologies included dietary deficiency, cystic fibrosis, malabsorption, and biliary atresia. Seven had a keratopathy and three had facial paresis. It is highly unlikely that any of these infants were deficient only in vitamin A, and it is impossible to be confident it was the vitamin A deficiency that was responsible for the elevated intracranial pressure. 3. Malnutrition and Renutrition Pseudotumor cerebri has been recognized in malnourished children and immediately upon renourishing such children.7~1’~z6~3’~33~g2~10’ In~103 Couch et al’s series26 of 37 cases of pseudotumor cerebri up to age 14, there were five with “failure to thrive,” one additional “failure to thrive” on being re-fed, and four cystic fibrosis patients within four days of being re-fed. One theory holds that re-feeding causes raised intracranial pressure by stimulating brain growth at a rate faster than that of the cranium.” Tibbles et al”’ reported four cases of pseudotumor cerebri in children between ages 20 and 61 months with deprivation dwarfism and microcephaly. The possibility of dural venous sinus thrombosis has not been addressed as a possible pathogenesis of pseudotumor cerebri from inanition, and the pathogenesis of the intracranial hypertension remains unexplained. Whether this entity is related pathogenetically to the pseudotumor cerebri that occasionally supervenes in adults receiving parenteral alimentation is unknown. Dr. Joseph Vitale (personal communication, October 6, 1991) is aware of a group of malnourished infants in Africa who developed increased intracranial pressure upon being renourished. They had retained considerable hepatic stores of vitamin A that contributed to vitamin A intoxication when supplementary vitamin A was administered. Pseudotumor cerebri in children with cystic fibrosis has also been blamed on vitamin A deficiency (see above) and on an obstructive so it is not clear what factor or bronchopathy,& factors are responsible. G. HEADTRAUMA Closed head trauma can cause increased intracranial pressure and accounts for some cases in most large series of pediatric pseudotumor cerebri patients. One consecutive series’ of 23 cases of

161 childhood pseudotumor cerebri included five instances. In other series,46748 there were five head trauma patients among 48, and three among 79 patients, respectively. The trauma may be “minor.” Cerebral venous sinus thrombosis is the putative mechanism, but is not always documented. Closed head trauma can thrombose the sagittal sinus, torcula, sigmoid or lateral sinus.48 Greer4* believes that pseudotumor cerebri from traumatic sinus occlusion can be distinguished from traumatic cerebral edema by the normal mental state of the pseudotumor cerebri patient.

H. SYSTEMIC LUPUS ERYTHEMATOSUS Systemic lupus erythematosus is sometimes complicated by pseudotumor cerebri in adolescents and (rarely) in preadolescents prior to any corticosteroid therapy.32~62~‘02~‘22 In some cases the diagnosis of systemic lupus erythematosus is only made after the patient has presented with elevated intracranial pressure. Dural venous thrombosis or renal failure may be factors in some cases. As mentioned elsewhere, reduction or cessation of corticosteroid therapy can also precipitate pseudotumor cerebri in patients with systemic lupus erythematosus. I. MISCELLANEOUS There are a variety of other associations and putative etiologies that have been mentioned in the literature. Among these are Aldrich syndrome,4s iron deficiency,48 viral infection,26s50 radiation vasculitis,“j acute lymphoblastic leukemia,*” autoimmune hemolytic anemia,50 various causes of venous sinus obstruction,26*48 arteriovenous malformation,“’ polycythemia and thrombocytosis,‘23 Sydenham chorea,’ craniostenosis (or its treatment), ‘*N Turner syndrome,“‘j galactosemia,57 galactokinase deficiency,73 nitrofirrantoin,gs roseola infantum,s6 uremia4’ and obstructive uropathy,s3 after repair of an atria1 septal defect,16 histiocytosis X,‘j” hypophosphatasia,‘2,3g and Aarskog (facialdigital-genital) syndrome.‘14 In some of these cases the data are inadequate or there are findings that would invalidate the diagnosis of pseudotumor cerebri ~ensustrictu. Others may merely be coincidental associations with pediatric pseudotumor cerebri.

IV. Visual Implications A. TRANSIENT OBSCURATIONS Visual symptoms are highly prevalent in adults when they first present with pseudotumor cerebri. Transient obscurations of vision were re-

ported in 72% of 50 consecutive patients (age 11-58 years) studied prospectively by Wall and George. I*’ Corbett et alz3 had found an identical percentage when they retrospectively investigated adults and older adolescents. This is much higher than the 2-53% that Wall and George found in the literature in retrospective studies. Only retrospective studies of pediatric pseudotumor cerebri case-series are available, and one would expect them to show a low incidence of transient visual obscurations, if only because of the admixture of preverbal children. Weisberg and Chutorian noted that transient obscurations of vision occurred in only two of their 38 patients.‘** Baker et al4 mentioned it in only one of their 36 patients. Transient obscurations as such were not reported in any of Rose and Matson’s 23. cases,g4 Szenasy and Paraicz’s 11 cases,1og Grant’s 79 cases,46 Couch’s 38 cases,26 or Hagberg and Sillanpaa’s 18 cases.” In some of the series, transient visual obscurations may have been included under some more general heading, or the examiners may have failed to inquire. Amacher and Spence15 published a series of 23 pediatric pseudotumor cerebri cases, which included 12 who did not have papilledema in either eye. Surprisingly, all nine of their patients with transient obscurations of vision were in the group without papilledema! Until there has been a prospective study that includes questions about visual symptoms, it would be premature to conclude that few children with pseudotumor cerebri have transient visual obscurations. B. IMPAIRED VISUAL FUNCTION Impaired visual function is present in a surprisingly large proportion of adult pseudotumor cerebri patients at the time of diagnosis. In Wall and George’s prospective study of 50 patients (only seven of them in the 1O-l 9 year age range), 26% had noticed visual 10~s.‘~~At the initial examination, Wall and George found that 22% of their patients had reduced Snellen visual acuity and over 90% had visual field defects (including blind spot enlargement to within 10 degrees of fixation). They found that an average of 16% of previously reported cases of pseudotumor cerebri had reduced Snellen visual acuities and 13-49% had visual field defects. In the pediatric pseudotumor cerebri cases reported by Baker et a1,4 the Snellen acuity had become reduced in at least one eye at the time of presentation in 6 of 36 and five had visual field defects. patients, “Blurred vision” was a symptom in six of 12 patients ages four or older reported by Hagberg

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and Sillanpaa.50 Loss of acuity or visual field is the only serious permanent complication of pseudotumor cerebri. Corbett et al’s landmark contribution alerted physicians to the risk of permanent loss of visual function in adults with this disease.” They provided longterm follow-up on 57 of 118 patients whose findings strictly conformed to the criteria for idiopathic intracranial hypertension. Only 58 of 114 eyes were free of visual loss or field defect, and six patients were bilaterally blind! Wall and George ‘*O also found a high incidence of visual wastage in their prospectively-studied cases. There is only one comparable report about children with pseudotumor cerebri. Baker et al4 had ophthalmic follow-up data on 36 of 60 pediatric pseudotumor cerebri patients. Five patients were left with “moderate” abnormalities and a sixth had severe visual impairment. Several other reports have also documented visual loss in with pseudotumor cerepediatric patients bri 46,27,72,107

V. Evaluation and Management Corbett and Thompson24 have promulgated eminently sensible guidelines for the management of idiopathic intracranial hypertension in adults, which the reader would do well to consult. A. EVALUATION

The initial examination of the pediatric pseudotumor cerebri patient is not a “quickie.” Evaluation begins with a meticulous history, with every effort being made to identify a toxic, metabolic or nutritional etiology. In addition to the elementary neurological examination, each patient should undergo as complete an ophthalmic examination as is possible by a neuro-ophthalmologist or ophthalmologist. The examination of the child should not be delegated, even in part, to a technician. It requires a caring physician who is willing to devote adequate time to the task, and is capable of improvisation. Regardless of the child’s age or capacity to cooperate, the pupils, eye movements, and fundi must be evaluated. It is essential that the pupils be dilated for the fundus examination. Additional elements of the examination are added, as possible. A persistent examiner can usually measure acuity in a 3-year-old, evaluate color sense in a 4-year-old, and map the visual fields on a perimeter in a 5-year-old, provided the child is cooperative. I have found that the

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Goldmann perimeter is an excellent device for testing children in the 5-10 year range. If the child cannot maintain fixation or reliably signal his responses, you can employ his tendency to peek to your advantage. While observing that the child is fixating on the central target, flash the 14e stimulus in the periphery and note if the child looks accurately to the location of the flash. If he looks at the appropriate area, it proves that the stimulus was seen. If there are areas in which the child fails to respond, larger stimuli can be used. Meticulous, creative testing is admittedly time-consuming, but a patient examiner can thus construct an accurate representation of the patient’s visual field. Perimetry can easily be made into an enjoyable game for children of elementary school age, since the action is similar to that of some television games. Some children arrive at the office eager to “shoot down the space ships.” The evaluation of older children is the same as that of adults, and should include automated threshold static perimetry of the central 30 degrees. Whenever possible, stereoscopic photographs of the discs should be obtained at the first visit. These photographs not only provide a basis for subsequent comparison, but they also permit the ophthalmologist to examine the critical portions of the fundus at leisure. Visual evoked response testing does not have a role in the detection or monitoring of visual dysfunction in this disease.‘18 Neuroimaging of the head is the next step, preferably by magnetic resonance imaging but, at minimum, by computerized tomographic scanning. Once this has been done and the brain found to be normal, a lumbar puncture is performed. The cerebrospinal fluid pressure can and should be measured if the child is relaxed. Cells are counted and characterized immediately, and then the various chemical determinations and serological tests are performed. Apart from routine urinalysis and complete blood count, a serum vitamin A determination is probably the only other test that is warranted. Whatever else is done beyond this stage, regular, careful neuro-ophthalmic examinations are the irreducible minimum. The frequency of the examinations is individually determined for each case. Visual function, pupils and firndi require frequent evaluation (probably monthly) during the first half year after diagnosis, but the intervals can be lengthened thereafter. Once the papilledema abates, the risk ofvisual loss is no longer a concern. Follow-up lumbar punctures are not

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necessary and could even prove counterproductive. B. MANAGEMENT When a toxic, metabolic, or nutritional cause is identified, it must be treated by correction of the underlying problem. Unfortunately, such cases are in the minority. Whether, how, and when to treat the remaining pediatric pseudomotor cerebri patients are questions yet to be definitively answered. The available literature on pediatric pseudotumor cerebri provides no guidelines for therapy. In some instances the disorder is short-lived and self-limited, but other patients have persistently elevated intracranial pressure. If the patient is asymptomatic and visual function is normal, treatment is unnecessary. If headache is the only symptom, many patients can be managed with “standard prophylactic vascular headache have been remedies.“24 Various medications used in adults24~‘“0 and in children4 but drug efficacy has yet to be studied systematically in a controlled, prospective and masked fashion in any age group. Corticosteroids and acetazolamide are the drugs whose use is most widely reported. Either could be elected as the first drug, but the neurologist or pediatric neurologist rather than the ophthalmologist will have primary responsibility for initiating drug therapy. If vision is severely affected at the time of diagnosis, or if it fails despite drug therapy, surgery is indicated to preserve or even restore vision. Subtemporal craniectomy is passe. The current options are lumboperitoneal shunt and optic nerve sheath fenestration. There is now considerable evidence that the latter is the procedure of choice in adults.‘3,22,g7 Optic nerve sheath fenestration is safe, effective, and not apt to require revision. Unilateral surgery sometimes alleviates papilledema in both eyes and may even relieve headache! Billson and Hudson9 have employed optic nerve sheath decompression to treat pseudotumor cerebri in an 11 -year-old with a lateral sinus thrombosis. However, I have failed to find any other reports of optic nerve sheath fenestration for pseudotumor cerebri in children. Nevertheless, there is every reason to expect that this will prove to be the procedure of choice in the pediatric age group. A final word ofcaution. An occasional pediatric patient with pseudotumor cerebri will manifest factitious visual loss, sometimes superimposed upon organic visual impairment. Needless-to-

say, failure to recognize the factitious nature of the visual loss is apt to lead to inappropriate management. Physicians should be alert to the possibility of factitious visual loss in patients with pseudotumor cerebri regardless of age.

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Outline I. Nomenclature and definitions II. Clinical characteristics A. Sex ratio B. Initial symptoms C. Papilledema D. Other signs III. Specific etiologies A. Introduction and lateral sinus thrombosis B. Mastoiditis and lateral sinus thrombosis

LESSELL

1992 Endocrine conditions 1. Hypoparathyroidism 2. Menarche 3. Adrenal corticosteroids 4. Thyroid replacement D. Obesity E. Drugs 1. Nalidixic acid 2. Tetracycline 3. Vitamin A F. Nutritional 1. Vitamin D deficiency 2. Vitamin A deficiency 3. Malnutrition and renutrition G. Head trauma H. Systemic lupus erythematosus Miscellaneous I. IV. Visual implications A. Transient obscurations B. Impaired visual function V. Evaluation and management A. Evaluation B . Management C.

Reprint address: Boston, MA 02114.

Dr. Simmons Lessell, 243 Charles St.,