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Pseudotumor cerebri in children
Pseudotumor Cerebri in Children P a u l H. Phillips, MD," M i c h a e l X. R e p k a , M D , ~ S c o t t R. L a m b e r t , M D c~
Purpose:Demographic and outcome data in the era of modern neuroimaging are needed to describe pseudotumor cerebri in children. Methods: We reviewed the medical records of children less than 18 years old who were diagnosed with pseudotumor cerebri between 1977 and 1997. We defined pseudotumor cerebri as (1)increased intracranial pressure, (2) normal or small ventricles, and (3) normal cerebrospinal fluid composition. The condition might be idiopathic or the result of a nontumor etiology. ResMts:Thirty-seven patients had an initial diagnosis of pseudotumor cerebri. Two patients were subsequently diagnosed with a central nervous system malignancy and were excluded from further analysis. The remaining 35 patients included 10 patients with idiopathic pseudotumor cerebri and 25 patients with disorders reported to be associated with pseudotumor cerebri. The mean age was 10.6 years with a range of 3 to 17 years. Twenty patients (57%) were female and 13 patients (37%) were obese. At presentation 4 patients had a visual acuity less than 20/40 in the best eye and 10 patients had visual field deficits. Seventeen patients (49%) had cranial nerve deficits, all of which resolved with normalization of the intracranial pressure. Follow-up data were obtained on 30 patients. Only one patient had a final visual acuity less than 20/40 in the best eye, whereas six patients had residual visual field deficits. Ten patients (33%) had optic nerve atrophy. Conclusions:Therewas no gender predominance, and associated etiologic factors were common in these children with pseudotumor cerebri. Permanent visual loss occurs in some children with pseudotumor cerebri. Quantitative perimetry and optic nerve examination were more sensitive than visual acuity determination in detecting damage to the visual sensory system. In rare instances the patient diagnosed with pseudotumor cerebri will be found after extended follow-up to harbor an intracranial neoplasm. (J AAPOS 1998;2:33-8)
p
seudotumor cerebri is a syndrome characterized by increased intracranial pressure, normal or small ventricles, and normal cerebrospinal fluid composition. 1 The condition may be idiopathic or the result of a nontumor etiology. Pseudotumor cerebri most frequently occurs in obese women of childbearing age) However, there are many series of children with pseudommor cerebri. 3-18Most series of children with pseudotumor cerebri found no gender predominance and less frequent obesity compared with surveys of adult pseudommor cerebri. 3,4,6-is Unfortunately, most of these series 3,4,7L~4have provided minimal details regarding visual acuity and visual fields. Permanent visual impairment was infrequently described and thus considered to be a rare occurrence in children with pseudotumor cerebri. In addition, all these series3-~4 included patients managed before the advent of modern neuroimaging.
From the l;VilmerOphthalmologicMInstitute" and the Deparlmem of Pediatrics,~a%bus Hopkins University School of Medicine, Baltimore, Maryland, and the Department of Ophthalmology, Emory University School of Medicine,cAtlanta, Georgia. Presented at the Twenty-third Annual Meeting of the American Associatioufor Pediatric Ophthalmology and Strabismus, Charleston, South Carolina,April 2-6, 1997. Reprint requests: Michael X. Repka, MD, Fgilmer OphthalmologicInstitute, Strabismus and Pediatric Ophthalmology, l~ilmer B1-3Y, Johns Hopkins Hospital, Baltimore, MD 21287-9009. Copyright © 1998 by theAmericanAssociationfor PediatricOphthalmologyand Strabismus. 1091-8Y31/98 SY.O0 + 0 7~;/1/8Y16Y
Journal of AAPOS
The purpose of this study is to describe the clinical profile of pseudotumor cerebri in children who were evaluated with detailed ophthalmologic examinations in the era of "modern neuroimaging." METHODS We reviewed our database for the records of children younger than 18 years old who were diagnosed with pseudotumor cerebri at the Wilmer Ophthalmological Institute and the Emory Eye Center between 1977 and 1997. For purposes of this report we used the following three component definition of pseudotumor cerebri: (1) increased intracranial pressure (>200 mm H20 by lumbar puncture), (2) normal- or small-sized ventricles, and (3) normal cerebrospinal fluid composition. 1 We included idiopathic cases as well as cases associated with a nontumor etiology. All patients had neuroimaging consisting of computerized tomography (C AT) with contrast or magnetic resonance imaging (MRI) with gadolinium. All patients had elevated intracranial pressure (>200 mm H20 ) documented by lumbar puncture. Children were sedated when necessary to obtain an accurate intracranial pressure measurement. All patients had detailed examinations by the pediatric ophthalmology service or the neurophthalmology service. Visual acuities were obtained with best refractive correction with use of Snellen optotypes in literate patients and Allen symbols in preverbal patients. Quantitative perim-
February 1998 33
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ffournal ofAAPOS Volume 2 Number I February 1998
Phillips, Repka, and Lambert
TABLE 1. Disorders associated with pseudotumor cerebri*
12
Associated conditions
10-
No. of patients
Venous sinus thrombosis Ear infection Steroid taper Tetracycline/minocycline use Major head trauma Minor head trauma Cyclosporin use Recent weight loss Nephrotic syndrome Medroxyprogesterone acetate (Depo-Provera)use Lyme disease Viral illness Recent weight gain Renal failure Severe anemia Down syndrome Tuberous sclerosis Tyrosinemia Pyruvate kinase deficiency None identified
5 5 4 4 2 2 2 2 2 1 1 1 1 1 1 1 1 1 1 10
8 642-
03-5
12-14
15-17
FIG. !. Age distribution of study patients at presentation.
gaze. Cycloplegic refractions (cyclopentolate 1%) and dilated fundus examinations were performed on all patients. Body habitus was determined from subjective descriptions in the patient's medical record.
TABLE 2. Symptoms at presentation* Headache Nausea Decreased vision Vomiting Fatigue Diplopia Transient visual obscurations Arthralgia/myalgia Neck pain Strabismus noted by parents Photophobia Tinnitus Decreased appetite Vertigo Limb paresthesias Ataxia Irritability Asymptomatic
9-11 Yrs
*Associated disorders of 35 children are listed. Some children had more than one associated disorder.
Symptom
6-8
No. of patients
%
30 16 13 11 11 10 7 5 3 3 3 2 2 1 1 1 1 1
86 46 37 31 31 29 20 14 9 9 9 6 6 3 3 3 3 3
*Symptoms at presentation in 35 children. Some children had more than one symptom.
etry including kinetic visual fields or Humphrey Automated Threshold Visual Fields were obtained when possible. Goldmann Kinetic Visual Fields usually included the II4e and V4e isopters. Quantitative perimetry was interpreted only if the test was reliable according to the perimetrist and one of the investigators. Ocular motility was assessed by a certified orthoptist and one of the investigators, including cover-uncover tests in the diagnostic positions of
RESULTS A total of 37 patients were initially diagnosed with pseudotumor cerebri. Two of these patients subsequently diagnosed with a central nervous system malignancy were excluded from further analysis. Both these patients initially had a normal MR[ scan and cerebrospinal fluid composition, including cytologic studies. One of these patients had a glioblastoma multiforme and the other had a primitive neuroectodermal tumor. The 35 remaining patients included 10 patients with idiopathic pseudotumor cerebri and 25 patients with associated disorders at presentation that have previously been reported with pseudotumor cerebri. These associated conditions are listed in Table 1. Several patients had more than one associated disorder. There were no significant differences in demographic or outcome variables between the idiopathic pseudotumor cerebri patients and the patients with associated disorders. These two groups were therefore combined in the following data analysis. Figure 1 shows the age distribution of the study patients at presentation. The age range of the 35 study patients at presentation was 3 to 17 years, with a mean age of 10.6 years and a median age of 11 years. Twenty patients (57%) were female and 13 patients (37%) were obese. Seventeen patients were younger than 11 years old. Nine of these patients were female (53 %) and 4 were obese (24%). Among the 18 patients 11 to 18 years old, 11 were female (61%) and nine were obese (50%). The symptoms at presentation are listed in Table 2. Headache was the most common symptom, occurring in 30 (86%) patients. Ophthalmologic symptoms included decreased vision (13 patients), diplopia (10 patients), transient visual obscurations (7 patients), strabismus noted by the parents (3 patients), and photophobia (3 patients). Children younger than 11 years old more frequently were first
Journal ofAAPOS Volume 2 Number I February 1998
Phillips, Repka, and Lambert
45 40 35 30 # 25 Eyes 20 15 10 5 0
3Y
TABLE 3. Visual field deficits at presentation*
Visual field deficit
No. of eyes
Constriction/depression Nasal step or nasal depression Arcuate defect Temporal defect
15 3 3 2
*Visual fieldswereobtainedin 48 eyesof 24 patients.Nineteeneyesof 10 patients hadvisualfield deficits.Someeyeshad morethan onevisualfield deficit.
20/1520•25
20/3020•40
20/5020/100
<20/100
FIG. 2. Distribution of visual acuities of 62 eyes at presentation.
seen with fatigue (8 patients, 47%) compared with the older children (3 patients, 17%). The youngest child (3 years old) had asyrnptomatic papilledema noted on a routine examination. Thirty-three patients were seen within 5 months of symptom onset. One patient had headache for 2 years before presentation. Reliable initial visual acuities were obtained in 62 eyes of 31 patients. Figure 2 shows the distribution of visual acuities in each eye at presentation. Ten eyes (17%) of 6 patients (19%) had a visual acuity less than 20/40. Four of these patients (13 %) had a visual acuity less than 20/40 in both eyes. One patient had decreased visual acuity as a result of macula edema. The remaining patients had no macula abnormality to explain the decreased vision. Twenty-four patients had reliable quantitative perimetry at presentation. Eighteen patients had kinetic visual fields and 6 patients had automated static threshold visual fields. Ten (42%) of these 24 patients had visual field deficits other than enlarged blind spots. A total of 19 eyes in these 10 patients were affected. Some eyes had more than one visual field deficit. The deficits are listed in Table 3. Thirty-one patients had bilateral papilledema, 1 patient had unilateral papilledema, and 3 patients had normal discs at presentation. Fourteen patients had severe bilateral papilledema. Seven patients had peripapillary hemorrhages, 4 patients had fundus exudates, 2 patients had peripapillary cotton-wool spots, 1 patient had macula edema, and 1 patient had choroidal folds. Seventeen patients (49%) had cranial nerve deficits at presentation, all of which resolved with normalization of the intracranial pressure. The ocular motor deficit was the primary reason for presentation in 8 of these patients. The ocular misalignment was either noted by the parents or the patient complained of diplopia. The cranial nerve deficits are listed in Table 4. Abducens nerve palsies were the most frequent deficit. Three patients had unilateral trochlear nerve palsies. These three patients had a hypertropia that increased with adduction of the hypertropic eye and with head tilt toward the hypertropic eye. One patient had both a cranial nerve IV palsy and a cranial nerve VI palsy. One
TABLE 4. Cranial nerve deficits at presentation
Cranial nerve palsy Cranial Cranial Cranial Cranial
nerve Vl, bilateral nerve Vl, unilateral nerve IV nerve VII
No. of patients*
%
9 5 3 1
26 14 9 3
*Seventeenpatients had cranial nervedeficits at presentation.One patient had cranial nerveIV palsyand cranial nerveVl palsy. TABLE 5. Treatment of patients*
Treatment Observation Repeat lumbar puncture Steroids Diuretics LP shunt 0NSF
No. of patients 7 4 10 24 5 1
LP,,Lumboperitoneal;ONSF, optic nervesheathfenestration. *Treatmentof 35 patients is listed. Many patientswere treated with multiple therapeuticapproaches,
patient had upbeat nystagmus in upgaze that resolved with normalization of the intracranial pressure. Children younger than 11 years old more frequently had cranial nerve deficits (10 patients, 59%) compared with the older children (7 patients, 39%). Neuroimaging with contrast (MR[ or C AT) was normal or revealed small ventricles in all the study patients. Twenty patients (57%) were evaluated with MR[. The intracranial pressure was elevated in all patients, with a range of 220 to 800 mm H20. The patients were treated with a variety of therapeutic modalities, as noted in Table 5. Many patients were treated with multiple therapeutic modalities. Only two patients had recurrent episodes of papilledema. In one patient this was associated with thecoperitoneal shunt failure, and the recurrent papilledema resolved comp]etely with repair of the shunt. The other patient had comp]ete resolution of the recurrent papilledema after one lumbar puncture. The mean time of follow-up was 1.25 years with a range of 2 weeks to 4 years 8 months. Twenty-seven patients (77%) had resolved papilledema at their most recent examinations. Three patients (9%) continued to have papilledema 1 year after presentation. These 3
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# Eye
Journal ofAAPOS Volume 2 Number 1 February 1998
Phillips, Repka, and Lambert 45 40 35 30 25 20 15 10 5 0
TABLE 6. Visual field deficits--follow-up*
Visual field deficit Constriction/depression Nasal step or nasal depression Arcuate defect
No. of eyes 7 4 1
*Visual fields were obtained in 36 eyes of 18 patients. Ten eyes of six patients had visual field deficits. Someeyes had more than one visual field deficit.
20/1520•25
20•3020140
20•5020/100
<20/100
FIG. 3. Distribution of visual acuities of 56 eyes at most recent follow-up visit.
patients had stable vision documented on at least two examinations. The follow-up data were derived from these 30 patients (86%). There was insufficient follow-up of five patients who had continued disc edema and less than 1 year of follow-up. Three of these five patients had visual acuities better than 20/40 and no visual field deficits at presentation or during follow-up examinations. Two of these five patients were unable to perform reliable visual acuity measurements or quantitative perimetry. Reliable follow-up visual acuities were obtained in 56 eyes of 28 patients. These 28 patients were among the 31 patients who provided reliable initial visual acuities and include all 6 patients who had visual acuities of less than 20/40 in at least one eye. Figure 3 shows the distribution of visual acuities at the most recent examination. Vision had improved with follow-up because only two eyes in one patient had a visual acuity less than 20/40 in both eyes. This patient was a 16year-old obese woman who was first seen for headache and decreased vision. At presentation, she had a visual acuity of 20/50 in both eyes, bilateral visual field constriction, severe papilledema, and bilateral cranial nerve VI palsies. She was treated immediately with acetazolamide and had a lumbar peritoneal shunt 2 days after presentation. Her headaches and papilledema resolved. Three years later she had a visual acuity of 20/70 in both eyes with severe visual field constriction and optic nerve atrophy. Reliable follow-up quantitative perimetry was obtained in 18 patients. These 18 patients were among the 24 patients who performed reliable initial quantitative perimetry and included all 10 patients who had visual field deficits. Nine patients had kinetic visual fields and nine patients had automated static threshold visual fields. Six of these 18 patients (33 %) had residual visual field deficits. A total of 10 eyes in these six patients were affected. Some eyes had more than one visual field deficit. The deficits are listed in Table 6. All 10 eyes with residual visual field deficits had optic nerve atrophy. The most recent fundus examination revealed bilateral optic nerve atrophy in 8 patients (27%), unilateral
optic nerve atrophy in 2 patients (6%), bilateral papilledema in 3 patients (10%), and normal optic nerves in 17 patients (57%). Compared with the older children, the children younger than 11 years old had no significant differences in initial visual function, final visual function, or optic nerve appearance. None of the patients with dural sinus thrombosis or renal disease had a residual deficit in visual acuity or visual fields. DISCUSSION This study describes the clinical profile of pseudotumor cerebri in a group of children who had detailed ophthalmologic evaluation and modern neuroimaging including C AT or MRI. Pseudotumor cerebri should have no identifiable etiology, according to the modified Dandy criteria. 19 However, several investigators is, 2orecommend expanding the definition to include cases associated with a nontumor etiology. Indeed, previous series of children with pseudotumor cerebri report associated "etiologic" conditions in 26% to 74% of the cases. 3' 7-9,12-14Similarly, the series of Babikian et al.4 of children with "idiopathic intracranial hypertension" contains five patients with ear infections, five patients with febrile illnesses, two patients with head trauma, one patient with renal disease, and two patients with tetracycline use associated with the onset of disease. Ear infections are typically the most common association identified. < 9,12,13 In our series associated "etiologic" conditions were demonstrated in 20 patients (57%). Ear infections occurred in 5 patients and were associated with documented venous sinus thrombosis in 4 of these patients. Some of the associated conditions, such as venous sinus thrombosis and steroid withdrawal, have been convincingly implicated as an etiology of pseudotumor cerebri, is, 21 Other associated conditions such as viral illness at presentation, minor head trauma, and birth control pill use have not been convincingly shown to cause pseudotumor cerebri and are of questionable significance, ix Several reports 3, 7, 12, 13 suggest that younger children are more likely to have associated conditions. In contrast, our series did not demonstrate any relationship between age and frequency of associated conditions in children between 3 and 18 years old. However, the high frequency of associated factors in
Journal ofAAPOS Volume2 Number 1 February1998 children younger than 18 years old with pseudotumor cerebri is in marked contrast to adult pseudotumor patients, who are generally healthy except for obesity.22 The definition of the age range for "childhood pseudotumor cerebri" is arbitrary. A logical age range might include only prepubescent patients because hormonal changes occurring at puberty have been implicated in the pathogenesis of pseudotumor cerebri. However, most previous series of childhood pseudotumor cerebri include teenagers 3-9, 12, 14 and have an age distribution similar to that in our series. In our series we analyzed the entire cohort but also divided the group into children younger and older than 11 years. More than 90% of adults with pseudotumor cerebri are female. 2I However, previous reports of children with pseudotumor cerebri show no significant gender predominance because females comprise only 39% to 70% of the children in these series. 3,4,7-9,12,13Similarly, our series had no significant gender predominance, with females comprising only 57% of our patients. Baker et al. 6 and Weisberg and Chutorian 14noted that younger children are less likely to have a female gender predominance. A similar trend was demonstrated in our series because females comprised 53 % of the children younger than 11 years old compared with 61% of the children more than or equal to 11 years old. Although obesity occurs in more than 90% of adult pseudotumor cerebri patients, 21 obesity is infrequently described in children with pseudotumor cerebri. 3,s-9,12,13 Two exceptions are the series by Babikian et al.4 and Weisberg and Chutorian, 14which reported a 30% and a 50% incidence of obesity, respectively. The 37% incidence of obesity in our series is comparable to that in these two series of childhood pseudotumor cerebri. Children younger than 11 years old had a lower incidence of obesity (24% obese) compared with children more than or equal to 11 years old (50% obese). Hormonal changes at puberty may account for the higher incidence of obesity in older children and adults. Most children with pseudotumor cerebri have headache), 4,6-9,12-i4In our series headache was the most frequent presenting symptom. Ocular complaints included decreased vision, diplopia, transient visual obscurations, and photophobia. There was a relatively low incidence of transient visual obscurations in our series (20%) compared with the incidence reported in adults (68%). 22 This may reflect the inability of young children to verbalize this symptom. Children are frequently seen with nonspecific symptoms such as fatigue, irritability, ataxia, neck pain, or limb paresthesias) s, 23 M1 of these symptoms occurred in our series. Children younger than 11 years old more frequently had fatigue (47 %) compared with children more or equal to 11 years old (17%). Seventeen of our children (49%) had cranial nerve deficits at presentation, and in eight of these patients the deficit was the presenting symptom. Children younger
Phillips, Repka, and Lambert
37
than 11 years old more frequently had cranial nerve deficits (59%) compared with the older children in our series (39 %). Previous series report cranial nerve deficits in 19 % to 57% of children with pseudotumo r cerebri. 4,6-9,12-1sIn comparison, cranial nerve palsies appear to occur less frequently in adults with pseudommor cerebri, in whom the reported incidence of sixth nerve palsies at presentation is 10% to 20%. 21 The high incidence of ocular motor deficits in our series may reflect selection bias in children who are seen on a pediatric ophthalmology service. Alternatively, children may be less likely to complain of subjective symptoms, whereas strabismus may be observed by parents or teachers. In agreement with previous reports, is sixth nerve palsies were the most common cranial nerve deficits. Three children had fourth nerve palsies and one patient had a seventh nerve palsy. Several investigators have described fourth nerve palsies and seventh nerve palsies in children with pseudotumor cerebri. 6, is, 24-29 Baker and Buncic24 reported three young patients with pseudotumor cerebri with vertical ocular motility disturbances attributed to either a fourth nerve palsy or a skew deviation. Our three children with vertical ocular motility disturbances had incomitance and a head tilt test most consistent with a fourth nerve palsy, although a skew deviation cannot be excluded. None of our patients had evidence of a cranial nerve deficit before the onset of the disease and all our patients had complete resolution of the cranial nerve deficits with normalization ofintracranial pressure. Therefore pseudotumor cerebri is the likely cause of these cranial nerve deficits. Most previous series of children with pseudommor cerebri provided minimal details regarding visual acuity and quantitative perimetry. 3,4, 7-9,,2-14Permanent visual impairment was thought to be a rare occurrence in pediatric patients with pseudommor. None of the series before 1985 report more than one child with visual loss.9,12q4 Baker et al. s described 36 children with pseudotumor cerebri. This is the only previous series in which detailed data regarding visual function was reported. In contrast to prior reports, visual loss was not rare. Indeed, six patients (17%) had at least moderate permanent visual impairment. The importance of serial quantitative perimetry to detect this visual impairment was emphasized because only one patient had a permanent reduction in visual acuity. Reliable quantitative perimetry was obtained in children as young as 5 years old. The failure to perform quantitative perimetry in previous reports of children with pseudotumor cerebri may account for the low incidence of visual loss reported in these series. Lessell and Rosman 3° also reported permanent visual impairment in five children with pseudotumor cerebri. A significant number of children in our series had visual impairment. Six patients had a reduction in visual acuity and 10 patients had visual field deficits. One patient had
38
Phillips,Repka, and Lambert
macular edema to account for the reduced vision. T h e remaining patients p r e s u m a b l y had decreased vision as the result of an optic neuropathy, although subtle macular edema m a y have been undetected. Although only one child had a p e r m a n e n t reduction in visual acuity, six children (17 %) had p e r m a n e n t visual field deficits consistent with an optic neuropathy. Fundus examination d e m o n s t r a t e d residual optic nerve a t r o p h y in 10 patients. All six children with residual visual field deficits had optic nerve atrophy. T h e 17 % frequency of p e r m a n e n t visual i m p a i r m e n t in our series is similar to that in the series by Baker et al. s Quantitative p e r i m e t r y was necessary to d o c u m e n t m u c h of the visual impairment. Quantitative p e r i m e t r y m a y be difficult to interpret in children. All visual fields included in this series appeared reliable to the perimetrist and one of the investigators. F u r t h e r m o r e , there was optic nerve a t r o p h y in all six children with residual visual field deficits, supporting the interpretation that these deficits were n o t artifacts. Unfortunately, m a n y of our children were unable to p e r f o r m reliable perimetry. T h u s the true frequency of visual field deficits in children with p s e u d o t u m o r cerebri is likely greater than that demonstrated in this series. P s e u d o t u m o r cerebri patients with venous sinus t h r o m bosis or renal failure have been reported to have a p o o r visual outcome, s, 31T h i s was not d e m o n s t r a t e d in our series because none of our patients with sinus thrombosis or renal disease had a p e r m a n e n t visual deficit. O u r series d e m o n s t r a t e d that children with p s e u d o t u m o r cerebri have no g e n d e r p r e d o m i n a n c e and less f r e q u e n t o b e s i t y c o m p a r e d with adult p s e u d o t u m o r patients. Associated factors and cranial n e r v e deficits w e r e c o m m o n at p r e s e n t a t i o n . D a m a g e to the visual s y s t e m o c c u r r e d in a significant n u m b e r o f c h i l d r e n with p s e u d o t u m o r cerebri. Q u a n t i t a t i v e p e r i m e t r y and optic n e r v e e x a m i n a t i o n were m o r e sensitive t h a n visual acuity d e t e r m i n a t i o n in d e t e c t i n g d a m a g e to the visual s e n s o r y system. C a r e f u l follow-up, including q u a n t i t a tive p e r i m e t r y , is n e c e s s a r y to d e t e c t visual d y s f u n c t i o n and the rare m a s q u e r a d e s y n d r o m e s t h a t c o n t i n u e to o c c u r even in the era o f m o d e r n n e u r o i m a g i n g . References
1. Miller NR. Walsh and Hoyt's Clinical neuro-ophthalmology. 4th ed. Vol 1. Baltimore: Williams & Wilkins; 1983. 2. Durcan FJ, Corbett JJ, Wall M. The incidence of pseudotumor cerebri: population studies in Iowa and Louisiana. Arch Neurol 1988;45:875-7. 3. Amacher AL, Spence JD. Spectrum of benign intracranial hypertension in children and adolescents. Child Nerv Syst 1985;1:81-6. 4. BabildanP, CorbettJ, Bell W. Idiopathic intracranial hypertension in children: the Iowa experience. J Child Neurol 1994;9:144-9.
Journal of AAPOS Volume 2 Number 1 February 1998 5. Baker RS, Carter D, Hendrick EB, Btmcic JR. Visual loss in pseudotumor cerebri in childhood: a follow-up study. Arch Ophthahnol 1985;103:1681-6. 6. Baker RS, Baumann RJ, Buncic JR. Idiopathic intracranial hypertension (pseudotumor cerebri) in pediatric patients. Pediatr Neurol 1989;5:5-11. 7. Couch R, Camfield PR, Tibbles JAR. The changing picture of pseudommor cerebri in children. CanJ Neurol Sci 1985;12:48-50. 8. Dhiravibulya K, Ouvrier R, Johnston I, Procopis P, AntonyJ. Benign intracranial hypertension in childhood: a review of 23 patients. J Paediatr Child Health 1991;27:304-7. 9. Grant DN. Benign intracranial hypertension: a review of 79 cases in infancy and childhood. Arch Dis Child 1971;46:65 I- 5. 10. Greer M. Benign intracranial hypertension (pseudotumor cerebri). Pediatr Clin North Am 1967;14:819-30. 11. Greer M. Benign intracranial hypertension, IV: menarche. Neurology 1964;14:569-73. 12. Hagberg B, Sillanpaa M. Benign intracranial hypertension (pseudotumor cerebri): review and report of 18 cases. Acta Paediatr Scand 1970;59:328-39. 13. Rose A, Matson DD. Benign intracranial hypertension in children. Pediatrics 1967;39:227-37. 14. Weisberg LA, Chutorian AM. Pseudommor cerebri in children. Arch Pediatr Adolesc Med 1977;131:1243-8. 15. Lessell S. Pediatric pseudotumor cerebri (idiopathic intracranial hypertension). Surv Ophthalmol 1992;37:155-66. 16. Rush JA. Pseudotumor cerebri: clinical profile and visual outcome in 63 patients. Mayo Clin Proc 1980;55:541-6. 17. Wall M, George D. Idiopathic intracranial hypertension: a prospective study of 50 patients. Brain 1991;114:155-80. 18. Foley J. Benign forms of intracranial hypertension-"toxic" and "otitic" hydrocephalus. Brain 1955;78:1-41. 19. Smith JL. Whence pseudotumor cerebri? J Clin Neuro Ophthalmol 1985;5:55-6. 20. Johnston I, Hawke S, Hahnagyi M, Teo C. The pseudotumor syndrome: disorders of cerebrospinal fluid circulation causing intracranial hypertension without ventriculomegaly. Arch Neurol 1991;48:740-7. 21. Wall M. Idiopathic intracranial hypertension. Semin Ophthahnol 1995;10:251-9. 22. Giuseffi V, Wall M, Siegel PZ, Rojas PB. Symptoms and disease associations in idiopathic intracranial hypertension (pseudotumor cerebri): a case-control study. Neurology 1991;41.:239-44. 23. Murray RS, Tait VF, Thompson JA. Spinal and radicular pain in pseudotumor cerebri. Pediatr Neurol 1986;2:106-7. 24. Baker RS, Buncic JR. Vertical ocular motility disturbance in pseudommor cerebri. J Clin Neur Ophthahnol 1985;5:41-4. 25. Halpern JI, Gordon WH Jr. Trochlear nerve palsy as a false localizing sign. Ann Ophthahnol 1981;13:53-6. 26. Lee AG. Fourth nerve palsy in pseudotumor cerebri. Strabismus 1995;3:57-9. 27. Selky AK, Dobyns WB, Yee RE). Idiopathic intracranial hypertension and facial diplegia. Neurology 1994;44:357. 28. Silhnan JS, Eavey RD, Reardon EJ, Thornton AR. Metabolic facial paralysis in an infant. Arch Otolaryngol 1985;111:822-5. 29. Chutorian AM, Gold AP, Braun CW. Benign intracranial hypertension and Bell's palsy. N EnglJ Med 1977;296:1214-5. 30. Lessell S, Rosman NP. Permanent visual impairment in childhood pseudotumor cerebri. Arch Neurol 1986;43:801-4. 31. Guy J, Johnston PK, Corbett JJ, Day AL, Glaser JS. Treatment of visual loss in pseudotumor cerebri associated with uremia. Neurology 1990;40:28-32.
Purpose:Demographic and outcome data in the era of modern neuroimaging are needed to describe pseudotumor cerebri in children. Methods: We reviewed the medical records of children less than 18 years old who were diagnosed with pseudotumor cerebri between 1977 and 1997. We defined pseudotumor cerebri as (1)increased intracranial pressure, (2) normal or small ventricles, and (3) normal cerebrospinal fluid composition. The condition might be idiopathic or the result of a nontumor etiology. ResMts:Thirty-seven patients had an initial diagnosis of pseudotumor cerebri. Two patients were subsequently diagnosed with a central nervous system malignancy and were excluded from further analysis. The remaining 35 patients included 10 patients with idiopathic pseudotumor cerebri and 25 patients with disorders reported to be associated with pseudotumor cerebri. The mean age was 10.6 years with a range of 3 to 17 years. Twenty patients (57%) were female and 13 patients (37%) were obese. At presentation 4 patients had a visual acuity less than 20/40 in the best eye and 10 patients had visual field deficits. Seventeen patients (49%) had cranial nerve deficits, all of which resolved with normalization of the intracranial pressure. Follow-up data were obtained on 30 patients. Only one patient had a final visual acuity less than 20/40 in the best eye, whereas six patients had residual visual field deficits. Ten patients (33%) had optic nerve atrophy. Conclusions:Therewas no gender predominance, and associated etiologic factors were common in these children with pseudotumor cerebri. Permanent visual loss occurs in some children with pseudotumor cerebri. Quantitative perimetry and optic nerve examination were more sensitive than visual acuity determination in detecting damage to the visual sensory system. In rare instances the patient diagnosed with pseudotumor cerebri will be found after extended follow-up to harbor an intracranial neoplasm. (J AAPOS 1998;2:33-8)
p
seudotumor cerebri is a syndrome characterized by increased intracranial pressure, normal or small ventricles, and normal cerebrospinal fluid composition. 1 The condition may be idiopathic or the result of a nontumor etiology. Pseudotumor cerebri most frequently occurs in obese women of childbearing age) However, there are many series of children with pseudommor cerebri. 3-18Most series of children with pseudotumor cerebri found no gender predominance and less frequent obesity compared with surveys of adult pseudommor cerebri. 3,4,6-is Unfortunately, most of these series 3,4,7L~4have provided minimal details regarding visual acuity and visual fields. Permanent visual impairment was infrequently described and thus considered to be a rare occurrence in children with pseudotumor cerebri. In addition, all these series3-~4 included patients managed before the advent of modern neuroimaging.
From the l;VilmerOphthalmologicMInstitute" and the Deparlmem of Pediatrics,~a%bus Hopkins University School of Medicine, Baltimore, Maryland, and the Department of Ophthalmology, Emory University School of Medicine,cAtlanta, Georgia. Presented at the Twenty-third Annual Meeting of the American Associatioufor Pediatric Ophthalmology and Strabismus, Charleston, South Carolina,April 2-6, 1997. Reprint requests: Michael X. Repka, MD, Fgilmer OphthalmologicInstitute, Strabismus and Pediatric Ophthalmology, l~ilmer B1-3Y, Johns Hopkins Hospital, Baltimore, MD 21287-9009. Copyright © 1998 by theAmericanAssociationfor PediatricOphthalmologyand Strabismus. 1091-8Y31/98 SY.O0 + 0 7~;/1/8Y16Y
Journal of AAPOS
The purpose of this study is to describe the clinical profile of pseudotumor cerebri in children who were evaluated with detailed ophthalmologic examinations in the era of "modern neuroimaging." METHODS We reviewed our database for the records of children younger than 18 years old who were diagnosed with pseudotumor cerebri at the Wilmer Ophthalmological Institute and the Emory Eye Center between 1977 and 1997. For purposes of this report we used the following three component definition of pseudotumor cerebri: (1) increased intracranial pressure (>200 mm H20 by lumbar puncture), (2) normal- or small-sized ventricles, and (3) normal cerebrospinal fluid composition. 1 We included idiopathic cases as well as cases associated with a nontumor etiology. All patients had neuroimaging consisting of computerized tomography (C AT) with contrast or magnetic resonance imaging (MRI) with gadolinium. All patients had elevated intracranial pressure (>200 mm H20 ) documented by lumbar puncture. Children were sedated when necessary to obtain an accurate intracranial pressure measurement. All patients had detailed examinations by the pediatric ophthalmology service or the neurophthalmology service. Visual acuities were obtained with best refractive correction with use of Snellen optotypes in literate patients and Allen symbols in preverbal patients. Quantitative perim-
February 1998 33
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ffournal ofAAPOS Volume 2 Number I February 1998
Phillips, Repka, and Lambert
TABLE 1. Disorders associated with pseudotumor cerebri*
12
Associated conditions
10-
No. of patients
Venous sinus thrombosis Ear infection Steroid taper Tetracycline/minocycline use Major head trauma Minor head trauma Cyclosporin use Recent weight loss Nephrotic syndrome Medroxyprogesterone acetate (Depo-Provera)use Lyme disease Viral illness Recent weight gain Renal failure Severe anemia Down syndrome Tuberous sclerosis Tyrosinemia Pyruvate kinase deficiency None identified
5 5 4 4 2 2 2 2 2 1 1 1 1 1 1 1 1 1 1 10
8 642-
03-5
12-14
15-17
FIG. !. Age distribution of study patients at presentation.
gaze. Cycloplegic refractions (cyclopentolate 1%) and dilated fundus examinations were performed on all patients. Body habitus was determined from subjective descriptions in the patient's medical record.
TABLE 2. Symptoms at presentation* Headache Nausea Decreased vision Vomiting Fatigue Diplopia Transient visual obscurations Arthralgia/myalgia Neck pain Strabismus noted by parents Photophobia Tinnitus Decreased appetite Vertigo Limb paresthesias Ataxia Irritability Asymptomatic
9-11 Yrs
*Associated disorders of 35 children are listed. Some children had more than one associated disorder.
Symptom
6-8
No. of patients
%
30 16 13 11 11 10 7 5 3 3 3 2 2 1 1 1 1 1
86 46 37 31 31 29 20 14 9 9 9 6 6 3 3 3 3 3
*Symptoms at presentation in 35 children. Some children had more than one symptom.
etry including kinetic visual fields or Humphrey Automated Threshold Visual Fields were obtained when possible. Goldmann Kinetic Visual Fields usually included the II4e and V4e isopters. Quantitative perimetry was interpreted only if the test was reliable according to the perimetrist and one of the investigators. Ocular motility was assessed by a certified orthoptist and one of the investigators, including cover-uncover tests in the diagnostic positions of
RESULTS A total of 37 patients were initially diagnosed with pseudotumor cerebri. Two of these patients subsequently diagnosed with a central nervous system malignancy were excluded from further analysis. Both these patients initially had a normal MR[ scan and cerebrospinal fluid composition, including cytologic studies. One of these patients had a glioblastoma multiforme and the other had a primitive neuroectodermal tumor. The 35 remaining patients included 10 patients with idiopathic pseudotumor cerebri and 25 patients with associated disorders at presentation that have previously been reported with pseudotumor cerebri. These associated conditions are listed in Table 1. Several patients had more than one associated disorder. There were no significant differences in demographic or outcome variables between the idiopathic pseudotumor cerebri patients and the patients with associated disorders. These two groups were therefore combined in the following data analysis. Figure 1 shows the age distribution of the study patients at presentation. The age range of the 35 study patients at presentation was 3 to 17 years, with a mean age of 10.6 years and a median age of 11 years. Twenty patients (57%) were female and 13 patients (37%) were obese. Seventeen patients were younger than 11 years old. Nine of these patients were female (53 %) and 4 were obese (24%). Among the 18 patients 11 to 18 years old, 11 were female (61%) and nine were obese (50%). The symptoms at presentation are listed in Table 2. Headache was the most common symptom, occurring in 30 (86%) patients. Ophthalmologic symptoms included decreased vision (13 patients), diplopia (10 patients), transient visual obscurations (7 patients), strabismus noted by the parents (3 patients), and photophobia (3 patients). Children younger than 11 years old more frequently were first
Journal ofAAPOS Volume 2 Number I February 1998
Phillips, Repka, and Lambert
45 40 35 30 # 25 Eyes 20 15 10 5 0
3Y
TABLE 3. Visual field deficits at presentation*
Visual field deficit
No. of eyes
Constriction/depression Nasal step or nasal depression Arcuate defect Temporal defect
15 3 3 2
*Visual fieldswereobtainedin 48 eyesof 24 patients.Nineteeneyesof 10 patients hadvisualfield deficits.Someeyeshad morethan onevisualfield deficit.
20/1520•25
20/3020•40
20/5020/100
<20/100
FIG. 2. Distribution of visual acuities of 62 eyes at presentation.
seen with fatigue (8 patients, 47%) compared with the older children (3 patients, 17%). The youngest child (3 years old) had asyrnptomatic papilledema noted on a routine examination. Thirty-three patients were seen within 5 months of symptom onset. One patient had headache for 2 years before presentation. Reliable initial visual acuities were obtained in 62 eyes of 31 patients. Figure 2 shows the distribution of visual acuities in each eye at presentation. Ten eyes (17%) of 6 patients (19%) had a visual acuity less than 20/40. Four of these patients (13 %) had a visual acuity less than 20/40 in both eyes. One patient had decreased visual acuity as a result of macula edema. The remaining patients had no macula abnormality to explain the decreased vision. Twenty-four patients had reliable quantitative perimetry at presentation. Eighteen patients had kinetic visual fields and 6 patients had automated static threshold visual fields. Ten (42%) of these 24 patients had visual field deficits other than enlarged blind spots. A total of 19 eyes in these 10 patients were affected. Some eyes had more than one visual field deficit. The deficits are listed in Table 3. Thirty-one patients had bilateral papilledema, 1 patient had unilateral papilledema, and 3 patients had normal discs at presentation. Fourteen patients had severe bilateral papilledema. Seven patients had peripapillary hemorrhages, 4 patients had fundus exudates, 2 patients had peripapillary cotton-wool spots, 1 patient had macula edema, and 1 patient had choroidal folds. Seventeen patients (49%) had cranial nerve deficits at presentation, all of which resolved with normalization of the intracranial pressure. The ocular motor deficit was the primary reason for presentation in 8 of these patients. The ocular misalignment was either noted by the parents or the patient complained of diplopia. The cranial nerve deficits are listed in Table 4. Abducens nerve palsies were the most frequent deficit. Three patients had unilateral trochlear nerve palsies. These three patients had a hypertropia that increased with adduction of the hypertropic eye and with head tilt toward the hypertropic eye. One patient had both a cranial nerve IV palsy and a cranial nerve VI palsy. One
TABLE 4. Cranial nerve deficits at presentation
Cranial nerve palsy Cranial Cranial Cranial Cranial
nerve Vl, bilateral nerve Vl, unilateral nerve IV nerve VII
No. of patients*
%
9 5 3 1
26 14 9 3
*Seventeenpatients had cranial nervedeficits at presentation.One patient had cranial nerveIV palsyand cranial nerveVl palsy. TABLE 5. Treatment of patients*
Treatment Observation Repeat lumbar puncture Steroids Diuretics LP shunt 0NSF
No. of patients 7 4 10 24 5 1
LP,,Lumboperitoneal;ONSF, optic nervesheathfenestration. *Treatmentof 35 patients is listed. Many patientswere treated with multiple therapeuticapproaches,
patient had upbeat nystagmus in upgaze that resolved with normalization of the intracranial pressure. Children younger than 11 years old more frequently had cranial nerve deficits (10 patients, 59%) compared with the older children (7 patients, 39%). Neuroimaging with contrast (MR[ or C AT) was normal or revealed small ventricles in all the study patients. Twenty patients (57%) were evaluated with MR[. The intracranial pressure was elevated in all patients, with a range of 220 to 800 mm H20. The patients were treated with a variety of therapeutic modalities, as noted in Table 5. Many patients were treated with multiple therapeutic modalities. Only two patients had recurrent episodes of papilledema. In one patient this was associated with thecoperitoneal shunt failure, and the recurrent papilledema resolved comp]etely with repair of the shunt. The other patient had comp]ete resolution of the recurrent papilledema after one lumbar puncture. The mean time of follow-up was 1.25 years with a range of 2 weeks to 4 years 8 months. Twenty-seven patients (77%) had resolved papilledema at their most recent examinations. Three patients (9%) continued to have papilledema 1 year after presentation. These 3
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# Eye
Journal ofAAPOS Volume 2 Number 1 February 1998
Phillips, Repka, and Lambert 45 40 35 30 25 20 15 10 5 0
TABLE 6. Visual field deficits--follow-up*
Visual field deficit Constriction/depression Nasal step or nasal depression Arcuate defect
No. of eyes 7 4 1
*Visual fields were obtained in 36 eyes of 18 patients. Ten eyes of six patients had visual field deficits. Someeyes had more than one visual field deficit.
20/1520•25
20•3020140
20•5020/100
<20/100
FIG. 3. Distribution of visual acuities of 56 eyes at most recent follow-up visit.
patients had stable vision documented on at least two examinations. The follow-up data were derived from these 30 patients (86%). There was insufficient follow-up of five patients who had continued disc edema and less than 1 year of follow-up. Three of these five patients had visual acuities better than 20/40 and no visual field deficits at presentation or during follow-up examinations. Two of these five patients were unable to perform reliable visual acuity measurements or quantitative perimetry. Reliable follow-up visual acuities were obtained in 56 eyes of 28 patients. These 28 patients were among the 31 patients who provided reliable initial visual acuities and include all 6 patients who had visual acuities of less than 20/40 in at least one eye. Figure 3 shows the distribution of visual acuities at the most recent examination. Vision had improved with follow-up because only two eyes in one patient had a visual acuity less than 20/40 in both eyes. This patient was a 16year-old obese woman who was first seen for headache and decreased vision. At presentation, she had a visual acuity of 20/50 in both eyes, bilateral visual field constriction, severe papilledema, and bilateral cranial nerve VI palsies. She was treated immediately with acetazolamide and had a lumbar peritoneal shunt 2 days after presentation. Her headaches and papilledema resolved. Three years later she had a visual acuity of 20/70 in both eyes with severe visual field constriction and optic nerve atrophy. Reliable follow-up quantitative perimetry was obtained in 18 patients. These 18 patients were among the 24 patients who performed reliable initial quantitative perimetry and included all 10 patients who had visual field deficits. Nine patients had kinetic visual fields and nine patients had automated static threshold visual fields. Six of these 18 patients (33 %) had residual visual field deficits. A total of 10 eyes in these six patients were affected. Some eyes had more than one visual field deficit. The deficits are listed in Table 6. All 10 eyes with residual visual field deficits had optic nerve atrophy. The most recent fundus examination revealed bilateral optic nerve atrophy in 8 patients (27%), unilateral
optic nerve atrophy in 2 patients (6%), bilateral papilledema in 3 patients (10%), and normal optic nerves in 17 patients (57%). Compared with the older children, the children younger than 11 years old had no significant differences in initial visual function, final visual function, or optic nerve appearance. None of the patients with dural sinus thrombosis or renal disease had a residual deficit in visual acuity or visual fields. DISCUSSION This study describes the clinical profile of pseudotumor cerebri in a group of children who had detailed ophthalmologic evaluation and modern neuroimaging including C AT or MRI. Pseudotumor cerebri should have no identifiable etiology, according to the modified Dandy criteria. 19 However, several investigators is, 2orecommend expanding the definition to include cases associated with a nontumor etiology. Indeed, previous series of children with pseudotumor cerebri report associated "etiologic" conditions in 26% to 74% of the cases. 3' 7-9,12-14Similarly, the series of Babikian et al.4 of children with "idiopathic intracranial hypertension" contains five patients with ear infections, five patients with febrile illnesses, two patients with head trauma, one patient with renal disease, and two patients with tetracycline use associated with the onset of disease. Ear infections are typically the most common association identified. < 9,12,13 In our series associated "etiologic" conditions were demonstrated in 20 patients (57%). Ear infections occurred in 5 patients and were associated with documented venous sinus thrombosis in 4 of these patients. Some of the associated conditions, such as venous sinus thrombosis and steroid withdrawal, have been convincingly implicated as an etiology of pseudotumor cerebri, is, 21 Other associated conditions such as viral illness at presentation, minor head trauma, and birth control pill use have not been convincingly shown to cause pseudotumor cerebri and are of questionable significance, ix Several reports 3, 7, 12, 13 suggest that younger children are more likely to have associated conditions. In contrast, our series did not demonstrate any relationship between age and frequency of associated conditions in children between 3 and 18 years old. However, the high frequency of associated factors in
Journal ofAAPOS Volume2 Number 1 February1998 children younger than 18 years old with pseudotumor cerebri is in marked contrast to adult pseudotumor patients, who are generally healthy except for obesity.22 The definition of the age range for "childhood pseudotumor cerebri" is arbitrary. A logical age range might include only prepubescent patients because hormonal changes occurring at puberty have been implicated in the pathogenesis of pseudotumor cerebri. However, most previous series of childhood pseudotumor cerebri include teenagers 3-9, 12, 14 and have an age distribution similar to that in our series. In our series we analyzed the entire cohort but also divided the group into children younger and older than 11 years. More than 90% of adults with pseudotumor cerebri are female. 2I However, previous reports of children with pseudotumor cerebri show no significant gender predominance because females comprise only 39% to 70% of the children in these series. 3,4,7-9,12,13Similarly, our series had no significant gender predominance, with females comprising only 57% of our patients. Baker et al. 6 and Weisberg and Chutorian 14noted that younger children are less likely to have a female gender predominance. A similar trend was demonstrated in our series because females comprised 53 % of the children younger than 11 years old compared with 61% of the children more than or equal to 11 years old. Although obesity occurs in more than 90% of adult pseudotumor cerebri patients, 21 obesity is infrequently described in children with pseudotumor cerebri. 3,s-9,12,13 Two exceptions are the series by Babikian et al.4 and Weisberg and Chutorian, 14which reported a 30% and a 50% incidence of obesity, respectively. The 37% incidence of obesity in our series is comparable to that in these two series of childhood pseudotumor cerebri. Children younger than 11 years old had a lower incidence of obesity (24% obese) compared with children more than or equal to 11 years old (50% obese). Hormonal changes at puberty may account for the higher incidence of obesity in older children and adults. Most children with pseudotumor cerebri have headache), 4,6-9,12-i4In our series headache was the most frequent presenting symptom. Ocular complaints included decreased vision, diplopia, transient visual obscurations, and photophobia. There was a relatively low incidence of transient visual obscurations in our series (20%) compared with the incidence reported in adults (68%). 22 This may reflect the inability of young children to verbalize this symptom. Children are frequently seen with nonspecific symptoms such as fatigue, irritability, ataxia, neck pain, or limb paresthesias) s, 23 M1 of these symptoms occurred in our series. Children younger than 11 years old more frequently had fatigue (47 %) compared with children more or equal to 11 years old (17%). Seventeen of our children (49%) had cranial nerve deficits at presentation, and in eight of these patients the deficit was the presenting symptom. Children younger
Phillips, Repka, and Lambert
37
than 11 years old more frequently had cranial nerve deficits (59%) compared with the older children in our series (39 %). Previous series report cranial nerve deficits in 19 % to 57% of children with pseudotumo r cerebri. 4,6-9,12-1sIn comparison, cranial nerve palsies appear to occur less frequently in adults with pseudommor cerebri, in whom the reported incidence of sixth nerve palsies at presentation is 10% to 20%. 21 The high incidence of ocular motor deficits in our series may reflect selection bias in children who are seen on a pediatric ophthalmology service. Alternatively, children may be less likely to complain of subjective symptoms, whereas strabismus may be observed by parents or teachers. In agreement with previous reports, is sixth nerve palsies were the most common cranial nerve deficits. Three children had fourth nerve palsies and one patient had a seventh nerve palsy. Several investigators have described fourth nerve palsies and seventh nerve palsies in children with pseudotumor cerebri. 6, is, 24-29 Baker and Buncic24 reported three young patients with pseudotumor cerebri with vertical ocular motility disturbances attributed to either a fourth nerve palsy or a skew deviation. Our three children with vertical ocular motility disturbances had incomitance and a head tilt test most consistent with a fourth nerve palsy, although a skew deviation cannot be excluded. None of our patients had evidence of a cranial nerve deficit before the onset of the disease and all our patients had complete resolution of the cranial nerve deficits with normalization ofintracranial pressure. Therefore pseudotumor cerebri is the likely cause of these cranial nerve deficits. Most previous series of children with pseudommor cerebri provided minimal details regarding visual acuity and quantitative perimetry. 3,4, 7-9,,2-14Permanent visual impairment was thought to be a rare occurrence in pediatric patients with pseudommor. None of the series before 1985 report more than one child with visual loss.9,12q4 Baker et al. s described 36 children with pseudotumor cerebri. This is the only previous series in which detailed data regarding visual function was reported. In contrast to prior reports, visual loss was not rare. Indeed, six patients (17%) had at least moderate permanent visual impairment. The importance of serial quantitative perimetry to detect this visual impairment was emphasized because only one patient had a permanent reduction in visual acuity. Reliable quantitative perimetry was obtained in children as young as 5 years old. The failure to perform quantitative perimetry in previous reports of children with pseudotumor cerebri may account for the low incidence of visual loss reported in these series. Lessell and Rosman 3° also reported permanent visual impairment in five children with pseudotumor cerebri. A significant number of children in our series had visual impairment. Six patients had a reduction in visual acuity and 10 patients had visual field deficits. One patient had
38
Phillips,Repka, and Lambert
macular edema to account for the reduced vision. T h e remaining patients p r e s u m a b l y had decreased vision as the result of an optic neuropathy, although subtle macular edema m a y have been undetected. Although only one child had a p e r m a n e n t reduction in visual acuity, six children (17 %) had p e r m a n e n t visual field deficits consistent with an optic neuropathy. Fundus examination d e m o n s t r a t e d residual optic nerve a t r o p h y in 10 patients. All six children with residual visual field deficits had optic nerve atrophy. T h e 17 % frequency of p e r m a n e n t visual i m p a i r m e n t in our series is similar to that in the series by Baker et al. s Quantitative p e r i m e t r y was necessary to d o c u m e n t m u c h of the visual impairment. Quantitative p e r i m e t r y m a y be difficult to interpret in children. All visual fields included in this series appeared reliable to the perimetrist and one of the investigators. F u r t h e r m o r e , there was optic nerve a t r o p h y in all six children with residual visual field deficits, supporting the interpretation that these deficits were n o t artifacts. Unfortunately, m a n y of our children were unable to p e r f o r m reliable perimetry. T h u s the true frequency of visual field deficits in children with p s e u d o t u m o r cerebri is likely greater than that demonstrated in this series. P s e u d o t u m o r cerebri patients with venous sinus t h r o m bosis or renal failure have been reported to have a p o o r visual outcome, s, 31T h i s was not d e m o n s t r a t e d in our series because none of our patients with sinus thrombosis or renal disease had a p e r m a n e n t visual deficit. O u r series d e m o n s t r a t e d that children with p s e u d o t u m o r cerebri have no g e n d e r p r e d o m i n a n c e and less f r e q u e n t o b e s i t y c o m p a r e d with adult p s e u d o t u m o r patients. Associated factors and cranial n e r v e deficits w e r e c o m m o n at p r e s e n t a t i o n . D a m a g e to the visual s y s t e m o c c u r r e d in a significant n u m b e r o f c h i l d r e n with p s e u d o t u m o r cerebri. Q u a n t i t a t i v e p e r i m e t r y and optic n e r v e e x a m i n a t i o n were m o r e sensitive t h a n visual acuity d e t e r m i n a t i o n in d e t e c t i n g d a m a g e to the visual s e n s o r y system. C a r e f u l follow-up, including q u a n t i t a tive p e r i m e t r y , is n e c e s s a r y to d e t e c t visual d y s f u n c t i o n and the rare m a s q u e r a d e s y n d r o m e s t h a t c o n t i n u e to o c c u r even in the era o f m o d e r n n e u r o i m a g i n g . References
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Journal of AAPOS Volume 2 Number 1 February 1998 5. Baker RS, Carter D, Hendrick EB, Btmcic JR. Visual loss in pseudotumor cerebri in childhood: a follow-up study. Arch Ophthahnol 1985;103:1681-6. 6. Baker RS, Baumann RJ, Buncic JR. Idiopathic intracranial hypertension (pseudotumor cerebri) in pediatric patients. Pediatr Neurol 1989;5:5-11. 7. Couch R, Camfield PR, Tibbles JAR. The changing picture of pseudommor cerebri in children. CanJ Neurol Sci 1985;12:48-50. 8. Dhiravibulya K, Ouvrier R, Johnston I, Procopis P, AntonyJ. Benign intracranial hypertension in childhood: a review of 23 patients. J Paediatr Child Health 1991;27:304-7. 9. Grant DN. Benign intracranial hypertension: a review of 79 cases in infancy and childhood. Arch Dis Child 1971;46:65 I- 5. 10. Greer M. Benign intracranial hypertension (pseudotumor cerebri). Pediatr Clin North Am 1967;14:819-30. 11. Greer M. Benign intracranial hypertension, IV: menarche. Neurology 1964;14:569-73. 12. Hagberg B, Sillanpaa M. Benign intracranial hypertension (pseudotumor cerebri): review and report of 18 cases. Acta Paediatr Scand 1970;59:328-39. 13. Rose A, Matson DD. Benign intracranial hypertension in children. Pediatrics 1967;39:227-37. 14. Weisberg LA, Chutorian AM. Pseudommor cerebri in children. Arch Pediatr Adolesc Med 1977;131:1243-8. 15. Lessell S. Pediatric pseudotumor cerebri (idiopathic intracranial hypertension). Surv Ophthalmol 1992;37:155-66. 16. Rush JA. Pseudotumor cerebri: clinical profile and visual outcome in 63 patients. Mayo Clin Proc 1980;55:541-6. 17. Wall M, George D. Idiopathic intracranial hypertension: a prospective study of 50 patients. Brain 1991;114:155-80. 18. Foley J. Benign forms of intracranial hypertension-"toxic" and "otitic" hydrocephalus. Brain 1955;78:1-41. 19. Smith JL. Whence pseudotumor cerebri? J Clin Neuro Ophthalmol 1985;5:55-6. 20. Johnston I, Hawke S, Hahnagyi M, Teo C. The pseudotumor syndrome: disorders of cerebrospinal fluid circulation causing intracranial hypertension without ventriculomegaly. Arch Neurol 1991;48:740-7. 21. Wall M. Idiopathic intracranial hypertension. Semin Ophthahnol 1995;10:251-9. 22. Giuseffi V, Wall M, Siegel PZ, Rojas PB. Symptoms and disease associations in idiopathic intracranial hypertension (pseudotumor cerebri): a case-control study. Neurology 1991;41.:239-44. 23. Murray RS, Tait VF, Thompson JA. Spinal and radicular pain in pseudotumor cerebri. Pediatr Neurol 1986;2:106-7. 24. Baker RS, Buncic JR. Vertical ocular motility disturbance in pseudommor cerebri. J Clin Neur Ophthahnol 1985;5:41-4. 25. Halpern JI, Gordon WH Jr. Trochlear nerve palsy as a false localizing sign. Ann Ophthahnol 1981;13:53-6. 26. Lee AG. Fourth nerve palsy in pseudotumor cerebri. Strabismus 1995;3:57-9. 27. Selky AK, Dobyns WB, Yee RE). Idiopathic intracranial hypertension and facial diplegia. Neurology 1994;44:357. 28. Silhnan JS, Eavey RD, Reardon EJ, Thornton AR. Metabolic facial paralysis in an infant. Arch Otolaryngol 1985;111:822-5. 29. Chutorian AM, Gold AP, Braun CW. Benign intracranial hypertension and Bell's palsy. N EnglJ Med 1977;296:1214-5. 30. Lessell S, Rosman NP. Permanent visual impairment in childhood pseudotumor cerebri. Arch Neurol 1986;43:801-4. 31. Guy J, Johnston PK, Corbett JJ, Day AL, Glaser JS. Treatment of visual loss in pseudotumor cerebri associated with uremia. Neurology 1990;40:28-32.