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case records of the massachusetts general hospital

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n engl j med 361;24 nejm.org december 10, 2009 2367

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From the Departments of Neurology (S.D.B., K.S.K.), Neuro-Ophthalmology (M.J.D.), Neurosurgery (Z.W.), Radiology (W.A.C.), and Pathology (S.H.F.), Massa-chusetts General Hospital; and the De-partments of Neurology (S.D.B., K.S.K.), Neurology (M.J.D.), Surgery (Z.W.), Radi-ology (W.A.C.), and Pathology (S.H.F.), Harvard Medical School.

N Engl J Med 2009;361:2367-78.Copyright 2009 Massachusetts Medical Society.

Pr esen tation of C a se

A 16-year-old boy was seen in the pediatric neurology clinic of this hospital because of paroxysmal headaches and visual changes.

The patient had been well until approximately 4 months earlier, when severe head-aches began to occur up to three times per month, lasting 3 hours to 8 days (usu-ally 1 to 2 days), and improving with tablets containing a combination of acetamino-phen, aspirin, and caffeine. His pediatrician prescribed sumatriptan orally and by means of a nasal spray. Three days before this evaluation, his father brought him to the emergency department of this hospital because of a severe headache that did not respond to sumatriptan. Magnetic resonance imaging (MRI) of the brain after the administration of gadolinium was normal. His pain improved; he was dis-charged home and referred to the pediatric neurology clinic of this hospital.

On evaluation in the clinic, the patient described the headaches as pounding and rated the pain as 5 to 7 on a scale of 1 to 10, where 10 is most severe. They began in the back of the head and extended to the forehead; were associated with dizzi-ness, nausea, neck pain, and mosaic vision; and were typically relieved by sleep. He did not have difficulty sleeping, motion sickness, or abdominal pain. He reported no stress and could not identify triggers for the headaches. He had recently had sinusitis, for which he had taken amoxicillinclavulanic acid, fluticasone nasal spray, loratadine, and pseudoephedrine.

Birth and development had been normal. The patient had no known allergies. He was a good student and had been a competitive athlete but had curtailed physical activity because of the headaches. He lived with his family. He did not smoke, drink, or use illicit drugs. He had not traveled recently. His father had had a transient ische-mic attack at the age of 45 years and had headaches and hyperlipidemia; his mother had diabetes mellitus and was ill from complications of the disease; his brother was healthy. There was no family history of migraine.

On examination, the vital signs were normal. The pupils were round and reac-tive to light. The optic-disk margins were sharp, and the visual fields were normal. The neck was supple, and the remainder of the physical and detailed neurologic examination was normal. Administration of atenolol was begun. Five weeks later,

Case 38-2009: A 16-Year-Old Boy with Paroxysmal Headaches and Visual Changes

Steven D. Brass, M.D., M.P.H., Marc J. Dinkin, M.D., Ziv Williams, M.D., Kalpathy S. Krishnamoorthy, M.D., William A. Copen, M.D.,

and Stefanie H. Freeman, M.D.

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n engl j med 361;24 nejm.org december 10, 20092368

an electroencephalogram (EEG) revealed continu-ous focal slowing in the right hemisphere over the posterior regions, with no epileptic activity. Ad-ministration of valproic acid was begun.

During the next 6 months, the headaches gradually resolved, but 6 months later, they re-curred daily. The patient rated their severity as 8 to 9 on a scale of 1 to 10; they were preceded at times by a bubbling feeling in his neck and were as-sociated with nausea and vomiting. On several occasions, his body stiffened and then went limp, while his eyes remained open and staring; this was followed by recovery within 30 seconds. An EEG showed mild, diffuse background slowing and superimposed intermittent posterior slowing, with right-sided predominance and no seizure ac-tivity. The serum prolactin level was 25.6 ng per milliliter (reference range, 0.0 to 15.0). MRI of the brain was again normal. Magnetic resonance an-giography (MRA) of the head and neck and com-puted tomographic angiography (CTA) showed no abnormalities. Ophthalmologic ultrasonography was normal.

During the next 3 years, the patient was seen in the emergency department of this and other hospitals multiple times; he was repeatedly ad-mitted to the hospital for headaches associated with visual changes, nausea, and occasionally leg cramps. Therapy included intravenous fluids, nar-cotic analgesics, valproic acid, and ondansetron for the acute episodes; trials of atenolol, nortrip-tyline, amitriptyline, gabapentin, butalbital, cypro-heptadine, topiramate, and riboflavin for prophy-laxis; and other medications (ibuprofen, naproxen, zolmitriptan, frovatriptan, and sumatriptan tab-lets and nasal spray) for abortive therapy, which he used with variable adherence. He saw a psy-chologist and had some improvement with bio-feedback techniques.

When the patient was between 19 and 20 years of age, episodes of syncope, weakness, and diffi-culty standing occurred with the headaches, and the pain radiated to the neck and upper back. Re-peated physical, neurologic, and ophthalmologic examinations disclosed no abnormalities. At 20 years of age, he described photopsias (the sensa-tion of perceiving lights or colors) and partial loss of vision in the right peripheral visual field. On evaluation by an ophthalmologist, bilateral optic-disk edema and splinter hemorrhages were seen. In the emergency department of this hospital, examination revealed bilateral blurred optic-disk

margins. MRI, MRA, and magnetic resonance venography (MRV) of the brain were normal. A lumbar puncture was performed. The opening pressure was 11 cm of water (reference range, 6 to 20). Results of cerebrospinal fluid (CSF) analysis are shown in Table 1. Flow cytometry revealed normal lymphocytes. The symptoms improved, and 1 week later, neuro-ophthalmologic examina-tion at this hospital was normal. The complete blood count; serum levels of electrolytes, magne-sium, free thyroxine, thyrotropin, prolactin, insu-lin-like growth factor 1, norepinephrine, dopamine, epinephrine, normetanephrine, and metanephrine; and the level of 5-hydroxyindoleacetic acid in a timed urine specimen were normal.

When the patient was 20 years 10 months of age, low back pain and transient inability to move or respond occurred during a severe headache. In the emergency department, he was most comfort-able sitting forward with his head down. CT of the head showed no evidence of hemorrhage, and he declined lumbar puncture. Neurologic exami-nation, including funduscopic examination, was normal. Narcotic analgesia was given, with im-provement. MRI of the spine was ordered, but the patient returned to college without undergoing the study.

When the patient was 21 years of age, severe headaches associated with pain in the shoulder and low back, visual changes, and loss of con-sciousness occurred three times in 1 week (once with incontinence). He was admitted to the neu-rology service of this hospital for video EEG monitoring. On examination, there was bilateral optic-disk edema; the vital signs and the remain-der of the examination were normal. The com-plete blood count, serum electrolyte levels, and tests of liver and renal function were normal.

On the second hospital day, during a severe headache, the patient reported numbness in his groin and perineum and sensory loss down the back of his left leg and heel, which resolved in 10 minutes. Neurologic examination and MRI and MRV of the brain were normal. He remained in bed, crouched on his elbows and knees, moan-ing, until morphine was administered. EEG dur-ing the headache showed theta-range slowing of the background, most prominent over the tem-poral regions. On the third day, neuro-ophthal-mologic evaluation revealed elevated optic disks with blurred margins (more blurred on the left than the right), obscuration of the vessels at the



case records of the massachusetts gener al hospital


disk margins, and a small flame-shaped hemor-rhage at the superior nasal margin of the left optic disk.

On the fifth day, the patient again reported numbness in the left groin and leg; neurologic examination was normal. A lumbar puncture was performed. The opening pressure was 25 cm of water; results of CSF analysis are shown in Table 1. Cytopathological examination revealed no malig-nant cells, and flow cytometry showed normal lymphocytes. Two hours after the procedure, the patient felt constipated; he was able to defecate but

reported a lack of rectal sensation. On examina-tion, there was decreased sensation in the area of the dermatomes of sacral nerves S1 through S4 on the left and decreased perianal sensation and rec-tal tone; strength in the legs was normal, but the left patellar and ankle reflexes were diminished.

A diagnostic procedure was performed.

Differ en ti a l Di agnosis

Dr. Kalpathy S. Krishnamoorthy: On initial evaluation, when he was 16 years of age, and on several follow-

Table 1. Cerebrospinal Fluid Analysis.

VariableReference Range,

Adults* Age of Patient

20 Yr 1 Mo21 Yr 1 Mo,

5th Hospital Day

Color Colorless Colorless Colorless

Turbidity Clear Clear Clear

Xanthochromia None None None

Red cells (per mm3)

Tube 1 0 1667 202

Tube 4 0 1244 92

White cells (per mm3)

Tube 1 05 3 1

Tube 4 05 2 0

Differential count (%)

Neutrophils 0 28 0

Lymphocytes 0 68 72

Monocytes 0 4 28

Protein (mg/dl) 555 47 32

Glucose (mg/dl) 5075 73 69

Venereal Disease Research Laboratory test Nonreactive Nonreactive Nonreactive

Grams stain No organisms No organisms No organisms

Acid-fast bacilli smear No organisms No organisms No organisms

Fungal wet prep No organisms Test not performed No organisms

Cultures

Routine No growth No growth No growth

Fungal No growth No growth No growth

Mycobacterial No growth No growth No growth

Cryptococcal antigen Negative Negative Negative

Herpes simplex virus (DNA amplification) Negative Test not performed, white-cell count and protein too low

Test not performed, white-cell count and protein too low

* Reference values are affected by many variables, including the patient population and the laboratory methods used. The ranges used at Massachusetts General Hospital are for adults who are not pregnant and do not have medical condi-tions that could affect the results. They may therefore not be appropriate for all patients.

To convert the values for glucose to millimoles per liter, multiply by 0.05551.





up evaluations until he was 19.5 years of age, the patient had a normal neurologic examination, in-cluding the funduscopic examination. He was in-telligent, articulate, and seemed to be a reliable historian. At the time of his first visit, I requested MRI to rule out mass lesions, hydrocephalus, vas-cular malformations, and type I Chiari malfor-mation, none of which were detected. He met all the criteria described by the International Head-ache Society for migraine without aura, and some of the criteria for migraine with aura, including recurrent attacks of bilateral or unilateral throb-bing headaches, with visual aura, lasting for hours and sometimes days, often associated with nau-sea, vomiting, and photophobia. Migraine is one of the common causes of headaches in teenagers, with a prevalence of 4 to 11% among teenagers who have headaches.1 The onset in boys is usu-ally before puberty, and in girls it is more com-mon at or after puberty, but the age of onset is not helpful in diagnosis. After an initial response to valproic acid, this patient had multiple attacks that were resistant to all prophylactic and rescue migraine medications. I considered transformed migraine (also known as chronic migraine) and mixed headaches.1 In both conditions, progressive and recurrent migrainous episodes are superim-posed on chronic daily headaches. Many patients have substantial psychosocial stress. Approximate-ly 18% of teenagers with migraines have mixed headaches.1

Over time, unusual symptoms developed, in-cluding transient loss of consciousness, weakness, obtundation, and lethargy. Some of these features suggested basilar migraines, which affect the brain stem, cerebellum, and parieto-occipital re-gions. Symptoms of basilar migraines include diplopia, ataxia, vertigo, motor deficits (e.g., hemi-paresis and quadriparesis), syncope, vomiting, lethargy, and obtundation. Some basilar migraines may mimic strokes or seizures. None of the defi-cits lasted long enough to make this diagnosis.

As the patients headaches became intractable, psychosocial stresses were also considered among the causes. He had started college and was leav-ing home. His mother had a chronic illness, and sometimes he seemed depressed and anxious. He was going to emergency rooms and requesting morphine, and he had no satisfactory response to therapy with antimigraine drugs. However, a psychiatrist did not find evidence of depression or other psychiatric illness. At one admission, when he presented with vomiting, flushing, and

sweating, I considered catecholamine-releasing tumors or a carcinoid tumor, but the workup was negative.

My working diagnosis was intractable mi-graine. The patient was transferred to the adult neurology service at 19.5 years of age.

Dr. Steven D. Brass: This now 21-year-old man had recurrent paroxysmal headaches with episodic loss of consciousness and intermittent bilateral op-tic-disk edema. Neuroimaging of the brain and head and neck vasculature on several occasions did not reveal a mass, an aneurysm, or central venous thrombosis. An EEG showed no epilepti-form activity. We requested consultation with neuro-ophthalmology to assess whether his op-tic-disk abnormality was in fact papilledema.

Dr. Marc J. Dinkin: The differential diagnosis of optic-disk edema, when it is referred to as a papil-litis, includes ischemia, compression, and infiltra-tion and inflammation of the anterior optic nerve. Such cases are characterized by decreased visual acuity, dyschromatopsia, an afferent pupillary de-fect, and arcuate, central, or centrocecal scotomas, whereas transient visual obscurations are rare. In-creased intracranial pressure tends to cause bi-lateral optic-disk edema, called papilledema, and papilledema may result from stasis of axoplasmic transport down the nerves, secondary to increased intracranial pressure.2 The visual symptoms and signs of papilledema differ from those of other types of optic-disk edema in that acuity and color vision are typically preserved until the late stages, as they were in this case. The preservation of acu-ity, color vision, central field, and papillary func-tion all result from the relative sparing of the maculopapillary bundle (axons leading from cen-tral macular ganglion cells to the optic nerve) in early papilledema. Transient visual obscurations, which our patient had, are also more common with papilledema, occurring in 68% of patients,3 and may result from transient ische mia to the nerve in the context of high intracranial pressure and changes in position.4 The temporal scotoma in his right eye that we observed reflected the en-larged physiological blind spot of papilledema, which results from the presence of peripapillary fluid between the retina and retinal pigment epi-thelium.5,6

When the patient was first seen by our neuro-ophthalmology service, disk edema had been observed a week earlier; however, his optic disks were normal on our examination. A lumbar punc-ture performed between the examinations showed





a normal opening pressure. A year later, when he was seen closer to the time of a headache, his optic nerves showed many signs of papilledema, includ-ing elevation of the edge of the disks, venous dis-tention, and one splinter hemorrhage (Fig. 1A). A subsequent lumbar puncture showed increased intracranial pressure.

The finding of papilledema helped identify in-creased intracranial pressure as the cause of the patients symptoms, but its sporadic nature led to a delay in the diagnosis. In experiments in non-human primates, papilledema developed as early as 24 hours after an increase in intracranial pres-sure7; in studies in humans, papilledema typically develops only after several days of elevated intra-cranial pressure.8 Papilledema may resolve with-in 2 weeks after abrupt normalization of intrac-ranial pressure,3,9 whereas in medically treated patients with idiopathic intracranial hypertension, it resolves at an average of 5 months.10 Thus, the fleeting nature of the papilledema in this patient suggested the presence of paroxysmal, short-lived elevations in intracranial pressure.

Dr. Brass: Of the many disorders that can pre-sent with headache and papilledema,11,12 I focused my differential diagnosis on idiopathic intracra-nial hypertension, vascular causes, infectious dis-eases, and a central nervous system neoplasm as the most likely possibilities.

Idiopathic Intracranial Hypertension

Idiopathic intracranial hypertension was initially the leading diagnosis. This condition typically occurs in overweight adolescent girls and young women.11-13 An unremitting headache with epi-sodic worsening is accompanied by pulsatile tin-nitus, transient visual obscuration, and diplopia. Visual acuity is preserved until late in the course of the disease. Examination often shows bilateral disk edema. The diagnostic criteria include a nor-mal cranial MRI and MRV and an opening pres-sure on lumbar puncture greater than 25 cm of water, with a normal CSF composition.11 Most of the reported neuroimaging signs on cross-sectional imaging are not helpful in establishing or ruling out this diagnosis.14 Treatment includes weight reduction, lowering the intracranial pressure with acetazolamide, and surgical treatment with either fenestration of the optic-nerve sheath or lumbar-to-peritoneal shunting.11-13 Because of a high sus-picion for this diagnosis, I started treatment with acetazolamide after the lumbar puncture. How-

ever, arguing against this diagnosis was the fact that his headaches were paroxysmal, as was his disk edema, unlike the chronic findings reported in cases of this condition. In addition, he was thin and male, features atypical for this condition.

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Figure 1. Funduscopic Examination.

On funduscopic examination during the current admission (Panel A), there is hyperemia of the nasal disks and blurring of the nasal margins, as well as a splinter hemorrhage. Examination 3 months after the first operation, at the time of recurrent visual symptoms (Panel B), discloses persistent ve-nous dilatation (arrowhead), a tortuous artery (white arrow), and severe 360-degree swelling of the optic-nerve head (black arrow), features consis-tent with worsening papilledema.





Vascular causes

Cerebral venous thrombosis may present with an acute onset or chronic progressive headache, papilledema, transient visual symptoms, seizures, focal neurologic signs, or subacute encephalopa-thy, depending on the location and extent of the thrombus.15,16 MRI will show evidence of infarct, hemorrhage, or thrombosis, and there may be a lack of signal on MRV.17 Our patients presenta-

tion of paroxysmal headache and papilledema, with completely asymptomatic intervals and nor-mal examinations between episodes, and normal MRV ruled out this diagnosis.

Meningitis

Recurrent meningitis may cause intermittent men-ingeal symptoms and CSF abnormalities, followed by periods of normal clinical and CSF findings.18-20

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Figure 2. Images of the Spine.

Sagittal T2-weighted (Panel A) and T1-weighted (Panel B) MRIs show a cystic-appearing mass lesion that nearly fills the sacral canal, compressing the sacral nerve roots. An axial T2-weighted image of the sacrum (Panel C) shows a bulbous component of the sacral mass protruding through the left S2S3 anterior sacral foramen. The urinary blad-der is distended. Reformatted images from a CT myelogram (Panel D) show an absence of visible injected contrast material in most of the lesion, confirming that there is no wide-open communication with the thecal sac, but a small quantity of contrast material seen in the bulbous sacral portion of the cyst (Panel E, arrow) confirms that some communication is present. Surface rendering produced from CT myelogram images (Panel F) shows spinal dysraphism with congenital nonfusion of the posterior sacral elements (arrows).





Patients may have recurrent headache, fever, nau-sea, change in mental status, nuchal rigidity, sei-zures, focal neurologic findings, cranial-nerve pal-sies, and papilledema.21 Examination of the spinal f luid reveals an elevated opening pressure with pleocytosis, normal or elevated protein levels, and a variable glucose level, depending on the cause. MRI may show meningeal enhancement. Our pa-tient had no fever or meningismus, repeated neu-roimaging did not show meningeal enhancement, and repeated CSF examinations did not support this diagnosis.18-20

Central Nervous System Neoplasm

Brain tumors may present with headaches, and headaches occur in 60% of patients who have brain tumors.22,23 The headaches are usually worse dur-ing a Valsalva maneuver and on wakening, prob-ably because of increases in intracranial pressure. Headaches may be migraine or tensionlike and may be accompanied by papilledema, focal neu-rologic findings, nausea, or seizures. When head-aches are paroxysmal and associated with vomit-ing, visual symptoms, drop attacks, and loss of consciousness, one must consider that a neoplasm, such as colloid cyst of the third ventricle, may be causing intermittent hydrocephalus.24 Other in-traventricular or periventricular tumors, such as pinealoma, craniopharyngioma, and ependymoma, should also be considered.25 In this patient, mul-tiple cranial MRIs ruled out an intracranial tumor.

Patients with spinal cord tumors such as ependymomas and neurofibromas may on rare occasions present with headache and papillede-ma as the initial symptoms, although typically when headache and papilledema develop in a pa-tient with a spinal-cord neoplasm, spinal symp-toms have already evolved.26-31 It has been sug-gested that a spinal cord tumor may lead to mechanical and physiological changes in both CSF absorption and flow, causing a rise in intracra-nial pressure.26-31 Our patients spinal symptoms were not initially prominent, and his normal ex-amination indicated that a spinal tumor was un-likely. However, during the year before this ad-mission, low back pain developed; during this admission, he described numbness of the left groin and leg, and several hours after the lumbar punc-ture, neurologic deficits indicating compression of the spinal cord or thecal sac developed, suggest-ing the presence of an intraspinal mass. Imaging studies were obtained.

Dr. William A. Copen: T2-weighted MRI of the lum-

bar spine (Fig. 2A) reveals a hyperintense extra-dural mass that nearly fills the sacral canal, mark-edly compressing the sacral-nerve roots. The mass is markedly hypointense on T1-weighted MRI and has no enhancing components (Fig. 2B), suggest-ing a cyst. Expansion of the sacral canal, with deep scalloping of the bodies of the sacral segments, indicates a chronic, gradually expanding cyst. A bulb-shaped component extends through and expands the left S2S3 anterior sacral foramen (Fig. 2C), suggesting a perineural cyst arising from the sheath of the left S2 nerve root.

A sagittal reformatted image from a CT myelo-gram (Fig. 2D) shows injected contrast material in the subdural space and the thecal sac but not within the cyst, indicating that the wide-open communication between the thecal sac and the cyst that would be expected in a meningocele or pseudomeningocele is missing. However, a left parasagittal image (Fig. 2E) shows contrast lay-

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Figure 3. Biopsy Specimen of the Cyst Wall.

There is a fibrous connective-tissue wall with a thin, flat-tened arachnoid cell lining and an arachnoid granulation (Panel A, hematoxylin and eosin). Immunohistochemi-cal staining for epithelial membrane antigen highlights the thin epithelial lining of the cyst (Panel B, immu-noperoxidase stain for epithelial membrane antigen).





Figure 4 (facing page). Follow-up Imaging Studies.

Sagittal T2-weighted MRI of the lumbar spine, obtained 1 month after spinal surgery (Panel A), shows that mass effect on the sacral nerve roots has increased be-cause of a fluid collection in the operative bed that may indicate cyst reaccumulation or pseudomeningo-cele. Two and a half months after the operation (Panel B), the mass effect has resolved. An axial T2-weighted image through the orbits (Panel C), obtained 3 months after surgery, when the patient was having visual symp-toms, shows expansion of the optic-nerve sheaths (short arrows), kinking of the optic nerves (long ar-row), and concavity of the globe at the insertion of the right optic nerve (arrowhead), features that indicate in-creased intracranial pressure.

ering in the portion of the cyst within the left S2S3 anterior sacral foramen, indicating some communication between the cyst and the thecal sac and suggesting that the cyst may have arisen from the nerve-root sheath at this level. However, enlargement of a perineural cyst to this size with-in the sacral canal would be unusual. A surface rendering of the CT myelogram images (Fig. 2F) shows nonfusion of the sacral posterior elements, indicating occult congenital spinal dysraphism. This abnormality has been reported in association with spinal arachnoid cysts, making this the most likely diagnosis in this case.32,33

Dr. Ziv Williams: This patient presented with acute cauda equina syndrome due to compression of the descending lumbosacral nerve roots by the cyst. Decompression within 48 hours after symp-tom onset was necessary to avoid a permanent neurologic deficit.34 However, the patient also had chronic symptoms associated with elevated intra-cranial pressure. Two approaches were considered. Fenestration would provide direct decompression of the cyst and immediately relieve pressure on the nerve roots but might not relieve the elevated intracranial pressure. Diversion of the CSF by means of a shunt might decrease the intracra-nial pressure. The CT myelogram showed a small amount of contrast material within the cyst, but it was uncertain whether f luid communicated freely between the subarachnoid space and the entire cyst. If communication between the cyst contents and the subarachnoid space was incom-plete or inconsistent, shunting might not pro-vide adequate decompression.35

We elected to perform cyst fenestration, with the option of performing a diversionary proce-dure for the CSF at a later date, if elevated intra-cranial pressure persisted. By decompressing the cyst into the subarachnoid space, the pressure gradient between the two compartments is equil-ibrated and the pressure on the nerves is relieved. Either complete excision or wide fenestration with or without placement of a shunt into the sub-arachnoid space or peritoneum is recommended to limit the possibility of recurrence.36

In the operating room, a midline incision was made over the lumbosacral area, and the L4 through S2 lamina were removed. An intraspinal, extradural cyst protruded through a large dorsal dural defect extending from the L5 to S1 levels. The cyst was gray and translucent, consistent in appearance with an arachnoid cyst. Microdissec-tion was performed intradurally through the de-

fect, and the cyst wall was widely removed. The descending nerve roots appeared to be displaced ventrolaterally and did not traverse the cyst cavity. Some of the cyst that was adherent to the nerve roots was left behind. A lumbosacral fascial graft was used to close the dural defect.

Clinic a l Di agnosis

Spinal arachnoid cyst.

Pathol o gic a l Discussion

Dr. Stefanie H. Freeman: A portion of the cyst wall was submitted for histopathological examination. It is composed of fibrous connective tissue with a thin lining composed of flattened epithelial cells, con-sistent with an arachnoid cyst (Fig. 3A). Immu-nohistochemical staining for epithelial membrane antigen highlights the thin epithelial lining of the cyst (Fig. 3B). The differential diagnosis of spinal cysts includes ependymal cysts (which are com-posed of cuboidal to columnar ciliated cells and may resemble the ependymal lining), dermoid and epidermoid cysts (which have a keratinizing, strat-ified, squamous epithelium and contain keratin debris), and enterogenous cysts (which have a lin-ing of well-differentiated cuboidal to columnar epithelium and may contain mucin or have cilia).37 The findings in this case are consistent with the diagnosis of an arachnoid cyst.

Arachnoid cysts may be intradural or, more commonly, extradural.38 The cause is unknown, but most extradural cysts are associated with a defect in the dura that is presumed to be congeni-tal, as in this case.38-40 Meningeal cysts can be classified on the basis of their location.41 Type I cysts are extradural, without involvement of nerve-





root fibers. Type II cysts are extradural but in-volve nerve-root fibers (Tarlov perineurial cysts), and type III cysts are intradural. The cyst in this case would be classified as type I.

Discussion of M a nagemen t

Dr. Brass: After the operation, the patients neuro-logic symptoms resolved and his paroxysmal head-

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aches ceased. However, he now described a per-sistent low-grade (1 on a scale of 1 to 10) headache. Three months postoperatively, he reported trans-lucencies in his vision.

Dr. Dinkin: Neuro-ophthalmologic examination revealed worsening papilledema, with hemor-rhage (Fig. 1B), and on visual-field testing, there was a constriction of the inferior nasal visual field, reflecting damage to the retinal nerve-fiber layer, which occurs with chronic papilledema and tends to affect the superior fibers.42

Dr. Copen: A sagittal T2-weighted image of the lumbar spine approximately 1 month after spinal surgery (Fig. 4A) shows a large fluid collection extending from the original location of the cyst through a new multilevel laminectomy defect. It is unclear whether this represents cyst reexpansion, a postoperative seroma, or a pseudomeningocele. Mass effect on the thecal sac has increased since the preoperative examination. A repeat examina-

tion performed 6 weeks later (Fig. 4B) shows the resolution of mass effect on the thecal sac.

An axial T2-weighted image through the orbits obtained 3 months postoperatively, at the onset of new visual symptoms (Fig. 4C), shows marked expansion of the optic-nerve sheaths bilaterally and kinking of the optic nerves, reflecting in-creased intracranial pressure. There also appears to be slight concavity of the globe at the insertion of the optic nerve, a feature that suggests pap-illedema.

Dr. Dinkin: This case is unusual in that although fenestration of the cyst led to remission of the paroxysmal headaches, it also resulted in long-standing papilledema associated with significant visual-field defects. The operation may have re-moved a slit-valve mechanism between the cyst and the subarachnoid space, preventing further abrupt fluid shifts and elevations in the intracranial pres-sure (Fig. 5). However, the open connection be-

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Muller

Figure 5. Diagram of the Spinal Arachnoid Cyst and Its Possible Effect on the Flow of Cerebrospinal Fluid (CSF).

The cyst was located in the subarachnoid space and protruded through a small defect in the dura, compressing the lumbosacral nerve roots (Panel A). There are several possible explanations for the associated increased intracranial pressure. First, the cyst may have produced a mass effect on the subarachnoid space. Second, its presence may have led to a reduction in the surface area of arachnoid granulations in that region and therefore a reduction in the absorption of CSF. A third theory specifically addresses the presence of paroxysmal spikes in intracranial pressure associated with changes in position a slitlike opening in the cyst could have acted as a valve, resulting in intermit-tent release of cyst fluid into the subarachnoid space. (Panel B, arrow), with consequent sudden increases in intracranial pressure. After partial removal of the cyst wall, the slit valve would have been removed, but the open connection between the subarachnoid space and a developing pseudomeningocele (Panel C, arrows) may have further reduced the capacity of spinal arachnoid granulations to absorb CSF, leading to a more protracted (although milder) state of high intracranial pressure that was necessary for the emergence of chronic papille-dema and associated visual-field changes.





tween the subarachnoid space and a developing pseudomeningocele may have reduced the capacity of spinal arachnoid granulations to absorb CSF, leading to a more protracted (although milder) state of high intracranial pressure necessary for the emergence of chronic papilledema and associ-ated visual-field changes.

Dr. Williams: To relieve the increased intracra-nial pressure, we considered performing a lumbar-to-peritoneal shunt or a ventricular-to-peritoneal shunt. The lumbar-to-peritoneal approach is less invasive, but after the shunt is placed, the rate of flow cannot be adjusted. The patient also had re-cent lumbar spinal surgery, and the CSF dynam-ics leading to his elevated intracranial pressure were unclear. Placement of a ventricular-to-peri-toneal shunt is a more invasive procedure, with a higher likelihood of complications, but the rate of flow can be modified with the use of a valve that can be controlled with an external magnet. Depending on the patients symptoms, the shunt can be adjusted to provide greater or lesser flow through the catheter. This was an important con-sideration in this case, since we did not know how the patients symptoms would respond to CSF di-version. We elected to place a ventricular-to-peri-toneal shunt; the procedure was performed with-out complications.

Dr. Brass: After placement of the shunt, the pa-tients headaches, papilledema, and retinal hem-orrhages resolved. In retrospect, it is clear that elevated intracranial pressure caused his signs and symptoms. Spinal arachnoid cysts are rare in chil-dren, with fewer than 40 cases reported, and they are rarely associated with elevated intracranial pressure.32,38,43-46 It has been postulated that spi-nal arachnoid cysts may develop as a result of ele-vated intracranial pressure or that these cysts may cause elevated intracranial pressure by means of a mass effect on the thecal sac. The thecal sac

normally expands and responds to physiologic changes in CSF volume without causing apprecia-ble changes in pressure. The mass effect of a spi-nal lesion may affect this distensibility, ultimately leading to an elevation in intracranial pressure. The slitlike valve between the cyst and the sub-arachnoid space may explain the paroxysmal symptoms and postural effects seen in this pa-tient.32,38,43-46

This case serves as an important reminder that increased intracranial pressure may be caused by abnormalities outside the cranial cavity. It also emphasizes the need to continually reassess a pa-tient with atypical symptoms until a diagnosis that explains all the symptoms is established.

A Physician: Were there any cutaneous manifes-tations over the base of the spine, such as a he-mangioma, that might have suggested the pres-ence of a spinal lesion?

Dr. Williams: I saw no external manifestations.Dr. Ronald E. Kleinman (Pediatrics): What has

been the emotional effect of this protracted ill-ness on this young man?

Dr. Brass: He missed a lot of school, but he is well adjusted and is doing well in college. Despite all that has happened, he is not bitter and has taken an interest in his case. After the diagnosis of the spinal arachnoid cyst, he asked me, Do you think I am going to be in the New England Journal of Medicine?

A nat omic a l Di agnosis

Spinal arachnoid cyst.This case was presented at Pediatric Grand Rounds, April 1,

2008.Dr. Brass reports receiving consulting fees from EMD Serono

and Teva Neuroscience; and Dr. Freeman, holding equity owner-ship in Abbott Laboratories. No other potential conflict of inter-est relevant to this article was reported.

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