Pseudotumor cerebri during pregnancy

ELS E V I E R

Clinical Eye and Vision Care 10 (1999) 189-194

Pseudotumor cerebri during pregnancy Rita Ellent, O.D." East New YorkDiagnostic and Treatment Center, SUNYState College of Optometry, 100 East24th Street, New York,NY10010, USA

Abstract

Pseudotumor cerebri (PTC) is characterized by increased intracranial pressure and papilledema in the absence of other neurologic localizing signs. Headaches and other visual disturbances may also exist. PTC or benign intracranial hypertension has been estimated to have an incidence of approximately 1 in 1000 pregnancies [Katz VL, Peterson R, Cefalo RC. Am J Perinatol 1989;6:442-4451. The following case report presents a patient with PTC during pregnancy. The clinical features of the disease, management strategies and differential diagnoses will be discussed. 0 1999 Elsevier Science Ireland Ltd. All rights reserved.

Keywords: Benign intracranial hypertension; Pseudotumor cerebri; Idiopathic intracranial hypertension; Pregnancy; Papilledema; Visual fields

1. Introduction

Ocular complications during pregnancy can be due to hormonal, metabolic, hematologic, immunologic and cardiovascular alterations [ 1,2]. PTC, which presents as papilledema secondary to increased intracranial pressure can occur during pregnancy. A normal CT scan of the head and a normal spinal fluid composition confirm this diagnosis. The following represents a case of PTC during pregnancy which subsequently resolved without medical treatment. 2. Case report

A 25-year-o1d7African American female presented to the Eye Clinic at the East New York Diagnostic and Treatment Center complaining of a swollen left lid for 1 week. Her medical history was remarkable for anemia. She reported taking no medications or any history of allergies. Upon examination the diagnosis of an internal hordeolum on the left upper lid was made and she was treated with bacitracin ointment and warm compresses. She was scheduled for follow-up in 1 week.

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The patient missed her follow-up appointment and returned 1 month later complaining of seeing red spots in the right eye and intermittent headaches for 1 week. On examination her best corrected visual acuity was 20/20 in both eyes. Pupils were equal, round, briskly reactive to light with no relative afferent pupillary defect. Extraocular motilities were unrestricted and confrontation visual fields were full in both eyes. Slit lamp examination was unremarkable in each eye except for a resolving internal hordeolum on the left upper lid. Applanation tonometry was 14 mmHg OD and 0s. Dilated fundus examination revealed bilateral swollen optic nerves with mild venous tortuosity. In the right eye, a small flame-shaped retinal hemorrhage was present inferior to the disc. The maculae were clear and a foveal reflex was present in both eyes (Figs. 1 and 2). She was diagnosed with bilateral disc edema and referred for a CT scan and neurologic evaluation. During this examination a routine pregnancy test revealed that the patient was 5 weeks pregnant. The CT scan of the head and orbits and neurologic examination were unremarkable. The patient refused a spinal tap. A working diagnosis of PTC was made, but without a lumbar puncture the diagnosis was inconclusive. No medical treatment was initiated.

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Fig. 1. Marked swelling of the optic nerve head with a small flame-shaped retinal hemorrhage inferior to the disc O.D. Fig. 2. Marked swelling of the optic nerve head with exudative material superior-temporally along the disc O.S.

The patient returned to our eye clinic 1 week later and reported that the headaches had subsided. Visual

acuity remained 20/20 in each eye. Humphrey 120 point visual field screening showed no neurological

R. Ellent /Clinical Eye and Vuion Cave 10 (1999)189-194

defects or enlarged blind spots in either eye. Color vision with Ishihara plates and red desaturation testing were normal in both eyes. Bilateral optic nerve head edema was still present. At 6 weeks follow-up, her vision remained stable. Dilated fundus examination showed reduced optic nerve swelling. Vision continued to be stable on a subsequent 3 month follow-up examination, and at this time the papilledema had resolved. Distinct disc margins were evident with no optic atrophy present. The patient was scheduled to return for follow-up after delivery, in 6 months. 3. Discussion

The patient's history and clinical findings are consistent with a diagnosis of pseudotumor cerebri occurring during her first trimester. There have been multiple reports of PTC occurring during pregnancy, which is most frequently detected in obese women of childbearing age. In the past, many authors believed PTC occurred with dramatically increased frequency during pregnancy. However, recent studies have concluded that PTC is no more frequent in pregnancy than one would expect in an age-matched non-pregnant population [3,4,51. Patients with PTC may present asymptomatically, or with focal neurological symptoms, such as headaches, vomiting on awakening or with physical straining, and diplopia from a sixth nerve palsy. Nonneurological symptoms may include transient blurring of vision lasting 5-30 s or mildly reduced visual acuity. PTC is not life threatening, but optic nerve damage can develop as a result of long-standing disc edema. The etiology of the increase in intracranial pressure in patients with PTC remains obscure. It has been found that a decrease in corticosteroid levels that occurs with the increase in estrogen levels towards the end of the first trimester coincides with the onset of PTC during pregnancy [7]. When presenting during pregnancy, PTC usually occurs within the first 5 months, although it may occur in any trimester [3,6,8]. Recurrence of PTC in subsequent pregnancies has been reported, however, with a 10% recurrence rate in subsequent pregnancies, the rate is the same as in non-pregnant women [3,8]. 3.1. Diagnosis and management Before the diagnosis of PTC can be made in a pregnant patient, a number of other conditions causing disc edema must be ruled out including preeclampsia, eclampsia, diabetic retinopathy, central retinal vein occlusion, and intracranial mass. In pregnant patients dilation with 1% tropicamide is accept-

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able with no risk of adverse effects to the fetus or the mother. One should avoid using phenylepherine [9]. When disc edema is present, it is crucial to refer patients for neuroimaging first, and then possibly lumbar puncture. A CT scan or MRI of the head and orbits is performed to rule out an intracranial mass or disease process. MRI and CT scan with contrast enhancement has been shown to be safe in pregnant patients 181. CSF pressure is then measured to determine if there is evidence of increased intracranial pressure and for fluid studies. Patients should also be evaluated by their obstetrician to rule out preeclampsia, eclampsia, and diabetes. Once PTC is diagnosed, the decision to treat is based on visual acuity and visual field loss. PTC is generally a self-limited process with a high remission rate and little visual compromise. However, papilledema can cause optic atrophy in 4 ~ 1 2 %of cases, leading to severe vision loss, and up to 50% of patients may develop a reduction in visual field after resolution of papilledema [3,8,10,111. Thus, it is important to follow these patients closely after resolution of symptoms. Pregnancy does not affect the eventual visual outcome of patients with PTC [1,12]. A number of treatment options are available for PTC including corticosteroids, analgesics, repeated lumbar punctures, lumboperitoneal shunt, and optic nerve sheath decompression. Lumbar puncture reduces the intracranial pressure, however, it is considered risky because of the chance of spontaneous abortion [13]. Many obstetricians feel that steroids can be used with caution during pregnancy, however, steroids pose a possible adverse effect to both the mother and fetus [8,13].Dehydrating agents, including carbonic anhydrase inhibitors, loop diuretics, and mannitol appear to be contraindicated during pregnancy because of their risk of congenital deformities 1141. It is critical for the eye care practitioner to consult with the obstetrician and neurosurgeon. The decision to initiate treatment is dependent upon the visual status. It is crucial to monitor this patient very closely with visual acuities, visual fields and dilated fundus examinations, with any signs of worsening prompting treatment initiation (Fig. 3). An enlarged blind spot is the most frequent visual field defect found in patients with PTC. When treatment is warranted, blind spot measurements are important to monitor effectiveness of therapy. Patients may also present with concentric constriction, usually seen in chronic stages, with the inferior nasal quadrant being most often involved [10,15,16]. 3.2. Differential diagnosis A swollen optic nerve head during pregnancy may

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Optic disc edema absent

edema present

head and orbits

'

Increased intracranial pressure Normal CSF

Increased intracranial

Abnormal CSF composition

Neurological Evaluation

Diagnosis: Pseudotumor cerebri

Evaluate visual acuity and visual fields: Treatment indicated based on vision

reduction in visual acuity andor visual

acuity Normal visual

visual acuity, visual fields, and fundus grounds

-Treatment: Corticosteroids,

Repeated lumbar punctures

Improvement

-

No improvement

Consider Surgery: Lumboperitoneal shunt,

Fig. 3. Flow diagram for the diagnosis and management of pseudotumor cerebri during pregnancy.

be due to numerous possible causes (Table 1). Papilledema is always a sequela of increased intracranial pressure. When secondary to a hypercoagulable state during pregnancy, the risk of venous sinus thrombosis is greater, resulting in significantly increased intracranial pressure. Pregnancy is one of the wide variety of conditions which predispose patients to sagittal sinus thrombosis. This rare condition presents with sudden and severe headache and marked papilledema. Initial presenta-

tion may also include transient obscurations of vision and diplopia from a sixth nerve palsy. Visual acuity is often normal or only mildly reduced. An enlarged blind spot is seen on visual field testing. In rare cases severe visual field constriction may be present 1241. Because symptomatology is nonspecific imaging studies play a vital role in the diagnosis. Sagittal sinus thrombosis is not evident on CT examination. It is seen with MR images and MR angiography. Though rare because of its potentially devastating result it is

R. Ellent /Clinical Eye and Vuion Cave 10 (1999)189-194 Table 1 Differential diagnosis of swollen optic nerve head during pregnancy:

(1)Papilledema/pseudotumor cerebri (2) Pregnancy-induced hypertension (3) Diabetic retinopathy

(4) Central retinal vein occlusion

(5) Intracranial mass (6) Optic neuritis

(7) Ischemic optic neuropathy (8) Optic disc drusen or an anomalously elevated disc (9) Sagittal sinus thrombosis

critical for the clinician to consider it as part of the differential diagnosis in pregnant patients presenting with disc edema. Elevated blood pressure which develops during pregnancy in a previously normotensive female is called pregnancy-induced hypertension (PIH). This includes preeclampsia, the development of hypertension with proteinuria and edema, usually seen after the fifth month of pregnancy, and eclampsia, the occurrence of seizures in a preeclamptic woman, generally seen late in pregnancy. Preeclampsia occurs in 5% of first pregnancies. It is more likely to occur in younger and older women, multifetal pregnancy, hemolytic disease of the fetus and in women with hypertension, diabetes and renal disease [1,17,18]. The earliest and most common retinal change of preeclampsia is focal arteriolar spasm. In the later stage of this condition generalized narrowing of retinal arterioles is observed. These changes are reversible post-partum in most patients. Hemorrhages, cotton wool spots, retinal edema and papilledema can occur in women with PIH. Symptoms of scotoma, diplopia, dimness of vision and photopsia are reported by 25% of patients with severe preeclampsia and by 50% of patients with eclampsia [MI. The Diabetes 2000 program of the American Academy of Ophthalmology includes pregnancy as one of the risk factors requiring more careful followup for progression of diabetic retinopathy [l]. Unilateral or bilateral disc edema (diabetic papillitis) can occur in diabetic patients often accompanied by telangiectasia of disc vessels. There is no relationship between the severity of diabetic retinopathy and the development of disc edema. Diabetic papillitis is more commonly encountered in patients with juvenile-onset diabetes. No change in vision to a mild reduction in visual acuity can occur, which usually resolves within 6-8 weeks without treatment. Patients with gestational diabetes are not at risk for diabetic retinopathy [19,201.

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Disc edema may also present in conjunction with a central retinal vein occlusion. There are a number of reported cases in the literature of a central retinal vein occlusion occurring during pregnancy. It has been found that during pregnancy alterations in immunologic function and increased levels of blood coagulating factors increases the risk of vascular occlusions 131. Intracranial tumors can also cause swelling of the disc. Pituitary adenomas and meningiomas do appear to become more symptomatic and grow more rapidly during pregnancy, although intracranial tumors overall do not appear to be more frequent in pregnant vs. non-pregnant women [ 17,211. During pregnancy the pituitary gland normally undergoes a small enlargement, however, this change is too small to cause visual field defects. Postpartum the pituitary gland returns to normal size [17]. Optic neuritis may present with a swollen disc in addition to peripapillary flame-shaped hemorrhages. It presents as a typically unilateral loss of vision with a relative afferent pupillary defect and decreased color vision. There is a lower incidence of optic neuritis occurring during pregnancy. This may be due to the immunosuppressive effect of pregnancy [1,17,221. Ischemic optic neuropathy is a very rare complication during pregnancy causing disc swelling and elevation, but a characteristically pale disc. Buried drusen of the optic nerve head often give the discs a swollen appearance, especially in younger patients. They evolve slowly with age, with calcium-like refractile deposits becoming more apparent. Disc drusen often result in visual field defects which may present as scotomas, concentric constriction, or generalized depressions. B-scan ultrasonography is useful in the differential diagnosis and should be considered in all suspected cases to avoid unnecessary neurological evaluation. Pregnancy has not been found to effect the course or outcome of optic nerve head drusen 1231. 4. Conclusion

PTC presenting during pregnancy is a diagnosis by exclusion. Although rare, the challenge to manage these patients requires a cohortive effort between the optometrist or ophthalmologist, obstetrician, and neurosurgeon. Patients with PTC should be monitored closely to ensure complete resolution of the disc edema and good visual outcome. Acknowledgements

I would like to thank Dr Ray Corbin-Simon, Chief of Optometry Services, East New York Diagnostic

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and Treatment Center, Brooklyn, New York for her help with the preparation of this manuscript and Dr. Christine Dumestre for consultation on this case. I am grateful for their support and abundant knowledge during my residency. References Sunness JS. The pregnant woman’s eye. Surv Ophthalmol 1988;32219-238. Digre KB, Varner MW. Pregnancy. In: Gold DH, Weingeist TA, editor. The eye in systemic disease. Philadelphia: JB Lippincott, 1990;(15)160:483-486. 131 Digre KB, Varner MW, Corbett JJ. Pseudotumor cerebri and pregnancy. Neurology 1984;34:721-729. 141 Kassam SH, Hadi HA et al. Benign intracranial hypertension in pregnancy: current diagnostic and therapeutic approach. Obstet Gynecol Surv 1983;38:314-321. 151 Guiseffi V et al. Symptom and disease associations in idiopathic intracranial hypertension (pseudotumor cerebri): a case-control study. Neurology 1991;41:239-244. Greer M. Benign intracranial hypertension: pregnancy. Neurology 1963;13:670-672. Ireland B et al. The search for causes of idiopathic intracranial hypertension: a preliminary case-control study. Arch Neurol 1990;47:315-320. Katz VL, Peterson R, Cefalo RC. Pseudotumor cerebri and pregnancy. Am J Perinatol 1989;6:442-445. Samples JR, Meyers SM. Use of opthalmic medications in pregnant and nursing women. Am J Opthalmol 1988;106:616-623. Krogsaa B et al. Ophthalmologic prognosis in Benign intracranial hypertension. Acta Ophthalmologica 1985;Suppl. 6173x62-64. Keltner JL, Miller NR, Gittinger JW,Burde RN. Pseudotumor cerebri. Surv Ophthalmol 1979;23:315-322.

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