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Show Spontaneous Postpartum Resolution of Vision Loss Caused by a Progesterone Receptor-Positive Tuberculum Sellae Meningioma Joseph G. Chacko, MD, Jessica L. Miller, MD, Edgardo J. Angtuaco, MD Abstract: A 27-year-old pregnant woman reported pro-gressive loss of vision. Brain MRI disclosed an intracranial mass compressing the optic nerves and chiasm with imaging features suggestive of meningioma. Because delivery was imminent, surgical removal was deferred. Within a few days after delivery, the patient noted im-provement in vision. Subsequent neuro-ophthalmological evaluations documented almost complete visual recovery within 2 months postpartum. The mass was removed surgically at 10 months postpartum. It was a grade I meningioma with progesterone cell surface receptors. The patient's visual function has remained stable during a follow-up of 14 months. This is the first reported case with full visual function and MRI documentation of spon-taneous postpartum visual recovery in a progesterone receptor-positive meningioma compressing the anterior visual pathway. Journal of Neuro-Ophthalmology 2010;30:132-134 doi: 10.1097/WNO.0b013e3181da9d59 2010 by North American Neuro-Ophthalmology Society Intracranial meningiomas compressing the optic nerve in which progressive visual loss has been documented are often removed surgically, even in pregnancy. These tumors can be hormonally responsive and be positive for progesterone and/or estrogen receptors (1-3). Thus, they can display an accelerated growth pattern when these hormone levels are highest, such as during the second half of pregnancy. We report a case of a gravid patient with a hormonally responsive tuberculum sellae meningioma causing optic nerve dysfunction from compression who did not undergo intervention because of imminent delivery. Following de-livery, she sustained dramatic spontaneous visual recovery with only mild regression in tumor size, a previously in-completely documented phenomenon. CASE REPORT A 27-year-old woman in her 26th week of pregnancy presented with a 2-week history of vision loss in the right eye. She also complained of headaches with nausea and vomiting for 4 months. Medical history included migraine and spastic diplegic cerebral palsy. She had had 2 preterm deliveries at 28 and 29 weeks. Visual acuity was count fingers at 3 feet in the right eye and 20/15 in the left eye. Color vision by Ishihara plates was 1/11 in the right eye and 11/11 in the left eye. Pupils measured 7 mm bilaterally in dim illumination, with trace reaction to light in the right eye and brisk reaction in the left eye and a 1.5 log unit afferent pupillary defect in the right eye. Ophthalmoscopy was normal. Automated visual fields showed dense temporal loss in the right eye and a mild inferotemporal defect in the left eye (Fig. 1A). Brain MRI without contrast showed a suprasellar mass measuring 11 3 14 mm that was compressing the optic chiasm, most sus-picious for meningioma. At 29 weeks of pregnancy, she was complaining of contractions and a further decline in vision. Visual acuity had decreased to hand motion in the right eye and 20/30 in the left eye. The afferent pupillary defect had decreased to 0.9 log units, perhaps owing to increasing dysfunction of the left optic nerve. Ophthalmoscopy remained normal. Visual fields (Fig. 1B) showed worsening defects. Repeat Department of Ophthalmology, Jones Eye Institute (JGC, JLM); and Department of Radiology (EJA), University of Arkansas for Medical Sciences, Little Rock, Arkansas. Supported in part by an unrestricted grant from Research to Prevent Blindness (New York, NY) and the Pat & Willard Walker Eye Research Center, Jones Eye Institute, University of Arkansas for Medical Sciences (Little Rock, AR). Presented in part at the North American Neuro-Ophthalmology Society Annual Meeting, February 24, 2009, Lake Tahoe, NV. The authors declare no conflicts of interest. Address correspondence to Joseph G. Chacko, MD, Departments of Ophthalmology and Neurology, University of Arkansas for Medical Sciences, 4301 W. Markham #523, Little Rock, AR 72205; E-mail: jchacko@uams.edu 132 Chacko et al: J Neuro-Ophthalmol 2010; 30: 132-134 Original Contribution Copyright © North American Neuro-ophthalmology Society.Unauthorized reproduction of this article is prohibited. MRI with contrast (Fig. 2A) was consistent with mild tu-mor enlargement since the previous study. Because of im-minent delivery, surgery was postponed and instead she underwent uncomplicated cesarean section at 30 weeks of pregnancy. Within a few days after delivery, she noted improvement in her vision. At 1 month after delivery, visual acuity was 20/40 in the right eye and 20/20 in the left eye. Color vision had improved to 11/11 in both eyes. The right afferent pupillary defect had decreased to 0.6 log units. Visual fields were markedly improved. At the 8-week postpartum visit, left visual acuity had further improved to 20/25. The right afferent pupillary defect now measured only 0.3 log units. Visual fields were nearly normal (Fig. 1C). At 24 weeks postpartum, the MRI tumor dimensions in the coronal plane had slightly regressed (Fig. 2B). Visual function remained stable. At 40 weeks postpartum, the tumor was electively re-moved. It was a World Health Organization grade I me-ningioma. Tumor cells were strongly positive for progesterone receptor and negative for estrogen receptor. Neuro-ophthalmologic follow-up has documented stable visual function for 14 months since her recovery. DISCUSSION This is the first reported case with visual field and neuro-imaging documentation of spontaneous postpartum reso-lution of visual loss due to an anterior visual pathway compressive intracranial meningioma with progesterone cell surface receptors. The meningioma probably displayed an accelerated growth pattern during the second half of pregnancy due to increasing levels of progesterone. Most cases involving hormonally responsive intracranial meningiomas detected during pregnancy have been urgently removed surgically, either during the pregnancy or within a few days after delivery (4-8). Adverse events can FIG. 1. Automated 30-2 visual fields at 26 weeks of pregnancy (A), 29 weeks of pregnancy (B), and 8 weeks after delivery (C). FIG. 2. A. Coronal postcontrast T1 MRI at 29 weeks of pregnancy reveals an enhancing mass with features of a tuberculum sellae meningioma. B. Coronal postcontrast T1 MRI at 24 weeks after delivery reveals only slight reduction in tumor size. Original Contribution Chacko et al: J Neuro-Ophthalmol 2010; 30: 132-134 133 Copyright © North American Neuro-ophthalmology Society.Unauthorized reproduction of this article is prohibited. accompany neurosurgery on gravid patients. Among 12 gravid patients with visual loss caused by compressive in-tracranial meningiomas who have undergone craniotomy, the fetus died in 2 (4,6-8). The phenomenon of spontaneous visual improvement after delivery in patients with anterior visual pathway compressive meningiomas has been previously reported but with less documentation. Kanaan et al (7) mentioned 2 patients with compressive meningiomas whose vision im-proved spontaneously postpartum but did not report visual acuity, visual field, or MRI changes. Johnson et al (8) also reported one patient whose vision spontaneously improved postpartum. Optic neuritis had been the initial clinical diagnosis, but a postpartum brain MRI showed a tuberc-ulum sellae meningioma. We theorize that our patient improved postpartum be-cause progesterone levels decreased with delivery of the placenta. Without this trophic factor present, the menin-gioma regressed mildly in size, and the optic nerves were decompressed. Prior to surgery, the patient was advised not to breast-feed or become pregnant because the increased progesterone levels could result in a return of her vision loss. The pituitary also enlarges during late pregnancy and thus can further elevate a meningioma to compress the optic nerve/chiasm (5). It is important to note that our patient did not observe visual improvement immediately after delivery but rather a few days later. Because the patient became asymptomatic and repeat automated visual fields showed resolution of visual field loss, no immediate neurosurgical intervention was indi-cated. Urgent surgery was thus avoided at a critical time when mother-baby bonding normally occurs. REFERENCES 1. Saitoh Y, Oku Y, Izumoto S, Go J. Rapid growth of a meningioma during pregnancy: relationship with estrogen and progesterone receptors-case report. Neurol Med Chir (Tokyo). 1989;29:440-443. 2. Hsu DW, Efird JT, Hedley-White ET. Progesterone and estrogen receptors in meningiomas: prognostic considerations. J Neurosurg. 1997;86:113. 3 Black PM. Hormones, radiosurgery, and virtual reality: new aspects of meningioma management. Can J Neurol Sci. 1997; 24:302-306. 4. Wan WL, Geller JL, Feldon SE, Sadun AA. Visual loss caused by rapidly progressive intracranial meningiomas during pregnancy. Ophthalmology. 1990;97:18-21. 5. Ebner FH, Bornemann A, Wilhelm H, Ernemann U, Honegger J. Tuberculum sellae meningioma symptomatic during pregnancy: pathophysiological considerations. Acta Neurochir (Wien). 2008;150:189-193. 6. Balki M, Manninen PH. Craniotomy for suprasellar meningioma in a 28-week pregnant woman without fetal heart rate monitoring. Can J Anesth. 2004;51:573-576. 7. Kanaan I, Jallu A, Kanaan H. Management strategy for meningioma in pregnancy: a clinical study. Skull Base. 2003; 13:197-203. 8. Johnson N, Sermer M, Lausman A, Maxwell C. Obstetric outcomes of women with intracranial neoplasms. Int J Gynecol Obstet. 2009;105:56-59. Original Contribution 134 Chacko et al: J Neuro-Ophthalmol 2010; 30: 132-134 Copyright © North American Neuro-ophthalmology Society.Unauthorized reproduction of this article is prohibited. |