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Sir,

Diffuse or multifocal seeding of the leptomeninges by carcinoma, so-called meningeal carcinomatosis, often presents as simultaneous or rapidly sequential cranial neuropathy, with or without headache, altered mental status, or signs of meningeal irritation. Visual loss may occur in up to 30% of these patients, usually rapid, painless, and unilateral, and often progressing to the other eye.1 Only a few cases of meningeal carcinomatosis owing to ovarian carcinoma have been reported. To the best of our knowledge there are no reports in the literature on acute bilateral blindness occurring during hours owing to meningeal carcinomatosis or ovarian malignancies. We present a case of acute bilateral blindness secondary to ovarian adenocarcinoma with meningeal infiltration.

Case report

A 50-year-old woman presented in May 2000 with abdominal distention and ill-defined abdominal pain. Examination revealed a firm pelvic mass. Ultrasound showed a mass compatible with ovarian cancer. Surgical exploration and pathologic examination assessed an ovarian serous cystoadenocarcinoma stage IIIc (with abdominal implants over 2 cm in diameter and positive retroperitoneal and inguinal nodes). Total hysterectomy with bilateral salpingo-oophorectomy was performed, and three cycles of systemic chemotherapy of cisplatine and cyclophosphamide were administered. During the follow-up period, no visual complaints were reported. After 2 years, the patient presented with altered mental status and headaches, without visual complaints, or pupil abnormalities. Lumbar puncture revealed elevated cerebrospinal fluid (CSF) protein, and cytology of the centrifugated CSF sediment demonstrated numerous cells consistent with adenocarcinoma. Magnetic resonance imaging (MRI) Tl-weighted scan of the orbit showed thickening of both apical intraorbital optic nerves with slight enhancement postgadolinium (Figures 1 and 2). MRI of the brain demonstrated minimal meningeal enhancement and normal postchiasmatic visual pathways.

Figure 1
figure 1

Gadolinium-enhanced axial MRI Tl-weighted SPIR demonstrates bilateral slight enhancement of the apical optic sheath.

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Figure 2
figure 2

Gadolinium-enhanced axial MRI Tl-weighted SPIR demonstrates bilateral slight enhancement of the apical optic sheath.

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During admission, the patient complained of acute painless bilateral loss of vision, and mental status deteriorated. The patient reported that the loss of vision occurred within less than 12 h. At examination, visual acuity was no light perception in both eyes. Anterior segment and intraocular pressure were normal in both eyes. Pupils were amaurotic and nonreactive to light. Optic disc and fundus examination were normal in both eyes. Full ocular ductions were observed. Fluorescein angiography did not reveal any pathology. Despite radiation therapy, the patient died 1 week after the presentation of the visual loss. No autopsy was performed.

Comment

The most common tumours to metastasize to the meninges are breast carcinoma, lung carcinoma, and melanoma. Leptomeningeal involvement has been estimated to occur in 2.5–5% of all breast cancers, 9–25% of small cell lung cancers, and 23% of melanomas.2 A history of known malignancy is absent in 6–38% of patients with meningeal carcinomatosis.

The combination of headache, rapidly progressive visual loss, sluggish pupil reactions, and normal optic discs should bring to mind the diagnosis of meningeal carcinomatosis.3 The pathophysiologic mechanisms of visual loss include migration of tumour cells along pial septae with invasion of the optic nerve mesenchyma, ‘tumour cuffing’ with compression of the nerve, and invasion of the arachnoid surrounding the intracranial optic nerves and chiasm with secondary interruption of the neural microvasculature.4 The diagnosis of meningeal carcinomatosis is based on cytological confirmation, by either CSF cytology or leptomeningeal biopsy, and can also be supported by MRI findings. Median survival in untreated patients is 4–8 weeks, which is extended to 6 months by aggressive treatment in selected patients.5 The differential diagnosis of visual loss in a patient with a known malignancy besides meningeal carcinomatosis includes cancer-associated retinopathy (CAR). But in CAR patients, visual loss is usually slowly progressive, ophthalmoscopy reveals attenuated retinal arterioles and changes in retinal pigment epithelium and vitreal cells; electroretinogram (ERG) is almost always abnormal, and in many patients autoantibodies to a 23 kDa retinal antigen may be detected by Western blot immunoelectrophoresis.

In our patient, ERG and CAR antibody testing were considered unnecessary because of the CSF confirmation of the tumour, and the MRI findings. Visual loss as a result of CAR caused by ovarian carcinoma has been described, but to the best of our knowledge there are no reports in the literature on meningeal carcinomatosis with acute bilateral blindness owing to ovarian adenocarcinoma.