ABSTRACT
A 79-year-old male patient was followed for unilateral uveitis with 3 attacks in 10 months, despite initial improvement with steroid therapy. The patient had visual acuity (VA) of counting fingers in right eye, hypopyon and vitritis with no chorioretinal lesions. The left eye was normal. The patient was evaluated for intraocular foreign body, intraocular lymphoma and associated systemic disease and malignancy. Computed tomography of the abdomen showed a mass in the bladder. Biopsy confirmed bladder carcinoma. After resection of the mass, intraocular inflammation improved completely and no attack was noted in the follow-up. In his last examination, two years after the operation, VA was light perception; seclusio pupilla and mature cataracts were seen on biomicroscopy. There was no sign of vitritis on ocular ultrasonography. Evidence is discussed that suggests a link and potential etiology between refractory uveitis with hypopyon and bladder carcinoma. This is the first case of unilateral recurrent uveitis with hypopyon as the initial presenting sign of bladder carcinoma.
Introduction
The association between uveitis and concurrent malignancies has been described as pseudouveitis. Masquerade syndromes are a group of disorders that mimic ocular inflammatory disease, which are thought to be immune responses to concurrent intraocular tumors.1Masquerade syndromes usually do not respond well to steroid treatment. Another possible diagnosis to be considered is the paraneoplastic syndrome, the remote effect of a systemic malignancy presenting as ocular inflammatory disease. The pathogenesis of paraneoplastic syndromes is thought to be autoimmune, and they also do not respond well to steroid treatment.2
To our knowledge, the presentation of bladder carcinoma with unilateral anterior uveitis with hypopyon has not been reported to date. Here we report a case in which unilateral uveitis led to the detection of bladder carcinoma.
Discussion
The incidence of true immune-mediated uveitis declines in the elderly. Infection endophthalmitis, especially arising after surgery, and malignancy occur at higher frequency. In this age group, the possibility of a masquerade or paraneoplastic syndrome should always be considered.3,4
We report a patient with bladder carcinoma whose initial symptoms led him to consult an ophthalmologist. The patient presented with hypopyon and anterior chamber reactions three times within a span of ten months.
Carcinomas predominate as the primary lesions that produce ocular metastases. Ocular metastases are hematogenously disseminated and most commonly affect the uveal tract. In our case, the presentation was in the form of a rather benign unilateral anterior uveitis without the involvement of the posterior segment, which misled us to a diagnosis of idiopathic anterior uveitis after an initial etiological investigation overlooking the possibility of a malignancy. The patient had no sign of an intraocular mass at initial presentation or during follow-up.
Initial clinical presentation of urinary bladder carcinoma with distant metastasis is rare.4Urothelial metastases of the most common sites are regional lymph nodes, liver, lungs, and bone.5To our knowledge, initial clinical presentation with ophthalmologic pathology such as anterior uveitis and hypopyon has not been previously reported for bladder carcinoma.
Intraocular inflammation presenting as hypopyon may occur in acute lymphoblastic leukemia (ALL), acute myeloid leukemia (AML), and chronic myeloid leukemia (CML). Isolated presentation of hypopyon uveitis is very rare in AML, with only two similar reported cases.6Due to the patient’s age being over 60 years and isolated presentation of hypopyon and uveitis, the investigation of the patient for leukemia and lymphoma was recommended by other reports.3,4There was no clinical or laboratory evidence of hematologic malignancy.
The term ‘masquerade syndrome’ refers to a wide variety of disorders whose clinical features include the presence of cells either in the anterior chamber, vitreous or both, but are unrelated to any immune-mediated uveitic diseases. Paraneoplastic retinopathies constitute a subgroup of this syndrome, which is defined as carcinoma-associated retinopathy (CAR) and melanoma-associated retinopathy. CAR patients usually present with bilateral vision loss due to extensive retinopathy and uveitis. Small-cell lung, gynecologic and breast cancers are diagnosed in most of the patients.7
The coexistence of bladder carcinoma and ocular inflammation in our patient may be explained by several possible pathophysiological mechanisms. The presence of bladder carcinoma with the anterior chamber reaction may be completely coincidental. Nevertheless, the cessation of ocular inflammatory findings after the resection of the tumor led us to search for a relationship between the two entities. The cessation of ocular inflammation despite the absence of a curative treatment for the ocular disease or a systemic chemotherapy supported the evidence of absence of any malignant cells in the ocular tissues and pointed to the remote effects of the malignancy presenting as ocular inflammation. The cytologic examination of the aqueous humor or the vitreous, which we failed to obtain, may be of great help in the diagnosis.
Conclusion
To our knowledge, this is the first case of bladder carcinoma with an initial presentation of anterior uveitis. The case demonstrates the problems encountered in reaching a diagnosis and stresses the importance of a high index of suspicion of metastatic disease in elderly patients presenting with uveitis. Timely diagnosis is essential, particularly if the suspicion of ocular or systemic malignancy needs to be validated, as early diagnosis in ocular malignancy or metastasis can preserve sight and improve survival.
