PITUITARY ADENOMA PRESENTING AS BILATERAL BULLOUS EXUDATIVE RETINAL DETACHMENTS: A CLINICOPATHOLOGIC REPORT Patrick Williams, MD,* Chi Chao Chan, MD,† Ronald Buggage, MD,† Sunil Srivastava, MD,†‡ David Callanan, MD*
Purpose: To describe a case of pituitary adenoma presenting as bilateral serous retinal detachments and the histopathologic and hematologic evidence of a linkage. Methods: Record review for a single patient including testing to determine the etiology of the retinal detachments. Results: The retinal detachments were determined to be a variant of central serous chorioretinopathy. A pituitary adenoma was discovered by neuroimaging. Histopathologic analysis of the tumor and serum cortisol levels demonstrated the lesion to be a cortisolsecreting adenoma. Conclusion: Bilateral serous retinal detachments are a variant of central serous chorioretinopathy and a rare presentation of pituitary adenoma. RETINAL CASES & BRIEF REPORTS 3:230 –232, 2009
From *Texas Retina Associates, Dallas, Texas; †Laboratory of Immunology, National Eye Institute, Bethesda, Maryland; and ‡Emory Eye Clinic, Atlanta, Georgia.
Case Report A 55-year-old Native American man was referred for evaluation of blurry vision (worse in the left eye) for 2 months. He had been treated with oral prednisone by the referring physician for possible Vogt-Koyanagi-Harada disease and had finished a tapering schedule days before presentation. Subjectively, his symptoms were worse since stopping prednisone therapy. He denied any discomfort around the eyes and denied any headaches, tinnitus, difficulty hearing, or neck stiffness. He noticed no skin changes. Recent computed tomography of the abdomen revealed only a renal cyst. Magnetic resonance imaging (MRI) of the head had been ordered, but the patient failed to comply with this recommendation. At examination, visual acuity was 20/30 in the right eye and 20/40 in the left eye. Intraocular pressure was within normal limits, and results of anterior segment examination were unremarkable bilaterally. No anterior cells were appreciated, but vitreous examination revealed trace cells in the right eye and 1⫹ cells in the left eye. Several placoid lesions (1 to 2 disk diameters in size) were present subretinally in both eyes (Fig. 1). The right fundus had slight subretinal fluid inferiorly, and the left fundus had a bullous serous retinal detachment inferiorly. No pigment epithelial detachments were appreciated. The patient was observed initially with continued steroid treatment and further recommendation for MRI of the head. Six weeks later, MRI had again not been performed, and steroid treatment yielded no improvement. In fact, the left retinal detach-
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entral serous chorioretinopathy (CSC) is characterized by a serous detachment of the neurosensory retina and/or retinal pigment epithelium, retinal pigment epithelium leakage, and angiographic retinal pigment epithelium and choroidal hyperpermeability. The disorder has been associated with hypercortisolism from both endogenous and exogenous sources.1–5 This case represents an endogenous source of hypercortisolism, a pituitary adenoma, manifesting as bilateral CSC. Bilateral retinal detachments are an uncommon but well described variant of CSC.2 A review of the literature found no other cases of pituitary adenoma associated with bilateral serous retinal detachments.
Reprint requests: David Callanan, MD, Texas Retina Associates, 1001 North Waldrop Drive #512, Arlington, TX 76012; e-mail:
[email protected]
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Fig. 2. Subretinal fluid shows foamy macrophages with pigment granules (Giemsa stain; original magnification, ⫻640).
National Eye Institute for analysis, which demonstrated only chronic inflammation (Fig. 2). The patient subsequently developed a right third cranial nerve palsy. He was referred to the National Institutes of Health for repeated biopsy of the intracranial mass, which demonstrated an atypical, adrenocorticotropic hormone–secreting, highly cellular pituitary adenoma (corticotroph cell adenoma) infiltrating fibrous tissue and bone (Fig. 3). Systemic hypercortisolism was discovered, with highly elevated levels of serum cortisol (42.8 g/dL), 24-hour urine cortisol (896.6 g/dL), and adrenocorticotropic hormone (938.0 pg/mL). The patient underwent radiotherapy for the remaining residual pituitary adenoma but subsequently died due to infiltrating tumor.
Discussion This patient presented with the unusual symptoms of mild vitritis and large dependent serous retinal
Fig. 1. Color photograph (A) and angiogram (B) at initial presentation. Fibrinous subretinal lesions are visible in the right eye (A). Multiple focal points of leakage are evident on the angiogram (B).
ment had progressed into the macula. A consideration of possible atypical idiopathic CSC prompted a rapid taper and discontinuation of steroid therapy and another admonition to perform MRI. The patient did not return for his scheduled appointment but returned 3 months later complaining of double vision and headaches. A right sixth cranial nerve palsy was noted at examination. In addition, there was an increase of subretinal fluid in the right eye. MRI of the head was again reordered. The patient complied with the recommendation, and MRI demonstrated a mass at the sella turcica and right cavernous sinus. Lumbar puncture was performed, but cerebrospinal fluid analysis revealed no neoplastic cells. Biopsy of the intracranial mass revealed only necrotic tissue. Vitreous and subretinal fluid biopsy to rule out intraocular lymphoma was then performed, and the biopsy sample was sent to the
Fig. 3. Brain biopsy shows a noncapsulated mass eroding the sella turcica. The mass is composed of periodic acid–Schiff (PAS) stain– positive, relatively uniformed, large polygonal cells arrayed in sheets. These cells contain some pleomorphic nuclei and basophilic densely granulated or chromophobic sparsely granulated cytoplasm (PAS stain; upper: original magnification, ⫻100).
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detachments. The differential diagnoses of serous retinal detachment include Vogt-Koyanagi-Harada disease, choroidal tumors, malignant hypertension, toxemia of pregnancy, and choroidal inflammatory diseases including sarcoidosis, sympathetic ophthalmia, posterior scleritis, choroidal mucosa–associated lymphoid tissue lymphoma, idiopathic uveal effusion, ocular contusion, bilateral diffuse uveal melanocytic proliferation, and CSC.2 The patient’s Native American heritage, along with large serous retinal detachments and vitritis, supported the initial diagnosis of Vogt-Koyanagi-Harada disease by the referring physician, but no other symptoms were present. On the basis of the angiographic findings and the lack of improvement with corticosteroid treatment, the idea of atypical CSC was entertained. Although vitritis is not typically seen with CSC, the course of this patient’s retinal detachments has been described by Gass as a variant of CSC.2 Chronic dependent serous detachments can occur, with associated pigmentary degeneration, retinal capillary loss, vitreous hemorrhage, and electroretinogram changes.6 In addition, fibrinous exudates can occur in 10% of cases of CSC.7 These exudates are more commonly associated with larger areas of retinal detachment and more prominent fluorescein leakage. The eventual diagnosis of an adrenocorticotropic hormone–secreting pituitary adenoma confirmed the diagnosis of CSC. Unfortunately, the diagnosis was delayed. The first reason for the delay was the patient noncompliance with the recommendation for MRI of the head. The second reason was the negative result of the initial pituitary mass biopsy. The development of CSC due to exogenous and endogenous sources of hypercortisolism is well known.1–5 However, a cortisol-secreting pituitary adenoma manifesting as bilateral serous retinal detachments is not well
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documented. Our case is similar to one of the six cases reported by Harada and Harada3 in 1985, although their case was unilateral. In that case, the patient had already undergone excision of the adenoma. The patient had pigment migration in the macula but no serous detachment. Fluorescein angiography revealed a hyperfluorescent spot in the macula but no leakage. Three years later, the patient returned with worsening scotoma and a serous detachment. Both cases of pituitary adenoma emphasize the need for neuroimaging when serous retinal detachments do not fit the characteristics of typical etiologies or respond to initial therapy. In addition, determination of serum cortisol levels should be considered in atypical presentations of bilateral retinal detachments. Key words: central serous chorioretinopathy, central serous retinopathy, serous retinal detachment, exudative retinal detachment, pituitary adenoma, hypercortisolism. References 1.
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Bouzas EA, Karadimas P, Pournaras CJ. Central serous chorioretinopathy and glucocorticoids. Surv Ophthalmol 2002;47: 431–448. Gass JDM. Stereoscopic Atlas of Macular Diseases: Diagnosis and Treatment. 4th ed. St. Louis, MO: Mosby; 1997:52–68. Harada T, Harada K. Six cases of central serous choroidopathy induced by systemic corticosteroid therapy. Doc Ophthalmol 1985;60:37–44. Bouzas EA, Scott MH, Mastorakos G, Chrousos GP, KaiserKupfer MI. Central serous chorioretinopathy in endogenous hypercortisolism. Arch Ophthalmol 1993;111:1229–1233. Haimovici R, Koh S, Gagnon DR, et al. Risk factors for central serous chorioretinopathy: a case-control study. Ophthalmology 2004;111:244–249. Gass JD, Little H. Bilateral bullous exudative retinal detachment complicating idiopathic central serous chorioretinopathy during systemic corticosteroid therapy. Ophthalmology 1995; 102:737–747. Gass JD. Central serous chorioretinopathy and white subretinal exudation during pregnancy. Arch Ophthalmol 1991;109:677– 681.
