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PituitaryAdenomaPresentingAsBilateralBullousExudativeRetinalDetachments:AClinicopathologicReport

ARTICLEinRETINALCASES&BRIEFREPORTS·DECEMBER2008

DOI:10.1097/ICB.0b013e31815f3cac

5AUTHORS,INCLUDING:

Chi-ChaoChan

NationalInstitutesofHealth

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RonaldBuggage

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DavidGCallanan

TexasRetinaAssociates

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PITUITARY ADENOMA PRESENTING ASBILATERAL BULLOUS EXUDATIVERETINAL DETACHMENTS: ACLINICOPATHOLOGIC 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 retinaldetachments and the histopathologic and hematologic evidence of a linkage.

Methods: Record review for a single patient including testing to determine the etiologyof the retinal detachments.

Results: The retinal detachments were determined to be a variant of central serouschorioretinopathy. A pituitary adenoma was discovered by neuroimaging. Histopathologicanalysis of the tumor and serum cortisol levels demonstrated the lesion to be a cortisol-secreting adenoma.

Conclusion: Bilateral serous retinal detachments are a variant of central serous chori-oretinopathy and a rare presentation of pituitary adenoma.

RETINAL CASES & BRIEF REPORTS 3:230–232, 2009

From *Texas Retina Associates, Dallas, Texas; †Lab-oratory of Immunology, National Eye Institute, Be-thesda, Maryland; and ‡Emory Eye Clinic, Atlanta,Georgia.

Central serous chorioretinopathy (CSC) is character-ized by a serous detachment of the neurosensory

retina and/or retinal pigment epithelium, retinal pigmentepithelium leakage, and angiographic retinal pigmentepithelium and choroidal hyperpermeability. The disor-der has been associated with hypercortisolism from bothendogenous and exogenous sources.1–5 This case repre-sents an endogenous source of hypercortisolism, a pitu-itary adenoma, manifesting as bilateral CSC. Bilateralretinal detachments are an uncommon but well describedvariant of CSC.2 A review of the literature found noother cases of pituitary adenoma associated with bilateralserous retinal detachments.

Case Report

A 55-year-old Native American man was referred for evaluationof blurry vision (worse in the left eye) for 2 months. He had beentreated with oral prednisone by the referring physician for possibleVogt-Koyanagi-Harada disease and had finished a tapering sched-ule days before presentation. Subjectively, his symptoms wereworse since stopping prednisone therapy. He denied any discom-fort around the eyes and denied any headaches, tinnitus, difficultyhearing, or neck stiffness. He noticed no skin changes. Recentcomputed 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 and20/40 in the left eye. Intraocular pressure was within normal limits,and results of anterior segment examination were unremarkablebilaterally. No anterior cells were appreciated, but vitreous exam-ination revealed trace cells in the right eye and 1� cells in the lefteye. Several placoid lesions (1 to 2 disk diameters in size) werepresent subretinally in both eyes (Fig. 1). The right fundus hadslight subretinal fluid inferiorly, and the left fundus had a bullousserous retinal detachment inferiorly. No pigment epithelial detach-ments were appreciated. The patient was observed initially withcontinued steroid treatment and further recommendation for MRIof the head.

Six weeks later, MRI had again not been performed, and steroidtreatment yielded no improvement. In fact, the left retinal detach-

Reprint requests: David Callanan, MD, Texas Retina Associates,1001 North Waldrop Drive #512, Arlington, TX 76012; e-mail:dcallanan@texasretina.com

230

ment had progressed into the macula. A consideration of possibleatypical idiopathic CSC prompted a rapid taper and discontinuationof steroid therapy and another admonition to perform MRI.

The patient did not return for his scheduled appointment butreturned 3 months later complaining of double vision and head-aches. A right sixth cranial nerve palsy was noted at examination.In addition, there was an increase of subretinal fluid in the righteye. MRI of the head was again reordered. The patient compliedwith the recommendation, and MRI demonstrated a mass at thesella turcica and right cavernous sinus. Lumbar puncture wasperformed, but cerebrospinal fluid analysis revealed no neoplasticcells. Biopsy of the intracranial mass revealed only necrotic tissue.Vitreous and subretinal fluid biopsy to rule out intraocular lym-phoma was then performed, and the biopsy sample was sent to the

National Eye Institute for analysis, which demonstrated onlychronic inflammation (Fig. 2).

The patient subsequently developed a right third cranial nervepalsy. He was referred to the National Institutes of Health forrepeated biopsy of the intracranial mass, which demonstrated anatypical, adrenocorticotropic hormone–secreting, highly cellularpituitary adenoma (corticotroph cell adenoma) infiltrating fibroustissue and bone (Fig. 3). Systemic hypercortisolism was discov-ered, with highly elevated levels of serum cortisol (42.8 �g/dL),24-hour urine cortisol (896.6 �g/dL), and adrenocorticotropic hor-mone (938.0 pg/mL). The patient underwent radiotherapy for theremaining residual pituitary adenoma but subsequently died due toinfiltrating tumor.

Discussion

This patient presented with the unusual symptomsof mild vitritis and large dependent serous retinal

Fig. 1. Color photograph (A) and angiogram (B) at initial presenta-tion. Fibrinous subretinal lesions are visible in the right eye (A).Multiple focal points of leakage are evident on the angiogram (B).

Fig. 2. Subretinal fluid shows foamy macrophages with pigmentgranules (Giemsa stain; original magnification, �640).

Fig. 3. Brain biopsy shows a noncapsulated mass eroding the sellaturcica. 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 denselygranulated or chromophobic sparsely granulated cytoplasm (PAS stain;upper: original magnification, �100).

231PITUITARY ADENOMA PRESENTING AS BILATERAL BULLOUS EXUDATIVE RETINAL DETACHMENTS

detachments. The differential diagnoses of serousretinal detachment include Vogt-Koyanagi-Haradadisease, choroidal tumors, malignant hypertension,toxemia of pregnancy, and choroidal inflammatorydiseases including sarcoidosis, sympathetic ophthal-mia, posterior scleritis, choroidal mucosa–associatedlymphoid tissue lymphoma, idiopathic uveal effusion,ocular contusion, bilateral diffuse uveal melanocyticproliferation, and CSC.2 The patient’s Native Ameri-can heritage, along with large serous retinal detach-ments and vitritis, supported the initial diagnosis ofVogt-Koyanagi-Harada disease by the referring phy-sician, but no other symptoms were present.

On the basis of the angiographic findings and thelack of improvement with corticosteroid treatment, theidea of atypical CSC was entertained. Although vitri-tis is not typically seen with CSC, the course of thispatient’s retinal detachments has been described byGass as a variant of CSC.2 Chronic dependent serousdetachments can occur, with associated pigmentarydegeneration, retinal capillary loss, vitreous hemor-rhage, and electroretinogram changes.6 In addition,fibrinous exudates can occur in 10% of cases of CSC.7

These exudates are more commonly associated withlarger areas of retinal detachment and more prominentfluorescein leakage. The eventual diagnosis of an ad-renocorticotropic hormone–secreting pituitary ade-noma confirmed the diagnosis of CSC. Unfortunately,the diagnosis was delayed. The first reason for thedelay was the patient noncompliance with the recom-mendation for MRI of the head. The second reasonwas the negative result of the initial pituitary massbiopsy.

The development of CSC due to exogenous and en-dogenous sources of hypercortisolism is well known.1–5

However, a cortisol-secreting pituitary adenoma mani-festing as bilateral serous retinal detachments is not well

documented. Our case is similar to one of the six casesreported by Harada and Harada3 in 1985, although theircase was unilateral. In that case, the patient had alreadyundergone excision of the adenoma. The patient hadpigment migration in the macula but no serous detach-ment. Fluorescein angiography revealed a hyperfluores-cent spot in the macula but no leakage. Three years later,the patient returned with worsening scotoma and a se-rous detachment. Both cases of pituitary adenoma em-phasize the need for neuroimaging when serous retinaldetachments do not fit the characteristics of typical eti-ologies or respond to initial therapy. In addition, deter-mination of serum cortisol levels should be considered inatypical presentations of bilateral retinal detachments.

Key words: central serous chorioretinopathy, centralserous retinopathy, serous retinal detachment, exudativeretinal detachment, pituitary adenoma, hypercortisolism.

References

1. Bouzas EA, Karadimas P, Pournaras CJ. Central serous cho-rioretinopathy and glucocorticoids. Surv Ophthalmol 2002;47:431–448.

2. Gass JDM. Stereoscopic Atlas of Macular Diseases: Diagnosisand Treatment. 4th ed. St. Louis, MO: Mosby; 1997:52–68.

3. Harada T, Harada K. Six cases of central serous choroidopathyinduced by systemic corticosteroid therapy. Doc Ophthalmol1985;60:37–44.

4. Bouzas EA, Scott MH, Mastorakos G, Chrousos GP, Kaiser-Kupfer MI. Central serous chorioretinopathy in endogenoushypercortisolism. Arch Ophthalmol 1993;111:1229–1233.

5. Haimovici R, Koh S, Gagnon DR, et al. Risk factors for centralserous chorioretinopathy: a case-control study. Ophthalmology2004;111:244–249.

6. Gass JD, Little H. Bilateral bullous exudative retinal detach-ment complicating idiopathic central serous chorioretinopathyduring systemic corticosteroid therapy. Ophthalmology 1995;102:737–747.

7. Gass JD. Central serous chorioretinopathy and white subretinalexudation during pregnancy. Arch Ophthalmol 1991;109:677–681.

232 RETINAL CASES & BRIEF REPORTSℜ ● 2009 ● VOLUME 3 ● NUMBER 2