Bilateral Choroiditis from Prototheca Wickerhamii Algaemia
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nal stalk, or retina.2,3 It is a neoplasm traction folds, but no evidence of in- chemical characteristics. Ophthalmology. 1988; 95:1565-1575. of childhood that usually becomes traretinal involvement was present. 8. Orellana J, Moura RA, Font RL, Boniuk M, Mur- clinically symptomatic during the first Our patient had an unusual mass phy D. Medulloepithelioma diagnosed by ul- decade of life (mean age, 5 years).1 that disclosed extensive seeding of trasound and vitreous aspirate: electron mi- croscopic observations. Ophthalmology. 1983; However, there are well-docu- tumor cells along the internal lim- 90:1531-1539. mented cases in which the tumor had iting membrane of the retina with 9. Shields JA, Eagle RC Jr, Shields CL, Potter PD. Congenital neoplasms of the nonpigmented cili- become symptomatic in adult- foci of intraretinal involvement. In ary epithelium (medulloepithelioma). 4,5 hood. The most frequent clinical addition, seedings of tumor cells Ophthalmology. 1996;103:1998-2006. signs are leukocoria; notching or sub- were present along the anterior seg- 10. Shields JA, Eagle RC Jr, Shields CL, Singh AD, Robitaille J. Pigmented medulloepithelioma of luxation of the lens; cataract; and a ment structures, surrounding the the ciliary body. Arch Ophthalmol. 2002;120: mass in the iris, ciliary body, or an- remnants of the anterior lens cap- 207-210. terior chamber. Almost all tumors are sule. We believe it is quite unlikely unilateral. There is no predilection of that the pattern of spread of the tu- this tumor for race, sex, and lateral- mor to the retinal surface and onto ity.6 It also has a strong tendency to the lens capsule is related to the prior Bilateral Choroiditis From induce secondary glaucoma due to cyclectomy specimen. To our knowl- Prototheca wickerhamii iris neovascularization. edge, this is a unique case of medul- Algaemia Broughton and Zimmerman1 es- loepithelioma of ciliary body with tablished the histopathological cri- diffuse intraretinal involvement oc- Algae are a diverse group of eukary- teria for malignancy that includes the curring in a young adult. otic, photosynthetic microbes. They presence of poorly differentiated are not plant, animal, or fungus but Ramon L. Font, MD neuroblastic cells, numerous mito- are—along with molds and mil- ses, pronounced pleomorphism, sar- Kirtee Rishi, MD dews—members of the kingdom Pro- comatous areas, or invasion of other tista. Though generally considered ocular structures with or without ex- Financial Disclosure: None. noninfectious agents in mammals, a traocular extension. Mostly these tu- Correspondence: Dr Font, Ophthal- few algae are pathogenic. One ex- mors are nonpigmented; however, mic Pathology Laboratory, Cullen ample is Prototheca, a nonphotosyn- a few cases of clinically pigmented Eye Institute, Baylor College of thetic alga. Prototheca organisms ex- medulloepithelioma of ciliary body Medicine, Houston, TX 77030 (rfont ist worldwide and are readily isolated have been reported.10 Metastases are @bcm.tmc.edu). from rivers, lakes, ponds, and soil. very rare and usually occur in cases Funding/Support: This study was While protothecosis in canine and with extraocular extension.6 supported in part by grants from the bovine species is common, infec- Immunohistochemically, the Retina Research Foundation, Hous- tion in humans is rare. In the past 25 neuroblastic cells are positive for ton, Tex, and Research to Prevent years, approximately 100 human neuron-specific enolase and synap- Blindness, Inc, New York, NY. Dr cases of protothecosis have been re- tophysin while the spindle cells in Font is the recipient of the Senior In- ported. Most of these cases have been the stroma stain positively for vi- vestigator Award from Research to caused by Prototheca wickerhamii, al- mentin, glial fibrillary acidic pro- Prevent Blindness, Inc. though Prototheca zopfi has been re- tein, and S100 protein.7 Ultrasonog- Acknowledgment: We thank Al- covered from 2 patients.1 raphy and cytologic examination of fonso de la Torre, MD, for provid- The most common site of proto- vitreous aspirate have led to cor- ing the clinical information on this thecosis is the skin, and this may rect pre-enucleation diagnosis of me- patient. manifest as erythema, papulonod- dulloepithelioma in a few cases.8 ules, plaques, and ulcers. Immuno- Shields et al9 concluded that lo- 1. Broughton WL, Zimmerman LE. A clinico- compromised patients tend to have pathologic study of 56 cases of intraocular cal surgical resection (iridocyclec- medulloepitheliomas. Am J Ophthalmol. 1978; more severe lesions that may be vesi- tomy) of the tumor is usually insuf- 85:407-418. cobullous with purulent discharge. ficient, and enucleation ultimately 2. Vadmal M, Kahn E, Finger P, Teichberg S. Disseminated infection such as Nonteratoid medulloepithelioma of the retina will be necessary because of tumor with electron microscopic and immunohisto- peritonitis, endocarditis, urinary recurrence. Six of the 10 patients in chemical characterization. Pediatr Pathol Lab tract infection, or meningitis may Med. 1996;16:663-672. 1 their series were managed by local 3. Green WR, Iliff WJ, Trotter RR. Malignant tera- also occur less commonly. Ocular resection, and 5 eventually re- toid medulloepithelioma of the optic nerve. Arch manifestations of protothecosis quired enucleation, 4 because of lo- Ophthalmol. 1974;91:451-454. have never been reported in a hu- 4. Husain SE, Husain N, Boniuk M, Font RL. cal tumor recurrence. One of the 10 Malignant nonteratoid medulloepithelioma of man; however, several cases have cases in the same series had intrareti- the ciliary body in an adult. Ophthalmology. been reported in animals (primar- nal involvement (J. A. Shields, oral 1998;105:596-599. ily in dogs).2-10 9 5. Carrillo R, Streeten BW. Malignant teratoid me- communication, April 2004). Car- dulloepithelioma in an adult. Arch Ophthalmol. We report what is to our knowl- rillo and Streeten5 reported a case of 1979;97:695-699. edge the first case in the literature 6. Shields JA, Shields CL. Tumors of the nonpig- malignant teratoid medulloepithe- mented ciliary epithelium. In: Shields JA, Shields of bilateral choroiditis due to algae lioma in an adult in which the tu- CL, eds. Intraocular Tumors: A Text and Atlas. in a human. mor had extended over the inner Philadelphia, Pa: WB Saunders Co; 1992:465- 481. retinal surface (in the macular re- 7. Kivela T, Tarkkanen A. Recurrent medulloepi- Report of a Case. Three years prior, gion), producing large retinal con- thelioma of the ciliary body: immunohisto- a 58-year-old white man was diag- (REPRINTED) ARCH OPHTHALMOL / VOL 123, AUG 2005 WWW.ARCHOPHTHALMOL.COM 1138 ©2005 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021 nosed with myelodysplastic syn- drawn 3 days apart. A complete dif- lar corneal epithelium—the constel- drome for which he received che- ferential blood cell count performed lation of which was consistent with motherapy and a bone marrow on the day that the first blood cul- keratoconjunctivitis sicca second- transplant. Over the following year, ture was obtained revealed anemia ary to chronic graft-vs-host disease. he developed chronic graft-vs-host and severe thrombocytopenia (he- Fundus examination results were disease that required systemic matocrit=24.2%, hemoglobin=8.1 normal with no vitritis at this time. immunosuppression. g/dL, platelets=17ϫ103/µL). His Visual acuity measured at the bed- One month prior to ophthalmo- white blood cell count was within the side was moderately decreased to the logic consultation, the patient was ad- normal range at 7.1ϫ103/µL (differ- 20/60 level (with correction) in each mitted to the intensive care unit for ential cell count [normal range]: 94% eye owing to irregular corneal epi- a new skin rash and hepatitis second- neutrophils [44%-74%], 1% bands thelium and mild nuclear sclerotic ary to graft-vs-host disease. His hos- [0%], 1% lymphocytes [0%-2%], 3% cataracts. Aggressive lubrication, topi- cal steroids, and cyclosporine eye pital course was complicated by liver monocytes [4%-14%], and 1% eosin- drops were initiated. failure and neutropenic fevers. His fe- ophils [0%-6%]). The ophthalmol- When the patient was reexam- vers persisted, and he developed sev- ogy department was consulted to ined 1 week later, he had a marked eral vesicobullous lesions on his arms evaluate ocular discomfort on the day improvement in corneal epithelial (Figure 1) and legs over the follow- the first blood culture was obtained. regularity but still complained of ing 2 weeks. Fluid from 1 of the arm Initial examination of both eyes vague ocular discomfort. The visual lesions was aspirated for culture. Ad- was notable for mild conjunctival acuity had dropped to 20/100 in each ditionally, 2 blood cultures were chemosis, icteric sclera, and irregu- eye. Repeat fundus examination re- vealed new bilateral, multiple, slightly raised, whitish-yellow lesions at the level of the retinal pigment epi- thelium with overlying vitritis (Figure 2). Lesion sizes were vari- able, ranging from 100 to 400 µm. Over the course of the week, the blood cultures (Figure 3) as well as cultures from fluid aspirated from the vesicobullous lesion had be- come positive for P wickerhamii. The diagnosis of choroiditis secondary to algaemia was made, and intrave- nous amphotericin B therapy was initiated. Voriconazole and flucona- zole therapy were considered; how- ever, the severity of the patient’s liver disease precluded their use. The patient died 3 days after the second eye examination owing to multiple organ failure. A full body Figure 1. Photograph of a ruptured vesicobullous lesion discovered on the right arm. autopsy was performed after receiv- A B Figure 2. Bedside fundus photographs of right (A) and left (B) eyes. Note multiple, slightly raised, whitish-yellow lesions at the level of the retinal pigment epithelium with overlying vitritis. (REPRINTED) ARCH OPHTHALMOL / VOL 123, AUG 2005 WWW.ARCHOPHTHALMOL.COM 1139 ©2005 American Medical Association.