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Vascular Tumors of the Iris in 45 Patients the 2009 Helen Keller Lecture

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Vascular Tumors of the Iris in 45 Patients the 2009 Helen Keller Lecture CLINICAL SCIENCES Vascular Tumors of the Iris in 45 Patients The 2009 Helen Keller Lecture Jerry A. Shields, MD; Carlos Bianciotto, MD; Brad E. Kligman, BS; Carol L. Shields, MD Objective: To report on a series of vascular tumors of temic involvement occurred in a child. Of the 41 eyes the iris. with iris racemose hemangioma, none showed systemic involvement. Of all 54 eyes, transient hyphema was the Design: Noncomparative case series. A retrospective medi- main complication, found at some point in 30% or more cal record review of all patients with an iris vascular tu- of each affected eye except for iris capillary and race- mor was performed to identify the clinical features and de- mose hemangioma. Surgical resection was necessary in velop a simple classification of these lesions. Included were 1 cavernous hemangioma and 1 varix. The remainder were demographics, clinical features, systemic associations, com- managed with observation. plications, management, and histopathology. Conclusions: There are now well-documented ex- Results: There were 54 eyes in 45 patients with an iris amples of iris racemose hemangioma, cavernous heman- vascular tumor. These were categorized as racemose he- gioma, capillary hemangioma, varix, and microheman- mangioma (41 eyes: 29 simple and 12 complex), cav- giomatosis. Transient hyphema is the main complication. ernous hemangioma (3 eyes: 2 localized and 1 sys- Observation is usually advised. Most are solitary lesions temic), capillary hemangioma (1 eye, localized), varix (3 confined to the iris and some (cavernous hemangioma eyes, localized), and microhemangiomatosis (6 eyes, and microhemangiomatosis) can have important sys- localized). The hemangiomas occurred in adults at a temic associations. median age of 55 years, whereas capillary hemangioma occurred in infancy and cavernous hemangioma with sys- Arch Ophthalmol. 2010;128(9):1107-1113 HE EXISTENCE OF TRUE IRIS In our literature review and personal vascular tumors was once experience, there have been genuine questioned. In 1972, cases of iris vascular tumors and there Ferry1 reviewed reported are some that remain controversial.2-52 cases of histopathologi- In a recent case report of an iris varix, Tcally proven iris and ciliary body heman- Broaddus and associates3 provided a giomas on file at the Armed Forces Insti- review on iris vascular tumors. The pur- tute of Pathology and other sources. He pose of this contribution is to describe a came to the surprising conclusion that series of iris vascular tumors seen on an all cases had been misdiagnosed histo- ocular oncology service and to review pathologically and they actually repre- the English language literature of the sented vascular melanoma, juvenile subject. xanthogranuloma, florid iris neovascu- larization, or other lesions.1 He raised METHODS doubts about the existence of iris hemangioma and proposed that most reported cases in humans were not hem- This study was approved by the institutional angiomas. Since that time, however, review board of the Wills Eye Institute of there have been several reports of iris Thomas Jefferson University. A classification 2-34 was designed as listed below. Although not all hemangiomas. of these conditions represent true tumors in the Author Affiliations: Oncology Since the inception of our oncology classic sense of a distinct mass, they were in- Service Wills Eye Institute, service in 1974, we have coded and filed cluded in the classification of vascular tumors Thomas Jefferson University, all cases of iris vascular tumors and in this communication, in keeping with clas- Philadelphia, Pennsylvania. pseudotumors examined by our team. sic usage in the literature. (REPRINTED) ARCH OPHTHALMOL / VOL 128 (NO. 9), SEP 2010 WWW.ARCHOPHTHALMOL.COM 1107 ©2010 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/28/2021 Table 1. Patients With Vascular Tumors of the Iris Table 3. Referring Diagnosis in 54 Eyes of 45 Patients With Iris Hemangioma No. Referring Diagnosis No. (%) Tumor Type Patients Eyes Bilateral Iris Racemose 35 41 6 Hemangioma 8 (15) Cavernous 3 3 0 Melanoma 2 (4) Capillary 1 1 0 Nevus 5 (9) Varix 3 3 0 Cyst 1 (2) Microhemangiomatosis 3 6 3 Prominent vessels nonspecific 6 (11) Total 45 54 9 Ciliary body melanoma 2 (4) Choroidal melanoma 2 (4) No diagnosis submitted 27 (50) Table 2. Vascular Tumors of the Iris by Category RESULTS Tumor Type No. of Eyes Racemose 41 There were 54 eyes in 45 patients with an iris vascular Simple 29 tumor (Table 1). The tumors were classified as race- Complex 12 mose hemangioma in 41 eyes of 35 patients (29 simple Cavernous 3 and 12 complex), cavernous hemangioma in 3 eyes of 3 Localized 2 Systemic 1 patients (2 localized and 1 systemic), capillary heman- Capillary 1 gioma in 1 eye of 1 patient, localized varix in 3 eyes of 3 Localized 1 patients, and microhemangiomatosis in 6 eyes of 3 pa- Systemic 0 tients (Table 2). Histopathologic confirmation of the Varix 3 diagnosis was available in a single case of solitary cav- Localized 3 ernous hemangioma and a single case of varix, both fol- Systemic 0 Microhemangiomatosis 6 lowing removal of the lesion by iridectomy. The referral Localized 6 diagnoses are shown in Table 3. The diagnosis of iris Systemic 0 hemangioma was suspected in 8 of the 54 eyes and no Total 54 diagnosis was submitted in 27 (50%) cases. Uveal mela- noma (iris, ciliary body, and choroid) was the referral diagnosis in 6 cases. Based on our prior observations and literature review, we The demographic data are shown in Table 4. The me- classified iris vascular tumors and pseudotumors into the fol- dian patient age was 55 years at original examination in lowing categories: all cases with the exception of iris capillary hemangioma 1. Racemose hemangioma (0.1 year) and cavernous hemangioma with systemic in- Simple volvement (7 years). All but 1 tumor was found in white Complex patients. The iris color was blue or green in 48 cases (89%). 2. Cavernous hemangioma The intraocular pressure was under 22 mm Hg except for Localized 1 patient with open-angle glaucoma and a pressure of 37 Systemic mm Hg. Ectropion was absent in all cases. Corectopia was 3. Capillary hemangioma Localized found in 1 case of iris varix and 1 case of solitary cavern- Systemic ous hemangioma. Individual tumor features are shown in 4. Varix Table 4 and Table 5, and specific information per tumor Localized classification will be discussed. Systemic Racemose hemangioma (arteriovenous communica- 5. Microhemangiomatosis tion) was the most common lesion in our series, account- Localized ing for 35 of the 45 patients. In 29 patients, it was uni- Systemic lateral and in 6 patients bilateral. It appeared as a solitary Using this classification, a retrospective medical record review was large tortuous iris blood vessel, approximately twice the conducted on all patients coded with 1 of these diagnoses, and normal caliber and located within the iris stroma. The the patient, ocular, and tumor features were evaluated. The pa- racemose hemangioma intertwined in a sinuous fashion tient features that were recorded included age at diagnosis, race, within the iris stroma from iris root to the pupillary bor- sex, symptoms, and presence of related systemic hemangiomas der and then back to the root.8-14 In 29 patients, the ra- or syndromes. The ocular features recorded included iris color, cemose hemangioma was classified as simple, character- visual acuity, and intraocular pressure. The tumor features that ized by a single tortuous vessel (Figure 1A and B). Six were recorded included referring diagnosis, tumor quadrant lo- cation, size (base in millimeters and clock-hours), blood vessel of the simple racemose hemangiomas were bilateral. In feeding from the sclera or iris, and intrinsic blood vessels. Re- 12 cases, it was classified as complex, characterized by lated ocular findings such as glaucoma, cataract, hyphema, and multiple ramifying, intertwining blood vessels forming corneal changes were listed. Medical and surgical intervention a sessile vascular mass (Figure 1C and D).13 The com- were reviewed and the ultimate outcome was listed. plex cases were all unilateral. Racemose hemangioma was (REPRINTED) ARCH OPHTHALMOL / VOL 128 (NO. 9), SEP 2010 WWW.ARCHOPHTHALMOL.COM 1108 ©2010 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/28/2021 Table 4. Iris Hemangioma in 54 Eyes of 45 Patients No. (%) by Hemangioma Type Racemose Cavernous Capillary, Microhemangiomatosis, Simple Complex Solitary Systemic Localized Varix Localized Characteristic (n=29) (n=12) (n=2) (n=1) (n=1) (n=3) (n=6) Age, median (mean, range), ya 56 (61, 30-82) 55 (52, 28-82) 56 (56, 55-56) 7 (7, 7-7) 0.1 (0.1, 0.1-0.1) 59 (57, 39-78) 70 (70, 66-73) Racea White 23 (51) 12 (27) 2 (4) 1 (2) 1 (2) 2 (4) 3 (7) African American 0 0 0 0 0 0 0 Hispanic 0 0 0 0 0 1 (2) 0 Asian 0 0 0 0 0 0 0 Sexa F 14 (31) 5 (11) 1 (2) 1 (2) 1 (2) 0 3 (7) M 9 (20) 7 (16) 1 (2) 0 0 3 (7) 0 Iris colorb Blue 24 (44) 10 (19) 2 (4) 1 (2) 1 (2) 1 (2) 6 (11) Green 2 (4) 1 (2) 0 0 0 0 0 Brown 2 (4) 0 0 0 0 2 (4) 0 Hazel 1 (2) 1 (2) 0 0 0 0 0 Visual acuityb 20/20-20/40 25 (46) 12 (22) 2 (4) 1 (2) 0 3 (6) 6 (11) 20/50-20/100 4 (7) 0 0 0 0 0 0 20/200-HM 0 0 0 0 1 (2) 0 0 Intraocular pressure, median 24 (16, 10-37) 15 (15, 10-19) 18 (18, 12-23) FTNc FTNd 17 (17, 15-19) 15 (14, 12-17) (mean, range), mm Hgb Abbreviations: FTN, finger tension normal; HM, hand motions.
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