[Skip to Content]
Access to paid content on this site is currently suspended due to excessive activity being detected from your IP address 54.167.149.128. Please contact the publisher to request reinstatement.
[Skip to Content Landing]
Download PDF
Figure 1.
Clinical and angiographic features of vascular tumors of the iris. A, Clinical appearance of simple racemose hemangioma of iris. B, Fluorescein angiogram of lesion shown in A, better depicting the dilated, tortuous blood vessels. There is a sparsity of blood vessels in the area between the 2 components of the lesion. C, Clinical appearance of complex racemose hemangioma of iris. D, Fluorescein angiogram of lesion shown in C, depicting a more complex arrangement, tortuous blood vessels, forming a vascular mass. Again, there is sparsity of blood vessels in the area between the 2 components of the lesion. E, Cavernous hemangioma of the iris inferiorly in the right eye. The temporal iris nevus is coincidental and unrelated. The vascular lesion was relatively hypofluorescent throughout the angiogram, suggesting minimal blood flow through the tumor. F, Goniophotograph of lesion shown in E, showing the typical blood-filled cavernous vessels. G, Iris cavernous hemangioma adjacent to the pupil, causing slight peaking of the pupil. The lesion was removed by sector iridectomy. H, Photomicrograph of lesion shown in G. There are large blood-filled cavernous channels lined by endothelial cells.

Clinical and angiographic features of vascular tumors of the iris. A, Clinical appearance of simple racemose hemangioma of iris. B, Fluorescein angiogram of lesion shown in A, better depicting the dilated, tortuous blood vessels. There is a sparsity of blood vessels in the area between the 2 components of the lesion. C, Clinical appearance of complex racemose hemangioma of iris. D, Fluorescein angiogram of lesion shown in C, depicting a more complex arrangement, tortuous blood vessels, forming a vascular mass. Again, there is sparsity of blood vessels in the area between the 2 components of the lesion. E, Cavernous hemangioma of the iris inferiorly in the right eye. The temporal iris nevus is coincidental and unrelated. The vascular lesion was relatively hypofluorescent throughout the angiogram, suggesting minimal blood flow through the tumor. F, Goniophotograph of lesion shown in E, showing the typical blood-filled cavernous vessels. G, Iris cavernous hemangioma adjacent to the pupil, causing slight peaking of the pupil. The lesion was removed by sector iridectomy. H, Photomicrograph of lesion shown in G. There are large blood-filled cavernous channels lined by endothelial cells.

Figure 2.
Further clinical and angiographic features of vascular tumors of the iris. A, Capillary hemangioma in an infant, showing involvement of the face and eyelid. Reprinted with permission from Slack Inc. B, Anterior segment of child seen in A, showing capillary hemangioma involving superotemporal quadrant of the iris. The skin and iris lesion both subsequent spontaneous regression. Reprinted with permission from Slack Inc. C, Iris varix located in iris stroma adjacent to the pupil. D, Fluorescein angiogram of lesion shown in C, revealing that the varix is hypofluorescent with slight leakage of dye from the adjacent iris vessels. E, Thrombosed iris varix. The brown homogeneous lesion temporal to the pupil is in the iris stroma, some overlying rarification of the more superficial stroma. The lesion was removed by sector iridectomy. F, Histopathology of lesion shown in E, demonstrating a large blood clot. It was lined by thin endothelial cells. G, Microhemangiomatosis. There are numerous small vascular tufts at the pupillary margin that are difficult to visualize and photograph. They are barely visible at 7- and 11-o’clock. H, Fluorescein angiogram of lesion shown in G, revealing numerous hyperfluorescent lesions at the pupillary border.

Further clinical and angiographic features of vascular tumors of the iris. A, Capillary hemangioma in an infant, showing involvement of the face and eyelid. Reprinted with permission from Slack Inc.24 B, Anterior segment of child seen in A, showing capillary hemangioma involving superotemporal quadrant of the iris. The skin and iris lesion both subsequent spontaneous regression. Reprinted with permission from Slack Inc.24 C, Iris varix located in iris stroma adjacent to the pupil. D, Fluorescein angiogram of lesion shown in C, revealing that the varix is hypofluorescent with slight leakage of dye from the adjacent iris vessels. E, Thrombosed iris varix. The brown homogeneous lesion temporal to the pupil is in the iris stroma, some overlying rarification of the more superficial stroma. The lesion was removed by sector iridectomy. F, Histopathology of lesion shown in E, demonstrating a large blood clot. It was lined by thin endothelial cells. G, Microhemangiomatosis. There are numerous small vascular tufts at the pupillary margin that are difficult to visualize and photograph. They are barely visible at 7- and 11-o’clock. H, Fluorescein angiogram of lesion shown in G, revealing numerous hyperfluorescent lesions at the pupillary border.

Table 1. 
Patients With Vascular Tumors of the Iris
Patients With Vascular Tumors of the Iris
Table 2. 
Vascular Tumors of the Iris by Category
Vascular Tumors of the Iris by Category
Table 3. 
Referring Diagnosis in 54 Eyes of 45 Patients With Iris Hemangioma
Referring Diagnosis in 54 Eyes of 45 Patients With Iris Hemangioma
Table 4. 
Iris Hemangioma in 54 Eyes of 45 Patients
Iris Hemangioma in 54 Eyes of 45 Patients
Table 5. 
Tumor Features in 54 Eyes of 45 Patients With Iris Hemangioma
Tumor Features in 54 Eyes of 45 Patients With Iris Hemangioma
1.
Ferry  AP Hemangiomas of the iris and ciliary body: do they exist? a search for a histologically proved case. Int Ophthalmol Clin 1972;12 (1) 177- 194
PubMed
2.
Shields  JAShields  CL Intraocular Tumors: An Atlas and Textbook. Vol 2. Philadelphia, PA Lippincott Williams & Wilkins2008;256- 261
3.
Broaddus  ELystad  LDSchonfield  LSingh  AD Iris varix: report of a case and review of iris vascular anomalies. Surv Ophthalmol 2009;54 (1) 118- 127
PubMedArticle
4.
Ashton  N Primary tumours of the iris. Br J Ophthalmol 1964;48650- 668
PubMedArticle
5.
Amasio  EBrovarone  FVMusso  M Angioma of the iris. Ophthalmologica 1980;180 (1) 15- 18
PubMedArticle
6.
Spencer  WHFerguson  WJ  Jr Hemangioma of the iris. Arch Ophthalmol 1963;70811- 812
PubMedArticle
7.
Stallard  HB Haemangioma of the iris. Br J Ophthalmol 1966;50 (7) 434- 435
PubMedArticle
8.
Perkins  SAMagargal  LE Arteriovenous malformations of the iris. Ann Ophthalmol 1985;17 (11) 679- 680, 682, 685
PubMed
9.
de Corral  LRConway  MPeyman  GAConstanteras  A Argon laser treatment of an abnormal angle vessel producing recurrent hyphema. Int Ophthalmol 1985;8 (3) 179- 182
PubMedArticle
10.
Prost  M Arteriovenous communication of the iris. Br J Ophthalmol 1986;70 (11) 856- 859
PubMedArticle
11.
Parodi  MBBondel  ESaviano  SDa Pozzo  SBergamini  LRavalico  G Iris arteriovenous communication: clinical and angiographic features. Int Ophthalmol 1998;22 (1) 1- 5
PubMedArticle
12.
Shields  JAShields  CLO'Rourk  T Racemose hemangioma of the iris. Br J Ophthalmol 1996;80 (8) 770- 771
PubMedArticle
13.
Shields  JAStreicher  TFESpirkova  JHJStubna  MShields  CL Arteriovenous malformation of the iris in 14 cases. Arch Ophthalmol 2006;124 (3) 370- 375
PubMedArticle
14.
Lee  BJJeng  BHSingh  AD OCT and ultrasound biomicroscopic findings in iris arteriovenous malformation. Ophthalmic Surg Lasers Imaging 2008;39 (5) 426- 428
PubMedArticle
15.
Prost  M Cavernous hemangioma of the iris. Ophthalmologica 1987;195 (4) 183- 187
PubMedArticle
16.
Lam  S Iris cavernous hemangioma in a patient with recurrent hyphema. Can J Ophthalmol 1993;28 (1) 36- 39
PubMed
17.
Shields  JAShields  CLEagle  RC  Jr Cavernous hemangioma of the iris. Arch Ophthalmol 2008;126 (11) 1602- 1603
PubMedArticle
18.
Larson  SAOetting  TA Presumed iris hemangioma associated with multiple central nervous system cavernous hemangiomas. Arch Ophthalmol 2002;120 (7) 984- 985
PubMed
19.
Thangappan  AShields  CLDinowitz  MShields  JA Iris cavernous hemangioma associated with multiple cavernous hemangiomas in the kidney, brain, and skin. Cornea 2007;26 (4) 481- 483
PubMedArticle
20.
Naidoff  MAKenyon  KRGreen  WR Iris hemangioma and abnormal retinal vasculature in a case of diffuse congenital hemangiomatosis. Am J Ophthalmol 1971;72 (3) 633- 644
PubMed
21.
Weiss  MJErnest  JT Diffuse congenital hemangiomatosis with infantile glaucoma. Am J Ophthalmol 1976;81 (2) 216- 218
PubMed
22.
Ebenezer  GJDaniel  EJob  CK Cavernous haemangioma of the iris in a leprosy patient. Br J Ophthalmol 1997;81 (7) 610- 612
PubMedArticle
23.
Cashell  GT Angioma of the iris. Br J Ophthalmol 1967;51 (9) 633- 635
PubMedArticle
24.
Ruttum  MSMittelman  DSingh  P Iris hemangiomas in infants with periorbital capillary hemangiomas. J Pediatr Ophthalmol Strabismus 1993;30 (5) 331- 333
PubMed
25.
Woo  SJKim  CJYu  YS Cavernous hemangioma of the iris in an infant. J AAPOS 2004;8 (5) 499- 501
PubMedArticle
26.
Winnick  MMargalit  ESchachat  APStark  WJ Treatment of vascular tufts at the pupillary margin before cataract surgery. Br J Ophthalmol 2003;87 (7) 920- 921
PubMedArticle
27.
Chang  CWRao  NAStout  JT Histopathology of the eye in diffuse neonatal hemangiomatosis. Am J Ophthalmol 1998;125 (6) 868- 870
PubMedArticle
28.
Bryce  IGPai  VBradbury  JA Spontaneous resolution of iris and cutaneous haemangiomata in diffuse neonatal haemangiomatosis. Eye (Lond) 1999;13 (pt 3a) 388- 390
PubMedArticle
29.
Haik  BGClancy  PEllsworth  RMPerina  AZimmerman  K Ocular manifestations in diffuse neonatal hemangiomatosis. J Pediatr Ophthalmol Strabismus 1983;20 (3) 101- 105
PubMed
30.
Shields  JAShields  CLPulido  JEagle  RC  JrNothnagel  AF Iris varix simulating an iris melanoma. Arch Ophthalmol 2000;118 (5) 707- 710
PubMedArticle
31.
Andersen  SROther  A Varix of the iris. Arch Ophthalmol 1975;93 (1) 32- 33
PubMedArticle
32.
Küchle  MNaumann  GOH Varix of the iris spontaneous regression. Klin Monbl Augenheilkd 1992;200 (3) 233- 236
PubMedArticle
33.
Ang  LPSim  DHChiang  GSYong  VS Iris varix. Eye (Lond) 1997;11 (pt 5) 733- 735
PubMedArticle
34.
Rohrbach  JMEckstein  ASchuster  I Varicose vein of the iris. Klin Monbl Augenheilkd 1995;207 (3) 206- 207
PubMedArticle
35.
Rosen  ELyons  D Microhemangiomas at the pupillary border demonstrated by fluorescein photography. Am J Ophthalmol 1969;67 (6) 846- 853
PubMed
36.
Sellman  A Hyphaema from microhaemangiomas. Acta Ophthalmol (Copenh) 1972;50 (1) 58- 61
PubMedArticle
37.
Israel  MPLorenzetti  DW Bilateral microhemangiomas of the pupillary border with later hyphema. Can J Ophthalmol 1974;9 (1) 138- 140
PubMed
38.
Perry  HDMallen  FJSussman  W Microhaemangiomas of the iris with spontaneous hyphaema and acute glaucoma. Br J Ophthalmol 1977;61 (2) 114- 116
PubMedArticle
39.
Coleman  SLGreen  WRPatz  A Vascular tufts of pupillary margin of iris. Am J Ophthalmol 1977;83 (6) 881- 883
PubMed
40.
Mason  GIFerry  AP Bilateral spontaneous hyphema arising from iridic microhemangiomas. Ann Ophthalmol 1979;11 (1) 87- 91
PubMed
41.
Francis  ICKappagoda  MB Iris microhaemangiomas. Aust J Ophthalmol 1982;10 (3) 167- 171
PubMedArticle
42.
Meades  KVFrancis  ICKappagoda  MBFilipic  M Light microscopic and electron microscopic histopathology of an iris microhaemangioma. Br J Ophthalmol 1986;70 (4) 290- 294
PubMedArticle
43.
Thomas  RAylward  GMBillson  FA Spontaneous hyphaema from an iris microhaemangioma. Aust N Z J Ophthalmol 1988;16 (4) 367- 368
PubMedArticle
44.
Bandello  FBrancato  RLattanzio  RMaestranzi  G Laser treatment of iris vascular tufts. Ophthalmologica 1993;206 (4) 187- 191
PubMedArticle
45.
Ah-Fat  FGCanning  CR Recurrent visual loss secondary to an iris microhaemangioma. Eye (Lond) 1994;8 (pt 3) 357
PubMedArticle
46.
Akram  IReck  ACSheldrick  J Iris microhaemangioma presenting with total hyphaema and elevated intraocular pressure. Eye (Lond) 2003;17 (6) 784- 785
PubMedArticle
47.
Strauss  ECAldave  AJSpencer  WH  et al.  Management of prominent iris vascular tufts causing recurrent spontaneous hyphema. Cornea 2005;24 (2) 224- 226
PubMedArticle
48.
Bakke  EFDrolsum  L Iris microhaemangiomas and idiopathic juxtafoveolar retinal telangiectasis. Acta Ophthalmol Scand 2006;84 (6) 818- 822
PubMedArticle
49.
Cobb  B Vascular tufts at the pupillary margin: a preliminary report on 44 patients. Trans Ophthalmol Soc U K 1968;88211- 221
50.
Cobb  BShilling  JSChisholm  IH Vascular tufts at the pupillary margin in myotonic dystrophy. Am J Ophthalmol 1970;69 (4) 573- 582
PubMed
51.
Rodin  FH Angioma of the iris: first case to be reported with histologic examination. Arch Ophthal 1929;2679- 682Article
52.
Savir  HManor  RS Spontaneous hyphema and vessel anomaly. Arch Ophthalmol 1975;93 (10) 1056- 1058
PubMedArticle
Clinical Sciences
Sepetmber 2010

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

Author Affiliations

Author Affiliations: Oncology Service Wills Eye Institute, Thomas Jefferson University, Philadelphia, Pennsylvania.

Arch Ophthalmol. 2010;128(9):1107-1113. doi:10.1001/archophthalmol.2010.188
Abstract

Objective  To report on a series of vascular tumors of the iris.

Design  Noncomparative case series. A retrospective medical record review of all patients with an iris vascular tumor was performed to identify the clinical features and develop a simple classification of these lesions. Included were demographics, clinical features, systemic associations, complications, management, and histopathology.

Results  There were 54 eyes in 45 patients with an iris vascular tumor. These were categorized as racemose hemangioma (41 eyes: 29 simple and 12 complex), cavernous hemangioma (3 eyes: 2 localized and 1 systemic), capillary hemangioma (1 eye, localized), varix (3 eyes, localized), and microhemangiomatosis (6 eyes, localized). The hemangiomas occurred in adults at a median age of 55 years, whereas capillary hemangioma occurred in infancy and cavernous hemangioma with systemic involvement occurred in a child. Of the 41 eyes with iris racemose hemangioma, none showed systemic involvement. Of all 54 eyes, transient hyphema was the main complication, found at some point in 30% or more of each affected eye except for iris capillary and racemose hemangioma. Surgical resection was necessary in 1 cavernous hemangioma and 1 varix. The remainder were managed with observation.

Conclusions  There are now well-documented examples of iris racemose hemangioma, cavernous hemangioma, capillary hemangioma, varix, and microhemangiomatosis. Transient hyphema is the main complication. Observation is usually advised. Most are solitary lesions confined to the iris and some (cavernous hemangioma and microhemangiomatosis) can have important systemic associations.

The existence of true iris vascular tumors was once questioned. In 1972, Ferry1 reviewed reported cases of histopathologically proven iris and ciliary body hemangiomas on file at the Armed Forces Institute of Pathology and other sources. He came to the surprising conclusion that all cases had been misdiagnosed histopathologically and they actually represented vascular melanoma, juvenile xanthogranuloma, florid iris neovascularization, or other lesions.1 He raised doubts about the existence of iris hemangioma and proposed that most reported cases in humans were not hemangiomas. Since that time, however, there have been several reports of iris hemangiomas.234

Since the inception of our oncology service in 1974, we have coded and filed all cases of iris vascular tumors and pseudotumors examined by our team. In our literature review and personal experience, there have been genuine cases of iris vascular tumors and there are some that remain controversial.252 In a recent case report of an iris varix, Broaddus and associates3 provided a review on iris vascular tumors. The purpose of this contribution is to describe a series of iris vascular tumors seen on an ocular oncology service and to review the English language literature of the subject.

METHODS

This study was approved by the institutional review board of the Wills Eye Institute of Thomas Jefferson University. A classification was designed as listed below. Although not all of these conditions represent true tumors in the classic sense of a distinct mass, they were included in the classification of vascular tumors in this communication, in keeping with classic usage in the literature.

Based on our prior observations and literature review, we classified iris vascular tumors and pseudotumors into the following categories:

  • Racemose hemangioma

    Simple

    Complex

  • Cavernous hemangioma

    Localized

    Systemic

  • Capillary hemangioma

    Localized

    Systemic

  • Varix

    Localized

    Systemic

  • Microhemangiomatosis

    Localized

    Systemic

Using this classification, a retrospective medical record review was conducted on all patients coded with 1 of these diagnoses, and the patient, ocular, and tumor features were evaluated. The patient features that were recorded included age at diagnosis, race, sex, symptoms, and presence of related systemic hemangiomas or syndromes. The ocular features recorded included iris color, visual acuity, and intraocular pressure. The tumor features that were recorded included referring diagnosis, tumor quadrant location, size (base in millimeters and clock-hours), blood vessel feeding from the sclera or iris, and intrinsic blood vessels. Related ocular findings such as glaucoma, cataract, hyphema, and corneal changes were listed. Medical and surgical intervention were reviewed and the ultimate outcome was listed.

RESULTS

There were 54 eyes in 45 patients with an iris vascular tumor (Table 1). The tumors were classified as racemose hemangioma in 41 eyes of 35 patients (29 simple and 12 complex), cavernous hemangioma in 3 eyes of 3 patients (2 localized and 1 systemic), capillary hemangioma in 1 eye of 1 patient, localized varix in 3 eyes of 3 patients, and microhemangiomatosis in 6 eyes of 3 patients (Table 2). Histopathologic confirmation of the diagnosis was available in a single case of solitary cavernous hemangioma and a single case of varix, both following removal of the lesion by iridectomy. The referral diagnoses are shown in Table 3. The diagnosis of iris hemangioma was suspected in 8 of the 54 eyes and no diagnosis was submitted in 27 (50%) cases. Uveal melanoma (iris, ciliary body, and choroid) was the referral diagnosis in 6 cases.

The demographic data are shown in Table 4. The median patient age was 55 years at original examination in all cases with the exception of iris capillary hemangioma (0.1 year) and cavernous hemangioma with systemic involvement (7 years). All but 1 tumor was found in white patients. The iris color was blue or green in 48 cases (89%). The intraocular pressure was under 22 mm Hg except for 1 patient with open-angle glaucoma and a pressure of 37 mm Hg. Ectropion was absent in all cases. Corectopia was found in 1 case of iris varix and 1 case of solitary cavernous hemangioma. Individual tumor features are shown in Table 4 and Table 5, and specific information per tumor classification will be discussed.

Racemose hemangioma (arteriovenous communication) was the most common lesion in our series, accounting for 35 of the 45 patients. In 29 patients, it was unilateral and in 6 patients bilateral. It appeared as a solitary large tortuous iris blood vessel, approximately twice the normal caliber and located within the iris stroma. The racemose hemangioma intertwined in a sinuous fashion within the iris stroma from iris root to the pupillary border and then back to the root.814 In 29 patients, the racemose hemangioma was classified as simple, characterized by a single tortuous vessel (Figure 1A and B). Six of the simple racemose hemangiomas were bilateral. In 12 cases, it was classified as complex, characterized by multiple ramifying, intertwining blood vessels forming a sessile vascular mass (Figure 1C and D).13 The complex cases were all unilateral. Racemose hemangioma was better visualized in a blue iris than a brown one. Fluorescein angiography clearly depicted the abnormal blood vessels with sparse vascularity in the adjacent iris and no leakage (Figure 1B and Figure 2B). A dilated, slightly tortuous episcleral blood vessel, termed sentinel vessel, was visible in the limbal sclera at the entry point of the hemangioma in the iris root in 22 of the 35 affected eyes.13 All racemose hemangiomas remained stable on follow-up examinations. Only 1 racemose hemangioma produced hyphema. This occurred twice, spontaneously and transiently, leaving no complications in a 40-year-old healthy woman.

There were 3 patients (3 eyes) coded as iris cavernous hemangioma, 2 of which were solitary lesions localized to the iris in adults and 1 in a child who had additional brain, kidney, and skin hemangiomatosis.19 Two of the cavernous hemangiomas produced multiple recurrent hyphemas. In each case, it appeared as a multilobular blue-red iris stromal mass (Figure 1E and F), with features identical to the well-known orbital cavernous hemangioma. One localized lesion was removed by iridectomy and confirmed histopathologically after more than 30 hyphemas during 18 years (Figure 1G and H).17

There was 1 patient coded as having an iris capillary hemangioma in our series. An infant girl was referred for a red iris lesion in her right eye that was first noted by her parents at age 4 weeks. Our examination results were entirely normal except for a 3-mm diameter red lesion inferiorly in the iris stroma of her right eye. There was no distinct periocular capillary hemangioma, but a few prominent dilated blood vessels were present on the right upper eyelid. The iris lesion was purely vascular and did not resemble a juvenile xanthogranuloma. The iris and skin lesions were subsequently documented by the referring pediatric ophthalmologist to have disappeared within 6 weeks. Although photographs were not possible in our case, iris capillary hemangioma is well documented in the literature (Figure 2A and B).

Three of our cases, all of which were in adults, were classified as iris varix, each of which was considered to be a thrombosed varix. The varix appeared in the iris stroma as a dark brown mass that resembled a blood clot and showed hypofluorescence on fluorescein angiography (Figure 2C and D). Two of the patients had transient hyphema preceding our examination, one of which achieved intraocular pressure of 52 mm Hg. In 1 case, varix removal by iridectomy confirmed the diagnosis (Figure 2E and F).30

The series included 3 patients with microhemangiomatosis. It was bilateral and characterized by multiple dilated vascular channels arranged circumferentially around the pupillary margin. The microhemangiomatoses were hyperfluorescent with angiography and showed leakage of fluorescein into the aqueous humor (Figure 2G and H).

COMMENT

Ferry's publication1 in 1972 temporarily raised doubts as to the existence of iris hemangiomas. In fact, he titled his article “Hemangiomas of the Iris and Ciliary Body: Do They Exist? A Search for a Histologically Proved Case.” In his meticulous histopathologic study of reported cases, he either disproved or rejected virtually every reported and unreported case on file at the Armed Forces Institute of Pathology.

The high rate of misdiagnosis in Ferry's series is understandable when one considers the histopathology of the most frequently misdiagnosed conditions in his review, namely iris melanoma in adults and iris juvenile xanthogranuloma in children. Iris melanoma often clinically and histopathologically contains large cavernous blood vessels that may be so prominent that the pathologist can easily overlook more subtle spindle melanoma cells and make the erroneous diagnosis of hemangioma. Similarly, juvenile xanthogranuloma is often highly vascular such that the pathologist may overlook the granulomatous inflammation and make the erroneous diagnosis of hemangioma.

It is clearly established that vascular tumors occur in other ocular structures, including the eyelid, conjunctiva, orbit, choroid, and retina, and hence similar lesions would be expected to occur in the iris. Judged from subsequent personal experience and scrutiny of the literature, it is now evident that vascular tumors of the iris also exist.

There are shortcomings in determining the types and frequency of the vascular tumors of the iris. First, reports in the literature of iris hemangiomas are often confusing and not well documented. Some authors have reported cases of iris “angiomas” or “hemangiomas” but have not specified the type of vascular tumor.5,8,1012 Second, most reported cases have not had histopathologic confirmation of the diagnosis. Third, even when histopathologic material is available, it may be extremely difficult in some cases to differentiate cavernous hemangioma from capillary hemangioma from varix and other lesions, because there may be histopathologic overlap of these lesions.1,47 Fourth, some skeptics might challenge the diagnosis of hemangioma to define lesions like racemose hemangioma and microhemangiomatosis in which there is not a distinct mass. However, such lesions have traditionally been defined as hemangiomas in other parts of the eye and elsewhere in the body and it seems reasonable, for purposes of the communication, to do the same for iris vascular tumors. Hence, we will discuss the features of iris vascular lesion using our classification. A similar variation of this classification is used in a recent textbook2 and a recent literature review.3

By far, the most common vascular lesion in our series was racemose hemangioma (arteriovenous communication). The clinical features of this iris lesion are described in the “Results” section and in the recent literature.814 Until recently, there were only a few reported cases. In a report of 14 cases in 2006, the authors emphasized that iris racemose hemangioma may be more common than previously believed, but it may be subtle and difficult to recognize clinically, particularly in a brown iris.13 Since that publication, we have seen more referrals for this lesion, perhaps owing to increased clinical awareness. Fluorescein angiography can dramatically depict the full extent of the lesion. It tends to occur in asymptomatic adults, some of whom have diabetes or hypertension, though no predisposing factors have been identified. None of the affected patients in our series have experienced hyphema or other complications. In 1 recorded case of bleeding from an angle vessel, the lesion did not appear to meet our anatomic criteria for a racemose hemangioma.9 Iris racemose hemangioma has no recognized association with the retinal and brain racemose hemangioma seen with the Wyburn-Mason syndrome.13 An interesting association with iris racemose hemangioma is a contiguous dilated episcleral blood vessel seen in 22 of the 41 eyes in our series. This raises concerns for an underlying ciliary body melanoma, which was not present in any of our cases.13

Cavernous hemangioma is rare, accounting for only 3 eyes in 3 patients in our series. A review of the literature reveals few well-documented cases.1519 Two of our 3 cases were localized to the iris and 1 was part of a systemic cavernous hemangiomatosis. Concerning the localized type, it appears as a fairly well-circumscribed grapelike mass composed of large blue-red cavernous blood vessels that are remarkably similar grossly and histopathologically to the better known cavernous hemangioma of the orbit (Figure 1E and G). Our review of the case reported by Prost15 suggests that the lesion may be microhemangiomatosis (discussed subsequently) and not a true cavernous hemangioma, based on clinical images and fluorescein angiograms. A case reported by Lam16 may be a cavernous hemangioma, but the images also suggest a possible thrombosed varix. There may be overlap between cavernous hemangioma and varix, and it is possible that they represent parts of a spectrum, with varix being a thrombosed variant of cavernous hemangioma. Despite some doubt about its existence, there is a recently reported clinicopathologic correlation of a classic cavernous hemangioma of the iris in a man who had experienced more than 30 hyphemas over about 20 years. There is no doubt about the diagnosis in that case.17 Our second patient had a localized cavernous hemangioma that we considered to be a classic case (Figure 1E), though histopathology is not available. The patient has been followed up and the lesion has remained stable for 4 years. These 2 cases are excellent examples of cavernous hemangiomas localized to the iris.

Importantly, there is another variant of iris cavernous hemangioma that is not confined only to the iris but is associated with multiple systemic cavernous hemangiomas.1821 There are several vascular lesions that have a systemic association and they appear to represent different syndromes. Larson and Oetting18 described a 48-year-old woman with a cavernous hemangioma of the iris and multiple central nervous system hemangiomas. One of our cases was a child who developed hydrocephalus at age 3 months and was found to have hemangiomas of the skin, brain, and kidney, requiring multiple treatments. We saw the child at age 7 years, and 1 year later the iris hemangioma had spontaneously deflated, leaving a fibrous scar.19 Iris cavernous hemangiomas can also be a component of diffuse congenital hemangiomatosis, which is often fatal in infants.20,21 An iris cavernous hemangioma has been reported following evisceration for a perforated globe in a patient with leprosy, but the diagnosis is questionable and the association may be coincidental.22

True capillary hemangioma of the iris is extremely rare. There was 1 case in our series that possibly represented a capillary hemangioma based on its clinical appearance and a very subtle ipsilateral eyelid vascular lesion. It was not possible to obtain adequate photographs of the iris lesion in that infant. Both lesions showed spontaneous regression. Iris capillary hemangioma has been seen mainly in association with periocular cutaneous capillary hemangioma of infancy. A well-documented case was reported by Ruttum and associates.24 Other possible cases are less well documented.23,25 The classic case appears as a periocular cutaneous hemangioma with an ipsilateral iris component (Figure 2A and B). Like the cutaneous counterpart, the iris component shows similar spontaneous regression.24 A case reported by Cashell23 as an angioma of the iris in a 2-year-old child was likely a capillary hemangioma based on the published histopathology. Based on our review, we believe that the case reported by Woo and associates25 as a cavernous hemangioma in an infant was possibly a capillary hemangioma.

There were 3 cases of iris varix in our series (Table 1). These are discussed briefly in the “Results” section. As with other iris vascular lesions, their diagnosis is often difficult to confirm clinically and histopathologically.3034 Two recently reported cases, however, had good histopathologic confirmation of the diagnosis.3,30 Unlike some iris vascular lesions, iris varix apparently has no known systemic associations.3

There were 3 patients with iris microhemangiomatosis, also called iris vascular tufts, in our series. A literature review suggests that it is by far the most common iris vascular tumor, with hundreds of cases having been reported.3550 It is most often detected by ophthalmologists following a spontaneous hyphema sometimes with secondary elevated intraocular pressures. Hence, it is important to differentiate microhemangiomatosis from iris neovascularization due to diabetic retinopathy or retinal vein obstruction. The pattern of iris neovascularization is different in that the distribution of the blood vessels in iris neovascularization is more widespread on the iris and in the anterior chamber angle. The hyphema often occurs after cataract surgery and does not create enough suspicion for a true neoplasm to warrant referral to an oncology center, explaining the low number of cases in our series. However, the hyphema and vascular lesion sometimes raise suspicion for an iris melanoma.

Microhemangiomatosis is occasionally asymptomatic and is found on routine slitlamp biomicroscopy. Cobb49 looked carefully at the pupillary margin of a series of patients and identified 44 patients, none of whom had any symptoms related to the iris lesions. It generally appears as multiple very subtle dilated vascular channels that arise from the pupillary vascular arcade. Fluorescein angiography reveals that they are more numerous than suspected clinically, become rapidly fluorescent, and often leak dye into the aqueous.35 Microhemangiomatosis that causes recurrent hyphema has been treated successfully with laser photocoagulation.39,43,44 There is little histopathology available for microhemangiomatosis, but a report of 1 lesion removed at cataract surgery described it as a hamartoma of capillary hemangioma type with features specific for iris blood vessels.42

Microhemangiomatosis is 1 of the iris vascular lesions that has been found to have intriguing systemic associations, mainly with myotonic dystrophy and possibly with diabetes mellitus.3,49,50 Cobb and associates50 found iris microhemangiomatosis in 5 of 8 men in a series of 10 patients with myotonic dystrophy. The reason for this odd association is not known.

In summary, although the existence of iris vascular tumors was once challenged, there are examples of racemose hemangioma, cavernous hemangioma, capillary hemangioma, varix, and microhemangiomatosis in our series and in the literature. Each type has characteristic clinical features and different ocular complications, mainly spontaneous hyphema. This study has reviewed a series of 45 patients with iris vascular tumor and related conditions seen on an ocular oncology service. A simple classification is proposed and the demographics, clinical features, complications, management, and histopathology are discussed. Although most are solitary lesions, some have serious systemic implications.

Back to top
Article Information

Correspondence: Jerry A. Shields, MD, Ocular Oncology Service, Wills Eye Institute, 840 Walnut St, Philadelphia, PA 19107 (jas.cls@comcast.net).

Submitted for Publication: January 31, 2010; final revision received February 14, 2010; accepted February 16, 2010.

Author Contributions: Dr J. A. Shields had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.

Financial Disclosure: None reported.

Funding/Support: This study was supported by a donation from the Eye Tumor Research Foundation, Philadelphia, Pennsylvania (Drs C. L. Shields and J. A. Shields), Mellon Charitable Giving from the Martha W. Rogers Charitable Trust (Dr C. L. Shields), the Paul Kayser International Award of Merit in Retina Research, Houston, Texas (Dr J. A. Shields), the LuEsther Mertz Retina Research Foundation (Dr C. L. Shields), and a donation from the Michael, Bruce, and Ellen Ratnor Foundation, New York, New York (Drs C. L. Shields and J. A. Shields).

Role of the Sponsor: The funders had no role in the design or conduct of the study; in the collection, analysis, or interpretation of the data; or in the preparation, review, or approval of the manuscript.

Previous Presentation: This paper was presented as part of the Helen Keller Lecture (Drs C. L. Shields and J. A. Shields); May 7, 2009; University of Alabama, Birmingham.

References
1.
Ferry  AP Hemangiomas of the iris and ciliary body: do they exist? a search for a histologically proved case. Int Ophthalmol Clin 1972;12 (1) 177- 194
PubMed
2.
Shields  JAShields  CL Intraocular Tumors: An Atlas and Textbook. Vol 2. Philadelphia, PA Lippincott Williams & Wilkins2008;256- 261
3.
Broaddus  ELystad  LDSchonfield  LSingh  AD Iris varix: report of a case and review of iris vascular anomalies. Surv Ophthalmol 2009;54 (1) 118- 127
PubMedArticle
4.
Ashton  N Primary tumours of the iris. Br J Ophthalmol 1964;48650- 668
PubMedArticle
5.
Amasio  EBrovarone  FVMusso  M Angioma of the iris. Ophthalmologica 1980;180 (1) 15- 18
PubMedArticle
6.
Spencer  WHFerguson  WJ  Jr Hemangioma of the iris. Arch Ophthalmol 1963;70811- 812
PubMedArticle
7.
Stallard  HB Haemangioma of the iris. Br J Ophthalmol 1966;50 (7) 434- 435
PubMedArticle
8.
Perkins  SAMagargal  LE Arteriovenous malformations of the iris. Ann Ophthalmol 1985;17 (11) 679- 680, 682, 685
PubMed
9.
de Corral  LRConway  MPeyman  GAConstanteras  A Argon laser treatment of an abnormal angle vessel producing recurrent hyphema. Int Ophthalmol 1985;8 (3) 179- 182
PubMedArticle
10.
Prost  M Arteriovenous communication of the iris. Br J Ophthalmol 1986;70 (11) 856- 859
PubMedArticle
11.
Parodi  MBBondel  ESaviano  SDa Pozzo  SBergamini  LRavalico  G Iris arteriovenous communication: clinical and angiographic features. Int Ophthalmol 1998;22 (1) 1- 5
PubMedArticle
12.
Shields  JAShields  CLO'Rourk  T Racemose hemangioma of the iris. Br J Ophthalmol 1996;80 (8) 770- 771
PubMedArticle
13.
Shields  JAStreicher  TFESpirkova  JHJStubna  MShields  CL Arteriovenous malformation of the iris in 14 cases. Arch Ophthalmol 2006;124 (3) 370- 375
PubMedArticle
14.
Lee  BJJeng  BHSingh  AD OCT and ultrasound biomicroscopic findings in iris arteriovenous malformation. Ophthalmic Surg Lasers Imaging 2008;39 (5) 426- 428
PubMedArticle
15.
Prost  M Cavernous hemangioma of the iris. Ophthalmologica 1987;195 (4) 183- 187
PubMedArticle
16.
Lam  S Iris cavernous hemangioma in a patient with recurrent hyphema. Can J Ophthalmol 1993;28 (1) 36- 39
PubMed
17.
Shields  JAShields  CLEagle  RC  Jr Cavernous hemangioma of the iris. Arch Ophthalmol 2008;126 (11) 1602- 1603
PubMedArticle
18.
Larson  SAOetting  TA Presumed iris hemangioma associated with multiple central nervous system cavernous hemangiomas. Arch Ophthalmol 2002;120 (7) 984- 985
PubMed
19.
Thangappan  AShields  CLDinowitz  MShields  JA Iris cavernous hemangioma associated with multiple cavernous hemangiomas in the kidney, brain, and skin. Cornea 2007;26 (4) 481- 483
PubMedArticle
20.
Naidoff  MAKenyon  KRGreen  WR Iris hemangioma and abnormal retinal vasculature in a case of diffuse congenital hemangiomatosis. Am J Ophthalmol 1971;72 (3) 633- 644
PubMed
21.
Weiss  MJErnest  JT Diffuse congenital hemangiomatosis with infantile glaucoma. Am J Ophthalmol 1976;81 (2) 216- 218
PubMed
22.
Ebenezer  GJDaniel  EJob  CK Cavernous haemangioma of the iris in a leprosy patient. Br J Ophthalmol 1997;81 (7) 610- 612
PubMedArticle
23.
Cashell  GT Angioma of the iris. Br J Ophthalmol 1967;51 (9) 633- 635
PubMedArticle
24.
Ruttum  MSMittelman  DSingh  P Iris hemangiomas in infants with periorbital capillary hemangiomas. J Pediatr Ophthalmol Strabismus 1993;30 (5) 331- 333
PubMed
25.
Woo  SJKim  CJYu  YS Cavernous hemangioma of the iris in an infant. J AAPOS 2004;8 (5) 499- 501
PubMedArticle
26.
Winnick  MMargalit  ESchachat  APStark  WJ Treatment of vascular tufts at the pupillary margin before cataract surgery. Br J Ophthalmol 2003;87 (7) 920- 921
PubMedArticle
27.
Chang  CWRao  NAStout  JT Histopathology of the eye in diffuse neonatal hemangiomatosis. Am J Ophthalmol 1998;125 (6) 868- 870
PubMedArticle
28.
Bryce  IGPai  VBradbury  JA Spontaneous resolution of iris and cutaneous haemangiomata in diffuse neonatal haemangiomatosis. Eye (Lond) 1999;13 (pt 3a) 388- 390
PubMedArticle
29.
Haik  BGClancy  PEllsworth  RMPerina  AZimmerman  K Ocular manifestations in diffuse neonatal hemangiomatosis. J Pediatr Ophthalmol Strabismus 1983;20 (3) 101- 105
PubMed
30.
Shields  JAShields  CLPulido  JEagle  RC  JrNothnagel  AF Iris varix simulating an iris melanoma. Arch Ophthalmol 2000;118 (5) 707- 710
PubMedArticle
31.
Andersen  SROther  A Varix of the iris. Arch Ophthalmol 1975;93 (1) 32- 33
PubMedArticle
32.
Küchle  MNaumann  GOH Varix of the iris spontaneous regression. Klin Monbl Augenheilkd 1992;200 (3) 233- 236
PubMedArticle
33.
Ang  LPSim  DHChiang  GSYong  VS Iris varix. Eye (Lond) 1997;11 (pt 5) 733- 735
PubMedArticle
34.
Rohrbach  JMEckstein  ASchuster  I Varicose vein of the iris. Klin Monbl Augenheilkd 1995;207 (3) 206- 207
PubMedArticle
35.
Rosen  ELyons  D Microhemangiomas at the pupillary border demonstrated by fluorescein photography. Am J Ophthalmol 1969;67 (6) 846- 853
PubMed
36.
Sellman  A Hyphaema from microhaemangiomas. Acta Ophthalmol (Copenh) 1972;50 (1) 58- 61
PubMedArticle
37.
Israel  MPLorenzetti  DW Bilateral microhemangiomas of the pupillary border with later hyphema. Can J Ophthalmol 1974;9 (1) 138- 140
PubMed
38.
Perry  HDMallen  FJSussman  W Microhaemangiomas of the iris with spontaneous hyphaema and acute glaucoma. Br J Ophthalmol 1977;61 (2) 114- 116
PubMedArticle
39.
Coleman  SLGreen  WRPatz  A Vascular tufts of pupillary margin of iris. Am J Ophthalmol 1977;83 (6) 881- 883
PubMed
40.
Mason  GIFerry  AP Bilateral spontaneous hyphema arising from iridic microhemangiomas. Ann Ophthalmol 1979;11 (1) 87- 91
PubMed
41.
Francis  ICKappagoda  MB Iris microhaemangiomas. Aust J Ophthalmol 1982;10 (3) 167- 171
PubMedArticle
42.
Meades  KVFrancis  ICKappagoda  MBFilipic  M Light microscopic and electron microscopic histopathology of an iris microhaemangioma. Br J Ophthalmol 1986;70 (4) 290- 294
PubMedArticle
43.
Thomas  RAylward  GMBillson  FA Spontaneous hyphaema from an iris microhaemangioma. Aust N Z J Ophthalmol 1988;16 (4) 367- 368
PubMedArticle
44.
Bandello  FBrancato  RLattanzio  RMaestranzi  G Laser treatment of iris vascular tufts. Ophthalmologica 1993;206 (4) 187- 191
PubMedArticle
45.
Ah-Fat  FGCanning  CR Recurrent visual loss secondary to an iris microhaemangioma. Eye (Lond) 1994;8 (pt 3) 357
PubMedArticle
46.
Akram  IReck  ACSheldrick  J Iris microhaemangioma presenting with total hyphaema and elevated intraocular pressure. Eye (Lond) 2003;17 (6) 784- 785
PubMedArticle
47.
Strauss  ECAldave  AJSpencer  WH  et al.  Management of prominent iris vascular tufts causing recurrent spontaneous hyphema. Cornea 2005;24 (2) 224- 226
PubMedArticle
48.
Bakke  EFDrolsum  L Iris microhaemangiomas and idiopathic juxtafoveolar retinal telangiectasis. Acta Ophthalmol Scand 2006;84 (6) 818- 822
PubMedArticle
49.
Cobb  B Vascular tufts at the pupillary margin: a preliminary report on 44 patients. Trans Ophthalmol Soc U K 1968;88211- 221
50.
Cobb  BShilling  JSChisholm  IH Vascular tufts at the pupillary margin in myotonic dystrophy. Am J Ophthalmol 1970;69 (4) 573- 582
PubMed
51.
Rodin  FH Angioma of the iris: first case to be reported with histologic examination. Arch Ophthal 1929;2679- 682Article
52.
Savir  HManor  RS Spontaneous hyphema and vessel anomaly. Arch Ophthalmol 1975;93 (10) 1056- 1058
PubMedArticle
×