Because CNM and IPCV share many ocular manifestations, IPCV may be mistaken as other conditions that are associated with CNM. Differential diagnoses of IPCV include, but are not limited to, AMD, inflammatory retinopathy, myopic degeneration, idiopathic central serous choroidopathy, and ocular histoplasmosis. In addition, the reddish-orange nodular lesion of IPCV may be confused with choroidal hemangioma and other choroidal malignancies (Table 3). Unlike the conditions mentioned, IPCV is not correlated with retinal features of lacquer cracks, angioid streaks, drusen, or inflammation. 1
Because IPCV and CNM can resemble each other clinically, FA and/or ICG videoangiography are beneficial in making the diagnosis. 17 CNM and IPCV lesions begin to fill simultaneously with the choroid and display hyperfluorescence in later phases. The classic CNM angiography is a lacy vascular early hyperfluorescent area with late staining. However, there are many FA pattern variations of CNM. IPCV does not encompass the intense hyperfluorescence observed with CNM angiography. 1 The typical IPCV FA reveals mottled hyperfluorescence in the early stage of the angiogram with late staining of the polypoidals. 18 Two distinct phases of FA filling can be identified in the IPCV lesion. In the early phase, IPCV will first show filling of the underlying choroidal network concurrent with choroidal vasculature. During late phases, IPCV polypoidal lesions become more hyperfluorescent.
The acute phase of IPCV may result in massive exudation and/or bleeding, obscuring the polypoidal findings on an angiogram. For cases when this occurs, the ability of ICG videoangiography can enhance imaging of the choroidal circulation and aid in confirming the diagnosis of IPCV. This technique initially unveils choroidal hyperfluorescence. Early phases reveal a branching vascular network with terminating polypoidals. 5 Areas within and just around the IPCV network appear to fill slowly. During the late stages, there is a uniform fading of the ICG dye, with the exception of an active polypoidal structure, which tends to leak. 4 A study using ICG analysis for patients who were previously diagnosed with CNM associated with AMD revealed two previously undiagnosed cases of IPCV. 19 Because ICG reveals the primary vascular network lesion related with IPCV, it provides a more reliable diagnosis for IPCV than conventional FA. Again, unlike our case, this is primarily in cases where hemorrhage obscures the polypoidal views.
Ultrasonography and optical coherence tomography (OCT) may also aid in the diagnosis of IPCV. 10, 13, 20 Ultrasonography reveals enlarged choroidal thickness associated with the IPCV choroidal vascular network polypoidals. Perkovich 21 demonstrated retinal elevation and irregular choroidal thickening when performing ultrasonographic evaluations on patients with IPCV. OCT has been identified as a method to evaluate patients with IPCV. OCT, a noninvasive laser-based clinical imaging technique, reveals cross-sectional information of the retina and choroid, similar to that of ultrasonography but with a higher resolution image.
OCT performed at the site of an IPCV lesion reveals a characteristic hyper-reflectivity in the choroidal layers. 10 The choroidal layers were localized under a small domelike elevation of the RPE. 20 Otsuji et al. 13 argued that the highly reflective band does not correspond to RPE changes but rather to the choriocapillaris. Giovannini 10 noted that a larger RPE detachment (≥1/2 disc diameter) gives an image with “optical silence” below the RPE detachment. Unfortunately, when OCT was used to identify the choroidal branching vascular network, no unique corresponding image was found. 13, 10 Although OCT may provide a histopathologic representation of a lesion, interpretation also is required and may vary among examiners. Therefore, although OCT may be able to identify a characteristic pattern of reflectivity in some patients with IPCV, a unique OCT pattern specific to a diagnosis of IPCV has not been identified. In summary, OCT or ultrasonography alone cannot determine the existence of IPCV in a patient but may serve to complement other data acquired.
Histopathology and Pathophysiology
MacCumber et al. 22 reported a case with clinical findings similar to those of IPCV. The patient also had severe diabetic retinopathy, which led to enucleation of the eye, and consequently allowed for histopathologic investigation. MacCumber et al. identified polypoidal lesions as choroidal vascularization traveling through Bruch's membrane with additional fibrovascular proliferation. However, in a study by Rosa et al., 18 polypoidals were identified as thin-walled cavernous vascular channels within Bruch's membrane that were thought to originate from the short posterior ciliary arteries. Postulation of the variable histologic characteristics between the two studies are believed to be a result of the progressive changes in IPCV—MacCumber's evaluation took place at the end stage of IPCV, whereas Rosa's evaluation took place at an earlier stage.
One systemic correlation of IPCV is hypertension. 17 Rosa et al. 18 noted IPCV shared common features with ocular changes associated with chronic hypertension, such as ocular manifestations primarily in the posterior pole and representations of vascular remodeling. Shared qualities were also relevant in the studies of MacCumber et al. and Ross et al. 22, 23 The correlation between hypertension and IPCV suggests a possible mechanism of action for the pathogenesis of the vascular anomalies of IPCV. Both lesions associated with IPCV and hypertension predominately occur in the juxtapapillary region possibly because it corresponds to an ocular location of decreased vascular support; with less vascular redundancy and support, the juxtapapillary area within the choroid may be predisposed to hypertensive insult. 23 Moreover, the choroidal vascular IPCV changes appear to mimic retinal vascular hypertensive changes, such as retinal arterial macroaneurysm (RAM). RAM and IPCV lesions are examples of vascular remodeling secondary to hypertensive insult to the retina or choroid. 23 Besides the association with hypertension, other studies by Lip et al. 7 and Smith et al. 24 noted distinctive systemic associations with IPCV, namely, increased plasma viscosity levels and thrombocytopenia. However, no conclusive data have linked a systemic condition to the etiology of IPCV.
Based on OCT findings, Otsuji et al. 13 suggested that the IPCV lesions may simply be variants of CNM with dilatations, contributing to direct leakage of the fine vessels. Using the OCT, Iijima et al. 20 reported that the choroidal polypoidal structures had their own distinct patterns separate from that of CNM, protruding anteriorly, raising the overlying neurosensory retina. Iijima et al. theorized that because the RPE undergoes sustained pressure from the underlying IPCV lesion, thinning defects result in weakened adhesive forces and lead to recurrent RPE detachments from either serous or hemorrhagic factors. Because of the absence of conclusive studies, the exact nature of the choroidal polypoidal vascular anomalies associated with IPCV remains ambiguous.
The course of IPCV begins with the development of a polypoidal vascular lesion beneath the RPE at the level of the inner choroid (external to the choriocapillaris) that grows and expands anteriorly toward the inner retinal layers. Anterior expansion of the polypoidal can result in hemorrhagic or serous RPE detachments and vitreous hemorrhage. The quintessential course of IPCV is associated with recurrent RPE detachments. In time, the vascular lesion will flatten, and the RPE detachments will resolve. 5 As the detachment and leakage resolve, pigmentary and atrophic changes may occur. New polypoidal vessels may form at the level of the inner choroid near the site of the flattened lesion, leading to the recurrence of IPCV. CNM represents direct proliferation of the small choroidal capillaries through Bruch's membrane. This proliferation also results in RPE detachments and extensive leakage.
Studies in the Japanese population have given us more information regarding the natural course of IPCV. Uyama et al. 3 reported that most untreated eyes showed a stable course, whereby the choroidal polypoidal flattened and serosanguineous RPE detachments resolved; 28% of cases showed recurrence of serous RPE detachment(s) with few cases advancing to a more pathologic stage of scarring. In contrast to IPCV lesions, CNM associated with AMD generally has a more rapid progression corresponding to a poorer prognosis because of a declining course. The disciform scars and fibrovascularization after CNM rupture often result in devastating visual loss as a complication of “wet” AMD.
A recent complication noted in patients with IPCV is retinal microangiopathy. 25 The cases discussed shared specific characteristics of exudative retinal detachment secondary to the IPCV lesions, lipid deposition, and associated microangiopathy, which was apparent on FA. On FA this microangiopathy included telangiectatic microaneurysmal alterations and adjacent capillary nonperfusion and leakage. Although the exact mechanism of development of retinal microangiopathy in these cases was not determined, these retinal vascular abnormalities have been associated with cases of “chronic” retinal elevations resulting from retinal or macular detachments. 25
Management and Treatment
Vision is usually preserved in patients with IPCV, although some patients may experience severe vision loss because of secondary complications. When this occurs, it is usually attributed to vitreous hemorrhage or macular involvement, including cystic degeneration and/or chronic atrophy. 4 If the macula develops areas of atrophy or a classic CNM in addition to a primary IPCV lesion, visual prognosis is poor. Moorthy et al. 26 noted that when a patient has sudden, severe vision loss caused by a hemorrhagic RPE detachment associated with IPCV, spontaneous resolution may still occur, resulting in an excellent prognosis. Thus, patients without significant vision loss, as in the case presented, may be managed by monitoring for regression. Observation may entail performing repeat visual fields, dilated fundus examination, fundus photography, and FA and/or ICG videoangiography. Patient education regarding the recurrent nature of the condition also is essential.
IPCV lesions that linger or demonstrate excessive leakage associated with RPE detachments tend to be progressive and result in a poorer prognosis when left untreated. Treatment options include surgical and laser therapy. Surgical intervention may be beneficial for patients with IPCV who also experienced submacular hemorrhages. Surgical procedures that have been performed are pars plana vitrectomy with tissue plasminogen activator (TPA) and intravitreal 100% sulfur hexafluoride gas injection without TPA. 6 Postoperatively, any remaining polypoidal lesions outside the foveal avascular zone may be treated with laser. In addition, cases using macular translocation have also been reported. Unfortunately, the sample size was too small to present any statistically significant results. 27 A more recent therapeutic option is photodynamic therapy (PDT) with verteporfin, considered useful for the management of subfoveal IPCV. 28 This treatment modality has been proven to be successful for patient with CNM associated with AMD. Reported IPCV cases treated with PDT had an increase in improvement over mean vision within the sample cases. However, complication of progressive hemorrhage and exudation did occur among some subjects. 29
Laser photocoagulation management of IPCV may also be used in cases of severe vision loss caused by serosanguineous RPE detachments threatening or involving the macula. Only mild applications are essential for the management of IPCV. The rapid reabsorption of fluid after photocoagulation treatment of patients with IPCV can decrease blood toxicity to the retina, allowing for restoration of normal retinal function and decreasing atrophic and fibrovascular scar formation. The laser treatment may allow for normal macular function to be reinstated in patients with IPCV. 30, 31
Historically, argon laser photocoagulation was applied to areas of leakage associated with the polypoidal structures and adjacent areas of RPE detachment. Repeat treatment may be needed in cases of persistent leakage. Moreover, argon laser photocoagulation can be used as prophylactic treatment in cases of polypoidals located within the maculae. 7 In 1998, Gomez et al. 32 discussed the benefits of diode laser photocoagulation over argon laser photocoagulation as treatment for a patient with finger counting vision caused by excessive subretinal fluid at the macular level. Because the diode laser has deep choroidal penetration with relative sparing of the RPE and nerve fiber layer, treatment led to progressive improvement of visual acuity to 20/200; thus, it has been suggested as the preferred laser treatment for patients with IPCV. 32
The case depicted here demonstrates a classic representation of IPCV with a simultaneous presentation of CNM. Dilated examination and FA revealed RPE detachments associated with choroidal polypoidals, which is the diagnostic finding of IPCV. In addition, FA also revealed a lacy hyperfluorescent vascular lesion, consistent with CNM. ICG has been regarded as an essential component for the diagnosis of IPCV by some authors. 17, 25 However, ICG is primarily used to visualize the choroidal circulation, especially in the presence of blood, which may obscure the polypoidal views. ICG was not performed in our patient because we believed that the polypoidals were easily delineated in the fundus photograph (see Fig. 2) and the FA (see Figs. 4 and 5), making the diagnosis apparent.
In summary, serosanguineous RPE detachments are a prevalent ocular presentation of IPCV and CNM commonly associated with AMD, leading many to believe that IPCV may be a variant of CNM. Careful clinical evaluation of the fundus and FA and/or ICG videoangiography analysis is beneficial in making the diagnosis, in addition to helping differentiate IPCV from CNM. Although IPCV and CNM share many common features, the contrast in demographics, associated retinal findings, visual prognosis, and natural course leads one to accept IPCV and CNM as separate clinical entities. It is imperative to correctly diagnose IPCV over CNM associated with AMD because they each have ultimately different prognosis and management options. Further studies delineating the pathophysiology of IPCV are needed to clearly comprehend this rare retinal condition, ultimately leading to the correct management option.
A portion of this paper was presented in part at the February SECO 2000 meeting in Atlanta, Georgia.
1. Yannuzzi LA, Sorenson J, Spaide RF, Lipson B. Idiopathic polypoidal choroidal vasculopathy (IPCV). Retina 1990;10:1–8.
2. Stern RM, Zakov ZN, Zegarra H, Gutman FA. Multiple recurrent serosanguineous retinal pigment epithelial detachments in black women. Am J Ophthalmol 1985;100:560–9.
3. Uyama M, Matsubara T, Fukushima I, Matsunaga H, Iwashita K, Nagai Y, Takahashi K. Idiopathic polypoidal choroidal vasculopathy in Japanese patients. Arch Ophthalmol 1999;117:1035–42.
4. Yannuzzi LA, Ciardella A, Spaide RF, Rabb M, Freund KB, Orlock DA. The expanding clinical spectrum of idiopathic polypoidal choroidal vasculopathy. Arch Ophthalmol 1997;115:478–85.
5. Spaide RF, Yannuzzi LA, Slakter JS, Sorenson J, Orlach DA. Indocyanine green videoangiography of idiopathic polypoidal choroidal vasculopathy. Retina 1995;15:100–10.
6. Shiraga F, Matsuo T, Yokoe S, Takasu I, Okanouchi T, Ohtsuki H, Grossniklaus HE. Surgical treatment of submacular hemorrhage associated with idiopathic polypoidal choroidal vasculopathy. Am J Ophthalmol 1999;128:147–54.
7. Lip PL, Hope-Ross MW, Gibson JM. Idiopathic polypoidal choroidal vasculopathy: a disease with diverse clinical spectrum and systemic associations. Eye 2000;14(Pt 5):695–700.
8. Yannuzzi LA, Nogueira FB, Spaide RF, Guyer DR, Orlock DA, Colombero D, Freund KB. Idiopathic polypoidal choroidal vasculopathy: a peripheral lesion. Arch Ophthalmol 1998;116:382–3.
9. Kleiner RC, Brucker AJ, Johnston RL. The posterior uveal bleeding syndrome. Retina 1990;10:9–17.
10. Giovannini A, Amato GP, D'Altobrando E, Giuliani M. Optical coherence tomography (OCT) in idiopathic polypoidal choroidal vasculopathy (IPCV). Doc Ophthalmol 1999;97:367–71.
11. Gass JDM. Stereoscopic Atlas of Macular Disease: Diagnosis and Treatment, vol 1. 4th ed. St. Louis: Mosby, 1997:250.
12. Ahuja RM, Stanga PE, Vingerling JR, Reck AC, Bird AC. Polypoidal choroidal vasculopathy in exudative and haemorrhagic pigment epithelial detachments. Br J Ophthalmol 2000;84:479–84.
13. Otsuji T, Takahashi K, Fukushima I, Uyama M. Optical coherence tomographic findings of idiopathic polypoidal choroidal vasculopathy. Ophthalmic Surg Lasers 2000;31:210–4.
14. Yannuzzi LA, Wong DW, Sforzolini BS, Goldbaum M, Tang KC, Spaide RF, Freund KB, Slakter JS, Guyer DR, Sorenson JA, Fisher Y, Maberley D, Orlock DA. Polypoidal choroidal vasculopathy and neovascularized ARMD. Arch Ophthalmol 1999;117:1503–10.
15. Ferris FL 3rd. Senile macular degeneration: review of epidemiologic features. Am J Epidemiol 1983;118:132–51.
16. Sho K, Takahashi K, Yamada H, Wada M, Nagai Y, Otsuji T, Nishikawa M, Mitsuma Y, Yamazaki Y, Matsumura M, Uyama M. Polypoidal choroidal vasculopathy: incidence, demographic features, and clinical characteristics. Arch Ophthalmol 2003;121:1392–6.
17. Uyama M, Wada M, Nagai Y, Matsubara T, Matsunaga H, Fukushima I, Takahashi K, Matsumura M. Polypoidal choroidal vasculopathy: natural history. Am J Ophthalmol 2002;133:639–48.
18. Rosa RH Jr, Davis JL, Eifrig CW. Clinicopathologic reports, case reports, and small case series: clinicopathologic correlation of idiopathic polypoidal choroidal vasculopathy. Arch Ophthalmol 2002;120:502–8.
19. Escano MF, Fujii S, Ishibashi K, Matsuo H, Yamamoto M. Indocyanine green videoangiography in macular variant of idiopathic polypoidal choroidal vasculopathy. Jpn J Ophthalmol 2000;44:313–6.
20. Iijima H, Imai M, Gohdo T, Tsukahara S. Optical coherence tomography of idiopathic polypoidal choroidal vasculopathy. Am J Ophthalmol 1999;127:301–5.
21. Perkovich BT, Zakov ZN, Berlin LA, Weidenthal D, Avins LR. An update on multiple recurrent serosanguineous retinal pigment epithelial detachments in black women. Retina 1990;10:18–26.
22. MacCumber MW, Dastgheib K, Bressler NM, Chan CC, Harris M, Fine S, Green WR. Clinicopathologic correlation of the multiple recurrent serosanguineous retinal pigment epithelial detachments syndrome. Retina 1994;14:143–52.
23. Ross RD, Gitter KA, Cohen G, Schomaker KS. Idiopathic polypoidal choroidal vasculopathy associated with retinal arterial macroaneurysm and hypertensive retinopathy. Retina 1996;16:105–11.
24. Smith RE, Wise K, Kingsley RM. Idiopathic polypoidal choroidal vasculopathy and sickle cell retinopathy. Am J Ophthalmol 2000;129:544–6.
25. Iida T, Yannuzzi LA, Freund KB, Ciardella AP, Costa DL, Huang SJ, Golub BM. Retinal angiopathy and polypoidal choroidal vasculopathy. Retina 2002;22:455–63.
26. Moorthy RS, Lyon AT, Rabb MF, Spaide RF, Yannuzzi LA, Jampol LM. Idiopathic polypoidal choroidal vasculopathy of the macula. Ophthalmology 1998;105:1380–5.
27. Terasaki H, Miyake Y, Suzuki T, Nakamura M, Nagasaka T. Polypoidal choroidal vasculopathy treated with macular translocation: clinical pathological correlation. Br J Ophthalmol 2002;86:321–7.
28. Quaranta M, Mauget-Faysse M, Coscas G. Exudative idiopathic polypoidal choroidal vasculopathy and photodynamic therapy with verteporfin. Am J Ophthalmol 2002;134:277–80.
29. Spaide RF, Donsoff I, Lam DL, Yannuzzi LA, Jampol LM, Slakter J, Sorenson J, Freund KB. Treatment of polypoidal choroidal vasculopathy with photodynamic therapy. Retina 2002;22:529–35.
30. Hochman MA, Seery CM, Zarbin MA. Pathophysiology and management of subretinal hemorrhage. Surv Ophthalmol 1997;42:195–213.
31. Bennett SR, Folk JC, Blodi CF, Klugman M. Factors prognostic of visual outcome in patients with subretinal hemorrhage. Am J Ophthalmol 1990;109:33–7.
© 2004 American Academy of Optometry
32. Gomez-Ulla F, Gonzalez F, Torreiro MG. Diode laser photocoagulation in idiopathic polypoidal choroidal vasculopathy. Retina 1998;18:481–3.