Unilateral giant coloboma of the upper eyelid associated with other congenital anomalies (33 years follow-up of surgical repair).

PURPOSE: To describe a case of congenital unilateral giant coloboma and its successful surgical repair with 33 years of follow-up. CASE REPORT: A 6-year-old boy presented with a congenital unilateral giant coloboma of the right upper eyelid associated with madarosis of the eyebrows, microphthalmos, dystopia of the hair, and coloboma of the apex of the nose. The patient underwent surgical repair of the multiple anomalies in different steps. DISCUSSION: A multiple-step, two-layer technique for the reconstruction of the right upper eyelid was performed in a 6-year-old boy with congenital unilateral giant coloboma associated with multiple ocular and facial anomalies. After 33 years of follow-up, the cosmetic results are excellent, although it has not been possible to preserve the visual function of the right eye, which had to be enucleated.

Polypoidal choroidal vasculopathy in Italy.

PURPOSE: To report on the frequency and clinical features of polypoidal choroidal vasculopathy (PCV) in a consecutive series of elderly Italian patients presenting with macular exudation. METHODS: The authors conducted a retrospective study on a series of 194 consecutive patients 50 years or older with newly diagnosed exudative maculopathy and the presumed diagnosis of age-related macular degeneration (ARMD). Color and/or red-free photographs and fluorescein and indocyanine green angiography were performed in all patients. RESULTS: Of the 194 patients, 19 (9.8%) were diagnosed with PCV. The remaining 175 (90.2%) patients had ARMD complicated by choroidal neovascularization. No age or sex differences were observed between the two groups. The disease was unilateral in 103 (58.9%) of 175 ARMD cases compared with 15 (78.9%) of 19 PCV cases (P = 0.09). Nine (47.3%) of 19 patients with PCV had an extramacular choroidal neovascularization, compared with only 5 (2.9%) of 175 patients with ARMD (P < 0.0001). Significant drusen were present in the fellow eyes of 66 (64.1%) of 103 unilateral cases in the ARMD group and in 4 (26.7%) of 15 patients with unilateral disease in the PCV group (P = 0.006). CONCLUSIONS: Polypoidal choroidal vasculopathy is not an uncommon disease in Italy and should be suspected in patients presenting with extramacular lesions and no large drusen in the fellow eye.

Chorioretinal involvement in primary systemic nonfamilial amyloidosis.

PURPOSE: To report a case of primary systemic nonfamilial amyloidosis studied by fluorescein angiography and indocyanine green angiography. METHODS: Case report. A 59-year-old woman with primary systemic nonfamilial amyloidosis presented bilateral diffuse deep hemorrhages and pigmentary mottling at the posterior pole. RESULTS: On fluorescein angiography bilateral diffuse areas of hypofluorescence were present. Indocyanine green angiography showed large hypofluorescent areas with hypofluorescent lines in the midperiphery and hyperfluorescent streaks in the peripapillary area. CONCLUSIONS: In this case of primary systemic nonfamilial amyloidosis, diffuse bilateral chorioretinal abnormalities included hemorrhages and pigmentary mottling at the posterior pole, with hypofluorescent areas on fluorescein angiography and indocyanine green angiography, as well as hypofluorescent lines in the midperiphery.

Optical coherence tomography findings in diabetic macular edema before and after vitrectomy.

BACKGROUND AND OBJECTIVE: To assess the role of optical coherence tomography (OCT) in the evaluation and follow-up after vitrectomy for diabetic macular edema. MATERIALS AND METHODS: The 18 eyes of 12 patients affected by presumed diabetic vitreous-induced macular edema underwent biomicroscopy with a Goldmann contact lens, fluorescein angiography, and OCT. RESULTS: OCT revealed two patterns of edema. The first group (15 eyes) was characterized by widespread thickening of the neurosensory retina with an increased nonhomogeneous reflectivity of the inner retinal layers; cystoid-like spaces of absent or reduced reflectivity in the neurosensory retina were also present. In the second group (3 eyes), a cystoid macular edema with a dome-shaped foveal profile because of a markedly increased retinal thickness in the foveal region was observed. The disappearance of the physiologic foveal profile was always seen. Biomicroscopy revealed an increased reflex of the inner limiting membrane in the first group and minimal alterations in the second one. Four patients (7 eyes) underwent vitrectomy with posterior hyaloid removal. In 5 eyes, the patients experienced a visual improvement greater than two Snellen lines. The restoration of the normal foveal profile and the reduction of the retinal thickness on the OCT were evident in all cases one month after surgery. CONCLUSION: OCT appears to be a useful tool in the diagnosis and management of diabetic macular edema and in the monitoring of the morphological changes after vitrectomy.

Polypoidal choroidal vasculopathy masquerading as central serous chorioretinopathy.

OBJECTIVE: To differentiate polypoidal choroidal vasculopathy (PCV) from central serous chorioretinopathy (CSC). DESIGN: A retrospective, observational case series. PARTICIPANTS: Thirteen patients originally diagnosed with CSC proved to have PCV after more extensive evaluation and follow-up. METHODS: A clinical and angiographic review of patients with manifestations of CSC, including macular detachment. MAIN OUTCOME MEASURES: Demographic data, funduscopic examination, and fluorescein and indocyanine green (ICG) angiographic findings. RESULTS: Thirteen patients initially suspected of having CSC were ultimately diagnosed as having PCV. These eyes had exudative macular detachments secondary to a small caliber, polypoidal choroidal vascular abnormality or so-called polypoidal choroidal neovascularization. The clinical manifestations in the fundus varied. They included multiple, variably sized serous pigment epithelial detachments, neurosensory retinal detachment, lipid deposition, patchy atrophy of the pigment epithelium and indistinct staining from decompensation of the posterior blood-retinal barrier on fluorescein angiography. In reality, the suspected PEDs proved to be polypoidal lesions of PCV when imaged with ICG angiography. CONCLUSIONS: The clinical diagnosis of CSC or PCV generally poses little challenge to the experienced retinal specialist. However, in CSC with persistent and/or recurrent exudation, a myriad of retinal pigment epithelial changes may evolve that make it difficult to differentiate these two entities. In such patients, ICG angiography is useful in differentiating CSC from PCV. An accurate clinical diagnosis is important since each of these entities, CSC and PCV, may differ in terms of their risk factors, natural course, and visual prognosis.

Estrogen and visual hallucinations in a patient with Charles Bonnet syndrome.

PURPOSE: To describe the occurrence of visual hallucinations in a patient with Charles Bonnet syndrome associated with estrogen intake. METHOD: Case report. RESULTS: An 84-year-old woman with poor visual acuity secondary to bilateral, nonexudative, age-related macular degeneration had nonthreatening visual hallucinations 2 weeks after starting oral estrogen for osteoporosis. The estrogen was stopped, and the hallucinations subsided. The patient was given estrogen twice more and each time the hallucinations recurred. CONCLUSION: We report a case of Charles Bonnet syndrome associated with estrogen intake in an 84-year-old woman. Estrogen may have promoted release phenomena and triggered the hallucinatory episodes in our patient.

Optical coherence tomography in the assessment of retinal pigment epithelial tear.

PURPOSE: To study the findings of optical coherence tomography (OCT) in retinal pigment epithelial (RPE) tears. METHODS: Sixteen eyes of 16 consecutive patients with age-related macular degeneration complicated by RPE tear were studied using OCT. Fluorescein angiography also was performed. Thirteen eyes were at the acute stage and three eyes were at the scarring stage, still with a recognizable tear. RESULTS: Optical coherence tomography identified an RPE detachment (PED) with focal interruption of the RPE in all cases. Optical coherence tomography always highlighted a peculiar non-dome-shaped profile of the serous PED, as opposed to that of the PED not complicated by an RPE tear. A very intense hyperreflectivity was observed in the OCT scans performed through the retracted RPE. A deep hyperreflectivity under the line corresponding to the RPE was evident in the area of the bare choroid. No choroidal neovascularization could be visualized using OCT, either at the acute or at the scarring stages. CONCLUSIONS: Optical coherence tomography, a noncontact, noninvasive imaging technique, may be a useful tool, complementary to fluorescein angiography, in the clinical assessment of RPE tears.

OCT imaging of choroidal neovascularisation and its role in the determination of patients' eligibility for surgery.

AIM: To evaluate the optical coherence tomographic characteristics of choroidal neovascularisation (CNV) in age related macular degeneration (AMD) and in idiopathic and inflammatory CNV. The use of this technique in the selection of patients for surgery is discussed. METHODS: Ocular coherence tomography (OCT), fluorescein, and indocyanine green angiography were performed in 23 patients affected by AMD complicated by well defined CNV and in 10 patients affected by inflammatory or idiopathic CNV. The neovascular membrane was surgically removed in five age related CNVs, two inflammatory choroidopathies, and two idiopathic CNVs. RESULTS: In inflammatory and idiopathic CNV, the OCT displayed a neovascularisation on the retinal pigment epithelium (RPE). In three cases the CNV was excised with an improvement of visual acuity equal to or greater than two Snellen lines; in a fourth case, the visual acuity after surgery was unchanged. In the cases of AMD the OCT fell into three different patterns: (A) CNV above the RPE (five cases); (B) focal, irregular thickening of the retinal pigment epithelial band (12 cases); (C) CNV above and below the RPE (six cases). The five pattern A CNV patients underwent the surgical excision of the neovascularisation. In four cases the visual acuity improved by two or more Snellen lines; in the fifth case the visual acuity remained unchanged. CONCLUSIONS: The authors suggest that the surgical removal of early age related CNV could be performed in those cases where the OCT shows a neovascular membrane on the RPE, as in idiopathic and inflammatory CNVs.

Usefulness of fluorescein angiography in predicting the size of the atrophic area after surgical excision of choroidal neovascularization.

Twenty consecutive patients affected by age-related macular degeneration (AMD) complicated by subfoveal and juxtafoveal choroidal neovascularization (CNV) underwent a surgical removal of the membrane. The patients were divided into two groups: group A = CNVs 2 months. The surgical excision of the CNV was also performed in 10 cases of multifocal choroidopathies and idiopathic CNV. The atrophic area after surgery was larger in AMD than in multifocal choroidopathies or idiopathic CNV (p < 0.001). In AMD the atrophic area after surgical excision of the CNV was larger in group B than in group A (p < 0.05). The area of the CNV + the hyperfluorescent halo observed in the late phase of fluorescein angiography before surgery was 84.6% of the atrophic area after surgery. Our observations could be helpful to the surgeon for a more accurate evaluation of the expected size of the atrophic area after surgical removal of a CNV, thus allowing a better selection of the patients for whom surgery could be of some benefit.

Comparison of fluorescein and indocyanine green angiography in angioid streaks.

BACKGROUND: The main cause of vision loss in patients with angioid streaks is choroidal neovascularization and subsequent macular degeneration. Indocyanine green angiography allows visualization of the choroidal circulation and may be superior to fluorescein angiography in the evaluation of patients with angioid streaks. METHODS: The ophthalmoscopic, fluorescein and indocyanine green angiographic characteristics of angioid streaks were studied in 34 patients with such streaks. Nineteen patients had pseudoxanthoma elasticum and 15 patients had isolated angioid streaks. The fluorescence characteristics of the 'peau d'orange' and of choroidal neovascularization, when present, were also analyzed. RESULTS: Angioid streaks may be hyperfluorescent, hypofluorescent or invisible on indocyanine green angiography. Hyperfluorescent streaks were found in 88% of eyes, hypofluorescent streaks in 11%; in 18% of eyes some streaks were not visualized by indocyanine green angiography. The peau d'orange stained as a speckled pattern in the midperiphery; the flecks were concentrated temporal to the macula. Eighteen eyes presented classic and 6 occult choroidal neovascularization. In several eyes a plaque-like lesion was seen on indocyanine angiography that did not correspond to occult choroidal neovascularization on fluorescein angiography. CONCLUSION: Indocyanine angiography outlines angioid streaks as well as the peau d'orange appearance better than fluorescein angiography in the majority of cases. In some cases, however, funduscopically visible streaks can not be visualized. Sometimes classic choroidal neovascular membranes are not visualized by conventional indocyanine green angiography. Occult choroidal neovascularization is better defined by indocyanine green angiography. The fluorescence of angioid streaks and of plaque-like lesions makes the interpretation of indocyanine green angiography difficult.

Indocyanine green angiography of multifocal choroiditis.

PURPOSE: The purpose of the study is to determine indocyanine green (ICG) angiographic characteristics of patients with multifocal choroiditis (MC) and to identify features that may assist in the differentiation of MC from other ocular inflammatory diseases. METHODS: After complete ophthalmologic examination, fluorescein angiography and ICG angiography were performed in a series of 14 patients with MC. The ICG findings were then correlated with the clinical and fluorescein angiographic appearance of these patients to determine specific characteristics and distinguishing features of the entity. These findings then were compared with those of angiographic patterns observed in patients with ocular histoplasmosis syndrome to determine whether differentiating features could be identified. RESULTS: Fourteen (50%) of the 28 eyes were found to have large hypofluorescent spots in the posterior pole on ICG angiography, which, in most cases, did not correspond to clinically or fluorescein angiographically detectable lesions. Seventeen (61%) had smaller hypofluorescent lesions (approximately 50 pm in size) in the posterior pole on the ICG study. In seven eyes exhibiting enlarged blind spots on visual field testing, ICG angiography showed confluent hypofluorescence surrounding the optic nerve. The ICG angiogram was found useful in evaluating the natural course in two patients with MC as well as a response to oral prednisone therapy in four others. The ICG angiographic findings differed from those seen in patients with ocular histoplasmosis. CONCLUSIONS: Indocyanine green angiography can provide information that is not detectable by clinical or fluorescein angiographic examination in patients with MC. This information may prove useful in differentiating this condition from the ocular histoplasmosis syndrome, provide a better understanding of the natural course and progression of the disease, and provide a potential adjunct in the clinical evaluation of patients undergoing therapeutic regimens for active inflammatory lesions.

Choroidal findings in the course of idiopathic serous pigment epithelium detachment detected by indocyanine green videoangiography.

PURPOSE: To analyze the choroidal alterations associated with idiopathic serous pigment epithelium detachment. METHODS: Twenty-five consecutive patients affected by idiopathic serous pigment epithelium detachment underwent ophthalmoscopy, fluorescein angiography, and indocyanine green videoangiography. RESULTS: On indocyanine green videoangiography an early, complete, and homogeneous filling of the pigment epithelium detachment was always observed. In the late phases, the indocyanine green pattern depended on the size of the detachment. An idiopathic serous pigment epithelium detachment larger than the diameter of one optic disk was still hyperfluorescent in the late phases of indocyanine green videoangiography and was surrounded by a ring of brighter hyperfluorescence. An idiopathic serous pigment epithelium detachment smaller than the diameter of one optic disk usually could be visualized in the late phases as a hypofluorescent area surrounded by a hyperfluorescent ring. In 30 eyes (83.3%), choroidal hyperpermeability was observed. An irregular dilatation of the choroidal veins at the site or within an area the size of one disk diameter from the detachments could be visualized on indocyanine green videoangiography in 12 of 36 affected eyes (33.3%); in three cases an active focus of central serous chorioretinopathy with subretinal leakage developed in the follow-up period. CONCLUSION: The observation that pigment epithelium detachments frequently are associated with choroidal leakage and venous dilatation supports the hypothesis that an idiopathic serous pigment epithelium detachment is a variant of central serous chorioretinopathy. Moreover, the choroidal permeability alterations detected by indocyanine green videoangiography would support the theory of Gass that idiopathic serous pigment epithelium detachments could be caused by exudation of fluids from the choroidal vessels.

Indocyanine green angiographic findings in multifocal choroidopathies.

With high definition videoangiography (TOPCON IMAGEnet H1024) the Authors studied 41 patients affected by multifocal choroidopathies (MC) (68 eyes with ophthalmoscopic or indocyanine green angiographic evidences): 29 females and 12 males; age 21-51 years with a follow up of 6-29 months. In the light of the evidence provided by FA and ICG the Authors present a classification of MC in three stages: Stage 1 of subclinical choroidal activity (5 eyes) characterised by the presence of hypofluorescent or hyperfluorescent spots visible only in the late phases of ICGA; stage 2 of clinically evident choroidal activity (45 eyes) in FA the spots are hypofluorescent in the early phases and hyperfluorescent with a slight diffusion in the late phases, in ICGA either hypofluorescent spots or less frequently hyperfluorescent spots and choroidal permeability alterations can be observed; stage 3 or healed stage (18 eyes) in FA the spots are hyperfluorescent without late leakage, in ICGA hypofluorescence can be observed during all angiographic phases. In 5 patients in stage 1 of subclinical activity, a systemic steroid therapy induced a regression of the hypofluorescent spots in ICGA, in 2 cases the regression of hyperfluorescent spots in ICGA was observed after systemic antibiotic therapy. The authors underline that ICGA could be a particularly useful tool for an early diagnosis and clinical monitoring of MC.

Isolated lesion of the medial orbital wall following endonasal surgery. Isolated fractures of the medial orbital wall.

BACKGROUND: Isolated fractures of the medial orbital wall are infrequent. The diagnostic triad includes: adduction block, exotropia with diplopia in all directions of gaze, positive passive duction in abduction. Sometimes a slight enophthalmos is present. Computed tomography shows the extension and the seat of the fracture. CASE REPORT: The authors illustrate the case of a 60 year old male who presented with a breach of the medial orbital wall following endonasal surgery. RESULTS: The patient was successfully operated using an iliac bone graft inserted via an eyebrow-nasal cutaneous approach, after a previous attempt with a transconjunctival approach performed in another hospital had failed. A good functional and aesthetic result was observed within the first year after surgery. After almost 11 years a full adduction is still present and diplopia is absent. CONCLUSION: The authors underline the importance of an early diagnosis and prompt surgical treatment. The fat-muscle entrapment should be removed and the bone defect closed. A close cooperation between ophthalmologist and plastic surgeon is suggested.

Indocyanine green angiography in serpiginous choroidopathy.

BACKGROUND: Manifestations of serpiginous choroidopathy have been well described, but very little is known about its true pathogenesis. By virtue of the enhanced imaging of the choroidal circulation, indocyanine green angiography may offer information on the causative factors of the disorder. METHODS: Indocyanine Green (ICG) videoangiography was carried out in 17 patients with serpiginous choroidopathy. A full ophthalmological examination and intravenous fluorescein angiography were simultaneously done as well. The patients were studied at 3 different stages of the disease as follows: 1) with acute manifestations; 2) with subacute lesions; 3) in the inactive or healed state. RESULTS: Acute Lesions: Indocyanine angiography showed active choroidal involvement in the acute stage beyond the limits delineated by corresponding fluorescein studies. Subacute Lesions: ICG angiography showed resolution of choroidal involvement in advance of clinical and fluorescein angiographic changes in some eyes. Healed Lesions: ICG angiography showed better delineation of the atrophic choroid with clearer definitions than corresponding fluorescein studies. Late staining of fibrovascular tissue within atrophic zones was similar to fluorescein findings. Two patients in the healed state showed multifocal hypofluorescence (one patient) and hyperfluorescent choroidal lesions (one patient) with no clinical or fluorescein counterparts, possibly representing occult lesions. CONCLUSIONS: ICG angiography may be useful in understanding certain clinical features of the entity, such as a clearer documentation of the extent and nature of the choroidal damage and possible sites at risk for future recurrences. However, it adds little to our current management of the disorder.

Indocyanine green angiographic findings in serpiginous choroidopathy.

AIMS: Analysis of the choroidal findings in patients affected by serpiginous choroidopathy (SC). METHODS: Thirteen patients (23 eyes; 11 males and two females; age range 50-68 years; mean age 59.1 years) affected by SC were examined with fluorescein angiography (FA) and indocyanine green angiography (ICGA). The follow up period was 7-33 months. RESULTS: Using ICGA the disease could be divided into the following stages: (1) subclinical or choroidal stage (hypofluorescent lesions without FA evidence); (2) active stage (with ICGA and FA evidence); (3) subhealing stage (slight late hyperfluorescent lesions with ICGA, with no evidence on FA); (4) inactive or healed stage (hypofluorescent areas with ICGA and hyperfluorescent areas with FA). CONCLUSIONS: Although FA showed a clear distinction between active and healed stages, ICGA allowed a greater subdivision of the disease. In particular, ICGA allowed: (1) better staging of SC, revealing choroidal alterations when there was no ophthalmoscopic or FA evidence; (2) better identification of the active lesions which appear to be larger at the choroidal level in comparison with the corresponding retinal lesions; and (3) revealed a persistence of choroidal activity even when the signs of retinal activity had disappeared. Thus, ICGA should be a particularly useful clinical and therapeutic monitoring tool of SC.

Indocyanine green angiographic findings in idiopathic choroidal neovascularization.

PURPOSE: Evaluation of choroidal alterations associated with idiopathic choroidal neovascularization (ICNV) and the possible relation between this affection and Multifocal Choroidopathies (MC). METHODS: The authors analysed, using high definition videoangiography, the choroidal findings in 21 consecutive patients affected by ICNV (7 males and 14 females; 19-46 years; mean age: 31.8 years); with a follow-up at 5-30 months (mean 13 months). Moreover, a retrospective study of 20 cases of ICNV (11 males and 9 females; age: 17-39 years; mean age: 29.3 years) with a follow-up at 6-11 years (mean 8.9), was performed. RESULTS: In 7 eyes, the indocyanine green angiography (ICGA) showed choroidal hypofluorescent spots similar to those observed in MC (in 3 cases even in the fellow unaffected eye), in 2 of them the regression of the spots was observed after steroid therapy. In 2 eyes, the ICGA revealed hyperfluorescent spots; in one of them the complete regression of the spots after oral cyprofloxacine was observed. In 6 patients (10 eyes), choroidal permeability alterations could be visualized (in 4 cases even in the unaffected eye). CONCLUSIONS: The indocyanine green angiographic findings (hypo and hyperfluorescent spots, choroidal permeability alterations) could support the theory of Gass which considers that ICNV is not idiopathic but secondary to a widespread choroidal inflammatory disease. The similarity of the ICGA alterations in ICNV and MC, the observation that cases of ICNV would become MC in the follow-up, could allow the hypothesis of a close connection between these two affections.

Indocyanine green angiographic findings in multifocal choroidopathies.

With high definition videoangiography (TOPCON IMAGEnet H1024) the Authors studied 41 patients affected by multifocal choroidopathies (MC) (68 eyes with ophthalmoscopic or indocyanine green angiographic evidences): 29 females and 12 males; age 21-51 years with a follow up of 6-29 months. In the light of the evidence provided by FA and ICG the Authors present a classification of MC in three stages: Stage 1 of subclinical choroidal activity (5 eyes): characterised by the presence of hypofluorescent or hyperfluorescent spots visible only in the late phases of ICGA; stage 2 of clinically evident choroidal activity (45 eyes): in FA the spots are hypofluorescent in the early phases and hyperfluorescent with a slight diffusion in the late phases, in ICGA either hypofluorescent spots or less frequently hyperfluorescent spots and choroidal permeability alterations can be observed; stage 3 or healed stage (18 eyes): in FA the spots are hyperfluorescent without late leakage, in ICGA hypofluorescence can be observed during all angiographic phases. In 5 patients in stage 1 of subclinical activity, a systemic steroid therapy induced the regression of the hypofluorescent sports in ICGA, in 2 cases the regression of hyperfluorescent spots in ICGA was observed after systemic antibiotic therapy. The authors underline that ICGA could be a particularly useful tool for an early diagnosis and clinical monitoring of MC.