Optical coherence tomography angiography (OCTA) is a new and non-invasive imaging technique that is able to detect blood flow signal in the retina and the choroid within seconds. OCTA is different from the traditional angiography methods. The major advantages of OCTA are that it can observe blood flow signal in different layers of the retina and the choroid without injecting any dye, provide blood flow information that traditional angiography cannot provide, and enrich pathophysiological knowledge of the retinal and choroidal vascular diseases., which help us to make an accurate diagnosis and efficient evaluation of these diseases. However there is a large upgrade potential either on OCTA technique itself or on clinical application of OCTA. We need to fully understand the advantage and disadvantage, and differences of OCTA and traditional angiography. We also need to know how to interpret the result of OCTA. With that we could make a fast diagnosis in a non-invasive way and improve our knowledge of the retinal and choroidal vascular diseases.
ObjectiveTo observe the imaging features of branching vascular network (BVN) in polypoidal choroidal vasculopathy (PCV). MethodsEighty PCV patients (90 eyes) were enrolled in this study. The patients included 58 males and 22 females. The age was ranged from 49 to 85 years, with a mean age of 61.4 years. All the patients were examined for fundus photography, fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA) and optical coherence tomography (OCT). The fibrovascular retinal pigment epithelium detachment (PED) was defined as a well-demarcated subretinal heterogeneous plaque with increasing fluorescence on FFA. The late lichenoid hyperfluorescent plaque was defined as a well-demarcated lichenoid hyperfluorescent plaque on late phase ICGA. The double-layer sign on OCT was defined as a wide range of shallow PED from Bruch membrane. ResultsBVN were found on early ICGA in 76 eyes among the 90 eyes (84.4%). Among these 76 eyes, 18 eyes (23.7%) demonstrated the subretinal reddish-orange branches corresponding to BVN. Fifty-six eyes (73.7%) demonstrated all or part of the BVN on early FFA. Three eyes (3.9%) demonstrated branching transmitted fluorescence corresponding to BVN throughout the FFA. Seventy-three eyes (96.1%) were manifested by occult choroidal vascularization on FFA, and 21 eyes (27.6%) of them were fibrovascular PED. Among the 76 eyes with BVN, all BVN appeared earlier than polypoidal lesions on ICGA. Polypoidal lesions located on the terminal of BVN in 62 eyes (81.6%). Sixty-nine eyes (90.8%) on ICGA demonstrated the late lichenoid hyperfluorescent plaque, whose area was equal to or greater than the area of BVN shown on early ICGA. Seventy-two eyes (94.7%) had the double-layer sign. Among these 72 eyes, 15 eyes (20.8%) had lumen-like structure within the double-layer sign. Sixty-five eyes (90.3%) had punctate and linear hyper-reflectance within the double-layer sign. Two eyes (2.8%) demonstrated a hyporeflective short segment and a gap of Bruch membrane on OCT corresponding to the origin of the BVN. Sixty-three eyes (87.5%) had an area of double-layer sign that matched the area of late lichenoid hyperfluorescent plaque on ICGA. ConclusionsBVN in PCV can be noted as reddish-orange branches on fundus examination. Most of the BVN are shown as early branching transmitted fluorescence but collectively an occult choroidal vascularization on FFA, as lichenoid hyperfluorescent plaque on late ICGA, and as double-layer sign on OCT whose area matches late lichenoid hyperfluorescent plaque.
Purpose To observe the features of the hyperfluorascent areas in the posterior ocular fundus detected by indocyanine green angiography(ICGA) in healthy volunteers, and to study circulatory properties of choroid. Methods Routine ICGA was performed on each of fifty consecutive normal eyes. Results ⑴Hyper fluorescence began at an average time of (30.80plusmn;5.42) seconds. ⑵The patterns of the hyperfluorescence revealed themselves in doubling areas divided symmetrically by the relatively hypoer fluorescence blelt running horizontally across the fovea in 29 eyes(58%), and single area in 21 eyes(42%).⑶The average area of the hyper fluorescence was (57.27plusmn;14.08)mm2.⑷ The sustaining time of the hyper fluorescence was (172.44plusmn;59.70) seconds at average. Conclusion During ICGA, a very patchy filling pattern of hyper fluorescence was visible in posterior fundus in normal eyes, and its filling time and shape presented choroidal blood supply and circulation. These parameters would offer consulted bases for clinical diagnosis of the choroidal diseases. (Chin J Ocul Fundus Dis,1999,15:1-3)
Objective To observe the clinical features, phenotypes and genotypes in a Chinese family with choroideremia (CHM). Methods A Chinese four-generation family (15 members) with CHM, including 5 patients (4 males/1 female), 2 female carriers and 8 healthy members, was enrolled in this study. Initially all family members underwent best corrected visual acuity (BCVA), indirect ophthalmoscopy, fundus fluorescein angiography, optical coherence tomography (OCT), visual field and full view electroretinogram (ERG). BCVA was followed up for 3 years. Venous blood samples were collected, and all of the 15 coding exons and flanking intron regions were amplified in the proband by polymerase chain reaction followed by direct sequencing. Protein structure was modeled based on the protein data bank and mutations in DeepView v4.0.1 to predict the effect of the mutations. A total of 180 healthy volunteers were enrolled as control group to matching CHM gene sequences. Results The visual acuity (VA) of 3/4 adult male patients began to decrease at less than 10, 10 and 30 years old, the average BCVA was 0.43. There were characteristic signs and symptoms of CHM including narrow visual field, extinguished rod and cone response in ERG, disappeared junction line and intermediate line of photoreceptor inner segment/outer segment on OCT. After 3 years, the mean BCVA decreased to 0.11. The BCVA of one young male patient was 1.0 in both eyes with minor changes fundus and visual field. The VA of the female patient began to decrease at 50 years old, her BCVA of two eyes were 0.5 and 0.25, respectively. The fundus changes were typical of CHM, with relative scotomas in the peripheral visual field of OD, and big scotomas in the OS. After 3 years, her mean BCVA decreased to 0.2. Of 2 female carriers, one had minor fundus changes (patches of pigmentary deposits, atrophy spots of retinal pigment epithelium cells), and the other was normal. A novel heterozygous c.1837G＞A mutation in exon 15 of CHM was detected in the proband, which resulted in the substitution of serine by proline at codon 613 (p.D613N). Based on molecular modeling, the misfolded protein caused by the mutation might destabilize the structure of the helix that potentially could affect the global stability of the Rep-1 protein. Conclusions A novel c.1837G＞A (p.D613N) mutation may be the causative mutation for CHM in this family. Female CHM carriers may have some signs and symptoms.