Anophthalmic socket syndrome determines functional deficits and facial deformities, and may lead to poor psychological outcomes. This review aims to comprehensively evaluate the features of the syndrome, based on literature review and authors’ clinical and surgical experience. An electronic database (PubMed,MEDLINE and Google Scholar) search of all articles written in English and non-English language with abstract translated to English on anophthalmic socket syndrome was performed. Data reviewed included demographics, presentations, investigations, management, complications and outcomes. Different types of orbital implants were evaluated; the management of implant exposure was examined; different orbital volume enhancement procedures such as secondary implantation, subperiosteal implants and the use of fillers in anophthalmic patients were described; the problems related to socket contraction were outlined; the treatment options for chronic anophthalmic socket pain and phantom eye syndrome were assessed; the most recent advances in the management of congenital anophthalmia were described. Current clinical evidence does not support a specific orbital implant; late exposure of porous implants may be due to pegging, which currently is seldom used; filler absorption in the orbit appears to be faster than in the dermis, and repeated treatments could be a potential source of inflammation; socket contraction results in significant functional and psychological disability, and management is challenging. Patients affected by anophthalmic socket pain and phantom eye syndrome need specific counseling. It is auspicable to use a standardized protocol to treat children affected by clinical congenital anophthalmia; dermis fat graft is a suitable option in these patients as it helps continued socket expansion. Dermis fat graft can also address the volume deficit in case of explantation of exposed implants and in contracted sockets in both children and adults. Appropriate clinical care is essential, as adequate prosthesis wearing improves the quality of life of anophthalmic patients.

Purpose . To evaluate the effects of combined intense pulsed light therapy (IPL) and low‐level light therapy (LLLT) in dry eye disease (DED) in patients affected by Sjögren’s syndrome. Patients and Methods . This is a monocentric, prospective, interventional study. At baseline, all the study patients ( n = 20) were on tear substitute therapy and underwent Schirmer type‐1 test and breakup time (BUT) test. After baseline measurements, tear substitute therapy was suspended, and patients underwent IPL and LLLT. The same investigations were carried out at one (T1) and at three (T3) months after treatment. The Ocular Surface Disease Index (OSDI) survey was used to measure the severity of DED. Results . BUT test showed an increase in tear film breakup time in patients with DED 1 month after the beginning of the treatment (T0 vs T1: p = 0,01). This increase was even more statistically significant after 3 months of the IPL and LLLT treatment (T0 vs T3: p < 0.0001). Schirmer test values increased too, but there was not statistically significance between values at T0 and T1 or T3. The patients perceived an improvement in their condition, which resulted in a lower score on the OSDI survey. The OSDI score was lower at T1 than T0 (T0 vs T1: p = 0.0003), while it tended to increase again after 3 months although it was still lower than baseline (T0 vs T3: p = 0.02). No facial or ocular side effects were reported. Conclusions . The use of combined IPL/LLLT for the treatment of DED in patients affected by Sjögren’s syndrome appears to be beneficial.

Purpose: To assess the retinal and choroidal vasculature in patients with genetically confirmed Marfan syndrome (MfS). Methods: This prospective, case-control, observational study included 48 eyes of 24 patients with a genetic diagnosis of MfS and compared them with 52 eyes of 26 healthy controls. Best-corrected visual acuity, choroidal and retinal thickness measured by spectral domain-optical coherence tomography, retinal and choroidal vasculature characterized by optical coherence tomography angiography, were collected. A genetic counseling was carried out. A transthoracic echocardiogram was performed to evaluate the dimension of the aortic root, the ascending aorta and the left ventricle function and dimensions. Results: A significant decrease in the superficial and deep retinal capillary plexi vessel density (VD) was evident, such as a decrease in the choriocapillaris plexus VD. In patients with MfS, a negative correlation between left ventricular diameter and the VD of the superficial and deep plexi was observed. Patients with MfS with greater posterior wall and interventricular septum dimensions had lower VD in both plexi (P < 0.05). Moreover, there was a negative correlation between the dimension of the ascending aorta and foveal choriocapillary VD. In patients with MfS, increasing diameter of the ascending aorta was associated with a lower foveal choriocapillary VD (P < 0.05). Conclusions: The severity of MfS correlates with the impairment of the retinal and choroidal vasculature. Translational Relevance: Optical coherence tomography angiography may be a reproducible and noninvasive tool to study retinal blood flow in patients with MfS, with potential diagnostic and prognostic value.

Purpose: To assess the structure and function of the retinal posterior pole in patients with early Parkinson’s disease (PD) and to identify possible biomarkers correlated with clinical features. Patients and Methods: A cross-sectional case-control study of 21 patients with PD and 22 age-matched healthy controls (HC) was conducted. All subjects underwent full ophthalmological examinations, fundus perimetry (FP) and spectral domain-OCT (SD-OCT) of the entire retinal posterior pole and peripapillary retinal nerve fiber layer (pRNFL). Results: We analyzed 41 eyes from 21 patients (14 males and 7 females) with early PD (Hoehn and Yahr scale (H&Y) equal to or less than stage 2) and 41 eyes from 22 HC (12 males and 10 females). We found no significant difference in the pRNFL thickness between patients with PD and HC. The statistical analysis of the SD-OCT posterior pole area, consisting of 64 values for each retinal layer, revealed a decrease in the outer nuclear layer (ONL) thickness in patients with PD (p < 0.0001). On the contrary, a significant increase in the thickness of the outer plexiform layer (OPL) (p < 0.0001) and of the retinal pigmented epithelium (RPE) (p= 0.002) compared to healthy controls was detected. Other retinal layers showed no significant statistical differences. The differential light sensitivity (DLS) values measured by FP were significantly lower in patients than the healthy controls (15 [13– 16.2] vs 17.95 [16.08– 18.96] p< 0.0001). Conclusion: Our results showed that DLS and retinal structure differed in the posterior pole between patients with early PD and controls. Thickening of the OPL may represent accumulation of α-synuclein in the OPL of patients with PD.

There are consolidated data about multiple sclerosis (MS)–dependent retinal neurodegeneration occurring in the optic disk and the macula, although it is unclear whether other retinal regions are affected. Our objective is to evaluate, for the first time, the involvement of the entire retinal posterior pole in patients diagnosed with relapsing remitting multiple sclerosis (RRMS) unaffected by optic neuritis using Spectral Domain–Optical Coherence Tomography (SD–OCT). The study protocol was approved by Tor Vergata Hospital Institutional Ethics Committee (Approval number 107/16), and conforms to the tenets of the Declaration of Helsinki. After a comprehensive neurological and ophthalmological examination, 53 untreated RRMS patients (aged 37.4 ± 10) and 53 matched controls (aged 36.11 ± 12.94) were enrolled. In addition, each patient underwent an examination of the posterior pole using the SD-OCT built-in Spectralis posterior pole scanning protocol. After segmentation, the mean thickness, as well as the thickness of the 64 single regions of interest, were calculated for each retinal layer. No statistically significant difference in terms of average retinal thickness was found between the groups. However, MS patients showed both a significantly thinner ganglion cell layer (p < 0.001), and, although not statistically significant, a thinner inner nuclear layer (p = 0.072) and retinal nerve fiber layer (p = 0.074). In contrast, the retinal pigment epithelium (p = 0.014) and photoreceptor layers p < 0.001) resulted significantly thicker in these patients. Interestingly, the analysis of the region of interest showed that neurodegeneration was non-homogeneously distributed across each layer. This is the first report that suggests a complex rearrangement that affects, layer by layer, the entire retinal posterior pole of RRMS retinas in response to the underlying neurotoxic insult.

To explore retinal abnormalities using spectral domain optical coherence tomography (SD-OCT) and OCT-angiography (OCT-A) in a highly selective cohort of patients with type I hereditary angioedema (HAE). This prospective case-control study included 40 type I HAE patients and 40 age-/sex-matched healthy subjects (HC). All participants underwent SD-OCT-scanning of retinal posterior pole (PP), peripapillary retinal nerve fiber layer (pRNFL), and optic nerve head (ONH). Superficial/deep capillary density was analyzed by OCT-A. A total of 80 eyes from 40 HAE and 40 eyes from HC were evaluated. The pRNFL was thicker in HAE than in HC in nasal superior (p < 0.0001) and temporal quadrants (p = 0.0005 left, p = 0.003 right). The ONH thickness in HAE patients was greater than in HC in the nasal (p = 0.008 left, p = 0.01 right), temporal (p = 0.0005 left, p = 0.003 right), temporal inferior (p = 0.007 left, p = 0.0008 right), and global (p = 0.005 left, p = 0.007 right) scans. Compared to HC, HAE showed a lower capillary density in both superficial (p = 0.001 left, p = 0.006 right) and deep (p = 0.008 left, p = 0.004 right) whole images, and superficial (p = 0.03 left) and deep parafoveal (p = 0.007 left, p = 0.005 right) areas. Our findings documented subclinical retinal abnormalities in type I HAE, supporting a potential role of the retinal assessment by SD-OCT/OCT-A as a useful tool in the comprehensive care of HAE patients.