Visual Acuity, and Macular and Peripapillary Thickness in High Myopia.

PURPOSE: To investigate best corrected visual acuity (BCVA), and choroidal and retinal thickness values between high myopes without myopic maculopathy and emmetropes. MATERIALS AND METHODS: Case control study where 53 myopes with axial length (AL) above 26 mm without myopic maculopathy and 53 age-matched emmetropes with AL between 21.50 and 24.50 were included as controls. Complete ophthalmological examination and biometry were performed. Choroidal and individual retinal layer thickness maps using spectral domain optical coherence tomography were obtained in the macular and peripapillary area with enhanced depth imaging. Peripapillary retinal nerve fiber layer (pRNFL) thickness was obtained using the circular 12°diameter scan. RESULTS: Mean age was 31.9 ± 9.9 and 32.5 ± 9.3 years in the myopes and controls, respectively (p > 0.05). Mean BCVA was 55.32 ± 2.50 versus 57.04 ± 2.27 ETDRS letters, in the myopes and controls, respectively (p = 0.0004). AL was the principal predictive factor for macular and peripapillary CT in myopes and macular CT in controls. BCVA was not influenced by choroidal thickness (CT). BCVA positively correlated with global pRNFL, following correction for age and AL, in both groups (r = 0.38, p = 0.008 and r = 0.38, p = 0.007 in the myopic and control groups, respectively). Statistical analysis following correction for the potential confounding factors of age, gender, AL, gender, AL, macular CT, and peripapillary CT, showed no significant differences in macular and peripapillary thicknesses between the two groups. CONCLUSIONS: AL is the principal predictive factor for macular and peripapillary CT in high myopes without maculopathy, and CT is not an independent predictor of visual acuity. Global pRNFL thickness is the only independent predictive factor of BCVA.

Hyperreflective Choroidal Vessels in Geographic Atrophy Secondary to Age-Related Macular Degeneration.

BACKGROUND AND OBJECTIVE: To describe choroidal vessels in areas of geographic atrophy (GA) secondary to age-related macular degeneration that appear as hyperreflective choroidal vessels (HRCVs) on multicolor (MC) imaging. PATIENTS AND METHODS: Retrospective case series of patients with GA. Multimodal imaging evaluation was performed. RESULTS: HRCVs, which seem to be sclerotic on MC imaging, appeared as hyperautofluorescent on fundus autofluorescence, clearly distinguishable over the background of hypo-autofluorescence, and correlated with late-phase hypocyanescence areas on indocyanine green angiography. Average size of GA areas was significantly larger in eyes with (4.19 mm ± 0.83 mm) compared to eyes without (3.22 mm ± 1.05 mm) HRVCs (P = .0002). Similarly, mean choroidal thickness (CT) was significantly thinner in eyes with (78.5 µm ± 33.8 µm) compared to eyes without (155.4 µm ± 69.8 µm) HRVCs (P < .0001). CONCLUSIONS: HRCVs are more clearly distinguishable than other choroidal vessels on MC imaging in GA. HRCV identification is more frequent in eyes with larger areas of atrophy and reduced CT, and thus possibly represent a maker of more advanced GA. [Ophthalmic Surg Lasers Imaging Retina. 2016;47:1106-1114.].

An Update on the Ophthalmologic Features in the Phakomatoses.

Neurofibromatosis type 1, tuberous sclerosis complex, and Von Hippel-Lindau disease, historically classified as the phakomatoses, are hereditary multisystem disorders characterized by the presence of hamartoma, which carry the risk of malignant transformation. The alteration of tumor suppressor genes seems to be at the basis of their pathophysiogenetic mechanism. Lisch and choroidal nodules in neurofibromatosis type 1, retinal astrocytomas in tuberous sclerosis complex, and retinal capillary hemangioma in Von Hippel-Lindau disease are the principal ophthalmic hamartomatous manifestations. The advent of novel imaging techniques such as near infrared reflectance and optical coherence tomography has provided unprecedented insight on the choroidal and retinal features of these diseases. These methods have improved early diagnosis and the ongoing surveillance in these conditions. Among an array of treatment modalities, antivascular endothelial growth factor therapy has been used in the management of retinal hamartomas but results have been varied. This review is an update on the pathophysiogenetic mechanisms, ophthalmic manifestations, and novel treatment strategies in the phakomatoses with emphasis on the role of imaging techniques.

Glaucoma Management in Carotid Cavernous Fistula.

Carotid cavernous fistulas (CCF) are vascular communications between the carotid artery and the cavernous sinus. Ophthalmologists are called to diagnose and manage the condition in cases that present with ocular features. A 73-year-old female was referred to our glaucoma center clinic. Eight years before, she had started receiving medication for glaucoma and had undergone laser iridotomy, but a satisfactory management of intraocular pressure (IOP) had not been achieved. The patient was complaining of intermittent diplopia, bilateral proptosis, and conjunctival chemosis over the past 6 months. Best-corrected visual acuity in the right (OD) and left eye (OS) was 9/10 and 10/10, respectively. Visual field testing showed slight paracentral field defects mostly in OS. IOP was 20 mm Hg in OD and 34 mm Hg in OS. We referred the patient to neuroradiology, and MRI angiography revealed a CCF with angiographic classification of Cognard grade 2. Closure of the CCF by transarterial embolization was performed in the neuroradiology department. One week following the procedure, the clinical signs of diplopia, proptosis, and conjunctival chemosis had greatly improved, and IOP was reduced to 12 mm Hg OD and 19 mm Hg in OS. Glaucoma treatment was maintained with topical brimatoprost, brinzolamide, and timolol. Owing to the risk of vision loss associated with vascular stasis, retinal ischemia, and high IOP, ophthalmologists must be aware of the clinical features of CCF and should request appropriate imaging studies such as MRI angiography in order to confirm the diagnosis and plan multidisciplinary treatment.

Spectral domain optical coherence tomography and B-scan ultrasonography in the evaluation of retinal tears in acute, incomplete posterior vitreous detachment.

BACKGROUND: The purpose of this study was to evaluate the extension and traction effects of posterior vitreous detachment (PVD) complicated with retinal tears using spectral domain optical coherence tomography (OCT) and B-scan ultrasonography. METHODS: Complete ophthalmological examination, B-scan ultrasonography and spectral domain OCT were performed in patients with acute PVD and retinal tears. Vitreous detachment was classified as complete or incomplete, based on extent of posterior pole or peripheral vitreous detachment. Retinal tear location and persistent traction on the retinal flap was evaluated with B-scan ultrasonography and OCT. Categorical data were evaluated with Fisher's exact test. Statistical significance was considered as P < 0.05. RESULTS: Twenty-six eyes of 25 patients were assessed. Four eyes (15 %) presented complete PVD with detachment at the posterior pole and periphery. 22 eyes (85 %) presented incomplete PVD with detachment in the periphery. Twenty eyes presented retinal tears in the superior quadrants with respect to only 6 in the inferior quadrants (p = 0.006). There was a higher incidence of retinal tears in the pre with respect to post-equatorial areas (19 vs 7 eyes, p = 0.019). B-scan ultrasonography and OCT revealed persistent traction on the retinal tear flap in 19 and 15 eyes, respectively. CONCLUSIONS: In acute PVD, retinal tears are prevalently associated with peripheral vitreous detachment. The impact of complete or incomplete PVD can be of clinical value when evaluating patients with retinal tears.

Spectral Domain Optical Coherence Tomography of Choroidal and Outer Retinal Layer Thickness in the Sturge Weber Syndrome.

PURPOSE: To evaluate choroidal thickness and its effect on the outer retinal layers in patients with Sturge Weber syndrome (SWS). MATERIALS AND METHODS: Twenty eyes of 10 patients with SWS and 20 eyes of 10 healthy controls were evaluated at the ophthalmology unit of the Umberto I Policlinic, Rome from December 2015 to May 2015. Manual segmentation measurements of choroidal and retinal pigment epithelium (RPE)-photorec eptor layer (PHL) thickness were performed at the subfovea and at 500 µm intervals over 3 mm-long horizontal and vertical segments using enhanced depth spectral domain optical coherence tomography. RESULTS: Mean choroidal thickness of the affected (561.6 µm ± 208.8) and fellow eyes (322.0 µm ± 56.6) of patients with SWS was significantly higher with respect to controls (266.5 µm ± 48.5 µm), p = 0.001 and p = 0.017, respectively. Mean RPE-PHL thickness was significantly lower in both the affected and fellow eyes of patients with respect to controls (p = 0.039 and p = 0.025, respectively). CONCLUSIONS: The choroid is thickened in patients with SWS, but the RPE-PHL is thinner. Choroidal thickening may lead to functional impairment causing disruption in the fine equilibrium between the choroid and retina and consequent outer retinal layer thinning.

Abnormalities of retinal ganglion cell complex at optical coherence tomography in patients with type 2 diabetes: a sign of diabetic polyneuropathy, not retinopathy.

AIMS: To compare optical coherence tomography (OCT)-derived neuro-retinal parameters in patients with type 2 diabetes and non-diabetic controls and to evaluate their correlation with diabetic retinopathy (DR) and polyneuropathy (DPN). METHODS: One-hundred consecutive patients with type 2 diabetes were examined by spectral-domain (SD) OCT for evaluating ganglion cell complex (GCC) and retinal nerve fibre layer (RNFL) thickness and two new pattern-based quantitative measures of GCC damage, global and focal loss volume (GLV and FLV). Fifty sex- and age-matched non-diabetic subjects served as control. RESULTS: RNFL thickness (101.0±10.6 vs. 106.4±10.3 µm, P=0.003) was significantly lower and GLV (6.58±4.98 vs. 4.52±3.10 %, P=0.008) and FLV (1.90±1.97 vs. 0.89±0.84 %, P<0.0001) were significantly higher in diabetic versus control subjects. The OCT parameters did not differ significantly according to DR grade. Conversely, RNFL thickness was lower and GLV and FLV were higher in patients with versus those without DPN, and the extent of changes increased significantly with quartiles of DPN score. At both bivariate and multivariate analysis, OCT parameters, especially FLV, correlated significantly with DPN measures. CONCLUSIONS: The GCC is significantly affected in patients with type 2 diabetes and SD-OCT might represent a useful tool to detect DPN, but not DR in these individuals.

Ocular Alterations in a Rare Case of Segmental Neurofibromatosis Type 1 with a Non-Classified Mutational Variant of the NF-1 Gene.

BACKGROUND: Neurofibromatosis type 1 (NF-1) is an autsomal dominant disorder which can occasionally result from somatic mosaicism and manifest as segmental forms of the disease. METHODS: A 37-year-old woman with ascertained NF-1, based on clinical diagnostic criteria and genetic analysis, was referred for ophthalmological evaluation. Genetic analysis, magnetic resonance imaging (MRI), complete ophthalmological examination, and near infrared reflectance (NIR) images at 815 nm of the retina were obtained. RESULTS: Genetic analysis revealed a non-classified mutational variant of the NF-1 gene identified as NM_000267.3:c2084T > C (p.Leu695Pro.T). MRI demonstrated non-symptomatic bilateral optic nerve gliomas. The only cutaneous sign was a subcutaneous neurofibroma of the posterior cervical region. Slit-lamp examination showed bilateral Lisch nodules. NIR images of the retina did not show any choroidal hamartomas. DISCUSSION: We report a rare case of segmental neurofibromatosis with a non-classified mutational variant of the NF-1 gene described in only one previous case in the literature. The patient presented with clinical features of NF-1 localized to the head and neck region, compatible with diagnosis of segmental NF-1. Interestingly, ocular manifestations included bilateral optic nerve gliomas and Lisch nodules, but no choroidal hamartomas.

Ocular Inflammation: Can It Be a Sign of Activity of Weber-Christian Disease? A Case Report and Review of Literature.

PURPOSE: To present a case of Weber-Christian disease with symptomatic ocular involvment. Weber-Christian disease is a relapsing febrile nodular nonsuppurative panniculitis. It is characterized by malaise and fever accompanied by subcutaneous inflammatory nodules on the trunk and extremities. It can affect several organs, but ocular signs have been infrequently described in literature. METHODS: A 20-year-old woman with Weber-Christian disease presented with severe bilateral ocular inflammation. A complete ophthalmologic examination was performed. RESULTS: Visual acuity was 20/100 in both eyes and slit-lamp examination showed bilateral iridocyclitis. Bilateral cortico-nuclear cataract did not allow funduscopy and she underwent cataract extraction. Retinal vasculitis was detected. CONCLUSIONS: Patients with Weber-Christian disease can develop severe ocular inflammation. A complete ophthalmolgic examination should be done in these patients, especially when ocular involvement is the main sign of the disease. A further understanding of the severity of ocular inflammation proved fundamental in the management of the disease.

SPECTRAL DOMAIN OPTICAL COHERENCE TOMOGRAPHY EVIDENCE OF RETINAL NERVE FIBER LAYER AND GANGLION CELL LOSS IN ADULT PATIENTS WITH NEUROFIBROMATOSIS TYPE 1.

PURPOSE: To evaluate peripapillary retinal nerve fiber layer, macular retinal nerve fiber layer, and ganglion cell layer-inner plexiform layer thickness and analyze their correlations in adult patients with neurofibromatosis Type 1 (NF1) and disease-free controls. METHODS: This cross-sectional study was performed at the Azienda Policlinico Umberto I, University of Rome "La Sapienza." All participants underwent complete ophthalmologic examination. Spectral domain optical coherence tomography was used to evaluate peripapillary retinal nerve fiber layer and obtain retinal segmentation measurements to assess macular retinal nerve fiber layer and ganglion cell layer-inner plexiform layer at 1,000 µm nasal, temporal, superior, and inferior to the fovea. RESULTS: Thirty-four eyes of 17 patients with NF1 (mean age, 42.2 ± 14.3 years) and 34 eyes of 17 disease-free control subjects (mean age, 41.4 ± 12.2 years) were included. All participants had best-corrected visual acuity of 20/20. The mean thickness of peripapillary retinal nerve fiber layer, macular retinal nerve fiber layer, and ganglion cell layer-inner plexiform layer was lower in patients with NF1 with respect to controls (P = 0.003, P = 0.022, P < 0.001, respectively). Regression analysis showed a significant correlation (P < 0.001) between mean ganglion cell layer-inner plexiform layer thickness and mean peripapillary retinal nerve fiber layer thickness in patients with NF1. CONCLUSION: Retinal nerve fiber layer and ganglion cell loss correlate well with each other in adult patients with NF1 in comparison with a healthy control population.

Surgical treatment of a rare case of bilateral ptosis due to localized ocular amyloidosis.

We describe a rare case of a 31-year old woman with bilateral ptosis due to localized amyloidosis. She referred a nine-year history of ptosis and surgical treatment with frontalis suspension three years previously. Following complete ophthalmological examination and evaluation of the ptosis we carried out tarsal and fornix biopsy, which revealed accumulation of a weakly eosinophilic amyloid positive substance. We performed surgical correction using the levator aponeurosis-Müller's muscle complex re-adaptation technique and amyloid substance debulking in all the palpebral layers in the left eye. The material obtained was stained with hematoxylin-eosin, Congo Red, PAS and alpha-actin, which confirmed amyloid deposition. Successively, the right eye was operated in the same manner and entropion was managed by dissection and removal of amyloid from subconjunctival layers. Five years following surgery, the corrective procedure for ptosis was still effective. Surgical treatment of ptosis is very complex and requires precise indications. Appropriate management depends on the etiopathogenesis, accurate diagnosis, and clinical findings.

Twelve-Month Results of a Single or Multiple Dexamethasone Intravitreal Implant for Macular Edema following Uncomplicated Phacoemulsification.

The clinical efficacy of one or two intravitreal injections of a continued deliverance dexamethasone 700 µg implant in ten patients with persistent macular edema following uncomplicated phacoemulsification was evaluated. Complete ophthalmological examination and spectral domain optical coherence tomography were carried out. Follow-up was at day 7 and months 1, 2, 4, 6, 8, and 12. At baseline mean best corrected visual acuity was 62 Early Treatment Diabetic Retinopathy Study Chart letters, which showed statistically significant improvement at each follow-up, except at month 6, to reach 79 letters at month 12 (P = 0.018). Prior to treatment mean central foveal thickness was 622 µm, which showed statistically significant improvement at each follow-up to reach a mean value of 282 µm (P = 0.012) at month 12. Five patients received a second dexamethasone implant at month 7. Two patients were excluded from the study at months 4 and 8. Intraocular pressure remained stable during the study period with the exception of mild increase in two patients requiring topical therapy. In conclusion there was statistically significant improvement of best corrected visual acuity and mean central foveal thickness with one or two intravitreal dexamethasone implants over 12 months.

Rare Diseases Leading to Childhood Glaucoma: Epidemiology, Pathophysiogenesis, and Management.

Noteworthy heterogeneity exists in the rare diseases associated with childhood glaucoma. Primary congenital glaucoma is mostly sporadic; however, 10% to 40% of cases are familial. CYP1B1 gene mutations seem to account for 87% of familial cases and 27% of sporadic cases. Childhood glaucoma is classified in primary and secondary congenital glaucoma, further divided as glaucoma arising in dysgenesis associated with neural crest anomalies, phakomatoses, metabolic disorders, mitotic diseases, congenital disorders, and acquired conditions. Neural crest alterations lead to the wide spectrum of iridocorneal trabeculodysgenesis. Systemic diseases associated with childhood glaucoma include the heterogenous group of phakomatoses where glaucoma is frequently encountered in the Sturge-Weber syndrome and its variants, in phakomatosis pigmentovascularis associated with oculodermal melanocytosis, and more rarely in neurofibromatosis type 1. Childhood glaucoma is also described in systemic disorders of mitotic and metabolic activity. Acquired secondary glaucoma has been associated with uveitis, trauma, drugs, and neoplastic diseases. A database research revealed reports of childhood glaucoma in rare diseases, which do not include glaucoma in their manifestation. These are otopalatodigital syndrome, complete androgen insensitivity, pseudotrisomy 13, Brachmann-de Lange syndrome, acrofrontofacionasal dysostosis, caudal regression syndrome, and Wolf-Hirschhorn syndrome.

Ophthalmic Alterations in the Sturge-Weber Syndrome, Klippel-Trenaunay Syndrome, and the Phakomatosis Pigmentovascularis: An Independent Group of Conditions?

The phakomatoses have been traditionally defined as a group of hereditary diseases with variable expressivity characterized by multisystem tumors with possible malignant transformation. The Sturge-Weber syndrome, Klippel-Trenaunay syndrome, and the phakomatosis pigmentovascularis have the facial port-wine stain in common. Numerous pathophysiogenetic mechanisms have been suggested such as venous dysplasia of the emissary veins in the intracranial circulation, neural crest alterations leading to alterations of autonomic perivascular nerves, mutation of the GNAO gene in the Sturge-Weber syndrome, PIK3CA mutation in malformative/overgrowth syndromes such as the Klippel-Trenaunay syndrome, and the twin-spotting phenomenon in phakomatosis pigmentovascularis. Other features linked to the port-wine stain and typical to all of the three conditions are glaucoma and choroidal alterations. Glaucoma can be due to malformations of the anterior chamber or high episcleral venous pressure and in phakomatosis pigmentovascularis it can also be associated with angle hyperpigmentation. The choroid can be thickened in all diseases. Furthermore, choroidal melanocytosis in the phakomatosis pigmentovascularis can lead to malignant transformation. Although the multiple pathophysiological mechanisms still require clarification, similarities in ophthalmic manifestations make it reasonable to classify these diseases in an independent group.

An unusual case of acute glaucoma in Sturge-Weber syndrome.

PURPOSE: Sturge-Weber syndrome is a phakomatosis with involvement of the eyelids, conjunctiva, choroid, and retina. Congenital glaucoma is the most common ocular complication resulting from raised episcleral venous pressure. We present an unusual case of glaucoma induced by pupillary block in an 81-year-old woman with Sturge-Weber syndrome. METHODS: The patient was referred for acute loss of vision OD and right-sided headache, pain, and nausea. The visual acuity was light perception OD with an intraocular pressure (IOP) of 41 mm Hg. Slit-lamp examination showed diffuse corneal edema OD associated with pupillary occlusion leading to angle closure and acute glaucoma attack. No pathologic changes were evidenced in the left eye. RESULTS: The raised IOP was partly relieved following administration of intravenous acetazolamide 250 mg, accompanied by relief of both pain and nausea. Laser iridotomy was performed OD, reducing the IOP to 22 mm Hg. The patient underwent cataract surgery with pupil membrane removal, leading to IOP of 17 mm Hg, but visual acuity remains unchanged due to optic nerve atrophy. CONCLUSIONS: This case supports the importance of involving an ophthalmologist in the management of Sturge-Weber syndrome, with monitoring of IOP, since early management of glaucoma can modify the visual outcome.

Ganglion Cell Complex Evaluation in Exudative Age-Related Macular Degeneration after Repeated Intravitreal Injections of Ranibizumab.

Purpose. To detect the effects of intravitreal ranibizumab injections on GCC in patients with wet AMD. Methods. 32 wet AMD eyes were selected and submitted at three ranibizumab injections. RTVue-OCT GCC and MM5 protocol were performed before treatment and twenty days after each injection. Results. At baseline mean GCC thickness was 93.9 ± 18.5 µm. Twenty days after each intravitreal injection it was, respectively, 85.8 ± 10.1, 86.5 ± 9.3, and 91.1 ± 11.5 µm, without statistical significance. A significant improvement in visual acuity (P = 0.031) and a reduction of mean foveal (P = 0.001) and macular thickness (P = 0.001) were observed. Conclusion. The clinical results confirm therapeutic efficacy of intravitreal injections of ranibizumab in wet AMD. A contemporary not statistically significant reduction of GCC thickness suggests that the loading phase of ranibizumab does not have any toxic effects on ganglion cell complex.

Morphologic and vasculature features of the choroid and associated choroid-retinal thickness alterations in neurofibromatosis type 1.

BACKGROUND/AIMS: A normal structural and functional choroid is essential in supplying blood flow to the retina. Neurofibromatosis type 1 (NF1) is a neurocristopathy where the choroid is altered due to the presence of nodules. The present transversal study was conducted to examine choroidal nodules and their effect on choroidal and retinal thickness in NF1 patients. METHODS: Near-infrared reflectance and optical coherence tomography with enhanced depth imaging were used to evaluate choroidal morphology and vasculature in 19 patients with NF1 and 19 healthy, age-matched control subjects. Choroidal thickness, neuroepithelium thickness, photoreceptors together with retinal pigment epithelium (RPE) thickness and outer nuclear layer (ONL) thickness were measured at the fovea and 1000 µm nasal, temporal, superior and inferior to the fovea in NF1 patients and control subjects. Choroidal and neuroepithelium thickness were assessed overlying and adjacent to nodules in NF1 patients. RESULTS: Choroidal nodules were classified as 'dome-shaped' or 'placcoid' subtypes in 17 patients. Small and medium calibre choroidal vessels were observed above dome-shaped nodules where choroidal thickness was significantly reduced. There was a statistically significant reduction in mean choroidal thickness (p=0.013) in NF1 patients with respect to control subjects. The neuroepithelium, photoreceptors together with RPE and ONL had a statistically significant reduction in mean thickness in NF1 patients (p<0.001, p<0.001, p=0.012, respectively). CONCLUSIONS: In NF1, there are dome-shaped and placcoid choroidal nodules which alter choroidal morphology and thickness. There is reduction in mean choroid thickness with generalised thinning of the neuroepithelium, photoreceptors together with RPE and ONL in NF1 patients.

Retinal microvascular abnormalities overlying choroidal nodules in neurofibromatosis type 1.

BACKGROUND: Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder involving aberrant proliferation of multiple tissues of neural crest origin. Retinal vascular alterations in NF1 have rarely been reported in the literature and their nature is not clear. This study describes distinctive retinal microvascular alterations and their relationship to choroidal nodules in patients with neurofibromatosis type 1. METHODS: This was a retrospective study where records of seventeen consecutive patients with diagnosis of NF1, presenting Lisch nodules and choroidal alterations, and 17 age and gender-matched healthy control patients were evaluated. Fundus photographs, near infrared reflectance and enhanced depth imaging - optical coherence tomography images were reviewed. Retinal microvascular abnormalities and choroidal and retinal alterations in proximity of the retinal microvacular alterations were carefully noted. RESULTS: 6 patients (35%) presented distinctive microvascular abnormalities. These consisted of small, tortuous vessels with a "spiral" or "corckscrew" aspect. They were second or third order, small tributaries of the superior or inferior temporal vein. These vessels were all located overlying choroidal alterations as observed with near infrared reflectance. Enhanced depth imaging - optical coherence tomography showed alteration of choroidal vasculature due to the presence of choroidal nodules but otherwise retinal and choroidal cross-sections were unremarkable for morphology. CONCLUSIONS: Retinal microvascular alterations overlying choroidal nodules in patients with NF1 can be considered another distinctive characteristic of the disease. Although the nature of these alterations is not clear, the authors speculate that functional disorders of vasomotor nerve cells, which originate in the embryonal neural crest can lead to their formation.

Intravitreal injection of dexamethasone implant and ranibizumab in cystoid macular edema in the course of irvine-gass syndrome.

PURPOSE: To evaluate the efficacy of 2 dexamethasone intravitreal implants and 1 ranibizumab intravitreal injection after a bilateral postoperative complication of cataract surgery as pseudophakic cystoid macular edema. PATIENTS AND METHODS: A 70-year-old male patient with systemic hypertension developed a progressive cystoid macular edema (CME) in both eyes starting between 10 and 20 days after cataract surgery. Two intravitreal dexamethasone implants and 1 ranibizumab injection were administered; first in the right eye (RE) and then in the left eye (LE). The patient was checked for 1 whole week and then once a month for 5 months after the injections. RESULTS: One month after the first dexamethasone implant in his RE, the spectral domain optical coherence tomography (SD-OCT) showed a progressive reduction of the foveal thickness until a complete resolution of the CME occurred, which was associated with an improvement of visual acuity. After 3 months, the SD-OCT showed a relapse of the CME, which was then treated with 1 injection of ranibizumab. One month after this injection, there was a complete resolution of the CME. A new CME in his RE was diagnosed 2 months after the last ranibizumab injection; it was treated with a new dexamethasone implant. A complete resolution of the CME was obtained; a normal foveal profile was still present 5 months after the last injection, and the best-corrected visual acuity was 20/20. His LE developed a CME 40 days after surgery. One intravitreal injection of ranibizumab was first administered in his LE, with a complete resolution of the CME at SD-OCT 2 weeks later. As observed in his RE, 40 days after the ranibizumab injection, there was a relapse of the CME that was treated with 1 intravitreal injection of dexamethasone implant. Five months later, the patient showed a worsening of the CME, but it was completely resolved with a second dexamethasone injection. After 3 months, the foveal thickness was back to normal with a BCVA of 20/20. CONCLUSION: Treatment with dexamethasone implants (Ozurdex(®)) and ranibizumab injections (Lucentis(®)) induced a progressive reduction of our patient's CME after cataract surgery (Irvine-Gass syndrome) until a complete normal foveal thickness was restored and his visual function was improved despite the order of injections.

Intravitreal Injection of Dexamethasone Implant in Serous Macular Detachment Associated with Waldenström's Disease.

PURPOSE: To evaluate the efficacy of one intravitreal injection of dexamethasone (Ozurdex(®); Allergan, Inc., Irvine, Calif., USA) in serous macular detachment (SMD) of one eye, associated with bilateral central retinal vein occlusion (CRVO) in a patient affected by Waldenström's macroglobulinemia (WM). PATIENTS AND METHODS: A female patient, affected by WM, complained of a progressive decrease in visual acuity, mainly in the left eye (LE). SMD in the LE associated with bilateral CRVO was diagnosed. One intravitreal injection of dexamethasone was administered in the LE and the patient was tested 1, 2, and 6 months after the injection. RESULTS: 1, 2, and 6 months after the injection, the spectral domain optical coherence tomography (SD-OCT) showed a progressive slight reduction of foveal thickness that was not related to any improvement of visual function. CONCLUSIONS: Treatment with dexamethasone (Ozurdex) induced a progressive slight reduction of SMD but no improvement of visual acuity, and it is possible that this is related to the condition of hematic hyperviscosity that is present in WM.