scholarly journals Multimodal Imaging Findings and Treatment with Dexamethasone Implant in Three Cases of Idiopathic Macular Telangiectasia Type 1

2021 ◽  
pp. 92-97
Author(s):  
Paola Cirafici ◽  
Maria Musolino ◽  
Michela Saccheggiani ◽  
Carlo Enrico Traverso ◽  
Massimo Nicolò
Keyword(s):  
Visual Acuity ◽  
Macular Edema ◽  
Multimodal Imaging ◽  
Diagnostic Errors ◽  
Wide Field ◽  
Vascular Changes ◽  
Macular Telangiectasia ◽  
First Choice ◽  
Idiopathic Macular Telangiectasia

This is a report of 3 patients diagnosed with idiopathic macular telangiectasia type 1 (MacTel 1) at Medical Retina Center of the University of Eye Clinic of Genova and then prospectically followed for a mean time of 26 months between 2016 and 2019. It is the first report of patients affected by MacTel 1 treated with dexamethasone (DEX) implant as a first choice of treatment. Aim of our study is to better characterize the disease using a multimodal wide-field imaging and to determine efficacy of DEX implant on MacTel 1 in terms of central macular thickness (CMT) and best-corrected visual acuity (BCVA). MacTel 1 is a rare unilateral disease, characterized by telangiectatic retinal capillaries, cystoid macular edema, and lipid deposition occurring temporal to the fovea. Patients underwent a comprehensive ophthalmic examination, BCVA, swept-source optical coherence tomography (SS-OCT), SS-OCT angiography (SS-OCTA), ultra-widefield (UWF) color, and fluorescein angiography (FA) fundus photograph. All the patients presented monolateral reduced BCVA and macular edema with increased CMT evaluated by SS-OCT. With SS-OCTA, we showed that the telangiectasia-associated vascular changes originate in the deep retinal vascular plexus and as a consequence macular edema and exudation develop causing vision loss. Furthermore, UWF imaging helped us to highlight vascular changes typical of Coats Disease at the far retinal periphery. All the patients were treated with DEX intravitreal implant, showing a decrease in CMT and a stabilization of visual acuity. Due to the recurrent nature of macular edema, patients underwent a mean of 4 DEX implants during the follow-up period. In order to address the clinical features of this uncommon disease avoiding diagnostic errors, it might be important to use a multimodal imaging approach. The anatomical and functional beneficial effects of DEX implant were well evident although transient.

scholarly journals Bilateral Type 1 Idiopathic Macular Telangiectasia in a Female Patient: Multimodal Imaging of a Rare Presentation

2021 ◽  
pp. 373-379
Author(s):  
Mohamad Reza Niyousha ◽  
Narges Hassanpoor ◽  
Sasan Jafari
Keyword(s):  
Optical Coherence Tomography ◽  
Macular Edema ◽  
Female Patient ◽  
Multimodal Imaging ◽  
Rare Presentation ◽  
Macular Telangiectasia ◽  
Healthy Female ◽  
Capillary Plexus ◽  
Idiopathic Macular Telangiectasia

We report a bilateral case of type 1 idiopathic macular telangiectasia (IMT) in a female patient. A 40-year-old otherwise healthy female with gradual vision loss since 2 years ago with best-corrected visual acuity of 20/400 in both eyes was referred. Her past medical history was negative for any systemic disease including diabetes and systemic hypertension. Color fundus photography showed bilateral loss of normal foveal reflex with macular edema. Fluorescein angiography demonstrated symmetric perifoveal telangiectasia mainly in the superior and nasal macula in both eyes with late staining and leakage. Spectral-domain optical coherence tomography revealed significant intraretinal fluid bilaterally and subretinal fluid in the left eye. Optical coherence tomography angiography (OCTA) revealed obvious saccular parafoveal capillary telangiectasia and capillary dropout as well as decreased vascular density in both superficial and deep capillary plexus. Deep capillary plexus involvement in OCTA was more evident than superficial plexus. Based on the patient’s medical history and multimodal imaging, the diagnosis of bilateral IMT type 1 was made. The patient underwent 5 intravitreal monthly injection of bevacizumab in both eyes, which resulted in macular edema resolution. However, after 3 months of discontinuation of intravitreal bevacizumab, macular edema relapsed. In conclusion, type 1 IMT can occur bilaterally in an otherwise healthy female patient as a very rare presentation. To the best of our knowledge, this case is the 4th case of bilateral type 1 IMT reported in a female.

scholarly journals Efficacy of Laser Photocoagulation for Type 1 Idiopathic Macular Telangiectasia: A Retrospective Study

2021 ◽  
pp. 9-16
Author(s):  
Hirofumi Kono ◽  
Katsuhiko Yokoyama ◽  
Daiji Kishi ◽  
Kenichi Kimoto ◽  
Toshiaki Kubota
Keyword(s):  
Visual Acuity ◽  
Macular Edema ◽  
Laser Photocoagulation ◽  
Macular Thickness ◽  
University Hospital ◽  
Type I ◽  
Central Macular Thickness ◽  
Macular Telangiectasia ◽  
Idiopathic Macular Telangiectasia

Aims: The aim of this study was to evaluate the efficacy of laser photocoagulation for type 1 idiopathic macular telangiectasia (MacTel). Study Design: Retrospective case series Place and Duration of Study: Department of Ophthalmology, Oita University Hospital during the period from 2004 to 2013. Patients and Methods: Six eyes of 6 patients with type 1 MacTel diagnosed consecutively in the Oita University Hospital during the period from 2004 to 2013 were studied. They showed macular edema and their visual acuity were deteriorated due to leakage from the aneurysms. Four patients were male and two were female with an average age of 64 years (range: 41 to 74 years). Informed consent was obtained from each patient and the laser photocoagulation was applied to leaky aneurysms in all eyes. The central macular thickness (CMT) was measured by optical coherence tomography (OCT). Mean follow up period was 44 months (range: 24 - 63 months).  Results: Mean visual acuity significantly increased from logMAR 0.25±0.27 at baseline to logMAR 0.11±0.14 (P<0.05) at the last visit. Visual acuity improved more than 0.2 logMAR in five eyes and was stable (gain or loss of < 0.3 logMAR) in one eye. Mean central macular thickness significantly decreased from 454±181 μm at baseline to 231±67 μm (P<0.05) at the last visit. Macular edema was not detected by OCT inany patients at the last visit. Conclusion: In type I MacTel, laser photocoagulation may achieve a visual improvement and normalize the central macular thickness.

MULTIMODAL IMAGING IN TYPE 2 IDIOPATHIC MACULAR TELANGIECTASIA

Retina ◽  
2015 ◽  
Vol 35 (4) ◽  
pp. 742-749 ◽  
Author(s):  
Ferenc B. Sallo ◽  
Irene Leung ◽  
Traci E. Clemons ◽  
Tunde Peto ◽  
Alan C. Bird ◽  
...  
Keyword(s):  
Multimodal Imaging ◽  
Macular Telangiectasia ◽  
Idiopathic Macular Telangiectasia

scholarly journals Pars Plana Vitrectomy Combined with Focal Endolaser Photocoagulation for Idiopathic Macular Telangiectasia

2014 ◽  
Vol 2014 ◽  
pp. 1-6 ◽  
Author(s):  
Gaku Terauchi ◽  
Celso Soiti Matsumoto ◽  
Kei Shinoda ◽  
Harue Matsumoto ◽  
Yutaka Imamura ◽  
...  
Keyword(s):  
Fluorescein Angiography ◽  
Pars Plana Vitrectomy ◽  
Late Phase ◽  
Macular Telangiectasia ◽  
Before And After ◽  
Antiangiogenic Drugs ◽  
Pars Plana ◽  
Idiopathic Macular Telangiectasia ◽  
Sd Oct

Background. To report the outcome of pars plana vitrectomy (PPV) combined with intraoperative endolaser focal photocoagulation (PC) on eyes with idiopathic macular telangiectasis (MacTel) type 1.Methods. This was a retrospective study of two female patients with MacTel type 1 who were resistant to focal photocoagulation, sub-Tenon triamcinolone injection, and/or antiangiogenic drugs. The best-corrected visual acuity (BCVA) was determined, and fluorescein angiography (FA) and spectral domain optical coherence tomography (SD-OCT) were performed before and after surgery for up to 19 months.Results. After surgery, the BCVA gradually improved from 20/100 to 20/20 at 19 months in Case 1 and from 20/50 to 20/13 at 13 months in Case 2. Fluorescein angiography (FA) showed leakage at the late phase, and OCT showed that the cystoid macular edema was resolved and the fovea was considerably thinner postoperatively.Conclusion. Patients with MacTel type 1 who are refractory to the other types of treatments can benefit from PPV combined with intraoperative endolaser focal PC with functional and morphological improvements.

scholarly journals Multimodal imaging of Hurler syndrome-related keratopathy treated with deep anterior lamellar keratoplasty

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Elodie Da Cunha ◽  
Cristina Georgeon ◽  
Nacim Bouheraoua ◽  
Marc Putterman ◽  
Françoise Brignole-Baudouin ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Multimodal Imaging ◽  
Lamellar Keratoplasty ◽  
Deep Anterior Lamellar Keratoplasty ◽  
Imaging Features ◽  
Hurler Syndrome ◽  
Hematopoietic Stem ◽  
First Choice ◽  
Anterior Lamellar Keratoplasty ◽  
Sd Oct

Abstract Background Hurler syndrome-associated keratopathy is an exceedingly rare corneal disorder that requires corneal transplantation in advanced stages. Precise assessment of the corneal condition is necessary for deciding which type of keratoplasty (i.e., deep anterior lamellar or penetrating) should be proposed. We aimed to confront the results of multimodal imaging with those of histology in a case of Hurler syndrome-associated keratopathy. Case presentation A 16-year-old patient with Hurler’s syndrome treated with hematopoietic stem cell transplantation was referred for decreased vision related to advanced keratopathy. The patient was treated with deep anterior lamellar keratoplasty (DALK) in both eyes with uncomplicated outcome. Visual acuity improved from 0.1 (20/200) preoperatively to 0.32 (20/63) and 0.63 (20/32) after transplantation. The corneal endothelial cell density was 2400 cells/mm2 in both eyes 3 years after transplantation. In vivo confocal microscopy (IVCM) and spectral domain optical coherence tomography (SD-OCT) were performed preoperatively. The corneal buttons retrieved during keratoplasty were processed for histology. In SD-OCT scans, corneal opacities appeared as diffuse stromal hyperreflectivity associated with increased corneal thickness. IVCM showed diffuse cytoplasmic granular hyperreflectivity and rounded/ellipsoid aspects of keratocytes, presence of small intracellular vacuoles, and hyperreflective epithelial intercellular spaces. Bowman’s layer was thin and irregular. The corneal endothelium was poorly visualized but no endothelial damage was observed. Histology showed irregular orientation and organization of stromal lamellae, with the presence of macrophages whose cytoplasm appeared clear and granular. A perinuclear clear halo was visible within the epithelial basal cells. Bowman’s layer featured breaks and irregularities. Conclusions The observed corneal multimodal imaging features in mucopolysaccharidosis-related keratopathy were concordant with histology. Compared with standard histology, multimodal imaging allowed additional keratocyte features to be observed. It revealed both morphological and structural changes of all corneal layers but the endothelium. This information is essential for therapeutic management which should include DALK as the first-choice treatment in case of impaired visual acuity.

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