scholarly journals Retinal and optic nerve functions in incontinentia pigmenti: long-term elctrophysiological follow-up

2020 ◽  
Vol 13 (2) ◽  
pp. 15-20
Author(s):  
Márta Janaky ◽  
Ágnes Jánossy ◽  
Attila Kovács ◽  
Daniella Lőrincz ◽  
Dóra Nagy ◽  
...  
Keyword(s):  
Vision Loss ◽  
Clinical Symptoms ◽  
Incontinentia Pigmenti ◽  
Inherited Disease ◽  
Multisystem Disorder ◽  
Inflammatory Reactions ◽  
Asymmetric Pattern ◽  
Functional Involvement ◽  
The Right

Incontinentia pigmenti (IP) is a rare, X-linked, dominantly inherited disease affecting mostly females, which is best characterized as an autoimmune disease. It is a multisystem disorder affecting ectodermal tissues. Ocular abnormalities usually occur early in childhood, with subsequent retinal detachment and vision loss. Vision rarely remains intact until adulthood. We present the 17-year visual electrophysiological follow-up of such a rare patient and her mother. The mother was only a carrier, but the daughter developed various manifestations of IP. The aim of our investigations was to obtain information on the progression of functional deterioration in IP. Electroretinography (ERG), multifocal electroretinography (mfERG), visual evoked potentials (VEP), ultrasound (US) and optical coherence tomography (OCT) were performed at regular intervals between the patient’s ages of 9 and 26 years (2003 to 2020). From 9 to 22 years of age, a characteristic picture of spared vision with minimal ophthalmoscopic alterations and fluctuating ERG anomalies were observed in the left eye. It was only between the ages of 22 and 23 that subjective symptoms developed, and then complete loss of vision in the affected eye ensued rapidly. The right eye remained clinically asymptomatic throughout the observation period. The mother remained completely asymptomatic, but she showed similar ERG alterations. Electroretinography is a sensitive indicator of the activity of the ocular immune or inflammatory reactions in IP, and it readily detects their functional effect even in the absence of clinical symptoms. Thus, it is recommendable not only for the longterm functional follow-up of these patients, but probably also for early disease-specific screening. ERG recordings from the presented case suggest that the characteristic, asymmetric pattern of retinal functional involvement may be traced back to the different degrees to which the two eyes were exposed to the intermittent reactivations of the disease.

scholarly journals Recurrent Intraocular Lens Dislocation in a Patient with Familial Ectopia Lentis

2021 ◽  
Vol 18 (9) ◽  
pp. 4545
Author(s):  
Tomasz K. Wilczyński ◽  
Alfred Niewiem ◽  
Rafał Leszczyński ◽  
Katarzyna Michalska-Małecka
Keyword(s):  
Intraocular Lens ◽  
Pars Plana Vitrectomy ◽  
Vision Loss ◽  
Silicone Oil ◽  
Single Case ◽  
Recurrent Dislocation ◽  
Ectopia Lentis ◽  
Pars Plana ◽  
The Right

A 36-year-old patient presented to the hospital with recurrent dislocation of the intraocular lens (IOL). The patient with the diagnosis of familial ectopia lentis was first operated on for crystalline lens subluxation in the left eye in 2007 and in the right eye in 2009. In both eyes, lens extraction with anterior vitrectomy and transscleral fixation of a rigid IOL was performed. In 2011, the IOL in the right eye luxated into the vitreous cavity due to ocular trauma. The patient underwent a pars plana vitrectomy with the IOL resuturation to the sclera. Seven years later, a spontaneous vision loss in the right eye was caused by a retinal detachment. The pars plana vitrectomy with silicone oil tamponade and a consequential oil removal three months later were performed in 2018. The follow-up examination revealed recurrent IOL dislocation in the same eye. Due to a history of previous suture-related complications a decision was made to remove the subluxated rigid polymethyl-methacrylate (PMMA) IOL and fixate to sclera a sutureless SOLEKO FIL SSF Carlevale lens. The purpose of this report is to present a single case of a 36-year-old patient who was presented to the hospital with recurrent dislocation of the intraocular lens. In a three-month follow-up period, a good anatomical and functional outcome was finally obtained with transscleral sutureless intraocular lens. This lens is an option worth considering especially in a young patient with a long life expectancy and physically active.

scholarly journals Surgical Management of a Patient with Anterior Megalophthalmos, Lens Subluxation, and a High Risk of Retinal Detachment

2017 ◽  
Vol 8 (1) ◽  
pp. 61-66 ◽  
Author(s):  
María Carmen Guixeres Esteve ◽  
Augusto Octavio Pardo Saiz ◽  
Lucía Martínez-Costa ◽  
Samuel González-Ocampo Dorta ◽  
Pedro Sanz Solana
Keyword(s):  
Retinal Detachment ◽  
Vision Loss ◽  
Lens Subluxation ◽  
Intraocular Lens Dislocation ◽  
Common Causes ◽  
History Of ◽  
Anterior Megalophthalmos ◽  
The Right ◽  
Iris Claw

The early development of lens opacities and lens subluxation are the most common causes of vision loss in patients with anterior megalophthalmos (AM). Cataract surgery in such patients is challenging, however, because of anatomical abnormalities. Intraocular lens dislocation is the most common postoperative complication. Patients with AM also seem to be affected by a type of vitreoretinopathy that predisposes them to retinal detachment. We here present the case of a 36-year-old man with bilateral AM misdiagnosed as simple megalocornea. He had a history of amaurosis in the right eye due to retinal detachment. He presented with vision loss in the left eye due to lens subluxation. Following the removal of the subluxated lens, it was deemed necessary to perform a vitrectomy in order to prevent retinal detachment. Seven months after surgery, an Artisan® Aphakia iris-claw lens was implanted in the anterior chamber. Fifteen months of follow-up data are provided.

scholarly journals The Use of Intravitreal Ranibizumab for Choroidal Neovascularization Associated with Vogt-Koyanagi-Harada Syndrome

2011 ◽  
Vol 2011 ◽  
pp. 1-3 ◽  
Author(s):  
A. M. Kolomeyer ◽  
M. S. Roy ◽  
D. S. Chu
Keyword(s):  
Vision Loss ◽  
Corticosteroid Treatment ◽  
Exudative Retinal Detachment ◽  
Intravitreal Ranibizumab ◽  
Loss To Follow Up ◽  
Finger Counting ◽  
Combinational Treatment ◽  
Vkh Syndrome ◽  
The Right

Purpose. To describe the use of intravitreal ranibizumab for choroidal neovascular membrane (CNVM) secondary to Vogt-Koyanagi-Harada (VKH) syndrome.Methods. Interventional case report.Results. A 50-year-old woman presented with conjunctival injection and bilateral eye pain. Vision was 20/400 and 20/80 in the right and left eyes, respectively. Bilateral iritis, vitritis, and choroidal thickening were evident. Exudative retinal detachment was present in the left eye. Corticosteroid treatment improved vision to 20/40 bilaterally. Methotrexate (MTX) was initiated and vision remained stable for 3 months. After a 5-month loss to follow-up, vision in the left eye decreased to finger counting (CF) and a parafoveal CNVM was identified. After 3 intravitreal ranibizumab injections, vision improved to 20/40. Twelve months later, despite inflammation control, vision decrease to CF due to recurrent CNVM. A fourth ranibizumab injection was given. Twenty months later, best-corrected vision was 20/400, and an inactive CNVM was present in the left eye.Conclusion. After initial CNVM regression and visual acuity improvement due to ranibizumab, the CNVM recurred and became refractory to treatment. Despite control of inflammation and neovascularization, VKH chronicity lead to permanent vision loss in our patient. A combinational treatment approach may be required in such patients.

Anterior and posterior ischemic optic neuropathy following liposuction surgery in a previously healthy young woman

2020 ◽  
pp. 112067212096206
Author(s):  
Kaveh Abri Aghdam ◽  
Ali Sadeghi ◽  
Mostafa Soltan Sanjari ◽  
Ali Aghajani ◽  
Saba Gholamalizadeh
Keyword(s):  
Optic Neuropathy ◽  
Vision Loss ◽  
Ischemic Optic Neuropathy ◽  
Laboratory Studies ◽  
Level Of Evidence ◽  
Healthy Young Woman ◽  
Posterior Ischemic Optic Neuropathy ◽  
The Right ◽  
Liposuction Surgery

A previously healthy 28-year-old female developed bilateral painless vision loss, more prominent in the right eye than in the left, following abdominoplasty and liposuction surgery. Laboratory studies showed severe peri- and post-operative anemia. Over a 5-month follow-up, visual function remained decreased but stable in the right eye and improved in the left eye. This is the second reported case of anterior ischemic optic neuropathy in one eye and posterior ischemic optic neuropathy in the other eye after liposuction. Level of evidence: Level VI, case report study.

Retinal vasoproliferative tumour secondary to retinitis pigmentosa sine pigmento

2021 ◽  
Vol 14 (5) ◽  
pp. e240878
Author(s):  
Albert John Bromeo ◽  
Sweet Jorlene Lerit ◽  
Amadeo Veloso ◽  
Gary John Mercado
Keyword(s):  
Retinal Detachment ◽  
Retinitis Pigmentosa ◽  
Laser Photocoagulation ◽  
Vision Loss ◽  
Peripheral Retina ◽  
Focal Laser Photocoagulation ◽  
Feeder Vessel ◽  
Eye Examination ◽  
The Right

Retinitis pigmentosa can be associated with exudative vasculopathy in rare instances, which can manifest as retinal vasoproliferative tumours. We present the case of a 33-year-old woman previously diagnosed with retinitis pigmentosa sine pigmentosa in both eyes. She was asymptomatic and just came in for a routine follow-up eye examination. Thorough examination of the peripheral retina on the right eye revealed a dome-shaped retinal tumour with a feeder vessel and surrounding exudative changes at the superotemporal periphery, consistent with a secondary retinal vasoproliferative tumour from retinitis pigmentosa. She subsequently underwent focal laser photocoagulation of the tumour which resulted in tumour stabilisation. While exudative vasculopathy is very uncommon in retinitis pigmentosa, ophthalmologists need to be aware of its occurrence in such patients. Vision loss may occur from exudation, haemorrhage, retinal detachment and neovascularisation. A thorough examination of the peripheral retina is warranted in these cases.

scholarly journals Endoscopic Endonasal Approach to a Suprasellar Craniopharyngioma

2018 ◽  
Vol 79 (S 03) ◽  
pp. S237-S238 ◽  
Author(s):  
Alexandre Todeschini ◽  
Alaa Montaser ◽  
Mostafa Shahein ◽  
Juan Revuelta ◽  
Bradley Otto ◽  
...  
Keyword(s):  
Vision Loss ◽  
Third Ventricle ◽  
Collagen Matrix ◽  
Endoscopic Endonasal ◽  
Stereotactic Radiation ◽  
The Third ◽  
Head Clamp ◽  
The Right ◽  
Adamantinomatous Craniopharyngioma

AbstractWe present the case of a 57-year-old male who presented with progressive right side vision loss whose workup revealed a large suprasellar lesion with invasion of the third ventricle. The pituitary stalk was not visible.Hormonal panel showed no hormonal deficits. The initial diagnosis was of a type II transinfundibular craniopharyngioma (as classified by Kassam et al).An endoscopic endonasal transplanum transtuberculum approach was done using a standard binostril four-hand technique, with the patient positioned supine with the head turned to the right side and tilted to the left, fixed in a three-pin head clamp, under imaging guidance. The tumor was carefully dissected away from the optic apparatus while preserving the vessels, mainly the superior hypophyseal artery. The stalk was identified around the tumor and preserved. The third ventricle was entered and inspected at the end of the procedure and a near-total resection (a small residual in the right hypothalamus) with decompression of the optic apparatus was achieved. Reconstruction was done in a multilayered fashion, using collagen matrix and a nasoseptal flap. Patient had an uneventful postoperative stay and was discharged on POD 4, neurologically stable with no hormonal deficits. Pathology confirmed an adamantinomatous craniopharyngioma. Due to a small growth of the residual, patient underwent fractionated stereotactic radiation (50.4Gy in 28 sessions). He presented with panhypopituitarism 2 years after radiation therapy. At 3-month follow-up, his vision was back to normal and 6-year postoperative magnetic resonance imaging showed no signs of recurrence.The link to the video can be found at: https://youtu.be/chG7XIz7a_A.

scholarly journals Corneal crosslinking in a case with Axenfeld–Rieger syndrome and unilateral pellucid marginal degeneration

2019 ◽  
Vol 11 ◽  
pp. 251584141882228 ◽  
Author(s):  
Mustafa Koc ◽  
Pinar Kosekahya ◽  
Merve Inanc ◽  
Kemal Tekin
Keyword(s):  
Visual Acuity ◽  
Male Patient ◽  
Vision Loss ◽  
Slit Lamp ◽  
Slit Lamp Examination ◽  
Corneal Crosslinking ◽  
Rieger Syndrome ◽  
Corrected Distance Visual Acuity ◽  
The Right

A 31-year-old male patient presented with the complaint of progressive vision loss in his left eye. Slit-lamp examination showed posterior embryotoxon, iris hypoplasia, and iridocorneal adhesion in both eyes, corectopia in the right, and peripheral inferior thinning and ectasia in the left eye. Corneal topography showed slightly asymmetric bowtie pattern in the right eye and crab-claw pattern in the left eye. Topographic examination was compared with his previous topography. The comparison showed 1.6-D steepening of maximum keratometry ( Kmax) and 22-µm decrease of thinnest corneal pachymetry. Corneal crosslinking treatment was performed on the left eye. At the postoperative 28-month follow-up visit, Kmax decreased from 54.1 to 53.0 D and corrected distance visual acuity improved to 20/20 with scleral lens. This is the first reported a case with Axenfeld–Rieger syndrome and pellucid marginal degeneration association. We suggest that corneal crosslinking can be useful for management of pellucid marginal degeneration and longer follow-up might be needed in order to corroborate the effectiveness of the corneal crosslinking procedure.

Choroidal Neovascularization in Multifocal Choroiditis after Dabrafenib and Trametinib

2017 ◽  
Vol 27 (6) ◽  
pp. e184-e186 ◽  
Author(s):  
Giorgia C. Albertini ◽  
Eleonora Corbelli ◽  
Maurizio Battaglia Parodi ◽  
Francesco Bandello
Keyword(s):  
Combination Therapy ◽  
Metastatic Melanoma ◽  
Choroidal Neovascularization ◽  
Multimodal Imaging ◽  
Vision Loss ◽  
Functional Improvement ◽  
Multifocal Choroiditis ◽  
History Of ◽  
The Right

Purpose To describe a case of bilateral choroidal neovascularization (CNV) in multifocal choroiditis (MFC) associated with dabrafenib and trametinib chemotherapy for metastatic melanoma. Case We present a case of a 57-year-old man with MFC who underwent combination therapy with dabrafenib plus trametinib for metastatic melanoma. The patient presented to our ophthalmology department complaining of bilateral vision loss of 2 days’ duration. He underwent multimodal imaging showing a MFC reactivation complicated by bilateral CNV. The patient underwent 3 ranibizumab injections in the right eye and 7 ranibizumab injections in the left eye. Anatomical and functional improvement has been observed. Conclusions This report emphasizes the importance of strict ophthalmologic follow-up in patients with metastatic melanoma treated with dabrafenib plus trametinib since rare severe ocular toxicities can occur, especially in patients with a history of uveitis.

scholarly journals Renal Endometriosis Mimicking Cystic Renal Tumor: Case Report and Literature Review

2021 ◽  
Vol 8 ◽  
Author(s):  
Ye Yang ◽  
Xinxin Zhao ◽  
Ying Huang
Keyword(s):  
Renal Mass ◽  
Clinical Symptoms ◽  
Renal Tumor ◽  
Cystic Mass ◽  
Case Presentation ◽  
Contrast Enhanced ◽  
The Right ◽  
Enhanced Computed Tomography ◽  
Contrast Enhanced Computed Tomography

Background: Endometriosis mainly affects female pelvic tissues and organs, and the presence of endometriosis in the kidney is extremely rare.Case Presentation: We report a case of a 48-year-old woman who presented with intermittent hematuria. She was found to have a cystic mass on renal ultrasonography, and contrast-enhanced computed tomography (CT) showed slight enhancement of the cystic wall and septa. These findings were indicative of cystic renal tumor. The patient subsequently underwent partial right nephrectomy. Histopathology revealed endometriosis of the right renal parenchyma. The patient recovered well and had no evidence of a recurrent renal mass at the 3 months' follow up.Conclusion: The possibility of renal endometriosis should be considered in a female patient with a cystic renal mass and clinical symptoms related to the menstrual cycle.

scholarly journals Case Report and Literature Review: Catastrophic Embolism Following Cosmetic Injection of Autologous Fat in the Face

2021 ◽  
Vol 8 ◽  
Author(s):  
Chunyu Liu ◽  
Zhaolun Cai ◽  
Lingli Zhang ◽  
Muke Zhou ◽  
Li He
Keyword(s):  
Vision Loss ◽  
Fat Embolism ◽  
External Carotid Artery ◽  
External Carotid ◽  
Significant Heterogeneity ◽  
Autologous Fat ◽  
Retinal Arterioles ◽  
The Face ◽  
The Right

Injection of autologous fat on the face is a commonly performed procedure in plastic surgery. However, it can lead to rare but devastating complications due to fat embolism. In this study, we presented two cases of cerebral infarction and/or sudden vision loss after cosmetic injections of autologous fat on the face. Two women underwent injections into the temporal and frontal areas, respectively. In case 1, the patient underwent decompressive craniectomy as her condition deteriorated continuously and died. In case 2, the patient's vision had not improved at the 3-month follow-up visit. Imaging examinations showed occlusion of the right external carotid artery in case 1, and multiple retinal arterioles were segmentally occluded in case 2. We also screened relevant studies via a systematic search of PubMed (last updated on May 9, 2020) and performed a narrative review due to the significant heterogeneity between the studies. To prevent this catastrophic event, the autologous fat injection should be performed carefully. If embolization does occur, early diagnosis and timely treatment may help improve functional outcomes.

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