Clinical characteristics and treatment progress of microphthalmos-anophthalmos-coloboma / 国际眼科杂志(Guoji Yanke Zazhi)

Microphthalmos-anophthalmos-coloboma(MAC)is structurally and clinically correlated congenital ocular malformation. Although MAC is relatively rare, they still become an important cause of blindness in children. At present, the etiology of MAC is not clear; a variety of genetic or environmental factors may cause MAC. Because of its special anatomical structure, MAC has complex clinical manifestations and multiple complications. The complications including cataract, angle-closure glaucoma, uveal effusion, retinal detachment, orbital cyst, which are often accompanied with increased risks for surgery and poor prognosis. By summarizing the clinical features, the common types of ocular complications, and the progress of the treatment, this article will enhance clinicians' cognition of MAC and guide clinicians to formulate personalized and safe treatment plans for patients.

Clinical presentation and treatment outcomes of an algorithmic approach to uveal effusion syndrome

Purpose: To describe the clinical features and treatment outcomes in spontaneous uveal effusion syndrome (UES). Methods: A 10?year retrospective chart review of UES patients from a tertiary eye center was carried out. Optical coherence tomography (OCT), fundus fluorescein angiography, and ultrasound biomicroscopy (UBM) scans were performed. UES was managed based on presenting best?corrected visual acuity (BCVA), symptoms, and fundus findings. Patients with secondary causes of uveal effusion were excluded. Results: Twenty?five eyes of 16 patients were included. Of the 16 patients, 14 (88%) were male and 9 (56%) had bilateral disease. Fifteen of 25 affected eyes had nanophthalmos (axial length (AL) <20.5 mm) and 6 had hyperopia with AL >20.5 mm. The presenting mean distance BCVA was 0.74 ± 0.64 logMAR (mean Snellen: 20/100). Eleven eyes had exudative retinal detachment, and 4 also had exudative choroidal detachment (CD). Choroidal thickness (CT) was increased in 11 eyes on B?scan ultrasonography, and the mean CT was 1.74 ± 0.38 mm. Sub?retinal fluid (SRF) and retinal folds were the most common OCT findings. UBM findings included shallow angles, peripheral CD, and supra?ciliary effusion. A combination of local and systemic corticosteroids was used to successfully treat 12 eyes, 6 needed surgery, and 7 were observed. Partial sclerectomy with anterior chamber maintainer?assisted SRF drainage was the favored surgery. The median period of follow?up was 6.5 months (0.1–76 months), and the mean distance BCVA at the last follow?up was 0.58 ± 0.42 logMAR (mean Snellen: 20/80). Conclusion: UES can be suitably managed both medically and surgically based on clinical presentation

Bilateral acute retinal necrosis associated with bilateral uveal effusion in an immunocompetent patient: A challenging association

Bilateral uveal effusion syndrome associated with bilateral acute retinal necrosis is a diagnostic and therapeutic challenge. A 52 year old man presented with bilateral angle closure with choroidal detachment. With restricted fundus view, parenteral steroid was started. During close follow up bilateral discrete areas of peripheral retinitis were noted. Parenteral steroids were promptly stopped and parenteral antivirals with oral steroids were continued. It showed healing response with nil recurrences till last follow up. Aggressive treatment of bilateral uveal effusion with parenteral steroids can cause progression of bilateral acute retinal necrosis leading to phthisis bulbi. However early diagnosis, prompt intervention and close follow up are the key elements to therapeutic success even during diagnostic surprises and avoid costly mistakes.

Diagnosis and a minimum effective management for nanophthalmic uveal effusion syndrome.

A 38‑year‑old male presented with a 2‑week history of sudden blurred vision (best‑corrected visual acuity: 20/200) and pain in his right eye. Spectral domain optical coherence tomography (OCT) revealed focal thickening of the retinal pigment epithelium layer as leopard spots [Fig. 1a]. Fundus examination demonstrated exudative retinal detachment and retinal vascular dilation [Fig. 1c] whereas the contralateral eye remained unremarkable [Fig. 1b and d]. B‑scan ultrasonography showed a short axial length (17.3 mm). Fundus fluorescein angiography and indocyanine green angiography revealed leopard spots of granular hyperfluorescence in the posterior and inferior quadrants [Fig. 1e], which were increased with time and persisted until the late phase [Fig. 1f]. On the basis of clinical findings, we diagnosed him and it revealed to be nanophthalmic uveal effusion syndrome (UES).[1] After 10 mg dexamethasone was applied intravenously, two‑third thickness scleral flaps measuring 4 mm × 5 mm were performed at inferotemporal and inferonasal sites [Fig. 2a]. Under them, the remaining sclerae were excised measuring 1 mm × 2 mm accordingly [Fig. 2b and c]. Biopsy [Fig 2d] and pathologic evaluation revealed fibroblastic proliferation and hyaline degeneration of lamellar sclera [Fig. 2e and f]. The effusion was not detected at least on 6‑month follow‑up [Fig. 3], and the visual acuity was stabled at 20/100.

Full-thickness Sclerotomy for Uveal Effusion Syndrome

To report the surgical outcome of full-thickness sclerotomy in five cases of uveal effusion syndrome (UES). Full-thickness sclerotomy without sclerectomy was performed on five eyes of four patients with UES with or without nanophthalmos. In four of the eyes, exudative retinal detachment associated with UES resolved after the sclerotomy. The subretinal fluid in one eye, which had a normal axial length, was relieved after undergoing three sclerotomy procedures. Full-thickness sclerotomy without vortex vein decompression or sclerectomy is an effective surgical option for the management of significant UES.

Clinical feature and management of uveal effusion syndrome / 中华实验眼科杂志

Background Uveal effusion syndrome is uncommon in clinic.To understand the clinical characteristics of uveal effusion syndrome is helpful for rescuing visual acuity of patient.Objective This study was to discuss the diagnosis,classification and surgical outcome of uveal effusion syndrome.Methods This was a descriptive study.The clinical data of 14 eys from 10 patients with uveal effusion syndrome,ineluding ophthalmologic examination,B-scan sonography,ultrasound biomicroscopy (UBM),fundus fluorescence angiography (FFA),indocyanine green angiography (ICGA),surgical treatment and prognosis,were retrospectively analyzed.The follow-up period was 6 months.Results The fundus findings of all impacted eyes showed bullous-shape retinal detachment (RD).B-scan sonography revealed retinal and choroidal detachment.A annular peripheral ciliochoroidal detachment was observed in the cases under the UBM.FFA exhibited leopard spots without any leakage from choroid into the subretinal space.ICGA demonstrated diffusely choroidal granular hyperfluorescence in the very early phase,which presented with an increasing intensity as time lapse until the late phase.Full-thickness sclerectomy was performed on 4 eyes of 2 patients and subscleral sclerectomy was performed in 1 eye of 1 patient,achieving a retinal anatomic reattachment after surgery.All of the patients finished the fellow-up.No recurrence of RD was seen during the followup duration.Conclusions Comprehensive preoperative evaluation,including ophthalmologic ultrasonography,MRI and CT,is crucial for accurate classification of uveal effusion syndrome and determine of proper management strategy.

Scleral Window Surgery with Mitomycin C for Nanophthalmic Uveal Effusion: Case Report

PURPOSE: To report the case of a 36-year-old patient with nanophthalmic uveal effusion was treated with scleral window surgery and topical administration of mitomycin C (MMC). CASE SUMMARY: A 36-year-old woman presented with decreased visual acuity and blurred vision in the both eyes during 3 months. Fundus examination revealed choroidal effusion and retinal detachment with thickend sclera wall and short axial lengh. Partial-thickness sclera flap with deep sclerostomy was performed and topical MMC was administered to one quadrant of the equatorial sclera. The subretinal fluid resorbed gradually and visual acuity improved. CONCLUSIONS: Scleral window surgery and topical mitomycin C might relieve the blocked transscleral outflow of intraocular fluid in the small area of a sclerostomy in young patient with nanophthlamos.

Castleman's Disease Presenting with Uveal Effusion Syndrome

We report a rare case of multicentric Castleman's disease that presented with ophthalmic involvement, along with a review of the literature. A 63-year-old male presented with decreased visual acuity in both eyes. Both eyes had serous elevations of the retinas with shifting subretinal fluid and annular choroidal detachment. No retinal breaks were found. Laboratory tests revealed pancytopenia, hypergammaglobulinemia, and an increased erythrocyte sedimentation rate. Chest and abdominal computed tomographies showed multiple lymphadenopathies in the mediastinum, abdomen, and in both inguinal areas. Histological examination of the inguinal lymph node biopsy was consistent with Castleman's disease. After combination chemotherapy, the serous elevations of both retinas and the annular choroidal detachments of both eyes disappeared. Ophthalmic involvement in Castleman's disease is very rare, and to the authors' knowledge, this is the first report of ophthalmic involvement of Castlemans's disease in Korea.

Two Cases of Uveal Effusion Syndrome

PURPOSE: To report a case of uveal effusion syndrome associated with hypotony and a case of uveal effusion syndrome in nanophthalmos. METHODS: The first case was a 25-year-old man who presented with decreased visual acuity in the left eye and hypotony. Fundus examination revealed choroidal effusion and retinal detachment with a thickened eyeball. Partial thickness sclerotomy and sclerectomy were performed. The second case was a 13-year-old boy who had uveal effusion syndrome with a nanophthalmic eye. RESULTS: In the patient with hypotony, intraocular pressure was well maintained following partial thickness sclerotomy and sclerectomy, and choroidal effusion and retinal detachment were reduced. The visual acuity of the nanophthalmic patient was well maintained during a 3-year follow-up period without treatment. CONCLUSIONS: appropriate treatment modalities should be considered depending on the ophthalmic condition of the individual patient.

Change of Intraocular Pressure Induced by Viscoelastics in Cataract Surgery with Intentional Posterior Capsule Ruptrue

The cataract surgery with viscoelastic agent was performed in forty eyes of twenty rabbits which were divided into two groups, one group with intact posterior capsule and the other group with ruptured posterior capsule of the lens, to evaluate the effect of viscoelastic agents on IOP according to presence of the intact posterior capsule of the lens after cataract surgery. The viscoelastics used were Healon in control group, and Healon, Viscoat and Occucoat in experimental group. We checked the IOP during one month. Peak IOP after surgery and the time from surgery to the peak IOP showed no difference between two groups. In experimental group, there was no difference in the change of postoperative IOP according to the kind of viscoelastics. Results suggest that the rupture of posterior capsule of the lens plays a role in delayed excretion of the viscoelastics, and the postoperative elevation of IOP was not different regardless of the physical difference of Viscoelastics.

A Case of Nanophthalmos

Nanophthalmos is a rare disease that results from arrested development of the globe after closure of the embryonic fissure and is characterized by a small eye with thickened sclera, uveal effusion, non-rhegmatogenous retinal detachment and narrow angle glaucoma. Treatments include medical, laser and surgical theraphy for glaucoma, and surgical treatments like scleral resection, anterior sclerotomy and vortex vein decompression surgery for the drainage of the uveal effusion, etc. We experienced a case of nanophthalmos with narrow angle glaucoma and choroidal effusion in 34 year-old male patients. Anterior sclerotomy for the drainage of the uveal effusion was performed and the scleral tissue obtained. Histopathologic study on the sclera showed abnormal collagen fibers.