Rapid resolution of severe exudation in uveal effusion syndrome with anti-vascular endothelial growth factor alone in a case of bilateral nanophthalmos: a case report

Background Uveal effusion syndrome is a rare disease characterized by exudative detachments of the choroid, ciliary body, and retina. Various surgical procedures and nonsurgical strategies have been described to treat uveal effusion syndrome with limited success. The treatment for uveal effusion syndrome remains a serious challenge for clinicians. To the best of our knowledge, no previous report has described a severe uveal effusion syndrome patient with nanophthalmos treated by using an anti-vascular endothelial growth factor agent alone. We report here one such case with unexpected positive results. Case presentation A 30-year-old Chinese male patient presented with painless vision loss in both eyes that had persisted for 2 months. Examination of the right eye revealed a best corrected visual acuity of 0.03; the best corrected visual acuity of the left eye was finger count/20 cm. The intraocular pressure was normal on both eyes. A-scan revealed an right eye axial length of 15.88 mm and a left eye axial length of 16.21 mm. In the right eye, half of the peripheral choroid and nearly three-fourths of the retina were detached. The left fundus was not visible because of the total retinal detachment located just behind the lens, which could be clearly observed directly with a slit lamp. Considering all the possibilities and available treatments as well as the patient’s intentions after discussion, we first administered an intravitreal injection of ranibizumab 0.5 ml into both eyes. The patient’s visual perception improved 3 days after the injection. One month later, most of the effusion under the choroid and retina was absorbed. Visual acuity improved from finger count to 0.05 in both eyes, and vision quality was remarkably improved. Encouraged by this good result, the patient opted to undergo a second injection 1 month later. Choroidal and retinal detachment completely vanished 30 days after the second injection. Conclusions Using an anti-vascular endothelial growth factor agent alone may be a potentially effective and safe method for managing some types of uveal effusion syndrome, such as in nanophthalmos. The injection may be administered before considering more aggressive procedures in some uveal effusion syndrome patients.

Choroidal Effusion Mimicking Uveal Melanoma: A Novel Presentation of Idiopathic Systemic Capillary Leak Syndrome

<b><i>Background:</i></b> Idiopathic systemic capillary leak syndrome (ISCLS, also known as Clarkson’s disease) is a rare medical condition characterized by episodes of capillary endothelial cell dysfunction with leakage of fluid into the interstitial space resulting in severe hypotension, hemoconcentration, hypoalbuminemia, and generalized edema. Each episode can result in multiorgan failure due to systemic hypoperfusion. <b><i>Case Presentation:</i></b> We report a case of uveal effusion, mimicking uveal melanoma, associated with ISCLS following viral infection. A 74-year-old white male was evaluated in our ocular tumor clinic for a large intraocular mass in the right eye concerning for choroidal melanoma. We completed a review of the literature and list clinical recommendations for these cases. ISCLS, although rare, was a significant diagnostic consideration in this patient. Due to the high mortality rate of this condition, accurate diagnosis and prompt treatment was critical. We hypothesize that the mechanism of choroidal effusion development was due to reduced oncotic pressure from rapid decrease in serum albumin. Increased permeability of choroidal capillaries may be an additional mechanism leading to uveal effusion. <b><i>Conclusion:</i></b> With treatment, the patient had complete resolution of his choroidal effusion with no recurrence of his ISCLS. Further research should be considered on the role of viral infections in the pathogenesis of ISCLS.

Surgical Management of Choroidal Diseases

Accumulation of serous fluid in the suprachoroidal space, known as uveal effusion, and choroidal or suprachoroidal haemorrhage (SCH) following rupture of ciliary blood vessels are considered rare, but serious, events with extremely poor functional prognosis. As a result, uveal effusion, and expulsive suprachoroidal haemorrhage in particular, continue to be considered as more or less fatal complications. However, clinical experience demonstrates that both clinical entities can be managed by conservative as well as surgical strategies, depending on their severity and localisation, with sometimes surprisingly favourable visual outcome. In addition to prognostic factors, timely recognition and prompt, if possible preventive, acute care, as well as carefully considered timing of adequate surgical measures taking advantage of the specific characteristics of the choroidal tissue, are crucial to treatment success. Along with technical advances in the field of vitreoretinal surgery, numerous variants of therapeutic approaches to the treatment of choroidal effusion and suprachoroidal haemorrhage have been proposed to date. This review presents some of the most important surgical techniques and strategies in the field.

Acute Angle-Closure—A Rare but Vision-Threatening Ocular Adverse Effect of Selective Serotonin Reuptake Inhibitors

Acute angle closure (AAC) is a relatively rare but serious ophthalmological condition in which early diagnosis and intraocular pressure (IOP) -lowering treatment play a key role in prognosis. The aim of this review is to present current understanding of the pathophysiology, symptoms, diagnosis, potential mechanisms as well as management of AAC associated with the use of selective serotonin reuptake inhibitors (SSRIs). In most cases, AAC in individuals receiving SSRIs occurs in the pupillary block mechanism, secondary to SSRI-induced mydriasis. However, SSRIs may also cause uveal effusion and, consequently, iridocorneal angle closure. Other factors such as impaired metabolism and elimination of SSRIs, individual genetic conditions, as well as inhibition of SSRIs metabolism due to the effects of other drugs used may also promote AAC.

Multimodal imaging to aid in diagnosis of uveal effusion syndrome type 3

A 47-year-old man presented to our retina clinic with gradual onset diminution of vision in his right eye for the last 3 months. Anterior segment evaluation was normal in both eyes. Fundus evaluation showed the presence of leopard spot appearance in the right eye with inferior exudative retinal detachment. Multimodal imaging was done to evaluate retinal pigment epithelium function, choroidal thickness, choroidal vascularity and scleral thickness. Multimodal imaging showed increased choroidal thickness, choroidal congestion on indocyanine green angiography and peripheral choroidal detachment with normal sclera thickness in our patient, thus pointing towards a diagnosis of uveal effusion syndrome type 3.

Partial Thickness Sclerectomy for Uveal Effusion Syndrome in nanophthalmic eyes

Introduction: Nanophthalmos characterized by short axial length, high lens-to-eye ratio and thick sclera, is more prone to develop uveal effusion syndrome (UES). This rare entity can result in idiopathic exudative detachment of the choroid, ciliary body and retina. Abnormality in the scleral thickness with resultant obstruction of the vortex veins and reduced trans-scleral drainage of fluid is responsible for exudative retinal detachment (ERD). Methods: A retrospective study of UES in nanophthalmic patients treated with partial thickness sclerectomy in tertiary eye care centre from January 2015 to June 2019. Five eyes of five patients (four males and one female) with a diagnosis of nanophthalmos suffered from angle closure glaucoma associated with ERD. Raised intra-ocular pressure (IOP) not amenable to conservative medical management were subjected to surgery. Lamellar sclerectomy was performed in two or more quadrants without drainage which was judged on the basis of maximum amount of exudative fluid present in the subsequent quadrants. Results: The average age at surgery was 39.2 years and the mean follow-up duration was 9.2 months (6 to 18 months). Revision sclerectomy was required in 2/5 (60%) eyes. The serous fluid gradually resolved and retina remained reattached at the end of final follow up. The useful vision was preserved and IOP was normalized. Conclusion: Nanophthalmic UES remains a challenging clinical entity. Partial thickness sclerectomy may be an effective option in the treatment of nanophthalmic UES, not amenable to the conventional medical management in a low resource setup.

Prevalence of Complications in Eyes With Nanophthalmos or Microphthalmos: A Systematic Review and Meta-analysis

IntroductionMicrophthalmos and nanophthalmos are uncommon ocular conditions, whereby affected eyes have smaller dimensions compared to the normal population. Microphthalmos and nanophthalmos present several challenges to ophthalmologists; They have spontaneous and post-operative sequelae such as high hyperopia, angle-closure glaucoma, uveal effusion syndrome, and retinal detachment.This systematic review and meta-analysis intends to assess the prevalence of both the spontaneous complications associated with nanophthalmos and microphthalmos, as well as the post-surgical complications associated with nanophthalmos or microphthalmos. Methods and analysisArticles will be searched for, on four online databases: PubMed, EMBASE, Scopus and Web of Science. Two independent reviewers will identify the studies according to prespecified inclusion and exclusion criteria. Relevant data will be extracted and assessed for the risk of bias in each article. The data will then be pooled to determine the prevalence of complications among patients with microphthalmos and nanophthalmos. If any preventative measures are associated with lower risks of developing complications, these will be expressed as odds ratios.DiscussionAlthough nanophthalmos is an uncommon condition that affects the eye, its management and complications can be sight-threatening. Thus, it is important to counsel parents and patients correctly upon diagnosis and prior to any surgical intervention. This can only be done if the overall prevalence of complications is known.RegistrationThis systematic review has been submitted to PROSPERO for registration.