|Year : 2021 | Volume
| Issue : 1 | Page : 9
Scleral windows for exudative retinal detachment in nanophthalmos
Silvia Nerantzoulis da Cunha Diniz, Inês Figueiredo Matias, Pedro Prata Gomes, Pedro Pereira Neves, Mário Correa Ornelas
Department of Ophthalmology, Setúbal Hospital Center, Setúbal, Portugal
|Date of Submission||10-Nov-2020|
|Date of Decision||31-Dec-2020|
|Date of Acceptance||01-Jan-2021|
|Date of Web Publication||12-Mar-2021|
Dr. Silvia Nerantzoulis da Cunha Diniz
Quinta da Matela - Batudes, 2950-056 - Palmela, Setúbal
Source of Support: None, Conflict of Interest: None
Nanophthalmic eyes are associated with increased scleral thickness, decreased uveoscleral outflow, and increased incidence of uveal effusion syndrome, exudative retinal detachment, and angle closure glaucoma. Lamellar sclerectomies can enhance uveoscleral permeability. We report a case of nanophthalmos with exudative retinal detachment, submitted to anterior lamellar sclerectomies. A 50-year-old male patient presented with high hyperopia who complained of progressive visual loss in the right eye. The best-corrected visual acuity was 20/400 in both eyes; fundoscopy showed peripheral choroidal detachments in both eyes and retinal detachment in the right eye. Ocular ultrasound revealed thickened scleral walls and short axial lengths in both eyes. Fluorescein angiography confirmed an exudative retinal detachment in the right eye. Two inferior lamellar sclerectomies were performed in the right eye. Postoperative period was uneventful; subretinal fluid was progressively reabsorbed with retinal reattachment.
Keywords: Exudative detachment, nanophthalmos, sclerectomies, uveoscleral outflow
|How to cite this article:|
da Cunha Diniz SN, Matias IF, Gomes PP, Neves PP, Ornelas MC. Scleral windows for exudative retinal detachment in nanophthalmos. Pan Am J Ophthalmol 2021;3:9
|How to cite this URL:|
da Cunha Diniz SN, Matias IF, Gomes PP, Neves PP, Ornelas MC. Scleral windows for exudative retinal detachment in nanophthalmos. Pan Am J Ophthalmol [serial online] 2021 [cited 2021 Apr 21];3:9. Available from: https://www.thepajo.org/text.asp?2021/3/1/9/311126
| Introduction|| |
Nanophthalmos is a rare developmental eye disorder characterized by shortened axial length (20 mm or less, at least two standard deviations below the mean for age), high corneal curvature, shallow anterior chamber, high lens/eye volume ratio with narrow iridocorneal angle, high hyperopia (error ranging from +8.00 to +25.00), scleral thickening, and collagen fibrils derangement; the functionality and organization of the eye are usually preserved.
The majority of cases of nanophthalmos are sporadic, but autosomal dominant and recessive inheritance has been reported. This condition may present as an isolated disorder or associated with other malformations, such as colobomas and anterior-segment dysgenesis, or as part of a rare syndrome,, such as the “nanophthalmos, retinitis pigmentosa, foveoschisis and optic drusen syndrome,”, the “oculo-dento-digital syndrome,” the “autosomal dominant vitreoretinochoroidopathy with nanophthalmos syndrome,” and the “Kenny–Caffey syndrome.”
Nanophthalmic eyes also seem to present scleral inelasticity which contributes to the vortex venous system outflow blockage and sequestration of extracellular fluid, resulting in increased incidence of choroidal congestion, choroidal detachment, exudative retinal detachment, and angle closure glaucoma.
The performance of anterior lamellar sclerectomies, “scleral windows,” has been described in the treatment of exudative retinal detachment in nanophthalmic eyes.,,
The authors present a case of nanophthalmos with exudative retinal detachment, submitted to two inferior lamellar sclerectomies.
| Case Report|| |
A 50-year-old male patient with high hyperopia (+17.0 D sphere in both eyes) presented with progressive visual loss in the right eye (OD) for 1 year. His medical history included arterial hypertension. A positive first-degree family history of high hyperopia was present, including three siblings and his maternal grandmother.
On examination, the best-corrected visual acuity was 20/400 in both eyes (OU). The patient was phakic and slit lamp examination showed transparent media and shallow anterior chambers, which by gonioscopy the trabecular meshwork could be identified in OU; intraocular pressure (IOP) was 16 mmHg in the OD and 17 mmHg in the OS. Dilated fundus examination showed subretinal fluid in the OD [Figure 1].
|Figure 1: Right eye exudative retinal detachment and vascular tortuosity|
Click here to view
Posterior-segment optical coherence tomography (OCT) revealed cystoid macular edema (CME) in OU, with a cystoid foveal degeneration-like pattern associated with great disruption and atrophy of outer layers in the OS; OCT also showed a neurosensory retinal detachment in the OD [Figure 2] and [Figure 3].
|Figure 2: Posterior-segment optical coherence tomography image showing neurosensory retinal detachment and intraretinal fluid in the right eye|
Click here to view
|Figure 3: Posterior-segment optical coherence tomography image showing severe macular edema and ellipsoid zone disruption in the left eye|
Click here to view
B-scan ultrasonography showed thickened sclera in OU [Figure 4]. Ultrasound biometry results were as follows: right eye – axial length of 15.74 mm, K1 50.60 D and K2 51.25 D; and left eye – axial length 15.41 mm, K1 49.65 D and K2 51.48 D.
|Figure 4: Ocular ultrasonography image showing scleral thickening and peripheral choroidal detachments (a: right eye, b: left eye)|
Click here to view
Fluorescein angiography of the OD showed tortuous retinal vessels and areas of hyperfluorescence and leakage accentuated during the arteriovenous phase, corresponding to an exudative retinal detachment [Figure 5].
|Figure 5: Right eye fluorescein angiography showing areas of leakage in the posterior pole|
Click here to view
A diagnosis of nanophthalmos with uveal effusion syndrome and exudative retinal detachment in OD was made. The patient underwent scleral resection surgery with the creation of two “scleral windows” in the inferotemporal and inferonasal quadrants of the OD. First, a 360° conjunctival peritomy was performed. Then, rectus muscles were isolated on a muscle hook and 2-0 black silk traction sutures were placed around the muscles using a fenestrated muscle hook [Figure 6]. We proceeded with the creation of two 6 mm × 6 mm square-shaped anterior lamellar sclerectomies in the inferotemporal and inferonasal quadrants, distanced 4 mm from the corneoscleral limbus, using a scleral MVR knife (Alcon®) [Figure 7]. By using a crescent knife SatinCrescent™ (Alcon®), 2/3 thickness sclerectomies were dissected [Figure 8] and completely excised [Figure 9]. Finally, the conjunctiva was sutured with a 7-0 polyglactin suture.
The patient was medicated with dexamethasone eye drops 4 times a day for 4 weeks, ofloxacin eye drops 4 times a day for 1 week, gentamicin ointment at night for 1 week, and flurbiprofen eye drops 3 times a day for 4 weeks.
The postoperative period was uneventful; the intraretinal and subretinal fluid was progressively reabsorbed with retinal reattachment 5 weeks after surgery [Figure 10]. Although there were retinal reattachment and uveal effusion resolution, the patient had macular edema recurrence 10 weeks after surgery. Despite the anatomical improvement after 2 monthly loading doses of aflibercept 2 mg, best-corrected visual acuity remained 20/400 in OU, and the patient decided not to continue with intravitreal injections, being referred to a low vision clinic.
|Figure 10: Posterior-segment optical coherence tomography image showing attached retina without macular edema|
Click here to view
| Discussion|| |
Diagnosis criteria for nanophthalmos vary across the literature. High hyperopia in nanophthalmic eyes may cause bilateral amblyopia; the best-corrected visual acuity is rarely above 20/40 at any point in those patients' lives. Complications can worsen the patients' vision. Pronounced iris convexity and peripheral anterior synechiae formation can cause blockage of aqueous humor outflow and consequently angle closure glaucoma. Other complications include uveal effusion syndrome, retinal folds, exudative detachments, and CME.,, It is of extreme importance considering other causes of choroidal effusion and exudative retinal detachment as differential diagnosis, such as posterior scleritis and uveitis, uveal lymphoma, paraneoplastic syndromes, metastatic tumors of the choroid or retina, hypertensive choroidopathy, postoperative inflammation from cryotherapy, or photocoagulation and persistent hypotony.
Nanophthalmic eyes are often prone to serious complications during or after intraocular surgery, such as expulsive hemorrhage, persistent shallow anterior chamber, and aqueous misdirection syndrome. Managing choroidal effusion and exudative retinal detachment represent a clinical and surgical challenge. The performance of lamellar sclerectomies, or “scleral windows,” has been described in such cases to increase uveoscleral flow/drainage, by relaxing the scleral tension or reducing resistance to the outflow of large molecules.,,,,, Ozgonul et al. proposed a phased approach beginning with partial thickness sclerectomies; if a second surgery is necessary, flap revision using mitomycin C to inhibit further scarring can be considered; in refractory cases, small sclerostomy under the scleral flap or unroofing of the vortex veins may be considered., Kong et al. reported that single full-thickness sclerotomy without sclerostomy under the scleral flap can produce good results, simplifying the surgical procedure.
Despite the few studies, the use of anti-vascular endothelial growth factor therapy, oral carbonic anhydrase inhibitors, and local prostaglandin analogs has a certain value for patients who show unsatisfactory results after surgical approach or have severe scleral scarring after multiple surgeries.
In our case, despite the poor functional outcome, this surgical approach proved to be effective and safe in the resolution of the choroidal effusion and exudative retinal detachment in a nanophthalmic eye.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Sundin OH, Dharmaraj S, Bhutto IA, Hasegawa T, McLeod DS, Merges CA, et al
. Developmental basis of nanophthalmos: MFRP is required for both prenatal ocular growth and postnatal emmetropization. Ophthalmic Genet 2008;29:1-9.
Serrano JC, Hodgkins PR, Taylor DS, Gole GA, Kriss A. The nanophthalmic macula. British J Ophthalmol 1998;82:276-9.
Carricondo PC, Andrade T, Prasov L, Ayres BM, Moroi SE. Nanophthalmos: A review of the clinical spectrum and genetics. J Ophthalmol 2018;2018:2735465.
Ayala-Ramirez R, Graue-Wiechers F, Robredo V, Amato-Almanza M, Horta-Diez I, Zenteno JC. A new autosomal recessive syndrome consisting of posterior microphthalmos, retinitis pigmentosa, foveoschisis, and optic disc drusen is caused by a MFRP gene mutation. Mol Vis 2006;12:1483-9.
Zenteno JC, Buentello-Volante B, Ayala-Ramirez R, Villanueva-Mendoza C. Homozygosity mapping identifies the Crumbs homologue 1 (Crb1) gene as responsible for a recessive syndrome of retinitis pigmentosa and nanophthalmos. Am J Med Genet A 2011;155A: 1001-6.
Sugar HS, Thompson JP, Davis JD. The oculo-dento-digital dysplasia syndrome. Am J Ophthalmol 1966;61:1448-51.
Yardley J, Leroy BP, Hart-Holden N, Lafaut BA, Loeys B, Messiaen LM, et al
. Mutations of VMD2 splicing regulators cause nanophthalmos and autosomal dominant vitreoretinochoroidopathy (ADVIRC). Invest Ophthalmol Vis Sci 2004;45:3683-9.
Bergada I, Schiffrin A, Abu Srair H, Kaplan P, Dornan J, Goltzman D, et al.
Kenny syndrome: Description of additional abnormalities and molecular studies. Hum Genet 1988;80:39-42.
Venkatesh P, Chawla R, Tripathy K, Singh HI, Bypareddy R. Scleral resection in chronic central serous chorioretinopathy complicated by exudative retinal detachment. Eye Vis (Lond) 2016;3:23.
Krohn J, Seland JH. Exudative retinal detachment in nanophthalmos. Acta Ophthalmol Scand 1998;76:499-502.
Moradian S, Kanani A, Esfandiari H. Nanophthalmos. J Ophthalmic Vis Res 2011;6:145-6. [Full text]
Yang N, Jin S, Ma L, Liu J, Shan C, Zhao J. The pathogenesis and treatment of complications in nanophthalmos. J Ophthalmol 2020;2020:6578750.
Ozgonul C, Dedania VS, Cohen SR, Besirli CG. Scleral surgery for uveal effusion. Retina 2017;37:1977-83.
Kong M, Kim J. H, Kim S. J, Kang S. W. Full-thickness sclerotomy for uveal effusion syndrome. Korean Journal of Ophthalmology. 2013;27:294-8. doi: 10.3341/kjo.2013.27.4.294.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10]