Nanophthalmic Eyes Research Articles

The Pathogenesis and Treatment of Complications in Nanophthalmos.

Microphthalmos is a type of developmental disorder ophthalmopathy, which can occur isolated or combined with other ocular malformations and can occur secondary to a systemic syndrome. Nanophthalmos is one of the clinical phenotypes of microphthalmos. Due to the special and complex structure of nanophthalmic eyes, the disorder is often associated with many complications, including high hyperopia, angle-closure glaucoma, and uveal effusion syndrome. The management of these complications is challenging, and conventional therapeutic methods are often ineffective in treating them. The purpose of this paper was to review the concept of nanophthalmos and present the latest progress in the study of the pathogenesis and treatment of its complications. As it is considerably challenging for ophthalmologists to prevent or treat these nanophthalmos complications, timely diagnosis and a suitable clinical treatment plan are vital to ensure that nanophthalmos patients are treated and managed effectively.

Extensive circumferential partial-thickness sclerectomy in eyes with extreme nanophthalmos and spontaneous uveal effusion

AimTo describe an extensive scleral excision technique to treat uveal effusion in nanophthalmic eyes.MethodsThis prospective, interventional series of eight eyes of five consecutive patients with nanophthalmos underwent scleral window surgeries....

Unmeasurable small size superficial and deep foveal avascular zone in nanophthalmos: the Collaborative Nanophthalmos OCTA Study

AimTo study the macular structure and vasculature in consecutive nanophthalmic eyes using optical coherence tomography angiography.MethodsThis is a prospective, multicentre, cross-sectional study of patients with nanophthalmos (one or both eyes)....

Successful recovery from misdirection syndrome in nanophthalmic eyes by performing an anterior vitrectomy through the anterior chamber

PurposeTo determine the effectiveness of iridectomy, capsulotomy and anterior vitrectomy through the anterior chamber to treat misdirection syndrome in pseudophakic nanophthalmic eyes.MethodsThis was a non-comparative study of seven nanophthalmic eyes from four consecutive patients. All eyes developed misdirection syndrome after successful cataract surgery. Treatment for misdirection syndrome involved capsulotomy and anterior vitrectomy through a peripheral iridectomy from the anterior chamber using a 25-gauge vitreous cutter. The best-corrected visual acuity, intraocular pressure and anterior and posterior segment findings were recorded before and after surgery.ResultsResolution of the aqueous misdirection was achieved in all but one eye. The single case of recurrence was observed after a mean follow-up of 45.6 ± 21.5 months and was caused by closure of the capsule hole by Elschnig’s pearls. This eye was successfully treated by enlargement of the lens capsule hole with a vitreous cutter. The mean intraocular pressure before surgery was 28.7 ± 4.4 mmHg, and this was significantly reduced to 13.7 ± 1.3 mmHg at the final visit. All but one patient, who had uveal effusion, maintained their best-corrected visual acuity.ConclusionIn this study, we investigated an alternative option for the treatment of misdirection syndrome in nanophthalmic eyes. We undertook a lens capsulotomy and anterior vitrectomy through a peripheral iridectomy from the anterior chamber using a 25-gauge vitreous cutter, which was able to create a communication hole between the anterior and posterior chambers.

A Randomized Controlled Trial Comparing Outcomes of Cataract Surgery in Nanophthalmos With and Without Prophylactic Sclerostomy

To prospectively evaluate visual outcomes and complications during and after cataract surgery with or without prophylactic sclerostomy in nanophthalmic eyes with visually significant cataract. Randomized controlled trial. Sixty nanophthalmic eyes of 60 patients with visually significant cataract were randomly assigned to cataract surgery alone (control group, n= 31) or cataract surgery with concomitant prophylactic sclerostomy (sclerostomy group, n= 29). Surgery was performed using phacoemulsification or manual small-incision cataract surgery (SICS) based on the LOCS III grading score. Group differences in intraoperative and postoperative complications were analyzed and risk factors assessed. Fewer complications were noted in eyes receiving sclerostomy (5/29, 17.2%) as compared to control group eyes (12/31, 38.7%), though differences were marginally significant (P= .065). Four control group, but no sclerostomy group, eyes developed postoperative uveal effusions (P= .04). In multivariable models, sclerostomy decreased the odds of an intraoperative or postoperative complication by 80% (odds ratio [OR]= 0.2, 95% confidence interval [CI]= 0.04-0.92, P= .039); SICS was associated with a significantly higher risk of complications as compared to phacoemulsification (OR= 5.95, 95% CI= 1.49-23.73, P= .012), while high preoperative intraocular pressure (OR= 4.54, 95% CI= 0.99-20.9, P= .052) and greater lens thickness (OR=3.38, 95% CI= 0.88-12.91, P= .075) demonstrated a marginally significant association. Cataract surgery in eyes with nanophthalmos is associated with a high risk for vision-threatening complications. Performing a simultaneous prophylactic sclerostomy with cataract surgery reduces complication rates, particularly uveal effusions. Cataract surgery at earlier stages by phacoemulsification may be more beneficial than undergoing manual SICS.

Refractive lens exchange and piggyback intraocular lens implantation in nanophthalmos: Visual and structural outcomes

To evaluate the best piggyback intraocular lens (IOL) implantation method and the outcomes in nanophthalmos eyes and to define the postoperative structural changes. Farabi Eye Hospital, Tehran, Iran. Prospective case series. Candidates for refractive surgery who were nanophthalmic were recruited. Patients had refractive lens exchange followed by implantation of both IOLs in the bag or 1 IOL in the bag and 1 IOL in the sulcus. The baseline and follow-up visual acuity, refractive status, and structural Scheimpflug imaging were evaluated. Ultrasound biomicroscopy (UBM) was performed 6months postoperatively. The study comprised 9 nanophthalmic patients (18 eyes) with a mean preoperative uncorrected distance visual acuity (UDVA) of 1.53 logarithm of the minimum angle of resolution (logMAR)±0.3 (SD), mean corrected distance visual acuity (CDVA) of 0.34±0.2 logMAR, and mean spherical equivalent (SE) of +13.55±4.0 diopters (D). The mean postoperative UDVA improved from baseline. There was no significant difference in the mean UDVA and CDVA between the 2 IOL groups. Postoperatively, both groups had a significant improvement in SE, a significant rise in anterior chamber depth and angle, and similar UBM measurements. Piggyback IOL implantation was an effective refractive procedure in nanophthalmic eyes. Both implantation methods resulted in similar outcomes; however, the small number of patients in each group made it less likely that possible differences would be found. The increase in angle values might help prevent the development of closed-angle glaucoma.

Effectiveness of scleral decompression procedures for preventing choroidal effusion in nanophthalmic eyes: A retrospective review

Purpose: The purpose of this study is to study role of prophylactic scleral decompression procedures in the prevention of choroidal effusion in nanophthalmos. Materials and Methods: Nanophthalmos was defined as eye with axial length (AL) ( +5.00 D) and scleral thickness (>1.7 mm). In a retrospective case series, we analyzed 21 eyes of 21 patients with nanophthalmos, who underwent cataract and/or glaucoma surgery with concurrent sclerotomy or sclerotomy with sclerectomy. Results: The mean age at the time of surgery was 51 ± 7 years. The mean AL was 17.18 ± 1.74 mm. Surgery-related complications were documented in 8 (38%) eyes. The prophylactic scleral decompression procedures did not offer protection against postoperative choroidal effusion (P = 0.6). On univariate logistic regression, preoperative intraocular pressure (P = 0.01) was significantly associated with postoperative complications. Conclusion: Prophylactic scleral decompression does not offer absolute protection against postoperative complications during cataract and/or glaucoma surgery in nanophthalmic eyes.

Analysis of the Optical Quality of Spherical and Aspheric Intraocular Lenses in Simulated Nanophthalmic Eyes.

To analyze the optical performance of different implant strategies in simulated nanophthalmic eyes. An optical design software was used. Analysis included eye models that required 30.00, 45.00, and 60.00 diopters (D) intraocular lenses (IOLs) to achieve emmetropia. Spherical and aspheric IOLs were designed. They were tested either with a single implant setting S or by splitting the power into two lenses. Setting P1 had an even split of the power between the lenses and setting P2 had an uneven power split with one-third of the power in the anterior lens and two-thirds in the posterior IOL. The area under the modulation transfer function (MTF) curve was calculated and spherical aberration was recorded in each setting. Setting S had the worst optical performance in the spherical group and the best performance in the aspheric group. A statistically significant difference was found between setting S and the piggyback options (settings P1 and P2) in all analyzed variables for the spherical and aspheric groups for the 45.00 and 60.00 D IOL requirement. No statistically significant difference was found between the piggyback settings. Single aspheric IOLs had better optical performance than piggybacking lower-power aspheric IOLs. In the spherical lenses group, the results were the opposite, with the piggyback options having higher optical quality than the single IOL. MTF shows that single aspheric lenses provide the highest contrast sensitivity among all of the analyzed settings.

Refractive outcomes in nanophthalmic eyes after phacoemulsification and implantation of a high-refractive-power foldable intraocular lens

To evaluate the refractive and postoperative outcomes of a high-power foldable intraocular lens (IOL) in nanophthalmic eyes. Six ophthalmic surgical centers in Canada. Retrospective case series. Consecutive charts of nanophthalmic patients having cataract extraction and insertion of the CT Xtreme D IOL were reviewed. Demographic and clinical data were collected, including age, sex, axial length (AL), minimum keratometry (K) value and maximum K value, corneal white-to-white (WTW), anterior chamber depth, lens thickness (LT), and complications. The following preoperative and operative data were collected: uncorrected distant visual acuity (UDVA), corrected distant visual acuity (CDVA), sphere, cylinder, and spherical equivalence (SE). The primary outcome measure was change in SE. The secondary outcome measures were changes in UDVA and CDVA. A total of 21 eyes from 13 patients with a mean follow-up time of 9.6 ± 8.5 months were studied. Mean preoperative data were: age (51.4 ± 15.2 years), AL (16.63 ± 0.68 mm), minimum K value (46.20 ± 2.26 D), maximum K value (47.55 ± 2.34 D), anterior chamber depth (2.60 ± 0.49 mm), WTW (11.08 ± 1.38 mm), LT (4.70 ± 0.97 mm), and IOL power implanted (+49.9 ± 3.3 diopters [D]). SE improved from +16.11 ± 3.26 D preoperatively to +2.00 ± 2.37 D postoperatively (P < .0001). UDVA improved from 1.47 ± 0.30 logMAR preoperatively to 0.74 ± 0.43 logMAR postoperatively (P = .016). CDVA did not change significantly. Five eyes (23.8%) had serious postoperative complications. Of these eyes, 2 had malignant glaucoma, 2 had vitreous hemorrhages, and 1 eye had a vitreous hemorrhage with retinal detachment resulting in visual acuity of no light perception. Implanting foldable high-power IOLs in a series of nanophthalmic eyes yielded significant improvement in UDVA and SE. Cataract surgery in these eyes carries increased risk. Iqbal Ike K. Ahmed is a consultant to Carl Zeiss Meditec AG. No other author has a financial or proprietary interest in any material or method mentioned.

RETINAL AND CHOROIDAL CHANGES OF NANOPHTHALMIC EYES WITH AND WITHOUT SECONDARY GLAUCOMA.

To analyze the retinal and choroidal changes and their relationship in nanophthalmic eyes quantitatively and to evaluate the influence of axial length (AL) and secondary glaucoma on the retina and choroid in these eyes. Thirty-seven eyes of 19 nanophthalmic patients (23 eyes with secondary glaucoma, SG group; 14 eyes without secondary glaucoma, NG group) and 38 eyes of 38 age/sex-matched controls were recruited. The retinal and choroidal changes, the foveal retinal thickness (FRT), and the subfoveal choroidal thickness (SFCT) were measured by enhanced depth imaging optical coherence tomography (EDI-OCT, Heidelberg). The parameters were compared between nanophthalmic eyes and controls, as well as between the NG and SG group. The relationship between AL and FRT, AL and SFCT, and FRT and SFCT were analyzed. The optical coherence tomography images revealed broadened outer nuclear layer, and aberrant preservation of the inner retinal layer to different extents existed in the fovea in all nanophthalmic cases. The ganglion cell layer was found much thinner in the SG group than that of the NG group. The low-reflective areas near the outer border of the choroid in nanophthalmic eyes seemed larger than that of controls. The average FRT and SFCT of the total nanophthalmic cases were significantly thicker than those of controls (P < 0.001, P < 0.001, respectively). The FRT of the SG group was thinner than that of the NG group (P < 0.001). No significant difference was found between the SG group and NG group in SFCT (P = 0.83). Axial length was negatively related to FRT and SFCT (P < 0.001, P < 0.001, respectively). The foveal retinal thickness and SFCT were positively related (P = 0.02). Retina and choroid are thickened accordantly in nanophthalmic eyes and are negatively related to AL. Secondary glaucoma could affect the FRT but would do nothing to the SFCT. The combined measurement of retina and choroid by enhanced depth imaging optical coherence tomography provides valuable information to facilitate comprehensive understanding of the pathology of nanophthalmos and its complications in vivo.

Cataract surgery in patients with nanophthalmos: results and complications.

To evaluate phacoemulsification surgery results and complications in patients with nanophthalmos. The records of 9 patients with nanophthalmos who had cataract surgery from January 2011 through January 2015 were retrospectively reviewed. Nanophthalmos was diagnosed by the presence of an eye with an axial length (AL) less than 20.5 mm. The records were reviewed for ocular diagnosis, keratometry, anterior chamber depth (ACD), AL, ocular surgeries, visual acuity, and complications. A total of 14 eyes of 9 patients (8 women, 1 man) with a mean age of 72 years (range 48-86) were reviewed. Mean AL was 18.72 mm and mean ACD was 2.30 mm. Mean preoperative spherical equivalent (+8.55 ± 6.44 diopters (D)) was reduced to +0.30 ± 2.17 D at last follow-up visit. Mean best-corrected visual acuity (BCVA) was +0.68 ± 0.55 logarithm of the minimum angle of resolution (logMAR) preoperatively and improved to +0.55 ± 0.73 logMAR at last follow-up visit. Visual acuity remained stable or improved in 11 eyes. Five eyes did not achieve final BCVA ≥+0.3 logMAR. Complications occurred in 3 eyes (21.4%) and included 1 case of posterior capsule rupture, 1 case of severe iritis and rhegmatogenous retinal detachment 2 weeks postoperatively, and 1 other case of iritis. No postoperative uveal effusion was seen. Although phacoemulsification seems to be relatively safe in nanophthalmic eyes, surgeons need to be aware of the challenges of this procedure in these high-risk eyes. Nevertheless, with careful preoperative evaluation and planning, most cases were uncomplicated and resulted in improvement of BCVA.

Optical coherence tomography of bilateral nanophthalmos with macular folds and high hyperopia.

Importance. There is a conflict about the content of the macular folds in nanophthalmic eyes in the literature. Our study clearly demonstrated that papillomacular folds seen in nanophthalmos or posterior microphthalmos were only composed of neurosensory retina without involvement of retinal pigment epithelium and choroid. Observations. This is a report of two consecutive nanophthalmic patients with macular folds at Maltepe University School of Medicine, Department of Ophthalmology, from January to June 2012. Anterior segment dimensions were near normal. The axial lengths of the eyes were short with markedly shortened posterior segment. A macular fold extending from the center of the fovea towards the optic nerve head was present in all eyes. Optic coherence tomography clearly demonstrated that folds were only composed of neurosensory retina. Binocular visual acuities and refractive errors of the cases were 0.3, 0.2 and +16.00, +15.75 diopters, respectively. Conclusions and Relevance. Our study proposes a surgical option to treat these folds like serous retinal detachments by showing the true content of the folds, although there is not any surgical operation accepted for this condition yet. Further studies dealing with the surgical interventions of these folds should be performed to support this option.

Subfoveal choroidal thickness measurements with enhanced depth imaging optical coherence tomography in patients with nanophthalmos

PurposeTo compare subfoveal choroidal thickness (SFCT) between patients with nanophthalmos and age-matched controls.MethodsThis prospective, cross-sectional and comparative study included 31 eyes from 31 patients with nanophthalmos (study group) and 31...

Small eyes big problems: is cataract surgery the best option for the nanophthalmic eyes?

Nanophthalmos refers to an eyeball of short axial length, usually less than 20 mm which leads to angle closure glaucoma due to relatively large lens. Intra-ocular lens extraction relieves the angle closure in nanophthalmos. Cataract surgery in a nanophthalmic eye is technically difficult with high risk of complications such as posterior capsular rupture, uveal effusion, choroidal haemorrhage, vitreous haemorrhage, malignant glaucoma, retinal detachment and aqueous misdirection. Various options are explained in the literature to perform cataract surgery in nanophthalmos, like extracapsular cataract extraction with or without sclerostomy; small-incision cataract extraction by phacoemulsification which not only helps maintain the anterior chamber during surgery but also reduces the incidence of complications due to less fluctuation of intraocular pressure (IOP) during the surgery. Cataract surgery deepens and widens the anterior chamber angle in nanophthalmic eyes and has beneficial effects on IOP in eyes with nanophthalmos but is associated with a high incidence of complications.

A Novel Technique for Choroidal Fluid Drainage in Uveal Effusion Syndrome

Uveal effusion syndrome (UES) is a rare disorder associated with thickening of the choroid and secondary retinal detachment as a possible serious complication. UES occurs in hypermetropic or nanophthalmic eyes or can be idiopathic and is a diagnosis of exclusion. Vortex vein decompression, sclerectomy or sclerotomy, and drainage of choroidal fluid are surgical treatment options. The authors describe a novel surgical technique for drainage of serous choroidal detachment using a penetrating diathermy probe in two patients with UES. The procedure involves drainage of choroidal fluid by using a penetrating diathermy probe (2 mm tip length) after placing a 20-gauge infusion line in the anterior chamber. Choroidal fluid drainage was successful in both patients, with satisfactory outcome on follow-up. The proposed simple and feasible surgical drainage technique may improve the management of patients with UES.

Resolution of Serous Retinal Detachment Following Partial Sclerectomy With Mitomycin C in Nanophthalmos

The authors report the results of partial-thickness sclerectomy combined with mitomycin C in a nanophthalmic eye with serous retinal detachment. Partial sclerectomy in two quadrants with adjuvant use of mitomycin C without drainage of subretinal fluid was performed. The patient was monitored with serial fundus photographs and spectral-domain optical coherence tomography (SD-OCT) for up to 16 weeks postoperatively. Best corrected visual acuity improved from counting fingers at 1 meter preoperatively to 6/60 at final follow-up. There was gradual but complete resolution of serous retinal detachment both clinically and on SD-OCT. Partial-thickness sclerectomy with mitomycin C is an effective and safe procedure in the management of nanophthalmic eyes with uveal effusion.

Assessment of the Anterior Chamber Angle in Patients with Nanophthalmos: An Anterior Segment Optical Coherence Tomography Study

ABSTRACTPurpose: To quantitatively assess the parameters of anterior chamber and angle dimensions by anterior segment optical coherence tomography (AS-OCT) in nanophthalmos.Methods: In this prospective, cross-sectional and comparative study, 34 eyes of 34 healthy individuals (control group) and 37 eyes of 37 patients with nanophthalmos (study group) were enrolled. A single experienced ophthalmologist imaged and measured the right eye with the Visante AS-OCT. The anterior chamber angle (ACA), angle opening distance (AOD) at 500 and 750 µm from the scleral spur, and trabecular–iris space area (TISA) at 500 and 750 µm at the nasal and temporal angles were measured.Results: Mean nasal/temporal ACA was 44.90 ± 9.09/47.07 ± 8.69° in the control group and 32.15 ± 10.30/29.77 ± 10.57° in the study group (p < 0.001). Mean nasal/temporal AOD500 and AOD750 was 0.52 ± 0.18/0.53 ± 0.15 mm and 0.71 ± 0.26/0.71 ± 0.23 mm in the control group. The mean nasal/temporal AOD500 and AOD750 were 0.33 ± 0.12/0.31 ± 0.15 mm and 0.48 ± 0.16/0.47 ± 0.22 mm in the study group. Mean nasal/temporal TISA500 and TISA750 were 0.16 ± 0.06/0.17 ± 0.05 mm2 and 0.31 ± 0.11/0.33 ± 0.09 mm2 in the control group. Mean nasal/temporal TISA500 and TISA750 were 0.11 ± 0.04/0.09 ± 0.05 mm2 and 0.20 ± 0.07/0.20 ± 0.10 mm2 in the study group. All values were significantly lower in the study group compared with the control group.Conclusion: Determination of angle parameters using AS-OCT is a rapid noncontact method that is useful in patients with nanophthalmos. The findings demonstrated that nanophthalmic eyes had lower ACA parameters including ACA, AOD500, AOD750, TISA500 and TISA750 at the nasal and temporal angles.

Phakoemulsifikation bei nanophthalmischen Augen

The aim of this study was to determine the prevalence of nanophthalmos among cataract patients and to evaluate intraoperative and postoperative complications in nanophthalmic eyes. Medical and operating records of all 3287 patients who had undergone clear cornea phacoemulsification cataract surgery with foldable acrylic IOL implantation during a period of two years (January 2009 to December 2010) were retrospectively reviewed. Nanophthalmos was diagnosed according to a shorter axial length than 20 mm, a shallow anterior chamber, hyperopia and scleral thickness greater than 1.7 mm as determined by echography. Before surgery all patients received an intravenous 20 % mannitol solution. From 3287 eyes, six eyes of four patients were identified as nanophthalmic with an axial length below 20 mm and a scleral thickness greater than 1.7 mm. The prevalence of nanophthalmos was 0.18 %. The average hyperopia was + 7.87 D (+ 6.0 to + 9.50). The mean axial length was 19.58 mm (18.94 to 20.00). The mean biometry calculation was 33.16 D (31.5 to 36.0). In all cases no complications were observed during the surgery. In two eyes a mild iritis was observed one day after surgery. During a follow-up of 3 months no further complications occurred. Surgical manipulation in a narrow and crowded anterior chamber in nanophthalmic eyes with increased vitreous pressure is always a challenge and must be performed by an experienced surgeon. Preoperative examination of scleral thickness and preoperative administration of hyper-osmotic solution for reduction of vitreous pressure are very important to prevent unexpected complications. There was no need for a prophylactic surgical procedure in our patients.

Angle-closure glaucoma in a patient with the nanophthalmos-ocular cystinosis-foveoschisis-pigmentary retinal dystrophy complex

BackgroundTo report clinical features of bilateral angle-closure glaucoma in a patient with nanophthalmic eyes associated with ocular cystinosis, foveoschisis and pigmentary retinal dystrophy. This is probably the first published report of the possible association of all these five entities in the same patient.Case presentationA 50-year-old white male was referred for uncontrolled glaucoma in both eyes. He was previously diagnosed with angle-closure glaucoma in association with ocular cystinosis. Ocular examination revealed high hyperopia (+13.5 OD and +14 OS diopters) with reduced axial length (16.27 mm OD and 15.93 mm OS). Despite being on 3 topical medications, his IOP measured 37 mmHg OD and 35 mm Hg OS. Slit-lamp biomicroscopy showed refractile, polychromatic crystalline deposits throughout the cornea and conjunctiva in both eyes. Gonioscopy revealed an extremely narrow angle with peripheral anterior synechiae (PAS). Anterior chamber depths were shallow. Fundus examination disclosed punctate hypopigmentation of the retinal pigment epithelium mainly at the posterior pole. Optical coherence tomography showed foveal schisis appearing as small retinal cysts. The patient did not display any systemic abnormalities.ConclusionsThis case brings into discussion a new clinical entity of angle closure glaucoma in nanophthalmos accompanied by ocular cystinosis-foveoschisis-pigmentary retinal dystrophy complex.

Nanophthalmos: quantitative analysis of anterior chamber angle configuration before and after cataract surgery

Background/aimsTo analyse quantitatively the anterior segment configuration in eyes with nanophthalmos before and after cataract surgery.MethodsThis was a retrospective, non-comparative, interventional case series. Eleven eyes in eight patients with nanophthalmos...