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Figure 2. Statistical analysis of regional damage in the macular map. (a) The numbers in each grid point represent P values, and the colors indicate the statistical significance of the damage (red: higher, blue: lower). The two temporal grid points above the horizontal line in the superior hemifield and the arcuate region of 23 grids within 3–7 degrees in the inferior hemifield were the most significantly damaged. * indicates P < 0.001. (b) Regional vulnerability in the inferior arcuate area. The distal portion (temporal side, in red) of the arcuate damaged region was more vulnerable than the proximal portion (nasal side, in blue). * indicates P < 0.001. Taken together, each grid point included, on average, about 40% of the patients with mild or moderate NTG. Furthermore, the most vulnerable area in the macular map was the arcuate area in the temporal and inferior region (Fig. 2). These findings for regional distribution and abnormal grid point frequency in the mGCL + IPL are consistent with previous reports on vulnerable areas of the mRNFL in OAG patients,3 as well as with HFA findings on macular visual field damage in NTG.5 Thinning of the mGCL + IPL represents in situ damage caused by glaucoma, and thickness measurements are highly consistent with the results of visual field testing. We thus believe that this new information on mGCL + IPL regional damage should improve comparative investigations of visual field damage. Our finding that the distal area of the inferior arcuate region had a higher vulnerability to macular damage may be explained by the susceptibility to peripheral lesions of the lamina cribrosa. In conclusion, we found regional vulnerabilities to glaucomatous damage in OCT macular maps of NTG patients. This information may help improve the management of NTG, the major type of glaucoma in Asia.

REFERENCES 1. Yang Q, Reisman CA, Wang Z et al. Automated layer segmentation of macular OCT images using dual-scale gradient information. Opt Express 2010; 18: 21293–307. 2. Raza AS, Zhang X, De Moraes CG et al. Improving glaucoma detection using spatially correspondent clusters of damage and by combining standard automated perimetry and optical coherence tomography. Invest Ophthalmol Vis Sci 2014; 55: 612–24. 3. Hood DC, Raza AS, de Moraes CG, Liebmann JM, Ritch R. Glaucomatous damage of the macula. Prog Retin Eye Res 2013; 32: 1–21. 4. Lee EJ, Kim TW, Weinreb RN, Park KH, Kim SH, Kim DM. Trend-based analysis of retinal nerve fiber layer thickness measured by optical coherence tomography in eyes with localized nerve fiber layer defects. Invest Ophthalmol Vis Sci 2011; 52: 1138–44. 5. Araie M, Arai M, Koseki N, Suzuki Y. Influence of myopic refraction on visual field defects in normal tension and primary open angle glaucoma. Jpn J Ophthalmol 1995; 39: 60–4.

ACKNOWLEDGEMENTS The authors thank Mr. Tim Hilts for editing this manuscript and Dr. Masahiro Akiba for useful discussion.

Kazuko Omodaka MD,1 Naoko Takada MD,1 Hidetoshi Takahashi MD PhD1 and Toru Nakazawa MD PhD1,2,3 1 Departments of Ophthalmology, 2Retinal Disease Control, Ophthalmology and 3Advanced Ophthalmic Medicine, Tohoku University Graduate School of Medicine, Sendai, Japan Received 13 May 2014; accepted 18 May 2014.

Noonan syndrome with bilateral acute angle-closure Noonan syndrome (NS) is a common and heterogeneous developmental disorder characterized by facial dysmorphism, congenital heart defect, reduced growth, skeletal anomalies, short stature and other comorbidities. There are Competing/conflicts of interest: No stated conflict of interest. Funding sources: No stated funding sources.

© 2014 Royal Australian and New Zealand College of Ophthalmologists

Letters to the Editor Figure 1. (a) Gonioscopy of the OD showing appositional closure of the angle. (b) Optic nerves did not show signs of glaucoma damage. (c) UBM shows iridotrabecular apposition in OD. (d) Slit-lamp biomicroscopy photograph of the OD after phaco/IOL. Sectoral iris atrophy (arrows) is observed resulting from ischaemia of acute attack.

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(b)

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(d)

several gene mutations identified in NS. Nine genes (SOS1, PTPN11, NRAS, KRAS, RAF1, SHOC2, MEK1, BRAF and CBL) related with RAS/MAPK (Rat sarcoma/ mitogen-activated protein kinase) pathway have been identified.1 Clinically and genetically heterogeneous, NS is transmitted as an autosomal dominant trait (complete penetrance and variable expressivity); although as other dominant disorders, there is a significant percentage of cases because of new mutations.1 We report a case of NS with a bilateral attack of acute angle-closure (AAC) induced by pharmacologic mydriasis. A 33-year-old woman con NS documented clinically and genetically, mutation in the gene SOS1 (Met269Thr), came to the emergency room complaining of bilateral loss of vision, painful eyes associated with photophobia and haloes. In the previous 24 h, she had been explored under cycloplegic eye drops. Refraction had shown low hypermetropia. She referred previous several intermittent episodes of headache. Ophthalmological exploration revealed bestcorrected visual acuity (BCVA) of 6/12 in her right eye (OD, from latin oculus dexter) and 6/18 in her left eye (OS, from latin oculus sinister). Both eyes (OU, from latin oculi uterque) were injected with corneal epithelial oedema, shallow anterior chambers and fixed mid-dilated pupils. Intraocular pressure (IOP) was 58 mmHg in OD and 54 mmHg in OS. A diagnosis of bilateral AAC was made, and intravenous manitol, timolol, brimonidine, dorzolamide, pilocarpine and dexamethasone eye drops were administered. Two hours later, IOP was 24 mmHg in OU, and laser peripheral iridotomy (LPI) were performed successfully. One week later, IOP was 28 mmHg in OD and 22 mmHg in OS under maximum medication. Gonioscopy showed extensive peripheral anterior synechiae (PAS) in 270° in OD (Fig. 1a) and in 90° in OS. Optic nerves (Fig. 1b) and visual fields did not show glaucomatous damage. Ultra-

biomicroscopy (UBM) revealed narrow angles (Fig. 1c). Considering ocular findings and insufficient control of IOP in OD, a phacoemulsification with intraocular lens implantation (phaco/IOL) was planned. Finally, a trabeculectomy with mitomycin C was necessary in OD. Eight months after both surgeries, BCVA was 6/7.5 in OD and 6/6 in OS. IOP was 16 mmHg in OD without medication and 20 mmHg in OS with topical timolol 0.5% twice daily. Most common ophthalmic manifestations of NS reported in the literature correspond to external eye abnormalities (e.g. hypertelorism, epicanthal folds and ptosis). Other findings as prominent corneal nerves, refractive errors, amblyopia and strabismus have been commonly described.2 After a comprehensive literature search, there were no reports of association between NS and ocular hypertension or glaucoma. Our patient referred intermittent episodes of headache probably related with subacute angle-closure episodes. We presume that midryatics induced an attack of AAC. The clinical management was very similar to any case of AAC, lowering IOP with topical and systemic medications, and relieving the pupillary block performing LPI. Phaco/IOL performed within 1 week of medically controlled AAC does result in superior IOP control at 2 years compared with LPI, the standard treatment, with few complications.3 A satisfactory control of IOP after AAC depends on the extension of the PAS and damage of trabecular meshwork. LPI does not achieve a desired reduction of IOP in eyes with an episode of AAC and extensive PAS.4,5 Concerning phaco/IOL in AAC, Greve6 concluded that IOP decreased after cataract extraction even the presence of extensive PAS. The phaco-goniosynechialysis could be a treatment option to manage this case. Teekhasaenee et al.7 showed that this technique is effective in reducing PAS and IOP in

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eyes with persistent chronic angle-closure glaucoma within 6-month treatment for AAC. If PAS affect more than 270° filtering, surgery may be necessary to control IOP. In conclusion, few ocular manifestations have been described in NS. Based on literature search, this is the first case report of a NS with hypermetropia and an attack of AAC.

Noel Padrón-Pérez MD FEBO, Silvia Sanz-Moreno MD, Juan Lillo-Sopena MD and Jorge Arruga MD PhD Department of Ophthalmology, Hospital Universitari de Bellvitge, Barcelona, Spain Received 4 June 2014; accepted 10 June 2014.

REFERENCES 1. Roberts AE, Allanson JE, Tartaglia M, Gelb BD. Noonan syndrome. Lancet 2013; 381: 333–42. 2. Lee NB, Kelly L, Sharland M. Ocular manifestations of Noonan syndrome. Eye (Lond) 1992; 6 (Pt 3): 328–34. 3. Husain R, Gazzard G, Aung T, Chen Y, Padmanabhan V, Oen FTS. Initial management of acute primary angle closure. A randomized trial comparing phacoemulsification with laser peripheral iridotomy. Ophthalmology 2012; 119: 2274–81. 4. Nolan WP, Foster PJ, Devereux JG, Uranchimeg D, Johnson GJ, Baasanhu J. YAG laser iridotomy treatment for primary angle closure in east Asian eyes. Br J Ophthalmol 2000; 84: 1255–9. 5. Alsagoff Z, Aung T, Ang LPK, Chew PTK. Long-term clinical course of primary angle-closure glaucoma in an Asian population. Ophthalmology 2000; 107: 2300–4. 6. Greve EL. Primary angle closure glaucoma: extracapsular cataract extraction or filtering procedure? Int Ophthalmol 1988; 12: 157–62. 7. Teekhasaenee C, Ritch R. Combined phacoemulsification and goniosynechialysis for uncontrolled chronic angle-closure glaucoma after acute angleclosure glaucoma. Ophthalmology 1999; 106: 669–74.

Analysis of higher-order aberrations in eyes having undergone intraocular lens suturing When treating dislocated intraocular lenses (IOLs), incisions larger than 2.4 mm are commonly used to replace Competing/conflicts of interest: No stated conflict of interest. Funding sources: This paper was supported in part by a JST grant from JSPS KAKENHI Grants-in-Aid for Scientific Research (B) (T.N. 26293372), for Scientific Research (C) (H.K. 26462629), and for Exploratory Research (T.N. 26670751). This paper was partially presented at the Annual Meeting of the Japanese Society of Ophthalmic Surgeons, Fukuoka, 2013.

irrecoverable IOLs with new, sutured IOLs. However, a new, less invasive approach to this procedure, combining 25-gauge microincision vitrectomy surgery and haptic externalization, leads to good postoperative vision1–3 and requires only two 1-mm corneal incisions. Furthermore, as only micro-corneal incisions are made intraoperatively, it reduces postoperative astigmatism and prevents expulsive haemorrhages. However, postoperative visual quality after this procedure and the induction of highorder aberrations (HOAs) have not been reported.4,5 Here, we compared HOAs and coma-like aberrations in a sutured IOL group and a simple cataract surgery group. This retrospective analysis included 43 eyes. Eighteen eyes underwent phacoemulsification and intraocular lens in-the-bag implantation. Twenty-five eyes received sutured IOLs (Fig. 1) 14 with incisions larger than 2.4 mm (standard group) and 11 with 1-mm incisions (microincision group). Small 1-mm corneal incisions were used when the original IOL was sutured, as in earlier studies, and incisions larger than 2.4 mm were used when new IOLs were sutured. Patient characteristics including age, sex and preoperative best-corrected visual acuity were measured. A 1-month postoperative assessment included best-corrected visual acuity and corneal, internal and ocular cylinder power, as well as corneal, internal and ocular HOAs, assessed with the Hartmann–Shack wavefront analyzer (Wave-Front Analyzer KR-9000PW, TOPCON CORPORATION, Tokyo, Japan) and corneal, internal and ocular coma-like aberrations, assessed with a program for Zernike vector analysis. Mean age and pre- and postoperative best-corrected visual acuity were similar in the three groups (Table 1). Ocular HOAs, ocular coma-like aberrations, internal coma-like aberrations, ocular cylinder power and corneal cylinder power were significantly different in the phacoemulsification and intraocular lens in-the-bag implantation and microincision groups; but HOAs, comalike aberrations, and cylinder power were similar in the standard and microincision groups. Internal coma-like aberrations were significantly higher in the standard and microincision groups than the phacoemulsification and intraocular lens in-the-bag implantation group, although internal coma-like aberrations were similar in the standard and microincision groups. We thus found that internal coma-like aberrations were higher in sutured IOL eyes, regardless of incision length, than in standard cataract surgery eyes. This finding generally supports existing studies showing that IOL tilt in sutured IOLs is significantly correlated with increased ocular coma-like aberrations.5 The microincision method, which requires the dislocated IOL to be properly positioned, is a closed procedure and does not require IOL optic extraction. Thus, we anticipated postoperative IOL positional complications, which increase internal coma-like aberrations, but we observed no such differences between the standard and microincision groups. This retrospective study was limited by including only a small number of eyes (approximately 40), omitting an evaluation of tilt and decentering of the IOLs with Scheimpflug-based corneal

© 2014 Royal Australian and New Zealand College of Ophthalmologists

Noonan syndrome with bilateral acute angle-closure.

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