- Email: [email protected]
Plateau iris in children∗
Volume 19 Number 4 / August 2015 7. Kolb BA, Buller RE, Connor JP, DiSaia PJ, Berman ML. Effects of early postoperative chemotherapy on wound healing. Obstet Gynecol 1992;79:988-92. 8. Kim JH, Chung HK, Kim NJ, Lee MJ, Khwarg SI. The effect of autologous serum eye drops on the conjunctivalization over exposed porous polyethylene orbital implant in the rabbit model. Orbit 2011;30:83-7. 9. Ang LP, Tan DT, Phan TT, Li J, Beuerman R, Lavker RM. The in vitro and in vivo proliferative capacity of serum-free cultivated human conjunctival epithelial cells. Curr Eye Res 2004;28:307-17. 10. Matsuo H, Tomidokoro A, Tomita G, Araie M. Topical application of autologous serum for the treatment of late-onset aqueous oozing or point-leak through filtering bleb. Eye 2005;19:23-8.
Belovay et al
377
Plateau iris is more commonly observed in the young female adult population between 30-50 years of age, much older than the youngest previously reported patient, a 12year-old boy.3 Other children and adolescents have been reported in the literature with angle closure, but none with a definitive diagnosis of plateau iris.4-6 We report 5 cases of plateau iris in children who presented to either the Hospital for Sick Children, Toronto (HSC), Canada, or Centre Hospitalier Universitaire Sainte-Justine (CHUSJ), Montreal, Canada.
Case 1
Plateau iris in children* Graham W. Belovay, MD, FRCSC,a Talal Alabduljalil, MD, FRCSC,a Charles J. Pavlin, MD, FRCSC,a,b,c Patrick Hamel, MD, FRCSC,d,e and Asim Ali, MD, FRCSCa,f Narrow iridocorneal angles, a very rare condition in the pediatric population, can lead to visual loss through angle closure glaucoma. In the workup for patients with narrow iridocorneal angles, plateau iris must be considered in the differential diagnosis. We describe 5 children with plateau iris, the youngest 5 years of age. All were confirmed using ultrasound biomicroscopy and were offered iridotomy for treatment.
A 5-year-old, white healthy girl was referred to the HSC for narrow angles. Uncorrected visual acuity was 20/25 in the right and 20/30 in the left eye. There was no family history of ocular disease. Intraocular pressures measured by rebound tonometry (Icare-Finland,Vantaa) predilation were 16 mm Hg in the right eye and 20 mm Hg in the left eye, increasing slightly after dilation to 21 mm Hg in the right eye and 25 mm Hg in the left eye. Cycloplegic refraction showed mild hyperopia in both eyes. Gonioscopy was Shaffer grade 2, with a narrow peripheral angle, flat profile, and no peripheral anterior synechiae (PAS) in each eye. The cup:disk ratio was 0.2 in each eye. Ultrasound biomicroscopy (UBM) performed under general anesthesia confirmed PIC in each eye (Figure 1). Options for treatment were discussed, and the patient underwent a bilateral laser peripheral iridotomy (LPI) under general anesthesia with no change in the angles.
P
Case 2
Author affiliations: aDepartment of Ophthalmology and Vision Sciences, University of Toronto, Canada; bMount Sinai Hospital, Toronto, Canada; cPrincess Margaret Hospital, Toronto, Canada; dCentre Hospitalier Universitaire Sainte-Justine, Montreal, Canada; e Universite de Montreal, Montreal Canada; fThe Hospital for Sick Children, Toronto, Canada Presented in part at the Annual Meeting of the Canadian Ophthalmological Society, Montreal, June 15-17, 2013. * This publication is dedicated to our late colleague, coauthor, and pioneer in ophthalmic ultrasound, Dr. Charles J Pavlin. Submitted October 13, 2014. Revision accepted February 27, 2015. Published online July 31, 2015. Correspondence: Dr. Asim Ali, MD, FRCSC, The Hospital for Sick Children, 555 University Avenue, Toronto, Canada ON M5G 1X8 (email: [email protected]). J AAPOS 2015;19:377-379. Copyright Ó 2015 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2015.02.017
A 13-year-old white girl initially presented to the HSC with papillophlebitis in the left eye and a left relative afferent pupillary defect. Her presenting visual acuity in each eye was 20/20; IOP was 22 mm Hg in each eye. Her cup:disk ratio was 0.2 in the right eye. Her papillophlebitis was confirmed with an intravenous fluorescein angiogram and gradually improved without treatment. All investigations to rule out a causative etiology for her papillophlebitis were negative. At 1 month’s follow-up she presented with an IOP of 18 mm Hg in the right eye and 28 mm Hg in the left eye by Goldmann applanation tonometry, with no change in visual acuity. Gonioscopy showed plateau configuration of the iris in both eyes with the typical double hump sign. The right eye was Shaffer grade 1-2, with no PAS. In the left eye there was steep iris configuration, with PAS nasally and Shaffer grade 0-1. She was started on dorzolamide hydrochloride 2%–timolol maleate 0.5% in the left eye. An UBM assessment confirmed bilateral PIC. She did not undergo the offered intervention of LPI and was lost to follow-up for several years, returning with transient blurry vision with associated headache. Her examination was unchanged, with normal Humphrey 24-2 visual field testing.
lateau iris configuration (PIC) has been shown by ultrasound biomicroscopy to be caused by an anterior positioning of the ciliary processes behind the iris, preventing the peripheral angle from opening after iridotomy.1 PIC is typically diagnosed clinically by gonioscopy showing a persistently narrow angle following iridotomy. Plateau iris syndrome (PIS) is a subgroup of PIC with a patent iridotomy and increased intraocular pressure (IOP) on dilation.2
Journal of AAPOS
378
Belovay et al
Volume 19 Number 4 / August 2015
Cases 4 and 5
FIG 1. Ultrasound biomicroscopy images of case 1 showing the forward positioning of the ciliary processes (CP) supporting the peripheral iris and absence of ciliary sulcus (A). After laser peripheral iridotomy (B) the iris profile flattens, but the angles remain narrow. The arrow represents the location of the scleral spur.
Case 3 A 7-year-old white boy was referred from a local optometrist to the CHUSJ for high IOP in each eye. His presenting visual acuity was 20/70 in the right eye and 20/ 100 in the left eye, with an IOP of 31 mm Hg (right eye) and 32 mm Hg (left eye) by Tono-Pen applanation tonometry (Reichert Technologies, Depew, New York). Gonioscopy showed 90 of inferior angle closure and Shaffer grade 1 elsewhere in each eye. UBM showed PIC in each eye. The patient’s mother had a history of narrow-angle glaucoma treated from age 25 years. The patient underwent bilateral LPI under general anesthesia, with no change in angles. Afterward the pressure remained elevated, at 25 mm Hg in the right eye and 27 mm Hg in the left eye; he was therefore started on timolol maleate 0.25%. His cup:disk ratio at the time was 0.4 in the right eye and 0.65 in the left eye. Cycloplegic refraction showed him to be hyperopic (13.50 10.50 70, 14.00 10.75 10) and glasses were prescribed. His IOPs gradually increased over the year and he was switched to brimonidine tartrate 0.2%–timolol maleate 0.5% in both eyes. Five years after presentation his visual acuity was 20/40 in both eyes, with a cup:disk ratio of 0.6 in the right eye and 0.8 in the left eye. The diffuse depression (in each eye) and an inferior nasal step (left eye) on Humphrey 24-2 visual field testing has remained stable since diagnosis. He was also diagnosed with X-linked juvenile retinoschisis several years after initial presentation. Due to the chronic angle closure circumstances of his presentation it was not possible to determine whether he had PIS.
Twin 5-year-old white boys were referred to the CHUSJ for assessment of narrow angles. They were born at 34 weeks’ gestation and their maternal grandfather had glaucoma with low vision at age 45. Case 4 had presenting visual acuity of 20/20 in both eyes, with IOP by Tono-Pen of 20 mm Hg in the right eye and 19 mm Hg in the left eye. Cycloplegic refraction showed mild hyperopia in each eye. The cup:disk ratio was 0.2 in each eye. Gonioscopy in each eye showed Shaffer grade 2 angles, with the ciliary body only visible in the superior and inferior quadrants on indentation. UBM confirmed PIC. The patient underwent bilateral LPI under general anesthesia with follow-up IOP by Goldmann applanation tonometry of 14 mm Hg in the right eye and 21 mm Hg in the left eye and no change in angles. Visual acuity and optic disks have since remained unchanged. Case 5 had visual acuity of 20/25 in the right eye and 20/ 30 in the left eye, with presenting IOP of 18 mm Hg in the right eye and 17 mm Hg in the left eye by Tono-Pen. His cup:disk ratio was 0.2 in each eye. Gonioscopy was the same as his twin. UBM showed PIC. He also underwent bilateral PI under general anesthesia, with follow-up IOP of 13 mm Hg in the right eye and 15 mm Hg in the left eye by Tono-Pen and no change in angles. His follow-up cycloplegic refraction showed mild hyperopia in each eye. His most recent visual acuity was 20/20 in both eyes, with no optic disk changes.
Discussion The assessment of narrow iridocorneal angles in children can be challenging. Many will require an examination under anesthesia to perform gonioscopy and confirmatory imaging. The various etiologies reported in this population include iridociliary cysts,7,8 primary angle closure, retinopathy of prematurity,7 nanophthalmos,7 plateau iris, microspherophakia, iris tumors, and anterior segment dysgenesis. As pediatric patients are not routinely screened for narrow irdocorneal angles until later in life, glaucoma could develop insidiously. The prevalence of plateau iris in the pediatric population is unknown. Only single patient case reports exist; population-based studies have examined adults exclusively.9 As performing reliable gonioscopy can be difficult in children, UBM should be considered the imaging modality of choice to make a definitive diagnosis. In one study, the prevalence of PIC in nonglaucomatous eyes of adults was between 20.9% and 25.2%, but the long-term conversion rate to glaucoma is unknown.9 The initial recommended treatment for plateau iris is LPI to eliminate the pupil block, followed by pilocarpine, argon laser iridoplasty, or clear lens removal as needed.10 We empirically recommend that children with PIC should be followed every 3 months to ensure that they do not develop glaucoma.
Journal of AAPOS
Volume 19 Number 4 / August 2015 References 1. Pavlin CJ, Ritch R, Foster FS. Ultrasound biomicroscopy in plateau iris syndrome. Am J Ophthalmol 1992;113:390-95. 2. Cheng J, Buys YM, Spaeth GL. Confusion with the misuse of plateau iris terminology. J Glaucoma 2013;22:265-6. 3. Llinas A, Dorairaj S, Liebmann JM, Ritch R. Plateau iris syndrome in a child. Eye 2008;22:597-8. 4. Fivgas GD, Beck AD. Angle-closure glaucoma in a 10-year-old girl. Am J Ophthalmol 1997;124:251-3. 5. Appleby RS Jr, Kinder RS. Bilateral angle-closure glaucoma in a 14year-old boy. Arch Ophthalmol 1971;86:449-50. 6. Jones DE, Watson DM. Angle-closure glaucoma precipitated by the use of phospholine iodide for esotropia in a child. Br J Ophthalmol 1967;51:783-5. 7. Ritch R, Chang BM, Liebmann JM. Angle closure in younger patients. Ophthalmology 2003;110:1880-89. 8. Shields JA, Shields CL, Lois N, Mercado G. Iris cysts in children: classification, incidence, and management. The 1998 Torrence A Makley Jr Lecture. Br J Ophthalmol 1999;83:334-8. 9. Li Y, Wang YE, Huang G, et al. Prevalence and characteristics of plateau iris configuration among American Caucasian, American Chinese and mainland Chinese subjects. Br J Ophthalmol 2014;98:474-8. 10. Ng WT, Morgan W. Mechanisms and treatment of primary angle closure: a review. Clin Experiment Ophthalmol 2012;40:e218-28.
Evolution of focal choroidal excavation underlying combined hamartoma of the retina and retinal pigment epithelium in a child Meera D. Sivalingam, BS, Emil Anthony T. Say, MD, and Carol L. Shields, MD Combined hamartoma of the retina and retinal pigment epithelium (RPE) is a benign tumor seen mostly in children. Enhanced-depth imaging optical coherence tomography (OCT) of these tumors often shows an epiretinal membrane, tangential traction, disorganization of the retinal layers, and underlying uniform choroidal thinning. We describe the evolution over 9 years of focal choroidal excavation, a novel finding on OCT character-
Author affiliations: Ocular Oncology Service, Wills Eye Institute, Thomas Jefferson University, Philadelphia, Pennsylvania Support provided by Eye Tumor Research Foundation, Philadelphia, PA (CLS). The funders had no role in the design and conduct of the study, in the collection, analysis and interpretation of the data, and in the preparation, review or approval of the manuscript. Carol L. Shields, MD, has had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis. Submitted January 2, 2015. Revision accepted March 2, 2015. Published online July 18, 2015. Correspondence: Carol L. Shields, MD, Ocular Oncology Service, 840 Walnut Street, Suite 1440, Philadelphia, PA 19107 (email: [email protected]). J AAPOS 2015;19:379-381. Copyright Ó 2015 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2015.03.016
Journal of AAPOS
Sivalingam, Say, and Shields
379
ized as a “microstaphyloma,” in a girl with combined hamartoma of the retina and RPE.
n 2006 Jampol and colleagues1 described a myopic patient with a peculiar finding on time domain optical coherence tomography (OCT), which showed unilateral cup-shaped depression of the subfoveal retinal pigment epithelium (RPE) and choroid with outward bowing. The overlying retina was flat and visual acuity was preserved. Margolis and colleagues2 later more precisely illustrated the enhanced-depth imaging spectral domain OCT (EDI-OCT) features of this “microstaphyloma” in 13 eyes and used the term focal choroidal excavation (FCE). They found this condition most often in eyes with mild myopia, manifesting as a yellow spot in the foveal region with EDI-OCT features of an abrupt cup-shaped RPE-choroidal depression with focal choroidal thinning. They noted 2 retinal patterns: in one, the retina appeared attached to the RPE (conforming); in the other, separated from the RPE (nonconforming). They suspected that conforming FCE might transform slowly into nonconforming FCE, but their follow-up was less than 1 year.2 Little is known about the cause of FCE, but a developmental origin has been hypothesized because most demonstrate no previous insult.1,2 However, secondary FCE has been associated with age-related macular degeneration, central serous chorioretinopathy, polypoidal choroidal vasculopathy, and Best disease.3-6 We report the evolution of conforming FCE by EDI-OCT from an undulation in the RPE to a focal cuplike excavation over 9 years in a child with combined hamartoma of the retina and RPE.
I
Case Report A 19-month-old girl was referred to the Ocular Oncology Service at Wills Eye Hospital for exotropia in the left eye that was first noticed by her mother at 9 months of age. On examination, visual acuity in each eye was central, steady, and maintained in the office, but parents reported a right eye preference. Intraocular pressure and anterior segment examination were normal in both eyes. Ultrasonography showed similar axial lengths in both eyes. Fundus examination of the right eye was normal. The left fundus showed a lightly pigmented epimacular membrane with retinal vascular dragging and slight gray retinal discoloration, consistent with combined hamartoma of the retina and RPE. A trial of patching of the right eye was advised. At 5 years of age, visual acuity was 20/20 in the right eye and 20/400 in the left eye. The hamartoma remained stable clinically and time domain OCT was first performed, revealing an undulating topography in the retina, RPE, choroid, and sclera, with dense epiretinal membrane, thickening and disorganization of all retinal layers, thin choroid, and subtle outward bowing of the RPE, choroid, and sclera. At 10 years of age, fundus examination and vision were stable and time domain OCT showed a smooth epiretinal membrane with retinal thickening and more
Belovay et al
377
Plateau iris is more commonly observed in the young female adult population between 30-50 years of age, much older than the youngest previously reported patient, a 12year-old boy.3 Other children and adolescents have been reported in the literature with angle closure, but none with a definitive diagnosis of plateau iris.4-6 We report 5 cases of plateau iris in children who presented to either the Hospital for Sick Children, Toronto (HSC), Canada, or Centre Hospitalier Universitaire Sainte-Justine (CHUSJ), Montreal, Canada.
Case 1
Plateau iris in children* Graham W. Belovay, MD, FRCSC,a Talal Alabduljalil, MD, FRCSC,a Charles J. Pavlin, MD, FRCSC,a,b,c Patrick Hamel, MD, FRCSC,d,e and Asim Ali, MD, FRCSCa,f Narrow iridocorneal angles, a very rare condition in the pediatric population, can lead to visual loss through angle closure glaucoma. In the workup for patients with narrow iridocorneal angles, plateau iris must be considered in the differential diagnosis. We describe 5 children with plateau iris, the youngest 5 years of age. All were confirmed using ultrasound biomicroscopy and were offered iridotomy for treatment.
A 5-year-old, white healthy girl was referred to the HSC for narrow angles. Uncorrected visual acuity was 20/25 in the right and 20/30 in the left eye. There was no family history of ocular disease. Intraocular pressures measured by rebound tonometry (Icare-Finland,Vantaa) predilation were 16 mm Hg in the right eye and 20 mm Hg in the left eye, increasing slightly after dilation to 21 mm Hg in the right eye and 25 mm Hg in the left eye. Cycloplegic refraction showed mild hyperopia in both eyes. Gonioscopy was Shaffer grade 2, with a narrow peripheral angle, flat profile, and no peripheral anterior synechiae (PAS) in each eye. The cup:disk ratio was 0.2 in each eye. Ultrasound biomicroscopy (UBM) performed under general anesthesia confirmed PIC in each eye (Figure 1). Options for treatment were discussed, and the patient underwent a bilateral laser peripheral iridotomy (LPI) under general anesthesia with no change in the angles.
P
Case 2
Author affiliations: aDepartment of Ophthalmology and Vision Sciences, University of Toronto, Canada; bMount Sinai Hospital, Toronto, Canada; cPrincess Margaret Hospital, Toronto, Canada; dCentre Hospitalier Universitaire Sainte-Justine, Montreal, Canada; e Universite de Montreal, Montreal Canada; fThe Hospital for Sick Children, Toronto, Canada Presented in part at the Annual Meeting of the Canadian Ophthalmological Society, Montreal, June 15-17, 2013. * This publication is dedicated to our late colleague, coauthor, and pioneer in ophthalmic ultrasound, Dr. Charles J Pavlin. Submitted October 13, 2014. Revision accepted February 27, 2015. Published online July 31, 2015. Correspondence: Dr. Asim Ali, MD, FRCSC, The Hospital for Sick Children, 555 University Avenue, Toronto, Canada ON M5G 1X8 (email: [email protected]). J AAPOS 2015;19:377-379. Copyright Ó 2015 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2015.02.017
A 13-year-old white girl initially presented to the HSC with papillophlebitis in the left eye and a left relative afferent pupillary defect. Her presenting visual acuity in each eye was 20/20; IOP was 22 mm Hg in each eye. Her cup:disk ratio was 0.2 in the right eye. Her papillophlebitis was confirmed with an intravenous fluorescein angiogram and gradually improved without treatment. All investigations to rule out a causative etiology for her papillophlebitis were negative. At 1 month’s follow-up she presented with an IOP of 18 mm Hg in the right eye and 28 mm Hg in the left eye by Goldmann applanation tonometry, with no change in visual acuity. Gonioscopy showed plateau configuration of the iris in both eyes with the typical double hump sign. The right eye was Shaffer grade 1-2, with no PAS. In the left eye there was steep iris configuration, with PAS nasally and Shaffer grade 0-1. She was started on dorzolamide hydrochloride 2%–timolol maleate 0.5% in the left eye. An UBM assessment confirmed bilateral PIC. She did not undergo the offered intervention of LPI and was lost to follow-up for several years, returning with transient blurry vision with associated headache. Her examination was unchanged, with normal Humphrey 24-2 visual field testing.
lateau iris configuration (PIC) has been shown by ultrasound biomicroscopy to be caused by an anterior positioning of the ciliary processes behind the iris, preventing the peripheral angle from opening after iridotomy.1 PIC is typically diagnosed clinically by gonioscopy showing a persistently narrow angle following iridotomy. Plateau iris syndrome (PIS) is a subgroup of PIC with a patent iridotomy and increased intraocular pressure (IOP) on dilation.2
Journal of AAPOS
378
Belovay et al
Volume 19 Number 4 / August 2015
Cases 4 and 5
FIG 1. Ultrasound biomicroscopy images of case 1 showing the forward positioning of the ciliary processes (CP) supporting the peripheral iris and absence of ciliary sulcus (A). After laser peripheral iridotomy (B) the iris profile flattens, but the angles remain narrow. The arrow represents the location of the scleral spur.
Case 3 A 7-year-old white boy was referred from a local optometrist to the CHUSJ for high IOP in each eye. His presenting visual acuity was 20/70 in the right eye and 20/ 100 in the left eye, with an IOP of 31 mm Hg (right eye) and 32 mm Hg (left eye) by Tono-Pen applanation tonometry (Reichert Technologies, Depew, New York). Gonioscopy showed 90 of inferior angle closure and Shaffer grade 1 elsewhere in each eye. UBM showed PIC in each eye. The patient’s mother had a history of narrow-angle glaucoma treated from age 25 years. The patient underwent bilateral LPI under general anesthesia, with no change in angles. Afterward the pressure remained elevated, at 25 mm Hg in the right eye and 27 mm Hg in the left eye; he was therefore started on timolol maleate 0.25%. His cup:disk ratio at the time was 0.4 in the right eye and 0.65 in the left eye. Cycloplegic refraction showed him to be hyperopic (13.50 10.50 70, 14.00 10.75 10) and glasses were prescribed. His IOPs gradually increased over the year and he was switched to brimonidine tartrate 0.2%–timolol maleate 0.5% in both eyes. Five years after presentation his visual acuity was 20/40 in both eyes, with a cup:disk ratio of 0.6 in the right eye and 0.8 in the left eye. The diffuse depression (in each eye) and an inferior nasal step (left eye) on Humphrey 24-2 visual field testing has remained stable since diagnosis. He was also diagnosed with X-linked juvenile retinoschisis several years after initial presentation. Due to the chronic angle closure circumstances of his presentation it was not possible to determine whether he had PIS.
Twin 5-year-old white boys were referred to the CHUSJ for assessment of narrow angles. They were born at 34 weeks’ gestation and their maternal grandfather had glaucoma with low vision at age 45. Case 4 had presenting visual acuity of 20/20 in both eyes, with IOP by Tono-Pen of 20 mm Hg in the right eye and 19 mm Hg in the left eye. Cycloplegic refraction showed mild hyperopia in each eye. The cup:disk ratio was 0.2 in each eye. Gonioscopy in each eye showed Shaffer grade 2 angles, with the ciliary body only visible in the superior and inferior quadrants on indentation. UBM confirmed PIC. The patient underwent bilateral LPI under general anesthesia with follow-up IOP by Goldmann applanation tonometry of 14 mm Hg in the right eye and 21 mm Hg in the left eye and no change in angles. Visual acuity and optic disks have since remained unchanged. Case 5 had visual acuity of 20/25 in the right eye and 20/ 30 in the left eye, with presenting IOP of 18 mm Hg in the right eye and 17 mm Hg in the left eye by Tono-Pen. His cup:disk ratio was 0.2 in each eye. Gonioscopy was the same as his twin. UBM showed PIC. He also underwent bilateral PI under general anesthesia, with follow-up IOP of 13 mm Hg in the right eye and 15 mm Hg in the left eye by Tono-Pen and no change in angles. His follow-up cycloplegic refraction showed mild hyperopia in each eye. His most recent visual acuity was 20/20 in both eyes, with no optic disk changes.
Discussion The assessment of narrow iridocorneal angles in children can be challenging. Many will require an examination under anesthesia to perform gonioscopy and confirmatory imaging. The various etiologies reported in this population include iridociliary cysts,7,8 primary angle closure, retinopathy of prematurity,7 nanophthalmos,7 plateau iris, microspherophakia, iris tumors, and anterior segment dysgenesis. As pediatric patients are not routinely screened for narrow irdocorneal angles until later in life, glaucoma could develop insidiously. The prevalence of plateau iris in the pediatric population is unknown. Only single patient case reports exist; population-based studies have examined adults exclusively.9 As performing reliable gonioscopy can be difficult in children, UBM should be considered the imaging modality of choice to make a definitive diagnosis. In one study, the prevalence of PIC in nonglaucomatous eyes of adults was between 20.9% and 25.2%, but the long-term conversion rate to glaucoma is unknown.9 The initial recommended treatment for plateau iris is LPI to eliminate the pupil block, followed by pilocarpine, argon laser iridoplasty, or clear lens removal as needed.10 We empirically recommend that children with PIC should be followed every 3 months to ensure that they do not develop glaucoma.
Journal of AAPOS
Volume 19 Number 4 / August 2015 References 1. Pavlin CJ, Ritch R, Foster FS. Ultrasound biomicroscopy in plateau iris syndrome. Am J Ophthalmol 1992;113:390-95. 2. Cheng J, Buys YM, Spaeth GL. Confusion with the misuse of plateau iris terminology. J Glaucoma 2013;22:265-6. 3. Llinas A, Dorairaj S, Liebmann JM, Ritch R. Plateau iris syndrome in a child. Eye 2008;22:597-8. 4. Fivgas GD, Beck AD. Angle-closure glaucoma in a 10-year-old girl. Am J Ophthalmol 1997;124:251-3. 5. Appleby RS Jr, Kinder RS. Bilateral angle-closure glaucoma in a 14year-old boy. Arch Ophthalmol 1971;86:449-50. 6. Jones DE, Watson DM. Angle-closure glaucoma precipitated by the use of phospholine iodide for esotropia in a child. Br J Ophthalmol 1967;51:783-5. 7. Ritch R, Chang BM, Liebmann JM. Angle closure in younger patients. Ophthalmology 2003;110:1880-89. 8. Shields JA, Shields CL, Lois N, Mercado G. Iris cysts in children: classification, incidence, and management. The 1998 Torrence A Makley Jr Lecture. Br J Ophthalmol 1999;83:334-8. 9. Li Y, Wang YE, Huang G, et al. Prevalence and characteristics of plateau iris configuration among American Caucasian, American Chinese and mainland Chinese subjects. Br J Ophthalmol 2014;98:474-8. 10. Ng WT, Morgan W. Mechanisms and treatment of primary angle closure: a review. Clin Experiment Ophthalmol 2012;40:e218-28.
Evolution of focal choroidal excavation underlying combined hamartoma of the retina and retinal pigment epithelium in a child Meera D. Sivalingam, BS, Emil Anthony T. Say, MD, and Carol L. Shields, MD Combined hamartoma of the retina and retinal pigment epithelium (RPE) is a benign tumor seen mostly in children. Enhanced-depth imaging optical coherence tomography (OCT) of these tumors often shows an epiretinal membrane, tangential traction, disorganization of the retinal layers, and underlying uniform choroidal thinning. We describe the evolution over 9 years of focal choroidal excavation, a novel finding on OCT character-
Author affiliations: Ocular Oncology Service, Wills Eye Institute, Thomas Jefferson University, Philadelphia, Pennsylvania Support provided by Eye Tumor Research Foundation, Philadelphia, PA (CLS). The funders had no role in the design and conduct of the study, in the collection, analysis and interpretation of the data, and in the preparation, review or approval of the manuscript. Carol L. Shields, MD, has had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis. Submitted January 2, 2015. Revision accepted March 2, 2015. Published online July 18, 2015. Correspondence: Carol L. Shields, MD, Ocular Oncology Service, 840 Walnut Street, Suite 1440, Philadelphia, PA 19107 (email: [email protected]). J AAPOS 2015;19:379-381. Copyright Ó 2015 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2015.03.016
Journal of AAPOS
Sivalingam, Say, and Shields
379
ized as a “microstaphyloma,” in a girl with combined hamartoma of the retina and RPE.
n 2006 Jampol and colleagues1 described a myopic patient with a peculiar finding on time domain optical coherence tomography (OCT), which showed unilateral cup-shaped depression of the subfoveal retinal pigment epithelium (RPE) and choroid with outward bowing. The overlying retina was flat and visual acuity was preserved. Margolis and colleagues2 later more precisely illustrated the enhanced-depth imaging spectral domain OCT (EDI-OCT) features of this “microstaphyloma” in 13 eyes and used the term focal choroidal excavation (FCE). They found this condition most often in eyes with mild myopia, manifesting as a yellow spot in the foveal region with EDI-OCT features of an abrupt cup-shaped RPE-choroidal depression with focal choroidal thinning. They noted 2 retinal patterns: in one, the retina appeared attached to the RPE (conforming); in the other, separated from the RPE (nonconforming). They suspected that conforming FCE might transform slowly into nonconforming FCE, but their follow-up was less than 1 year.2 Little is known about the cause of FCE, but a developmental origin has been hypothesized because most demonstrate no previous insult.1,2 However, secondary FCE has been associated with age-related macular degeneration, central serous chorioretinopathy, polypoidal choroidal vasculopathy, and Best disease.3-6 We report the evolution of conforming FCE by EDI-OCT from an undulation in the RPE to a focal cuplike excavation over 9 years in a child with combined hamartoma of the retina and RPE.
I
Case Report A 19-month-old girl was referred to the Ocular Oncology Service at Wills Eye Hospital for exotropia in the left eye that was first noticed by her mother at 9 months of age. On examination, visual acuity in each eye was central, steady, and maintained in the office, but parents reported a right eye preference. Intraocular pressure and anterior segment examination were normal in both eyes. Ultrasonography showed similar axial lengths in both eyes. Fundus examination of the right eye was normal. The left fundus showed a lightly pigmented epimacular membrane with retinal vascular dragging and slight gray retinal discoloration, consistent with combined hamartoma of the retina and RPE. A trial of patching of the right eye was advised. At 5 years of age, visual acuity was 20/20 in the right eye and 20/400 in the left eye. The hamartoma remained stable clinically and time domain OCT was first performed, revealing an undulating topography in the retina, RPE, choroid, and sclera, with dense epiretinal membrane, thickening and disorganization of all retinal layers, thin choroid, and subtle outward bowing of the RPE, choroid, and sclera. At 10 years of age, fundus examination and vision were stable and time domain OCT showed a smooth epiretinal membrane with retinal thickening and more