- Email: [email protected]
Topical autologous serum promotes enucleation wound healing in retinoblastoma patients
Volume 19 Number 4 / August 2015 4. Kobayashi A, Sugiyama K. Successful removal of a large conjunctival cyst using colored 2.3% sodium hyaluronate. Ophthalmic Surg Lasers Imaging 2007;38:81-3. 5. Kothari M. A novel method for management of conjunctival inclusion cysts following strabismus surgery using isopropyl alcohol with paired injection technique. J AAPOS 2009;13:521-2.
Topical autologous serum promotes enucleation wound healing in retinoblastoma patients Madeline K. Kwok, FRCSEd, FCOphthHK,a,b Jason C. S. YAM, FRCSEd, FCOphthHK,a,b Christopher B. O. YU, FRCOphth, FCOphthHK,b and Flora H. S. LAU, MRCSEd, FCOphthHKa,b Two retinoblastoma patients underwent uneventful enucleations while undergoing perioperative chemotherapy. In both cases the postoperative course was complicated by poor conjunctival wound healing. Administration of topical autologous serum was associated with wound healing and conjunctivalization of these wounds.
C
hemoreduction has been used to manage retinoblastoma since the 1990s: most centers adopt the three-agent protocol of vincristine, etoposide, and carboplatin (VEC).1 Chemotherapeutic agents affect cutaneous wound healing by decreasing wound contraction and fibroblast proliferation.2 Acute surgical wounds are susceptible to chemotherapy, which disrupts the critical inflammatory and proliferative phases of early wound healing. In the conjunctival stroma wound healing is thought to simulate other vascularized tissues in the body.3 Thus chemotherapy can similarly jeopardize conjunctival wound healing. The efficacy of topical autologous serum (TAS) in promoting cell migration on corneal epithelium has been well documented.4 We postulate that this applies similarly to the conjunctival epithelium and suggest that TAS plays a role in promoting healing of conjunctival wounds, which are affected by perioperative systemic chemotherapy. To our knowledge, this is the first report describing such use in post-enucleation conjunctival wounds of patients with retinoblastoma.
Author affiliations: aDepartment of Ophthalmology, Hong Kong Eye Hospital, Hong Kong SAR, China; bDepartment of Ophthalmology and Visual Sciences, The Chinese University of Hong Kong, Hong Kong SAR, China Submitted October 15, 2014. Revision accepted February 25, 2015. Published online July 22, 2015. Correspondence: Dr. Madeline K. Kwok, FRCSEd, FCOphthHK, Hong Kong Eye Hospital, 147K Argyle Street, Kowloon, Hong Kong (email: [email protected]). J AAPOS 2015;19:375-377. Copyright Ó 2015 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2015.02.016
Journal of AAPOS
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Case Reports Two eyes of 2 retinoblastoma patients, both grade E retinoblastoma using the International Classification of Retinoblastoma, underwent uneventful enucleations and orbital implant (MedPor; Porex Surgical Inc, Newnan, GA) insertions under general anesthesia. The diagnoses were confirmed histologically. Both patients had bilateral retinoblastoma and received perioperative VEC as chemoreduction therapy. TAS was used postoperatively to promote conjunctival wound healing. All surgeries and postoperative assessments were performed at Hong Kong Eye Hospital (HKEH) by a single surgeon (FHSL). Enucleation involved careful removal of the entire globe and a long segment of the optic nerve, followed by insertion of donor sclera–wrapped MedPor implant. Four rectus muscles were sutured to four openings created in the donor sclera using 6-0 polyglactin 910 sutures. Then Tenon’s fascia and conjunctiva were closed in two layers using interrupted 6-0 and 8-0 polyglactin 910 sutures, respectively. Poor conjunctival wound healing was initially treated with topical antibiotics after obtaining a conjunctival wound swab. TAS, manufactured according to the protocol by Tsubota and colleagues,5 was initiated based on the clinical judgment of no response to the antibiotic regimen. Case 1 was a 2-month old boy with leukocoria who was diagnosed with bilateral retinoblastoma. At presentation, the right eye tumor occupied the entire vitreous and enucleation was recommended. However, parents opted for intravenous chemoreduction therapy and laser photocoagulation as the initial treatment. After 3 cycles of chemotherapy, the right eye responded poorly and developed lens subluxation and phthisical changes. Enucleation was performed and the conjunctival wound was wellapposed, with healthy epithelium on postoperative day one. Topical 0.1% dexamethasone-neomycin-polymyxin B combination eyedrops was commenced 4 times daily, followed by the fourth cycle of chemotherapy on postoperative day 5. Five days later, the conjunctival wound showed necrosis. The regimen was changed to levofloxacin 0.5% eyedrops every 2 hours and ofloxacin ointment at bedtime, with no response after 3 days. TAS was added 4 times daily. The conjunctival wound began healing on day 2 and was completely healed on day 13 following initiation of TAS. Wound culture showed no growth. TAS was discontinued after 4 weeks and the patient completed 5 more cycles of chemotherapy without complications. The patient was followed for 27 months postoperatively. Case 2 was a 25-month-old boy with leukocoria who was diagnosed with bilateral retinoblastoma and treated with intravenous chemoreduction therapy and laser photocoagulation. Twenty-six days after the ninth cycle of chemotherapy, the left eye was enucleated due to exudative retinal detachment. The conjunctival wound was well apposed, with healthy epithelium on postoperative day 2. Dexamethasone-neomycin-polymyxin B combination
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eyedrops were initiated 4 times daily. The conjunctival wound healed poorly over 3 weeks (Figure 1A) and the regimen was switched to levofloxacin eyedrops every 2 hours and tobramycin ointment twice daily with poor response after 19 days. TAS was added 3 times daily and 2 days later, 12.5 mg amikacin sulphate was administered subconjunctivally. The conjunctival wound started healing on day 7 and completed healing on day 14 after initiation of TAS (Figure 1B). TAS was continued for 21 days without further complication. Wound swab showed limited growth of Staphylococcus aureus. The patient was followed for 8 months postoperatively.
Discussion Chemotherapy works by interfering with specific components of the cell cycle to prevent cancer cell proliferation. Unfortunately, this effect is nonselective and affects all rapidly dividing cells, whether benign or malignant. Studies of perioperative chemotherapy effects on wound healing have shown inconsistent results. Some animal studies demonstrated decreased wound tensile strength with a single dose of alkylating agent, whereas other human studies failed to correlate wound healing complications with standard therapeutic doses of chemotherapy.6,7 In HKEH, a tertiary referral center managing 90% of retinoblastoma patients in Hong Kong, most enucleations are performed after completion of chemoreduction therapy. In our experience, the same chemotherapeutic agents and postoperative regimen have not affected conjunctival wound healing in the absence of perioperative chemotherapy. Both patients reported here received 9 cycles of chemoreduction therapy due to the aggressive nature of the disease. No systemic complication was identified throughout treatment. Patient 1 underwent enucleation followed by 6 more cycles of chemotherapy; patient 2 underwent enucleation 26 days after the last dose of chemotherapy. Both encountered impediment of wound healing, although the time between enucleation and chemotherapy varied. TAS appeared to promote healing of these conjunctival wounds within 7 days of use and this appears to be especially evident in patient 1. TAS contains epitheliotrophic factors such as epidermal growth factors (EGF) and vitamin A, that play a vital role in its therapeutic effect on ocular surface disorders.8 Although the therapeutic effect of TAS on the conjunctiva has not been fully studied, vitamin A and EGF were found to be essential in maintaining normal conjunctival histology and be required in the cultivation of conjunctival epithelial cells in serum-free media.9,10 Furthermore, clinical studies have demonstrated the benefit of TAS in treating hyperpermeability of avascular bleb walls.10 All these support a therapeutic role of TAS in promoting conjunctivalization. In both patients, topical steroids were discontinued when TAS was initiated. This may itself have promoted wound healing and is a limitation of our report. Neverthe-
FIG 1. Clinical photographs of patient 2. A, Pre-topical autologous serum (TAS) conjunctival wound. B, Healing conjunctival wound after 2 weeks of TAS treatment.
less, TAS appears to play a role in promoting healing of conjunctival wounds.
References 1. Shields CL, Shields JA. Retinoblastoma management: advances in enucleation, intravenous chemoreduction, and intra-arterial chemotherapy. Curr Opin Ophthalmol 2010;21:203-12. 2. Stadelmann WK, Digenis AG, Tobin GR. Impediments to wound healing. Am J Surg 1998;176(2A suppl):39S-47S. 3. Pepperl JE, Ghuman T, Gill KS, Zieske JD, Trocme SD. Conjunctiva. Duane’s Ophthalmology on CD-ROM. Philadelphia: Lippincott Williams & Silkins; 2006:29. 4. Geerling G, MacLennan S, Hartwig D. Autologous serum eye drops for ocular surface disorders. Br J Ophthalmol 2004;88:1467-74. 5. Tsubota K, Goto E, Shimmura S, Shimazaki J. Treatment of persistent corneal epithelial defect by autologous serum application. Ophthalmology 1999;106:1984-9. 6. Shirafuji T, Oka T, Sawada T, et al. The importance of peripheral blood leukocytes and macrophage infiltration on bronchial wall wound healing in rats treated preoperatively with anticancer agents. Surg Today 2001;31:308-16.
Journal of AAPOS
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
Topical autologous serum promotes enucleation wound healing in retinoblastoma patients Madeline K. Kwok, FRCSEd, FCOphthHK,a,b Jason C. S. YAM, FRCSEd, FCOphthHK,a,b Christopher B. O. YU, FRCOphth, FCOphthHK,b and Flora H. S. LAU, MRCSEd, FCOphthHKa,b Two retinoblastoma patients underwent uneventful enucleations while undergoing perioperative chemotherapy. In both cases the postoperative course was complicated by poor conjunctival wound healing. Administration of topical autologous serum was associated with wound healing and conjunctivalization of these wounds.
C
hemoreduction has been used to manage retinoblastoma since the 1990s: most centers adopt the three-agent protocol of vincristine, etoposide, and carboplatin (VEC).1 Chemotherapeutic agents affect cutaneous wound healing by decreasing wound contraction and fibroblast proliferation.2 Acute surgical wounds are susceptible to chemotherapy, which disrupts the critical inflammatory and proliferative phases of early wound healing. In the conjunctival stroma wound healing is thought to simulate other vascularized tissues in the body.3 Thus chemotherapy can similarly jeopardize conjunctival wound healing. The efficacy of topical autologous serum (TAS) in promoting cell migration on corneal epithelium has been well documented.4 We postulate that this applies similarly to the conjunctival epithelium and suggest that TAS plays a role in promoting healing of conjunctival wounds, which are affected by perioperative systemic chemotherapy. To our knowledge, this is the first report describing such use in post-enucleation conjunctival wounds of patients with retinoblastoma.
Author affiliations: aDepartment of Ophthalmology, Hong Kong Eye Hospital, Hong Kong SAR, China; bDepartment of Ophthalmology and Visual Sciences, The Chinese University of Hong Kong, Hong Kong SAR, China Submitted October 15, 2014. Revision accepted February 25, 2015. Published online July 22, 2015. Correspondence: Dr. Madeline K. Kwok, FRCSEd, FCOphthHK, Hong Kong Eye Hospital, 147K Argyle Street, Kowloon, Hong Kong (email: [email protected]). J AAPOS 2015;19:375-377. Copyright Ó 2015 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2015.02.016
Journal of AAPOS
Kwok et al
375
Case Reports Two eyes of 2 retinoblastoma patients, both grade E retinoblastoma using the International Classification of Retinoblastoma, underwent uneventful enucleations and orbital implant (MedPor; Porex Surgical Inc, Newnan, GA) insertions under general anesthesia. The diagnoses were confirmed histologically. Both patients had bilateral retinoblastoma and received perioperative VEC as chemoreduction therapy. TAS was used postoperatively to promote conjunctival wound healing. All surgeries and postoperative assessments were performed at Hong Kong Eye Hospital (HKEH) by a single surgeon (FHSL). Enucleation involved careful removal of the entire globe and a long segment of the optic nerve, followed by insertion of donor sclera–wrapped MedPor implant. Four rectus muscles were sutured to four openings created in the donor sclera using 6-0 polyglactin 910 sutures. Then Tenon’s fascia and conjunctiva were closed in two layers using interrupted 6-0 and 8-0 polyglactin 910 sutures, respectively. Poor conjunctival wound healing was initially treated with topical antibiotics after obtaining a conjunctival wound swab. TAS, manufactured according to the protocol by Tsubota and colleagues,5 was initiated based on the clinical judgment of no response to the antibiotic regimen. Case 1 was a 2-month old boy with leukocoria who was diagnosed with bilateral retinoblastoma. At presentation, the right eye tumor occupied the entire vitreous and enucleation was recommended. However, parents opted for intravenous chemoreduction therapy and laser photocoagulation as the initial treatment. After 3 cycles of chemotherapy, the right eye responded poorly and developed lens subluxation and phthisical changes. Enucleation was performed and the conjunctival wound was wellapposed, with healthy epithelium on postoperative day one. Topical 0.1% dexamethasone-neomycin-polymyxin B combination eyedrops was commenced 4 times daily, followed by the fourth cycle of chemotherapy on postoperative day 5. Five days later, the conjunctival wound showed necrosis. The regimen was changed to levofloxacin 0.5% eyedrops every 2 hours and ofloxacin ointment at bedtime, with no response after 3 days. TAS was added 4 times daily. The conjunctival wound began healing on day 2 and was completely healed on day 13 following initiation of TAS. Wound culture showed no growth. TAS was discontinued after 4 weeks and the patient completed 5 more cycles of chemotherapy without complications. The patient was followed for 27 months postoperatively. Case 2 was a 25-month-old boy with leukocoria who was diagnosed with bilateral retinoblastoma and treated with intravenous chemoreduction therapy and laser photocoagulation. Twenty-six days after the ninth cycle of chemotherapy, the left eye was enucleated due to exudative retinal detachment. The conjunctival wound was well apposed, with healthy epithelium on postoperative day 2. Dexamethasone-neomycin-polymyxin B combination
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Volume 19 Number 4 / August 2015
eyedrops were initiated 4 times daily. The conjunctival wound healed poorly over 3 weeks (Figure 1A) and the regimen was switched to levofloxacin eyedrops every 2 hours and tobramycin ointment twice daily with poor response after 19 days. TAS was added 3 times daily and 2 days later, 12.5 mg amikacin sulphate was administered subconjunctivally. The conjunctival wound started healing on day 7 and completed healing on day 14 after initiation of TAS (Figure 1B). TAS was continued for 21 days without further complication. Wound swab showed limited growth of Staphylococcus aureus. The patient was followed for 8 months postoperatively.
Discussion Chemotherapy works by interfering with specific components of the cell cycle to prevent cancer cell proliferation. Unfortunately, this effect is nonselective and affects all rapidly dividing cells, whether benign or malignant. Studies of perioperative chemotherapy effects on wound healing have shown inconsistent results. Some animal studies demonstrated decreased wound tensile strength with a single dose of alkylating agent, whereas other human studies failed to correlate wound healing complications with standard therapeutic doses of chemotherapy.6,7 In HKEH, a tertiary referral center managing 90% of retinoblastoma patients in Hong Kong, most enucleations are performed after completion of chemoreduction therapy. In our experience, the same chemotherapeutic agents and postoperative regimen have not affected conjunctival wound healing in the absence of perioperative chemotherapy. Both patients reported here received 9 cycles of chemoreduction therapy due to the aggressive nature of the disease. No systemic complication was identified throughout treatment. Patient 1 underwent enucleation followed by 6 more cycles of chemotherapy; patient 2 underwent enucleation 26 days after the last dose of chemotherapy. Both encountered impediment of wound healing, although the time between enucleation and chemotherapy varied. TAS appeared to promote healing of these conjunctival wounds within 7 days of use and this appears to be especially evident in patient 1. TAS contains epitheliotrophic factors such as epidermal growth factors (EGF) and vitamin A, that play a vital role in its therapeutic effect on ocular surface disorders.8 Although the therapeutic effect of TAS on the conjunctiva has not been fully studied, vitamin A and EGF were found to be essential in maintaining normal conjunctival histology and be required in the cultivation of conjunctival epithelial cells in serum-free media.9,10 Furthermore, clinical studies have demonstrated the benefit of TAS in treating hyperpermeability of avascular bleb walls.10 All these support a therapeutic role of TAS in promoting conjunctivalization. In both patients, topical steroids were discontinued when TAS was initiated. This may itself have promoted wound healing and is a limitation of our report. Neverthe-
FIG 1. Clinical photographs of patient 2. A, Pre-topical autologous serum (TAS) conjunctival wound. B, Healing conjunctival wound after 2 weeks of TAS treatment.
less, TAS appears to play a role in promoting healing of conjunctival wounds.
References 1. Shields CL, Shields JA. Retinoblastoma management: advances in enucleation, intravenous chemoreduction, and intra-arterial chemotherapy. Curr Opin Ophthalmol 2010;21:203-12. 2. Stadelmann WK, Digenis AG, Tobin GR. Impediments to wound healing. Am J Surg 1998;176(2A suppl):39S-47S. 3. Pepperl JE, Ghuman T, Gill KS, Zieske JD, Trocme SD. Conjunctiva. Duane’s Ophthalmology on CD-ROM. Philadelphia: Lippincott Williams & Silkins; 2006:29. 4. Geerling G, MacLennan S, Hartwig D. Autologous serum eye drops for ocular surface disorders. Br J Ophthalmol 2004;88:1467-74. 5. Tsubota K, Goto E, Shimmura S, Shimazaki J. Treatment of persistent corneal epithelial defect by autologous serum application. Ophthalmology 1999;106:1984-9. 6. Shirafuji T, Oka T, Sawada T, et al. The importance of peripheral blood leukocytes and macrophage infiltration on bronchial wall wound healing in rats treated preoperatively with anticancer agents. Surg Today 2001;31:308-16.
Journal of AAPOS
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