Multiple reports describe adverse events during and following ROP screening, including oxygen desaturation, apnea, and bradycardia, 6 calling into question the effect and stress of the actual examination on the neonate contrib- uting to these events. 1,4 One study showed a significant rise in heart rate (P 5 0.0272) and a significant decrease in oxygen saturation levels (P 5 0.0275) in 30 neonates immediately after the ROP examination. 7 In addition, apnea and bradycardia of prematurity (ABP) is common and occurs in the majority of infants weighing \1,000 g at birth. By 36 weeks’ corrected gestational age, however, most infants outgrow ABP. 6 Our patient was already 41 weeks old, with no known history of ABP; moreover, the cardiopulmonary arrest occurred prior to the ROP examination and only after administration of the eyedrops. This suggests that the Cy- clomydril contributed to the cardiopulmonary arrest. Furthermore, cyclopentolate was the likely culprit given that phenylephrine would have mostly been cleared by the time the patient had a second event of apnea and bradycardia, almost 3 hours after the eyedrops were administered. 8 This infant did have undiagnosed pulmonary hypertension, which probably contributed to her cardiopulmonary arrest. The ef- fect of cyclopentolate or phenylephrine on infants with pul- monary hypertension has not been studied. The use of dilating eye drops for ROP screening has known risks. Punctal occlusion of the nasolacrimal duct after drop administration can reduce systemic absorption, although further research is necessary to determine its true effectiveness. 1 Reducing the drop size may also be of benefit: research has shown that a smaller drop size is as effective at dilating pupils as a standard drop size. 1,4 However, even with these modifications neonates may still experience episodes of apnea and bradycardia during screening. In the hospital setting the infants are in a carefully controlled and monitored environment with extensive support personnel. However in the outpatient office, this is not the case, so pediatric ophthalmologists should be equipped to handle this type of emergency, either personally or with ancillary services that are immediately available. Literature Search PubMed was last searched on October 24, 2013, without date or language restriction, using the following keywords: cyclomydril, cyclopentolate, cyclogyl, tropicamide, and phenyleph- rine in combination with the following terms: 0.2%, compli- cations, adverse effects, apnea, infant, pulmonary hypertension. References 1. Mitchell AJ, Green A, Jeffs DA, Roberson PK. Physiologic effects of retinopathy of prematurity screening examinations. Adv Neonatal Care 2011;11:291-7. 2. Lim DL, Batilando M, Rajadurai VS. Transient paralytic ileus following the use of cyclopentolate-phenylephrine eye drops during screening for retinopathy of prematurity. J Paediatr. Child Health 2003;39:318-20. 3. Shiuey Y, Eisenberg MJ. Cardiovascular effects of commonly used ophthalmic medications. Clin Cardiol 1996;19:5-8. 4. Wheatcroft S, Sharma A, McAllister J. Reduction in mydriatic drop size in premature infants. Br J Ophthalmol 1993;77:364-5. 5. Young TE. Pharmacology review: topical mydriatics: the adverse effects of screening examinations for retinopathy of prematurity. Neoreviews 2003;4:e163-6. 6. Wood MG, Kaufman LM. Apnea and bradycardia in two premature in- fants during routine outpatient retinopathy of prematurity screening. J AAPOS 2009;13:501-3. 7. Rush R, Rush S, Nicolau J, Chapman K, Naqvi M. Systemic manifes- tations in response to mydriasis and physical examination during screening for retinopathy of prematurity. Retina 2004;24:242-5. 8. Understanding phenylephrine metabolism, pharmacokinetics, bioavailability and activity. Briefing document for the Nonprescription Drugs Advisory Committee. Summit, NJ: Schering-Plough Corpora- tion; December 14, 2007. Early detection of recurrent primary iris stromal cyst using ultrasound biomicroscopy Pavel Pochop, MD, PhD, Gabriela Mahelkova, MD, PhD, Jir ı Cendel ın, MD, PhD, and Denisa Petruskova, MD Primary iris stromal cyst characteristically presents as a smooth, round, translucent mass in the anterior chamber. In small children it tends to grow rapidly and may be confused with intraocular malig- nancy. Management is difficult, and recurrences are frequent. We report 2 cases of primary iris stromal cyst in which recurrence was detected relatively early using ultrasound biomicroscopy and successfully managed with iridectomy. Case 1 A n 18-month-old girl with a 12-month history of growing iris lesion was referred to Department of Ophthalmology, 2nd Faculty of Medicine, Charles University, Prague, and Motol University Hospital. An ex- amination of the left eye revealed a large, translucent cystic lesion on the inferomedial iris measuring 5 5 4 mm and distorting the pupil. Fine vessels covered the surface of the clear fluid-filled cyst (Figure 1A). The underlying iris Author affiliations: Department of Ophtalmology, 2nd Faculty of Medicine, Charles University in Prague and Motol University Hospital, Czech Republic Supported by the Ministry of Health, Czech Republic—conceptual development of research organization, University Hospital Motol, Prague, Czech Republic, 00064203. Submitted July 3, 2013. Revision accepted September 14, 2013. Correspondence: Pavel Pochop, MD, PhD, Department of Ophtalmology, University Hospital Motol, V Uvalu 84 15006, Prague 5, Czech Republic (email: pavel.pochop@gmail. com). J AAPOS 2014;18:184-186. Copyright Ó 2014 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2013.09.017 Journal of AAPOS 184 Pochop et al Volume 18 Number 2 / April 2014
Transcript
184 Pochop et al Volume 18 Number 2 / April 2014
Multiple reports describe adverse events during andfollowing ROP screening, including oxygen desaturation,apnea, and bradycardia,6 calling into question the effectand stress of the actual examination on the neonate contrib-uting to these events.1,4 One study showed a significant risein heart rate (P 5 0.0272) and a significant decrease inoxygen saturation levels (P 5 0.0275) in 30 neonatesimmediately after the ROP examination.7 In addition, apneaand bradycardia of prematurity (ABP) is common and occursin the majority of infants weighing\1,000 g at birth. By 36weeks’ corrected gestational age, however, most infantsoutgrow ABP.6 Our patient was already 41 weeks old, withno known history of ABP; moreover, the cardiopulmonaryarrest occurred prior to the ROP examination and only afteradministration of the eyedrops. This suggests that the Cy-clomydril contributed to the cardiopulmonary arrest.Furthermore, cyclopentolate was the likely culprit giventhat phenylephrine would have mostly been cleared by thetime the patient had a secondevent of apnea andbradycardia,almost 3 hours after the eyedrops were administered.8 Thisinfant did have undiagnosed pulmonary hypertension,whichprobably contributed to her cardiopulmonary arrest. The ef-fect of cyclopentolate or phenylephrine on infants with pul-monary hypertension has not been studied.
The use of dilating eye drops for ROP screening hasknown risks. Punctal occlusion of the nasolacrimal ductafter drop administration can reduce systemic absorption,although further research is necessary to determine its trueeffectiveness.1 Reducing the drop sizemay also be of benefit:research has shown that a smaller drop size is as effective atdilating pupils as a standard drop size.1,4 However, evenwith these modifications neonates may still experienceepisodes of apnea and bradycardia during screening. In thehospital setting the infants are in a carefully controlled andmonitored environment with extensive support personnel.However in the outpatient office, this is not the case, sopediatric ophthalmologists should be equipped to handlethis type of emergency, either personally or with ancillaryservices that are immediately available.
Author affiliations: Department of Ophtalmology, 2nd Faculty of Medicine, Charles
Literature Search
PubMed was last searched on October 24, 2013, withoutdate or language restriction, using the following keywords:cyclomydril, cyclopentolate, cyclogyl, tropicamide, and phenyleph-rine in combination with the following terms: 0.2%, compli-cations, adverse effects, apnea, infant, pulmonary hypertension.
University in Prague and Motol University Hospital, Czech RepublicSupported by the Ministry of Health, Czech Republic—conceptual development of
research organization, University Hospital Motol, Prague, Czech Republic, 00064203.Submitted July 3, 2013.Revision accepted September 14, 2013.Correspondence: Pavel Pochop, MD, PhD, Department of Ophtalmology, University
Hospital Motol, V Uvalu 84 15006, Prague 5, Czech Republic (email: [email protected]).J AAPOS 2014;18:184-186.Copyright � 2014 by the American Association for Pediatric Ophthalmology and
1. Mitchell AJ, Green A, Jeffs DA, Roberson PK. Physiologic effects ofretinopathy of prematurity screening examinations. Adv NeonatalCare 2011;11:291-7.
2. Lim DL, Batilando M, Rajadurai VS. Transient paralytic ileusfollowing the use of cyclopentolate-phenylephrine eye drops duringscreening for retinopathy of prematurity. J Paediatr. Child Health2003;39:318-20.
3. Shiuey Y, Eisenberg MJ. Cardiovascular effects of commonly usedophthalmic medications. Clin Cardiol 1996;19:5-8.
4. Wheatcroft S, Sharma A, McAllister J. Reduction in mydriatic dropsize in premature infants. Br J Ophthalmol 1993;77:364-5.
5. Young TE. Pharmacology review: topical mydriatics: the adverseeffects of screening examinations for retinopathy of prematurity.Neoreviews 2003;4:e163-6.
6. WoodMG,KaufmanLM. Apnea and bradycardia in two premature in-fants during routine outpatient retinopathy of prematurity screening.J AAPOS 2009;13:501-3.
7. Rush R, Rush S, Nicolau J, Chapman K, Naqvi M. Systemic manifes-tations in response to mydriasis and physical examination duringscreening for retinopathy of prematurity. Retina 2004;24:242-5.
8. Understanding phenylephrine metabolism, pharmacokinetics,bioavailability and activity. Briefing document for the NonprescriptionDrugs Advisory Committee. Summit, NJ: Schering-Plough Corpora-tion; December 14, 2007.
Primary iris stromal cyst characteristically presents as a smooth,round, translucent mass in the anterior chamber. In small childrenit tends to grow rapidly and may be confused with intraocular malig-nancy. Management is difficult, and recurrences are frequent. Wereport 2 cases of primary iris stromal cyst in which recurrencewas detected relatively early using ultrasound biomicroscopy andsuccessfully managed with iridectomy.
Case 1
An 18-month-old girl with a 12-month history ofgrowing iris lesion was referred to Department ofOphthalmology, 2nd Faculty of Medicine, Charles
University, Prague, and Motol University Hospital. An ex-amination of the left eye revealed a large, translucent cysticlesion on the inferomedial iris measuring 5� 5� 4mm anddistorting the pupil. Fine vessels covered the surface of theclear fluid-filled cyst (Figure 1A). The underlying iris
stroma was atrophic, allowing visualization of the posterioriris pigment epithelium. Ultrasound biomicroscopy(UBM) revealed a relatively thin-walled anechogenic struc-ture inferotemporally in the anterior chamber. Because ofits amblyogenic potential, surgery was scheduled for3 weeks later. In the meantime, the lesion enlarged tooccupy 90% of the anterior chamber, opposing the cornealendothelium.Introperatively, a clear corneal incision was made and
sodium hyaluronate was injected into the anterior cham-ber. The anterior cyst wall was dissected free from cornealendothelium with viscoelastic and the cyst aspirated suc-cessfully. Iris resection, including the entire cystic lesionand iridoplasty, was performed. Histopathological of thecyst revealed a lining of nonkeratinizing, multilayeredstratified squamous epithelium with goblet cells, confirm-ing the diagnosis of primary iris stromal cyst (Figure 1B).Recurrence of the cyst near the iridoplasty suture 6weeks
postoperatively was confirmed on UBM (Figure 1C), andlarger total iridectomy was carried out. There was no evi-dence of recurrence 3 months after surgery: the corneawas clear, and there was pigment on the anterior lenscapsule, with subcapsular opacity within the total iridec-tomy. The clinical findings remained stable through thelast follow-up, at 15months after surgery. The visual acuityof the left eye was 5/5 without correction.
Case 2
A 7-year-old girl with a 4-year history of a slowlygrowing iris lesion in the right eye was referred to ourdepartment. Examination of the right eye revealed a large,translucent cystic lesion on the inferomedial iris measuring5 � 5 � 7 mm and distorting the pupil (Figure 2A). All theother characteristics of the cystic lesion were the same as inthe first case. The surface was covered with fine vessels, thecyst contained clear fluid, and the underlying iris stromawas atrophic. Our intervention was managed as in the firstcase. In this case too recurrence was discovered on UBM(Figure 2B) 1 month postoperatively. Again, we performeda larger, total iridectomy. At 2 years’ follow-up there wasno evidence of recurrence. Best-corrected visual acuity inthe right eye was 5/5. The lens remained clear.
FIG 1. Congenital intrastromal iris cyst in the left eye of an 18-month-old girl (case 1). A, Clinical photograph at presentation. B, Histopathol-ogy of (hematoxylin and eosin, original magnification � 40).C, Ultrasound biomicroscopy showing the cyst extending into theiris stroma.
Discussion
Congenital intrastromal epithelial cysts are rare space-occupying lesions of the anterior chamber. Only 9 casesof primary iris stromal cysts have been reported before20 years of age.1 These cysts are defined as epithelial-lined structures arising within the iris stroma in an eyewith no history of prior surgery or penetrating oculartrauma.2 Several theories have been proposed for its em-bryonic tissue origin. The most accepted is entrapment ofsurface epithelium within the eye at the time of lensvesicle invagination.3,4 The primary iris stromal cyst isusually clearly visible in the anterior chamber as a
Journal of AAPOS
smooth, round translucent mass. Although in adults, thelesion may become stable over time, in children it has atendency to enlarge.3 It may obscure the visual axis andlead to poor vision, amblyopia, secondary glaucoma,corneal decompensation, and a painful eye. In these cases,surgical intervention is necessary. Treatment approacheshave ranged from normal tissue sparing to moreradical approaches (needle aspiration, cryotherapy,
FIG 2. Cystic lesion on the inferomedial iris in a 7-year-old girl (case 2).A, Clinical photograph at presentation. B, Ultrasound biomicroscopyshowing several small cystic lesions on the rim of coloboma.
Author affiliations: Department of Ophthalmology, University of California–San Francisco,San Francisco, CaliforniaSubmitted May 19, 2013.Revision accepted September 14, 2013.Correspondence: Ian R. Gorovoy, Department of Ophthalmology, University of
California–San Francisco, 10 Koret Way, K301, San Francisco, CA 94143-0730 (email:[email protected]).J AAPOS 2014;18:186-188.Copyright � 2014 by the American Association for Pediatric Ophthalmology and
186 Gorovoy and de Alba Campomanes Volume 18 Number 2 / April 2014
endophotocoagulation, microdiathermy, injection ofchemical substances, partial lamellar iridectomy, iridec-tomy or iridogoniocyclectomy).4-6 Due to the smallnumber of cases and the variety of managementtechniques used, however, it is difficult to determine thebest approach. All available treatments present somerisk. Recurrence after even gentle treatment isfrequent—Lois and colleagues5 reported recurrence in 4of 9 pediatric cases. Use of more radical approaches isassociated with serious complications and poor visual out-comes.
New technologies have made diagnosis easier3,7,8 andallow early detection of recurrence.9 In combination withclose patient monitoring, these technologies permit moreconservative treatment to reduce the risk of serious compli-cations, such as diffuse corneal edema, peripheral bandkeratopathy5 and secondary glaucoma.10 In both casesdescribed here, the lesion was managed by a pupil-sparingsurgical approach; early recurrence was detected usingUBMand successfully treated, with good final visual acuity.
Literature Search
PubMed was searched from 1960 to 2013 for the followingterms: ultrasound biomicroscopy, iris cyst, and treatment.
References
1. Shields JA, Shields CL, Lois N, Mercado G. Iris cysts in children:classification, incidence, and management. Br J Ophthalomol 1999;83:334-8.
2. Shields JA. Primary cysts of the iris. Trans AmOphthalmol Soc 1981;79:771-809.
3. Rao A,Gupta V, Bhadange Y, SharmaR, Shields JA. Iris cyst: a review.Semin Ophthalmol 2011;26:11-22.
5. Lois N, Shields CL, Shields JA,MercadoG, De Potter P. Primary irisstromal cyst: a report of 17 cases. Ophthalmology 1998;105:1317-22.
6. Wiwatwongwana D, Ittipunkul N, Wiwatwongwana A. Ab externolaser photocoagulation for the treatment of spontaneous iris stromalcyst. Graefes Arch Clin Exp Ophthalmol 2012;250:155-6.
7. Zhou M, Xu G, Bojanowski CM, et al. Differential diagnosis of ante-rior chamber cysts with ultrasound biomicroscopy: ciliary bodymedulloepithelioma. Acta Ophthalmol Scand 2006;84:137-9.
8. Augsbureger JJ, Affel LL, Benarosch DA. Ultrasound biomicroscopyof cystic lesions of the iris and cilliary body. Trans Am OphthalmolSoc 1996;94:259-74.
9. Kawaguchi K, Yamamoto S, Nagae Y, Okada A, Iwasaki N, Tano Y.Treatment of recurrent giant iris cyst with intracyst administration ofmitomycin C. Br J Ophthalmol 2000;84:799.
10. Brooks SE, Baerveldt G, Rao NA, Smith RE. Primary iris stromalcysts. J Pediatr Ophthalmol Strabismus 1993;30:194-8.
A potential life-savingdiagnosis—recognizing TurcotsyndromeIan R. Gorovoy, MD, and Alejandra de AlbaCampomanes, MD
A previously healthy 9-year-old girl presented with ataxia, head-aches, and nausea of 1 month’s duration. Magnetic resonanceimaging demonstrated a large posterior fossa mass. Posteriorsegment examination revealed pigmented ocular fundus lesions(POFLs), which included cometoid dark lesions with depigmentedtails and smaller, dark midperipheral lesions. The patient under-went resection for a medulloblastoma. Because of these specificretinal lesions in combination with her medullobastoma, a diag-nosis of Turcot syndrome was made and subsequently confirmedby genetic testing. Turcot syndrome is one of the familial adenoma-tous polyposis (FAP) syndromes. This diagnosis may be life-savingbecause 100% of FAP patients develop colon cancer that can becured only early with timely colectomy.
