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Online Advanced Release
Articles appearing in this "Online Advanced Release" section have been peer-reviewed and accepted for publication in the Journal of Pediatric Ophthalmology & Strabismus and posted online before print publication. Articles appear on this site after copyediting and author corrections, but before final proofing. The content of the article will usually remain unchanged, and possible further corrections are fairly minor. The final published article will specify the issue and page numbers. Once the final version is available in print, the version posted here will be removed from this site.
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Table of Contents
Management of Disinserted Inferior Rectus Muscle by Fixation of Globe to the Inferior Orbital Margin
Rohit Saxena, MD; Swati Phuljhele, MD; Pavan Lohiya, MD; Pradeep Sharma, MD; Vimla Menon, MSOriginally submitted: 7/12/09; Accepted: 9/2/09; Posted online: 2/23/10
Trauma to the orbital floor is often associated with trauma to the inferior rectus muscle. The currently available options yield inconsistent postoperative results. The authors describe a new technique in which the globe was fixed to the inferior orbital margin with the help of a silicone band and titanium orbital plate.
doi: 10.3928/01913913-20100218-09 [get full text]
Mehmet Saldir, MD; Serdar Umit Sarici, MD; Fatih Mehmet Mutlu, MD; Cem Mocan, MD; Halil Ibrahim Altinsoy, MD; Okan Ozcan, MDOriginally submitted: 11/3/08; Accepted: 7/27/09; Posted online: 2/23/10
Background: To determine the frequency of ophthalmologic problems and the risk factors that affect the occurrence of these problems in premature newborns with a gestational age of 32 weeks or less.
Methods: Premature newborns observed at a neonatal intensive care unit between January 2002 and March 2006 were included. A control visit including an ophthalmologic examination was performed at 10 months of age or later. Primary ocular morbidities were studied, and the association between these parameters and prenatal, perinatal, and neonatal characteristics were evaluated.
Results: A total of 169 premature newborns were included in the study, and they were examined at a mean age of 25.85 ± 11.79 months (range: 10 to 42 months). There was complete vision loss (blindness) in 1 (0.6%) case, strabismus in 15 (8.9%) cases, and refractive errors in 10 (5.9%) cases. Twenty (77%) cases with any abnormality and 50 (35%) cases with a normal examination at follow-up had a history of ROP at any stage during the neonatal period (P = .001). Short gestational age (P = .018), low birth weight (P = .002), and the presence of ROP requiring retinal surgery during the neonatal period (P = .007) were determined to be significant risk factors for the development of vision loss, strabismus, and refractive errors.
Conclusion: Neonates with a gestational age of 32 weeks or less, especially those younger than 30 weeks, should not only be screened for ROP in the neonatal period, but should also have regular follow-up examinations to check for the development of other ophthalmologic problems during infancy and early childhood.
doi: 10.3928/01913913-20100218-08 [get full text]
Congenital Levator-Inferior Rectus Synkinesis
Junyeop Lee, MD; Myungmi Kim, MD, PhD; Junhyuk Son, MDOriginally submitted: 2/23/09; Accepted: 8/4/09; Posted online: 2/23/10
Aberrant innervation involving oculomotor nerve without evidence of antecedent oculomotor nerve palsy is rare. Three patients with unilateral eyelid retraction on infraduction without a history of previous oculomotor palsy were examined. Unilateral eyelid retraction on attempted down gaze was demonstrated in all cases and all presented with ipsilateral inferior oblique overaction to a greater or lesser degree. Ipsilateral ptosis of the upper eyelids was observed in primary gaze in two cases. There were no abnormal findings in biomicroscopic and funduscopic examinations. Pupillary examinations were also unremarkable. After repair of ptosis, the upper eyelid retraction remained and became increased on down gaze. Surgery for repair of ipsilateral ptosis in congenital levator-inferior rectus synkinesis should be performed cautiously because it may aggravate the eyelid retraction on down gaze.
doi: 10.3928/01913913-20100218-07 [get full text]
Two Cases of Congenital Retrobulbar Cyst or Peripapillary Staphyloma With an Alcoholic Mother
Ai Kitano, MD, PhD; Kumi Shirai, MD, PhD; Yuka Okada, MD, PhD; Shizuya Saika, MD, PhDOriginally submitted: 12/10/08; Accepted: 9/2/09; Posted online: 2/23/10
The authors report two cases of congenital retrobulbar cyst or large peripapillary staphyloma with alcoholic mothers. In the first case, a 53-day-old newborn was referred for evaluation of microphthalmia in her left eye. Ophthalmic examination showed microcornea, microphthalmia, and an abnormally enlarged optic disc with an excavation in her left eye. Imaging revealed the presence of a cystic lesion in her left orbit that seemed to be communicated with the vitreous cavity. She did not have chromosomal abnormality. In the second case, a large peripapillary staphyloma was detected in the right eye of a 7-month-old newborn. The mother of each patient was an alcoholic. Retrobulbar cystic lesion or peripapillary staphyloma could be considered to be a series of abnormalities caused by the degree of the failure of the optic cup. Effects of each mother’s alcohol intake on the development of the eye, especially the closure of the optic cup, is to be further investigated.
doi: 10.3928/01913913-20100218-06 [get full text]
Silicone-Eyelid Closure to Improve Vision in Deeply Amblyopic Eyes
Ossama M. Hakim, MD, FRCS; Yasser Gaber El-Hag, MD, FRCS; Ahmed Samir, MDOriginally submitted: 8/24/09; Accepted: 11/12/09; Posted online: 2/23/10
Background: Deep amblyopia is a recognized cause of monocular visual deficit in children with a high rate of poor compliance with conventional occlusion therapy. This study evaluates a new occlusion technique that can be used for children with failed amblyopia treatment.
Methods: In a prospective study, surgical silicone-eyelid closure was done for 15 children (age: 4 to 6 years; median age: 5.2 years) with a history of deep amblyopia and poor compliance with occlusion methods. In this technique, the good eye was closed by passing one limb of a double armed 5-0 polypropylene suture from one eyelid margin through a silicone sleeve and through the corresponding eyelid margin. The other polypropylene limb was passed behind the silicone sleeve and then through the opposite eyelid margin to form a barrier between the sleeve and the cornea. After 2 to 4 weeks, the suture was cut and the silicone sleeve was removed.
Results: Preoperative visual acuity ranged between counting fingers at 1 meter and counting fingers at 4 meters. Following eyelid closure, visual acuity was improved to between 20/40 and 20/200 for 12 patients, whereas three patients did not improve. Complications were seen in 8 patients: marginal eyelid irritation and erythema in 6 and suture break in 2.
Conclusion: Silicone-eyelid closure can be used safely and effectively for management of deep amblyopia. This technique affords a good option to restore vision in a deeply amblyopic eye and grants the child a lifetime of useful vision.
doi: 10.3928/01913913-20100218-05 [get full text]
Malignant Mixed Tumor of the Lacrimal Gland in a Teenager
Francesca M. Giliberti; Roman Shinder, MD; Diana Bell, MD; Aaron M. Savar, MD; Justin M. Sacks, MD; Nicholas B. Levine, MD; Bita Esmaeli, MDOriginally submitted: 6/11/09; Accepted: 9/2/09; Posted online: 2/23/10
A 17-year-old boy presented with a painless, right superotemporal orbital mass. Imaging demonstrated a lacrimal gland mass extending into the temporalis muscle through the lateral orbital wall. The patient underwent an orbital exenteration. Histopathology revealed malignant mixed tumor (carcinoma ex-pleomorphic adenoma) of the lacrimal gland with perineural and vascular invasion.
doi: 10.3928/01913913-20100218-04 [get full text]
Termination of Amblyopia Treatment: When to Stop Follow-up Visits and Risk Factors for Recurrence
Christine De Weger, MSc; Henry J. B. Van Den Brom, MD; Robert Lindeboom, PhDOriginally submitted: 5/6/09; Accepted: 8/4/09; Posted online: 2/23/10
Background: This study estimated when it is safe to stop follow-up visits after cessation of amblyopia treatment and to identify factors associated with deterioration of visual acuity.
Methods: Study patients included 282 patients aged 7 to 13 years who were monitored for deterioration after cessation of amblyopia treatment (median follow-up: 3.9 years).
Results: Six (2.1%) patients lost 2 or more logarithm of the minimum angle of resolution levels of visual acuity and 77 (27.3%) patients lost 1 or more Snellen lines of visual acuity. Good compliance with re-treatment stopped further deterioration and lost visual acuity was regained (average follow-up after re-treatment: 3.3 years). Life-table-analysis indicated that 95% of the cases that deteriorated occurred within 24 months after cessation of treatment. Multivariable analysis corrected for duration of treatment uncovered factors independently associated with deterioration.
Conclusion: A clinically important risk of deterioration of visual acuity was found during the first 2 years after cessation of amblyopia treatment. Follow-up time longer than 2 years is recommended in the presence of a developing risk factor such as increasing anisometropia. With prompt re-treatment and good compliance, deterioration can be stopped and visual acuity can be restored.
doi: 10.3928/01913913-20100218-03 [get full text]
Coats’ Disease and Neovascular Glaucoma in a Child With Neurofibromatosis
Shin-Lin Chiu, MD; San-Ni Chen, MD; Yan-Ting Chen, MD; Pao-Ju Chen, MDOriginally submitted: 6/25/09; Accepted: 9/22/09; Posted online: 2/23/10
This report describes an extremely rare case of Coats’ disease and neovascular glaucoma in a child with neurofibromatosis 1. Intraocular pressure control and vision restoration were achieved with intravitreal injection with bevacizumab, hemiretinal laser photocoagulation, peripheral retinal cryotherapy, and trabeculectomy with mitomycin C.
doi: 10.3928/01913913-20100218-02 [get full text]
Nalini K. Aggarwal, MD; Sai B. Gandham, MD; Rebecca Weinstein, MD; Robert Saltzmann, MD; David S. Walton, MDOriginally submitted: 2/23/09; Accepted: 8/4/09; Posted online: 2/23/10
Purpose: To examine the clinical and gonioscopic findings in patients with glaucoma associated with Sturge–Weber syndrome.
Methods: Retrospective review of clinical findings of all patients with Sturge–Weber syndrome who presented between January 1978 and December 2003. Koeppe gonioscopy was performed under general anesthesia and findings were photographed when feasible. Iris color was documented by color sketch or photograph at initial presentation. Fisher exact test was used to determine whether the presence of heterochromia was statistically higher in the glaucomatous group.
Results: Fifty-five patients who met inclusion criteria were identified, of whom 44 (80%) had glaucoma. Unilateral glaucoma was diagnosed in 30 eyes and bilateral glaucoma in 14 eyes, yielding 58 eyes with glaucoma and 52 eyes without. Gonioscopic examination revealed distinct anatomic abnormalities in the anterior chamber angle in 32 (55%) of the glaucomatous eyes in contrast to the normal fellow eyes in patients with unilateral glaucoma and to the patients without glaucoma. Heterochromia iridis with darker iris on the glaucomatous side was present in 11 of the 30 patients with unilateral glaucoma (37%, P < .001) but in none of the patients without glaucoma.
Conclusion: The characteristics observed in the anterior chamber angle in this large, uniquely examined series are the first reported to the authors’ knowledge and may support a role for neural crest derived anomalies in the pathogenesis of glaucoma in these infant eyes. Careful examination for heterochromia iridis may be judicious in these patients.
doi: 10.3928/01913913-20100218-01 [get full text]
Augmentin Duo in the Treatment of Childhood Blepharokeratoconjunctivitis
Jasmina Cehajic-Kapetanovic, MA Phy Sci, BM BCh Oxon, MRCOphth; Jeff Kwartz, FRCOphthOriginally submitted: 10/10/08; Accepted: 8/4/09; Posted online: 1/22/10
Purpose: To report the use of Augmentin Duo 400/57 (GlaxoSmithKline, Middlesex, UK) in the treatment of childhood blepharokeratoconjunctivitis (BKC).
Methods: This is a retrospective interventional case series. The case notes of 7 consecutive patients treated with Augmentin Duo 400/57 for BKC during 18 months were reviewed. Diagnostic criteria for BKC were blepharitis including recurrent chalazia and meibomian gland dysfunction, eyelid margin telangiectasia and facial rosacea, recurrent episodes of chronic red eye, photophobia, watering, punctate superficial keratopathy, corneal neovascularization, and corneal ulcers.
Results: Seven children (age range: 6 to 14 years) were diagnosed as having BKC. All children received systemic Augmentin Duo 400/57 and showed considerable improvement within the first month of therapy. Six children had no recurrences during a mean follow-up of 6 months. No patients experienced any side effects from this treatment.
Conclusions: Augmentin Duo 400/57 has not previously been reported in the treatment of BKC in children. In this case series, Augmentin Duo 400/57 proved to be at least as effective as current treatments with systemic erythromycin or doxycycline with the advantage of a twice-daily dosage and a superior side-effect profile.
doi: 10.3928/01913913-20100118-01 [get full text]
Posterior Polymorphous Dystrophy and Keratoglobus in a Child
Sangita P. Patel, MD, PhD; Manoj M. Sajnani, MBBS, FRCS (Glasgow); Roberto Pineda, MDOriginally submitted: 5/7/09; Accepted: 9/2/09; Posted online: 1/22/10
A 13-year-old boy presented with gradually progressive deterioration of vision in both eyes, bilateral photophobia, and regular headaches. Clinical examination, anterior segment findings, and specular microscopy findings were consistent with the diagnosis of posterior polymorphous dystrophy and keratoglobus. To the authors’ knowledge, this is the first pediatric case and the second case overall of the simultaneous occurrence of posterior polymorphous dystrophy and keratoglobus.
doi: 10.3928/01913913-20100118-05 [get full text]
Abnormal Posterior Insertion of the Lateral Rectus Muscle
Yeon-hee Lee, MD; Sang-hyuk Kim, MD; Su-Na Lee, MD; Kwang-sun Seo, MD; Youngjoon Jo, MDOriginally submitted: 12/11/08; Accepted: 9/2/09; Posted online: 1/22/10
Ectopic insertion of the lateral rectus muscle is a rare congenital abnormality. The authors present the case of an exotropic patient with an abnormal posterior insertion of the lateral rectus muscle.
doi: 10.3928/01913913-20100118-04 [get full text]
Conjunctival Marginal Zone B-cell Lymphoma in a 13-Year-Old Child
Shahar Frenkel, MD, PhD; Sujata S. Gaitonde, MD; Nathalie Azar, MD; Michael G. Wood, DOOriginally submitted: 11/1/08; Accepted: 8/4/09; Posted online: 1/22/10
Ocular adnexal lymphoma is a hematopoietic tumor that arises in the conjunctiva, orbit, eyelid, lacrimal gland, or lacrimal sac. The treatment options in children have not been addressed in the literature. The authors describe a 13-year-old child with ocular adnexal lymphoma and discuss the treatment options.
doi: 10.3928/01913913-20100118-02 [get full text]
Tess Garretty, DBOOriginally submitted: 10/6/08; Accepted: 6/17/09; Posted online: 1/22/10
Background: Convergence excess esotropia defines an esotropia on accommodation at near fixation with binocular single vision and motor fusion at distance fixation associated with a high accommodative convergence: accommodation (Ac/A) ratio. The effect of occlusion on exotropia has been well described and is known to alter the Ac/A ratio. This study examines the effect of monocular occlusion on convergence excess esotropia.
Methods: The Ac/A ratio was measured using the gradient method at 1/3 and 6 m in 10 patients with convergence excess esotropia immediately prior to and following a 30-minute period of monocular occlusion.
Results: Clinically and statistically significant differences to the angle of deviation and the Ac/A ratio were found in some patients following occlusion.
Conclusion: Ac/A ratio measurement in convergence excess esotropia is influenced by fusional tenacity. Disrupting this fusion with a period of monocular occlusion results in changes to the angle of deviation in some patients, suggesting subcategorization of this condition into true and simulated convergence excess esotropia. Measurements of the Ac/A ratio are influenced by fixation distance.
doi: 10.3928/01913913-20100118-03 [get full text]
Anomalous Medial Rectus Muscle Insertion in a Patient With Nonrefractive Comitant Esotropia
Jun Ho Choi, MD; Chang Hyun Park, MD; So Young Kim, MDOriginally submitted: 1/7/09; Accepted: 7/27/09; Posted online: 12/23/09
An 8-year-old girl with nonrefractive comitant estropia revealed the abnormal insertion of the medial rectus muscle during the muscle recession surgery. Strabismus surgeons should be aware of the possibility that anomalous muscles can cause unusual types of strabismus.
doi: 10.3928/01913913-20091218-01 [get full text]
Off-Axis Digital Flash Photography: A Common Cause of Artefact Leukocoria in Children
Heather C. Russell, MBChB(Hons); Pankaj K. Agarwal, MBBS; John E. A. Somner, MBChB; Richard J. C. Bowman, MBBChir; Gordon N. Dutton, MDOriginally submitted: 6/18/09; Accepted: 7/27/09; Posted online: 12/23/09
Leukocoria, a presenting sign in several significant pediatric ocular conditions, can be artefactually produced by off-axis flash photography in healthy eyes. The authors demonstrate the conditions needed to produce this phenomenon, which is more commonly seen in children due to their larger-sized pupils, photogenicity, and frequent off-axis shots.
doi: 10.3928/01913913-20091218-04 [get full text]
Unilateral Congenital Eyelid Eversion Causing Marked Chemosis in a Newborn
Roman Shinder, MD; Paul D. Langer, MDOriginally submitted: 6/15/09; Accepted: 7/27/09; Posted online: 12/23/09
A generally healthy male infant was born with unilateral congenital eversion of the upper eyelid causing considerable conjunctival injection and chemosis. Conservative medical management with topical lubrication and antibiotics resolved the condition within a few days.
doi: 10.3928/01913913-20091218-05 [get full text]
Acquired Pediatric Blepharoptosis Related to Linear Scleroderma
Tina Y. Kao, BS; Michael K. Yoon, MD; Thomas N. Hwang, MD, PhD; Michele M. Bloomer, MD; Timothy J. McCulley, MDOriginally submitted: 3/16/09; Accepted: 7/8/09; Posted online: 12/23/09
Periocular linear scleroderma coup de sabre has been seen in association with blepharoptosis. The authors describe a 4-year-old girl who developed left blepharoptosis simultaneously with a remote scalp lesion consistent with linear scleroderma coup de sabre. This case is unique in that the scalp lesion was distant from the affected eye.
doi: 10.3928/01913913-20091218-02 [get full text]
Rhegmatogenous Retinal Detachment Following Treatment for Retinoblastoma
Swakshyar Saumya Pal, MD; Lingam Gopal, MD, FRCSEd; Vikas Khetan, MD; Amit Nagpal, MD; Tarun Sharma, MD, FRCSEd, MBAOriginally submitted: 2/17/09; Accepted: 6/17/09; Posted online: 12/23/09
Purpose: To elucidate characteristics of rhegmatogenous retinal detachment that developed in children who underwent treatment for retinoblastoma and to analyze the outcome following vitreoretinal surgery in such clinical settings.
Methods: This was a retrospective case series of 9 eyes of 9 patients who underwent vitreoretinal surgery for tumor control and retinal reattachment.
Results: At the time of diagnosis of rhegmatogenous retinal detachment, retinoblastoma was regressed in 6 eyes (67%). The retinal detachment involved less than two quadrants in 7 eyes (78%) and was caused by an atrophic break in 7 eyes. Seven eyes underwent a non-drainage scleral buckling procedure. Three eyes underwent vitreous surgery; in 2 of these eyes, tumor excision along with retinectomy using melphalan infusion was performed and eventually silicone oil tamponade was used. Retinal reattachment was achieved in all eyes with eventual tumor control in 7 eyes. The median follow-up after retinal reattachment surgery was 24 months.
Conclusion: Rhegmatogenous retinal detachment in treated eyes with retinoblastoma is usually caused by an atrophic retinal break. Retinal detachment can be repaired successfully in most eyes.
doi: 10.3928/01913913-20091218-03 [get full text]
Choroidal Effusion With Serous Retinal Detachment as a Complication of Strabismus Surgery
Ingele Casteels, MD, PhD; Carl V. Gobin, MDSubmitted: 6/2/09; Accepted: 7/27/09; Posted: 11/23/09
A 12-year-old girl was referred because of unilateral visual loss in the right eye 5 days after uneventful strabismus surgery for a divergent squint. She had hypermetropia of +8 diopters and the axial length of both eyes was below normal for that age. Investigation with funduscopy, central visual field examination, optical coherence tomography, and fluorescein angiography revealed choroidal edema in the affected eye. The macular edema responded well to parabulbar and topical steroids and vision recovered completely within 2 weeks. Strabismus surgery in highly hypermetropic eyes with a small axial length can be complicated by choroidal edema.
doi: 10.3928/01913913-20091118-02 [get full text]
Atropine Treatment of Amblyopia: Is a Swap in Fixation Necessary?
Josephine Leone, BOrth&OphthSc(Hons); Zoran Georgievski, BAppSc(Orth)(Hons); Konstandina Koklanis, PhDSubmitted: 4/25/09; Accepted: 6/17/09; Posted: 11/23/09
Purpose: To investigate the impact of fixation on vision outcomes in patients with amblyopia undergoing atropine treatment and the validity of the “cyclo-swap test” (CST) as a method of predicting atropine efficacy.
Method: Sixteen children with amblyopia were included. The initial examination included a vision assessment and CST, in which fixation was assessed at 1/3 m, 6 m, and at distances less than 1/3 m. Children were treated for 10 weeks and reviewed at 5-week intervals with cessation of atropine 4 days prior. Vision and fixation were assessed at the follow-up visits. Children demonstrating no fixation swap initially were additionally observed after 1 week of atropine treatment under maximum cycloplegia.
Results: Eight children demonstrated a fixation swap (FS group) to the amblyopic eye during the CST at either 1/3 or 6 m, and 8 demonstrated no fixation swap (NFS group). By the end of the 10-week treatment period, both groups demonstrated a mean visual acuity improvement of between 2 and 3 lines (FS = 0.22 log units; NFS = 0.27 log units). Six of the 8 children in the NFS group demonstrated improvement in vision, all of whom also demonstrated a fixation swap at some stage.
Conclusion: Vision improvement in patients using atropine is likely to be attributable to a fixation swap that occurs during the treatment phase. The CST performed at 1/3 and 6 m has little value in predicting improvements in vision; however, when performed at distances of less than 1/3 m, it may provide valuable clinical information about atropine efficacy.
doi: 10.3928/01913913-20091118-05 [get full text]
Ufuk Elgin, MD; Bülent Cankaya, MD; Tulay Simsek, MD; Aygen Batman, MDSubmitted: 12/3/08; Accepted: 6/17/09; Posted: 11/23/09
Purpose: To compare the optic disc topography parameters of children with juvenile diabetes mellitus and normal children using the Heidelberg Retinal Tomograph (HRT III) (Heidelberg Engineering, Heidelberg, Germany).
Methods: The topographic optic disc parameters (cup volume, cup area, rim volume, rim area, disc area, mean cup-to-disc ratio, and mean cup depth) of 28 non-glaucomatous eyes of 28 children with type 1 diabetes mellitus and 28 eyes of 28 age-matched healthy children were compared using the nonparametric Mann–Whitney U test.
Results: No statistically significant differences were found between cup volume (P = .782), cup area (P = .878), rim volume (P = .853), disc area (P = .452), mean cup-to-disc ratio (P = .852), and mean cup depth (P = .711) of eyes of cases with diabetes mellitus and normal subjects.
Conclusion: This result suggests that non-glaucomatous eyes of children with type 1 diabetes mellitus and healthy subjects have similar topographic optic disc characteristics.
doi: 10.3928/01913913-20091118-03 [get full text]
Bilateral Retinoblastoma Presenting With Unilateral Phthisis
Erika Maka, MD; Béla Csákány, MD; Jeannette Tóth, MDSubmitted: 2/21/08; Accepted: 6/12/09; Posted: 11/23/09
Phthisis of the eye is an uncommon presenting sign of retinoblastoma. The authors report a case of bilateral retinoblastoma in a 14-month-old girl who presented with phthisis of the left eye. Enucleation of the painful left eye was performed. Spontaneously regressed, mostly calcified retinoblastoma was diagnosed. The patient underwent chemoreduction. Plaque radiotherapy and transpupillary thermotherapy were applied on the right side. Subsequent ophthalmoscopic examination revealed lesions showing “cottage-cheese” regression. Retinoblastoma should be suspected in any child presenting with phthisis bulbi of unknown origin and a detailed examination of the other eye should be performed.
doi: 10.3928/01913913-20091118-06 [get full text]
Kadircan H. Keskinbora, MD, PhDSubmitted: 5/4/09; Accepted: 6/9/09; Posted: 11/23/09
Purpose: To determine whether unilateral inferior oblique anterior transposition alone could be an effective procedure for treating superior oblique palsy with inferior oblique overaction.
Methods: The records of 38 patients who underwent unilateral inferior oblique anterior transposition for unilateral superior oblique palsy with inferior oblique overaction were evaluated. A comprehensive ocular examination including best-corrected visual acuity measurements, ductions, versions, and deviations at near and distance, head tilt test, abnormal head position, dilated fundus examination, and Titmus test was performed prior to and after surgery.
Results: The mean patient age was 29 years, the mean follow-up was 32 months, the mean preoperative hypertropia in primary position was 14.29 ± 7.7 prism diopters (PD), and the mean inferior oblique overaction was 3.63 ± 0.67. Anterior transposition of the inferior oblique muscle was effective across a wide range of preoperative primary position hypertropia (4 to 35 PD) with a mean reduction in postoperative hypertropia of 12 PD. Inferior oblique overaction was reduced in all patients. No patient demonstrated postoperative primary position hypotropia. Surgery improved stereoacuity nearly two units using the Titmus stereoacuity scale.
Conclusion: Anterior transposition of the inferior oblique muscle is effective in correcting inferior oblique overaction and primary position hypertropia in the treatment of unilateral superior oblique palsy.
doi: 10.3928/01913913-20091118-04 [get full text]
Incidence of Retinopathy of Prematurity in a Neonatal Intensive Care Unit in Nepal
Jyoti Baba Shrestha, MD; Sanyam Bajimaya, MD; Anand Sharma, MD; Jeevan Shresthal, FRCS, FRCOphth; Purna Karmacharya, MDSubmitted: 8/17/08; Accepted: 6/9/09; Posted: 11/23/09
Purpose: To determine the incidence of retinopathy of prematurity (ROP) in low birth weight premature infants admitted to the neonatal intensive care unit of a children’s hospital in Kathmandu.
Methods: A hospital-based prospective study was performed in neonates with a gestational age of less than 36 weeks or birth weight of less than 2,000 g during 1 year. Ophthalmologic examination was performed 2 to 4 weeks after birth and patients were observed until the retinal vascularization was complete. ROP was classified using the revised International Classification of Retinopathy of Prematurity.
Results: A total of 78 infants fulfilled the screening criteria. ROP was detected in 29.5% (n = 23) of neonates. Thirteen infants (16.7%) had stage 1 disease and 5 each (6.4%) were found to have stage 2 and stage 3 ROP. Threshold disease was present in 3.8% (n = 3) of infants with a gestational age of less than 32 weeks and birth weight less than 1,500 g who required laser treatment per Early Treatment of ROP criteria. Low birth weight (P < .01) and low gestational age (P < .01) were significantly associated with the incidence of ROP.
Conclusion: Prematurity and low birth weight were found to be major risk factors for ROP. ROP screening should be performed in such infants for early detection because its incidence is likely to increase as more premature infants survive with improved neonatal care.
doi: 10.3928/01913913-20091118-08 [get full text]
Comparative Study of Unilateral and Bilateral Surgery in Moderate Exotropia
Vimla Menon, MS; M. Ashok Singla, MD; Rohit Saxena, MD; Swati Phulijele, MDSubmitted: 2/23/09; Accepted: 5/15/09; Posted: 11/23/09
Purpose: To evaluate the surgical outcome of unilateral versus bilateral rectus recession for the correction of moderate exotropia.
Methods: This was a prospective study of 20 consecutive cases of intermittent exotropia ranging from 15 to 25 prism diopters (PD) randomized into two groups and operated on by a single surgeon. In the unilateral group, a recession of 7.0 to 7.5 and 8.0 to 8.5 was done for 15 to 20 PD and 21 to 25 PD of exotropia, respectively. In the bilateral group, a recession of 5.0 to 5.5 and 6.0 to 6.5 mm was done for 15 to 20 PD and 21 to 25 PD of exotropia, respectively. Surgical results of both groups were compared 3 months postoperatively.
Results: The mean age of patients was 8.5 years in the unilateral group and 11 years in the bilateral group. The mean preoperative exotropia was 21.0 ± 3.77 PD in the unilateral group and 22.9 ± 3.03 PD in the bilateral group. The mean postoperative deviation at 3 months was 5.5 ± 4.03 PD in the unilateral group and 2.8 ± 3.91 PD in the bilateral group. Consecutive persistent small-angle esotropia was seen in one patient in the bilateral group. There was no significant incomitance at 3 months in either group.
Conclusion: Unilateral and bilateral rectus recession are equally effective in mild to moderate exotropia.
doi: 10.3928/01913913-20091118-07 [get full text]
Unusual Presentation of Sympathetic Ophthalmia
Manisha Agarwal, MS, DNB; S. P. Chaudhary, MS; Ritesh Narula, MS; Kapil Arneja, MS; Jyotirmay Biswas, MDSubmitted: 4/4/09, Accepted: 2/27/09; Posted: 11/23/09
The authors describe an atypical presentation of sympathetic ophthalmia in a child caused by multiple surgical interventions in the exciting eye. Optical coherence tomography findings were noted. Early diagnosis and management with intravenous methylprednisolone and immunosuppressants helped in salvaging the only seeing eye of the child.
doi: 10.3928/01913913-20091118-01 [get full text]
Isolated Anterior Chamber Relapse in a Child With Chronic Myeloid Leukemia: Masquerade Syndrome
Mincheol Seong, MD; Myoung Joon Kim, MD; Hungwon Tchah, MD; Jong Jin Seo, MDSubmitted: 2/17/09; Accepted: 7/08/09; Posted: 10/22/09
The authors report a case of anterior uveitis with hypopyon that was refractory to topical corticosteroid therapy. A detailed history revealed that the patient had chronic myeloid leukemia. Cytology in the aqueous humor was consistent with relapse of chronic myeloid leukemia.
doi: 10.3928/01913913-20091019-09 [get full text]
Giant Cell Astrocytoma of the Retina in a 1-Month-Old Infant
Cecilia S. Jung, MD; G. Baker Hubbard, III, MD; Hans E. Grossniklaus, MD, MBASubmitted: 4/15/09; Accepted: 7/1/09; Posted: 10/22/09
A 1-month-old male infant with tuberous sclerosis presented with leukocoria for an evaluation of possible retinoblastoma. The patient had several Ash leaf spots and multiple subependymal lesions on computed tomography and magnetic resonance imaging. He was found to have a large intraocular tumor in the left eye. B-scan ultrasonography of the mass did not show any calcifications. Given the size of the tumor, poor visual prognosis, and a small possibility of a retinoblastoma, the enucleation of the eye was performed. The mass revealed a retinal giant cell astrocytoma mainly composed of two cell types—giant, round cells and spindle-shaped cells—with associated aggregates of mononuclear inflammatory cells. Immunohistochemical stains were positive for neuron-specific enolase, glial fibrillary acidic protein, and S-100 protein in both cell types. CD3 and CD68 staining were positive in the mononuclear inflammatory cells. Unlike previously reported cases of giant cell astrocytic hamartomas, the current case did not reveal calcifications or substantial inflammations.
doi: 10.3928/01913913-20091019-05 [get full text]
Successful Surgical Intervention for Childhood Pterygium Using a Conjunctival Autograft
Sameer Trikha, MRCOphth; Doreen Khan-Lim, FRCS, FRCOphth; Anthony R. Evans, FRCOphthSubmitted: 1/21/09; Accepted: 7/1/09; Posted: 10/22/09
Childhood pterygia are extremely rare and no consensus currently exists regarding surgical management in cases of significant symptomatology. The authors describe a case of a childhood pterygium in a 9-year-old boy successfully treated with a superior conjunctival autograft. At 6 months of follow-up, no signs of recurrence were detected and the patient reported marked improvement in symptoms. Use of this technique may be beneficial in certain cases of childhood pterygia.
doi: 10.3928/01913913-20091019-11 [get full text]
Terson Syndrome From a Significant Cerebral Infarct Occurring During the Peripartum Period
Edward Rickie L. Chu, MBBS; Teresa Sandinha, FRCEd(Ophth); Stewart Lake, FRCOphth, FRANZCO; John Pater, FRANZCOSubmitted: 10/02/08; Accepted: 6/17/09; Posted: 10/22/09
This case report describes a 23-day-old infant with significant infarct in the right middle cerebral artery territory associated with intraparenchymal hemorrhage within the infarct, subdural hygroma, and bilateral intraocular hemorrhage. The features, severity, and timing of the pathology are suggestive of Terson syndrome that has occurred during the peripartum period.
doi: 10.3928/01913913-20091019-02 [get full text]
Infant Botulism and Raised Intraocular Pressure
Matthew D. Eberly, MD; Ian Uber; Christopher R. Kieling, MD; Richard H. Birdsong, MDSubmitted: 5/26/09; Accepted: 6/12/09; Posted: 10/22/09
Infant botulism is an exceedingly rare disease. Because confirmatory laboratory testing is not available for several days after time of presentation, infant botulism remains a clinical diagnosis. The authors demonstrate how raised intraocular pressure may provide an additional clinical clue to making the diagnosis.
doi: 10.3928/01913913-20091019-03 [get full text]
Neelam Pushker, MD; Mandeep S. Bajaj, MD; Mridula Mehta, MS, DNB; Seema Kashyap, MD; Prashant Yadav, MS; Rachna Meel, MS; Madhu Sudhan, MDSubmitted: 4/4/09; Accepted: 6/12/09; Posted: 10/22/09
The authors describe a 5-year-old boy who had three congenital anomalies (clinical anophthalmos, meningocele, and glial heterotopia) in the orbit. These were associated with multiple neurological anomalies.
doi: 10.3928/01913913-20091019-08 [get full text]
Retinopathy of Prematurity and Shaken Baby Syndrome
Hassan A. Aziz, MD; Audina Berrocal, MD; Robert Sisk, MD; Timothy Murray, MDSubmitted: 3/26/09; Accepted: 5/15/09; Posted: 10/22/09
Retinopathy of prematurity is a vasoproliferative disease that occurs mostly in premature and low birth weight neonates. It is a potentially blinding disease that should be screened vigorously. On the other hand, shaken baby syndrome is a non-accidental trauma that is both common and avoidable. Ophthalmologists play a crucial role in diagnosing and treating the shaken infant. The authors describe a child with the combination of retinopathy of prematurity and shaken baby syndrome.
doi: 10.3928/01913913-20091019-01 [get full text]
A New Transconjunctival Muscle Reinsertion Technique for Minimally Invasive Strabismus Surgery
Daniel S. Mojon, MD, FEBO, ExecMHSASubmitted: 11/2/08; Accepted: 5/15/09; Posted: 10/22/09
Purpose: To describe a novel, minimally invasive strabismus surgery (MISS) technique for horizontal rectus muscle recessions, plications, and advancements.
Methods: Subjects for this prospective study were the first 20 consecutive patients who underwent unilateral horizontal rectus muscle surgery with a transconjunctival suturing (TRASU) technique for primary muscle displacements of 4.5 mm or greater or for repeat muscle displacements of 3.0 mm or greater.
Results: The TRASU technique was associated with no increase in complication rate and was accomplished through a conjunctival approach with an incision that was smaller by 31% ± 11% compared to incisions for previously described MISS approaches. Of the 19 patients (20 eyes) returning for follow-up, 11 (55%) had minimal redness as the only grossly visible sign of surgery on the first postoperative day. By 6 months postoperatively, visual acuity and refractive error were not significantly different from preoperative values (P > .10 for each).
Conclusion: The TRASU technique is safe and can be accomplished through a conjunctival incision that averages two-thirds the size of incisions for previously reported MISS techniques.
doi: 10.3928/01913913-20091019-07 [get full text]
Lihua Wang, MD; Leonard B. Nelson, MD, MBASubmitted: 3/18/09; Accepted: 4/23/09; Posted: 10/22/09
Purpose: To report an outcome study of 100 consecutive children with intermittent exotropia treated by unilateral lateral rectus recession for small to moderate angle exodeviation with a minimum follow-up of 6 months.
Methods: The records of patients with intermittent exotropia younger than 15 years who underwent 7- to 10-mm unilateral lateral rectus recession for exodeviation measuring 15 to 35 prism diopters (PD) from January 2000 to July 2008 were retrospectively reviewed. The surgery was performed according to the amount of distance deviation. A successful alignment was defined as an exodeviation of 5 PD or less and absence of any esotropia in primary and lateral gaze while viewing distant or near targets.
Results: Successful alignment was achieved in 99%, 88%, and 76% of patients at early postoperative, 6-month, and final follow-up, respectively. One overcorrected patient had an esodeviation of 20 PD at 6 months. The results of the final follow-up did not depend on age or refraction at the level of 0.05, whereas the amount of initial exodeviation was found to be significantly correlated with success at the final examination (P = .041). There was a positive significant relationship between results at 6 months and final follow-up (P = .000, r = 0.449). Eleven of the 13 patients who had a second surgery and were observed more than 6 months had successful alignment. Eighty-nine percent of the patients achieved a successful outcome with the combined primary and secondary surgery at the final follow-up.
Conclusion: Unilateral lateral rectus recession is a safe and effective treatment for small to moderate angle intermittent exotropia in children.
doi: 10.3928/01913913-20091019-12 [get full text]
Is Buckle Surgery Still the State of the Art for Retinal Detachments Due to Retinal Dialysis?
Bernhard M. Stoffelns, MD, PhD; Gisbert Richard, MD, PhDSubmitted: 1/15/09; Accepted: 3/19/09; Posted: 10/22/09
Purpose: Retinal dialysis is a frequent cause of retinal detachment in infants and young adults. The authors report long-term results obtained with conventional detachment surgery in a large consecutive series.
Methods: Fifty-two eyes of 50 patients with retinal detachment due to dialysis underwent a segmental buckling procedure between January 1990 and December 1998. Patient characteristics and surgical results at 1 year of follow-up were evaluated. In 2007, 40 eyes from these groups were reexamined for long-term results (follow-up: 9 to 17 years; median: 13.4 years).
Results: The mean age of the patients was 12.8 years (range = 6 to 28 years). Preferred locations of the dialyses were inferotemporal (72%) and superonasal (16%). The macula was detached in 82%. At 1 year of follow-up, the retina was completely reattached after one surgical procedure in 87% and after two procedures in 97%. Long-term follow-up of 40 of 52 eyes revealed no retinal redetachment, but additional surgeries had been performed. Visual acuity improved in 70% of the eyes, but only 40% reattained reading vision due to the high rate of macula-off retinal detachment preoperatively.
Conclusions: Scleral buckling for retinal detachment due to dialysis yields good results, even in the long term, and remains the treatment of choice for these usually young patients despite the increasing popularity of primary vitrectomy.
doi: 10.3928/01913913-20091019-10 [get full text]
Cut and Paste: Sutureless Conjunctival Closure in Strabismus Surgery
Suqin Guo, MD; Rudolph S. Wagner, MD; Brian J. Forbes, MD, PhD; Patrick A. DeRespinis, MD; Anthony R. Caputo, MDSubmitted: 8/14/08; Accepted: 2/3/09; Posted: 10/22/09
Purpose: This study investigated the use of fibrin glue and compared its effect with traditional sutures for conjunctival closure in strabismus surgery.
Methods: The study included 12 patients undergoing horizontal strabismus surgery, of whom 5 underwent bilateral medial rectus muscle recessions and 7 underwent bilateral lateral rectus muscle recessions. For each patient, fibrin glue was used to close the conjunctiva of one eye and 6-0 plain sutures were used to close the other.
Results: All eyes maintained adequate closure of the conjunctiva postoperatively and there were no intraoperative or postoperative complications for any eye. However, the average surgical time needed to apply fibrin glue was considerably less than that required for closure with sutures. Furthermore, eyes closed with fibrin glue were associated with significantly less postoperative inflammation and patient discomfort than those closed with sutures. All patients and parents reported significantly less discomfort from the eyes treated with fibrin glue.
Conclusion: These results are promising and demonstrate a safe and effective alternative to traditional suture closure in strabismus surgery. When compared to traditional suture closure, conjunctival closure with glue includes the following advantages: less postoperative patient discomfort, diminished postoperative inflammation, and potentially reduced surgical time with corresponding reduced time under general anesthesia.
doi: 10.3928/01913913-20091019-04 [get full text]
Recurrence of Congenital Pupillary-Iris-Lens Membrane After Surgical Excision
Alan Connor, MBChB, MRCOphth; James Innes, FRCS (G), FRCOphth; Lawrence Gnanaraj, FRCS (Ophth), FRCSEdSubmitted: 1/15/09; Accepted: 6/9/09; Posted: 9/22/09
The authors present a case of congenital pupillary-iris-lens membrane that demonstrated the previously unreported feature of membrane recurrence after surgical excision. [get full text]
Maculopathy due to Cobalamin C (cb1C) Disease in an Amish Child
Lorna W. Grant, BSc; Shawn E. McCandless, MD; Elias I. Traboulsi, MDSubmitted: 10/19/08; Accepted: 5/15/09; Posted: 9/22/09
A 5-year-old Amish boy diagnosed at birth as having a defect in intracellular cobalamin metabolism, cobalamin C disease (cb1C), presented to the pediatric ophthalmology service with severe visual impairment. Ophthalmoscopy showed bilateral bull’s eye macular lesions. Visual loss occurs from retinal degeneration in cb1C disease. This report highlights the importance of post-natal metabolic testing and ophthalmic evaluation in cb1C disease, especially in high-risk inbred populations. [get full text]
Pseudopapilledema in Kenny–Caffey Syndrome
Laurence Beral, MD; Angélique Donnio, MD; Albert Jean-Charles, MD; Harold Merle, MDSubmitted: 12/18/08; Accepted: 4/13/09; Posted: 9/22/09
A 21-year-old man with Kenny–Caffey syndrome had been observed since 1993 for hyperopia. Fundus examination revealed swollen optic disks. Further examinations (fluorescein angiography, B-scan ultrasonography, and optical coherence tomography) confirmed the optic nerve head elevation. The authors report a rare case of Kenny–Caffey syndrome with extreme pseudopapilledema. Although uncommon, ophthalmologists should be mindful of this disorder when a patient presents with characteristic findings because severe electrolyte disturbances may complicate the clinical course. [get full text]
Refractory Noninfectious Anterior Uveitis Associated With Systemic Mastocytosis
Hani S. Al-Mezaine, MD; Saleh Al-Obeidan, MD; Malak M. Al-Hakeem, MD; Ahmed M. Abu El-Asrar, MDSubmitted: 12/14/08; Accepted: 4/13/09; Posted: 9/22/09
A 10-year-old boy with biopsy-proven systemic mastocytosis presented with bilateral nongranulomatous anterior uveitis associated with high intraocular pressure. Anterior chamber paracentesis was performed for cytological evaluation, which demonstrated monocytes and macrophages but no evidence of mast cells. His uveitis was partially controlled with frequent topical steroid drops and weekly oral methotrexate therapy. The glaucoma was controlled with topical antiglaucoma medications in the right eye. Deep sclerectomy was required in the left eye to control the intraocular pressure. To the best of the authors’ knowledge, this is the first report of such an association. [get full text]
The Novel Concurrence of Noonan Syndrome and Bilateral Duane-like Synkinesis
Nathan M. Kerr, MBChB; Andrea L. Vincent, MBChB, FRANZCOSubmitted: 9/22/08; Accepted: 4/13/09; Posted: 9/22/09
A 9-year-old boy with Noonan syndrome was referred for evaluation of abnormal eye movements. Ocular motility examination revealed bilateral globe retraction with narrowing of the palpebral fissure in adduction and mild restriction of abduction consistent with a bilateral Duane-like synkinesis. This novel association expands the ocular phenotype of Noonan syndrome and may suggest a commonality in the pathogenesis of these two congenital malformation complexes. [get full text]
Ocular Findings in Pediatric Patients With Partial Agenesis of the Corpus Callosum
Raina Goyal, MS, FRCS; Patrick Watts, MS, FRCOphth; Margaret Hourihan, FRCRSubmitted: 6/22/08; Accepted: 3/4/09; Posted: 9/22/09
Purpose: To evaluate the ocular associations with corpus callosum agenesis.
Methods: All children who had corpus callosum agenesis diagnosed by computed tomography or magnetic resonance imaging scans during a 10-year period were included in the study. A complete ophthalmological assessment was undertaken. Data were collected for age, sex, neurological findings, visual acuity, refractive errors, ocular motility defects, and ocular abnormalities. There were no children with Aicardi syndrome in the study.
Results: Nineteen children with corpus callosum agenesis were identified, of whom 13 were examined. All had partial agenesis of the corpus callosum. Visual acuity was better than 20/60 in 5 children (38%), between 20/60 and 20/200 in 3 children (23%), and less than 20/200 in 5 children (38%). Refractive errors were present in 8 children (62%), strabismus in 6 children (46%), nystagmus in 2 children (15%), hypoplastic discs in 3 children (23%), and microphthalmos in 1 child (8%).
Conclusion: Non-syndromic corpus callosum agenesis may be associated with several ocular defects. Poor visual acuity, refractive errors, and strabismus predominate and need to be appropriately addressed to optimize visual function in these children. Global developmental delay (62%) and epilepsy (46%) were the most common neurological associations in this series. [get full text]
Shin Hae Park, MD; Sun Young Shin, MD, PhDSubmitted: 10/27/08; Accepted: 3/4/09; Posted: 9/22/09
Walker–Warburg syndrome, also known as HARD±E, is a congenital autosomal recessive disorder characterized by hydrocephalus, agyria, and retinal dysplasia, with or without encephalocele. The authors describe an infant with Walker–Warburg syndrome who presented with microphthalmia, retinal detachment, and leopard spot retinopathy. [get full text]
Judy E. Kim, MD; Alexander C. Lee, MDSubmitted: 9/8/08; Accepted: 2/19/09; Posted: 9/22/09
Purpose: To evaluate the outcome of indirect diode laser treatment for threshold retinopathy of prematurity (ROP) and to report demographic changes observed over 10 years.
Methods: Retrospective chart review and statistical analysis of 111 eyes of 61 infants treated for threshold ROP between 1991 and 2001 at a single academic institution.
Results: Ninety-six of 111 (86%) eyes showed regression of neovascularization after laser treatment. Fourteen (13%) eyes had zone 1 disease and 97 (87%) eyes had zone 2 disease. Thirty-six percent of eyes with zone 1 disease progressed to stage 4 or 5 disease despite treatment, whereas 10% of the zone 2 eyes progressed. Based on multivariate logistic regression, zone and female gender were found to be predictive of progression, whereas birth weight was predictive of zone. Between 1991 and 2001, a significant trend toward lower mean gestational age at time of birth (Pearson correlation= -0.36, P = .005) and mean birth weight (Pearson correlation= -0.35, P = .0041) was found. However, progression rates to stage 4 and 5 disease following laser treatment were similar for infants born before and after 1997 (P = .0796).
Conclusion: Regression of neovascularization was noted in 86% of eyes following laser treatment for threshold ROP. Zone was predictive of progression to stage 4 or 5 despite treatment. Premature infants being treated for threshold ROP tended to be born at an earlier gestational age and with lower birth weights over time, but progression rates to more severe stages did not increase following laser treatment, suggesting that optimal treatment can yield favorable ocular outcomes. [get full text]
Clinicopathological Study of a Case of Unsuspected Retinoblastoma
Jyotirmay Biswas, MS; Dipankar Das, MS; P. Vaijayanthi, BSc, PGDMLT; Vikas Khetan, MS; S. Krishna Kumar, MDSubmitted: 8/9/08; Accepted: 2/11/09; Posted: 9/22/09
Retinoblastoma is the most common primary malignant intraocular tumor of childhood. It may present with atypical features, particularly in older children. The authors report a case of unsuspected retinoblastoma following evisceration in a 9-year-old girl who had previous pars plana vitrectomy in the affected eye. [get full text]
Clinical Findings and Surgical Results of Duane Retraction Syndrome
Ayse Gul Kocak Altinas, MD; Hasan Basri Arifoglu, MD; Mucella Arikan, MD; Saban Simsek, MDSubmitted: 5/27/08; Accepted: 1/23/09; Posted: 9/22/09
Purpose: To evaluate clinical properties and surgical outcomes of Duane retraction syndrome (DRS).
Methods: Twenty-one patients with DRS were evaluated. Surgery was indicated for correction of strabismus, abnormal head position, upshoot–downshoot, and globe retraction. Six patients had undergone surgery. In 5 cases, the lateral rectus muscle was recessed from 4.0 to 7.0 mm combined with “Y-splitting” according to deviation in primary position and degree of globe retraction. In case of a significantly positive forced duction test on the medial rectus muscle intraoperatively, simultaneous recession of the ipsilateral medial rectus muscle was performed in addition to lateral rectus surgery.
Results: Type I DRS was diagnosed in 83.33% of cases. All 6 cases that underwent surgery had type I DRS. After surgery, all patients showed elimination of upshoot–downshoot. Two cases had minimally improved abduction from the midline. Case 1 underwent a second operation and it was observed that the middle split part disappeared and muscular fibers proliferated to the original insertion. To protect against refusion of the middle split part of the muscle due to proliferation, nonabsorbable separation sutures were placed around the split parts of the upper and lower halves without scleral fixation. Separation sutures were placed on both parts of the muscle to protect refusion in the other cases.
Conclusion: Y-splitting combined with recession of one or both horizontal recti is an effective procedure in patients with DRS and associated horizontal deviation. Prophylactic separation sutures as part of Y-splitting may protect against recurrence. [get full text]
Best's Disease in a 20-Month-Old Child
Jaclyn L. Kovach, MD; Stephen G. Schwartz, MD, MBA; Marcela Hickey, CRA; Craig A. McKeown, MDSubmitted: 10/6/08; Accepted: 2/3/09; Posted: 8/21/09
Best’s disease is an autosomal dominant disorder characterized by bilateral vitelliform macular lesions that progress through several stages throughout life. The authors describe a 20-month-old child with hereditary Best’s disease inherited from his father. To their knowledge, this is the youngest child to be diagnosed as having Best’s disease using photographic documentation. [get full text]
Optic Nerve Aplasia in Aicardi Syndrome
Hassan A. Aziz, MD; Robert A. Sisk, MD; Audina M. Berrocal, MD; Timothy G. Murray, MD, MBA, FACSSubmitted: 6/29/08; Accepted: 2/3/09; Posted: 8/21/09
Aicardi syndrome, first described by French neurologist Jean François Marie Aicardi in 1965, is a constellation of features characterized mainly by the triad of early infantile spasms, chorioretinal lacunae, and agenesis of the corpus callosum. The authors describe a 1-year-old child with Aicardi syndrome with previously unreported ophthalmic features of aplasia of the right optic nerve and bilateral iris colobomas. [get full text]
Use of Anterior Segment OCT in the Evaluation and Management of Congenital Aphakia
Vanissa W. S. Chow, AFCOphthHK; Amy L. Wong, MRCSEd, Mmed; Dorothy S. P. Fan, FRCS, MScSubmitted: 10/19/08; Accepted: 1/23/09; Posted: 8/21/09
Anterior segment optical coherence tomography (AS-OCT) is a noninvasive technology capable of capturing high-resolution images of the anterior segment. With AS-OCT, a 7-year-old boy with nystagmus was diagnosed as having secondary congenital aphakia. The presence of lens capsules on the AS-OCT was of significant prognostic and management value and the child later benefited from bilateral sulcus-fixated intraocular lens implantation surgery. AS-OCT also required less cooperation from the patient compared to ultrasound biomicroscopy. To the authors’ knowledge, this is the first reported case of congenital aphakia diagnosed by AS-OCT. [get full text]
Kevin Gregory-Evans, MD, PhD, FRCS, FRCOphth; Poornima Rai, FRCOphth; Julian Patterson, FRCOphthSubmitted: 8/18/08; Accepted: 1/23/09; Posted: 8/21/09
An 11-year-old boy presented with visual acuity reduced to 20/100 in his left eye. Investigations revealed optic disc drusen associated with a minimally classic choroidal neovascular membrane. The patient underwent a 3-month course of intravitreal ranibizumab. Six months of follow-up revealed remarkable visual acuity improvement to 20/20 and complete resolution of exudative maculopathy. [get full text]
Nasal Endoscopic Visualization and Management of the Leading Causes of Probing Failure
Ossama M. Hakim, FRCS; Walid Mandour, FRCS; Essam Elbaz, MSCSubmitted: 10/6/08; Accepted: 1/22/09; Posted: 8/21/09
Background: Conventional probing is a blind procedure with a variable success rate. This article evaluates the role of nasal endoscopy during probing to expose, explain, and manage the possible causes for probing failure.
Methods: In a prospective study, 169 eyes of 130 children (4 to 48 months old, median age = 1 to 6.2 months) with primary congenital nasolacrimal duct obstruction (NLDO) underwent probing under direct nasal endoscopic visualization. NLDO was diagnosed according to the patient’s history, clinical examination, and dye disappearance test. No patient had previously undergone lacrimal surgery. The main outcome measures were absence of tearing and discharge and negative dye disappearance test in the affected eye. Patients were observed at 1 week, 1 month, and 4 months postoperatively.
Results: Of the 169 obstructed ducts, 164 became patent after the first probing and 5 became patent after the second probing, with a total success rate of 100%. During probing, suspected causes for failure included thick membranous obstruction with false passage laterally, redundant membranous obstruction with trapdoor re-closure, stretchable membranous obstruction with elastic valve re-closure, and narrow terminal end of the duct with false passage medially. Such causes were predicted and managed intraoperatively.
Conclusion: Using nasal endoscopy during probing enables the surgeon to avoid the most likely causes for probing failure. Such intervention could increase the success rate of probing compared to probing without endoscopy. [get full text]
Complications and Visual Prognosis in Children With Aniridia
Helena Lee, MB; Katrina Meyers, MRCOphth; Bernadette Lanigan, MBA; Michael O’Keefe, FRCOphthSubmitted: 7/2/08; Accepted: 1/22/09; Posted: 8/21/09
Purpose: To characterize the ophthalmological findings, assess surgical outcomes, and review visual outcomes in aniridia.
Methods: A retrospective case review was performed and data were collected, including patient demographics, incidence of aniridia-associated keratopathy, glaucoma, cataract, retinal breaks or detachments, optic nerve hypoplasia, macular hypoplasia, poor vision, and nystagmus. All outcomes from surgery, including penetrating keratoplasty, trabeculectomy, Ahmed valve insertion, and cataract extraction, were recorded.
Results: Six children (12 eyes) had corneal abnormalities, 4 had optic nerve hypoplasia, 9 had nystagmus, and 2 had retinal detachments. Four patients (7 eyes) required penetrating keratoplasty. Five patients (9 eyes) developed glaucoma and only 1 of the 4 trabeculectomies performed succeeded. Of the 6 Ahmed valve procedures performed, all succeeded in maintaining a satisfactory intraocular pressure but some required needling and 5-fluorouracil. Eight patients developed cataract and 7 required surgery. Visual outcomes were poor despite treatment. Nine patients had Snellen acuity of 6/60 or less and required low visual aids to function.
Conclusion: Aniridia is a disorder that requires multiple surgeries. It has a poor visual prognosis despite early diagnosis and aggressive management. Newer techniques such as Ahmed valves and Boston keratoprostheses offer hope, but its proliferative nature makes treatment difficult. [get full text]
Absent Optic Chiasm Presenting With Horizontal Nystagmus
Daniel J. Salchow, MD; Jürgen Kohlhase, MD; Marijean Miller, MD; Nadja Kadom, MD; Edmond J. FitzGibbon, MD; Rafael C. Caruso, MD; Brian P. Brooks, MD, PhDSubmitted: 11/26/07; Accepted: 1/14/09; Posted: 8/21/09
A female infant with horizontal nystagmus and normal ophthalmic examination had isolated absence of the optic chiasm on magnetic resonance imaging. Eye movements were recorded on video and reviewed. Horizontal nystagmus without see-saw nystagmus was observed. Visual evoked potential showed inter-hemispheric asymmetry compatible with the absence of crossing chiasmal fibers. Systemic abnormalities in this patient included cleft lip, preauricular skin tags, broad thumbs, and an anteriorly positioned anus, suggestive of Townes–Brock syndrome. [get full text]
Visual Demands in Elementary School
Amy Langford, OD, FAAO; Timothy Hug, OD, FAAOSubmitted: 7/2/08; Accepted: 12/10/08; Posted: 8/21/09
Purpose: To evaluate the visual demands on elementary school students for both distance and near learning tasks. This information may be useful for prescribing glasses or amblyopia treatments such as occlusion therapy.
Methods: Classrooms for kindergarten to 5th grade were visited with consent from the school principal. Measurements of the vertical size of distance and near letters and the distances from the board to student desks were taken. A near visual working distance of 16 inches was assumed. The Snellen equivalent size of these target letters was calculated.
Results: Average visual acuity demands in these classrooms at distance ranged from 20/100 to 20/300 for children in grades kindergarten to 2 and from 20/60 to 20/100 for grades 3 to 5. The average near demand ranged from 20/100 to 20/500 for all grades at 16 inches.
Conclusion: Children with moderate to severe amblyopia may be able to visually perform in some classroom settings while undergoing occlusion therapy. Children with mild to moderate distance refractive errors may be able to perform in classroom activities without correction. A larger multi-school study may be indicated to provide more statistically significant data because visual demands may vary among different schools and school districts throughout the country. [get full text]
Vitrectomy in Cases of Nonaccidental Injury
Waheeda Rahman, MRCOphth; Sarah Osbourne, MRCOphth; Anna Bhan, FRCOphth; Gavin M. Orr, FRCOphth; Richard Gregson, DPhil, FRCS, FRCOphthSubmitted: 8/18/07; Accepted: 4/29/08; Posted: 7/22/09
Nonaccidental head injuries may present in several ways, requiring prompt recognition and management because of the risk of death or permanent neurologic and visual impairment. Dense amblyopia and high anisometropic myopia induced by prolonged occlusion of the macula by hemorrhage is a concern in these infants, although the prognosis also depends on the extent of associated brain injury. There are few reports of the visual and general outcome of children with a diagnosis of nonaccidental injury who have undergone vitrectomy. This report describes the outcome in six eyes of four patients diagnosed as having nonaccidental injury who underwent vitrectomy. The results are more favorable than in previous reports. [get full text]
Traumatic Rupture of the Superior Oblique Muscle Tendon Resulting in Acquired Brown’s Syndrome
Sunil Warrier, MBBS; Jane Wells, MBBS; Venkatesh C. Prabhakaran, MS, MRCOphth; Dinesh Selva, FRANZCOSubmitted: 9/9/07; Accepted: 4/29/08; Posted: 7/22/09
Traumatic rupture of the superior oblique tendon is a rare cause of acquired Brown’s syndrome. This report describes a case of traumatic rupture of the superior oblique tendon, distal to the trochlea, after injury with a plant hook. The ruptured tendon was reapproximated. Postoperatively, asymptomatic Brown’s syndrome developed, although residual function of the superior oblique muscle was retained. Full-thickness upper eyelid lacerations, especially those caused by a hook, must be explored carefully to exclude superior oblique tendon rupture. Careful reapproximation of the tendon can result in residual muscle function. [get full text]
The Emergence of Retinopathy of Prematurity in Guatemala
M. E. Sanchez, MD; B. J. Andrews, BS; D. Karr, MD; V. Lansingh, MD, PhD; Kevin L. Winthrop, MD, MPHSubmitted: 6/19/08; Accepted: 8/8/08; Posted: 7/22/09
Retinopathy of prematurity is an emerging cause of blindness in Latin America. In 2007, the authors examined 88 consecutive infants in Guatemala City, Guatemala, who met the screening criteria for retinopathy of prematurity; 14 (16%) had either “plus” or stage V disease. The incidence of retinopathy of prematurity in Guatemala City is likely high, and a formal screening program is warranted. [get full text]
Pradeep Sharma, MD, FAMS; Vivek Dave, MDSubmitted: 5/1/08; Accepted: 11/5/08; Posted: 7/22/09
Kabuki syndrome is a mental retardation–malformation syndrome affecting multiple organ systems. The typical facies resembles the make-up worn in Japanese Kabuki theater. Although there are several clinical findings, the ocular findings affecting vision have been underreported. The current patient shows esotropia and previously unreported nummular corneal opacities. [get full text]
Congenital Absence of Salivary and Lacrimal Glands Accompanied by Growth and Development Retardation
Faysal Gok, MD; Fatih Mehmet Mutlu, MD; Erkan Sari, MD; Erkan Demirkaya, MD; Halil Ibrahim Altinsoy, MD; Wollnik Bernd, MDSubmitted: 4/8/08; Accepted: 11/26/08; Posted: 7/22/09
Congenital absence or hypoplasia of some or all major salivary or lacrimal glands, nasolacrimal ducts, and puncta is a rare autosomal dominant disorder. Phenotypical expression may show great variability, and the diagnosis may be difficult. Only a few cases have been reported in the literature. This report describes a patient who has all of the essential features suggestive of aplasia of the lacrimal and salivary glands, as well as retardation of growth and development. Although the characteristic features of aplasia of the lacrimal and salivary glands have been described, an association with development and growth retardation has not been reported in the literature. [get full text]
Chorioretinal Lacunae: Pathognomonic Findings for Aicardi Syndrome
Silvana Guerriero, MD; Vittorio Sciruicchio, MD; Roberto De Blasi, MD; Claudio Furino, MD; Giuseppe Smaldone, MD; Lorenza Ciracì, MD; Dante Galeone, MDSubmitted: 11/15/08; Accepted: 12/10/08; Posted: 7/22/09
Aicardi syndrome is characterized by agenesis of the corpus callosum, chorioretinal lacunae, and infantile spasms. The authors describe the case of a girl with chorioretinal lacunae, seizures, and cerebral cyst, but a normal corpus callosum. Incomplete forms of Aicardi syndrome are reported in the literature, but typical choroidal lacunae are always present and are pathognomonic. [get full text]
Wenxin Wei, MD; Erica Zerfoss, BS; Lamees Ashker, MD; William A. Cantore, MDSubmitted: 9/3/08; Accepted: 12/10/08; Posted: 7/22/09
The authors report a case of a 16-year-old girl with a history of systemic lupus erythematosus who developed bilateral acute optic neuritis. Systemic lupus erythematosus can present with a vast array of neurological and ophthalmic complications, with optic neuritis being a rare but devastating manifestation and the major cause of blindness in these patients. The patient presented with an acute unilateral visual deficit that progressed to bilateral visual loss with no light perception over the course of days. Treatment included high-dose steroids, cyclophosphamide, intravenous immunoglobulin, and eventually rituximab. Furthermore, the patient was also seropositive for both antiphospholipid and neuromyelitis optica antibodies, which can have implications on prognosis and treatment options. [get full text]
Risk of Refractive Pathology After Spontaneously Regressed ROP in Emmetropic Patients
David G. Morrison, MD; Matthew Emanuel, BS; Sean P. Donahue, MD, PhDSubmitted: 6/11/08; Accepted: 12/10/08; Posted: 7/22/09
Purpose: To evaluate the development of refractive error abnormalities after a normal first examination in children with spontaneously regressed retinopathy of prematurity (ROP).
Methods: Two hundred twenty-six children with a history of spontaneously regressed ROP who had a cycloplegic refraction prior to 18 months of age were examined by a pediatric ophthalmologist. Children with a history of threshold ROP, strabismus, or a structural abnormality of the eye were excluded. A normal cycloplegic retinoscopy was defined as plano to less than +3.5 diopters of hypermetropia, less than +1.50 of symmetric astigmatism, and no anisometropia greater than 1.5 diopters in any meridian.
Results: Of the 226 children, 87 had a second examination and cycloplegic refraction at a mean of 2 years after the initial visit. In 63 of the children, the second examination and refraction remained normal. Three (3.4%) children had significant refractive error and were treated with spectacles: two had anisometropia and one had myopic astigmatism. Twenty-one had symmetric low magnitude myopia that did not warrant treatment.
Conclusion: Children with spontaneously regressed ROP and no significant refractive error on cycloplegic retinoscopy at first follow-up examination have a risk of developing ametropia that is similar to that of the general population. Such children can likely be observed with a verbal vision screening in the pediatrician’s office at 3 to 4 years of age. [get full text]
Ufuk Elgin, MD; Nilufer Berker, MD; Mehmet Yasin Teke, MD; Tulay Simsek, MD; Pinar Ozdal, MDSubmitted: 7/16/08; Accepted: 1/22/09; Posted: 7/22/09
A 12-year-old girl with neurofibromatosis type 1 was referred for pain and blurred vision in her right eye for the past 2 weeks. Neovascular glaucoma associated with peripheral retinal ischemia was diagnosed and she was treated with retinal photocoagulation after intravitreal bevacizumab injection and trabeculectomy. [get full text]
Usher Syndrome Associated With a Variant of Dandy–Walker Malformation
Tulay Simsek, MD; Yasemin Ozdamar, MD; Enver Simsek, MD; Gamze Men, MDSubmitted: 8/10/08; Accepted: 1/23/09; Posted: 7/22/09
Three cases of Usher syndrome associated with a variant of Dandy–Walker malformation in three siblings from consanguineous Turkish parents are described. The siblings had retinitis pigmentosa and hearing loss. Two of the siblings also had mental retardation, which is not a constant finding in Usher syndrome. Dandy–Walker malformation might have contributed to the mental retardation in two of these patients and might be a coincidental finding with Usher syndrome. [get full text]
Orbital Dermoid Cyst Presenting With a Discharging Sinus Tract
Alexandra M. Tien, MD; David Robbins Tien, MDSubmitted: 3/19/08; Accepted: 11/5/08; Posted: 6/23/09
This report describes a case of orbital dermoid cyst presenting with a discharging sinus tract. The embryologic basis for orbital dermoids in general is discussed, as well as their typical presentation and management. In the current case, patient presentation and management were somewhat different because of signs of infection and a communicating sinus tract, both highly unusual for orbital dermoids. However, after complete excision of the dermoid and sinus tract, the patient showed complete recovery.
doi: 10.3928/01913913-20090616-18 [get full text]
Parag K. Shah, DNB; Venkatapathy Narendran, DNB; Narendran Kalpana, DNBSubmitted: 8/24/08; Accepted: 11/26/08; Posted: 6/23/09
This report describes a case of macular pucker after triple freeze–thaw cryotherapy for retinoblastoma. Seven months after cryotherapy, the macular pucker resolved spontaneously.
doi: 10.3928/01913913-20090616-16 [get full text]
Intramuscular Bupivacaine Injection for the Treatment of Oculomotor Paresis
Olga Seijas, MD; Pilar Gómez de Liaño, PhD; Pilar Merino, PhDSubmitted: 4/12/08; Accepted: 11/12/08; Posted: 6/23/09
The main goal of this study is to examine the effect of intramuscular bupivacaine in oculomotor paresis, analyzing whether it is possible to obtain a stronger muscle contraction due to the muscle hypertrophy caused by the drug. An injection of 4.5 mL of a 0.50% solution of bupivacaine was administered in the paretic muscle of three patients. Magnetic resonance imaging was performed before and 3 months after injection to compare muscle cross-sectional areas. The symptoms of two patients improved and an increase of muscle cross-sectional area was observed. However, it is necessary to be prudent when employing intramuscular bupivacaine in oculomotor paresis treatment until there are more and larger studies.
doi: 10.3928/01913913-20090616-15 [get full text]
Unique Ocular Findings in an Infant With Pierson (Microcoria–Congenital Nephrosis) Syndrome
Taylor Sawyer, DO; David Young, MD; John Drouilhet, MD; Laurie Seaver, MD; Sherry Loo, MDSubmitted: 12/5/07; Accepted: 9/3/08; Posted: 6/23/09
Pierson syndrome is a recently described autosomal recessive genetic disorder that presents in the neonatal period with bilateral microcoria and congenital nephrotic syndrome. This report describes a case of Pierson syndrome with an emphasis on the ocular findings.
doi: 10.3928/01913913-20090616-14 [get full text]
D-dimer Evaluation of Hemorrhagic Orbital Lymphangioma
Elena Rizzardi, MD; Thea Tagliaferro, MD; Deborah Snijders, MD; Federica Bertuola, MD; Federica Spolaore, MD; Paolo Simioni, MD; Pierpaolo Maimone, MD; Luisa Pinello, MD; Monica Mazzarolo, PhD; Angelo Barbato, MDSubmitted: 5/27/08; Accepted: 11/5/08; Posted: 6/23/09
An 8-year-old boy with recurrent acute bleeding of lymphangioma of the left orbit is described. D-dimer levels increased as the size of the mass became stable, showing the effect of fibrinolysis within the hemorrhagic mass after clotting. D-dimer levels confirmed the possible use of conservative management of this lymphangioma.
doi: 10.3928/01913913-20090616-13 [get full text]
Carbon Monoxide Poisoning: A Patient With Bilateral Internuclear Ophthalmoplegia and Exotropia
Mohammad Etezad Razavi, MD; M. Khalife, MDSubmitted: 6/12/08; Accepted: 9/24/08; Posted: 6/23/09
Carbon monoxide poisoning, which occurs in poorly ventilated areas, is associated with different symptoms and signs. This report describes an 8-year-old girl with severe carbon monoxide intoxication, scattered retinal nerve fiber layer and subhyaloid hemorrhages, and exotropia with bilateral internuclear ophthalmoplegia.
doi: 10.3928/01913913-20090616-12 [get full text]
Congenital Anterior Staphyloma
Patrícia Lunardelli, MD; Suzana Matayoshi, PhDSubmitted: 7/21/08; Accepted: 9/24/08; Posted: 6/23/09
Staphyloma in the anterior segment of the eye is a rare abnormality of development. Histopathologic and electron microscopic studies suggest that the alteration occurs during embryogenesis. This report describes a 1-year-old girl with protrusion of the cornea between the eyelids in the left eye. Surgical removal of the staphyloma was performed, with reconstruction of the eyeball and total corneoscleral transplant. The clinical and histopathologic findings were typical of congenital anterior staphyloma. Although most affected patients are treated with enucleation, corneoscleral transplant provides a good cosmetic option in these cases.
doi: 10.3928/01913913-20090616-09 [get full text]
Presumed Atypical HDR Syndrome Associated With Band Keratopathy and Pigmentary Retinopathy
Cinoo Kim, MD; Hae Il Cheong, MD; Jeong Hun Kim, MD; Young Suk Yu, MD; Ji Won Kwon, MDSubmitted: 4/9/08; Accepted: 8/8/08; Posted: 6/23/09
This report describes presumed atypical hypoparathyroidism, deafness, and renal dysplasia (HDR) syndrome associated with unexpected ocular findings. The patient had exotropia, bilateral band keratopathy, and pigmentary retinopathy, including attenuated retinal vessels and atrophy of the retinal pigment epithelium. Even though the calcific plaques were successfully removed, visual acuity in both eyes gradually decreased and electroretinography was extinguished.
doi: 10.3928/01913913-20090616-08 [get full text]
Arif O. Khan, MD; Faisal E. Ghadfan, MDSubmitted: 8/11/08; Accepted: 11/26/08; Posted: 6/23/09
A 10-month-old child with unilateral visually insignificant anterior polar lens opacity was examined 6 years after initial presentation and found to have complete white cataract and apparent focal disruption of the anterior capsule in the affected eye. Although anterior lens opacities are generally static and visually insignificant, progression can occur.
doi: 10.3928/01913913-20090616-07 [get full text]
Relapse of Acute Lymphoblastic Leukemia as a Conjunctival Mass in a 9-Year-Old Girl
Mohammad-Ali Javadi, MD; Mozhgan Rezaei Kanavi, MD; Amir Faramarzi, MD; Nima Naghshgar, MD; Firooz Mirbabaei, MD; Asghar Ramyar, MDSubmitted: 8/7/08; Accepted: 11/12/08; Posted: 6/23/09
A 9-year-old girl with a history of acute lymphoblastic leukemia in remission presented with a right subconjunctival mass and ipsilateral preauricular lymphadenopathy despite normal findings on blood cell profile. Excisional biopsy of the lesion was performed to exclude extramedullary relapse of acute lymphoblastic leukemia. Histopathologic examination showed infiltration of leukemic cells at the conjunctival substantia propria. The patient was referred to her oncologist, and bone marrow aspiration showed medullary relapse of acute lymphoblastic leukemia for which systemic and intrathecal chemotherapy was administered. Leukemic infiltration of the conjunctiva in the presence of normal findings on blood cell profile can be a rare manifestation of relapsed acute lymphoblastic leukemia.
doi: 10.3928/01913913-20090616-06 [get full text]
Retinal Vasoproliferative Tumor in Association With Neurofibromatosis Type 1
Christopher T. Hood, MD; Linda Janku, COA; Careen Y. Lowder, MD, PhD; Arun D. Singh, MDSubmitted: 7/17/08; Accepted: 9/24/08; Posted: 6/23/09
Retinal vasoproliferative tumor is an uncommon benign vascular tumor that most often occurs sporadically. Vision loss can occur secondary to retinal fibrosis, subretinal exudation, and neovascular glaucoma. This report describes a functionally monocular patient with neurofibromatosis type 1 who had a vasoproliferative tumor with neovascularization of the iris that was successfully treated with intravitreal bevacizumab and cryotherapy.
doi: 10.3928/01913913-20090616-05 [get full text]
Cholesterolosis Bulbi of the Anterior Chamber in Coats Disease
Noopur Gupta, MS, DNB; Sarita Beri, MD; Pamela D'Souza, MSSubmitted: 2/27/08; Accepted: 10/29/08; Posted: 6/23/09
Coats disease is characterized by the presence of cholesterol crystals and lipid-laden macrophages in the subretinal space. These cholesterol crystals may rarely be found in the anterior chamber in Coats disease, a phenomenon known as cholesterolosis bulbi. This report describes a patient with advanced Coats disease who presented with anterior chamber cholesterolosis.
doi: 10.3928/01913913-20090616-04 [get full text]
Molluscum Contagiosum–Induced Periocular Toxic Dermatitis
Yajati K. Ghosh, FRCSEd, FRCSOphth; M. Freeth, MRCPath; S. Sandramouli, FRCS, FRCOphthSubmitted: 9/5/08; Accepted: 11/26/08; ; Posted: 6/23/09
This report describes an unusual case of a 9-year-old boy with a 6-month history of right lower eyelid lesion and excoriation of the adjoining skin that had not responded to topical treatment. The umbilicated lesion was electively excised and the base of the lesion was cauterized under general anesthesia, which resulted in complete resolution of the periocular dermatitis without additional treatment. It is important to consider molluscum contagiosum in the differential diagnosis in patients with periocular dermatitis, especially in the presence of an umbilicated vesicle, although it is classically taught that these lesions present with toxic conjunctivitis.
doi: 10.3928/01913913-20090616-03 [get full text]
Bilateral Optic Nerve Aplasia in a Child
Hidayet Erdol, MD; Mehmet Kola, MD; Adem Turk, MDSubmitted: 8/30/08; Accepted: 11/5/08; ; Posted: 6/23/09
An 3-month-old female infant was admitted to the authors’ clinic because of abnormal gaze position. On examination, the eyes were in the down gaze position. The left eye had a partial iris and lens coloboma. On funduscopic examination, the optic nerve and retinal vessels could not be detected. Optic nerve and optic chiasm were not observed on magnetic resonance imaging.
doi: 10.3928/01913913-20090616-02 [get full text]
Multiple Evanescent White Dot Syndrome After Vaccination for Human Papilloma Virus and Meningococcus
Steven M. Cohen, MDSubmitted: 7/10/08; Accepted: 10/29/08; Posted: 6/23/09
Multiple evanescent white dot syndrome is a rare chorioretinopathy causing transitory vision loss, usually in females and generally in one eye. In 2007, widespread vaccination of older children against human papilloma virus and meningococcus was recommended by the Centers for Disease Control and Prevention. A 17-year-old girl presented with multiple evanescent white dot syndrome 1 month after receiving these two vaccinations.
doi: 10.3928/01913913-20090616-01 [get full text]
Sawako Matsuno, MD; Ikue Takagi, MDSubmitted: 1/24/08; Accepted: 7/21/08; Posted: 6/23/09
The authors successfully assessed the lacrimal systems of two patients with dacryocystocele based on dacryocystographic observations using computed tomographic scanning. The results showed that both the lacrimal sac and the nasolacrimal duct were largely distended, and the duct terminated in a closed cyst in the nasal cavity.
doi: 10.3928/01913913-20090616-10 [get full text]
Self-Limiting Fourth and Sixth Cranial Nerve Palsy After Lyme Disease
Vinod Sharma, MRCOphth, FRCS (Edin); Susmito Biswas, FRCOphthSubmitted: 03/07/08; Accepted: 06/24/08; Posted: 6/23/09
Lyme disease, or borreliosis, is caused by a bacteria (Borrelia burgdorferi sensu lato and is transmitted to humans by the bite of ticks of the Ixodes genus. The authors describe a 16-year-old girl who presented with extraocular muscle palsies and had serology positive for Lyme disease. The muscle palsies resolved after antibiotic treatment with no residual limitation of ocular movements.
doi: 10.3928/01913913-20090616-17 [get full text]
Narman Puvanachandra, MBBChir MA(Hons), FRCOphth; Maryam Aroichane, MD, FRCSCSubmitted: 4/5/08; Accepted: 5/6/08; Posted: 6/23/09
This report describes a 7-year-old boy with multiple endocrine neoplasia type 2B who presented to the ophthalmology department because of peripheral corneal changes. In addition to prominent corneal nerves and eyelid neuromas, the patient was found to have substantial corneoscleral limbal thickening with corneal neovascularization.
doi: 10.3928/01913913-20090616-11 [get full text]
Ziaeddin Yazdian, MD; Mohammad Taher Rajabi, MD; Mohammad Ali Yazdian, MD; Mohammad Bagher Rajabi, MD; Mohammad Reza Akbari, MDSubmitted: 3/22/08; Accepted: 11/26/08; Posted: 6/23/09
Purpose: To report the clinical outcome and complications of the Scott Foster procedure for treating abduction deficiency in patients with Duane’s syndrome type 1 and sixth nerve palsy.
Methods: A retrospective, interventional case series included 62 consecutive patients (62 eyes: 38 eyes with Duane’s syndrome and 24 eyes with sixth nerve palsy) who underwent the Scott Foster procedure for treatment of abduction deficiency. The main outcome measures were deviation, face turn, and abduction deficiency.
Results: In patients with sixth nerve palsy, mean distance deviation improved from 44.7 ± 7.2 prism diopters (PD) before surgery to 12.5 ± 4.0 PD after surgery (P < .05), and in patients with Duane’s syndrome type 1, mean distance deviation improved from 31.5 ± 4.3 PD preoperatively to 9.2 ± 3.1 PD. Mean near deviation improved from 27.1 ± 4.5 PD to 6.2 ± 2.5 PD in patients with Duane’s syndrome and from 40.3 ± 6.2 PD to 13.7 ± 3.4 PD in patients with sixth nerve palsy. Mean abduction deficiency improved from -4 to -2 (P < .05) in both groups. Face turn improved significantly after surgery. No patient had an overcorrection. No recurrence was detected.
Conclusion: The Scott Foster procedure is effective for improving deviation, abduction deficiency, and face turn in patients with Duane’s syndrome type 1 and especially in those with sixth nerve palsy.
doi: 10.3928/01913913-20090616-19 [get full text]
Conformance With Preferred Practice Patterns in Caring for Children With Esotropia
Preeya K. Gupta, MD; Sharon F. Freedman, MD; Paul P. Lee, MD, JDSubmitted: 7/30/08; Accepted: 11/26/08; Posted: 5/20/09
Purpose: To evaluate documentation of the initial evaluation in patients with esotropia, with respect to history, physical examination, care management, and patient education, for the presence of key elements as defined in the American Academy of Ophthalmology’s Summary Benchmarks for Preferred Practice Patterns.
Methods: Two hundred charts of patients seen at the Pediatrics Service at the Duke Eye Center for an initial diagnosis of esotropia were reviewed relative to both the 2002 (all patients) and 2007 (for those seen in 2007) Preferred Practice Patterns benchmarks for esotropia. Documentation of key elements as defined in the Preferred Practice Patterns was recorded.
Results: The average total score was 85.8% for all evaluations. On average, 63.6% of the initial history key elements, 99.6% of the initial physical examination key elements, and 94.2% of the care management and patient education key elements were documented. For evaluations performed in 2007 and scored according to the 2007 Preferred Practice Patterns, scores were similar, except that the physical examination subset score average decreased to 90.3% and the family history documentation score increased to 45.5%.
Conclusion: In an academic practice, the process quality of care for strabismus often conforms well to professional practice patterns. However, there are opportunities for improvement in the documentation of key history elements. Additional data are needed from other practice settings and for other conditions to assess the quality of eye care for children. [get full text]
Congenital Ptosis and Amblyopia
Yesim Oral, MD; Ozlen Rodop Ozgur, MD; Levent Akcay, MD; Mehmet Ozbas, MD; Omer Kamil Dogan, MDSubmitted: 4/15/08; Accepted: 11/26/08; Posted: 5/20/09
Purpose: To investigate the incidence and causes of amblyopia in congenital ptosis.
Methods: The frequency and causes of amblyopia were evaluated in 83 eyes of 72 patients with congenital ptosis. Patient ages ranged between 10 months and 70 years. Amblyopia was defined as best-corrected visual acuity of 0.7 or less. After a complete ophthalmologic examination, the amount of ptosis and levator function was determined and the following amblyogenic factors were identified: ametropia, anisometropia, and strabismus. In cases with no other reason for amblyopia, stimulus deprivation amblyopia due to ptosis was assumed.
Results: The amount of ptosis was mild in 5 (6%), moderate in 26 (31%), and severe in 52 (63%) of 83 eyes with congenital ptosis. Strabismus was present in 17 (20%) eyes. Visual acuity could not be determined in 10 of 83 eyes with ptosis in children younger than 4 years. The overall incidence of amblyopia in the remaining 73 eyes was 48%. Of those patients with amblyopia, 54% had refactive errors such as anisometropia or ametropia, 20% had strabismus with or without refractive errors, and 26% had amblyopia solely attributed to ptosis. When all eyes with ptosis were included, the rate of stimulus deprivation amblyopia was 12% and all of these eyes had severe ptosis.
Conclusion: Early detection and treatment of amblyogenic factors such as refractive errors and strabismus is recommended and, when the pupillary axis is occluded with severe ptotic eyelids, surgery should be performed as soon as possible to prevent stimulus deprivation amblyopia. [get full text]
A New Muscle Transposition Procedure to Correct Cyclodeviation Without Tenotomy
Sanae Muraki, MD; Yasuhiro Nishida, MD; Yuri Harada, MD; Masashi Kakinoki, MD; Osamu Sawada, MD; Kenichi Yoshida, MD; Masahito Ohji, MDSubmitted: 12/4/07; Accepted: 8/8/08; Posted: 5/20/09
The authors developed a new surgical procedure for use in treating pure cyclodeviation. This procedure was accomplished by a simple muscle transposition technique without tenotomy. Satisfactory postoperative results were achieved without postoperative complications. This procedure may be useful for treating cyclodeviations, with minimal risk of surgical complications, particularly anterior segment ischemia. [get full text]
Bilateral Primary Congenital Glaucoma in Monozygotic Twins
Itay Ben-Zion, MD; Ameha Bogale, MD; Daniel B. Moore, MD; Eugene M. Helveston, MDSubmitted: 5/21/08; Accepted: 6/16/08; Posted: 5/20/09
Primary congenital glaucoma is an uncommon pediatric ocular disease contributing to a disproportionately high percentage of childhood blindness worldwide. This case report describes bilateral primary congenital glaucoma in monozygotic twins who first presented to an ophthalmologist in Ethiopia at 6 years of age. Both had severe, protracted disease, with only moderate improvement after surgical intervention. [get full text]
Usha K. Raina, MD, FRCS, FRCOph; Zia Chaudhuri, MS, MNAMS, FRCS (Glasg)Submitted: 12/20/07; Accepted: 6/16/08; Posted: 5/20/09
This report describes an unusual case of a neonate with giant congenital melanocytic nevi covering more than 80% of the body surface area, with cutis verticis gyrata and abnormal lacrimal glandular tissue rests in the epibulbar area. The patient’s mother had pigmented lesions in the periorbital region and sclera. [get full text]
Linear Nevus Sebaceous Syndrome in a Patient With Atypical Associated Abnormalities
Haotian Lin, MD, PhD; Jianhua Yan, MD, PhDSubmitted: 10/13/07; Accepted: 6/11/08; Posted: 5/20/09
A 3-year-old girl was diagnosed as having linear nevus sebaceous syndrome. The nevus sebaceous was located on the left side of the face and neck. An unusually large lacrimal gland was completely ectopic below the entire left conjunctiva, with secondary ectropion of the left upper eyelid. The patient had the unusual finding of dentigerous cysts in both maxillary sinuses. Computed tomography scan showed atrophy of the left cerebral hemisphere, accompanied by a history of seizures; furthermore, the patient was mentally retarded. This phenotypic pattern of linear nevus sebaceous syndrome is typical for patients with multiple associated abnormalities. [get full text]
Francesco Viola, MD; Edoardo Villani, MD; Chiara Mapelli, MD; Giovanni Staurenghi, MD; Roberto Ratiglia, MDSubmitted: 11/8/07; Accepted: 5/21/08; Posted: 5/20/09
The authors recently observed a boy with bilateral choroidal neovascularization associated with Best’s disease and compared the outcome of photodynamic therapy in one eye with the natural history of the untreated fellow eye. The final outcome of photodynamic therapy was inactive choroidal neovascularization, which seemed to be enveloped by the retinal pigment epithelium. The final appearance of the treated lesion was similar to the natural evolution of choroidal neovascularization in the untreated fellow eye. [get full text]
Strabismus Fixus Associated With Pierre Robin Sequence
Pranab Das, MD; Sushmita Kaushik, MD; Parul Ichhpujani, MD; Jaspreet Sukhija, MDSubmitted: 2/27/08; Accepted: 5/6/08; Posted: 5/20/09
This report describes an unusual case of Pierre Robin sequence with strabismus fixus in a 7-month-old male infant. Cosmetic and functional improvement was achieved with bilateral complete medial rectus muscle disinsertion. [get full text]
Silay Canturk, MD; Yonca Akova, MD; Veysi Oner, MDSubmitted: 1/8/08; Accepted: 4/29/08; Posted: 5/20/09
This study evaluated the outcome of corneal surface reconstruction with conjunctival limbal autograft and amniotic membrane transplantation in two children with unilateral ocular chemical burn who underwent surgery. Visual acuity improved from counting fingers at face to 20/60 in one patient and from counting fingers at 30 cm to 20/100 in the other. Both patients received simultaneous symblepharon lysis and regained deep fornices. When used in combination with amniotic membrane, conjunctival autograft allows correction of cicatricial abnormalities and early restoration of corneal surface integrity. Intervention within 6 months is desirable for rehabilitation of vision in the amblyogenic period. [get full text]
Nikolaos Mavrakanas, MD; James S. Schutz, MD; André A. Dosso, MDSubmitted: 10/9/07; Accepted: 4/29/08; Posted: 5/20/09
A case of severe pediatric ocular rosacea was effectively treated after 2.5 years of misdiagnosis. A high index of suspicion should be maintained in children with ocular surface disease, with or without dermatologic rosacea, to correctly diagnose ocular rosacea and avoid morbidity and complications. [get full text]
Spontaneous Bilateral Anterior Lens Dislocation in an 8-Year-Old Girl
Shatriah Ismail, MD, MMed; Sonny-Teo Khairy-Shamel, MD; Adil Hussein, MD, MMed; Bakiah Shaharuddin, MD, MMed; Zunaina Embong, MD, MMed; Mohtar Ibrahim, MD, MSurgSubmitted: 3/14/08; Accepted: 4/24/08; Posted: 5/20/09
The authors describe recurrent lens dislocation into the anterior chamber in a young girl with homocystinuria. The patient’s 12-year-old sister died after a cerebrovascular accident caused by the same disease. This report highlights the importance of early dietary restrictions and treatment. [get full text]
Severe Ocular Injuries from Improperly disposed Medical Syringes in Children
Ashraf K. Al-Hussaini, MD; Tarek A. Shazly, MDSubmitted: 12/9/07; Accepted: 4/8/08; Posted: 5/20/09
This report describes ocular injuries caused by syringes in children. The records of 12 children injured with syringes were reviewed. There were seven cases of endophthalmitis, two cases of panophthalmitis, one case of retinal detachment, one case of endophthalmitis with retinal detachment, and one case of traumatic cataract. All patients received treatment within 24 hours of presentation. Patients presented an average of 8.4 days after injury. Final visual acuity ranged from no light perception to counting fingers at 90 cm in eight patients and could not be assessed in four patients. Improperly disposed syringes may pose a significant risk for ocular injury and visual loss in children, especially when presentation is delayed. [get full text]
Diagnosis and Pathogenesis of Congenital X-Linked Retinoschisis With Optical Coherence Tomography
Sumit Dhingra, MRCOphth; C. K. Patel, FRCOphthSubmitted: 1/7/08; Accepted: 4/8/08; Posted: 4/20/09
This study shows how optical coherence tomography has been valuable in the diagnosis of congenital X-linked retinoschisis. An infant with convergent strabismus underwent serial optical coherence tomography examinations that helped to make the diagnosis of congenital X-linked retinoschisis. Optical coherence tomography is useful in the diagnosis of congenital X-linked retinoschisis and adds new information about pathogenesis. The authors recommend the use of optical coherence tomography in pediatric retinal disease, even if the macula appears normal on indirect ophthalmoscopy. [get full text]
Narman Puvanachandra, MBBChir MA(Hons), FRCOphth; Sameer Trikha, BM BSc(Hons), MRCOphth; Caroline J. MacEwen, MD, FRCS, FRCOphth; Robert J. Morris, BSc(Hons), MRCP, FRCS, FRCOphth; Peter R. Hodgkins, BSc (Hons), FRCS, FRCOphthSubmitted: 7/26/08; Accepted: 11/12/08; Posted: 4/20/09
Purpose: To survey national variation in the management of congenital nasolacrimal duct obstruction, particularly the timing of intervention and the use of nasolacrimal intubation, nasal endoscopy, and assistance from an ear, nose, and throat surgeon at different stages of management.
Methods: A telephone survey was conducted of 100 ophthalmologists in the United Kingdom who were involved in the management of congenital nasolacrimal duct obstruction. A sequential management protocol was established for each, including the nature of procedures, their timing, and the use of nasal endoscopy and ear, nose, and throat surgeons.
Results: Of those surveyed, 49% use the dye disappearance test for diagnosis. Eighty-four percent suggest lacrimal sac massage to parents. No surveyed ophthalmologists perform “office” probing or balloon dilation. Seventy-four percent perform initial probing after 1 year, with 25% using nasal endoscopy. If symptoms persist, 64.5% (60 of 93) repeat the probing, whereas 35.5% (33 of 93) intubate the lacrimal system. The use of nasal endoscopy increases to 50.5% (47 of 93). By the third intervention, 77.6% (45 of 58) perform lacrimal intubation, with 72.4% (42 of 58) using nasal endoscopy. All opt for dacryocystorhinostomy as the fourth intervention and 28.3% (13 of 46) perform this procedure themselves, whereas 71.7% (33 of 46) refer the patient to another practitioner. In total, 65% (65 of 100) use tubes at some stage of management, 58% (58 of 100) make some use of nasal endoscopy, and 33% (33 of 100) involve ear, nose, and throat surgeons.
Conclusion: Based on the results of this survey, huge variation exists in the management of congenital nasolacrimal duct obstruction in the United Kingdom. Most ophthalmologists intervene soon after patients reach 1 year of age. The rate of nasal endoscopy increases with successive interventions, especially to aid with nasal intubation, either alone or with the assistance of ear, nose, and throat surgeons. [get full text]
Confluent Laser Photocoagulation for the Treatment of Retinopathy of Prematurity
Victor H. Gonzalez, MD; Gian P. Giuliari, MD; Rodolfo M. Banda, MD; David A. Guell, BA; Michael Wingard, ODSubmitted: 2/17/08; Accepted: 10/29/08; Posted: 4/20/09
Purpose: To describe the efficacy, outcomes, and complications of confluent laser technique in a cohort of premature infants with threshold retinopathy of prematurity. Laser photocoagulation has a good treatment outcome in the management of retinopathy of prematurity; however, the number and density of laser spots remains debatable. Laser treatment can be done in a scattered pattern, a near-confluent pattern, or a confluent treatment pattern.
Methods: A retrospective chart review of patients with threshold retinopathy of prematurity treated between 2003 and 2006 was conducted. Confluent laser treatment was applied anterior to the ridge extending to the ora serrata 360°. Rate of progression, frequency of retreatment, postoperative complications, structural outcomes, and refractive error were evaluated.
Results: A total of 100 eyes from 51 patients were included. Mean gestational age was 28 weeks (range: 23 to 32 weeks) and mean birth weight was 1,065 g (range: 477 to 1,905 g). Patients had a mean follow-up of 13 months (range: 6 to 50 months). Progression to stage 4 or 5 occurred only in a total of 6 eyes (6%). Postoperative complications included cataract, vitreous hemorrhage, corneal edema, and macular dragging. Mean spherical equivalent at the last follow-up visit was -3.80 diopters (range: -19.00 to + 4.00 diopters).
Conclusion: Patients treated with confluent laser photocoagulation had a low rate of progression to stage 4 or 5 retinopathy of prematurity. Also, the need for additional laser treatment was small, with rates of complications and structural outcomes comparable to previous reports using a nonconfluent laser pattern. [get full text]
Intraocular Teratoma in Association With Sacrococcygeal Teratoma: A Clinicopathologic Report
Shahar Frenkel, MD, PhD; Michael T. Trese, MD; Mark K. Walsh, MD, PhD; Edward R. O'Malley, MD; Janet M. Poulik, MD; David H. Abramson, MD, FACS; Robert Folberg, MDSubmitted: 5/22/08; Accepted: 7/3/08; Posted: 4/20/09
A benign sacrococcygeal teratoma was resected from an infant at birth. Four weeks later, intraocular tumors were identified in the right eye. The eye expanded in size and was removed at 16 weeks. A benign cystic intraocular teratoma was identified. [get full text]
Major Pediatric Ocular Trauma in Taiwan
Mei-Ling Liu, MD; Yi-Sheng Chang, MD, PhD; Sung-Huei Tseng, MD; Hon-Chun Cheng, MD; Fu-Chin Huang, MD; Min-Hsiu Shih, MD; Sheng-Min Hsu, MD; Po-Hsiu Kuo, PhDSubmitted: 3/28/08; Accepted: 9/3/2008; Posted: 3/20/09
Purpose: To investigate major pediatric ocular trauma in Taiwan.
Methods: Retrospective review of medical records of all patients 15 years and younger who were hospitalized with a primary diagnosis of eye injury at National Cheng Kung University Hospital, Taiwan, between June 1988 and May 2006.
Results: There were 156 children (156 eyes) 1.1 to 15.0 years (mean ± standard deviation, 7.1 ± 0.3 years; boy:girl ratio: 2.1:1). Objects most often causing penetrating injury were scissors (13.5%), pencils/pens (12.2%), broken eyeglasses/spectacles (7.7%), and knives (6.4%). Most blunt trauma occurred in traffic accidents (5.8%). Most injuries occurred at home, followed by on the street, at school, and at sports venues. Injuries were classified as open globe (71.2%), adnexal only (18.6%), or closed globe (10.3%), and included corneal laceration (40.4%), lens damage (27.6%), hyphema (25.6%), and eyelid laceration (23.7%). Most surgical procedures were primary repair (88.5%) or removal of a damaged lens (22.4%). Additional surgery was performed in 19.9% of cases. After treatment, 56.4% of eyes had corneal opacity/scar and 7.1% became phthitic; 52.6% had good visual outcome, whereas 23.1% had poor final vision. Compared with visual acuity measured on admission, final visual acuity was improved in 76.1%, unchanged in 19.7%, and worse in 4.3%. Predictors of worse outcome were open-globe injury and larger wound size, posterior segment involvement, and presence of an intraocular foreign body.
Conclusions: Most of the children hospitalized for major ocular trauma are younger boys with penetrating injuries suffered at home. Most injuries could have been prevented by increased awareness and reduction of risk factors, and the authors urge better public education for improved safety. [get full text]
Alexander Demidenko, DO; Frederick A. Jakobiec, MD; Eissa Hanna, MD; David S. Walton, MDSubmitted: 3/31/08; Accepted: 6/16/08; Posted: 3/20/09
A 10-year-old boy with clinically confirmed congenital pupillary-iris-lens membrane with goniodysgenesis underwent light microscopic and immunolocalization examinations of the enucleated eye. The anterior segment changes consisted of the absence of the iridial pupillary sphincter muscle and dilator muscle processes. Endothelialization and descemetization of the anterior chamber angle and the anterior surface of the iris covered the original eccentric pupillary opening (occlusio pupillae), but grew around the fibrotic edges of the surgically created, patent pseudopupil. The anterior chamber findings in this case establish a localized syndrome that includes absence of the pupillary sphincter and dilator muscle processes. Incomplete development of the iris may be partly attributable to an abnormality of stromal development and inductions of the anterior neuroectodermal layer, together with anomalies of the pupillo-iridial vasculature. [get full text]
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