Publications by authors named "Iason S Mantagos"

26 Publications

  • Page 1 of 1

Retinopathy of prematurity screening and risk mitigation during the COVID-19 pandemic.

J AAPOS 2021 Apr 18. Epub 2021 Apr 18.

Boston Children's Hospital, Boston, Massachusetts; Harvard Medical School, Boston, Massachusetts.

Background: The coronavirus disease 2019 (COVID-19) pandemic has significantly disrupted the delivery of healthcare. Although most nonurgent ophthalmology visits at Boston Children's Hospital were canceled, premature infants at risk for retinopathy of prematurity (ROP) still required timely, in-person care during the initial 3-month period of the infection surge in Massachusetts. The purpose of the current study was to report our protocols for mitigating risk of exposure to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) between infants and eye care providers and to compare examination rates and results with the same 3-month period in 2019.

Methods: During the infection surge, we added new infection control measures and strengthened existing ones. Additional personal protective equipment was used, and the number of ophthalmologists rotating in the three high-capacity NICUs we service was limited.

Results: More infants required ROP examinations during the study period in 2020 than in the same period in 2019, but fewer examinations were performed. There were no cases of missed progression to severe ROP during this time and no known transmission of SARS-CoV-2 between ROP patients and ophthalmology staff.

Conclusions: Overall, effective ROP care was safely provided during the COVID-19 pandemic, and contact with this vulnerable population was minimized.
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http://dx.doi.org/10.1016/j.jaapos.2020.11.015DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8053364PMC
April 2021

Charting the Globe: How Technologies Have Affected Our Understanding of Retinal Findings in Abusive Head Trauma/Shaken Baby Syndrome.

Semin Ophthalmol 2021 May 1;36(4):205-209. Epub 2021 Apr 1.

Department of Ophthalmology, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts, USA.

Ocular findings such as retinal hemorrhages are common in abusive head trauma (AHT). Binocular indirect ophthalmoscopy has been the standard for assessing the eyes of children who are victims of AHT. However, technological advances have changed our understanding of retinal findings in AHT. Literature review on AHT - retinal findings, imaging technologies, models of representation, and telemedicine applications. Many studies suggest vitreoretinal traction from repetitive acceleration-deceleration shearing forces during shaking plays an important role in the development of retinal findings in AHT. This is further supported by different imaging modalities [optical coherence tomography (OCT); magnetic resonance imaging (MRI); fluorescein angiography (FA)] and models of representation (animal and mechanical models; finite element analysis). Emerging technologies have augmented our diagnostic abilities, enhanced our understanding regarding the pathophysiology of retinal findings, and strengthened the link between vitreoretinal traction and ocular pathology in AHT. Telemedicine is also starting to play an important role in AHT.
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http://dx.doi.org/10.1080/08820538.2021.1890150DOI Listing
May 2021

Amniotic membrane transplantation in a 2-month-old infant with toxic epidermal necrolysis.

Am J Ophthalmol Case Rep 2021 Mar 15;21:101017. Epub 2021 Jan 15.

Department of Ophthalmology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.

Purpose: To report a case of 2-month-old boy with Stevens-Johnson syndrome (SJS)/Toxic epidermal necrolysis (TEN) and ocular involvement that was successfully treated with cryopreserved amniotic membrane transplantation (AMT).

Observation: A 2-month-old otherwise healthy boy was referred to Boston Children's Hospital with extensive rash and desquamation concerning for SJS/TEN. A skin biopsy was performed which showed full-thickness epidermal necrosis. AMT was performed at the bedside under general anesthesia. A combination of tobramycin and dexamethasone ointment was prescribed four times per day. On reassessment two weeks following AMT, the entire ocular surface had healed with no signs of conjunctival and/or corneal inflammation or ulceration.

Conclusion And Importance: To the best of our knowledge, our case represents the youngest patient with SJS/TEN to be managed by AMT and one of very few cases where acetaminophen is suspected to be the offending agent. This case highlights the efficacy of AMT at such a young age and feasibility of performing the procedure at bedside in these patients It also highlights that SJS/TEN can develop at such young age.
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http://dx.doi.org/10.1016/j.ajoc.2021.101017DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7820027PMC
March 2021

Ocular involvement in recurrent infectious mucocutaneous eruption (RIME): a variation on a theme.

J AAPOS 2020 Dec 18. Epub 2020 Dec 18.

Department of Ophthalmology, Boston Children's Hospital, Boston, Massachusetts; Harvard Medical School, Boston, Massachusetts.

We present 2 cases of pediatric patients with reactive infectious mucocutaneous eruption with ophthalmic involvement. In both cases, the disease processes behaved similarly to that seen in patients with Mycoplasma pneumoniae-induced rash and mucositis. Visual outcomes were good.
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http://dx.doi.org/10.1016/j.jaapos.2020.10.003DOI Listing
December 2020

Mycoplasma Pneumoniae-Induced Rash and Mucositis: A Longitudinal Perspective and Proposed Management Criteria.

Am J Ophthalmol 2020 11 20;219:351-356. Epub 2020 Jun 20.

Department of Ophthalmology, Boston Children's Hospital, Boston, Massachusetts, USA; Harvard Medical School, Boston, Massachusetts, USA.

Purpose: To evaluate the natural history and ophthalmologic morbidity of Mycoplasma pneumoniae-induced rash and mucositis (MIRM) and propose a treatment algorithm.

Design: Retrospective, interventional case series.

Methods: Retrospective chart review of all MIRM patients examined by the department of ophthalmology at a tertiary children's hospital. Diagnosis was established clinically concomitant with either positive Mycoplasma pneumoniae IgM or PCR testing from January 1, 2010, until December 31, 2019. The main outcome measures were best-corrected visual acuity, long-term ocular sequelae, and duration and type of ophthalmic intervention.

Results: There were 15 patients (10 male and 5 female) aged 10.9 ± 4.2 years who had primary episodes of MIRM; of those, 4 had multiple episodes. All patients required topical steroid treatment, 3 required amniotic membrane transplantation, and 1 patient underwent placement of a sutureless biologic corneal badage device. There were no patients who suffered visual loss, but 1 was left with mild symblephara near the lateral canthus in each eye and 2 others had scarring of the eyelid margins and blepharitis.

Conclusions: The ocular morbidity is significantly less in MIRM than in other closely related syndromes such as erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis. However, these patients still require close observation and a low threshold for intervention to avoid permanent ophthalmic sequelae and possible blindness.
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http://dx.doi.org/10.1016/j.ajo.2020.06.010DOI Listing
November 2020

Trochleitis presenting with double vision in a patient with juvenile idiopathic arthritis.

Orbit 2020 Jun 5:1-2. Epub 2020 Jun 5.

Department of Ophthalmology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA.

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http://dx.doi.org/10.1080/01676830.2020.1772317DOI Listing
June 2020

Virtual Visits in Ophthalmology: Timely Advice for Implementation During the COVID-19 Public Health Crisis.

Telemed J E Health 2020 09 14;26(9):1113-1117. Epub 2020 May 14.

Harvard Medical School, Boston, Massachusetts, USA.

Virtual visits (VVs) are necessitated due to the public health crisis and social distancing mandates due to COVID-19. However, these have been rare in ophthalmology. Over 3.5 years of conducting >350 ophthalmological VVs, our group has gained numerous insights into best practices. This communication shares these experiences with the medical community to support patient care during this difficult time and beyond. We highlight that mastering the technological platform of choice, optimizing lighting, camera positioning, and "eye contact," being thoughtful and creative with the virtual eye examination, and ensuring good documenting and billing will make a successful and efficient VV. Moreover, we think these ideas will stimulate further VV creativity and expertise to be developed in ophthalmology and across medicine. This approach, holds promise for increasing its adoption after the crisis has passed.
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http://dx.doi.org/10.1089/tmj.2020.0121DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7640749PMC
September 2020

Prenatal diagnosis of intraconal lymphatic malformation on fetal magnetic resonance imaging.

J AAPOS 2020 04 23;24(2):113-115. Epub 2020 Jan 23.

Department of Ophthalmology, Boston Children's Hospital, Boston, Massachusetts.

Lymphatic malformations are benign hamartomatous tumors present at birth but usually diagnosed in early childhood. We report a case of prenatal diagnosis of an isolated unilateral retrobulbar lymphatic malformation with fetal magnetic resonance imaging (MRI). This was first detected at 27 weeks' gestational age. Postnatal ocular examinations at 4 days and 5 weeks of age showed no signs of optic nerve compromise. Postnatal MRI at 18 days of age showed slight increase in size of the lesion, and no intracranial vascular malformations were detected.
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http://dx.doi.org/10.1016/j.jaapos.2019.12.005DOI Listing
April 2020

Evaluation of computer-based retinopathy of prematurity (ROP) education for ophthalmology residents: a randomized, controlled, multicenter study.

J AAPOS 2019 04 16;23(2):86.e1-86.e7. Epub 2019 Mar 16.

Massachusetts Eye and Ear, Harvard Medical School, Department of Ophthalmology, Boston, Massachusetts. Electronic address:

Purpose: To evaluate the effect of a computer-based training program-Massachusetts Eye & Ear ROP Trainer-on residents' knowledge of retinopathy of prematurity (ROP) management.

Methods: In this prospective, randomized study, ophthalmology residents from nine different training programs consented to participate. Those who completed the study were randomly assigned to either the Trainer or the control group. The ROP Trainer was created using clinical cases encompassing the stages of ROP in digital pictures and videos. It includes sections on screening decisions, examination techniques, and diagnosis, and a reference section with the expert video clips and a searchable image library. Subjects in the control group were asked to study standard print material on ROP. A pre- and post-test, consisting of theoretical and practical (diagnosis) questions, and a post-intervention satisfaction test were administered. Accuracy of ROP diagnosis was assessed.

Results: A total of 180 residents agreed to participate, of whom 60 completed the study. Residents in the Trainer group had statistically significant improvements (P = 0.003) in ROP knowledge and diagnostic ability (P = 0.005). Residents randomized to the Trainer group were more satisfied with the training materials than were those in the control group. There was no significant difference in improving knowledge by year of training, sex, or country. Considering all training levels, a statistically significant increase was observed in sensitivity for the diagnosis of preplus or worse, zone I or II, ROP stage, category, and aggressive posterior ROP in the Trainer group.

Conclusions: In this study, the Trainer was shown to significantly improve ROP knowledge and diagnostic skills of residents, regardless of sex, year, of training, or country.
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http://dx.doi.org/10.1016/j.jaapos.2018.11.008DOI Listing
April 2019

Acquired reversible Brown syndrome caused by focal abscess of the superior oblique muscle.

J AAPOS 2019 06 30;23(3):172-174. Epub 2019 Jan 30.

Department of Ophthalmology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.

A 16-year-old boy with a history of relapsed acute myeloid leukemia and a right lower lobe lung abscess confirmed to be Aspergillus presented for a baseline eye examination prior to consideration of bone marrow transplantation. He noted double vision in up-and-left gaze, and his examination was consistent with an acquired right-sided Brown syndrome. Magnetic resonance imaging revealed a 4 mm rim-enhancing inflammatory focus in the right superior oblique muscle. His Brown syndrome resolved after treatment with systemic antimicrobials.
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http://dx.doi.org/10.1016/j.jaapos.2018.12.003DOI Listing
June 2019

A Dosing Study of Bevacizumab for Retinopathy of Prematurity: Late Recurrences and Additional Treatments.

Ophthalmology 2018 12 7;125(12):1961-1966. Epub 2018 Jun 7.

Jaeb Center for Health Research, Tampa, Florida.

Purpose: Intravitreal bevacizumab is increasingly used to treat severe retinopathy of prematurity (ROP), but it enters the bloodstream, and there is concern that it may alter development of other organs. Previously we reported short-term outcomes of 61 infants enrolled in a dose de-escalation study, and we report the late recurrences and additional treatments.

Design: Masked, multicenter, dose de-escalation study.

Participants: A total of 61 premature infants with type 1 ROP.

Methods: If type 1 ROP was bilateral at enrollment, then the study eye was randomly selected. In the study eye, bevacizumab intravitreal injections were given at de-escalating doses of 0.25 mg, 0.125 mg, 0.063 mg, or 0.031 mg; if needed, fellow eyes received 1 dose level higher: 0.625 mg, 0.25 mg, 0.125 mg, or 0.063 mg, respectively. After 4 weeks, additional treatment was at the discretion of the investigator.

Main Outcome Measures: Early and late ROP recurrences, additional treatments, and structural outcomes after 6 months.

Results: Of 61 study eyes, 25 (41%; 95% confidence interval [CI], 29%-54%) received additional treatment: 3 (5%; 95% CI, 1%-14%) for early failure (within 4 weeks), 11 (18%; 95% CI, 9%-30%) for late recurrence of ROP (after 4 weeks), and 11 (18%; 95% CI, 9%-30%) for persistent avascular retina. Re-treatment for early failure or late recurrence occurred in 2 of 11 eyes (18%; 95% CI, 2%-52%) treated with 0.25 mg, 4 of 16 eyes (25%; 95% CI, 7%-52%) treated with 0.125 mg, 8 of 24 eyes (33%; 95% CI, 16%-55%) treated with 0.063 mg, and 0 (0%; 95% CI, 0%-31%) of 10 eyes treated with 0.031 mg. By 6 months corrected age, 56 of 61 study eyes had regression of ROP with normal posterior poles, 1 study eye had developed a Stage 5 retinal detachment, and 4 infants had died of preexisting medical conditions.

Conclusions: Retinal structural outcomes are very good after low-dose bevacizumab treatment for ROP, although many eyes received additional treatment.
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http://dx.doi.org/10.1016/j.ophtha.2018.05.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6402322PMC
December 2018

The Effect of Botulinum Toxin Augmentation on Strabismus Surgery for Large-Angle Infantile Esotropia.

Am J Ophthalmol 2018 05 21;189:160-165. Epub 2018 Feb 21.

Department of Ophthalmology, Harvard Medical School, Boston, Massachusetts; Department of Ophthalmology, Boston Children's Hospital, Boston, Massachusetts. Electronic address:

Purpose: To determine whether botulinum toxin augments the effect of strabismus surgery in pediatric patients with large-angle infantile esotropia.

Design: Retrospective, comparative, case series.

Methods: Setting: Tertiary-care pediatric hospital.

Study Population: Patients with large-angle infantile esotropia.

Intervention: Treatment with botulinum toxin-augmented bilateral medial rectus muscle recessions ("augmented-surgery group") or traditional bilateral medial rectus muscle recessions ("surgery-only group").

Main Outcome Measure: The effect of surgery on ocular alignment at 4 months, measured in prism diopters of change per mm of surgery (PD/mm).

Results: There were 14 patients in the augmented-surgery group and 16 patients in the surgery-only group. The mean effect on alignment was significantly greater in the augmented-surgery group compared to the surgery-only group at 4 months (5.7 ± 1.3 vs 4.0 ± 1.4 PD/mm, P = .002) and at 1 year (5.4 ± 1.2 vs 3.7 ± 1.2 PD/mm, P = .002). There was a partial loss of treatment effect between 4 months and 1 year in both groups, which was similar in magnitude (P = .57). On linear regression, there was a trend toward a positive correlation between botulinum toxin dose and treatment effect, but this was not statistically significant (P = .09).

Conclusions: Botulinum toxin augments the surgical effect of medial rectus muscle recession. Botulinum toxin-augmented surgery may be an alternative to traditional options for large-angle infantile esotropia. A surgical dosing table is proposed for this technique.
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http://dx.doi.org/10.1016/j.ajo.2018.02.010DOI Listing
May 2018

Ophthalmologic Features of Progeria.

Am J Ophthalmol 2017 Oct 27;182:126-132. Epub 2017 Jul 27.

Department of Anesthesia, Boston Children's Hospital - Harvard Medical School, Boston, Massachusetts; Department of Pediatrics, Hasbro Children's Hospital - Warren Alpert Medical School of Brown University, Providence, Rhode Island.

Purpose: To establish the natural history of ophthalmic characteristics in Progeria patients and to determine incidence of ocular manifestations.

Design: Retrospective case series of patients with Progeria who were seen between 2007 and 2016.

Methods: Setting: Tertiary-care academic center.

Patient Population: Fourteen patients (28 eyes) with Hutchinson-Gilford Progeria syndrome were included for statistical analysis from a total of 84 patients who have been enrolled in clinical trials for Progeria at Boston Children's Hospital. Clinical treatment trial patients who were not seen at the Department of Ophthalmology at our hospital, but for whom we had detailed clinical ophthalmologic records, were also included. This essentially represents an estimated 20% of the world's known patients with Progeria. Interventions or Observation Procedures: Complete ophthalmic examination.

Main Outcome Measures: Visual acuity, stereoacuity, refraction, clinical findings of slit-lamp and dilated fundus examinations.

Results: Ophthalmic manifestations noted were hyperopia and signs of ocular surface disease owing to nocturnal lagophthalmos and exposure keratopathy. Additional ophthalmic manifestations included reduced brow hair, madarosis, and reduced accommodation. Most patients had relatively good acuity; however, advanced ophthalmic disease was associated with reduced acuity.

Conclusions: Children with Progeria are at risk for serious ophthalmic complications owing to ocular surface disease. Children with Progeria should have an ophthalmic evaluation at the time of diagnosis and at least yearly after that. Aggressive ocular surface lubrication is recommended, including the use of tape tarsorrhaphy at night.
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http://dx.doi.org/10.1016/j.ajo.2017.07.020DOI Listing
October 2017

Comparison of Botulinum Toxin With Surgery for the Treatment of Acute-Onset Comitant Esotropia in Children.

Am J Ophthalmol 2017 Apr 3;176:33-39. Epub 2017 Jan 3.

Department of Ophthalmology, Harvard Medical School and the Department of Ophthalmology, Boston Children's Hospital, Boston, Massachusetts. Electronic address:

Purpose: To determine whether botulinum toxin is as effective as strabismus surgery in the treatment of acute-onset comitant esotropia in children.

Design: Retrospective, nonrandomized, comparative clinical study.

Methods: Setting: Tertiary care pediatric hospital.

Study Population: Forty-nine children with acute-onset comitant esotropia.

Intervention: Treatment with either botulinum toxin ("chemodenervation group") or standard incisional strabismus surgery ("surgery group").

Main Outcome Measure: Success rate at 6 months (total horizontal deviation of 10 prism diopters or less and evidence of binocular single vision).

Results: There were 16 patients in the chemodenervation group and 33 patients in the surgery group. The success rate was not significantly different at 6 months (81% vs 61%, P = .20) or at 18 months (67% vs 58%, P = .74). The median angle of deviation and median stereoacuity were not significantly different at 6 or 18 months. The chemodenervation procedure was not inferior to incisional strabismus surgery at 6 months. The duration of general anesthesia (5 vs 71 min, P < .001) and time in the post-anesthesia care unit (37 vs 93 min, P < .001) were significantly shorter in the chemodenervation group. Botulinum toxin injection payment averaged $874 per procedure compared with $2783 for strabismus surgery.

Conclusions: Botulinum toxin is at least as effective as surgery in the treatment of acute-onset comitant esotropia at 6 months while reducing the duration of general anesthesia and healthcare costs.
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http://dx.doi.org/10.1016/j.ajo.2016.12.024DOI Listing
April 2017

Role of VEGF Inhibition in the Treatment of Retinopathy of Prematurity.

Semin Ophthalmol 2016 ;31(1-2):163-8

a Department of Ophthalmology , Boston Children's Hospital, Harvard Medical School , Boston , Massachusetts , USA.

Retinopathy of prematurity (ROP) is a potentially blinding disease characterized by retinal neovascularization, which eventually can lead to tractional retinal detachment. Improvements have been made regarding the management of retinopathy of prematurity (ROP) since it was described in the Cryotherapy for Retinopathy of Prematurity study. A more appropriate time for therapeutic intervention was defined by the Early Treatment for Retinopathy of Prematurity (ETROP) trial. Advances in screening strategies with the use of digital imaging systems are now available. All of this and the use of laser photocoagulation and vitreoretinal surgery have contributed to significant increases in favorable outcomes and decreases in child blindness secondary to ROP. Recently the use of vascular endothelial growth factor (VEGF) inhibitors has been introduced to the armamentarium for the treatment of ROP. The purpose of this review article is to evaluate the role of VEGF inhibition in the treatment of ROP.
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http://dx.doi.org/10.3109/08820538.2015.1114847DOI Listing
October 2016

Acute and Chronic Ophthalmic Involvement in Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis - A Comprehensive Review and Guide to Therapy. II. Ophthalmic Disease.

Ocul Surf 2016 04 13;14(2):168-88. Epub 2016 Feb 13.

Massachusetts Eye and Ear Infirmary, Harvard Medical School, USA. Electronic address:

Our purpose is to comprehensively review the state of the art with regard to Stevens- Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), with particular attention to improving the management of associated ocular surface complications. SJS and TEN are two ends of a spectrum of immune-mediated disease, characterized in the acute phase by a febrile illness followed by skin and mucous membrane necrosis and detachment. Part I of this review focused on the systemic aspects of SJS/TEN and was published in the January 2016 issue of this journal. The purpose of Part II is to summarize the ocular manifestations and their management through all phases of SJS/TEN, from acute to chronic. We hope this effort will assist ophthalmologists in their management of SJS/TEN, so that patients with this complex and debilitating disease receive the best possible care and experience the most optimal outcomes in their vision and quality of life.
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http://dx.doi.org/10.1016/j.jtos.2016.02.001DOI Listing
April 2016

Burn unit care of Stevens Johnson syndrome/toxic epidermal necrolysis: A survey.

Burns 2016 Jun 19;42(4):830-5. Epub 2016 Jan 19.

Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, 243 Charles Street, Boston, MA 02114, USA. Electronic address:

Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a systemic disease that can be associated with debilitating acute and chronic complications across multiple organ systems. As patients with acute SJS/TEN are often treated in a burn intensive care unit (BICU), we surveyed burn centers across the United States to determine their approach to the care of these patients. The goal of our study was to identify best practices and possible variations in the care of patients with acute SJS/TEN. We demonstrate that the method of diagnosis, use of systemic therapies, and involvement of subspecialists varied significantly between burn centers. Beyond supportive care provided to every patient, our data highlights a lack of standardization in the acute care of patients with SJS/TEN. A comprehensive guideline for the care of patients with acute SJS/TEN is indicated.
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http://dx.doi.org/10.1016/j.burns.2015.12.001DOI Listing
June 2016

Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis--A Comprehensive Review and Guide to Therapy. I. Systemic Disease.

Ocul Surf 2016 Jan 5;14(1):2-19. Epub 2015 Nov 5.

Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA. Electronic address:

The intent of this review is to comprehensively appraise the state of the art with regard to Stevens Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), with particular attention to the ocular surface complications and their management. SJS and TEN represent two ends of a spectrum of immune-mediated, dermatobullous disease, characterized in the acute phase by a febrile illness followed by skin and mucous membrane necrosis and detachment. The widespread keratinocyte death seen in SJS/TEN is rapid and irreversible, and even with early and aggressive intervention, morbidity is severe and mortality not uncommon. We have divided this review into two parts. Part I summarizes the epidemiology and immunopathogenesis of SJS/TEN and discusses systemic therapy and its possible benefits. We hope this review will help the ophthalmologist better understand the mechanisms of disease in SJS/TEN and enhance their care of patients with this complex and often debilitating disease. Part II (April 2016 issue) will focus on ophthalmic manifestations.
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http://dx.doi.org/10.1016/j.jtos.2015.10.002DOI Listing
January 2016

Unilateral Congenital Lacrimal Gland Agenesis With Contralateral Lacrimal Gland Hypoplasia.

J Pediatr Ophthalmol Strabismus 2015 Oct 15;52 Online:e52-4. Epub 2015 Oct 15.

Congenital alacrima is a form of primary lacrimal deficiency characterized by aplasia or hypoplasia of the lacrimal gland. The puncta and salivary glands may also be aplastic. The case of a 5-year-old girl with congenital alacrima secondary to lacrimal gland agenesis and hypoplasia without punctal or salivary gland involvement and without other systemic comorbidities is reported.
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http://dx.doi.org/10.3928/01913913-20151007-11DOI Listing
October 2015

A Novel Technique for Amniotic Membrane Transplantation in Patients with Acute Stevens-Johnson Syndrome.

Ocul Surf 2016 Jan 24;14(1):31-6. Epub 2015 Sep 24.

Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA, USA; Department of Ophthalmology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA. Electronic address:

Cryopreserved amniotic membrane (AM) transplantation is an emerging technique that is becoming the gold standard for the management of acute Stevens-Johnson syndrome (SJS) and its more severe variant, toxic epidermal necrolysis (TEN). We describe a novel surgical technique utilizing a single, large sheet of AM (5 x 10 cm) and a custom-made forniceal ring, which facilitates AM placement. Our technique is easy to use and minimizes suturing and manipulation of ocular tissues, resulting in decreased operative time. This technique may be applied in the management of multiple ocular surface disease processes, including chemical or thermal burns, severe ocular graft versus host disease (GVHD), and other autoimmune diseases.
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http://dx.doi.org/10.1016/j.jtos.2015.07.002DOI Listing
January 2016

Eyelid Lacerations Due to Dog Bite in Children.

J Pediatr Ophthalmol Strabismus 2015 Nov-Dec;52(6):360-3. Epub 2015 Sep 9.

Purpose: To evaluate the nature and extent of lacrimal apparatus injury in children after eyelid laceration from a dog bite.

Methods: A retrospective chart review of all eyelid lacerations treated between 1990 and 2012 at Boston Children's Hospital, Boston, Massachusetts, was conducted. Seventy-three patients who sustained an eyelid laceration due to dog bite were identified and were matched 5:1 with a randomly selected cohort of 365 patients from the group of 1,177 patients who had sustained eyelid lacerations from other causes during the same time period.

Results: Of the 73 patients who sustained an eyelid laceration due to a dog bite, 26 (35.62%) had damage to the lacrimal apparatus. This was statistically significant when compared to patients who sustained eyelid lacerations from other causes, in which 13 (3.56%) patients had damage to the lacrimal apparatus (P < .000001). The inferior canaliculus was the most commonly involved site of lacrimal apparatus trauma as a result of a dog bite. Success was defined as lack of epiphora at the time of the last follow-up. Early surgical management of eyelid lacerations with lacrimal apparatus involvement had a success rate of 82%.

Conclusions: Eyelid lacerations due to dog bites have a greater prevalence of involvement of the lacrimal apparatus and especially the inferior canaliculus than lacerations due to other causes in children. Clinicians should have a high index of suspicion for lacrimal apparatus involvement and be prepared for surgical repair, if indicated.
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http://dx.doi.org/10.3928/01913913-20150901-02DOI Listing
June 2016

Complications of Stevens-Johnson syndrome beyond the eye and skin.

Burns 2016 Feb 9;42(1):20-27. Epub 2015 Apr 9.

Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, 243 Charles St., Boston, MA 02114, USA. Electronic address:

Introduction: Ocular and cutaneous disease are common chronic sequelae of Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) and have been well described in the literature. Long-term complications affecting other organ systems have not been so well described. The purpose of this review article is to highlight non-ocular and non-cutaneous chronic complications of SJS/TEN.

Methods: The PubMed database was searched for the keywords "Stevens-Johnson syndrome" and "toxic epidermal necrolysis" through September 1, 2014. Relevant articles were then reviewed in full.

Results: 138 articles in the English language were found that described chronic sequelae of SJS/TEN. Our search revealed six affected organ systems other than the eyes and integument, with chronic sequelae from SJS/TEN: respiratory, gastrointestinal/hepatic, oral, otorhinolaryngologic, gynecologic/genitourinary, and renal. Complications involving these organs systems appeared likely to reduce the quality of life for SJS/TEN survivors.

Discussion: SJS/TEN is a multi-organ disease requiring multidisciplinary care from a variety of specialists. Affected patients have complex hospital stays, and their quality of life may be severely impacted by multiple long-term complications. We believe that preventative care in the acute setting might limit the development and progression of many of the sequelae described above.
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http://dx.doi.org/10.1016/j.burns.2015.03.012DOI Listing
February 2016

A 13-year-old boy with asthma, itchy eyes, and decreased vision.

J Pediatr 2014 Dec 11;165(6):1267. Epub 2014 Sep 11.

Department of Ophthalmology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.

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http://dx.doi.org/10.1016/j.jpeds.2014.07.044DOI Listing
December 2014

Adjustable nasal transposition of split lateral rectus muscle for third nerve palsy.

JAMA Ophthalmol 2014 Aug;132(8):963-9

Department of Ophthalmology, Boston Children's Hospital, Boston, Massachusetts3Department of Ophthalmology, Harvard Medical School, Boston, Massachusetts.

Importance: Third nerve palsy causes disfiguring, incomitant strabismus with limited options for correction.

Objective: To evaluate the oculomotor outcomes, anatomical changes, and complications associated with adjustable nasal transposition of the split lateral rectus (LR) muscle, a novel technique for managing strabismus associated with third nerve palsy.

Design, Setting, And Participants: Retrospective medical record review appraising outcomes of 6 consecutive patients with third nerve palsy who underwent adjustable nasal transposition of the split LR muscle between 2010 and 2012 with follow-up of 5 to 25 months at a tertiary referral center.

Intervention: Adjustable nasal transposition of the split LR muscle.

Main Outcomes And Measures: The primary outcome was postoperative horizontal and vertical alignment. Secondary outcomes were (1) appraising the utility of adjustable positioning, (2) demonstrating the resultant anatomical changes using magnetic resonance imaging, and (3) identifying associated complications.

Results: Four of 6 patients successfully underwent the procedure. Of these, 3 patients achieved orthotropia. Median preoperative horizontal deviation was 68 prism diopters of exotropia and median postoperative horizontal deviation was 0 prism diopters (P = .04). Two patients had preoperative vertical misalignment that resolved with surgery. All 4 patients underwent intraoperative adjustment of LR positioning. Imaging demonstrated nasal redirection of each half of the LR muscle around the posterior globe, avoiding contact with the optic nerve; the apex of the split sat posterior to the globe. One patient had transient choroidal effusion and undercorrection. Imaging revealed, in this case, the apex of the split in contact with the globe at an anterolateral location, suggesting an inadequate posterior extent of the split. In 2 patients, the surgical procedure was not completed because of an inability to nasally transpose a previously operated-on LR muscle.

Conclusions And Relevance: Adjustable nasal transposition of the split LR muscle can achieve excellent oculomotor alignment in some cases of third nerve palsy. The adjustable modification allows optimization of horizontal and vertical alignment. Imaging confirms that the split LR muscle tethers the globe, rotating it toward primary position. Case selection is critical because severe LR contracture, extensive scarring from prior strabismus surgery, or inadequate splitting of the LR muscle may reduce the likelihood of success and increase the risk of sight-threatening complications. Considering this uncertainty, more experience is necessary before widespread adoption of this technique should be considered.
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http://dx.doi.org/10.1001/jamaophthalmol.2014.756DOI Listing
August 2014

Emerging treatments for retinopathy of prematurity.

Semin Ophthalmol 2009 Mar-Apr;24(2):82-6

Department of Ophthalmology, Children's Hospital Boston, Boston, MA 02115, USA.

Retinopathy or prematurity (ROP) is a leading cause of potentially preventable blindness in children. With increased survival of infants born at earlier gestational ages the number of infants at risk from vision loss from ROP has increased. Current treatments consist of close monitoring of oxygen saturation levels, peripheral retinal ablation by cryotherapy or laser photocoagulation, and vitreoretinal surgery. Research in the area of angiogenesis has lead to numerous breakthroughs. Emerging treatments for ROP are targeting the Vascular Endothelial Growth Factor (VEGF) and Insulin-Like Growth Factor 1 (IGF-1) pathways, as well as dietary supplementation with omega-3-polyunsaturated fatty acids.
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http://dx.doi.org/10.1080/08820530902800322DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4028623PMC
May 2009

Transient monocular vision loss from acute rheumatic fever.

Pediatr Cardiol 2008 Sep 18;29(5):966-8. Epub 2008 Jan 18.

Department of Ophthalmology, Boston Medical Center, 85 East Concord St., 8th floor, Boston, MA 02118, USA.

A 15-year-old male developed transient monocular visual loss secondary to retinal artery flow disturbance, vasospasm, and possible microemboli associated with acute rheumatic heart disease. He had insufficiently treated streptococcal pharyngitis. Inadequately treated rheumatic carditis should be considered among the causes of transient monocular visual loss in children.
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http://dx.doi.org/10.1007/s00246-007-9159-yDOI Listing
September 2008