Iason S. Mantagos

Emerging Treatments for Retinopathy of Prematurity

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.

Role of VEGF Inhibition in the Treatment of Retinopathy of Prematurity

ABSTRACT 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.

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

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.

Corneal Endothelial Cell Characteristics After Pediatric Cataract Surgery

PURPOSE To describe the corneal endothelial cell characteristics after pediatric cataract surgery. METHODS Retrospective case series of 29 study eyes and 26 controls were evaluable. Specular microscopy was performed and endothelial cell features were correlated with clinical and surgical features. RESULTS Mean age at testing was 106 months in the study group and 116 months in the control group with a mean interval of 78 months after cataract surgery. Mean endothelial cell density in study eyes was 2,668 cells/mm(2) (control eyes, 3,002 cells/mm(2), P < .01). Mean coefficient of variation was 35% (control eyes, 31%, P < .01), and mean percentage of hexagonal cells was 40% (control eyes, 51%, P < .01). There was no significant difference based on age, duration since cataract surgery, contact lens use, glaucoma, number of intraocular procedures, or presence of intraocular lens. CONCLUSIONS This study demonstrates endothelial cell loss with polymegathism and pleomorphism in children undergoing cataract surgery. This poses a risk of developing long-term corneal complications and hence close monitoring of the endothelial cell count is advisable.

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

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.

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

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.

Unilateral Congenital Lacrimal Gland Agenesis With Contralateral Lacrimal Gland Hypoplasia

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.

Eyelid lacerations due to dog bite in children

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 Childrens 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.

Transient Monocular Vision Loss from Acute Rheumatic Fever

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.

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

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.