Jennifer A. Galvin

Atropine for the Prevention of Myopia Progression in Children: A Report by the American Academy of Ophthalmology.

PURPOSE To review the published literature on the efficacy of topical atropine for the prevention of myopic progression in children. METHODS Literature searches were last conducted in December 2016 in the PubMed database with no date restrictions, but were limited to studies published in English, and in the Cochrane Library database without any restrictions. The combined searches yielded 98 citations, 23 of which were reviewed in full text. Of these, 17 articles were deemed appropriate for inclusion in this assessment and subsequently were assigned a level of evidence rating by the panel methodologist. RESULTS Seventeen level I, II, and III studies were identified. Most of the studies reported less myopic progression in children treated with atropine compared with various control groups. All 8 of the level I and II studies that evaluated primarily myopic progression revealed less myopic progression with atropine (myopic progression ranging from 0.04±0.63 to 0.47±0.91 diopters (D)/year) compared with control participants (myopic progression ranging from 0.38±0.39 to 1.19±2.48 D/year). In studies that evaluated myopic progression after cessation of treatment, a rebound effect was noted. Several studies evaluated the optimal dosage of atropine with regard to myopic progression, rebound after treatment cessation, and minimization of side effects. Lower dosages of atropine (0.5%, 0.1%, and 0.01%) were found to be slightly less effective during treatment periods of 1 to 2 years, but they were associated with less rebound myopic progression (for atropine 0.01%, mean myopic progression after treatment cessation of 0.28±0.33 D/year, compared with atropine 0.5%, 0.87±0.52 D/year), fewer side effects, and similar long-term results for myopic progression after the study period and rebound effect were considered. The most robust and well-designed studies were carried out in Asian populations. Studies involving patients of other ethnic backgrounds failed to provide sufficient evidence of an effect of atropine on myopic progression. CONCLUSIONS Level I evidence supports the use of atropine to prevent myopic progression. Although there are reports of myopic rebound after treatment is discontinued, this seems to be minimized by using low doses (especially atropine 0.01%).

Contact Lens Correction of Aphakia in Children: A Report by the American Academy of Ophthalmology

PURPOSE To review the published literature to assess the visual outcomes and adverse events associated with the 2 most commonly used contact lenses for treating aphakia in children: silicone elastomer (SE) and rigid gas permeable (RGP). METHODS Literature searches were last conducted in January 2018 in the PubMed, Cochrane Library, and ClinicalTrials.gov databases with no date or language restrictions. These combined searches yielded 167 citations, 27 of which were reviewed in full text. Of these, 10 articles were deemed appropriate for inclusion in this assessment and subsequently assigned a level of evidence rating by the panel methodologist. RESULTS The literature search identified 4 level II studies and 6 level III studies. There were insufficient data to compare visual outcomes for eyes treated using SE lenses versus RGP lenses. Silicone elastomer lenses have the advantage that they can be worn on an extended-wear basis, but they were associated with more adverse events than RGP lenses. These adverse events included microbial keratitis, corneal infiltrates, corneal edema, corneal scars, lenses adhering to the cornea, superficial punctate keratopathy, lid swelling, and conjunctival hyperemia. The lens replacement rate was approximately 50% higher for RGP lenses in the only study that directly compared SE and RGP lenses. CONCLUSIONS Limited evidence was found in the literature on this topic. Silicone elastomer and RGP contact lenses were found to be effective for treating aphakia in children. Silicone elastomer lenses are easier to fit and may be worn on an extended-wear basis. Rigid gas permeable lenses must be removed every night and require a more customized fit, but they are associated with fewer adverse events. The choice of which lens a practitioner prescribes should be based on the particular needs of each patient.

Ophthalmic abnormalities of Pai syndrome: A case report and review of literature

We present a new case of Pai syndrome and review the current literature on this disease. Our PubMed search was conducted using the term “Pai syndrome.” We included original case reports and case series of Pai syndrome. Exclusion criteria consisted of cases not meeting the full phenotypic triad and text not available in the English language. Our patient was delivered at full term by primary cesarean section after an uncomplicated pregnancy. At three-and-a-half weeks, she was evaluated by plastic and reconstructive surgery for a facial cleft; her growth and development were otherwise normal. Examination revealed a midline cleft lip with intact palate, a mass in the left nose partially obstructing the left naris, and an epibulbar dermoid. Subsequent workup included an echocardiogram, renal ultrasound, chromosomal analysis, magnetic resonance imaging (MRI) of the head, and microarray-based comparative genomic hybridization (array CGH). Results were notable for an interhemispheric lipoma within the cranium on MRI and a 1.071-Mb duplication at 4q35.2, previously documented as benign in the database of genomic variants. Given the variant had no documented disease association, including cleft lip or palate, it was regarded as unrelated to our patient’s birth defects. There was no family history of congenital abnormalities, and the parents declined genetic testing. At 2 months old, her examination by pediatric ophthalmology showed a left inferior corneal limbal dermoid measuring 6 mm × 6 mm and a single iris membrane extending across the temporal pupil (Figure 1). Both findings contributed to amblyopia of the left eye. Her right eye and bilateral fundi were unremarkable. Next, our patient underwent a combined procedure of midline cleft lip repair, left limbal dermoid excision, and left pupilloplasty without any complications. At 7 months old, the left eye’s visual axis was unobstructed and the amblyopia was being closely monitored. At 9 months old, the patient and her family moved out of state; they were counseled about the importance of maintaining care for amblyopia management. Our literature search yielded 32 case reports of diagnosed Pai syndrome. However, only 17 of these cases fulfill the classic clinical triad consisting of midline orofacial cleft, cutaneous facial polyp, and pericallosal lipoma. Furthermore, only nine of these case discussions described ocular manifestations of the disease, including hypopigmented fundi, peripapillary pigmentation, hypopigmented macula, hypertelorism, epicanthus, persistent pupillary membrane, conjunctival lipoma, and mesodermal dysgenesis of the anterior segment of the eye (1–7) (Table 1). Our patient presented with findings of midline orofacial cleft, cutaneous facial polyp, and pericallosal lipoma. Ocular characteristics include a corneal limbal dermoid and a visually significant pupillary membrane. These two findings are consistent with prior reports. First described in 1987, Pai syndrome is an uncommon frontonasal dysplasia with a clinical constellation of three anomalies—midline orofacial cleft, cutaneous facial polyps, and pericallosal lipomas (8). In 1990, Reardon et al. proposed a broader definition of the syndrome consisting of only two obligate criteria: median cleft upper lip and midline lipomas (9). However, additional cases presenting with the original phenotype support the triad established by Pai et al. (1,10) In our patient, the diagnosis of Pai syndrome was made with the diagnosis of midline cleft lip, cutaneous polyp, and interhemispheric lipoma. The differential diagnosis of cleft lip and epibulbar dermoids includes Goldenhar syndrome, but our patient did not have the hemifacial macrosomia characteristic of the syndrome (11). Additionally, her genetic evaluation was negative for novel variants and mutations linked to cleft lip. Given the paucity of publications on Pai Syndrome and an even greater lack of discussion on the ophthalmic manifestations of this rare disease (13–23), it is difficult to typify ocular findings. Based on the present literature review, most descriptions of ophthalmic presentations with Pai syndrome are localized to the orbit and anterior segment. The genetic locus also has not been elucidated (3). Although most cases have been sporadic, an autosomal dominant inheritance pattern has been reported in the literature (3,12). Although there is limited literature on Pai syndrome and its variable phenotypic presentation, ophthalmic involvement occurs with sufficient frequency to warrant pediatric ophthalmological evaluation. Those with ocular findings of Pai syndrome may need medical and/or surgical management to prevent amblyopia, such as was the case in our patient.

Superior oblique palsy: A complication of endoscopic sinus surgery

A 34-year-old woman with chronic sinusitis and nasal polyps was treated with bilateral pansinusotomy. Postoperatively, she had a unilateral superior oblique paresis. We suspect that superior oblique damage occurred without involvement of the medial rectus because of inadvertent entry of the endoscopic probe through the superior lamina into the left orbit.

The Use of β-Blockers for the Treatment of Periocular Hemangiomas in Infants: A Report by the American Academy of Ophthalmology

PURPOSE To review the published literature assessing the efficacy of β-blockers for the treatment of periocular hemangioma in infants. METHODS Literature searches were conducted in May 2018 in PubMed with no date restrictions and limited to studies published in English and in the Cochrane Library database without any restrictions. The combined searches yielded 437 citations. Of these,16 articles were deemed appropriate for inclusion in this assessment and assigned a level of evidence rating by the panel methodologist. RESULTS None of the 16 studies included in this assessment were rated level I, 3 were rated level II, and 13 were rated level III. The most common treatment regimen was 2 mg/kg daily oral propranolol, but intralesional and topical β-blockers were also used. Treatment effect was most often measured in terms of reduction in the size of the lesions, which occurred in the majority of patients. β-Blockers were consistently shown to reduce astigmatism, but this reduction was shown to be statistically significant in only 2 series. The effect of β-blockers on amblyopia was not adequately documented. β-Blockers were generally well tolerated and had mild side effects (fatigue, gastrointestinal upset/diarrhea, restlessness/sleep disturbances, minor wheezing, and cold extremities). Complications severe enough to require cessation of treatment occurred in only 2 patients out of a total of 229 who received β-blockers. CONCLUSIONS There is limited evidence to support the safety and efficacy of both topical and systemic β-blockers to promote regression of periocular hemangiomas. Additional research may confirm the best dosage and route of administration to maximize efficacy in reducing induced astigmatism and amblyopia associated with periocular hemangiomas while minimizing side effects.

Clinical Uses of the Botulinum Toxin and Ophthalmology

Botulinum toxin therapy is now established in the field of ophthalmology for treatment of blepharospasm ,hemifacial spasm and strabismus and may help certain types of nystagmus, apraxia of the eyelid opening, eyelid myokyomia, facial nerve synkinesis, lacrimal hypersecretion syndromes, eyelid retraction, spastic entropion and corneal protective ptosis. In this chapter, I discuss these indications.