Advantages of Early Surgical Management of Periorbital Infantile Hemangiomas.

Background: Early evaluation and treatment of periorbital infantile hemangiomas (POIH) were associated with lower rates of ophthalmological complications. Objective: To evaluate age and characteristics associated with improved anisometropic astigmatism (anisoastigmatism) and eye symmetry measured by diopters and a 5-point scale, respectively, in patients with POIH treated with surgical excision. Methods: A retrospective study was performed on patients with POIH. Patient characteristics and eye symmetry were analyzed between patients with resolved and unresolved anisoastigmatism after surgery. Statistical analyses included the Mann-Whitney U tests, chi-square tests, and linear regression models. Results: In total, 54 patients were included (male: 20, female: 34). Upper medial eyelid was the most commonly affected site (resolved: 45%, unresolved: 43%), followed by upper lateral and upper central. Fifty-six percent (31/55) had postoperative resolution of anisoastigmatism, whereas 44% (24/55) did not. Earlier surgical evaluation (median: 4.5 vs. 6.0 months, p = 0.047) and excision (median: 5.0 vs. 12.0 months, p = 0.005) were associated with reversible anisoastigmatism. Good and suboptimal eye symmetry were not associated with earlier surgical excision (median: 6 vs. 6.5 months, p = 0.87). Follow-up ranged from 1 month to 12 years. Conclusion: Earlier surgical excision was associated with reversing anisoastigmatism but was not significant for improving eye symmetry.

Distribution and Clinical Characteristics of Periorbital Infantile Hemangiomas.

Background: Periorbital infantile hemangiomas (POIHs) are associated with a high incidence of visual complications. Objective(s): To analyze the sites of predilection of POIHs and to determine whether certain sites require earlier intervention due to their higher rate of visual complications. Methods: A retrospective case series study was conducted on patients from two tertiary care centers for 25 years. The location of POIHs was determined from clinical photographs, medical records, and radiological studies. The presence or absence of anisometropic astigmatism (anisoastigmatism) and amblyopia was recorded. Data were analyzed using a chi-square test. Results: There were 486 patients, of which 302 patients had ophthalmology evaluations and 245 patients had refractive error data. At presentation, 10% of patients already had amblyopia and 44% had anisoastigmatism. Medial eyelid lesions had the highest risk of developing anisoastigmatism (anisoastigmatism correlates with eyelid position, p = 0.0001). Segmental and upper medial lesions had the highest risk of amblyopia at initial evaluation. Conclusion: The site of POIH is an important indicator for developing clinically significant anisoastigmatism and amblyopia, underlining the need for early ophthalmologic assessment and management.

The Evolution of Full Thickness Macular Hole After Short Exposure to High Powered Hand-held Laser Pointer.

PURPOSE: To report a case of a full thickness macular hole (FTMH) after exposure to an extremely powerful handheld laser pointer. METHODS: We evaluated a 14-year-old male with a laser induced FTMH one month after a momentary exposure to a 5000 mW blue laser pointer. Imaging modalities including fundus color, autofluorescence, and spectral domain optical coherence tomography (SD-OCT), acquired both at our clinic and by the referring physician soon after the injury, are used to describe the clinical evolution of the case. RESULTS: Soon after the injury an intensely white, circular opacification of the retina approximately 400 µm in diameter was seen in the fovea. Early SD-OCT images showed full thickness hyperreflectivity, likely representing tissue necrosis. One month later, a FTMH and eradication of the retinal pigment epithelium at its base were evident in the fundus color, autofluorescence and SD-OCT images. CONCLUSION: High power laser pointers have become easily available online. The presenting findings after exposure to such high-power devices are distinct from those reported after exposure to weaker laser pointers. While long exposure to weaker lasers typically produces extensive, calligraphic figures and yellow placoid lesions involving only the outer retina, in our case a very brief exposure led to focal, full-thickness injury of the fovea.

Achromatopsia: a review.

PURPOSE OF REVIEW: The purposes of this article are to examine the literature published on achromatopsia and provide a comprehensive review of the clinical disease, genetic characteristics, and potential for therapy. Specifically, this article will describe recent advances in gene therapy in animal models, clinical features in human, and barriers to human translation. RECENT FINDINGS: Building on prior success with adeno-associated virus (AAV) therapy in mice models for achromatopsia with mutations in the CNGB3, CNGA3, or GNAT2 genes, multiple cone-specific promoters have recently been developed and shown success in mice and nonhuman primates. A sheep CNGA3 model has also been characterized. Two clinical trials are under way: one to better characterize humans with achromatopsia and another to study a ciliary neurotrophic factor (CNTF) implant as a treatment for patients with the CNGB3 mutation. SUMMARY: Genetic understanding and disease characterization of achromatopsia continues to evolve, as do gene therapy tools and animal models. The potential for the treatment of achromatopsia in humans with gene therapy shows great promise.

Bilateral posterior tenectomy of the superior oblique muscle for the treatment of A-pattern strabismus.

PURPOSE: To evaluate the efficacy of bilateral posterior superior oblique tenectomy for the treatment of A-pattern strabismus due to superior oblique overaction regardless of the magnitude of the pattern. METHODS: The medical records of patients with A-pattern esotropia or exotropia in the presence of superior oblique overaction who underwent combined horizontal muscle surgery along with bilateral superior oblique posterior 7/8 tenectomy from 2003 to 2013 were retrospectively reviewed. Patients with at least 3 months' follow-up were included. RESULTS: A total of 73 patients were included. Of these, 46 had esotropia; 27, exotropia. The preoperative A-pattern deviation for the study population was 19.6(Δ) ± 11.4(Δ) (range, 10-60), with a final postoperative patten collapse of 18.2 ± 3.6. Superior oblique overaction was 2.3 ± 0.7 preoperatively and 0.3 ± 0.7 postoperatively. Overall, 87.7% of patients had a successful collapse of their pattern to <10(Δ) following the initial bilateral superior oblique posterior tenectomy, with an additional 4.1% following a second procedure. Of patients with a pattern deviation of <25(Δ), 87.9% had successful collapse of the pattern following 1 surgery, and 86.7% of patients who had a pattern of ≥25(Δ) had successful collapse. Postoperatively, 7 patients demonstrated mild inferior oblique overaction. No surgical complications were noted. CONCLUSIONS: A uniform dose of bilateral posterior 7/8 tenectomy surgery successfully collapses A-pattern deviations of all magnitudes.

Treatment of capillary hemangiomas causing refractive and occlusional amblyopia.

PURPOSE: Capillary hemangiomas of the eyelid and orbit are treated when amblyopia secondary to anisometropic astigmatism or pupillary occlusion is present or when rapid growth of the hemangioma threatens to occlude the pupil. The goal of this study was to determine whether treatment of hemangiomas resolves or prevents occlusion or results in decrease in astigmatism. METHODS: The records of 54 patients who underwent treatment for reduction in the size of a capillary hemangioma causing amblyopia or threatened amblyopia in two pediatric ophthalmology practices were reviewed. RESULTS: Twenty-eight patients were treated for amblyopia due to anisometropic astigmatism. The average amount of pretreatment astigmatism was 2.71 D, while the average amount of post-treatment astigmatism was 0.46 D. Fifteen of these patients could be tested for optotype visual acuity and all had vision acuity of 20/40 or better. Only 1 of the 15 patients treated for threatened occlusion of the pupil developed occlusion. Six of these patients cooperated with optotype visual acuity and all had vision acuity of 20/30 or better. Eleven patients were treated for pupillary occlusion. Occlusion resolved in all cases. Of the five patients treated for occlusion who cooperated with optotype visual acuity, two had a vision acuity of 20/100 or worse. CONCLUSIONS: Treatment to reduce the size of capillary hemangiomas results in resolution of occlusion, reduction in astigmatism, and prevention of pupillary occlusion. Those with occlusion are at higher risk for severe residual amblyopia and require prompt and definitive treatment.

Surgical treatment of capillary hemangiomas causing amblyopia.

BACKGROUND: Capillary hemangiomas of the eyelids and orbit can cause refractive and occlusive amblyopia. Although oral and intralesional steroid injections are the most common treatment modalities, sometimes they are not successful. There is a paucity of information in the literature on the success of eliminating amblyogenic factors by treating these lesions with surgical resection. METHODS: Retrospective chart review of 10 patients in two pediatric ophthalmology practices who underwent surgical excision of a capillary hemangioma that was causing amblyopia and that had failed to regress with other treatment. RESULTS: Two patients had surgery secondary to pupillary occlusion, which was successful in relieving occlusion. Eight patients had surgery secondary to significant astigmatism. The average preoperative astigmatic difference between the affected and unaffected eye in five of these patients undergoing surgery before the age of 21 months was 2.15 D. The average postoperative astigmatic difference was 0.1 D. The average preoperative astigmatic difference between the affected and unaffected eye in three patients undergoing surgery after 21 months of age was 1.6 D. Surgery completely failed to reduce the astigmatism in two of these patients. The third patient had a decrease of 0.75 D of cylinder but still had a difference of 1.75 D between the two eyes postoperatively. Postoperative complications in this study included wound infection in one patient. CONCLUSIONS: Surgical excision of capillary hemangiomas that were resistant to other modes of treatment was useful in relieving pupillary occlusion and in decreasing the amount of astigmatism if performed before the age of 21 months in our series of patients. Our cases as well as the literature suggest that surgery should be performed at 13 months or earlier to reduce the amount of astigmatism.

Risk factors for amblyopia in children with capillary hemangiomas of the eyelids and orbit.

INTRODUCTION: Capillary hemangiomas are the most common orbital tumors of childhood and can cause amblyopia secondary to occlusion of the pupil, anisometropia, or strabismus. We undertook this study to describe the clinical characteristics of children with capillary hemangiomas and to propose a classification system to guide clinical treatment decisions. METHODS: A retrospective review of the records of 129 patients with 132 capillary hemangiomas in two pediatric ophthalmology practices was conducted. Hemangiomas were classified based on size. Presence of aniosometropic astigmatism, ptosis, pupillary occlusion, lid margin change, proptosis, globe displacement, and strabismus was recorded. RESULTS: Thirty-one hemangiomas measured less than 1 cm in greatest dimension and were not associated with amblyogenic factors. Seventy-five patients had hemangiomas that measured greater than 1 cm, 40 of which were associated with amblyopia. Eighteen children had diffuse hemangiomas that could not be measured and 14 of these were associated with amblyopia. Five of seven hemangiomas in six patients with PHACES syndrome were associated with amblyopia. CONCLUSION: This study is the largest review of capillary hemangiomas of the orbit and eyelids. Our findings suggest that size greater than 1 cm in largest diameter is an important predictor of amblyogenic factors and approximately half of these patients will require treatment. Diffuse hemangiomas and hemangiomas in patients with PHACES syndrome will cause amblyopia in the majority of cases.

Ocular and systemic manifestations of PHACES (Posterior fossa malformations, Hemangiomas, Arterial anomalies, Cardiac defects and coarctation of the Aorta, Eye abnormalities, and Sternal abnormalities or ventral developmental defects) syndrome.

INTRODUCTION: PHACES syndrome (Posterior fossa malformations, Hemangiomas, Arterial anomalies, Cardiac defects and coarctation of the aorta, Eye abnormalities, and Sternal abnormalities or ventral developmental defects) is a rare neurocutaneous syndrome with only 2 case reports published in the ophthalmic literature. This study was conducted to identify ocular and systemic manifestations of PHACES syndrome. METHODS: A retrospective chart review was performed on 8 children with a diagnosis of PHACES syndrome. Information recorded included age at first visit, length of follow-up, gender, race, vision, need for glasses, strabismus, amblyopia, ptosis, proptosis, anterior and posterior segment abnormalities, need for treatment of the hemangioma, type of treatment of the hemangioma, and systemic manifestations. RESULTS: Periocular and ocular findings in patients with PHACES syndrome included hemangioma involving ocular structures (n = 6), strabismus (n = 4), amblyopia (n = 5), proptosis (n = 2), ptosis (n = 5), anterior polar cataract (n = 1), optic atrophy from optic neuropathy (n = 1), heterochromia (n = 1), and refractive error requiring glasses (n = 2). All patients were treated with steroids for the hemangioma. Systemic manifestations of PHACES syndrome included posterior fossa malformation (n = 4), hemangioma (n = 8), arterial anomalies (n = 3), cardiac abnormalities (n = 3), and sternal or ventral deformities (n = 3). CONCLUSION: Children with PHACES syndrome may have significant ocular and systemic abnormalities and are at increased risk for strabismus and amblyopia. They often require steroid therapy of the hemangioma to prevent and/or treat ocular complications. These patients require careful monitoring by a pediatric ophthalmologist in addition to other subspecialists.

Periocular hemangiomas: what every physician should know.

Hemangiomas are the most common benign tumor of infancy. Most hemangiomas remain asymptomatic and can be managed by close observation; however, immediate treatment is indicated for hemangiomas that may cause significant complications. Periocular hemangiomas warrant close evaluation and early, active treatment of those with the potential to threaten or permanently compromise vision. Herein we review the clinical features of periocular hemangiomas, differential diagnosis, possible ophthalmologic complications and sequelae, and therapeutic modalities.

Ophthalmic issues in hemangiomas of infancy.

Hemangiomas are the most common benign tumor of infancy. Although most hemangiomas remain asymptomatic, certain hemangiomas can cause significant morbidity and require treatment. Periocular hemangiomas require close observation and early therapy for those lesions with potential for visual impairment. Hemangiomas typically cause visual morbidity by induction of amblyopia, strabismus, significant refractive error or optic nerve compromise. Diagnosis is typically straightforward but occasionally other entities may cause diagnostic confusion and radiologic evaluation can be helpful. This is particularly important if the hemangioma is one component of the PHACES syndrome. Therapeutic options which may be useful include steroids (oral, intralesional or topical), interferon alpha (usually reserved for life- or sight-threatening lesions due to serious potential side effects), laser, embolization and surgery. Ophthalmic treatment using patching, atropine, glasses and stabismus surgery may be necessary.