Infantile Hemangiomas Masquerading as Other Periocular Disorders

Periocular tumors are common in infancy. The most common periocular tumors are capillary hemangiomas, which are present in 1-2% of newborns and develop in 10%–12% of children by the age of 1 year old. Deep capillary hemangiomas may be more challenging to diagnose than superficial capillary hemangiomas and can be confused with other orbital lesions. Deep orbital hemangiomas can mimic teratoma, lymphangioma, rhabdomyosarcoma, metastatic neuroblastoma, and granulocytic sarcoma. In this paper, we describe 2 pediatric cases where previously diagnosed dermoid cyst and dacrocystocele were found to be capillary hemangiomas upon biopsy. Approaches to distinguish capillary hemangiomas from other periocular tumors are further discussed. To our knowledge, this is the first case report of periocular hemangiomas imitating a dermoid cyst and a dacrocystocele. These cases emphasize the importance of including infantile hemangiomas in the differential diagnosis of subcutaneous periocular abnormalities

The Relationship of Age and Other Baseline Factors to Outcome of Initial Surgery for Intermittent Exotropia

Purpose: To determine whether age at surgery is associated with surgical outcome of intermittent exotropia (IXT) at 3 years. Design: Secondary analysis of pooled data from a randomized trial. Methods: A total of 197 children 3 to <11 years of age with basic-type IXT of 15-40 prism diopters (Δ) were randomly assigned to 1 of 2 surgical procedures for treatment of intermittent exotropia. Masked examinations were conducted every 6 months for 3 years. The primary outcome was suboptimal surgical outcome by 3 years, defined as constant or intermittent exotropia of ≥10 Δ at distance or near by simultaneous prism and cover test (SPCT); constant esotropia of ≥6 Δ at distance or near by SPCT; or decrease in near stereoacuity of ≥2 octaves, at any masked examination; or reoperation without meeting any of these criteria. Results: The cumulative probability of a suboptimal surgical outcome by 3 years was 28% (19 of 72) for children 3 to <5 years of age, compared with 50% (57 of 125) for children 5 to <11 years of age (adjusted hazard ratio = 2.05; 95% confidence interval = 1.16 to 3.60). No statistically significant associations were found between suboptimal outcome and other baseline factors (magnitude of deviation, control score, fixation preference, or near stereoacuity) (P values ≥ .20). Conclusions: This analysis suggests that in children with IXT, younger age at surgery (3 to <5 years) is associated with better surgical outcomes; however, further evidence from a randomized trial comparing immediate with delayed surgery is needed for confirmation

Acquired Nonaccommodative Esotropia in Childhood

Purpose: Acquired nonaccommodative esotropia (ANAET) in childhood is reported to occur infrequently and is often associated with an underlying neurologic or neoplastic disorder. The primary objective of this study was to ascertain the prevalence and clinical characteristics of this form of childhood esotropia. Methods: A cohort of all children younger than 11 years with esotropia from a predominantly rural Appalachian region was prospectively identified from August 1, 1995, through July 31, 1998. The age at onset, family history of strabismus, perinatal and medical history, ophthalmologic findings, and surgical results were reviewed for all patients with ANAET. Results: Twenty-three (10.4%) of 221 consecutive children with esotropia were diagnosed with ANAET compared with 12 (5.4%) diagnosed with congenital esotropia. The median age at esotropia onset for the 23 children with ANAET was 31.4 months (range, 8-63 months) with a mean initial angle of esotropia of 24 PD. Although at least 2 children presented with diplopia, none of the 23 patients were known to have harbored intracranial tumors or other lesions of the central nervous system during the follow-up period. Fourteen of the 19 patients who underwent surgery attended follow-up visits for at least 6 months after their last surgical procedure: 13 were within 8 PD or less of orthotropia, whereas the final patient had persistent esotropia. Twelve of the 13 patients within 8 PD of orthotropia demonstrated some level of stereopsis, including 2 children with bifoveal fixation. Two (10.5%) of the 19 operated patients later required a low hyperopic spectacle correction to control their deviation. Conclusions: ANAET was more prevalent than congenital esotropia in this cohort of children with esotropia. This clinically distinct form of strabismus typically begins between 1 and 5 years of age and appears to be infrequently associated with underlying disease. The angle of deviation is relatively small and early surgical correction is more likely to achieve bifoveal fixation for these patients than for those with congenital esotropia

Common Forms of Childhood Esotropia

Objective: To determine the most common forms of childhood esotropia. Design: Prospective, consecutive, observational case series. Participants: All esotropic children younger than 11 years of age from a predominantly rural Appalachian region evaluated from August 1, 1995 through July 31, 1998. Methods: Demographic and clinical data were collected for all patients. Main Outcome Measures: The percentage ratio of the various forms of childhood esotropia. Results: Two hundred twenty-one consecutive children without prior surgical treatment were evaluated for esotropia. One hundred seventeen (52.9%) of the 221 children had some form of accommodative esotropia, 38 (17.2%) were associated with congenital or acquired abnormalities of the central nervous system, 23 (10.4%) displayed acquired nonaccommodative esotropia, 15 (6.8%) resulted from ocular sensory defects, 12 (5.4%) had confirmed congenital esotropia, seven (3.2%) had paralytic esotropia, and an unverified age at onset prevented an accurate categorization in the remaining nine (4.1%). Conclusions: Children with accommodative esotropia accounted for more than half of the study patients and were diagnosed nearly 10 times more frequently than children with congenital esotropia. Esotropic patients with central nervous system defects or with an acquired nonaccommodative deviation were also more common than children with congenital esotropia. Children with congenital esotropia or with a paralytic or sensory cause of their deviation were relatively uncommon

Cystoid Macular Edema Following Extraocular Muscle Surgery

Cystoid macular edema is a nonspecific response to a variety of stimuli and is characterized by an accumulation of fluid in the outer plexiform and inner retinal layers of the perifoveal region. This abnormal collection of fluid has been associated with intraocular surgery, retinal vascular disease, intraocular tumors, hereditary ocular conditions, inflammatory disorders, and the topical use of certain medications. Cystoid macular edema following cataract extraction is the most common postoperative presentation of this disorder, although it has been reported in other types of ophthalmic surgery. This report, to the best of our knowledge, describes the first known case of cystoid macular edema following uncomplicated extraocular muscle surgery in a phakic elderly patient

Common Forms of Childhood Exotropia

Objective: To determine the most common forms of childhood exotropia. Design: Retrospective, consecutive, observational case series. Participants: All exotropic children (with ≥10 prism diopters) younger than 19 years from a predominantly rural Appalachian region evaluated from August 1, 1995 through July 31, 2001. Methods: Demographic and clinical data were collected on all patients. Main Outcome Measures: The relative proportion of the various forms of childhood exotropia. Results: Two hundred thirty-five consecutive children without prior surgical treatment were evaluated for exotropia. Of the 235 study children, the specific forms of exotropia diagnosed and numbers were as follows: intermittent exotropia, 112 (47.7%); exotropia associated with congenital or acquired abnormalities of the central nervous system (CNS), 50 (21.3%); convergence insufficiency, 27 (11.5%); sensory exotropia, 24 (10.2%); paralytic exotropia, 5 (2.1 %); congenital exotropia, 4 (1.7%); neonatal exotropia that resolved after 4 months of age, 3 (1.3%), whereas the remaining 10 (4.3%) had an undetermined form of exodeviation. Conclusions: Intermittent exotropia was the most common form of divergent strabismus in this population. Exotropia associated with an abnormal CNS, convergence insufficiency, and sensory exotropia were also relatively common, whereas the congenital, paralytic, and late-resolving neonatal forms were uncommon

Second Nonocular Tumors in Survivors of Heritable Retinoblastoma and Prior Radiation Therapy

PURPOSE: The principal objectives of this study were to estimate the incidence of second tumors among children treated for heritable retinoblastoma during a 50-year period and to investigate the relationship between these tumors and previous radiation therapy. METHODS: The records of all retinoblastoma patients examined at the Mayo Clinic from 1941 through 1990 were retrospectively reviewed. The therapeutic modality used to manage the tumor, the occurrence of any second malignancy, and current follow-up on all patients were evaluated. RESULTS: Eighty-two (46%) of 180 children with retinoblastoma had bilateral tumors (76 patients) or unilateral disease and a positive family history (six patients) and were followed for an average of 21.8 years (range, 1 month to 53 years). The Kaplan-Meier estimates of second nonocular tumors among the 82 patients with heritable retinoblastoma were 12% at 10 years, 16% at 25 years, and 30% at 40 years. Although 14 of the 15 patients who developed second malignancies had received radiation therapy, only four of the malignancies occurred within the field of irradiation. CONCLUSIONS: The relatively low incidence of second tumors among long-term survivors of heritable retinoblastoma in this series of patients occurred predominantly outside the field of irradiation. The variable incidence of second nonocular malignancies in previous reports may reflect variations in radiation technique and dosage

Congenital esotropia in Olmsted County, Minnesota

Objective: To determine the birth prevalence of and risk factors associated with congenital esotropia. Design: Population-based prevalence study with nested case-control study. Participants: All residents of Olmsted County, Minnesota who were diagnosed with congenital esotropia and born between January 1, 1980 and December 31, 1989 (n = 47). Control subjects were chosen by selecting the next two sequential births to parents residing in Olmsted County, Minnesota (n = 94). Methods: Cases were identified through the Medical Diagnostic Index of Mayo and the Rochester Epidemiology Project. The community medical records were reviewed to confirm case status and ascertain risk factor information. Main Outcome Measure: Birth prevalence of congenital esotropia. Results: Forty-seven cases were identified from 17,536 live births, for a birth prevalence of 27 per 10,000 (95% confidence interval [Cl], 20-35). Congenital esotropia was associated with prematurity (odds ratio [OR], 11.5; 95% Cl, 3.4-39.2), a birth weight less than 2500 grams (OR, 4.6; 95% Cl, 1.7-12.9), a low Apgar score at 1 minute (OR, 4.3; 95% Cl, 1.7- 11.2) and at 5 minutes (OR, 6.3; 95% Cl, 1.3-30.7), and a family history of strabismus (OR, 3.5; 95% Cl, 1.5-8.3). Conclusions: The birth prevalence of congenital esotropia in Olmsted County, Minnesota is lower than previous estimates. Prematurity, low birth weight, low Apgar scores, and a family history of strabismus are significant risk factors for congenital esotropia