Irene H. Ludwig

Congenital Central Hypoventilation Syndrome: Ocular Findings in 37 Children

BACKGROUND Congenital central hypoventilation syndrome (CCHS) is a rare cause of central sleep apnea. Although ophthalmic abnormalities have been reported, the ocular findings have not been discussed in detail. METHODS We examined or obtained the records of 37 children with CCHS. RESULTS Twenty-seven patients were found to have abnormal pupils, most of which were miotic and reacted poorly to light. In 18 cases, the anterior surface of the iris was unusually smooth. Ten of the children with abnormal pupils also demonstrated light-near dissociation. Twenty had strabismus of various types, and 18 showed evidence of convergence insufficiency. CONCLUSIONS The high incidence of strabismus, pupillary abnormalities, and convergence insufficiency may be a result of neurologic defects in the midbrain.

Low-dose Methotrexate in the Treatment of Severe Juvenile Rheumatoid Arthritis and Sarcoid Iritis

OBJECTIVE To assess the efficacy of low-dose oral methotrexate (MTX) therapy for children with severe iritis. METHODS MTX in a weekly dose of 7.25 to 12.5 mg/m2 was administered orally to four patients (two with juvenile rheumatoid arthritis [JRA] and two with sarcoidosis) with severe iritis not adequately controlled by topical and systemic corticosteroid therapy. The treatment was initiated with half of the total dose and increased every 2 weeks until the final dose was reached. Iritis was graded from 0 to +4 according to the density of cells in the anterior chamber of the eye. RESULTS There were three girls and one boy with a mean age of 10.5 years. Two patients were African American and two were Caucasian. The mean age at onset of iritis was 6 years. The mean duration of MTX therapy was 28.8 months. Significant improvement was noted in two of the four patients in ocular inflammation, demonstrated by reduction of cell density from +4 to +1. Two patients had a mild improvement of the iritis. However, corticosteroids were significantly reduced in all patients. One patient was completely off steroids within 30 months of MTX therapy. In the remaining three cases, the steroid dose was successfully tapered from 0.82 mg/kg/d to 0.15 mg/kg/d (mean doses) within a mean duration of 20 months. No side effects were observed with MTX therapy. CONCLUSION Low-dose MTX therapy was effective and safe, and displayed steroid-sparing properties in four children with severe iritis.

Rate of deterioration in accommodative esotropia correlated to the AC/A relationship.

We analyzed the claim that accommodative esotropia tends to deteriorate with greater frequency if the accommodation convergence relationship (AC/A) is high. Records of 119 patients whose eyes were aligned with spectacles alone were studied. Their AC/A relationships were graded according to the difference between the distance and near measurements: normal included 0 to 9 prism diopters (delta) difference; grade 1 ranged from 10 to 19 delta difference; grade 2 from 20 to 29 delta difference; and in grade 3 the difference was 30 delta or greater. Deterioration is characterized by a nonaccommodative component of esotropia greater than 10 delta at distance becoming superimposed on the initial accommodative esotropia. Deterioration occurred in 7.7% of patients with a normal AC/A, 25% with grade 1 high AC/A, 44% with grade 2 high AC/A, and 52% with grade 3 high AC/A. Hypotheses were investigated using chi square, t-test, analysis of variance (ANOVA), and log linear analyses. Distributional differences were highly significant by chi square test (p = 0.001) with a rejection of the null hypothesis of no difference between the groups at the alpha = 0.05 level. An alternate analysis of average AC/A ratio in the deteriorated versus nondeteriorated patients was equally statistically significant by the t-test. Hypermetropia was significantly higher in the normal AC/A group. Multi-factor comparisons showed that time-to-deterioration, treatment delay, age of onset, and amblyopia were factors that did not relate significantly to the incidence of deterioration.

Long-term results of bifocal therapy for accommodative esotropia.

We studied the long-term course of 65 accommodative esotropes who required bifocals to maintain alignment at near. Average follow-up was 10.5 years. Forty patients (61.5%, group DC [bifocals discontinued]) were able to discontinue bifocal use after an average of 5.5 years wear. Twenty-five (38.5%) continued to wear bifocals (or a suitable alternative such as reading glasses), after an average 9.7 years of follow-up. Surgical correction of deteriorated accommodative esotropia was performed for 20 patients (50%) in group DC, and nine (36%) of those in group C[bifocals continued]. Surgery produced an average reduction in the accommodative convergence relationship (near esodeviation in prism diopters [pd] minus corrected distance measurement, AC/A) of approximately 10 pd in both groups. Surgical patients unable to discontinue bifocal wear began with a clinically higher AC/A than those in group DC. Non-surgical patients in group DC experienced spontaneous improvement of the AC/A over time (average, 6.2 pd). On average, this did not occur in those of group C. Average age of bifocal discontinuation was 9.7 years in surgical patients and 9.3 years in the non-surgical. Surgical patients had significantly lower hyperopia (+2.4 diopters [D]), than non-surgical (+3.5 D), and an earlier age of onset of bifocal wear (3.29 versus 4.64 years). Although bifocals may be successfully discontinued in a majority of patients at an average age of 9.5 years, a significant percentage require long-term wear, some, despite surgery. The only factor that predicted long-term bifocal wear was a relatively high AC/A.

Ideal Wound Healing Is Not a Stretch

From Eye Center South, Dothan AL, and Eye Health Partners, Nashville, Tennessee (IHL); the Department of Surgery, Section of Plastic Surgery, White Plains Hospital, White Plains, New York (RSR); and the Department of Ophthalmology and Visual Science, Albert Einstein College of Medicine, Bronx, New York (FMW). The authors have no financial or proprietary interest in the materials presented herein. Correspondence: Irene H. Ludwig, MD, 3215 Kinnard Springs Road, Franklin, TN 37064. E-mail: ihludwig@aol.com doi:10.3928/01913913-20180126-01 Editor’s Note: Most chefs don’t get involved in the clean-up process. Bon Appétit!!