These preliminary results suggest that silicone IOL implantation in children is a safe procedure with good and stable short-term anatomic results. Longer follow-up is necessary to answer questions about the long-term safety of silicone lens implantation in a child's eye.

De novo heterozygous mutations changing R179 to histidine, leucine, or cysteine in the ACTA2 gene are associated with Multisystemic Smooth Muscle Dysfunction Syndrome (MSMDS). Characteristic hallmarks of this condition, caused only by these specific ACTA2 mutations, are congenital mydriasis (mid-dilated, non-reactive pupils), a large persistent ductus arteriosus (PDA), aortic aneurysms evolving during childhood, and cerebrovascular anomalies. We describe two patients, a 3-day-old newborn and a 26-year-old woman, with this unique mutation in association with a huge PDA and an aorto-pulmonary window. In addition, one showed a coarctation of the aortic arch and the other a complete interruption of the aortic arch type A; thereby expanding the spectrum of cardiac congenital heart defect of this syndrome. Each patient displayed a huge PDA and an extra-cardiovascular phenotype consistent with MSMDS. These observations exemplify that a functional alpha 2 smooth muscle actin is necessary for proper cardiovascular organ development, and demonstrate that a very exceptional congenital heart defect (aortopulmonary window) can be caused by a mutation in a gene encoding a contractile protein of vascular smooth muscle cells. © 2017 Wiley Periodicals, Inc.

The authors have brought to our attention an error in their article title. R189H should be replaced with R179H. The corrected article title follows, "Two patients with the heterozygous R179H mutation in ACTA2 and Complex congenital heart defects expands the cardiac phenotype of multisystemic smooth muscle dysfunction syndrome". The authors and publisher regret this error.2566 |

The high incidence of PONV after BMF can be explained by the greater invasiveness of BMF than R&R surgery. PONV requiring antiemetic rescue medication can be reduced by preoperative administration of dimenhydrinate suppositories.

PurposeTo evaluate the outcomes of surgery for Brown syndrome.MethodsWe reviewed the charts of 15 patients who underwent surgery for Brown syndrome. The limitation of elevation in adduction (LEA) ranged from -2 to -4 degrees. A superior oblique muscle (SO) tenotomy was performed in 4 patients, a silicone expander was inserted in the SO of 9 patients, and a SO recession was performed in 2 patients. The results of surgery were analyzed with a follow-up period of more than 6 months, 42.3±48.42 months on average.ResultsNine female patients and 6 male patients with unilateral Brown syndrome were selected for this study. The left eye was the affected eye in 9 patients. The degree of preoperative LEA was -2 to -4 in 4 patients in whom SO tenotomy was performed, -3 to -4 in 9 patients treated with the silicone expander, and -2 to -4 in 2 patients treated with SO recession. The LEA was released after surgery in all patients without postoperative adhesion. However, unilateral overaction of the inferior oblique muscle due to excessive weakening of the SO occurred in 1 patient with tenotomy (25%) and in 1 patient with insertion of a silicone expander (11%).ConclusionsLEA was released after tenotomy, insertion of a silicone expander and recession of the SO in 13 of 15 patients with Brown syndrome. SO palsy due to overcorrection and under-correction with postoperative adhesion should be avoided.