Megalencephaly-capillary malformation-polymicrogyria syndrome: the first case report in Korea / 소아과

Megalencephaly-capillary malformation-polymicrogyria syndrome (MCAP), previously known as macrocephaly-cutis marmorata telangiectatica congenita and macrocephaly-capillary malformation syndrome, is a rare multiple-malformation syndrome that is characterized by progressive megalencephaly, capillary malformations of the midline face and body, or distal limb anomalies such as syndactyly. Herein, we report a female infant case that satisfies the recently proposed criteria of MCAP and describe the distinctive neuroradiological and morphological features. We have also reviewed recently published reports and the diagnostic criteria proposed by various authors in order to facilitate the clinical diagnosis of these children in pediatric neurology clinics.

Clinical Characteristics of Patients with Bronchopulmonary Dysplasia Admitted to Pediatric Intensive Care Unit

PURPOSE: As the incidence of bronchopulmonary dysplasia (BPD) has increased, it is important to understand the clinical outcomes of BPD patients discharged from neonatal intensive care units (NICU). The purpose of our study was to describe the characteristics of BPD patients who are re-hospitalized in a pediatric intensive care unit (PICU) and to evaluate the prognostic outcome factors. METHODS: We retrospectively reviewed the medical records of BPD patients who were admitted to our PICU between May 2006 and November 2014. In total, we identified 101 cases which were divided into two groups, group 1, those who required intensive care for an acute illness or disease aggravation (n=62), and group 2, those who were admitted for post-operative care unrelated to having BPD as a control group (n=39). We subsequently compared the characteristics. RESULTS: Most patients in group 1 were aged less than 1 year, with weight below the 3rd percentile for age at the time of their PICU admission. The main cause for their admission was respiratory failure, requiring mechanical ventilation. When comparing the two groups, group 1 showed higher gestational age at birth, and a longer duration of mechanical ventilation, oxygen support, and NICU hospitalization than group 2. However, we failed to identify any factor significantly associated with the duration of the PICU stay, hospital stay, and mortality. Further large-scale, long-term follow-up studies will be necessary. CONCLUSION: As the majority of patients are admitted to PICU because of respiratory symptoms during their infantile period, careful follow-up with supportive care and prevention of respiratory infection are required.