Neonatal Characteristic of Congenital Cystic Adenomatoid Malformation of the Lung Requiring Early Operation and Preoperative Intervention

PURPOSE: To identify neonatal characteristics associated with congenital cystic adenomatoid malformation (CCAM) who required early operations and to introduce preoperative interventions to delay definitive operations until stabilized. METHODS: A retrospective review of dataset was performed from January 2000 to December 2011 for neonates admitted to NICU at Asan Medical Center with prenatally diagnosed CCAM. Variable prenatal and postnatal factors were compared for those who required early operations to those asymptomatic neonates who required elective operations at later age. RESULTS: A total of 60 patients were enrolled and patients were divided into 2 groups according to time of operation. Median time of surgery for group 1 (n=12, 20%) and group 2 (n=48, 80%) was 5.5 days and 504 days, respectively. Maternal characteristics including age, parity, preterm labor, oligohydramnios were similar in between two groups. Factors associated with early operation included prenatal history of polyhydramnios (OR 23, P=0.001), who had undergone fetal interventions (OR 47, P=0.001), low 1 and 5 minute Apgar scores and increasing fetal fluid-filled cystic sizes (OR 26, P=0.013). Of those 3 neonates who required preoperative interventions to decrease air-filled cysts to relieve initial respiratory symptoms were successful and all underwent for definitive operations during NICU hospitalizations. All survived. CONCLUSION: Most neonates with CCAM undergo elective operations during infancy. However, about 20% neonates with polyhydramnios, fetal intervention, increasing fetal cystic mass and born with low Apgar scores required early operations for whom prenatal and postnatal interventions prior to definitive surgery can improve survival rate.

Persistent Pulmonary Hypertension of the Newborn in a Preterm Infant Exposed to Methotrexate During the Early Gestational Period / 대한주산의학회잡지

Methotrexate (MTX) is used in the reproductive aged females for the management of medical conditions such as ectopic pregnancy, autoimmune diseases and malignancies. Because of its antimetabolite effect, exposure to MTX during the fetal period can cause multiple anomalies. The most common anomalies related to intrauterine MTX exposure include growth retardation, craniofacial dysmorphism, central nervous system anomalies, cardiac anomalies and skeletal defects. We report a premature baby boy born after 27(+5) weeks of gestation who presented intrauterine growth restriction, single umbilical artery, small chest and anomalies of rib and thoracic vertebra. His mother had received 50 mg of MTX for the treatment of misdiagnosed ectopic pregnancy at 5th week of gestation. During the hospitalization, he was ventilator dependent and pulmonary hypertension persisted despite medical treatment including nitric oxide and sildenafil. Open lung biopsy revealed nonspecific findings suggestive of lung hypoplasia. He died at 141 days after birth due to respiratory failure.