Parenteral Nutrition-Associated Cholestasis in Very Low Birth Weight Infants: A Single Center Experience / 대한소아소화기영양학회지

PURPOSE: Parenteral nutrition (PN)-associated cholestasis (PNAC) is one of the most common complications in very low birth weight infants (VLBWIs). The aim of this study is to evaluate the risk factors of PNAC in VBLWIs. METHODS: We retrospectively reviewed the medical records of 322 VLBWIs admitted to the neonatal intensive care unit of our hospital from July 1, 2009 to December 31, 2013. We excluded 72 dead infants; 6 infants were transferred to another hospital, and 57 infants were transferred to our hospital at 2 weeks after birth. The infants were divided into the cholestasis and the non-cholestasis groups. PNAC was defined as a direct bilirubin level of ≥2.0 mg/dL in infants administered with PN for ≥2 weeks. RESULTS: A total of 187 VLBWI were enrolled in this study; of these, 46 infants developed PNAC. Multivariate logistic regression analysis showed that the risk factors of PNAC in VLBWI were longer duration of antimicrobial use (odds ratio [OR] 4.49, 95% confidence interval [95% CI] 4.42-4.58), longer duration of PN (OR 2.68, 95% CI 2.41-3.00), long-term lack of enteral nutrition (OR 2.89, 95% CI 2.43-3.37), occurrence of necrotizing enterocolitis (OR 2.40, 95% CI 2.16-2.83), and gastrointestinal operation (OR 2.19, 95% CI 2.03-2.58). CONCLUSION: The results of this study suggest that shorter PN, aggressive enteral nutrition, and appropriate antimicrobial use are important strategies in preventing PNAC.

A Case of Galloway-Mowat Syndrome with Classic Clinical Triad in the Neonatal Period / 대한주산의학회잡지

Galloway-Mowat syndrome (GMS) is a rare autosomal recessive disorder comprising of early-onset nephrotic syndrome and central nervous system involvement including microcephaly, seizure and developmental delay. Although hiatal hernia is no longer considered essential findings for diagnosis, clinical triad of GMS included nephrotic syndrome, neurological manifestations, and hiatal hernia in the original description. We experienced a case of newborn with GMS presenting these clinical triad in neonatal period. A male infant weighing 2,250 g was born at gestational week 39+3 by cesarean section. The patient revealed mild dysmorphic facial features and microcephaly. On day 7, Nissen fundoplication was done because of hiatal hernia with gastric volvulus. At the age of 2 weeks he developed nephrotic syndrome with proteinuria and hypoalubuminemia. This is the first case of GMS that three classic findings were present in neonatal period in Korea.

Neonatal Iliopsoas Abscess: The First Korean Case

Iliopsoas abscess (IPA) is rare in neonates. We present a case of neonatal IPA that was initially believed to bean inguinal hernia. A 20-day-old male infant was referred to our hospital for herniorrhaphy after a 2-day history of swelling and bluish discoloration of the left inguinal area and leg without limitation of motion. Abdominal and pelvic ultrasonography suggested a femoral hernia, but the anatomy was unclear. Abdominal computed tomography revealed a multi-septated cystic mass extending into the psoas muscle from the lower pole of the left kidney to the femur neck. Broad spectrum antibiotics were initiated, and prompt surgical exploration was planned. After opening the retroperitoneal cavity via an inguinal incision, an IPA was diagnosed and surgically drained. Culture of the abscess fluid detected Staphylococcus aureus, sensitive to methicillin. The patient was discharged without complication on the 17th postoperative day.

Clinical Characteristics of Meconium Aspiration Syndrome according to Ventilator Care / 대한주산의학회잡지

PURPOSE: Meconium aspiration syndrome is an important cause of neonatal morbidity and mortality, and sometimes patients need mechanical ventilator therapy. The aim of this study was to identify the risk factors which required mechanical ventilation. METHODS: We retrospectively analyzed 44 infants diagnosed as meconium aspiration syndrome from January 2009 to December 2013. We divided into 2 groups (ventilator group and oxygen therapy group) and analyzed the clinical characteristics, vital signs, laboratory findings and clinical courses. RESULTS: Ventilator group and oxygen therapy group included each 22 infants, and there were no significant differences in clinical characteristics between two groups. Time to transfer and time to start oxygen therapy were longer in ventilator group but not statistical difference. Initial mean blood pressure and pH were significantly lower in ventilator group than in oxygen therapy group (P=0.026, P=0.003). Ventilator group had longer treatment period and worse prognosis than oxygen therapy group. CONCLUSION: If the infants with meconium aspiration and respiratory distress represent low pH and hypotension, we have to pay close attention to early oxygen supplementation and proper treatment to prevent poor clinical course.

A Case of Cantrell's Syndrome with Left Ventricular Diverticulum in a Neonate / 대한주산의학회잡지

Cantrell's syndrome is a rare congenital anomaly, consists of five malformations. There are the midline abdominal wall defect, the defect of sterna lower part, an agenesis of the anterior part of the diaphragm, an absence of the diaphragmatic pericardium, and the congenital intracardiac problem. We diagnosed Cantrell's syndrome with left ventricular diverticulum in a female neonate with no perinatal problems. She experienced an one-staged operation and discharged with no complications. We report a case with brief review of the literature.

Scalp Abscess Developing Dura Mater Extension in a Newborn Infant : A Case Eeport / 대한주산의학회잡지

The risk factors associated with neonatal scalp abscess include electrode insertion for fetal scalp monitoring, traumatic scalp lacerations, and sepsis or meningitis. We report a case of neonatal scalp abscess with extension to dura mater, although our patient had no known risk factors. A 18-day-old, full-term baby was referred to the our hospital to evaluate a swelling over his left occipital scalp. A full sepsis work-up was performed and all were negative. Cranial sonography revealed echogenic cystic mass with peripheral vascularity consistent with ruptured epidermoid cyst or scalp abscess. The computed tomography (CT) scan of head was performed because of possibility of ruptured epidermoid cyt and revealed 2.0x2.0 cm sized hyperattenuating mass with suspicious intracranial extension. The magnetic resonance imaging (MRI) of head revealed the presence of 1.4x1.1 cm sized peripheral rim enhancing mass at left occipital area consistent with scalp abscess. And also, the lesion was suspicious dural exposure at left temporal area. Clinicians should be aware that scalp abscess may occur without any risk factors and perform diagnostic workup including a complete sepsis evaluation, cerebrospinal fluid analysis (CSF), and cranial imaging study to screen for intracranial extension.

Anorectal Malformations Associated with Esophageal Atresia in Neonates / 대한소아소화기영양학회지

PURPOSE: Anorectal malformations are often associated with other anomalies, reporting frequency with 40-70%. Gastrointestinal anomalies have been known to be relatively less common than associated anomalies of other organ system. This study was performed to assess a distinctive feature of cases associated with esophageal atresia. METHODS: Clinical data (from January 2000 through December 2011) on the 196 subjects with anorectal malformations, managed in our Hospital, were reviewed. Total 14 neonates were identified with accompanying esophageal atresia and retrospective analysis was conducted. RESULTS: The incidence was 7.1% and there were 8 male and 6 female subjects. Only 2 cases were associated with esophageal atresia without tracheoesophageal fistula. Although variable cases of anorectal malformation in female subjects, almost cases were anorectal malformations with rectourethral fistula in male. Other associated anomalies were identified in all cases, with more than 3 anomalies in 10 cases. There were 4 VACTERL (Vertebral abnormalities, Anal atresia, Cardiac anomalies, Tracheoesophageal fistula, Esophageal atresia, Renal and Limb anomalies) associations accounting for 28.6%, but could not identify chromosomal anomaly. Most cases were managed with staged procedure, usually primary repair of esophageal atresia and diverting colostomy. Overall mortality rate was 21.4%, mainly caused by heart problems. CONCLUSION: This study shows that early diagnosis and rational surgical approach with multidisciplinary plan are mandatory in managing anorectal malformations with esophageal atresia, when considering a high frequency of associated anomaly and a relative high mortality.

A Case of Infantile Hepatic Hemangioendothelioma Incidentally Detected during the Evaluation of Galactosemia

Galactosemia is a group of inherited enzyme deficiencies characterized by increase in the blood galactose levels. This condition may be associated with deficiencies of galactose-1-phosphate uridyl transferase, galactokinase, or uridine diphosphate galactose-4-epimerase. However, the elevated galactose identified by neonatal screening tests has several other possible etiologies, including hepatic hemangioendothelioma, hepatic hemangioma, and patent ductus venosus with hypoplasia of the portal vein. We report a 13-day-old Korean male with hepatic hemangioendothelioma, which was incidentally detected during the evaluation for suspected galactosemia. Laboratory studies revealed that mildly elevated levels of galactose, galactose-1-phosphate and alpha-fetoprotein, at the time of admission, were gradually decreased to the normal range over the 6 months of observation. Ultrasonography showed a well-defined heterogeneous hypoechoic mass in the liver, and magnetic resonance imaging study showed multiple enhanced mass lesions, which was compatible with the diagnosis of a hepatic hemangioendothelioma. Thus, hepatic imaging, especially ultrasonography, should be performed if neonatal screening suggests galactosemia.