Preoperative metabolic acidosis in infants with gastroschisis.

INTRODUCTION: There is little in literature regarding preoperative management of infants with gastroschisis. It is unclear if these infants develop metabolic acidosis as a consequence of prolonged intrauterine gut compromise or dehydration secondary to increased fluid loss. AIM: To assess the frequency of preoperative metabolic acidosis in infants with gastroschisis and investigate whether this acidosis reflects degree of gut compromise. METHODS: All infants with gastroschisis born between May 2005 and April 2013 in a single tertiary care center were reviewed. Metabolic acidosis was defined by the presence of pH <7.26 and serum bicarbonate <18.5 or base excess < -8.5 mmol/l. Infants with significant birth depression were excluded. Maternal and neonatal data were collected. Frequency of preoperative metabolic acidosis and its association with gastroschisis prognostic score (GPS), time to first and time to reach full feeds were investigated. RESULTS: Sixty infants were identified, 11 were excluded (birth depression/lack of preoperative blood gases). Median preoperative total fluid intake was 130 ml/kg/d. Nine infants (18%) had metabolic acidosis at a median age of 1.2 hours. No association was found between metabolic acidosis or serum lactate and GPS, age at first feed or age at full feeds. CONCLUSION: Preoperative metabolic acidosis was identified in a significant number of patients with gastroschisis despite high fluid intake. It does not appear to be associated with the degree of gut compromise. Using metabolic acidosis as an indication of dehydration in these patients needs more investigation.

Severe laryngeal stenosis in newly born twins with 22q11.2 deletion syndrome: A case report.

Chromosome 22q11.2 deletion syndrome is common and presents with a range of clinical features from cardiac malformations to hypocalcemia. Laryngeal anomalies are not a common feature of this syndrome. We describe newly born twins who presented with unexpected severe birth depression secondary to severe type IV glottic webs requiring extensive resuscitation and emergency tracheostomy. They were diagnosed postnatally to have deletion of 22q11.2. The successful resuscitation of these infants at birth was only possible because they were born in a tertiary care hospital. This report shows the critical nature of prenatal diagnosis of 22q11.2 deletion syndrome.

Coexistence of meconium ileus with duodenal atresia and trisomy 21 in a newborn: a case report.

The coexistence of duodenal atresia (DA) may mask the antenatal ultrasound findings of meconium ileus (MI) and delay its postnatal diagnosis. We report a rare case of MI in a newborn infant diagnosed antenatally to have trisomy 21 and DA. The diagnosis of MI was only established intraoperatively after the patient showed persistent signs of intestinal obstruction following the surgical repair of the DA.

Delivery room resuscitation of preterm infants in Canada: current practice and views of neonatologists at level III centers.

OBJECTIVE: To explore physicians' experiences and views related to resuscitation practice of preterm infants at birth, and determine whether the Canadian modifications of 2006 Neonatal Resuscitation Program (NRP) guidelines have been accepted by neonatologists. STUDY DESIGN: Neonatologists (n=146) at 25 tertiary neonatal intensive care units (NICUs) across Canada were contacted via email to participate in a web-based survey about their practice regarding resuscitation of preterm infants in the delivery room (DR). RESULT: In all, 78 respondents (53%) from 23 centres completed the survey. Participants reported significant variability in temperature control measures. Hypothermia, <36.5 °C on NICU admission, was reported by 49% of respondents. Room air is used by 59% of respondents to initiate resuscitation. The majority (91%) of participants use pulse oximetry to titrate oxygen administration. Although more than two thirds (69%) of respondents target an oxygen saturation range of 85 to 92%, 51% of respondents would allow 5 to 10 min for the oxygen saturation to reach the target level. Carbon dioxide detectors are commonly used to confirm endotracheal tube placement (90%). Although respondents (96%) agree on the use of positive end- expiratory pressure (PEEP), when providing positive pressure ventilation (PPV), only 60% would initiate PPV with a pre-set peak inspiratory pressure, mostly 20 cm H(2)O. CONCLUSION: DR resuscitation practices are highly variable in Canadian NICU's and the currently recommended NRP guidelines are not uniformly followed. Factors leading to variability and discordance in practice should be investigated to facilitate better compliance.

Phentolamine as a treatment for poor mixing in transposition of the great arteries with adequate intraatrial communication.

Patients with transposition of the great arteries often show poor mixing for different reasons, even after adequate balloon atrial septostomy. We present a patient with such a lesion whose clinical status improved dramatically after phentolamine was applied. We believe this improvement is due to reduction in afterload caused by the alpha(2) blocker and also possibly as a response to a presumptive effect of the drug on the diastolic function of the right ventricle, allowing more left-to-right shunt across the atrial septal defect. Both phenomena can improve cardiac output in such a situation.