Fifteen-minute consultation: When to consider extracorporeal membrane oxygenation.

Extracorporeal membrane oxygenation (ECMO) is a form of organ support which is used for severe, potentially reversible respiratory, cardiac or cardiorespiratory failure. While it is associated with significant risk of intracerebral injury in neonates and children, outcomes can be excellent, and timely referral is associated with improved survival and reduced morbidity. This article provides a concise summary of the technical aspects of ECMO support, indications for referral, complications, outcomes and important considerations for follow-up.

Mechanisms of ventilator dependence in children with neuromuscular and respiratory control disorders identified by monitoring diaphragm electrical activity.

OBJECTIVES: To report on the monitoring of diaphragm electrical activity (Edi) using neurally adjusted ventilator assist (NAVA) technology to investigate the mechanisms of ventilator dependence in children with neuromuscular and respiratory control disorders. PATIENTS AND METHODS: Using NAVA technology, electrical activity of the diaphragm (Edi) was monitored at the lowest achievable level of respiratory support in six ventilator-dependent patients with neuromuscular and respiratory control disorders, aged 6 weeks to 12 years, admitted to a tertiary paediatric intensive care unit between 2009 and 2011. RESULTS: Edi monitoring identified markedly abnormal respiratory dynamic patterns that were not always apparent clinically. These were associated with disorders of central respiratory control, muscle weakness and diaphragm pathology. CONCLUSIONS: Edi monitoring using NAVA technology is a valuable, minimally invasive, diagnostic adjunct in children with neuromuscular and respiratory control disorders who are ventilator-dependent.

Cor pulmonale due to congenital central hypoventilation syndrome presenting in adolescence.

OBJECTIVE: To report the first case of congenital central hypoventilation syndrome (CCHS) presenting with severe cor pulmonale in an adolescent. METHODS AND DESIGN: Case report and literature review. Our Institutional Review Board waived the need for consent. SETTING: Pediatric intensive care unit in a tertiary care children's hospital. PATIENT: A 12-year-old girl who developed profound hypoxia following routine dental extraction under intravenous opiate sedation and became progressively obtunded due to marked hypoventilation without hypoxic arousal, requiring mechanical ventilation. She had evidence of severe right heart failure, but no cardiac, pulmonary, neurologic, or neuromuscular cause was identified. The diagnosis of CCHS was suspected and subsequently confirmed by blood polymerase chain reaction analysis that revealed a heterozygous polyalanine expansion mutation of the PHOX2B gene (five polyalanine repeats). CONCLUSIONS: This report describes the unusual presentation of severe cor pulmonale in an adolescent with so-called "late-onset" CCHS. CCHS was previously thought to be a disease affecting only neonates, but the late-onset phenotype has now been well described in adults. It should be considered in any child presenting with unexplained right heart failure without an identifiable cause, particularly if central sleep apnea is present, because early initiation of ventilatory support can prevent cardiac and neurologic sequelae and improve outcome.