Neurally adjusted ventilatory assist [NAVA] mode as an adjunct diagnostic tool in congenital central hypoventilation syndrome

A full term female newborn was admitted to the neonatal intensive care unit [NICU] for continuous observation of apnea. Infant was noted to have apnea while asleep requiring intubation and mechanical ventilation. A video EEG was performed which demonstrated normal awake background without any seizure activity. Neurally adjusted ventilatory assist [NAVA] demonstrated the absence of electrical activity of the diaphragm [Edi] when the patient was in quiet phase of sleep. This finding on NAVA monitor raised the suspicion of central hypoventilation syndrome [CCHS] which was confirmed by genetic identification of the PHOX2B mutation

Congenital epulis

Epulis is a rare tumour, with female preponderance that is only seen in the newborns. It arises from the mucosa of the gingiva and protrudes out of the infant's mouth. It can potentially obstruct the airways and may require an EXIT [ex-utero intrapartum treatment] procedure which involves establishing an airway before the feto-maternal circulation is interrupted. We present a female newborn with such a mass, which was diagnosed antenatally. A multidisciplinary team including the neonatologist, anaesthesiologist and ENT specialist should be present in the delivery room to establish the airways, which may require an EXIT procedure. Recommended treatment is early surgical resection. Recurrences of the tumour and damage to future dentition have not been reported, suggesting that radical excision is not warranted

Neurally adjusted ventilatory assist mode used in congenital diaphragmatic hernia

A term baby with congenital diaphragmatic hernia [CDH] underwent surgical repair on the second day of life. Postoperatively; the oxygenation index increased to 85 despite high pressure ventilation with HFOV [high frequency oscillator ventilation] and inhaled nitric oxide therapy. Oxygenation index above 70 carries a mortality rate of 94% and merits starting extracorporeal membrane oxygenation [not available in the UAE]. A trial of neurally adjusted ventilatory assist [NAVA] on the 10th postoperative day was followed by a reduction of oxygenation index to 15 and marked improvement of the clinical parameters. The EAdi [electrical activity of diaphragm] signal was relatively weak [ +/- 5 micro volt] requiring augmentation witha high NAVA level [3 - 3.5]. The patient was successfully extubated after 3 weeks