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Editor—Neonates with mandibulo–facial anomalies and respiratory distress present a challenge for neonatologists. We report a newborn boy with severe micrognathia who failed to breathe adequately immediately after birth. Tracheal intubation was unsuccessful, but he was ventilated for several hours using a laryngeal mask.
The boy was born to a 29 year old primigravida after 39 weeks of gestation. During the pregnancy, absence of the corpus callosum had been noted at 33 weeks of gestation, and genetic amniocentesis had indicated a microdeletion of chromosome 21. No other malformation had been verified. Caesarean section was performed because of intrauterine growth retardation and breech presentation.
At birth the boy weighed 2300 g, had multiple contractures of the limbs, bilateral coloboma of the iris, severe mandibular hypoplasia with a small oral orifice and a massive glossoptosis, and a systolic heart murmur. He made feeble attempts to cry, but remained cyanotic and bradycardic despite the jaw thrust manoeuvre and bagging. Oro-tracheal intubation with a 3 mm and a 2 mm tracheal tube failed twice. A lubricated number 1 laryngeal mask was easily put into the right position while holding the tongue with a Magill forceps. After sedation with fentanyl and midalozam he tolerated intermittent mechanical ventilation well for three hours while investigations were carried out. At ventilator settings of PI 25 cm H2O, PEEP 4 cm H2O, RR 60 per minute, FIO2 0.4, I:E ratio of 1:2, flow rate of 12 l/min, capillary blood gas analysis was pH 7.3, pCO2 4, pO2 95, base excess −5 mmol/l. Blood pressure and heart rate were within the normal range.
Echocardiography revealed a severely hypoplastic distal aortic arch with a wide open ductus arteriosus, and cranial echocardiography confirmed the absence of the corpus callosum.
In view of the poor prognosis the parents requested the withdrawal of intensive care, and at necropsy the diagnoses were confirmed, with the addition of a micro larynx and a large pharyngo-oesophageal cleft. No signs of damage to the hypophraynx were found.
The use of the laryngeal mask in our patient gained us time to perform investigations to establish prognosis. In patients with isolated cranio-facial and mandibulo-facial malformations such as Goldenhar, Treacher–Collins, or Pierre–Robin syndromes, the laryngeal mask could be used to establish a definite airway either through bronchoscopic intubation or tracheotomy. The usefulness of the laryngeal mask during anaesthesia in such cases has been well documented,1-3 but we believe that the mask could also be a valuable tool for neonatologists who unexpectedly face a difficult airway.