Perinatal/Neonatal Case Presentation

A novel technique in airway management of neonates with occipital encephalocele


Airway stabilization in neonates with occipital encephalocele (OE) is critical during surgery or if they develop hypoxic-respiratory failure. Endotracheal intubation can be challenging due to difficulty in positioning the head in a patient with large occipital mass. We describe a novel technique for positioning neonates with large OE using a commonly used hospital apparatus which facilitated appropriate positioning of the baby and successful endotracheal intubation with ease and no additional staff.


Encephalocele results from failure of surface ectoderm to separate from the neuroectoderm. Its prevalence is 1 to 4 cases per 10 000 live births.1 Occipital encephalocele (OE) is usually characterized by herniation of rhombic roof elements, cerebellar vermis, caudal third ventricle and distended fourth ventricle through a widened posterior fontanel.

Airway stabilization in neonates with OE is critical during surgery or if they develop hypoxic-respiratory failure. Endotracheal intubation can be challenging due to difficulty in positioning the head in a patient with large OE. They may also have associated intra- or extra-cranial anomalies further complicating airway management. Although OE is rare, anticipating possible challenges associated with airway management is important. We describe a novel technique for positioning a neonate with large OE using a commonly used hospital apparatus, which facilitated appropriate positioning of the baby and successful endotracheal intubation with ease and no additional staff.


Baby B was a male baby with large OE weighing 2.97 kg delivered at LSU Health by cesarean section at 38 weeks gestation with Apgar score of 7 and 9 at 1 and 5 min respectively. The occipital mass measured about 15 × 11 cm. At 3 days of life, he required elective intubation in the neonatal intensive care unit for surgical repair of the OE. The level of the baby’s trunk was raised from the bed by placing him on two rectangular plastic bowls cushioned with warm towels (Figure 1a). The head along with occipital mass was placed over a firm, spongy apparatus called ‘prone headrest’. This apparatus is routinely used to position the head of the adult patients in the operating room if surgical procedures or interventions require them to be placed in a prone position. The OE mass was allowed to pass through the central hollow space in the ‘prone headrest’ (Figure 1b). Care was taken to ensure that the mass was supported and the head and trunk were in the same plane (Figure 1c) while ensuring that the mass was not compressed. The baby’s head was aligned with the trunk as done during routine intubation with no additional manpower. He was pre-medicated with fentanyl 1 mcg kg–1 per dose and midazolam 0.1 mg kg–1 per dose, followed by successful intubation in first attempt without compromising his clinical status. This technique was repeated during subsequent intubation for surgical repair of post-operative wound dehiscence.

Figure 1

(a) View from the top of the apparatus. From left to right: ‘prone headrest’ and the two plastic containers. (b) The occipital mass lying within the space of the ‘prone headrest’, the trunk lying on the plastic container, thus enabling the baby to lie supine with the head in neutral position. Endotracheal tube is seen. (c) Enlarged view of baby’s head and upper trunk. (d) Baby placed inside the isolette after intubation with the mass seen attached to the occiput.


In a series of 118 infants with encephalocele, wherein the average age of repair was 1.5 years, OE constituted two-thirds of them.2 The authors reported difficulty in bag and mask ventilation and intubation in 5.9 and 19.5% of them respectively. Of the patients with OE, 47.5% were intubated in a lateral position, and in those where it was unsuccessful, the head was brought to the edge of the table with an assistant supporting it and another holding the trunk followed by intubation. They also encountered difficulty in bag and mask ventilation in patients with OE during lateral positioning. Similarly, Yildirim et al. reported unsuccessful attempts at intubation by placing the head in a lateral position, which they managed by lifting the infant’s head off the table with two assistants.3 Several other reports have also documented the need for additional personnel to intubate neonates with OE after failing to intubate in a lateral position.4, 5, 6 Mowafi et al. used a ‘doughnut technique’, wherein they placed the OE mass inside a circular roll, and the trunk was supported on a silicon mattress.7 To minimize difficulties of intubation, Mahajan et al. aspirated cerebrospinal fluid to decompress the OE sac, but it resulted in severe bradycardia requiring extensive resuscitation in a few patients.8 They speculated that the rapid decompression of ventricular system led to cardiac compromise due to traction on the brainstem. Although several techniques have been used to intubate patients with OE, to date no single method has been consistently successful.

Apart from difficulty in airway management, other factors may compromise the management of neonates with OE. Jagger et al. reported multiple episodes of raised intracranial pressures and/or low cerebral perfusion pressures in patients with OE.9 In 31 patients with OE, Creighton et al. observed disturbances in central autonomic control and altered temperature regulation.10 More than 50% of their patients had non-neurological congenital malformations, such as Klippel-Feil deformity, cleft palate, micrognathia, subglottic stenosis, further compounding the difficulty in intubating these patients.

We propose a novel technique for endotracheal intubation in neonates with OE by using an apparatus readily available in most hospitals. Its advantages are being able to (1) maintain the usual neutral positioning of the head without pressure on the occipital mass; (2) provide adequate stabilization of the head, neck and trunk; and (3) conduct the procedure without assistance from additional staff.


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Correspondence to N Rangaswamy.

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Rangaswamy, N., Pramanik, A. A novel technique in airway management of neonates with occipital encephalocele. J Perinatol 34, 877–878 (2014).

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