Neonatal Pierre Robin sequence: An airway challenge addressed with retrograde intubation
Niveditha Karuppiah1, Surjya Kanta Mohanty2, Suraj Prasad2 1 Department of Anesthesia and Perioperative Medicine, University of Western Ontario, London, ON, Canada 2 Department of Anaesthesia, Rainbow Children's Hospital, Bengaluru, Karnataka, India
Date of Submission
11-Nov-2020
Date of Acceptance
11-Nov-2020
Date of Web Publication
25-Dec-2020
Correspondence Address: Dr. Niveditha Karuppiah Department of Anesthesia and Pain Medicine, Victoria Hospital, C2-166, 800 Commissioners' Road East, London, ON N6A 5W9 Canada
Source of Support: None, Conflict of Interest: None
DOI: 10.4103/arwy.arwy_49_20
Abstract
We report the case of a 25-day-old neonate posted for glossopexy. The child born at 35 weeks of gestation was diagnosed to have Pierre Robin sequence. The neonate was underweight (2.2 kg), had difficulty in feeding and had respiratory distress needing respiratory assistance and prone position to maintain saturation. Anticipating difficulty in intubation with respect to physiology, age and mismatch of equipment size, we planned retrograde intubation through a laryngeal mask airway with a 2.5 mm ID tube using a needle cricotracheotomy and a flexible-tipped paediatric urological guidewire. Based on our experience, we propose that neonatal difficult airway can be managed using the retrograde intubation technique.
Keywords: Difficult airway, neonate, Pierre Robin sequence, retrograde intubation
How to cite this article: Karuppiah N, Mohanty SK, Prasad S. Neonatal Pierre Robin sequence: An airway challenge addressed with retrograde intubation. Airway 2020;3:148-50
How to cite this URL: Karuppiah N, Mohanty SK, Prasad S. Neonatal Pierre Robin sequence: An airway challenge addressed with retrograde intubation. Airway [serial online] 2020 [cited 2023 Jun 4];3:148-50. Available from: https://www.arwy.org/text.asp?2020/3/3/148/304854
Introduction
Pierre Robin sequence (PRS) consisting of micrognathia, glossoptosis and cleft palate presents with airway obstruction and feeding difficulties with or without other congenital anomalies. Preterm, intrauterine growth restricted, underweight neonates add to this challenge in having smaller airways, restricted cardiorespiratory reserves along with issues with equipment availability and the requisite skills in handling such neonates.
Case Report
We present the case of a premature newborn with PRS. The child had feeding difficulties and respiratory distress needing ventilatory support with prone positioning to maintain the airway and prevent drastic drops in oxygen saturation. The child was posted for glossopexy.
The challenges for the anaesthesiologist in handling such a case are multiple. The child was a preterm neonate and underweight with compromised cardiorespiratory reserves. There was also a mismatch in equipment size. We anticipated the need for a small-sized endotracheal tube (2.5 mm ID) which could not be accommodated on the smallest fibreoptic bronchoscope (2.8 mm outer diameter flexible bronchoscope) available at our institution. Retrograde intubation was planned with the intention of minimising airway manipulation. A check laryngoscopy was planned followed by placement of a laryngeal mask airway (LMA) to maintain ventilation during the period when retrograde intubation was being performed.
After a thorough preanaesthetic evaluation and informed consent from the parents, the child was posted for glossopexy. Intravenous (IV) access was secured preoperatively for administering maintenance fluids to cover the period of fasting. On shifting to the operation theatre, standard monitoring was established. The child was given glycopyrrolate 10 μg and fentanyl 4 μg followed by propofol 5 mg as a slow IV injection while administering 100% oxygen using a Jackson Rees circuit. Anaesthesia was deepened with sevoflurane and a check laryngoscopy was attempted. We found it difficult to visualise the vocal cords or the epiglottis. An Ambu LMA #1 was inserted and secured after confirmation of free ingress and egress of gases. The child was then paralysed with atracurium 2 mg IV and anaesthesia was maintained with 2% sevoflurane in a 50:50 mixture of oxygen and air.
Following this, the child was positioned with slight extension of the head. Under aseptic precautions, a needle cricotracheotomy was performed using a 22G paediatric spinal needle (Quincke) connected to a saline syringe to check free aspiration of air [Figure 1] and [Video 1]. Once the needle tip was confirmed to be in the trachea, the syringe was disconnected and a soft-tipped paediatric urological guidewire was inserted through the needle and advanced until it emerged smoothly through the machine end of the LMA.
Figure 1: (a) Needle cricotracheotomy (b) Guidewire inserted through the spinal needle (c) Passed through the larynx, brought out through laryngeal mask airway (d) Endotracheal tube railroaded over the guidewire
The LMA was removed after securing the guidewire while the child received passive insufflation of oxygen at 10 L/min using nasal prongs. This was done to delay any desaturation that could occur during removal of the LMA. A 2.5 mm ID endotracheal tube was threaded through the Murphy's eye using the 'outside-in' technique and railroaded over the guidewire.[1] Air entry was confirmed by auscultation and capnography. The guidewire was then carefully removed from the proximal end and the endotracheal tube was secured. Anaesthesia was maintained with 2% sevoflurane in 50:50 oxygen and air. After the procedure, the child was electively ventilated overnight. The same airway equipment was kept ready as standby prior to extubation, which turned out to be uneventful.
Discussion and Conclusion
PRS is frequently encountered with anticipated difficult ventilation and difficult intubation.[1] Establishment of an airway is a challenge, especially in an infant, because of the limited options. It has been recognised that retrograde intubation is much more difficult in neonates than that in older children, and guiding the endotracheal tube into the trachea may be difficult.
In addition to the possible airway difficulty, management of diminished cardiorespiratory reserves also assumes importance. Maintenance of oxygenation and ventilation in this case was successful with an LMA in place. It also aided in maintaining the depth of anaesthesia during intubation.
The cricotracheal membrane was chosen as the point of needle insertion even though it is difficult to identify the structures in a neonate. This point of needle insertion gives the advantage of a few extra millimetres of the tube inside the trachea which compensates for tube movement during insertion and removal of the guidewire and increases the distance between the point of needle insertion and the vocal cords, thereby reducing the risk of trauma to the vocal cords.[2] A 22 G spinal needle was used for cricotracheotomy to provide stability and to maintain the direction of insertion.
The paediatric urological guidewire is soft tipped and gives sufficient stability while causing no harm to the soft neonatal airway tissues. The guidewire follows the path of least resistance and comes out smoothly through the LMA. The proximal end of the guidewire was threaded through the Murphy's eye of the endotracheal tube using the 'outside-in' technique. This step gave us a few additional millimetres, decreasing the chances of tube dislodgement when the guidewire was removed. We believe that this technique is a valuable option in low-resource settings to safely manage a difficult paediatric airway.
Acknowledgement
The authors would like to thank Dr Hariprasad, Consultant Anaesthesiologist, Rainbow Children's Hospital, Marathahalli, Bengaluru, India. He was part of the anaesthesia team for this case.
Declaration of patient consent
The authors certify that they have obtained the appropriate patient consent form. In the form, the parents have given their consent for clinical information to be reported in the journal. The parents understand that the name and initials of their child will not be published and due efforts will be made to conceal the child's identity, but anonymity cannot be guaranteed.
Portnoy JE, Tatum S. Retrograde nasal intubation via the cleft in Pierre-Robin Sequence neonates: A case series. Int J Pediatr Otorhinolaryngol 2009;73:1828-32.
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