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Year : 2020  |  Volume : 9  |  Issue : 2  |  Page : 149-151

Successful use of I-gel™ in a neonate with crouzon syndrome

1 Department of Critical and Intensive Care, JPN Apex Trauma Center, All India Institute of Medical Sciences, New Delhi, India
2 Department of Anaesthesiology and Critical Care, Pt. BD Sharma University of Health Sciences, Rohtak, Haryana, India

Date of Submission04-Oct-2019
Date of Decision18-Mar-2020
Date of Acceptance11-Mar-2020
Date of Web Publication21-Apr-2020

Correspondence Address:
Dr. Arnab Banerjee
Department of Critical and Intensive Care, JPN Apex Trauma Center, All India Institute of Medical Sciences, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcn.JCN_107_19

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Craniosynostosis is a birth defect syndrome characterized by the premature closure of one or more of the fibrous joints between the bones of the skull. The most common among these craniosynostosis syndromes is Crouzon syndrome. Airway management in such patients becomes challenging and requires an experienced and calm anesthesiologist for expert management. We report a case of successful usage of i-gel™ supraglottic airway device in the airway management of a newborn with craniosynostosis to be operated for enucleation for proptosis in the right eye. Well-planned anesthesia (induction-maintenance-extubation sequence) remains the key to success.

Keywords: Airway management, anesthesia, craniosynostosis, Crouzon syndrome, neonate

How to cite this article:
Banerjee A, Ahlawat G, Aggarwal R. Successful use of I-gel™ in a neonate with crouzon syndrome. J Clin Neonatol 2020;9:149-51

How to cite this URL:
Banerjee A, Ahlawat G, Aggarwal R. Successful use of I-gel™ in a neonate with crouzon syndrome. J Clin Neonatol [serial online] 2020 [cited 2021 Sep 17];9:149-51. Available from: https://www.jcnonweb.com/text.asp?2020/9/2/149/283022

  Introduction Top

Craniosynostosis is a birth syndrome characterized by the premature closure of one or more of the fibrous joints between the bones of the skull, i.e., the cranial sutures before brain growth is complete. Airway management in such patients is challenging and requires an experienced and calm anesthesiologist for expert management. We present a case of craniosynostosis with proptosis to be operated for enucleation with regard to the problems of difficult airway.

  Case Report Top

Our patient was a 1-day-old newborn who was brought by his parents to our institution with complaints of bulging of the right eye since birth which was increasing progressively. The child was born of a healthy mother at full term as a normal vaginal delivery at a nursing home. The child cried immediately after birth. Apgar score at 1, 5, and 10 min was 9, 10, and 10, respectively. The antenatal period was uneventful. There were no history of leaking or bleeding per vaginum, no history of fever or urinary tract infection, and no exposure to drugs or radiation during the antenatal period. The child was accepting breastfeeding well. There were no history of consanguinity and no history of similar disease in siblings.

At presentation, the child's general condition was fair. The child was normothermic. His heart rate was 134/min, with no murmurs on auscultation. The capillary filling time was <3 s. The respiratory rate was 34/min. The chest was clear with bilateral equal air entry. The abdomen on palpation was soft and nontender. Gross central nervous system examination was unremarkable. There was no pallor, icterus, or cyanosis. The skull shape was abnormal (frontal bossing) with proptosis of both eyeballs. The right one is bulging more than the left. There was chemosis present in the right eye. At the time of presentation, it was possible to replace the right eyeball into the eye socket partially, but with time and increasing swelling of the eyeball, it could no longer be placed to its anatomical position. Detailed examination revealed panophthalmitis in the right eye.

There were maxillary hypoplasia, relative prognathia, beaked nose, and a large tongue. There was no choanal atresia. Neck mobility was normal. No other associated anomaly was found. Routine blood and urine investigations were normal. Cranial ultrasound was inconclusive. Magnetic resonance imaging of the skull revealed a retrobulbar mass on the right side suggestive of neurofibroma or hemangioma.

With no useful vision left, the ophthalmologists decided to proceed to enucleate the right eye. On the 5th day, the weight was 2.1 kg. No premedication was prescribed. A difficult airway cart was kept ready for use. Adequate fasting of 4 h was confirmed, and the child shifted to the operation theater. Standard anesthesia monitoring, including electrocardiogram, noninvasive blood pressure, pulse oxygen saturation, temperature, precordial stethoscope, and end-tidal CO2 monitors, were attached, and intravenous line was secured in the operating room. The extremities were covered and the temperature of the operation theater was raised to 28°C, and fluid warmers were used. Preoxygenation was done with 100% for 3 min. Due to the proptosis and midface hypoplasia, a small-sized Rendell-Baker-Soucek mask (size 0) was chosen to ensure satisfactory seal. Anesthesia was induced with an incremental dosage of sevoflurane up to 8% till a minimum alveolar concentration of 1.67, and the end-tidal sevoflurane concentration of 2.5 was achieved. After ensuring bag-and-mask ventilation and adequate depth of anesthesia, the airway was secured with size 1 i-gel™ (Intersurgical Ltd., Wokingham, Berkshire, United Kingdom) supraglottic airway device. After confirming correct i-gel placement with bilateral equal air entry on auscultation and square wave capnography, the i-gel is secured in place, and anesthesia was maintained with sevoflurane 1%. Intravenous paracetamol (10 mg/Kg) was used intraoperatively. The surgery lasted for 90 min. Intraoperatively, the temperature was maintained between 36.3°C and 36.9°C. Blood loss was minimal, and the intraoperative period was uneventful. After the end of operation, all anesthetic agents were discontinued and 100% oxygen was given. Awake extubation was done. The patient was kept in the prone or lateral position to avoid any episodes of sleep apnea. The child was shifted to the ward on the same day. Postoperatively, intravenous paracetamol (10 mg/Kg) was used every 6 h. The patient was discharged from the hospital on the 5th day.

  Discussion Top

Crouzon syndrome consists of hypertelorism, craniosynostosis, shallow orbits with marked proptosis, and midface hypoplasia with an incidence of 16 per million newborns.[1] Octave Crouzon (1874–1938), a French neurologist, first described this syndrome characterized by cloverleaf skull, hydrocephalus (30% progressive), eye signs (proptosis, hypertelorism, rapidly falling vision progressing to complete blindness), mandibular prognathism, maxillary hypoplasia, parrot-beaked appearance of nose, narrow, high/cleft palate, short upper lip, small nasal passages with choanal stenosis, maloccluded teeth, strenuous breathing due to septal deviation, C2–C3 and C4–C5 cervical fusion (18%) limiting neck motion, and impaired hearing.[2]

These diseases can predispose the patients at high risk for anesthesia due to anticipated difficult airway, especially inadequate mask ventilation leading to hypoxia during induction of anesthesia. We did not find any similar case report in literature discussing the anesthetic concerns in a neonate with craniosynostosis for surgery other than repair of primary defect and use of i-gel™ (size 1) for securing the airway where mask ventilation of the newborn is adequate. Treatment of Crouzon syndrome is mainly surgical, for which general anesthesia is the preferred choice. The anesthetic challenges were due to proptosis, frontal bossing, beaked nose, and maxillary hypoplasia, so mask ventilation was anticipated to be difficult. We used a small-sized Rendell-Baker-Soucek mask to ensure satisfactory seal. In our case, satisfactory seal was achieved and bag-mask ventilation was satisfactory. There was no choanal atresia, and neck mobility was normal in our case. Airway was secured with i-gel™ size 1, and we were able to ventilate the patient successfully with no further resistance. It has been reported previously that the bulky design of the i-gel™ made insertion time longer and that the tongue size may have an influence on insertion.[3] However, in our case, we did not face any such problem as we have adequately lubricated the i-gel™ size 1 before insertion and stabilized the tongue with our fingers during insertion of i-gel™.[4] We did not have any difficulty stabilizing the tongue with fingers due to jaw relaxation after the adequate depth of anesthesia. We also assured that the tongue tip did not get trap between the lower teeth and the integral bite block of the i-gel™. In our case, we took all necessary preparations before induction including difficult airway cart and pediatric tracheostomy set as well as the presence of ENT surgeons. In case of difficulty in i-gel™ insertion, we could have secured the airway with endotracheal intubation. We did not use any benzodiazepines, opioids, and muscle relaxants during anesthesia as these patients are prone to difficult intubation and postoperative obstructive sleep apnea. Hypothermia was avoided intraoperatively.

The advantages of the successful use of i-gel™ in our case include avoiding the adverse effects of local tissue trauma, stress-response reflex common both during positioning and removal of laryngoscopy and tracheal intubation[5] but also the complications related to malpositioning of the tracheal tube in the esophagus or in the bronchial tree.[6] Moreover, postextubation complications of tracheal edema were avoided.[7] Moreover, as we used i-gel™, we could also avoid the use of any muscle relaxants during anesthesia as these patients are more prone to pharmacological risks.[8]

Moreover, laryngeal mask airway has been previously used successfully for life-saving of patients with malformations of the upper airway when both tracheal intubation and mask ventilation have failed.[9],[10]

  Conclusions Top

Crouzon syndrome should always be anticipated to present as a case of difficult airway. I-gel™ can be used successfully as airway device in the neonate with Crouzon syndrome, provided there is adequate mask ventilation and the stabilization of the tongue can be achieved during insertion and fixation of the i-gel™. In our case, we could overcome both these limitations efficiently and eventually avoid the complications intubation. We did not find any similar case report in literature discussing the anesthetic concerns in a neonate with craniosynostosis for surgery other than repair of primary defect and successful use of i-gel™ for securing the airway and maintaining adequate ventilation in a neonate with Crouzon syndrome.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Hughes C, Thomas K, Johnson D, Das S. Anesthesia for surgery related to craniosynostosis: A review. Part 2. Paediatr Anaesth 2013;23:22-7.  Back to cited text no. 1
Baldwin JL. Dysostosis craniofacialis of crouzon. A summary of recent literature and case reports with emphasis on involvement of the ear. Laryngoscope 1968;78:1660-76.  Back to cited text no. 2
Theiler LG, Kleine-Brueggeney M, Kaiser D, Urwyler N, Luyet C, Vogt A, et al. Crossover comparison of the laryngeal mask supreme and the i-gel in simulated difficult airway scenario in anesthetized patients. Anesthesiology 2009;111:55-62.  Back to cited text no. 3
Taxak S, Gopinath A. Insertion of the i-gel™ airway obstructed by the tongue. Anesthesiology 2010;112:500-1.  Back to cited text no. 4
Fujii Y, Saitoh Y, Tanaka H, Toyooka H. Cardiovascular responses to tracheal extubation or LMA removal in children. Can J Anaesth 1998;45:178-81.  Back to cited text no. 5
Asai T, Koga K, Vaughan RS. Respiratory complications associated with tracheal intubation and extubation. Br J Anaesth 1998;80:767-75.  Back to cited text no. 6
Tanaka A, Isono S, Ishikawa T, Sato J, Nishino T. Laryngeal resistance before and after minor surgery: Endotracheal tube versus laryngeal mask airway. Anesthesiology 2003;99:252-8.  Back to cited text no. 7
Trevisanuto D, Micaglio M, Ferrarese P, Zanardo V. The laryngeal mask airway: Potential applications in neonates. Arch Dis Child Fetal Neonatal Ed 2004;89:F485-9.  Back to cited text no. 8
Markakis DA, Sayson SC, Schreiner MS. Insertion of the laryngeal mask airway in awake infants with the Robin sequence. Anesth Analg 1992;75:822-4.  Back to cited text no. 9
Behar PM, Todd NW. Resuscitation of the newborn with airway compromise. Clin Perinatol 1999;26:717-32, viii.  Back to cited text no. 10


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