International Journal of Pediatric Otorhinolaryngology Extra
Volume 4, Issue 2 , Pages 84-87, March 2009

Anaesthetic management of a child having congenital macroglossia with mandibular deformity

Department of Anaesthesiology and Critical Care, Pt. B.D. Sharma Post Graduate Institute of Medical Sciences, Rohtak 124001, Haryana, India

Received 19 May 2008; received in revised form 3 August 2008; accepted 9 August 2008. published online 22 September 2008.

Article Outline

Summary 

Securing the airway is vital in the management of anaesthesia administration. Patients with difficult airway are extremely challenging. Paediatric tongue lesions represent 2.4% of all paediatric oral and maxillofacial lesions. Most lesions are benign and include various local neoplasms, solid tumors, cysts, polyps and diffuse hypertrophy. We present a case of a 7-year-old child having congenital macroglossia with mandibular deformity due to malocclusion, admitted for reduction glossectomy. It was predicted to be a difficult intubation and it was successfully achieved by blind nasal technique.

Keywords: Mouth opening, restricted, Macroglossia, Intubation, nasotracheal, Technique, blind

 

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1. Introduction 

Tongue development begins at 3–4 weeks gestation from the first to four branchial arches. Specifically, tongue arises from four swellings (median tongue bud, two lateral tongue buds and hypobranchial eminence) which merge to form the tongue [1]. Paediatric tongue lesions represent 2.4% of all paediatric oral maxillofacial tumors. Most lesions are benign and include various local neoplastic solid tumors, cysts, polyps, benign neoplasms and diffuse hypertrophy. Diffuse lesions can present with chronic protrusion, respiratory distress, dysphagia, dysarthria and salivation [2], [3].

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2. Case history 

A 7-year-old child presented with congenital macroglossia with mandibular malocclusion for reduction glossectomy. As narrated by her parents the child had large tongue since birth, anterior part of which remained out of the mouth. Gradually, the tongue size increased and attained the present size. No other congenital anomalies were present. The child had history of delayed milestones with difficulty in speech and feeding (Fig. 1, Fig. 2).

On examination, the child was malnourished, mentally retarded and appeared smaller than her age. The mouth was completely obliterated by large tongue. Lower jaw movements were restricted but neck movements were normal. Indirect laryngoscopy was not possible. The pliability of the mandibular space was limited. Temporomandibular joint (TMJ) movements were restricted due to occlusion defects. Teeth were hidden by large tongue. The cardiovascular and respiratory systems were normal. The investigations were within normal limits. The radiographs of face and chest (anteroposterior and lateral view), showed soft tissue mass in the oral cavity and airway passages were normal. The child's nostrils were patent, right more patent than left.

On the day of operation, child was kept fasting for 6h and a separate consent for tracheostomy was taken. Syrup promethazine 1tsf was given 2h before surgery as premedication. Xylometazoline nasal drops were instilled twice in the morning.

In operation theatre after application of standard monitoring (SpO2, EKG and NIBP), intravenous line was started. Patient was preoxygenated, nostrils were sprayed with lignocaine 10%. Injection glycopyrrolate 0.1mg and injection ketamine 10mg were given intravenously and anaesthesia was induced with inhalational agents (O2 100% and sevoflurane 3–6%), but mask ventilation became difficult due to enlarged tongue. A nasopharyngeal airway no. 4 was inserted and it was attached to the paediatric circuit. As paediatric fiberscope was not available, Blind nasal intubation was done with endotracheal tube no. 5.0mm i.d. through the other nostril.

Maintenance of anaesthesia was done with N2O 67% in O2 33%, sevoflurane, injection vecuronium 1.5mg and injection tramadol 15mg were given intravenously. At the end of surgery, patient was reversed with injection neostigmine 0.8mg and injection glycopyrrolate 0.1mg. Extubation was done after proper suctioning. Recovery was smooth and child responded to verbal commands. She was observed in recovery room for 2h and then shifted to the ward. Postoperatively, the child was unable to close her mouth due to mandibular malocclusion. She was referred to dental surgeon for TMJ malfunction and dentition problems (Fig. 3, Fig. 4).

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3. Discussion 

The American Society of Anaesthesiologists (ASA) defined a difficult airway as “The clinical situation in which a conventionally trained anaesthesiologist experiences difficulty with mask ventilation, difficulty with tracheal intubation or both” [4].

Macroglossia is defined as a resting tongue that protrudes beyond the teeth or alveolar ridge. Sequel owing to macroglossia includes articulation errors particularly in pronouncing consonants requiring tongue tip in approximation with the alveolar ridge or roof of the mouth. One may develop prognathism, increased ramus to body angle and flattening of the alveolar ridge. Deglutition problem is also important complication and results in failure to thrive owing to inadequate intake. Airway obstruction may be further complicated and may lead to pulmonary hypertension and corpulmonale. Acute respiratory distress owing to sudden respiratory obstruction has also been described. Neonatal hypothyroidism, cretinism has been associated with macroglossia. Here tongue is enlarged owing to myocyte hypertrophy and myxodematous tissue deposition [1], [2].

There are multiple syndromes associated with macroglossia. These are Beckwith–Wiedemann syndrome, generalised gangliosidosis, mannosidosis, mucopolysaccharidosis (Hurler), mucopolysaccharidosis (Hunter), neurofibromatosis, pycno-odysostosis or osteopetrosis, Simpson–Golabi–Behmel syndrome and X-linked α-thalassemia/mental retardation [1].

Lymphangiomas are the most common etiology of macroglossia in children. It can be apparent at birth 60% of the time, with 95% becoming symptomatic by 2 years of life. They typically involve the anterior two-third of the tongue. Hemangiomas, congenital vascular malformation may also present as macroglossia. Rhabdomyosarcoma of the tongue causes macroglossia and accounts for 20% of head and neck rhabdomyosarcomas. Neurofibromatosis may be associated with macroglossia when affected individual develop neurofibromatosis of the tongue [1], [2], [3], [4].

In a known difficult airway patient, preanaesthetic examination of upper airway and nostrils patency is important. Because of protruding tongue mask ventilation would be difficult to maintain. Soft nasopharyngeal airway offers better airway patency, as sharp tip of a blindly advancing nasotracheal tube may skewer adenoidal tissue, precipitate nasopharyngeal bleeding, trigger laryngospasm or aspiration of blood. When facial or oral pathology prevents visualization of larynx by direct laryngoscopy, then alternate means of tracheal intubation should be planned, so that excessive attempts to intubate the trachea with conventional techniques are avoided [5], [6].

Inhalational induction is the method of choice. This permits the anaesthesiologist to assess for any airway obstruction as the soft tissue tone decreases. Halothane and sevoflurane are the two currently available inhalational agents, which are least irritating to the airway and produce smooth induction. Sevoflurane has the advantage of a rapid and smooth induction. Halothane, in a gradually increasing concentration is a more suitable induction agent as the time available for performing a difficulty laryngoscopy and intubation is significantly more. In case of any doubt about ability to ventilate through the face mask, maintenance of spontaneous ventilation and use of topical anaesthesia in place of muscle relaxant is recommended. Spontaneous breathing may offer a patent airway and provide sight and sounds necessary to direct tracheal intubation [6]. Preanaesthetic medication with anticholinergic agents reduces the volume of secretions and prevents reflex bradycardia during airway manipulations. Preanaesthetic narcotics or barbiturates are contraindicated because of the potential for cardiorespiratory depression. The intubation techniques in these cases include fiberoptic nasotracheal intubation, blind nasal intubation and tracheostomy [5], [6], [7].

Fiberoptic intubation of the awake spontaneous breathing patient is the gold standard for management of an anticipated difficult or compromised airway. Its successful use in paediatric patients depends on several factors. Infants and children do not cooperate during awake fiberoptic intubation. So it is generally easier to keep them anaesthetized but breathing spontaneously. Loss of patency of child's airway can also occur rapidly with hypoxemia and desaturation due to high baseline oxygen consumption. Therefore, during airway manipulation administration of supplemental oxygen as well as atropine to prevent bradycardia and to dry secretions are essential steps to ensure success of the technique [8]. However, fiberoptic intubation is not a panacea and difficult or failed fiberoptic intubation is an ever present problem [9]. Common reasons for failure are inadequate training, lack of clinician's experience, limited field of view exacerbated by blood or secretions in the airway, poor airway anaesthesia, inability to advance the endotracheal tube over the fiberscope, distorted airway anatomy and limited space between epiglottis and posterior pharyngeal wall. In addition, fiberoptic bronchoscopes are expensive and fragile instruments and variety of sizes are needed in paediatric patients. Paediatric bronchoscopes are also smaller with reduced field of vision and have an insertion cord that is thinner and more flexible with different range of tip angulation. Bronchoscopes small enough for infants lack working channel [8].

In the absence of sophisticated paediatric fiberoptic equipment, blind nasal intubation remains the only non-surgical option for control of airway. When performed, it requires adequate sedation, topical anaesthesia, and vasoconstriction or general anaesthesia with preservation of spontaneous ventilation. The patient is placed in the classical intubating position with the neck flexed and head extended. After the endotracheal tube is inserted through the nostril, it is advanced blindly and directed into the glottis by listening for maximal breath sounds, observing fogging of the tube or by capnograph tracing [10]. Various maneuvers may be employed to aid intubation. Flexion or extension of the head or manipulating the larynx by external pressure may line up the tube and larynx [11]. Rotating the tube may be helpful [12]. A stylet can be inserted into the tube to help advance the tip through the vocal cords [13]. The cuff may be partially inflated in the oropharynx to elevate the tip from posterior pharyngeal wall and centre it [11]. The cuff is deflated before the tube is advanced into the trachea. A suction catheter that is inserted into the tube may facilitate passage through the larynx [14]. A bougie can also be used [15]. A success rate from 72% to 86% has been reported for blind nasal intubation [11].

Goldman et al. reported the successful control of airway with laryngeal mask in an infant with Beckwith–Wiedemann syndrome and hepatoblastoma for central line catheterization [16].

Treatment of macroglossia with surgical excision is based on the effect on feeding, dentition, speech and airway compromise. Management involves a multidisciplinary team of an otolaryngologist, a speech therapist and an orthodontist.

In conclusion, perioperative management of a case of difficult airway includes careful preoperative evaluation of the upper airway and provision for a secure perioperative airway.

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References 

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PII: S1871-4048(08)00055-5

doi:10.1016/j.pedex.2008.08.002

International Journal of Pediatric Otorhinolaryngology Extra
Volume 4, Issue 2 , Pages 84-87, March 2009

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