Acute tongue enlargement after surgery is an infrequently reported but known occurrence. This is a case report of a 17-yr-old patient with Crouzon's syndrome who developed severe postoperative macroglossia that required prolonged nasotracheal intubation. The macroglossia resolved rapidly once the trachea was extubated on Postoperative Day 14. This case was analyzed by comparing it with those described previously in the literature.
A 17-yr-old patient with Crouzon's syndrome diagnosed at infancy presented with recent onset of painful paresthesias over his left face, left upper extremity, and thorax. Subsequent investigations revealed a Chiari malformation with associated extensive syringohydromyelia. A suboccipital craniectomy with C1-2 laminectomy was planned. Previous surgeries included a skull morcellation shortly after birth with postoperative hydrocephalus requiring shunt placement and replacement 10 yr later. He had also undergone an inferior oblique myectomy at age 10 yr and Nissen fundoplication for severe gastroesophageal reflux at age 14 yr. Symptoms of reflux persisted, and the patient was treated with cimetidine. Additional surgical history included herniorrhaphy during infancy. Previous general anesthetics had been administered at our facility without complication. The airway examination performed on the morning of surgery revealed a Malampatti class II airway with full cervical range of motion.
A rapid-sequence IV induction of anesthesia was performed with cricoid pressure and administration of thiopental and succinylcholine. The airway was secured with a 7.5-mm oral endotracheal tube via direct laryngoscopy, and anesthesia was maintained with isoflurane and fentanyl. Vecuronium was used for muscle relaxation. A radial arterial line, antecubital right atrial catheter, precordial Doppler, and 10-mm diameter transesophageal echo (TEE) probe were placed after the induction of anesthesia to monitor for venous air embolism and paradoxical air embolism. An 18F esophageal stethoscope was used, but no oral airway was used. Positioning in the sitting position was performed with care to avoid excessive flexion of the neck. After final positioning and before antibiotic administration, a urticarial rash developed on the neck and trunk. The rash completely resolved after the administration of diphenhydramine 50 mg IV. Vital signs remained stable through this episode. The remainder of the intraoperative course of the 7.75-h procedure was unremarkable. The TEE probe and esophageal stethoscope were removed at the completion of surgery as the patient was being moved from the sitting to the supine position. The patient awoke and became responsive in the operating room (OR) soon after the surgical procedure; at that time, some swelling of the tongue was noted. The trachea was extubated to prevent coughing. Slight stridor occurred but improved before the patient left the operating room.
Thirty minutes after extubation, the patient's glossal edema increased and was initially treated with 10 mg of IV dexamethasone. The edema persisted, and increasing stridor and arterial desaturation developed within 45 min after extubation. Awake blind nasal reintubation was successfully performed with a 7-mm endotracheal tube in the recovery room.
The patient was admitted to the neurosurgical intensive care unit postoperatively and remained nasotracheally intubated for airway protection. The degree of macroglossia worsened over the first 24 h and persisted for the next 2 wk (Figure 1, Figure 2, and Figure 3). In addition, multiple ulcerated vesicular lesions were noted on the superior surface of the tongue. An extensive workup for the etiology of the macroglossia (including allergy testing for anesthetic and other medications, latex, and cow allergen) was obtained. This evaluation failed to document a clear etiology for the macroglossia. The macroglossia was initially treated with IV dexamethasone. The tongue showed significant dental impressions, and this mechanical dental pressure seemed to contribute to the distal macroglossia. Use of a bite block seemed to relieve the dental compression and to reduce tongue swelling slightly. Additional treatment was symptomatic with wet gauze dressings and humidified air via an open-face mask per patient request. On Postoperative Day 14, the tongue still could not be retracted into the oral cavity. Nevertheless, during propofol-induced sedation, physical examination by palpation and direct laryngoscopy revealed minimal swelling at the base of the tongue, and the trachea was extubated. No sign of upper airway obstruction followed. Rapid resolution of the swelling occurred within hours, with dramatic softening of the tongue and return to near normal size in 1 day. The patient recovered sensory and motor function of the tongue and was able to close his mouth normally when he was discharged 3 days after extubation.
Crouzon's syndrome is a form of congenital craniofacial synostosis first reported by Crouzon in 1912 as the association of a wide towering skull with proptosis, maxillary hypoplasia, and a beaked nose. Premature closure of cranial sutures begins well after birth and is usually complete by 2 yr of age. No characteristic cranial shape can be described. A high-arched palate is often short and occasionally cleft, and malocclusion is common. Increased intracranial pressure may be associated but is unusual; normal intelligence and life span are expected .
Payne and Cranston  reported postoperative airway problems in an 11-yr-old boy with Crouzon's syndrome who had a tracheostomy tube placed intra-operatively. The patient was mechanically ventilated postoperatively and developed acute respiratory distress when the tracheostomy tube was dislodged. A pneumothorax was diagnosed on return to the OR. Subsequently, the trachea was reintubated with an orotracheal tube, which was placed with great difficulty as only the epiglottis was visible on direct laryngoscopy .
The literature regarding postoperative macroglossia is sparse. In 1974, McAllister  reported two cases of postoperative macroglossia; one in a 22-mo-old child and the other in a 45-yr-old man during posterior fossa craniotomies performed in the sitting position. The resulting macroglossia was believed to be secondary to obstruction of venous drainage of the tongue (i.e., the lingual vein) resulting from the combination of extreme flexion of the head against the chest and compression of the base of the tongue from both the oral airway and the endotracheal tube.
In 1975, Ellis et al.  reported a case of massive edema of the head, neck, and tongue occurring after craniotomy in the sitting position. Bilateral internal jugular venous thrombosis was the proposed mechanism for this massive symmetrical edema.
Macroglossia has been described as a complication of sitting position craniectomy. In a patient with achondroplasia, macroglossia was presumed secondary to extreme neck flexion and a resultant bilateral lingual vein thrombosis [5,6]. The swelling took 12 wk to subside after lingual debridement and a tracheostomy had been performed in the OR. In a review of 107 pediatric patients undergoing transcranial craniofacial surgery, macroglossia with resultant supraglottic airway obstruction requiring reintubation was reported in 1 patient .
Sixteen cases of postoperative macroglossia have been previously reported in the literature [3-6,8-17]. Although macroglossia is a complication of endotracheal intubation, it has also been reported after the use of the laryngeal mask airway . Of the 15 cases complicating endotracheal intubation, 6 are in pediatric patients aged <2 yr and 9 are patients aged 12-67 yr. Of the 15 patients, 9 (60%) were male; 6 (40%) were female. Of the 15 patients, 7 (47%) underwent craniotomies, of which 86% (6 of 7) were sitting craniotomies. Of the remaining patients, 6 (40%) underwent either palatoplasty or palatorrhaphy procedures. The average length of the surgical procedure was 6.3 h. Of the 15 cases of macroglossia, 11 (73%) required postoperative intubation, and 6 (55%) of the reintubations were reported as difficult. In the data reported, the average duration of postoperative intubation was 7.25 days. Of the 15 patients, 3 (20%) died or suffered severe neurologic morbidity.
In this case, the exact etiology of the macroglossia is unclear. A possible explanation is that lingual compression occurred from multiple oral instrumentations (TEE, oral endotracheal tube, and esophageal stethoscope) during the surgery, which likely resulted in arterial and venous compromise and compression of the base on the tongue. After removal of all the tubes and reinsertion of the nasotracheal tube, persistent venous compromise remained, resulting in further tongue swelling with dental compression, perpetuating the cycle of continued swelling. The use of bite blocks in the postoperative period seemed beneficial. The macroglossia may have been exacerbated by the patient's midfacial hypoplasia and normal sized tongue, which made the degree of postoperative swelling that resulted even more significant with regard to airway compromise.
In addition to this case report, a number of others describe the insidious onset of postoperative macroglossia minutes or hours after extubation [4-6,9,17]. This rapid onset suggests ischemia of the tongue with reperfusion hyperemia and capillary leakage as important mechanisms. Venous and lymphatic obstruction due to kinking and even thrombosis of the internal jugular veins have been implicated in other case reports. The use of mouth packs and oral airways were thought to be contributory in several patients [4,17]. Although TEE is widely used for sitting procedures to monitor for venous air embolism and paradoxical air embolism, the contribution of the TEE probe in causing this patient's complication is impossible to quantify. Allergic reactions also frequently result in macroglossia . Hemorrhage and infection could be other mechanisms of this complication .
In the present case, the macroglossia could have been related to intraoperative cervical flexion, the endotracheal tube, TEE probe, esophageal stethoscope, and Crouzon's syndrome. The relative importance of each of these factors is impossible to quantify. The size of the patient's tongue delayed extubation for 14 days. Although rapid airway obstruction after extubation was still feared at that time, no stridor was noted, and the patient's swollen tongue shrank quickly after the nasal endotracheal tube was removed. This leads us to recommend earlier extubation in similar cases, especially if the base of the tongue is not swollen.
1. Ward CF. Pediatric head and neck syndromes. In: Katz J, Steward DJ, eds. Anesthesia and uncommon pediatric diseases. 2nd ed. Philadelphia: WB Saunders, 1993:319-63.
2. Payne JF, Cranston AJ. Postoperative airway problems in a child with Crouzon's syndrome. Paediatr Anaesth 1995;5:331-3.
3. McAllister RG. Macroglossia: a positional complication. Anesthesiology 1974;40:199-200.
4. Ellis SC, Bryan-Brown CW, Hyderally H. Massive swelling of the head and neck. Anesthesiology 1975;42:102-3.
5. Mayhew JF, Miner M, Katz J, et al. Anaesthesia for the achondroplastic dwarf. Can Anaesth Soc J 1986;33:216-21.
6. Mayhew JF, Miner M, Katz J. Macroglossia in a 16-month-old child after craniotomy. Anesthesiology 1985;62:683-4.
7. Jones BM, Jani P, Bingham RM, et al. Complications in paediatric craniofacial surgery: an initial four year experience. Br J Plast Surg 1992;45:225-31.
8. Maltby JR, Elwood T, Price B. Acute transient unilateral macroglossia following use of a LMA. Can J Anaesth 1996;43:94-5.
9. Chan MT, Chan MS, Mui KS, Ho BP. Massive lingual swelling following palatoplasty: an unusual case of upper airway obstruction. Anaesthesia 1995;50:30-4.
10. Tattersall MP. Massive swelling of the face and tongue: a complication of posterior cranial fossa surgery in the sitting position. Anaesthesia 1984;39:1015-7.
11. Kharasch ED. Angiotensin-converting enzyme inhibitor-induced angioedema associated with endotracheal intubation. Anesth Analg 1992;74:602-4.
12. Lee JT, Kingston HGG. Airway obstruction due to massive lingual oedema following cleft palate surgery. Can Anaesth Soc J 1985;32:265-7.
13. Bell C, Oh TH, Loeffler JR. Massive macroglossia and airway obstruction after cleft palate repair. Anesth Analg 1988;67:71-4.
14. Patane PS, White SE. Macroglossia causing airway obstruction following cleft palate repair. Anesthesiology 1989;71:995-6.
15. Teeple E, Maroon J, Rueger R. Hemimacroglossia and unilateral ischemic necrosis of the tongue in a long-duration neurosurgical procedure. Anesthesiology 1986;64:845-6.
16. Pivalizza EG, Katz J, Singh S, et al. Massive macroglossia after posterior fossa surgery in the prone position. J Neurosurg Anesth 1998;10:34-6.
17. Kawaguchi M, Sakamoto T, Ohnishi H, Karasawa J. Pharyngeal packs can cause massive swelling of the tongue after neurosurgical procedures. Anesthesiology 1995;83:434-5.
18. Renehan A, Morton M. Acute enlargement of the tongue. Br J Oral Maxillofac Surg 1993;31:321-4.