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Massive Macroglossia After Posterior Cranial Fossa Surgery: A Case Report

Ha, Jennifer, F., MBBS (Hons), FRACS*; Vitale, Lisa, N., MD; Pfarr, Marie, A., MD; Kawai, Yu, MD§; Zopf, David, A., MD, MS*

doi: 10.1213/XAA.0000000000000663
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A 16-year-old boy with Chiari 1 malformation presented for an elective suboccipital craniectomy and C1 laminectomy. His intraoperative course was uneventful. At the conclusion of the procedure, he met extubation criteria and followed commands. After extubation, he developed progressive upper airway obstruction and became obtunded. He was reintubated via videolaryngoscopy, which showed edema not only to the tongue, but also to the posterior pharynx and blisters over the vocal folds and epiglottis. The patient was transferred to the pediatric intensive care unit intubated and sedated. This report describes the clinical course of his massive macroglossia and discusses short- and long-term management.

From the Departments of *Pediatric Otolaryngology–Head and Neck Surgery, Pediatric Anesthesiology, Pediatrics and Communicable Diseases, and §Pediatric and Communicable Diseases, University of Michigan Medicine, CS Mott Children’s Hospital, Ann Arbor, Michigan.

Accepted for publication September 15, 2017.

Funding: None.

The authors declare no conflicts of interest.

Address correspondence to David A. Zopf, MD, MS, Department of Pediatric Otolaryngology–Head and Neck Surgery, CS Mott Children’s Hospital, Michigan Medicine, 1540 E Hospital Dr, SPC 4241, Ann Arbor, MI 48109. Address e-mail to davidzop@med.umich.edu.

Massive macroglossia is a known but unusual complication of posterior cranial fossa surgery. Pivalizza et al1 reported the first occurrence of this in 1998. This unusual complication has an unclear etiology, with multiple hypotheses that include positioning, vascular compromise, neurogenic factors, and local trauma.1–7 We present this unusual complication in a healthy adolescent and discuss its acute as well as long-term management and follow-up.

The patient’s family gave written permission for the authors to publish this case report. All authors participated in the care of the patient described.

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CASE DESCRIPTION

An otherwise healthy 85-kg, African American, 16-year-old boy with a 2-month history of intermittent headaches and numbness on his right chest and back presented for suboccipital craniectomy, C1 laminectomy, and duraplasty. Magnetic resonance imaging revealed a Chiari I malformation with cervical and thoracic syrinx.

After intravenous (IV) induction of anesthesia with propofol, lidocaine, and cisatracurium, easy, single-provider mask ventilation was achieved and a Cormack and Lehane grade 1 view was obtained under direct laryngoscopy with a Macintosh 3 blade. A 7.5-mm, single-lumen–cuffed endotracheal tube (ETT) was placed atraumatically. A soft bite block was placed between the left posterior molars.

He was placed prone. His head was flexed slightly in the midline and stabilized with a 3-point pin fixation using a Mayfield head clamp device. The cisatracurium used for intubation was allowed to wear off, and motor- and somatosensory-evoked potentials were monitored throughout the case. Anesthesia was maintained with isoflurane and sufentanil. He was given a prophylactic dose of cefazolin before surgical incision and redosed intraoperatively 4 hours later per our institutional protocol. The patient received intermittent morphine boluses IV throughout the latter half of the procedure to minimize postoperative pain. Intraoperatively, he received 2.8 L lactated Ringer’s solution and no blood products. Urine output during the case was 320 mL. Operative time was approximately 7 hours.

After the surgical dressing was completed, he was returned to the supine position and the 3-point pin device was removed. Consistent with prone positioning, he was noted to have slight periorbital and perioral edema, but no tongue swelling. As he emerged from anesthesia, he was breathing spontaneously and following commands. He received ondansetron IV 1 hour before extubation for prevention of postoperative nausea and vomiting. The ETT and bite block were removed when he met extubation criteria. Several minutes after extubation, he showed signs of mild upper airway obstruction manifested as snoring. This was relieved with a chin lift. Additional dexamethasone was administered due to concerns for airway swelling. He was then transported to the postanesthesia care unit (PACU).

On admission to PACU, he was noted to have increasing stertor not easily relieved by chin lift. Jaw thrust was maintained and a modified Jackson Rees non-rebreathing anesthesia circuit with facemask was applied. Oxygen saturation was in the high 90s. When he progressed to substernal retractions, a nasopharyngeal airway was inserted with ease. The isolated stertor without stridor led to a suspicion of pharyngeal obstruction from soft tissue edema, pharyngeal collapse due to decreased muscular tone after opioids or volatile anesthetics, or tongue obstruction. He then became unresponsive to commands. Arterial blood gas showed PaCO2 of 68 mm Hg (it was 36 mm Hg before extubation). Positive pressure was initiated with a Jackson Rees circuit.

His tongue now appeared to be swollen, so emergency airway equipment was prepared for possible reintubation. The otolaryngology critical airway team was also consulted. Mask ventilation was now difficult and required 2 practitioners. His Cormack and Lehane laryngoscopy grade evolved to grade 3. Otolaryngology was present for the videolaryngoscopy (GlideScope) reintubation, performed with a Macintosh 4 blade. A 7.0-mm cuffed ETT (0.5 mm smaller than the preoperative ETT) was placed on first pass with no apparent trauma. Blisters over the tongue, vocal folds, and epiglottis were noted. He remained hemodynamically stable throughout the PACU stay. Diphenhydramine 50 mg IV was administered. Serum tryptase and C1 esterase antigen levels were obtained. The PaCO2 corrected to 47 mm Hg after reintubation and mechanical ventilation. He was transferred to the pediatric intensive care unit 1.5 hours after reintubation.

His tongue swelling significantly increased such that it protruded beyond the interincisor line and out of the oral cavity (Figure 1). The oral cavity could not be viewed with a tongue depressor. His teeth caused concave marks on the dorsal tongue. There was no improvement of the lingual edema despite continuing the treatment for anaphylaxis in the first 24 hours. Dermatology was consulted for possible Stevens-Johnson syndrome given the mucosal involvement and known association with ondansetron and cefazolin.8 This was excluded due to the limited skin and mucus membrane involvement. His serum tryptase level (sensitivity, 0.36; specificity, 0.93)9 and C1 esterase inhibitor antigen (sensitivity, 65%; specificity, 94%) and functional level (sensitivity, 97%; specificity, 81%)10 results were normal. Creatinine phosphokinase levels peaked at 854 IU/L on postoperative day 10 and eventually normalized.

Figure 1

Figure 1

Nursing performed hourly moist, petroleum gauze and tongue dressing changes to the exposed mucosa to prevent desiccation. He was closely monitored for compartment syndrome of the tongue by frequently assessing the color, warmth, turgor, and capillary refill. After extensive discussion with the patient, his family, otolaryngologists, and intensivists, conservative management of his macroglossia was elected over early aggressive surgical approaches such as midline glossectomy or tongue reduction. The goal was to allow for delineation of necrotic tissue to achieve maximal preservation of lingual tissue, particularly the tongue tip, in hopes of providing optimal speech and swallowing functional outcomes. Surgical tracheostomy was performed 10 days after his reintubation due to the slow resolution of the tongue edema. Head and neck magnetic resonance imaging 15 days after the event did not show any vascular malformations contributing to the slow resolution. Evidence of ischemia was noted on the left lateral tongue. He was discharged after a 26-day stay in the hospital with a tracheostomy. His tongue remained swollen.

Figure 2

Figure 2

Figure 3

Figure 3

In the otolaryngology outpatient clinic on postoperative day 31, the tongue swelling was reduced, tongue mobility increased, and a necrotic area demarcated (Figure 2). On postoperative day 39, while the tongue remained protruded at baseline, he was able to fully retract it back into his oral cavity with effort. With delineated necrotic area stabilized, a partial glossectomy and debridement were performed (Figure 3A). His tracheostomy was decannulated successfully after being in situ for 47 days. His tongue had healed completely on postoperative day 72 (Figure 3B). The preserved lingual tip contributed to his intelligibility during speech and preservation of masticatory function of a normal diet with mixed texture food.

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DISCUSSION

It is postulated that the neck flexion and dependent position may result in lingual vein congestion and/or thrombosis, lymphatic obstruction, and arterial compromise.1–7 Previously published works ascribe the massive macroglossia to local trauma from oral airway or throat packing or to mechanical compression from the ETT that contributes to the development of regional ischemia and reperfusion hyperemia with capillary leakage.3–5 Other theories include neurogenic lingual edema due to abnormal brainstem signaling of sympathetic activity from surgical manipulation, tumor infiltration, or angioneurotic reaction.1,3,5 We hypothesize that in our patient, venous and/or lymphatic congestion and obstruction may have precipitated arterial insufficiency and ischemia with eventual reperfusion injury that led to severe macroglossia. In the postoperative course after the massive swelling had already occurred, the dentition may have focally restricted arterial supply resulting in worsened ischemic injury.

A majority of the literature is reported in adults with an estimated incidence of 1% in patients presenting for posterior fossa craniectomy.4 These are summarized in the Table. The onset of swelling ranged from intraoperatively at the end of the procedure to a delay of up to 36 hours.4 Kuhnert et al7 reported vesicles on the edematous tongue similar to our case. The time to resolution is variable, with the longest being 12 weeks in a 16-month-old.6 We present a treatment flowchart in Figure 4.

Table

Table

Figure 4

Figure 4

Suggested methods for prevention include avoiding extreme flexion, placing a bite block, avoiding oral instrumentation, reducing surgical time, and assessing the oral cavity at frequent intervals intraoperatively, especially if a chin bar is used.1,3,5,7 These methods did not prevent macroglossia in our patient. Lam and Vavilala5 suggested simultaneous monitoring of central and jugular venous pressure to diagnose venous obstruction should it occur (jugular venous pressure would increase while central venous pressure should remain unchanged). This was not performed in our case and is not currently the standard of care.

In summary, it is important to be aware of massive macroglossia as a complication during prone neurosurgical procedures because it can be potentially life threatening if not identified in a timely manner. In our 16-year-old boy who underwent posterior cranial fossa surgery, the timely identification and treatment of the impending airway obstruction and the conservative approach to lingual edema management optimized the outcome.

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DISCLOSURES

Name: Jennifer F. Ha, MBBS (Hons), FRACS.

Contribution: This author helped prepare the manuscript.

Name: Lisa N. Vitale, MD.

Contribution: This author helped prepare the manuscript.

Name: Marie A. Pfarr, MD.

Contribution: This author helped prepare the manuscript.

Name: Yu Kawai, MD.

Contribution: This author helped prepare the manuscript.

Name: David A. Zopf, MD, MS.

Contribution: This author helped prepare the manuscript.

This manuscript was handled by: Raymond C. Roy, MD.

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