AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

• Authors and Editors
• Introduction
• Indications
• RELEVANT ANATOMY
• Contraindications
• Workup
• Treatment
• Complications
• Outcome And Prognosis
• Future And Controversies
• References

INTRODUCTION

Section 2 of 11  

• Authors and Editors
• Introduction
• Indications
• RELEVANT ANATOMY
• Contraindications
• Workup
• Treatment
• Complications
• Outcome And Prognosis
• Future And Controversies
• References

Macroglossia, meaning large tongue, has been a documented anatomical anomaly for several centuries but remains an entity defined more by presentation than by strict cephalometric analysis. The earliest known written description of tongue lesions comes from the Egyptian Papyrus Ebers, originally thought to be from around 1550 BC.

Obviously, tongue lesions have since been categorized by their etiologies. Macroglossia has an extensive list of possible causes. Its treatment has been largely surgical in the modern era.

History of the Procedure

Historically, a number of medical interventions have been tried. Egyptians used milk gargles and various chewed concoctions to treat lesions of the tongue. Other forms of treatment have included sclerosing agents (mercury and potassium), leeches, and physical entrapment of the tongue within a bound oral cavity. All of these treatments have appeared in the literature as recently as the 20th century.

Similar to the history of medical intervention, surgical intervention for macroglossia began without an understanding of the cause and proceeded to truly barbaric forms of resection until its reform in the last 100 years.

Early surgical interventions included making cuts on the tongue to promote bleeding and the insertion of roughened instruments into the body of the tongue multiple times in order to promote scarring and retraction. As late as 1865, a physician went so far as to tie off the external carotid artery on one side and, when that did not prove helpful, tied off the common carotid on the other. The patient survived, and her tongue reportedly decreased to near normal in size, despite the blatant vascular risks.

In the late 1600s in Sweden, a young female patient was treated by amputating the protruded portion of the tongue. Her recovery allowed for normal swallowing. Hemorrhage must have been severe because the lingual arteries were not ligated. Surgical resection did not gain favor widely until after 1900. Prior to that time, the use of various ligatures dominated the attempts at surgical reduction. Tying a wire around the tongue was particularly gruesome and painful with the necrotic tongue taking up to 2 weeks to slough off.

The most common ligature instrument was the écraseur, which literally means crusher in French. An instrument that resembled a snare, the écraseur had, instead of a wire loop, one made of chain links like those found on a chain saw or bicycle. At the end of the snare handle was a screw that tightened the chain. This instrument was applied across the portion of the tongue that was to be removed and tightened one link every hour until the necrosing portion was removed. Sometimes, the écraseur was tightened at a rate of 1 notch every 2 minutes until the écraseur cut through the tongue rather than necrosing it. The complications reported were great, but those who survived did well in terms of swallowing and speech.

In 1900, Butlin and Spencer severely condemned all previous treatments and stated, "There is only one treatment—wedge shaped excision." This remains the standard today, although it has been modified in a number of different ways since then.

Problem

Multiple studies have attempted to define macroglossia by objective measurements based on a variety of clinical and radiographic tests. However, because of the difficulty in performing these tests and because intervention is not based on measurements but on clinical presentation, macroglossia is most often diagnosed subjectively.

Ueyama and others defined macroglossia as occurring when 1 of the following 3 criteria is met:¹ (1) extravasation of the lingual apex or lingual border onto or outside the dentition; (2) the impression of one or more teeth on the lingual border visualized when the mouth is open; or (3) following surgery for correction, a relapse of increased interdental space, open bite deformity, and/or jaw deformation with malocclusion occurs.

As with many lesions, medicine has identified a triad for those with macroglossia. It includes open bite deformity, mandibular prognathism, and malalignment.

Frequency

Although the exact incidence of macroglossia is unknown (because the etiologies are too numerous to quantify), some congenital syndromes often express macroglossia in their phenotypes, most commonly Down syndrome (1 per 700 live births) and Beckwith-Wiedemann syndrome (0.07 pre 1000 live births). In Beckwith-Wiedemann syndrome, 97.5% of patients have macroglossia. The literature documents 2 families with autosomal dominant inheritance of isolated macroglossia.

Etiology

Reports on the etiology of macroglossia are extensive. Historically, Virchow described it as a form of elephantiasis. In the last 100 years, Butlin and Spencer attributed it to the dilation of lymphatics, muscle hypertrophy, or inflammation. Because of the large number of possible etiologies, multiple classification schemes have been used to list the causes. Detailed below is the most comprehensive in the author's opinion.

The 2 broadest categories under the heading of macroglossia are true enlargement and pseudomacroglossia.

Pseudomacroglossia includes any of the following conditions, which force the tongue to sit in an abnormal position:

• Habitual posturing of the tongue
• Enlarged tonsils and/or adenoids displacing tongue
• Low palate and decreased oral cavity volume displacing tongue
• Transverse, vertical, or anterior/posterior deficiency in the maxillary or mandibular arches displacing the tongue
• Severe mandibular deficiency (retrognathism)
• Neoplasms displacing the tongue
• Hypotonia of the tongue

True macroglossia can be subdivided into 2 main subcategories: congenital causes and acquired causes.

• Congenital causes
• • Idiopathic muscle hypertrophy
  • Gland hyperplasia
  • Hemangioma
  • Lymphangioma
  • Down syndrome
  • Beckwith-Wiedemann syndrome
  • Behmel syndrome
  • Lingual thyroid
  • Gargoylism
  • Transient neonatal diabetes mellitus
  • Trisomy 22
  • Laband syndrome
  • Lethal dwarfism of Blomstrand
  • Mucopolysaccharidoses
  • Skeletal dysplasia of Urbach
  • Tollner syndrome
  • Autosomal dominant inheritance
  • Microcephaly and hamartoma of Wiedemann
  • Ganglioside storage disease type I
• Acquired causes (Categories have been assigned to simplify the list, but there can be overlap of a particular etiology into more than one of these categories.)
• • Metabolic/endocrine
  • • Hypothyroidism
    • Cretinism
    • Diabetes
  • Inflammatory/infectious
  • • Syphilis
    • Amebic dysentery
    • Ludwig angina
    • Pneumonia
    • Pemphigus vulgaris
    • Rheumatic fever
    • Smallpox
    • Typhoid
    • Tuberculosis
    • Actinomycosis
    • Giant cell arteritis
    • Candidiasis
    • Scurvy
    • Pellagra
  • Systemic/medical conditions
  • • Uremia
    • Myxedema
    • Hypertrophy
    • Acromegaly
    • Neurofibromatosis
    • Iatrogenic macroglossia
  • Traumatic
  • • Surgery
    • Hemorrhage
    • Direct trauma (eg, biting)
    • Intubation injury
    • Radiation therapy
  • Neoplastic
  • • Lingual thyroid
    • Lymphangioma
    • Hemangioma
    • Carcinoma
    • Plasmacytoma
  • Infiltrative
  • • Amyloidosis
    • Sarcoidosis

Pathophysiology

Because the pathophysiology of the enlarged tongue is related to the specific etiology, defining the pathophysiology of each is beyond the scope of this discussion. However, in all cases, the locoregional complications of macroglossia are generally the same relative to the magnitude of the enlargement. Mechanical obstruction in the oral cavity can directly occlude the airway. This occlusion is usually worsened by lying supine when an enlarged tongue base is more directly acted upon by gravity to block the oropharynx and hypopharynx. Depending on muscle mass and tone, speech and swallowing may be affected as well.

Several studies document the role of the tongue in shaping the oral cavity. Just as reduced pressure of the tongue on the palate and mandible may lead to an adenoid facies, increased pressure on the surrounding anatomy can have opposite effects. Upper incisors can be pushed horizontally, inducing forward maxillary growth. Other morphologic changes include open bite deformities, prognathism, class III malocclusion, anterior and/or posterior crossbites, buccal tipping of posterior teeth, accentuated curve of Spee in the maxillary arch, reverse curve of Spee in the mandibular arch, increased transverse width of mandibular and/or maxillary arches. Furthermore, difficulty with mastication may lead to temporomandibular joint pain.

If the tongue protrudes beyond the lips and is exposed to the air, drying of the tongue with resultant glossitis and bleeding is common. Prior to the 1900s, this was not an uncommon occurrence for patients.

Clinical

When obtaining the history of a patient with macroglossia, first direct questions toward determining whether an emergency situation exists. General presentation provides clear indication in most situations, but adequate assessment of the patient's ability to breathe, swallow, and speak should be obtained quickly.

If no emergent intervention is required, focus the history on possible causes for macroglossia because this determines appropriate treatment.

Congenital syndromes are often known prior to the presentation of macroglossia (eg, Down syndrome). Family history is crucial in discerning possible congenital causes. These syndromes are not always easy for the otolaryngologist or primary care physician to diagnose; therefore, consult a geneticist or other such specialist if warranted.

Should the cause be unknown to the patient and not obvious on initial presentation, further investigation is indicated. To simplify the process of narrowing the enormous list of differential diagnoses, direct questions toward the etiology class (eg, inflammatory versus neoplastic). Once the class is determined, specific causes are more easily discovered.

The physical examination can significantly aid diagnosis. Again, because of the nature of this entity, assess the airway. Typically, an astute clinician does this during the initial history gathering. Take note of signs related to potential airway compromise such as stridor, turgor, drooling, and poor or muffled speech.

Physical examination of the tongue alone should reveal signs of the cause or at least help rule out the classes that are not involved. For example, hemangiomas can often be observed on the surface of the tongue in a variety of positions. But, physical examination should always include palpation of the tongue to help diagnose discreet masses not prominent on the lingual surface. Infectious processes often present with characteristic findings, such as the white plaques pathognomonic for candidiasis. A lack of any unusual masses, discolorations, or other obvious lesions may lead the physician to consider amyloidosis or other more cryptic etiologies.

Physical examination of the oral cavity and head morphology is helpful to deduce true macroglossia from pseudomacroglossia. Severe retrognathia and unusually small maxillary and/or mandibular size may indicate the latter. In addition, note tongue tone and mobility to rule out simple atonia or hypotonia indicating poor posturing of the tongue (as is commonly observed in Down syndrome).

In addition to the oral cavity and airway, assess other features in the patient that may indicate congenital or systemic syndromes. Certain vitamin deficiencies may manifest with angular stomatitis, nonpitting edema of the lower extremities may indicate hypothyroidism, and unusual body morphologies may indicate the early signs of diseases like acromegaly.

The physical findings listed in Pathophysiology may be noted during oromaxillofacial examination.

Because of the large range of possible causes, take a broad history and perform a complete physical examination of the patient with macroglossia. Failure to do so may result in missing a diagnosis that may otherwise have been made.

INDICATIONS

Section 3 of 11  

• Authors and Editors
• Introduction
• Indications
• RELEVANT ANATOMY
• Contraindications
• Workup
• Treatment
• Complications
• Outcome And Prognosis
• Future And Controversies
• References

Indications for surgical intervention are varied. The most important is airway compromise. A tracheostomy may be required as a first step in surgical care (in some cases an elective tracheostomy is performed prior to surgical correction). Other indications include dysarthria, dysphagia, and cosmesis.

The goal of nearly all surgery is to return the patient to an anatomically and physiologically normal condition; the same is also true in surgery for macroglossia. The goal is to reduce tongue size and thereby improve function. Those main functions include articulation, mastication, deglutition, protection of the airway, and gustation. Of these, only gustation is not often improved with surgical intervention.

RELEVANT ANATOMY

Section 4 of 11  

• Authors and Editors
• Introduction
• Indications
• RELEVANT ANATOMY
• Contraindications
• Workup
• Treatment
• Complications
• Outcome And Prognosis
• Future And Controversies
• References

The lingual anatomy is relatively simple, although its complex 3-dimensional location makes it more interesting.

Four intrinsic and 4 extrinsic muscles control the motion of the tongue.

• Extrinsic muscles (named by their attachments, with their function and innervation)
  • Genioglossus - Protrusion of tongue apex from the mouth and depression of the tongue center allowing it to take a concave form, hypoglossal nerve
  • Hyoglossus - Depression of the tongue, hypoglossal nerve
  • Chondroglossus (often considered a portion of the hyoglossus) - Depression of the tongue (identified as separate entity based on embryologic origin), hypoglossal nerve
  • Styloglossus - Elevates and retracts the tongue, hypoglossal nerve
  • Palatoglossus - Elevates and retracts the tongue, vagus nerve
• Intrinsic muscles of the tongue (with their function, all are innervated by the hypoglossal nerve)
  • Superior longitudinal muscle - Shortens tongue, turns apex and sides upwards to create a concave dorsum
  • Inferior longitudinal muscle - Shortens tongue, turns apex and sides downwards to create a convex dorsum
  • Transverse muscle - Narrows and elongates the tongue
  • Vertical muscle - Flattens and widens the tongue

The main artery of the tongue is the lingual branch of the external carotid. However, contributing arteries include the tonsillar branch of the facial artery and the ascending palatine branch of the ascending pharyngeal artery. An extensive submucosal plexus is responsible for the vigorous bleeding with even superficial wounds.

• Sensory nerves (with their target innervation)
  • Lingual branch of V2 of CN V - General sensation for the anterior two thirds of the tongue
  • Chorda tympani of CN VII - Taste for the anterior two thirds of the tongue
  • Lingual branch of CN IX - General sensation and taste for the posterior one third
  • Superior laryngeal branch of CN X - Root of tongue at lingual base of epiglottis

A relatively avascular median fibrous septum creates a partition along the length of nearly the entire tongue. It anchors to the hyoid bone. Clinically it can serve as a site for placement of an anchor stitch when significant traction of the tongue is required in the operating room.

Multiple minor salivary glands are present in the tongue and consist of all 3 types, mucous, serous, and mixed.

The foramen caecum can be viewed in the midline of the tongue just posterior to the vallate papillae. It marks the origin of the thyroid gland in the embryo. Persistent thyroid tissue may be present in this location, or it may remain as the end orifice for a thyroglossal duct cyst.

CONTRAINDICATIONS

Section 5 of 11  

• Authors and Editors
• Introduction
• Indications
• RELEVANT ANATOMY
• Contraindications
• Workup
• Treatment
• Complications
• Outcome And Prognosis
• Future And Controversies
• References

As with all intervention, whether medical or surgical, the benefits of the operation must outweigh the risks. Relative contraindications are those associated with most surgeries and include coagulopathies and other comorbidities that make general anesthesia more dangerous. In the pediatric population, many cases of macroglossia are associated with syndromes that may have lesions that increase the risk of general anesthesia.

WORKUP

Section 6 of 11  

• Authors and Editors
• Introduction
• Indications
• RELEVANT ANATOMY
• Contraindications
• Workup
• Treatment
• Complications
• Outcome And Prognosis
• Future And Controversies
• References

Lab Studies

• No specific lab studies are useful for diagnosing macroglossia as an isolated entity. If it is considered to be a symptom of another disease (eg, vitamin deficiency, sarcoidosis, hypothyroidism), lab studies should be obtained to determine the cause. Although numerous etiologies are possible, careful consideration of patient history is appropriate in determining which lab studies to obtain.

Imaging Studies

• Imaging studies for macroglossia are not crucial to the diagnosis of the entity itself but may aid in the diagnosis of its etiology.
• An extremely useful imaging modality, CT scanning can provide ample information regarding the morphology of the tongue.
• • In combination with nasopharyngoscopy, a CT scan is highly useful in excluding neoplasm as the cause for the macroglossia and determining the safety of the airway.
  • Contrast material is recommended for this study. Vascular lesions account for many cases of macroglossia, with lymphangioma being the single most common cause. Without contrast material, in some cases, distinguishing solid lesions from cystic lesions may be difficult.
  • CT scanning also provides the clinician with an exact representation of the size of the tongue. As stated earlier, multiple studies have attempted to define macroglossia by specific cephalometric correlations, but knowing the size is the greatest aid in determining the amount of tissue to resect should surgery be indicated.
• Magnetic resonance imaging (MRI) is another useful modality in assessing the potential cause of macroglossia. It can also provide the definitive size of the tongue. Long recognized as the imaging study of choice when soft tissue resolution is paramount, MRI is not necessarily superior to CT scanning for macroglossia, particularly in the following circumstances:
• • First, when a faster test is desirable: CT scan can be obtained quickly; for instance, sedation may be necessary to perform an MRI in a pediatric patient, increasing the time required.
  • Second, when a neoplasm, and in particular a malignancy, is not suspected: Clearly identifying margins with MRI is not usually necessary.
• If a particular patient's macroglossia is associated with severe obstructive sleep apnea, chest radiography may be useful in evaluating heart size. Further cardiac imaging may then be indicated if an ECG confirms possible right heart failure.
• If lingual thyroid is a likely cause, an ultrasound of the neck is appropriate to determine if thyroid tissue exists in the normal anatomical position. More than 70% of patients with a lingual thyroid fail to have thyroid tissue in the anterior base of neck, which has obvious consequences when considering resection of the lingual mass.
• Panorex and other dental films may be useful if oromaxillofacial surgery for correction of abnormal dentition is required after primary treatment.

Other Tests

• In even mild cases, swallow studies may be indicated in a history of dysphagia or aspiration.

Diagnostic Procedures

• Diagnostic procedures must be performed according to the suspected etiology. Any physician who has taken a tongue biopsy knows that significant bleeding can occur. Certainly this potential is even greater with vascular or even inflammatory lesions.
• Much of the time, especially for base of tongue lesions, biopsy can be performed safely only in the operating room. This can be perilous because intubation may not be easily completed in those with obstructing tongue lesions. Nevertheless, a controlled environment is necessary for manipulation of the base of tongue.
• Biopsies of small anterior tongue lesions can usually be safely obtained in the clinic, but these entities are often not responsible for the global enlargement demonstrated in macroglossia.
• Fine-needle aspiration (FNA) may not be adequate for diagnosis. For example, an FNA cannot be used to diagnose lymphomas. Clinical judgment should be used to determine whether FNA is able to obtain an appropriate amount of tissue for assessment given a narrowed differential diagnosis.

Histologic Findings

Given the vast differential diagnosis, listing all the possible histological findings would be imprudent. However, in both Down syndrome and Beckwith-Wiedemann syndrome, tongue muscles show hypertrophy and hyperplasia.

Staging

Macroglossia alone does not have a specific staging system.

A staging system does exist for cancers of the tongue that is divided into whether the oral or hypopharyngeal tongue is involved.

Table 1. Oral Cavity Cancers

Table 2. Hypopharyngeal Cancers

Table 3. Regional Lymph Nodes

Table 4. Distant Metastases

Table 5. Staging System for Cancers of the Tongue

TREATMENT

Section 7 of 11  

• Authors and Editors
• Introduction
• Indications
• RELEVANT ANATOMY
• Contraindications
• Workup
• Treatment
• Complications
• Outcome And Prognosis
• Future And Controversies
• References

Medical therapy

Medical therapy for macroglossia is only useful when the etiology of the disease is a clearly defined medically treatable entity such as hypothyroidism, diabetes, infections, or inflammatory conditions such as amyloidosis. No medical treatments have proven useful when the etiology is unclear or the histology reveals simple hypertrophy/hyperplasia.

Many minor procedures that can be performed in the office have been attempted on large tongues thought to contribute to obstructive sleep apnea. Such interventions include sclerosing agents, cautery, or other forms of soft tissue destruction with Somnus or Coblation technology. These techniques are aimed at tongue bases slightly larger than the norm and not at the global enlargement of the tongue seen in macroglossia. However, the primary author has twice successfully used radiofrequency reduction of the anterior two thirds of the tongue on 2 patients with macroglossia from amyloidosis.

Neoplasms certainly may be managed by chemoradiation, depending on their type and susceptibility, but neoplasms often require surgical intervention as well. An excellent discussion of the decision making process is found in Malignant Tumors of the Base of the Tongue and Malignant Tumors of the Mobile Tongue.

One form of medical therapy has shown promise. The Castillo-Morales orofacial therapy was developed in the mid 1970s. This method has been most successful in cases of pseudomacroglossia where the problem is hypotonicity. A manual stimulation and facilitation treatment is performed using a palatal prosthesis. The prosthesis or plate has a pair of electrical stimulators in it. When placed in the mouth a reflexive action of the tongue to seek this foreign body occurs, moving the tongue backward and upward to meet it. When the tongue meets the plate, it activates the lingual electrode that further stimulates the tongue into the backward and upward position, rather than the hypotonic position of downward and forward.

Preoperative details

The scope of this article includes surgery for macroglossia as it pertains to reducing the hyperplastic, hypertrophic tongue. Surgery for macroglossia in a patient with a neoplastic lesion requires oncologic resection and is not discussed.

Preoperative planning should include a thorough knowledge of the patient's tongue size and the desired decrease in size. Nearly all of the surgical techniques used today allow for variation in the amount of tissue removed based on intraoperative findings. As all surgeons are aware, it is easy to take more but not easy to put back what was removed.

Probably the most important decision in the preoperative plan is to determine whether intubation or tracheostomy will be the primary means of airway control. Tracheostomy is almost universally short term but may be necessary, especially in younger patients in whom reintubation would be much more difficult.

Intraoperative details

Several authors have espoused multiple shapes of resection. All of these have proven to be effective in restoring function back to the tongue. However, some resection types have shown improved physiological outcome and closer return to homeostasis than others.

The keyhole method of resection has been the most popular resection type over the last 50 years. It reduces not only the anterior-posterior dimension of the tongue but also its width, yet the classic description of this procedure involves the resection of the tip of the tongue and a T-line closure. Although this allows a greater resection of the anterior extent of the tongue, it also prevents the use of the important tip musculature for articulation and other fine motor movements. Mixter et al also reported that this method of reduction could lead to an ankylosed globular tongue with an insensitive tip.² He advocated central debulking of the tongue using a W-shaped incision in the middle two-thirds. The greatest threat in this type of resection is more significant and prolonged swelling.

The type of surgical resection favored by many authors is a variation of the keyhole resection that does not involve the tip of the tongue. Described by Ueyama and others in 1999, this resection inverts the keyhole design so that the base of the keyhole is posterior on the tongue and the anterior incision does not come to the tip of the tongue, thus preserving function.¹ However, if an enormous anterior projection of the tongue is present, this type of resection may not be able to adequately reduce the size. Fortunately, extending the incision to involve the tip in such cases is not difficult.

However, this author no longer favors surgical excision of tongue tissue as a primary intervention. Rather, radiofrequency reduction of the tongue is used. Two methods are used. First (and least aggressive) is reduction of the tongue by submucosally inserting the turbinate Coblation wand (ArthroCare) into the area of the tongue that requires reduction, which is often the base of the tongue for obstructive sleep apnea (OSA). The insertion point is tested with a quick application of the Coblation technology to ensure that no fasciculation of tongue musculature is present. If fasciculation of the tongues is present, the wand is repositioned because this may indicate close proximity to the hypoglossal nerve. Typically, the wand is held in place for approximately 10 seconds on a setting of 4. It is withdrawn without any energy delivery. Hemostasis is spontaneous.

The second method, which is more aggressive, is to use the tonsil wand for a similar albeit greater submucosal reduction. The author is not aware of any literature that describes this procedure with long-term results for adults. Maturo and Mair described a similar technique on pediatric patients in 2006.³ The author uses a technique based on a presentation by Michael Friedman, MD, at the 2006 American Academy of Otolaryngology–Head & Neck Surgery meeting held in Toronto, Canada.

A 2-0 silk suture is placed through the midline of the tongue to help with retraction. Often a second, more posterior suture is placed for additional support. The tongue is retracted out of the oral cavity. At approximately half the distance between the tip of the tongue and the circumvallate papillae, local anesthetic with epinephrine is injected into the submucosa. After adequate time has been allowed for vasoconstriction, a 1 cm longitudinal incision is made at the same location, only deep enough to penetrate the mucosa.

A Coblation tonsil wand (ArthroCare) is then inserted into the incision and a submucosal lesion is created. The lesion extends from the incision to the base of the tongue posterior to the circumvallate papillae. The lesion is maintained within 1 cm of the midline on either side to prevent injury to the neurovascular bundles. Small fasciculations are common, but if any large ones are seen, the wand is kept closer to the midline. An index finger is placed over the mucosa of the base of the tongue to ensure the Coblation wand does not penetrate through the mucosa here. The wand is removed after a subjectively adequate lesion is created. Hemostasis is usually spontaneous but can be facilitated by closing the 1 cm incision with 3-0 chromic suture.

The author has performed 12 of these procedures, as of this article update, without postoperative taste, sensation, or movement abnormalities. These procedures also result in seemingly good results visually, as well as on postoperative polysomnography (as most of the patients who undergo this procedure have OSA). Results have yet to be assessed using statistical measures for significance. All patients are monitored overnight in the ICU. When performed alone, the postoperative pain does not seem to be as significant or long-lasting as it is for patients who undergo uvulopalatopharyngoplasty.

Postoperative details

Postoperatively, swelling is the greatest threat to the patient. If a tracheostomy is performed for surgery, this threat is largely bypassed. Postoperative antibiotics (for a contaminated but relatively low-risk wound) and steroids are recommended.

Pain control is essential. Limiting tongue motion postoperatively also aids in decreasing inflammation and pain. However, because the tongue is a muscle, a quick return to function greatly aids in shortening the time to normal recovery. Cold, clear liquids are excellent early troches for the tongue.

Follow-up

Follow-up care consists essentially of routine monitoring of wound healing and largely depends on the surgical method used for reduction. This author routinely sees the submucous Coblation patients 3 weeks postoperatively and then at 3 months. For surgical resection patients, they are often seen more frequently to assess the larger lingual incisions and ensure adequate wound healing.

Depending on the complications of the macroglossia, the patient may also need to be monitored by a dentist or oromaxillofacial surgeon. In addition, because of the enormous range of functions of the tongue, referral to a speech therapist may be appropriate, especially for children who demonstrate preoperative speech impediments.

COMPLICATIONS

Section 8 of 11  

• Authors and Editors
• Introduction
• Indications
• RELEVANT ANATOMY
• Contraindications
• Workup
• Treatment
• Complications
• Outcome And Prognosis
• Future And Controversies
• References

The most common complication of macroglossia surgery (really an expected sequelae of resection) is swelling. For this reason, the patient may need to remain intubated for a few days. Postoperative intubation has been used for surgical resection cases but has not yet been required for those patients who undergo submucous reduction with the Coblation technique. Tracheostomy clearly obviates the need for postoperative intubation but should be considered aggressive treatment only to be used in appropriate patients.

The tongue is a highly vascular entity and remarkably resistant to infection. Even in a contaminated field, infection is not likely. However, antibiotics given should cover for gram-positive and anaerobic organisms. Both amoxicillin and clindamycin have adequate coverage for most patients. This author routinely uses postoperative antibiotics for 1 week.

Despite its vascularity, hematoma is not as common as one would think because of the compressive strength inherent in the tongue musculature. The tongue has an enormous capacity for rapid healing. However, if a hematoma does occur, it represents an immediate airway risk and should be treated as an emergency. The avoidance of preoperative anticoagulants (including nonsteroidal anti-inflammatory drugs [NSAIDs]) is recommended.

Inappropriate reduction is another complication. Resecting too much of the tongue may be more detrimental than resecting too little, although the author favors neither. Leaving too much bulk can result in continued, albeit improved, symptoms. Skeletal defects are difficult to correct if reduction to appropriate size is not attained. Taking too much tissue may reduce function, especially mastication, deglutition, and articulation. Because of the remarkable ability of this organ to respond to its oral environment despite size, taking too much tissue from the tongue during this procedure is difficult. Many patients with cancer have lost significant portions of their tongue and have been able to communicate and eat without difficulty.

The final complication to consider, but one that will contribute to significant loss of quality of life for the patient, is neural injury. This can occur in the form of hypoglossal injury leading to unilateral and occasionally bilateral loss of tongue mobility, or from a sensory loss with injury to the lingual nerve. Unfortunately, surgical resection of tongue tissue often requires significant cautery, which may result in indirect trauma even if the nerve is not exposed. Nerve injury has not yet been encountered using the Coblation technique but is certainly a risk with which every potential patient needs to be familiar. Postoperative steroids may help prevent postoperative inflammatory injury and can help limit damage in a nonsevered nerve. If severed, return of function is difficult at best and repair is beyond the scope of this discussion.

OUTCOME AND PROGNOSIS

Section 9 of 11  

• Authors and Editors
• Introduction
• Indications
• RELEVANT ANATOMY
• Contraindications
• Workup
• Treatment
• Complications
• Outcome And Prognosis
• Future And Controversies
• References

The literature is riddled with small series studies that report mostly good results. The original wedge resection has even shown promising results in recent studies. Siddiqui and Pensler published a study of 9 patients with macroglossia evenly divided in cause among Beckwith-Wiedemann, Down syndrome, and lymphangioma. After treatment, 62% had improved speech intelligibility. Of the 6 patients with preoperative oral incompetence, 50% had long-term improvement. Postoperative improvement was shown by 4 of the 5 patients who had preoperative drooling and 4 of the 5 who had dysphagia.⁴

Morgan et al reported a series of 5 patients who underwent keyhole resections for congenital macroglossia. They stated all 5 had improved cosmesis and function of oropharyngeal airway without a change in speech or feeding.⁵

Harada and Enomoto originally described the inverted keyhole resection. Although they did not give quantitative results, they concluded that all patients have essentially normal speech, mobility, and taste postoperatively.⁶

In 3 case reports, Mixter et al reported that they had no worsening in speech with their W-shaped resection; conditions in sleep studies postoperatively improved in all their patients. This study did not provide objective data. They reported on an uncommon complication known as the lingual-cardiac reflex. This phenomenon occurs when excessive tongue retraction leads to bradycardia. In this case report, the bradycardia led to asystole, which was treated to resolution without sequelae with 30 seconds of advanced cardiac life support (ACLS) protocol.²

The overall results of the submucous Coblation techniques have not been extensively reported in the literature, but oral presentations at national meetings show promise.

FUTURE AND CONTROVERSIES

Section 10 of 11  

• Authors and Editors
• Introduction
• Indications
• RELEVANT ANATOMY
• Contraindications
• Workup
• Treatment
• Complications
• Outcome And Prognosis
• Future And Controversies
• References

If the radiofrequency techniques prove to have significant long-term results, their advantages over surgical resection may result in the largest paradigm shift in macroglossia intervention in the last 50 years. More published data are required before any conclusions can be drawn.

The surgical technique used is the main controversy. One author's success may be the bane of another. The surgeon should be familiar with the many options available and plan accordingly. The art of medicine truly still plays a large role in macroglossia from diagnosis to treatment.

REFERENCES

Section 11 of 11

• Authors and Editors
• Introduction
• Indications
• RELEVANT ANATOMY
• Contraindications
• Workup
• Treatment
• Complications
• Outcome And Prognosis
• Future And Controversies
• References

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Article Last Updated: Nov 6, 2007