Macroglossia Treatment & Management

In general, treatment approaches for macroglossia are complex, given the condition's association with systemic diseases and hereditary syndromes. Moreover, there is a wide range of surgical approaches that have been used but a lack of general consensus. ^[1]

Medical therapy for macroglossia is useful only when the etiology of the disease is a clearly defined, medically treatable entity such as hypothyroidism, ^[5] infection, or amyloidosis. No medical treatments have proven useful when the etiology is unclear or the histology reveals simple hypertrophy or hyperplasia.

Many minor procedures that can be performed in the office have been attempted on large tongues thought to contribute to obstructive sleep apnea. ^[6] Such interventions include the use of sclerosing agents, cautery, or other forms of soft tissue destruction. These techniques are aimed at tongue bases slightly larger than the norm and not at the global enlargement of the tongue seen in macroglossia.

Malignant neoplasms certainly may be managed by surgery and chemoradiation, depending on their type and susceptibility, but benign neoplasms in the anterior part of the tongue are often resected surgically.

Mechanical therapy may be useful in macroglossia with hypotonicity due to hypoglossal nerve deficit. Such treatment can be carried out using complex electrical stimulation and construction of a palatal prosthesis, which can be connected to the stimulation device. When the prosthesis is placed in the mouth, the tongue reflexively seeks this foreign body, moving backward and upward to meet it. When the tongue touches the prosthesis, it activates a lingual electrode, which further stimulates the tongue into the backward and upward position. ^[12]

For patients in whom macroglossia is protrusive from the oral cavity, compressive dressings using 3M Coban wrap have been tried with some success. ^[13]

 

 

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 can be seen elsewhere.

Preoperative planning for benign macroglossia management 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. 

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 if the posterior part of the tongue is involved.

Multiple shapes of keyhole tongue resection are utilized, depending on the size of the macroglossia. All of these have proven to be effective in restoring tongue function, but some resection types have shown better results than others with regard to physiologic outcome and return to homeostasis.

The keyhole method of resection remains the most popular resection type. 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. Although this allows a greater resection of the anterior extent of the tongue, it also may sacrifice tip musculature important for articulation and other fine motor movements. Central debulking of the tongue using wide-shaped, elliptical excisions in the middle two thirds may cause more significant and prolonged swelling.

The type of surgical resection favored by many surgeons is a variation of the keyhole resection that does not involve the tip of the tongue. However, if an enormous anterior projection of the tongue is present, this type of resection may not provide adequate size reduction. Extending the incision to involve the tip in such cases may be indicated.

To facilitate surgical excision, 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, and 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 passed for vasoconstriction, a longitudinal incision, only deep enough to penetrate the mucosa, is made at the same location.

The submucosal incision is extended to the base of the tongue, posterior to the circumvallate papillae. The incision is maintained within the midline on either side to prevent injury to the neurovascular bundles. The desired portion of the tongue is typically excised with monopolar cautery. Powered instrumentation such as bipolar or ultrasonic shears, or suture ligature, can be used for hemostasis.

After the excised portion of the tongue is removed, the adjacent segments are approximated and sutured from inside out with absorbable sutures such as Vicryl.

Although macroglossia due to neoplasms is not addressed in detail in this article, the following images demonstrate lesion removal and tongue repair.

Swelling is the greatest postoperative threat to the patient, although if a tracheostomy is performed prior to surgery, this threat is largely bypassed.

Antibiotics and steroids are administered postoperatively to the patient, while limiting tongue motion after surgery 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 for early soothing of the tongue. When tongue swelling no longer presents a threat to the airway and the patient is able to tolerate an appropriate oral diet, the patient may be released from the hospital. A hospital stay of several days may be required, particularly in pediatric patients.

Follow-up care consists essentially of routine monitoring of wound healing and largely depends on the surgical method used for reduction. Adequate oral intake should be assessed for those without gastrostomy tubes at baseline.  

Depending on the complications of the macroglossia, the patient may also need to be monitored by a dentist or oral and maxillofacial surgeon. In addition, referral to a speech therapist may be appropriate, especially for children who demonstrate preoperative speech impediments.

The most common complication of macroglossia surgery is postoperative swelling. For this reason, the patient may need to remain intubated for a few days. 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 organ and remarkably resistant to infection. Even in a contaminated field, infection is not likely.

Despite the tongue's vascularity, hematoma is not very common, because of the compressive strength inherent in the tongue musculature. The tongue tends to heal rapidly due to enhanced vascularity. However, if a hematoma does occur, it represents an immediate airway risk and should be treated as an emergency.

Inappropriate reduction is another surgical complication. Resecting too much of the tongue may be more detrimental than resecting too little, as this may reduce function, especially with regard to mastication, deglutition, and articulation. However, the tongue has a remarkable ability to respond to its oral environment despite its size.

Although injury to the hypoglossal nerve is rare, such injury, if it occurs, leads to unilateral and occasionally bilateral loss of tongue function and mobility. Injury to the lingual nerve leads to sensation loss.

The literature is riddled with small series studies that report mostly good results in macroglossia surgery. Although immediate postoperative dysphagia and drooling have been reported, most studies have shown that surgical procedures for macroglossia lead to improved cosmesis, speech, mastication and feeding. ^[14]

A study by Cohen et al indicated that in patients with Beckwith-Wiedemann syndrome, surgical tongue reduction can be safely and successfully carried out even prior to age 12 months. Tongue reduction in the study was carried out at median age 9.5 months. The obstructive apnea–hypopnea index for patients with severe obstructive sleep apnea was found to improve from 30.9 per hour to 10.0 per hour, while nadir oxyhemoglobin saturation rose from 72% to 83%. However, although clinically meaningful improvement in supplemental feeding tube or respiratory support occurred in specific patients, no significant change in these was seen overall. ^[15]