If you don't remember your password, you can reset it by entering your email address and clicking the Reset Password button. You will then receive an email that contains a secure link for resetting your password
If the address matches a valid account an email will be sent to __email__ with instructions for resetting your password
Address reprint requests and correspondence: Thomas D. Samson, MD, FAAP, FACS, Division of Plastic Surgery, Penn State Milton S. Hershey Medical Center, 500 University Dr, Hershey, PA 17033.
Macroglossia, although relatively uncommon, can lead to significant morbidity such as compensatory mandibular overgrowth, compromised oral competence, and chronic exposure and breakdown of tongue mucosa. Surgical debulking of the enlarged tongue is reserved for patients with significant morbidity. The methods of surgical correction of macroglossia are many, none of which are superior. Early techniques focused on reducing the tongue through nonspecific debulking, but more current techniques focus on preserving lingual mobility, maintaining adequate length and avoiding injury to the neurovascular bundles. The keyhole technique is a specific technique that incorporates both an anterior wedge resection and a central tongue reduction. This allows for protection of the lateral neurovascular bundles while performing significant debulking of the tongue. Postoperative results have demonstrated good mobility and positive effects on speech.
Macroglossia, or enlargement of the tongue, is a relatively uncommon condition in children. Although definitions of macroglossia have been proposed, there is no unified objective definition on what constitutes the deformity. A classification system for macroglossia has been formed that delineates true macroglossia and relative macroglossia.
True macroglossia is defined by intrinsic pathology within the tongue, such as increased or enlarged lingual structure or even the presence of atypical elements. Common examples for true macroglossia include vascular malformations, muscular enlargement, as seen in Beckwith-Wiedemann syndrome, and systemic diseases such as Hurler syndrome. These are distinct from relative macroglossia, in which the tongue is histologically normal but simply enlarged, as seen in Down syndrome.
The symptoms of macroglossia, whether true or relative in nature, are broad and result from the increased tongue size relative to the oral cavity. They can range from mild symptoms such as noisy breathing, increased drooling, and cosmetic and dental deformities to more severe symptoms such as difficulty in feeding, disrupted speech, and airway compromise.
The number of techniques is a testament to the lack of efficacy of each technique. Methods for surgical correction include wedge resection, central resection, de-epithelization, elliptical excisions, and peripheral excision. All these procedures aim to decrease overall tongue size by accounting for length, width, and vertical height reduction while preserving normal tongue function.
The keyhole technique, first described by Morgan et al,
combines both anterior wedge resection as well as a central tongue reduction. The benefits of this technique are that it preserves the lateral neurovascular bundles while avoiding complete deepithelialization of the tongue. The long-term studies of the keyhole technique have shown improved clinical outcomes. A review of patients with Beckwith-Wiedemann syndrome who underwent surgical correction for macroglossia demonstrated central tongue reduction and anterior wedge resection to be preferable when aiming to reduce tongue length and retain a tapered tip.
Similarly, a recent study investigating long-term outcomes regarding speech demonstrated this technique to have significant improvement in overall speech intelligibility, articulation, and oral behavior.
Long-term impact of tongue reduction on speech intelligibility, articulation and oromyofunctional behaviour in a child with Beckwith-Wiedemann syndrome.
Surgical correction of macroglossia is largely based on the patient׳s symptoms. Severe symptoms, such as airway compromise and dysphagia leading to failure to thrive, are usually identified early in the patient׳s life. Other symptoms, such as malocclusion, articulation disorders, drooling, troubles with speech, and cosmetic appearance, are also indications for performing surgery.
Surgical intervention should be delayed until at least 6 months of age, except for severe cases in which reduction may prevent the need for a surgical airway or prolonged nasogastric feeds. The risks of early intervention, however, carry an increased anesthetic risk, as well as a risk of tongue regrowth postoperatively.
The patient is nasotracheally intubated and the tube is sutured in with a #0 permanent suture through the membranous septum. The airway is securely sutured in place owing to the prolonged postoperative intubation secondary to lingual edema. Intravenous antibiotics are administered.
We begin by mobilizing the tongue to assess the true size with the patient relaxed. Two 3-0 nylon stay sutures are placed at the anterolateral aspect of the tongue. These sutures are important to allow complete mobilization of the tongue without manipulation of the muscle (Figure 1). The modified keyhole is drawn onto the tongue surface (Figure 2, Figure 3). The width of the anterior wedge is designed to correct for the degree of tongue protrusion. If tongue thickness is excessive, lateral wedges are designed into the excision. The surgical markings are reinforced with methylene blue tattooing. Local anesthesia with epinephrine is infiltrated along the proposed incision.
Figure 1Preoperative view of macroglossia. (Color version of figure is available online.)
Using the cut mode on a Bovie needle point coagulator, the mucosal edge is incised. We then use coagulation mode to come through the bulk of the muscle while protecting the lateral neurovascular bundles. Blood loss is well controlled using the coagulation mode.
Once the specimen is removed (Figure 4), hemostasis is assured and we begin closure. Closure is obtained with 3-0 and 4-0 VICRYL interrupted sutures. Multiple rows are placed through the bulk of the muscular tongue. We then use 4-0 VICRYL in a horizontal mattress fashion to evert the mucosal edges of the tongue (Figure 5). One of the stay sutures is left in place and Steri-Stripped to the side of the patient׳s cheek. This can be used for emergent control of the tongue if needed postoperatively. After the operation the patient remains intubated and is taken to the pediatric intensive care unit.
Figure 4Intraoperative resection of the tongue. (Color version of figure is available online.)
The patient is positioned with the head elevated and perioperative steroids are administered. The patient is extubated once the lingual edema subsides. The remaining stay suture is removed after extubation once the airway is felt to be stable. Postoperatively, the patient is fed with a soft diet for 3 weeks and no nipples or pacifiers are used.
Complications
Complications from the procedure include bleeding, neurovascular injury, wound breakdown, and prolonged lingual edema. Repeat tongue reduction is not uncommon and may be the result of a conservative reduction. Long-term speech difficulties and articulation errors can result from an excessive anterior resection. Similarly, lingual tip mobility may be reduced owing to scarring.
Discussion
Surgical treatment of macroglossia is indicated based on the patient׳s symptoms. Symptoms of macroglossia are typically due to mass effect and include airway compromise, difficulties in swallowing, chronic lingual exposure, malocclusion, and oral incompetency. Through the years, a variety of operative techniques have been described in the literature. The keyhole reduction technique combines an anterior wedge resection with central debulking. This procedure allows for a great deal of versatility for increased tongue reduction as noted by Morgan et al.
For greater reductions in the width of the tongue, the anterior wedge portion can be widened, whereas increasing the diameter of the central circular incision provides for further reduction in tongue bulk and length. This technique also avoids total deepithelialization of the tongue, so as not to disrupt the functionality of the taste buds.
The keyhole technique allows for improved tip mobility by preserving the lateral neurovascular bundles and by using an anterior wedge resection as opposed to a tip amputation.
By preserving mobility and function, the patient׳s speech can be improved. A study performed in 2010 looked at patients 9 years after undergoing surgical tongue reduction using the keyhole technique.
Long-term impact of tongue reduction on speech intelligibility, articulation and oromyofunctional behaviour in a child with Beckwith-Wiedemann syndrome.
They investigated the procedure׳s effect on speech development and oral behavior. They found that speech intelligibility went from severely impaired before the surgery to normal at 9 years after the operation. Regarding oral behavior, symptoms such as drooling and impaired chewing function disappeared by 9 years after the procedure. Overall, the keyhole technique provides a versatile method for correction of macroglossia that preserves the important structures of the tongue and has long-term benefit on patients׳ symptoms.
References
Vogel J.E.
Mulliken J.B.
Kaban L.B.
Macroglossia: A review of the condition and a new classification.
Long-term impact of tongue reduction on speech intelligibility, articulation and oromyofunctional behaviour in a child with Beckwith-Wiedemann syndrome.
00070-6&id=gr1.jpg){kind=link}
00070-6&id=gr2.jpg){kind=link}
00070-6&id=gr3.jpg){kind=link}
00070-6&id=gr4.jpg){kind=link}
00070-6&id=gr5.jpg){kind=link}