Referral for Pediatric Macroglossia
A child with macroglossia who is protruding their tongue should be referred to a pediatric otolaryngologist as the primary specialist, with pediatric plastic surgeon as an appropriate alternative option.
Primary Referral Pathway
The American Academy of Pediatrics explicitly recommends that infants and children with congenital malformations of head and neck structures, including oral cavity abnormalities, should be referred to a pediatric otolaryngologist. 1 The guidelines specifically state that a pediatric plastic surgeon, pediatric surgeon, pediatric dentist, or pediatric oromaxillofacial surgeon with appropriate training would also be appropriate in some cases. 1
Why These Specialists?
Pediatric Otolaryngologist
- Has completed 4-5 years of residency in otolaryngology/head and neck surgery plus 1-2 years of fellowship training in pediatric otolaryngology. 1
- Manages congenital malformations of the oral cavity and laryngotracheal airway, which is critical since macroglossia can cause airway obstruction. 1
- Can evaluate for potential airway compromise, which is a life-threatening complication of macroglossia. 2
Pediatric Plastic Surgeon (Alternative)
- Has completed 6+ years of surgical training plus additional year in pediatric plastic/craniofacial surgery. 1
- Performs tongue reduction procedures (partial glossectomy) for symptomatic macroglossia. 2, 3
- Manages craniofacial anomalies and congenital malformations that may be associated with macroglossia. 4, 5
Critical Clinical Considerations
Assess for Underlying Syndromes
- Macroglossia is frequently associated with Beckwith-Wiedemann syndrome, Down syndrome, mucopolysaccharidoses, and Pompe disease. 2, 6
- Children with Down syndrome have relative macroglossia due to hypotonia, midfacial hypoplasia, and a shortened palate. 1
- Mucopolysaccharidoses cause upper airway narrowing from hypertrophy of tongue, tonsils, and adenoids. 1
Evaluate for Airway Compromise
- Macroglossia can cause upper airway obstruction, feeding difficulties, and sleep-disordered breathing. 2, 6
- Children with craniofacial anomalies and macroglossia are at high risk for sleep-disordered breathing and may require preoperative polysomnography. 1
- Airway obstruction is a potentially life-threatening complication requiring urgent specialist evaluation. 2, 6
Document Associated Symptoms
- Look for feeding and swallowing difficulties, sialorrhea (drooling), speech problems, and recurrent upper airway infections. 2, 6
- Note presence of tongue fissures, ulcers, or signs of trauma from chronic protrusion. 7, 6
- Assess for dental malocclusion, anterior open bite, and proclination of incisors. 3
Common Pitfalls to Avoid
- Do not delay referral if there are any signs of airway compromise, as this can be life-threatening. 2, 6
- Do not assume all tongue protrusion is benign – acute macroglossia can occur from trauma, inflammation, or vascular malformations requiring urgent intervention. 8, 7
- Do not refer to general ENT or plastic surgeon when pediatric specialists are available, as children with medical conditions that increase operative risk should be managed by pediatric surgical specialists. 1
- Do not overlook the need for multidisciplinary care – these children may require coordination between pediatric otolaryngology, plastic surgery, genetics, and orthodontics. 3, 6
When Surgical Intervention May Be Needed
- Tongue reduction surgery (partial glossectomy) is indicated for symptomatic macroglossia causing airway obstruction, feeding difficulties, speech problems, or significant cosmetic concerns. 2, 3
- Surgery is typically performed between 4 months to 10 years of age, with good outcomes in airway, feeding, speech, and psychosocial wellbeing. 2, 3
- Early surgical intervention combined with orthodontic treatment can prevent development of malocclusions. 3