Abstract
BackgroundMacroglossia causes functional deficits such as airway obstruction, drooling, phonation difficulties, and leads to protrusion of dentoalveolar structures resulting in an anterior open bite and a prognathic mandibular appearance. Macroglossia is present in the majority of patients with Beckwith-Wiedemann syndrome (BWS) and surgical treatment may be indicated.Material and MethodsA retrospective review was conducted including BWS patients who underwent surgical tongue reduction between 2000 and 2015 at the Hospital Universitario La Paz, Madrid.ResultsOut of 16 patients with BWS, surgery was performed in 11 cases. Tongue protrusion with open bite was the main indication for surgical treatment. Reduction glossectomy was performed using the keyhole technique. We analysed the relationship between age at surgery and evolution of open bite. Complications were minimal and satisfactory outcomes were observed with a decrease in anterior open bite.ConclusionsIn this study we have observed that surgical treatment in patients with BWS and open bite accompanied by macroglossia seems to provide positive results with a satisfactory outcome in dentoskeletal alterations. Key words:Macroglossia, Beckwith- Wiedemann syndrome, glossectomy; tongue reduction, malocclusion.
Highlights
Beckwith-Wiedemann Syndrome (BWS) is a congenital overgrowth disorder with an estimated incidence of approximately 1 in 13,700 to 17,000 births [1,2]
2 of these patients are awaiting intervention; 3 cases of mild macroglossia did not require surgery and 11 patients underwent tongue reduction for various different conditions, all of which had an open bite at the time of surgery
The main indications for tongue surgery were open bite in 65% of patients (13 cases), airway obstruction in 15% (3 cases), oral incompetence with persistent drooling in 10% (2 cases), feeding problems in 5% (1 case) and phonation difficulties in 5% (1 case) (Table 1)
Summary
Beckwith-Wiedemann Syndrome (BWS) is a congenital overgrowth disorder with an estimated incidence of approximately 1 in 13,700 to 17,000 births [1,2]. Our objective is to analyse the anterior open bite evolution in these patients after surgery. Macroglossia causes functional deficits such as airway obstruction, drooling, phonation difficulties, and leads to protrusion of dentoalveolar structures resulting in an anterior open bite and a prognathic mandibular appearance. Macroglossia is present in the majority of patients with Beckwith-Wiedemann syndrome (BWS) and surgical treatment may be indicated. Results: Out of 16 patients with BWS, surgery was performed in 11 cases. Conclusions: In this study we have observed that surgical treatment in patients with BWS and open bite accompanied by macroglossia seems to provide positive results with a satisfactory outcome in dentoskeletal alterations
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