Abstract

ROA ROJAS, Pablo; ARANGO FERNANDEZ, Hernán; REBOLLEDO COBOS, Martha  and  HARRIS RICARDO, Jonathan. Surgical treatment of macroglossia in Beckwith-Wiedemann syndrome: case report. Arch. argent. pediatr. [online]. 2018, vol.116, n.2, pp.e341-e345. ISSN 0325-0075.  http://dx.doi.org/10.5546/aap.2018.e341.

Beckwith-Wiedemann syndrome is a rare congenital condition, characterized by presenting macroglossia, defects of the abdominal wall, hemihypertrophy, omphalocele, neonatal hypoglycemia, umbilical hernia, hepatomegaly, cardiac abnormalities, among others. Macroglossia occurs in 90% of cases, causing a problem in chewing, swallowing, phonation and breathing, resulting in a closure of the upper airway. The therapeutic option of choice is partial glossectomy. We present a 2-month-old pediatric patient with Beckwith-Wiedemann syndrome and area blockage due to severe macroglossia; in the medical history, congenital heart disease, interatrial communication, persistent ductus arteriosus, symptomatic epilepsy, renal failure, hypoglycemia, tracheotomy and gastrostomy, due to airway collapse and dysphagia. It was performed an anterior tongue reduction surgery as a surgical treatment with favorable results.

Keywords : Macroglossia; Tongue; Glossectomy; Beckwith-Wiedemann syndrome.