OBJECTIVE Wiedemann-Beckwith syndrome is a multisystemic pattern of congenital anomalies with overgrowth. The most characteristic clinical features are macroglossia, high birth weight, omphalocele, visceromegaly and hypoglycemia. PATIENTS AND METHODS We show the clinical and epidemiological characteristics of the 18 cases with Wiedemann-Beckwith syndrome identified in the consecutive series o...

Macroglossia is a morphological and volumetric alteration of the tongue, caused by muscular hypertrophy, vascular malformation, metabolic diseases, and idiopathic causes and also associated with Down and Beckwith-Wiedemann syndromes. This alteration can cause dental-muscle-skeletal deformities, orthodontic instability, masticatory problems, and alterations in the taste and speech. In this paper...

TOPIC: Critical Care TYPE: Medical Student/Resident Case Reports INTRODUCTION: Acquired macroglossia is uncommon but potentially life-threatening due to airway compromise. It might complicate intubation, extubation or both. Different etiologies are reported, among which Anti-Epileptic Agents (AED)1-4. Higher risk expected in Refractory Status Epilepticus patients multiple simultaneous AED use. ...

Upper airway obstruction is common in DS due to: midfacial hypoplasia, macroglossia, narrowing of the nasopharynx, tonsillar and adenoidal enlargement, laryngomalacia, tracheomalacia and congenital malformations of the larynx and the trachea. The incidence of OSA was reported to be 30-50%. Exacerbating factors including obesity and gastro-esophageal reflux may contribute to the occurrence of sl...

Beckwith-Wiedemann syndrome (BWS) is a rare congenital syndrome characterized by an overgrowth, macroglossia, exomphalos, and predisposition to embryonal tumors. Central nervous abnormalities associated with BWS are rare. We describe a one-day-old Congolese female who presented meningocele associated with BWS phenotype.

RESUMO O objetivo deste estudo foi relatar a abordagem interdisciplinar no manejo da macroglossia em um caso de paciente com síndrome Beckwith-Wiedemann, período dez anos. acompanhamento iniciou pela equipe Cirurgia Bucomaxilofacial, seguido Fonoaudiologia, função dificuldades alimentares. Após avaliação clínica e instrumental, aos 8 meses idade, iniciou-se intervenção fonoaudiológica foco na d...