Beckwith–Weidemann syndrome: partial glossectomy

Introduction: The Beckwith–Wiedemann Syndrome (BWS) is a condition that occurs with an overgrowth [1,2]. The most common complications: macroglossia, wall defects abdominal, hypoglycemia, nephromegaly, facial nevus flammeus [3]. BWS frequency is 1 per 13,700 births [4]. It has been observed alterations in gene segment 11p15.5 [5].

The macroglossia, can cause obstruction of the airway of the infant, dentoalveolar structures deformity, anterior open bite, maxillary hypoplasia and relative prognathism.

Objective: We present a case of the female patient, that was tried in conjunction with the plastic surgery service, which was valued macroglossia and partial glossectomy was performed technical Digman Grabb [6].

Case report: Female patient, 9 months old, came to hospital, with fever, productive cough and vomit. The first five days of age is admitted to neonatal intensive care for hypoglycemia and aspiration pneumonia, where diagnostic Beckwith–Weidemann syndrome. Subsequently present two events of aspiration pneumonia. Consultation to be plastic surgery and maxillofacial surgery. At physical examination with open mouth, large tongue and protruding. In lateral cephalogram and facial CT is observed diminished upper airway, tongue tissue abundant. Given the diagnosis of macroglossia and scheduled the patient for partial glossectomy.

Patients and method: Partial glossectomy was performed Digman Grabb technique under general anesthesia and nasotracheal intubation using fibroscopy made, without complications.

Conclusions management of BWS patients should be multidisciplinary. Partial glossectomy is not indicated in patients under 11 years. We decided to make it in this patient for ventilatory problems and recurrent aspiration pneumonia events.

Conflict of interest: None declared.

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Feb 5, 2018 | Posted by in Oral and Maxillofacial Surgery | Comments Off on Beckwith–Weidemann syndrome: partial glossectomy
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