Abstract

AGUILAR RAMIREZ, Niurys; FERNANDEZ MARRERO, Carlos Alberto; DE LA PAZ ESTRADA, Carlos  and  RABI LANG, Ana Elena. Beckwith-Wiedemann syndrome. Rev cuba anestesiol reanim [online]. 2019, vol.18, n.2  Epub June 01, 2019. ISSN 1726-6718.

Beckwith-Wiedemann syndrome is characterized by omphalocele, macroglossia, visceromegaly and neonatal hypoglycaemia, as well as a great diversity of clinical and laboratory abnormalities. This disease is also known as omphalocele, macroglossia and gigantism syndrome. The most significant problems related to anesthesia are hypoglycemia and macroglossia. It is imperative to perform a pre-anesthetic evaluation that includes the cardiovascular system, the urinary system, as well as the airway. Children with this syndrome may require different surgical procedures. A difficult approach to the airway should be predicted due to the growth of the tongue which can cause difficulty during ventilation and/or endotracheal intubation. Perioperative glycemia should be monitored in order to avoid neurological sequelae secondary to undiagnosed hypoglycemia. We report the perianesthesiological treatment of a four-year-old boy with Beckwith-Wiedemann syndrome who required surgical treatment for Wilms' tumor. After a thorough evaluation, orotracheal intubation was performed with a 5.0 tube, which was easily introduced with ketamine-vecuronium induction. Anesthesia was maintained without incident with isoflurane and fentanyl.

Keywords : Beckwith-Wiedemann syndrome; anesthetic approach; Wilms' tumor.

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