medigraphic.com
ISSN 1726-6718 (Electronic)
Revista Cubana de Anestesiología y Reanimación

2019, Number 2

<< Back

Revista Cubana de Anestesiología y Reanimación 2019; 18 (2)

Beckwith-Wiedemann syndrome

Aguilar RN, Fernández MCA, de la Paz EC, Rabí LAE

Language: Spanish
References: 22
Page: 1-10
PDF size: 196.37 Kb.


ABSTRACT

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.


REFERENCES

  1. Cordero EI. La vía aérea y su abordaje. En: Dávila CE, Gómez BC, Álvarez BM, Sainz CH, Molina LR. Anestesiología Clínica. España; 2001. p. 113-8.

  2. Cobley M, Vaughan RS. Recognition of management of the difficult airway problems. Br J Anesth. 1992;(4)68:90-8.

  3. Zamido VI. Vía aérea difícil en pediatría. Rev Chil Anest. 2002;31(1):45-56.

  4. Valera EA. Vía aérea en pediatría. Rev Ven Anest. 2002;7(2):202-7.

  5. Gloria MC. Manejo Adicional en Síndrome de Pierre Robin. Rev Chil Pediatr 2004;75(1):36-42

  6. Beckwith JB. Extreme citomegaly of the adrenal fetal cortex,"ophalocoele, hiperplasia of kidneys and pancreas and Leydig"cell hyperplasia. Another syndrome. Presented at The Annual" Meeting of the Western Society for Pediatric Research, Los" Angeles California, November 11, 1963.

  7. Wiedemann HR. Complexe malformatif familial avec hernie"ombilicale et maraglossieun “syndrome malformatif"nouveau”? J Genet Hum. 1964;13(3):223-32.

  8. Weng EY, Moeschler JB, Graham JM Jr. Longitudinal observations on 15 children with Wiedemann-Beckwith syndrome. Am J Med Genet. 1995;56(2):366-73.

  9. Begemann M, Spengler S, Gogiel M, Grasshoff U, Bonin M, Betz RC, et al. Clinical significance of copy number variations in the 11p15.5 imprinting control regions: new cases and review of the literature. J Med Genet. 2012;49(9):547-53.

  10. Weksberg R, Shuman C, Beckwith JB. Beckwith-Wiedemann syndrome. Eur J Hum Genet. 2010;18(1):8-14.

  11. Pettenati MJ, Haines JL, Higgins RR, Wappner RS, Palmer CG, Weaver DD. Wiedemann-Beckwith syndrome: presentation of"clinical and cytogenetic data on 22 new cases and review of the"literature. Hum Genet. 1986;74(6):143-54."

  12. Suan C, Ojeda R, García Perla JL, Pérez Torres MC. Anaesthesia and the Beckwith- Wiedemann syndrome. Paediatr Anaesth. 1996;6(3):231-33.

  13. Kim Y, Shibutani T, Hirota Y, Mahbub SF, Matsuura H. Anesthetic considerations of two sisters with Beckwith-Wiedemann syndrome. Anesth Prog. 1996;43(7):24-28."

  14. Nelson. Tratado de Pediatría. 15 ed. Madrid: Mc Graw-Hill, Interamericana; 1997. pp. 532 y 642.

  15. McManammys DS. Macroglosis as presentation of the Beckwith- Wiedemann Syndrome. Plast Reconstr Surg. 1985;75(2):67-89.

  16. Soltelo Ávila, C. Complete and incomplete forms of Beckwith-Wiedemann Syndrome: their oncogenic potencial. J Pediatr. 1986; 96:47.

  17. Patterson GT. Macroglosis and ankyloglosis, in Beckwith-Wiedemann Syndrome. Oral Surg Oral Med Oral Pathol. 1988;65(1):123-34.

  18. Viljoen D, Pearn J, Beighton P. Manifestations and natural" history of idiopathic hemihypertrophy: a review of eleven"cases. Clin Genet. 1984;26(4):81-6."

  19. Tubbs RS, Oakes WJ. Beckwith-Wiedemann syndrome in a" child with Chiari I malformation. Case report. J Neurosurg. 2005;103(3):172-74."

  20. Choyke PL, Siegel MJ, Oz O, Sotelo-Avila C, DeBaun MR. Nonmalignant renal disease in pediatric patients with BeckwithWiedemann syndrome. AJR Am J Roentgenol. 1998;171(7):733-37.

  21. Borer JG, Kaefer M, Barnewolt CE, Elias ER, Hobbs N, Retik AB, with Beckwith- Wiedemann syndrome. J Urol. 1999;161(5):235-39."

  22. Goldman M, Shuman C, Weksberg R, Rosenblum N. Hypercalciuria in Beckwith- Wiedemann syndrome. J of Pediatrics. 2003;142(2):206-08.




2020     |     www.medigraphic.com