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2017, Number 2

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Rev Mex Anest 2017; 40 (2)

Syndrome of Prader Willi. Presentation of a case

Sánchez-Hernández E, Álvarez-Betancourt AE, Soto-Blanquel JL, Echeto-Cerrato MA

Language: Spanish
References: 15
Page: 120-124
PDF size: 395.58 Kb.


ABSTRACT

Prader Willi syndrome is a rare genetic disorder; it affects 1:10,000-30,000 births. The symptoms can range from poor muscle tone during infancy to behavioral problems in early childhood. It results in hypothalamic disfunction, thermal disregulation, high pain threshold, hipersomnia, endocrine abnormalities, hypogonadism and adrenal insufficency. More aspects seen in a clinical overview include hypotonia and abnormal neurologic function, developmental and cognitive delays, short stature, behavioral and psychiatric disturbances, hyperphagia and obesity that lead to respiratory problems such as sleep apnea. Due to a variety of physical malformations they usually require surgical treatment and therefore their anesthetic management becomes a challenge. We describe a case of a thirteen month old patient with Prader Willi syndrome weighting 12.3 kg and a body mass index of 21.86 diagnosed with bilateral cryptorchidism scheduled for laparascopic orquidopexy. A combined technique (general anesthesia using laryngeal airway air-Q® and caudal block) with a continuous perfusion of short half life anesthetic (remifentanil) was used for anesthetic management. Surgery and anesthesia presented no complications; at the PACU the patient had mild stridor that ceased with nebulized steroids. Had no pain at his discharge.


REFERENCES

  1. Angulo MA, Butler MG, Cataletto ME. Prader-Willi syndrome: a review of clinical, genetic, and endocrine findings. J Endocrinol Invest. 2015;38:1249-1263.

  2. Siemensma EP, de Lind van Wijngaarden RF, Otten BJ, de Jong FH, Hokken-Koelega AC. Testicular failure in boys with Prader-Willi syndrome: longitudinal studies of reproductive hormones. J Clin Endocrinol Metab. 2012;97:E452-E459.

  3. Meco BC, Alanoglu Z, Cengiz OS, Alkis N. Anesthesia for a 16-month-old patient with Prader-Willi syndrome. J Anesth. 2010;24:949-950.

  4. Loker J, Rosenfield L. Anesthesia and Prader Willi syndrome, issues affecting Prader Willi syndrome and anesthesia. The Gathered View, Prader Willi Syndrome Association 2005. Disponible en: http://www.pwsausa.org

  5. Dearlove OR, Dobson A, Super M. Anaesthesia and Prader-Willi syndrome. Paediatr Anaesth. 1998;8:267-271.

  6. Orphananestethesia. Anesthesia recommendations for patients suffering from Prader-Willi Syndrome. [Citado en mayo de 2012] Disponible en: http://www.orpha.net

  7. Goyal R. Small is the new big: an overview of newer supraglottic airways for children. J Anaesthesiol Clin Pharmacol. 2015;31:440-449.

  8. Dearlove OR, Dobson A, Super M. Anaesthesia and Prader-Willi syndrome. Paediatr Anaesth. 1998;8:267-271.

  9. Nixon GM, Brouillette RT. Sleep and breathing in Prader-Willi syndrome. Pediatr Pulmonol. 2002;34:209-217.

  10. Legrand R, Tobias JD. Anesthesia and Prader-Willi syndrome: preliminary experience with regional anesthesia. Paediatr Anaesth. 2006;16:712-722.

  11. Lam H, Landsman IS. Are children with Prader Willi syndrome at higher risk for anesthetic complications? Paediatr Anaesth. 2014;24:457-459.

  12. Sánchez-Rodríguez JP, Guzmán-Nuques R, Rionda-Díaz E, Quintero-García MA, Jiménez-Casillas AV. Síndrome Ehlers-Danlos y anestesia. Acta Med. 2009;7:50-53.

  13. Bellas S, Marín F, Sepúlveda A. Síndrome de DiGeorge. Tratamiento anestésico. Rev Mex Anest. 2011;34:309-312.

  14. Coté CJ, Lerman J, Todres D. A practice of anesthesia for infants and children. Chapter 55. Philadelphia, PA: Elsevier; 2009. p. 137.

  15. Mortensen A, Lenz K, Abildstrøm H, Lauritsen TL. Anesthetizing the obese child. Paediatr Anaesth. 2011;21:623-629.




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