medigraphic.com
SPANISH

Mediciego

ISSN 1029-3035 (Electronic)
  • Contents
  • View Archive
  • Information
    • General Information        
    • Directory
  • Publish
    • Instructions for authors        
  • medigraphic.com
    • Home
    • Journals index            
    • Register / Login
  • Mi perfil

2016, Number 4

<< Back Next >>

Mediciego 2016; 22 (4)

Anesthetic management during orchiopexy in Prader-Willi syndrome. Case report

López EN, Ibarra LRE, Ortiz MN, Borroto AL, Santalla PMC
Full text How to cite this article

Language: Spanish
References: 12
Page: 54-59
PDF size: 118.37 Kb.


Key words:

Prader-Willi syndrome, endotracheal anesthesia, anesthesia adjuvants, delayed emergence from anesthesia, orchiopexy, case reports.

ABSTRACT

Introduction: Prader-Willi syndrome is a non-hereditary genetic disease, associated with disorders of the chromosome 15 of paternal origin. Although the diagnosis is based on suspicion of characteristic symptoms, it is only confirmed by genetic testing. The anesthetic peculiarities of these patients are derived from their characteristics at the time of the intervention; however, special attention should be paid to several aspects, including difficulties in the doctor-patient relationship, the possibility of a patient having a full stomach, difficult venous access, difficulty in managing the airway, increased anesthetics sensitivity and delayed awakening.
Case presentation: a nine year old male patient, white skin, 64 Kg of weight and 1,50 m of height, diagnosed with Prader-Willi syndrome. The examinations of the respiratory and cardiovascular systems did not provide positive data; the results of the supplementary examinations were within normal limits. On physical examination, characteristic features were found: large head and short neck with good flexion-extension, small mouth and large tongue, moderate mental retardation and bilateral cryptorchidism, reason for which surgical treatment was indicated by bilateral orchidopexy with the use of general orotracheal anesthesia. The patient had no anesthetic complications; he was hospitalized for 48 hours with a satisfactory evolution.
Conclusions: in the anesthetic management of patients with Prader-Willi syndrome, the associated comorbidities must be taken into account; Proper postoperative surveillance is necessary to prevent the occurrence of respiratory complications.


REFERENCES

  1. Fúnes R, Rivas V, Donaire MG, Henríquez A, Mejía JA. Síndrome de Prader Willi. Rev. Fac. Cienc. Méd [Internet]. Dic 2008 [citado 15 Ene 2016];5(2):37-45. Disponible en: http://65.182.2.242/RFCM/pdf/2008/pdf/RFCMVol5-2-2008-7.pdf

  2. Holm VA, Cassidy SB, Butler MG, Hanchett JM, Greenswag LR, Whitman BY et al. Prader-Willi syndrome: consensus diagnostic criterial. Pediatrics [Internet]. Feb 1993 [citado 15 Ene 2016];91(2):398-402. Disponible en: http://pediatrics.aappublications.org/content/pediatrics/91/2/398.full.pdf

  3. Angulo MA. Síndrome de Prader-Willi - Guía 2005 para familiares y profesionales [Internet]. New York: International Prader- Willi Syndrome Organization; 2005 [citado 15 Ene 2016]. Disponible en: http://pwsa-usa.org/spanish/SpanishGuideAngulo2005.pdf

  4. Huerta Rivas C, Barabash Bustelo A, Gallego Merlo J, Ramos Corrales C, Osorio Cabrero A, Robledo Batanero M, et al. Diagnóstico rápido del síndrome de Prader-Willi y de Angelman mediante test de metilación por PCR. An Esp Pediatr [Internet]. Jun 1998 [citado 15 Ene 2016];48(6):583-6. Disponible en: https://www.aeped.es/sites/default/files/anales/48-6- 3.pdf

  5. Camprubí Sánchez C, Gabau Vila E, Artigas Pallarés J, Coll Sandiumenge MD, Guitart Feliubadaló M. Del diagnóstico clínico al diagnóstico genético de los síndromes de Prader-Willi y Angelman. Rev Neurol [Internet]. Ene 2006 [citado 15 Ene 2016];42(1 Supl 1):S61-7. Disponible en: http://www.psyncron.com/wp-content/uploads/2011/05/angelman3.pdf

  6. Roberts J, Cataletto M, Quintos-Alagheband ML, Coste F, Angulo MA. Special anesthesia concerns for patients with Prader-Willi syndrome: The Winthrop University Hospital Center experience. The Gathered View [Internet]. May 2008 [citado 14 Ene 2016];32:[aprox. 2 p.]. Disponible en: http://pwsausa.org/wp-content/uploads/2015/11/Anesthesia-Concerns-for- Patients-abstract-w-logo.pdf

  7. Locker J, Rosenfield L. Issues affecting Prader-Willi syndrome and anesthesia. Anesthesia and Prader-Willi syndrome. The Gathered View [Internet]. Jun 2005 [citado 14 Ene 2016];1:[aprox 4 p.]. Disponible en: http://pwsaofwi.org/wpcontent/ uploads/2013/03/Anesthesia-and-PWS-MA-01.pdf

  8. Rodríguez H, Cuestas G, Leske V. Manifestaciones otorrinolaringológicas del síndrome de Prader-Willi. Revista FASO [Internet]. 2015 [citado 14 Ene 2016];2:11-3. Disponible en: http://www.faso.org.ar/revistas/2015/2/2.pdf

  9. Rodríguez Vargas N, Martínez Pérez T, Martínez García R, Calvo Luaces V, Guerrero Guerrero L. Síndrome de Prader-Willi. Presentación clínica de dos pacientes y revisión de la literatura. Rev Cubana Pediatr [Internet]. Mar 2006 [citado 21 Ene 2016];78(1):[aprox. 8 p.]. Disponible en: http://www.bvs.sld.cu/revistas/ped/vol78_1_06/ped11106.pdf

  10. Meco BC, Alanoglu Z, Cengiz OS Alkis N. Anesthesia for a 16-month-old patient with Prader– Willi Síndrome. J Anesth [Internet]. Dic 2010 [citado 27 Ene 2016];24:949–50. Disponible en: http://download.springer.com/static/pdf/34/art%253A10.1007%252Fs00540-010-1005- 3.pdf?originUrl=http%3A%2F%2Flink.springer.com%2Farticle%2F10.1007%2Fs00540-010- 1005- 3&token2=exp=1453916183~acl=%2Fstatic%2Fpdf%2F34%2Fart%25253A10.1007%25252 Fs00540-010-1005- 3.pdf%3ForiginUrl%3Dhttp%253A%252F%252Flink.springer.com%252Farticle%252F10.1007%252Fs00540-010-1005- 3*~hmac=c4f70cf23bddd2809f00c267c95da87c7a73223e12c85358d039462765ce4d96

  11. Biro P. Anaesthesia in a patient with Prader-Willi syndrome and severe burn injury: a case report. J Rom Anest Terap Int [Internet]. 2011 [citado 27 Ene 2016];18:149-52. Disponible en: http://www.jurnalul-anestezie.ro/2011/2/11.pdf

  12. Grass Pedrals J, Weissmann Marcuso K, Herrera Gallegos V. Escoliosis y Síndrome de Prader- Willi: a propósito de 5 casos intervenidos quirúrgicamente. Coluna/Columna [Internet]. Jun 2012 [citado 21 Ene 2016];11(2):127-30. Disponible en: http://www.scielo.br/pdf/coluna/v11n2/07.pdf




2020     |     www.medigraphic.com

Mi perfil

C?MO CITAR (Vancouver)

Mediciego. 2016;22