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Hipertrofia pilorica congenita

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Hipertrofia Pilorica Congenita

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Hipertrofia pilorica congenita

  1. 1. Definicion Es la disminución de la luz intestinal a nivel del píloro debido a hipertrofia e hiperplasia de la capa muscular de la porción antro-pilórica del estómago y se manifiesta clínicamente como obstrucción al vaciamiento gástrico (Roldán VE, 2007) Guia de Practica Clinica Cenetec 2012 Nelson Textbook of Pediatrics. 20th Edition. Chapter 321. Page 1275 - 1276. 2
  2. 2. Epidemiologia • 1 – 3 casos por 1,000 lactantes (EUA) • > Raza blanca • 4 – 6:1 varones primogénitos • Mayor incidencia en lactantes con padre o madre que presentaron estenosis pilórica. • Grupo sanguíneo B y O • Gemelos monocigoticos Nelson Textbook of Pediatrics. 20th Edition. Chapter 321. Page 1275 - 1276. 3
  3. 3. Patologias Concomitantes Defectos congénitos: • Fistula traqueo esofágica • hipoplasia del frenillo labial inferior Nelson Textbook of Pediatrics. 20th Edition. Chapter 321. Page 1275 - 1276. 4
  4. 4. Etiología • Causa desconocida • Multifactorial • Se desarrolla despues del nacimiento • Relación: • Gastroenteritis Eosinofilica • Síndrome de Apert • Síndrome de Zellweger • Trisomía 18 • Síndrome de Smith-Lemli-Opitz • Síndrome de Cornelia de Lange • Eritromicina en primeras 2 Semanas • Macrolidos Nelson Textbook of Pediatrics. 20th Edition. Chapter 321. Page 1275 - 1276. 5
  5. 5. Otras Asociaciones Inervación muscular anómala Concentraciones elevadas de prostaglandinas Hipergastrinemia Poca concentración de oxido nítrico sintasa neuronal (nNOS) Poca expresión del gen nNOS Poca concentración de NO Nelson Textbook of Pediatrics. 20th Edition. Chapter 321. Page 1275 - 1276. 6
  6. 6. Nelson Textbook of Pediatrics. 20th Edition. Chapter 321. Page 1275 - 1276. 7
  7. 7. Manifestaciones Clínicas Vomito no bilioso Proyectado o no proyectado Progresivo Posterior a la ingesta Inicia posterior a la 1ra - 3ra semana de vida  5to mes Nelson Textbook of Pediatrics. 20th Edition. Chapter 321. Page 1275 - 1276. 8
  8. 8. Manifestaciones Clínicas Hambre tras el vomito Deshidratación  Alcalosis metabólica hipocloremica Desnutrición Nelson Textbook of Pediatrics. 20th Edition. Chapter 321. Page 1275 - 1276. 9
  9. 9. Manifestaciones Clínicas Hiperbilirrubinemia «Síndrome Ictero-Pilorico» No Conjugada (alteracion enzimatica) Conjugada (descartar otros diagnosticos) Nelson Textbook of Pediatrics. 20th Edition. Chapter 321. Page 1275 - 1276. 10
  10. 10. Diagnostico Palpación de la masa pilórica Firme, movible, longitud de 2 cm, en forma de aceituna, dura, se localiza en la zona mesoepigastrica bajo el reborde hepático. Signo de la Oliva Se observan las ondas peristálticas progresivas. Nelson Textbook of Pediatrics. 20th Edition. Chapter 321. Page 1275 - 1276. 11
  11. 11. Signo de la Oliva Pilórica Nelson Textbook of Pediatrics. 20th Edition. Chapter 321. Page 1275 - 1276. 12
  12. 12. Nelson Textbook of Pediatrics. 20th Edition. Chapter 321. Page 1275 - 1276. 13
  13. 13. Estudios de Imagen Ecografía Abdominal Simple • Estudio confirmatorio • Criterios ecográficos • Sensibilidad de 95% Nelson Textbook of Pediatrics. 20th Edition. Chapter 321. Page 1275 - 1276. 14
  14. 14. Criterios Ecográficos para HPC 1. Grosor pilórico 3 – 4 mm 2. Longitud global pilórica de 15 – 19 mm 3. Diámetro pilórico de 10 – 14 mm Nelson Textbook of Pediatrics. 20th Edition. Chapter 321. Page 1275 - 1276. 15
  15. 15. Nelson Textbook of Pediatrics. 20th Edition. Chapter 321. Page 1275 - 1276. 16
  16. 16. Estudios de Imagen Ecografía contrastada • Demuestra la existencia del conducto pilórico alongado «Signo del cordón» • Protuberancia del musculo pilórico hacia el antro «Signo del hombro» • Presencia de estrías paralelas de bario en el conducto «Signo del Doble Riel» Nelson Textbook of Pediatrics. 20th Edition. Chapter 321. Page 1275 - 1276. 17
  17. 17. Diagnósticos Diferenciales Reflujo gastroesofagico Insuficiencia suprarrenal Alteraciones metabolicas congenitas Gastroenteritis Membrana pilorica Duplicacion pilorica Estenosis duodenal Nelson Textbook of Pediatrics. 20th Edition. Chapter 321. Page 1275 - 1276. 18
  18. 18. Tratamiento Preoperatorio: • Estabilización metabólica acido básica y electrolítica. Piloromiotomia de Ramstedt o Laparoscopica Alimentación naso-duodenal en pacientes Sulfato de atropina VO e IV Nelson Textbook of Pediatrics. 20th Edition. Chapter 321. Page 1275 - 1276. 19
  19. 19. Piloromiotomia de Ramstedt 1. Incisión transversa en la piel 2. Seccionar longitudinalmente la masa pilórica 3. Evitar cortar la mucosa 4. Cierre Nelson Textbook of Pediatrics. 20th Edition. Chapter 321. Page 1275 - 1276. 20
  20. 20. Pronostico Procedimiento quirúrgico curativo Mortalidad operatoria de 0 – 0.5% Dilatación endoscópica con balón en pacientes con piloromiotomia incompleta. Nelson Textbook of Pediatrics. 20th Edition. Chapter 321. Page 1275 - 1276. 21
  21. 21. Bibliografia Nelson Textbook of Pediatrics. 20th Edition. Chapter 321. Page 1275 - 1276. Guias de Practica Clinica CENETEC Nelson Textbook of Pediatrics. 20th Edition. Chapter 321. Page 1275 - 1276. 22
  22. 22. Jade Díaz G. Gracias por su Atencion Nelson Textbook of Pediatrics. 20th Edition. Chapter 321. Page 1275 - 1276. 23

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