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Sindrome prune belly por Fabricio Polo

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Sindrome prune belly por Fabricio Polo

  1. 1. Sindrome de abdomen en ciruela pasa. A propósito de un caso<br />Hospital General de las FF.AA No. 1 Servicio de Cirugía Pediátrica<br />Congreso Nal. de Urología<br />Octubre 2007<br />
  2. 2. Sindrome de abdomen en ciruela pasa<br />Objetivos<br />Describir a propósito de un caso los aspectos más importantes del SAC<br />Entender su patología<br />Discutir el manejo y pronóstico<br />
  3. 3. Sindrome de abdomen en ciruela pasa<br />Historia Clínica<br />Paciente neonato de 8 días de edad, sexo masculino, nacido en Ibarra, edad gestacional 41 semanas, hijo de madre de 37 años de edad, producto de cuarta gesta, parto cefalo-vaginal, con <br /> apgar 5 – 7 con distrés respiratorio moderado. <br />
  4. 4. Sindrome de abdomen en ciruela pasa<br />Examen físico<br />En la exploración física se le detectó peso 3850 gr., talla 52 cm., perímetro cefálico 35 cm., perímetro torácico 34 cm. , perímetro abdominal 38 cm., abdomen distendido con aspecto en “ciruela pasa”, criptorquidia bilateral. <br />
  5. 5. Sindrome de abdomen en ciruela pasa<br />Exámenes <br />Los exámenes de laboratorio demostraron urea 9 mg/dl, creatinina 0.4 mg/dl, electrolitos y biometría hemática normales.<br />Seguimiento de los niveles de creatinina se han mantenido por debajo de 0.7 mg/dL (2 por año)<br />
  6. 6. Sindrome de abdomen en ciruela pasa<br />Eco renal:<br />riñón izquierdo 60x34x41, derecho 44x33x31con moderada ectasia urétero-piélica bilateral, uréteres dilatados en todo su trayecto miden 14 mm.<br />10 días de edad. Julio 2001<br />
  7. 7. Sindrome de abdomen en ciruela pasa<br />Uretrocistograma retrógrado miccional<br />vejiga de moderada capacidad con un divertículo uracal prominente y alteración en la morfología de la uretra posterior secundario a valvas uretrales. <br />16 días de edad. Julio 2001<br />
  8. 8. Sindrome de abdomen en ciruela pasa<br />Evolución <br />Al 1 año 10 meses se realiza laparoscopia diagnostica, más liberación testicular retroperitoneal bilateral con fijación de testículos en anillo inguinal profundo.<br />A los 2 años 4 meses de edad se realiza orquidopexia bilateral (colocación de testículos en espacio subdartos y fijación a rafe escrotal).<br />Paciente ha presentado varios episodios de infecciones de vías urinarias, por lo que a los 3 años 4 meses se decide realizar talla vesical.<br />
  9. 9. Sindrome de abdomen en ciruela pasa<br />SAC<br />UROTAC. Preoperatoria<br />Edad: 4 años<br />
  10. 10. Sindrome de abdomen en ciruela pasa<br />Evolución <br />El seguimiento de la función se ha hecho con controles de ecografía y gammagrafía los cuales no han presentado gran variabilidad.<br />A los 4 años 8 meses se realiza remodelamiento de uréter izquierdo por gran dilatación, reimplante ureteral bilateral de los mismos por reflujo grado V, cistectomía parcial y recolocación de cistotomía.<br />
  11. 11. Sindrome de abdomen en ciruela pasa<br />UROTAC (postQx)<br />
  12. 12. Sindrome de abdomen en ciruela pasa<br />UROTAC (PostQx)<br />
  13. 13. Sindrome de abdomen en ciruela pasa<br />Gammagrafía<br />
  14. 14. Sindrome de abdomen en ciruela pasa<br />Gammagrafía<br />
  15. 15. Sindrome de abdomen en ciruela pasa<br />Gammagrafía<br />
  16. 16. Sindrome de abdomen en ciruela pasa<br />
  17. 17. Sindrome de abdomen en ciruela pasa<br />Gammagrafía<br />3 años 9 meses. 24 marzo 2005<br />
  18. 18. Sindrome de abdomen en ciruela pasa<br />Gammagrafía<br />
  19. 19. Sindrome de abdomen en ciruela pasa<br />4 años 6 m de edad. Enero 2006<br />
  20. 20. Sindrome de abdomen en ciruela pasa<br />Gammagrafía<br />
  21. 21. Sindrome de abdomen en ciruela pasa<br />
  22. 22. Sindrome de abdomen en ciruela pasa<br />Epidemiología<br />1 por cada 35 000 a 50 000 nacidos vivos<br />Más frecuente en varones<br />Las pruebas extensas no pudieron comprobar una base genética para el trastorno<br />Garlinger P, Ott J: Prune-belly syndrome—possible genetic implications. Birth Defects 1974 <br />
  23. 23. Sindrome de abdomen en ciruela pasa<br />Etiopatogenia<br />Obstrucción uretral. <br />Anormalidad primaria en la musculatura abdominal seguida de una sobre distensión de la vejiga y con cambios secundarios en el sistema urinario <br />Trastorno del desarrollo mesodérmico durante la embriogénesis temprana. <br />Moerman P, Fryns JP, Goddeeris P, Lauweryns JM: Pathogenesis of the prune-belly syndrome: A functional urethral obstruction caused by prostatic hypoplasia. Pediatrics 1984 <br />Wigger HJ, Blanc WA: The Prune Belly syndrome. Pathol Ann. 1977<br />
  24. 24. Sindrome de abdomen en ciruela pasa<br />Triada característica<br />Ausencia , deficiencia o hipoplasia congénita de la musculatura de la pared abdominal.<br />Anormalidades del tracto urinario <br />Criptorquidia bilateral<br />Eagle JF, Barrett GS: Congenital deficiency of abdominal musculature with associated genitourinary anomalies: A syndrome. Reports of 9 cases. Pediatrics 1950;6:721.<br />
  25. 25. Sindrome de abdomen en ciruela pasa<br />Clasificación<br />Grupo I: Las anormalidades urinarias son severas, existe un compromiso pulmonar importante.<br />Grupo II: el compromiso de las vías urinarias es moderado,. <br />Grado III: en este grupo las alteraciones son leves y no tienen comprometida la función renal.<br />
  26. 26. Sindrome de abdomen en ciruela pasa<br />Pared abdominal. (fisiopatología)<br />Los músculos afectados incluyen el transverso y el recto abdominales.<br />Los estudios de las zonas afectadas muestran músculo mal organizado que se intercala con tejido colágeno denso.<br />A nivel celular se reconoce desorganización ultraestructural inespecífica<br />Housden LG: Congenital abscense of the abdominal<br />Muscles. Arch Dis Child 9: 219-232, 1934<br />Minninberg DT, Mantoya F, Okada, K et al: Subcellular<br />Muscle studies in the Prune Belly Sindrome. J Urol 109, 1973<br />
  27. 27. Sindrome de abdomen en ciruela pasa<br />Pared abdominal (Relaciones clínicas)<br />Musculatura abdominal laxa<br />La gravedad del defecto abdominal no tiene importancia pronóstica<br />Problemas para la limpieza respiratoria (frecuentes infecciones)<br />Dificultades para la sedestación<br />Aschraft KW: Prune Belly syndrome. In Aschraft KE (ed): Pediatric Urology. Philadelphia, WB Saunders, 1990.<br />Bellah RD, States LJ, Duckett JW: Pseudoprune-belly syndrome: Imaging findings and clinical outcome. Am J Radiol 1996 <br />Karamanian A, Kravath R, Nagashima H, et al: Anesthetic management of the prune-belly syndrome in a 34 year old man. Int Urol Nephrol 1974 <br />
  28. 28. Sindrome de abdomen en ciruela pasa<br />Testículos (fisiopatología)<br />Localización a nivel de vasos ilíacos<br />Histológicamente, son indistinguibles de las gónadas normales<br />En adultos han demostrado poca maduración<br />Anormalidad de los anexos<br />
  29. 29. Sindrome de abdomen en ciruela pasa<br />Riñones (fisiopatología)<br />Displasia en el 50%<br />Desarrollo anormal<br />La displasia casi siempre es mas grave en aquellos que nacen con estenosis o agenesia uretral, megalouretra y ano imperforado.<br />
  30. 30. Sindrome de abdomen en ciruela pasa<br />Uréteres<br />Elongados, dilatados y tortuosos<br />Distribución en parches musculares lisos que se intercalan con fibrocitos y colágena.<br />Peristalsis inefectiva<br />El grado de dilatación no se vincula con el estado de los riñones ni el pronóstico del pcte<br />Nunn IN, Stephens FD: The triad system: A composite anomaly of the abdominal wall, urinary system and testes. J Urol 1961<br />
  31. 31. Sindrome de abdomen en ciruela pasa<br />Vejiga y uraco (Fisiopatología)<br />Vejiga grande y distorsionada<br />Algunas partes funcionan como divertículos<br />Casi siempre el trígono es grande y asimétrico<br />Ectopia ureteral<br />Berdon WE, Baker DH, Wigger HJ, et al: The radiologic and pathologic spectrum of the prune belly syndrome: The importance of urethral obstruction in prognosis. Radiol Clin North Am 1977 <br />
  32. 32. Sindrome de abdomen en ciruela pasa<br />Vejiga y uraco (Relaciones clínicas)<br />Dinámica anormal durante la micción con sensación tardía para orinar y volúmenes mayores a los normales por el reflujo y menor capacidad de contracción<br />El incremento de las presiones de reposo y el volumen residual afecta el vaciamiento de los uréteres.<br />Snyder HM, Harrison NW, Whitfield, HN, Williams DI: Urodynamics in the prune belly syndrome. Br J Urol 1976 <br />
  33. 33. Sindrome de abdomen en ciruela pasa<br />Próstata y uretra prostática (fp)<br />Hipoplasia generalizada del epitelio prostático<br />El tejido fibroso reemplaza su parte anterior<br />El verumontanum puede faltar por completo<br />Kroovand RL, Al-Ansari RM, Perlmutter AD: Urethral and genital malformations in prune-belly syndrome. J Urol 1982 <br />
  34. 34. Sindrome de abdomen en ciruela pasa<br />Diagnóstico prenatal<br />La validez diagnóstica del ultrasonido fetal no es óptima<br />La presencia de dilatación de las vías urinarias no supone a veces una obstrucción considerable<br />Los cambios ectásicos no son detectables casi nunca hasta después que se produce una agresión embriológica importante<br />
  35. 35. Sindrome de abdomen en ciruela pasa<br />Manifestaciones Clínicas<br />Durante el embarazo se puede detectar oligohidroamnios y en ocasiones observarse un aumento de la vejiga urinaria. <br />Triada característica<br />Otras malformaciones asociadas que se presentan con una ocurrencia del 10% son: hipoplasia pulmonar, cardiopatías congénitas, defectos gastrointestinales, anomalías en las extremidades como pie club o Bott. <br />
  36. 36. Sindrome de abdomen en ciruela pasa<br />Exámenes Complementarios<br />Se recomienda complementar la evaluación clínica con exámenes de gabinete como ser una ecografía reno-vesical, urografía excretora, cistouretrogafía retrograda, cintigafía renal. Por otro lado se deberán solicitar todos los exámenes necesarios para evaluar la función renal. <br />
  37. 37. Sindrome de abdomen en ciruela pasa<br />Right (A) and left (B) kidneys in a 1-day-old infant with typical prune-belly syndrome. The infant was seen at birth, prescribed antibiotics, and monitored closely with urine cultures, serum creatinine determinations, and renal ultrasound imaging. (From Woodard JR, Zucker I: Current management of the dilated urinary tract in prune-belly syndrome. Urol Clin North Am 1990;17:413.) <br />
  38. 38. Sindrome de abdomen en ciruela pasa<br />Cystogram showing massive bilateral vesicoureteral reflux. Note the marked difference in caliber of the left upper ureter compared with the lower ureter.<br />
  39. 39. Sindrome de abdomen en ciruela pasa<br />A and B, Voiding cystograms in two patients, showing the pseudodiverticulum configuration of the dome of the bladder. Note the wide, triangular prostatic urethra in both cystograms and the small utricular configuration in B.<br />
  40. 40. Sindrome de abdomen en ciruela pasa<br />A, Intravenous urogram of a 2-year-old boy with prune-belly syndrome shows the classic appearance of tortuous, dilated ureters with relatively well preserved kidneys. B, Urogram in the same patient at age 19 years shows improvement in ureteric dilation without surgical treatment and no deterioration in renal function.<br />
  41. 41. Sindrome de abdomen en ciruela pasa<br />Manejo<br />Debe individualizarse<br />Después del parto se dicta el pronóstico inmediato del lactante por su estado pulmonar.<br />El pronóstico a largo plazo del lactante depende de su estado renal<br />Berdon WE, Baker DH, Wigger HJ, et al: The radiologic and pathologic spectrum of the prune belly syndrome: The importance of urethral obstruction in prognosis. Radiol Clin North Am 1977 <br />Geary DF, MacLusky IB, Churchill BM, et al: A broader spectrum of abnormalities in the prune belly syndrome. J Urol 1986 <br />
  42. 42. Sindrome de abdomen en ciruela pasa<br />Controversias terapéuticas<br />Abordaje quirúrgico radical<br />Alrededor del 33% de pacientes intervenidos se libra de tener infecciones recurrentes<br />La insuficiencia renal crónica se instaura en la mayoría de niños con anomalías funcionales desde el aquellos nacimiento<br />Fallat ME, Skoog SJ, Belman AB, et al: The prune-belly syndrome: A comprehensive approach to management. J Urol 1989 <br />Woodard JR, Parrott TS: Reconstruction of the urinary tract in prune-belly uropathy. J Urol 1978 <br />
  43. 43. Sindrome de abdomen en ciruela pasa<br />Controversias terapéuticas<br />Se recomienda la intervención quirúrgica cuando la antibioprofilaxia y al observación no tiene éxito para conservar la función renal y prevenir la bacteriuria<br />Hasta un 75% de los pctes que presentaban función renal normal cuando fueron RN mantuvieron una creatinina estable de adultos.<br />Woodhouse CRJ, Ransley PG, Williams DI: Prune Belly <br />syndrome-report of 47 cases. Arch Dis Child 57. 1982<br />
  44. 44. Sindrome de abdomen en ciruela pasa<br />Controversia terapéuticas<br />La función ureteral y el flujo mejora en muchos pacientes que no se someten a cirugía<br />La reducción del calibre del uréter del SAC no incrementa su función peristáltica y el drenaje<br />La cirugía no revierte la displasia renal<br />Los sistemas de baja presión se deben a deficiencia del músculo liso ureteral, no a la obstrucción <br />Duckett JW Jr: The prune-belly syndrome. In Holder TM, Ashcroft KW (eds): The Surgery of Infants and Children. Philadelphia, WB Saunders, 1980 <br />Palmer JM, Tesluk J: Ureteral pathology in the prune belly syndrome. J Urol 1974 <br />
  45. 45. Sindrome de abdomen en ciruela pasa<br />Gracias<br />

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