World Journal of Surgery

, Volume 17, Issue 3, pp 301–309 | Cite as

Duodenal atresia and stenosis: Reassessment of treatment and outcome based on antenatal diagnosis, pathologic variance, and long-term follow-up

  • Jay L. Grosfeld
  • Frederick J. Rescorla
World Progress in Surgery

Abstract

Duodenal atresia and stenosis was observed in 103 infants and children from 1972 to 1991. There were 59 girls and 44 boys. Atresia was noted in 79 instances and stenosis in 24. Maternal hydramnios was detected in 33 cases, 46 babies were premature, and 31 had Down's syndrome. Fifty-four infants had significant associated anomalies including 35 with cardiac defects. Diagnosis was achieved by prenatal ultrasound examination in 14 cases, observation of a double-bubble sign on abdominal radiograph in 73, and contrast studies in 30 infants including 24 with stenosis. At operation annular pancreas was noted in 37 cases, malrotation in 37 cases, anterior portal vein in 4, and a second web in 3. Surgical treatment included duodenoduodenostomy in 85, duodenotomy and web excision in 8, and duodenojejunostomy in 10. Operative survival was 95%. Deaths were related to complex cardiac defects. Despite antenatal diagnosis, prompt intervention, and apparent early surgical success (95% survival), late deaths (5%) and late complications including motility disorders, megaduodenum, gastroesophageal reflux, duodenal-gastric reflux, gastritis, peptic ulcer disease, blind loop syndrome, and biliary-pancreatic conditions may be observed months to years after management during the neonatal period. Modifications in surgical technique including early tapering duodenoplasty may be useful, and close long-term follow-up is an essential component of patient care.

Résumé

Entre 1972 et 1991, on a observé 79 cas d'atrésie duodénale et 24 cas de sténose duodénale chez un total de 103 enfants, 59 filles et 44 garçons. Une hydramnios a été retrouvée chez 33 des mères, 46 enfants étaient des prématurés et 31 avait un syndrome de Down. Chez 54 enfants, il y avait des malformations associées, dont 35 cardiaques. Le diagnostic avait été établi dans 14 cas par une échographie anténatale, par l'existence du signe de la double bulle sur les abdomens sans préparation chez 73 patients et par une étude du transit intestinal en double contraste chez 34 enfants, dont 24 avec une sténose. A l'opération, on a retrouvé un pancréas annulaire chez 37 enfants, une malrotation chez 37, une veine porte antérieure chez 4, et une membrane intraluminale chez 3. Le traitement a été une anastomose duodénoduodénale chez 85 enfants, une excision de membrane après duodénotomie chez 8 et une duodénojéjunostomie chez 10. La mortalité opératoire était de 5%, en rapport essentiellement avec des malformations cardiaques complexes. En dépit d'un diagnostic anténatal, une intervention précoce et un succès chirurgical apparent, la morbidité tardive est toujours possible et comprend essentiellement des anomalies de la motilité duodénale, le mégaduodénum, un reflux gastroesophagien, un reflux duodénogastrique, une gastrite, la maladie ulcéreuse, un syndrome de l'anse borgne, et d'autres anomalies biliopancréatiques qui peuvent se voir des mois ou des années après la période néonatale. Les modifications de la technique chirurgicale et notamment la duodénoplastie précoce ainsi qu'une meilleure surveillance à distance, peuvent en améliorer le pronostic.

Resumen

Se encontró atresia duodenal y estenosis en 103 niños, 59 de sexo femenino y 44 de sexo masculino, en el período 1972–91: atresia en 79 casos y estenosis en 24. Se registró hidramnios materna en 33 casos; 46 niños fueron prematuros y 31 presentaban síndrome de Down; 54 exhibían anomalías de significación, incluyendo 35 con defectos cardiacos. El diagnóstico fue establecido por ultrasonido prenatal en 14 casos, mediante la observación de burbuja doble en la radiografía abdominal en 73 y por estudio con medio de contraste en 30, entre los cuales había 24 con estenosis. En la operación se encontró páncreas anular en 37 casos, malrotación en 37, vena porta anterior en 4 y segunda membrana en 3. El tratamiento quirúrgico incluyó duodenostomía en 85 pacientes, duodenotomía y resección de membrana en 8 y duodenoyeyunostomía en 10. La tasa de sobrevida operatoria fue de 95%. Las muertes estuvieron asociadas con los defectos cardfacos complejos. A pesar de su diagnóstico prenatal, una intervención quirúrgica precoz y un aparente éxito operatorio (sobrevida de 95%), se observan muertes tardías (5%) y complicaciones a largo plazo tales como desórdenes de la motilidad intestinal, megaduodeno, reflujo gastroesofágico, reflujo duodeno-gástrico, gastritis, enfermedad ulcerosa péptica, síndrome de asa ciega y alteraciones biliopancreáticas, meses a años después del tratamiento y manejo neonatales. Algunas modificaciones en la técnica quirúrgica, tales como duodenoplastia, pueden ser de utilidad; el seguimiento cuidadoso a largo plazo constituye un componente esencial de la atención de estos pacientes.

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Copyright information

© Société Internationale de Chirurgie 1993

Authors and Affiliations

  • Jay L. Grosfeld
    • 1
    • 2
  • Frederick J. Rescorla
    • 1
    • 2
  1. 1.Section of Pediatric Surgery, Department of SurgeryIndiana University School of MedicineIndianapolisUSA
  2. 2.James Whitcomb Riley Hospital for ChildrenIndianapolisUSA

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