Abstract
Malrotation of the intestinal tract is a product of a well defined aberrant embryology. Because the consequences of malrotation associated with a midgut volvulus may be catastrophic, an understanding of the anatomy, diagnostic criteria, and appropriate therapy for this putative emergency illness is imperative. This report summarizes a recent 18-month experience with this diagnosis and contrasts this experience with that in the published literature. More than half (14/22) of the patients presented during the first month of life, and all had vomiting, which in most cases was bilious. The barium upper gastrointestinal series was the preferred diagnostic study, being both sensitive (18/19, 95%) and accurate (18/21, 86%). In this series two-thirds of the patients presented with volvulus (15/22, 68%) of whom five had ischemic intestine requiring resection. One of these children died of overwhelming sepsis. A Ladd procedure was the preferred treatment, which as defined by us includes evisceration and inspection of the mesenteric root, counterclockwise derotation of a midgut volvulus, lysis of Ladd's bands with straightening of the duodenum along the right abdominal gutter, inversion-ligation appendectomy, and placement of the cecum into the left lower quadrant. A high index of suspicion in the neonate with vomiting, rapid diagnosis, and appropriate operative therapy results in a predictable favorable outcome for children with intestinal malrotatioh.
Résumé
La malrotation du tube intestinal est le résultat d'une anomalie embryologique bien connue. Les conséquences d'une malrotation intestinale associée à un volvulus de l'anse intestinale moyenne peuvent être catastrophiques. C'est pourquoi la compréhension parfaite de l'anatomie, des critères diagnostiques et une thérapeutique adaptée sont impératifs. Cet article résume une expérience récente de 18 mois à propos de ce diagnostic, comparé avec les données de la littérature. Plus de la moitié de nos patients (14/22) ont été vus dans le premier mois de vie; tous avaient des vomissements, le plus souvent bilieux. Un transit gastrointestinal du tube supérieur était l'examen diagnostique préféré, ayant à la fois une sensibilité (18/19, 95%) et une précision 18/21, 86% importantes. Dans cette séries, deux-tiers des malades ont présenté un volvulus (15/22, 68%), dont cinq ayant nécessité une résection intestinale en raison d'une ischémie. Un de ces enfants est mort d'un sepsis fulminant. Le procédé de Ladd a été la méthode thérapeutique de choix, qui comprend, selon nous, une éviscération complète, une inspection de la racine du mésentère, une dérotation de l'intestin dans le sens contraire des aiguilles d'une montre, une lyse des bandes de Ladd, redressant le duodénum le long de la gouttière droite, une appendicectomie avec enfouissement et le placement simple sans pexie du caecum dans la fosse iliaque gauche. Soupçonner cette malformation chez le nouveau-né qui vomit, faire un diagnositc précoce et réaliser une thérapeutique adaptée sont les clés du succès chez les enfants ayant une malrotation intestinale.
Resumen
La malrotación del tracto gastrointestinal es el resultado de una aberración embriológica bien definida. Puesto que las consecuencias de la malrotación asociada con volvulus del intestino medio pueden ser catastróficas, se hace imperioso poseer un buen conocimiento de la anatomía, los criterios diagnósticos y la adecuada terapia de esta emergencia. El presente informe resume la experiencia reciente en un período de 18 meses con esta entidad y la compara con la publicada en la literatura. Más de la mitad (14/22) de los pacientes se presentaron en el primer mes de su vida y todos exhibieron vómito que, en la mayoría de los casos, era bilioso. La serie gastrointestinal con bario constituyó el estudio diagnóstico de preferencia, y probó ser de alta sensibilidad (18/19, 95%) y certeza (18/21, 86%). En esta serie dos terceras partes de los pacientes se presentaron con volvulus (15/22, 68%), cinco de ellos con necrosis isquémica del intestino que requirió resección. Uno de estos niños murió por sepsis severa. El procedimiento de Ladd fue el tratamiento de elección, el cual, según nuestra propia definición, incluye la evisceración e inspección de la raiz del mesenterio, la desrotación del volvulus del intestino medio en el sentido del reloj, la lisis de las bandas de Ladd con alineación del duodeno sobre la gotera abdominal derecha, apendicetomía y colocación del ciego en el cuadrante inferior izquierdo. Un alto ídice de sospecha en el bebé con vómito, la pronta definición del diagnóstico y la instauración de la terapia adecuada resultan en un resultado predeciblemente favorable en los niños con malrotación intestinal.
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Torres, A.M., Ziegler, M.M. Malrotation of the intestine. World J. Surg. 17, 326–331 (1993). https://doi.org/10.1007/BF01658699
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DOI: https://doi.org/10.1007/BF01658699