Enfermedad de Hirschsprung asociada al síndrome de Mowat-Wilson: a propósito de un caso

Abstract

INTRODUCTION: Hirschsprung's disease (HD) or aganglionicmegacolon is a congenital disorder characterized by the absence of ganglion intramural cells of the submucosal myenteric plexus (namely Auerbach and Meissner,respectively) in distal sections of the intestine. This is due to a failure in the migration of the precursors of these cells from the neural chalk during the embryonic development and also due to other abnormalities associated(18 % of cases), in some cases involving specific polymalformation syndromes. OBJECTIVES: The aim of the work is to present the clinical and nutritional evolution of a 14 months aged pediatric patient who was diagnosed with HD since was born associated with Mowat-Wilson syndrome (MWS). In addition,it is also targeted to evaluate the clinical results from this patient. METHODS: Reviewing the medical history of the patient,the evolution of the anthropometric data (weight and height) as well as the analytical parameters for further studies were carried out. In addition, the upcoming issues associated with nutritional support and therapeutic strategies in the multidisciplinary context were evaluated. RESULTS: A male, 14 months aged patient, son of not consanguineous healthy immigrants parents from Colombia went to the emergency department of our hospital suffering abdominal distension and vomiting with no spontaneous bowels. A detailed description of the composition of parenteral nutrition administered within the period of 28 days of hospital admission. CONCLUSION: As observed in this patient, the association of HD and MWS can lead to the need of prolonged parenteral nutrition and frequently present diverting stoma leading to a greater number of postoperative complications in this population.