Clinical Presentation & Protocol
Patient Usually Complains Of
Patient presents with history of failure to pass meconium within the first 48 hours of life, persistent abdominal distension, bilious vomiting, and enterocolitis symptoms. Clinical suspicion for total colonic aganglionosis (TCA) supported by failure of conservative management and persistent obstructive symptoms.
Clinical Examination Findings
Abdominal examination reveals significant distension, visible bowel loops, and tympany to percussion. Rectal examination demonstrates an empty rectal vault with a tight anal sphincter and a positive "squirt sign" or explosive discharge of gas and stool upon withdrawal of the examining finger.
Treatment Protocol
Immediate stabilization with IV fluids and nasogastric decompression. Definitive management requires surgical intervention, typically involving a diverting ileostomy followed by a pull-through procedure (e.g., Duhamel, Soave, or Swenson technique) to restore intestinal continuity. Monitor closely for Hirschsprung-associated enterocolitis (HAEC).