| Popis: |
Hirschsprung’s disease and Hirschsprung-associated enterocolitis are functional disorders of the enteric nervous system leading to a functional bowel obstruction. Patients will often present in the first few days of life with delayed passage of meconium, abdominal distention, and poor feeding. Hirschsprung-associated enterocolitis is associated with increased morbidity and mortality and will present with signs and symptoms consistent with bowel obstruction, frankly bloody diarrhea, lethargy, fever, and, in severe cases, septic shock. Suspected cases should receive aggressive fluid resuscitation and broad-spectrum antibiotics. The gold standard for diagnosis is rectal biopsy and definitive management is surgical. Though many patients report some degree of bowel dysfunction later in life, a great number of patients do well. |
