1018
LOUIE
Management All infants will require admission for intravenous broad-spectrum antibiotics. For ill-appearing neonates, fluid resuscitation is a priority along with other supportive measures for airway, breathing, and circulation. A consultation with a neonatologist or pediatric infectious disease specialist is prudent for guidance on antimicrobial selection. A pediatric surgical consultation is warranted to remove infected tissue. Hirschsprung’s disease Pathophysiology From a histologic perspective, Hirschsprung’s disease is caused by aganglionosis, hypoganglionosis, or dysganglionosis of the bowel [54]. The congenital agangliosis always involves the anus, and the pathology proceeds proximally for a variable length of large bowel. Both the myenteric (Auerbach) and submucosal (Meissner) plexus are absent, resulting in diminished bowel peristalsis and function. The anus will have an abnormal or absent relaxation [55]. The precise mechanism underlying the development of Hirschsprung’s disease is unknown. Epidemiology It is estimated that 1 in 5000 newborns will have Hirschsprung’s disease [56]. Three quarters of the infants will be males [57]. Familial cases have been described [30]; thus it is not surprising that after one child has the disease, subsequent offspring are at much higher risk than the general population [58]. Other congenital abnormalities have been found with Hirschsprung’s disease: Trisomy 21, Smith-Lemli-Opitz syndrome, and Waardenburg’s syndrome [59]. This is an abbreviated list. Clinical presentation In the newborn period, the most common presentation is a failure to pass meconium in the first 48 hours of life [60]. For neonates who present to the ED, there are two well-described presentations: bowel obstruction and enterocolitis. The former often will present with complaints of abdominal distention, bilious or feculent vomiting, constipation, and other signs of obstruction [15,61,62]. Older patients and adults have been diagnosed with Hirschsprung’s disease. They usually complain of chronic constipation, vomiting, and failure to thrive [61]. For infants who present outside of the neonatal period, 30% will have diarrhea, which some researchers believe is a symptom of enterocolitis [63]. Enterocolitis occurs in 12% to 60% of patients with congenital agangliosis [63,64]. It is the most serious complication of Hirschsprung’s disease [65]. It may occur before a diagnosis of Hirschsprung’s disease is made, and it is described after corrective surgery. Mortality rates from treated Hirschsprung’s disease are as high as 30% [66]. The symptoms of enterocolitis