Hirschsprung Associated Enterocolitis

Abstract

HSD is a common cause of neonatal intestinal obstruction that is of great interest to pediatricsurgeons throughout the world. Although this congenital anomaly is curable and significant advancein the treatment was obtained during the last decades, a minority ofpatients can still develop serious complications and eventually died.The main cause is certainly to be found in HAEC, which can basically occur from birth toadulthood, regardless of the length of aganglionosis. The incidence of enterocolitis depends on the type ofrepair, presence or absence of predisposing factors, andinstitutional diagnostic criteria for enterocolitis. The incidence of HAEC has declined over the last 40 yearsdue to improved and more prompt diagnosis of HSD but ithas remained a major cause of morbidity and mortality. Despite significant advances in elucidating the geneticetiology of HSD and improvedsurgical techniques, little progress has been noted in discovering the etiology or prevention of HAEC. Manytheories have been proposed including mechanical dilatationand fecal stasis, alterations of mucin components,increased prostaglandin activity, Bacterial and viral infection, and impaired mucosalimmune defense mechanism. Unfortunately, the pathogenesisof enterocolitis is poorly understood. This perplexingproblem includes a wide range of clinical presentationsincluding abdominal distension, explosive diarrhea,vomiting, fever, lethargy, rectal bleeding, and shock. Pastor and co-workers, in 2008, developed a more standardizeddefinition of HAEC. They used a Delphi method, a technique forachieving consensus among a panel of experts. The Authorsdeveloped a scoring system and established HAEC definition basing onclinical-radiologic criteria including symptoms, blood tests, X-raysand personal history. Abdominal radiographs may reveal a distended loop ofcolon often associated with small bowel dilation and/ormultiple air fluid levels.Contrastenema shows an irregular mucosal lining, with ulceration. If repeated bouts of enterocolitis persist after definitivepull-through, investigation into mechanical causes shouldbe considered. Contrast enema, manometry, and rectalbiopsy may be necessary.