Pediatric Crohn Disease Surgery

Updated: Mar 23, 2018
  • Author: Patricia A Valusek, MD; Chief Editor: Harsh Grewal, MD, FACS, FAAP  more...
  • Print
Overview

Background

Crohn disease (CD) is a chronic disorder that primarily affects young individuals. [1] It has been found to occur in all parts of the gastrointestinal tract (GI) and to have widespread extraintestinal manifestations. The pathologic criteria to distinguish CD from other forms of inflammatory bowel disease (IBD), such as ulcerative colitis (UC), were not established until 1960. [2, 3]  Although the etiology is unknown, findings regarding the genetic and immunologic features of CD have provided insight into the possible mechanisms of pathogenesis.

CD is an incurable condition that requires ongoing medical management and causes long-term pain and disability. Frequent surgical interventions are often required to treat complications. Corticosteroids have been the mainstay of medical treatment in patients with active inflammation, but salicylates, antibiotics, and immune therapies have also shown benefit. Surgical treatment has been reserved for refractory disease and complications. Minimally invasive surgery provides an alternative to the traditional open surgical approach.

Next:

Pathophysiology

CD can affect any part of the alimentary tract from the mouth to the anus. The most common site is the terminal ileum. The appendix is also often involved. The disease is often segmental (unlike ulcerative colitis), and the rectum is frequently spared.

Endoscopically, CD typically appears as patchy areas of inflammation separated by uninvolved bowel. The earliest lesions are aphthous ulcers—tiny discrete erosions that typically develop over lymphoid follicles. As the disease progresses, interconnecting rows of aphthous ulcers create linear ulcers. These linear ulcers cross transverse folds, contributing to a cobblestone appearance. [4]

Grossly, surgical specimens are rigid and thickened owing to chronic inflammation and fibrosis during healing periods. The mesentery is typically thickened and foreshortened and may partly surround the bowel wall in a phenomenon known as “creeping fat” (see the image below). The transmural nature of inflammation contributes to possible fistulous connections between segments of bowel or other organs.

Laparoscopic view depicts creeping fat along the m Laparoscopic view depicts creeping fat along the mesentery of the terminal ileum.
Previous
Next:

Etiology

The etiology of CD is unknown. Its pathogenesis is likely multifactorial, involving a combination of infectious agents and environmental exposures that activate an immune response in a genetically susceptible host. Familial clustering of CD suggests a genetic etiology. A positive family history is found in 10-15% of patients. The relative risk for first-degree relatives of these patients is estimated to be 10-21 times that of the general population. Disease locations and types (eg, stricturing, inflammatory, or perforating) tend to be similar among family members.

Other genetic abnormalities linked with CD include hereditary conditions such as glycogen storage disease type 1b and complement pathway dysfunction. [5, 6, 7]

Bacterial agents have long been thought to be involved in the pathogenesis of CD, though no bacteria have been definitively identified. Thus, in this multifactorial model, the interaction of bacteria, immune mechanisms, and genetic susceptibility may be necessary for the development of the disease. [5, 6, 7]

Environmental factors such as smoking and second-hand smoke have been linked to the development of CD. [8] There has been a suggestion that a diet high in fatty foods may increase the risk for the disease. [9] Concerns about the measles vaccine and the development of the disease have proved to be unfounded. [10] Although appendectomy has been suggested to be protective in UC, it is not a protective factor in CD. [11]

Previous
Next:

Epidemiology

The incidence of CD in the general population is approximately 6.3-7.9 cases per 100,000 persons, with a reported prevalence of 43-174 per 100,000. [3, 12, 13, 14] The onset of the disease peaks in older adolescents and young adults. However, approximately 5% of new cases occur in children younger than 5 years. CD affects persons of every ethnic origin but is more common in the Ashkenazi Jewish and Caucasian populations. [5] The disease has no sexual predilection.

Previous
Next:

Prognosis

CD is a chronic, incurable condition. Among children with juvenile-onset CD, 86% require surgical intervention within 15 years of disease onset. Although our understanding of the disease is incomplete, advances in medical therapies and surgical procedures have led to more treatment options for these patients. With proper treatment, most achieve a healthy height and weight, and the mortality for the disease is low.

Previous