Chronic inflammatory bowel disease (IBD) is divided[JZ1] into two main forms: Crohn's disease (MC) and ulcerative colitis (UC). The age at first onset of symptoms (first manifestation) is usually between 15 and 25 years. While MC is more frequently observed in boys, the gender distribution is the same for CU. There is a familial clustering of IBD in 10-30% of patients, although this is more common in MC than in CU. The incidence of IBD is around 100-200 cases per 100,000 people. Several studies in children have shown an increasing frequency (incidence) of MC in recent years, while the frequency of IBD remains the same or, in some studies, is decreasing. Countries with high rates of IBD are industrialized nations such as Northern Europe and North America, which is referred to as the "North-South divide".

 

Causes:

It is assumed that IBD is caused by a combination of genetic, immunological and environmental factors. In genetically predisposed people, an excessive reaction of the immune system to environmental influences (inadequate immune response) probably leads to an excessive inflammatory reaction in the intestine.

 

Main forms of IBD:

Crohn's disease (MC)

Crohn's disease is a chronic inflammatory reaction that can affect the entire intestinal tract (Fig. 1). The disease typically spreads to different areas, so that healthy areas (skip leasons) are also located between the diseased sections of the intestine. The intestinal inflammation affects all wall layers of the intestine (transmural infiltration). The most common localization is the lowest section of the small intestine (terminal ileum), which is why MC is also referred to as "Ileitis terminalis". With a longer course of the disease, encapsulated inflammations (abscesses), constrictions (strictures) or connections between two sections of the intestine or hollow organs (fistulas) often develop. In contrast to adults, isolated infestation of the terminal ileum is less common in children, whereas infestation of the colon and upper gastrointestinal tract is more frequent. In up to 25% of cases, Crohn's disease is associated with diseases outside the gastrointestinal tract. These include diseases of the muscles or skeleton, the respiratory tract, the skin or the eyes. Children and adolescents in particular can suffer from social exclusion, a drop in performance at school and strong feelings of guilt as a result of illness-related deficits.

Ulcerative colitis (CU)

Ulcerative colitis is also an inflammatory disease of the intestinal mucosa (Fig. 1). In contrast to MC, however, the inflammation spreads continuously along the colon mucosa from below (rectum) towards the upper sections of the colon and remains almost exclusively limited to the rectum and colon. In CU, the mucosal lesions remain limited to certain intestinal layers (mucosa and submucosa). When children are diagnosed with ulcerative colitis, up to 90% of cases are already at an advanced stage with inflammation of the entire colon (pancolitis).

 

Progression of IBD

Chronic inflammatory bowel disease usually progresses in phases, interrupted by intervals with few or no symptoms (remission). The most common clinical symptoms are increased stool frequency and diarrhea. In addition, the clinical picture is often characterized by cramp-like abdominal pain and weight loss. Bleeding from the anus or bloody diarrhea can also occur and are more common in CU than in MC.

Drug therapy

The main features of drug therapy are aggressive treatment of acute relapses and support for maintaining remission for as long as possible (relapse prophylaxis). Cortisone(corticosteroids) is one of the most commonly used medications for acute flare-ups and initial therapy, even in children, despite severe side effects. Anti-inflammatory drugs(5-aminosalicylates), antibiotics and immunosuppressants (e.g. azathioprine, 6-mercaptopurine, methotrexate) can be used in children and adults to maintain remission or prevent relapses. So-called biologics (biologic agents = antibodies against certain body substances) are available for severe courses or if corticosteroids fail. The value of the individual substance groups can be illustrated using a step-by-step scheme (Fig. 2).

Primary enteral nutrition therapy with sip and tube feeds can achieve very good results in the treatment of an acute inflammatory flare-up in children with Crohn's disease. Compared to systemic corticosteroid therapy, nutritional therapy has only minor side effects and at the same time improves the patient's quality of life.

 

Surgical therapy

The fact that there is no curative treatment for Crohn's disease and only the removal of the entire colon (colectomy) for ulcerative colitis underlines the central importance of conservative, remission-preserving therapy for IBD. Accordingly, surgical therapy is particularly important in the management of complications.

 

Due to complications, 70 - 90 % of patients with M. Crohn's disease require surgical intervention during the course of the disease. Emergency indications for surgery include intestinal perforation (formation of a hole in the intestine) and severe bleeding into the abdominal cavity. In children, failure of drug therapy with persistent symptoms such as growth disturbances is one of the most common reasons (47%) for surgical intervention, followed by intra-abdominal abscesses and perforations (16%), obstructions (narrowing, 16%), fistula formation (14%), severe bleeding (4%) and appendectomies (appendectomy, 3%). The surgical procedure used in MC is the removal of a specific segment of the bowel with inflammatory changes (segmental resection). In order to reduce the risk of short bowel syndrome (absence of large parts of the small bowel), many surgeons prefer stricturoplasty (see below) to resection in cases of small bowel involvement. In addition to the open surgical technique, bowel resections can also be performed minimally invasively (laparoscopically).

Patients with ulcerative colitis are treated surgically in 30 - 40 % of cases during the course of their disease. In addition to failure of drug therapy, indications for this disease include intestinal perforations, severe bleeding, the occurrence of a toxic megacolon (acute dilatation and severe inflammation of the colon) and degeneration of the intestinal mucosa such as colorectal carcinoma. In contrast to Crohn's disease, surgical treatment of ulcerative colitis can be causal and curative. Today, proctocolectomy with ileopouchanal anastomosis (IPAA, removal of the rectum and colon while leaving the anal canal and sphincter intact) is the most common procedure. A pouch is formed from the last part of the small intestine = terminal ileum, which serves as a stool reservoir; this pouch is sutured to the anal canal), a frequently used surgical procedure. However, this surgical technique is contraindicated in Crohn's disease and perianal or ileal involvement, as it has been shown that postoperatively there is a high risk of anorectal recurrence, fistulas and abscesses in the area of the pouch. The IPAA can also be performed laparoscopically in addition to the traditional open procedure.

Drug therapy

The main features of drug therapy are aggressive treatment of acute relapses and support for maintaining remission for as long as possible (relapse prophylaxis). Cortisone (corticosteroids) is one of the most commonly used medications for acute flare-ups and initial therapy, even in children, despite severe side effects. Anti-inflammatory drugs (5-aminosalicylates), antibiotics and immunosuppressants (e.g. azathioprine, 6-mercaptopurine, methotrexate) can be used in children and adults to maintain remission or prevent relapses. So-called biologics (biologic agents = antibodies against certain body substances) are available for severe courses or if corticosteroids fail. The value of the individual substance groups can be illustrated using a step-by-step scheme.

Primary enteral nutrition therapy with sip and tube feeds can achieve very good results in the treatment of an acute inflammatory attack in children with Crohn's disease. Compared to systemic corticosteroid therapy, nutritional therapy has only minor side effects and at the same time improves the patient's quality of life.

Surgical therapy

The fact that there is no curative treatment for Crohn's disease and only the removal of the entire colon (colectomy) for ulcerative colitis underlines the central importance of conservative, remission-preserving therapy for IBD. Accordingly, surgical therapy is particularly important in the management of complications.

 

Due to complications, surgical intervention is required in 70 - 90 % of patients with Crohn's disease during the course of the disease. Emergency indications for surgery include intestinal perforation (formation of a hole in the intestine) and severe bleeding into the abdominal cavity. In children, failure of drug therapy with persistent symptoms such as growth disturbances is one of the most common reasons (47%) for surgical intervention, followed by intra-abdominal abscesses and perforations (16%), obstructions (narrowing, 16%), fistula formation (14%), severe bleeding (4%) and appendectomies (appendectomy, 3%). The surgical procedure used in MC is the removal of a specific segment of the bowel with inflammatory changes (segmental resection). In order to reduce the risk of short bowel syndrome (absence of large parts of the small bowel), many surgeons prefer stricturoplasty (see below) to resection in cases of small bowel involvement. In addition to the open surgical technique, bowel resections can also be performed minimally invasively (laparoscopically).

Patients with ulcerative colitis are treated surgically in 30 - 40 % of cases during the course of their disease. In addition to failure of drug therapy, indications for this disease include intestinal perforations, severe bleeding, the occurrence of a toxic megacolon (acute dilation and severe inflammation of the colon) and degeneration of the intestinal mucosa such as colorectal carcinoma. In contrast to Crohn's disease, surgical treatment of ulcerative colitis can be causal and curative. Today, proctocolectomy with ileopouchanal anastomosis (IPAA, removal of the rectum and colon while leaving the anal canal and sphincter intact) is the most common procedure. A pouch is formed from the last part of the small intestine = terminal ileum, which serves as a stool reservoir; this pouch is sutured to the anal canal), a frequently used surgical procedure. However, this surgical technique is contraindicated in Crohn's disease and perianal or ileal involvement, as it has been shown that postoperatively there is a high risk of anorectal recurrence, fistulas and abscesses in the area of the pouch. The IPAA can also be performed laparoscopically in addition to the traditional open procedure.

 

 

Two weeks after the operation, the surgeon will see you in our pediatric surgery outpatient clinic. The integrity of the intestinal suture will then be checked by palpating the anus. We will also instruct you to perform daily irrigations and fillings of the pouch via the ileostomy until the artificial bowel outlet (ileostomy) can be surgically closed again after approx. 2-3 months.

We provide long-term care after the operation on an interdisciplinary basis in close cooperation with the pediatric gastroenterologists at the MHH (headed by Prof. Baumann).