![]() |
|
||
![]() |
![]() |
![]() |
|
Doctors warn that using domestic spoons to give children medicine increases overdose risk |
What difference can a pediatric surgeon make?Pediatric surgeons specialize in the surgical care of children. They are surgeons who, by training, are oriented toward working with children and understanding their special needs. Learn More |
More than 50% of all child injuries are either caused or related to car or bicycle crashes. Like Mother |
Crohn’s disease (CD), also known as regional enteritis, is a type of inflammatory bowel disease (IBD) described by Crohn in 1932. The most common affected site of bowel inflammation is the distal part of the small intestine known as the ileum, although the colon and other segments of the intestinal tract can be involved as well. The other common type of IBD is termed ulcerative colitis, although this disease is limited to the colon (large intestine).
Crohn’s disease occurs with equal frequency in men and women, although it is more prevalent in whites than in blacks, and has a marked increase among Jews. Although the peak onset is in the mid to late teens, approximately 5% of patients are younger than 5 years of age. Children of parents with CD and siblings of affected children have a higher (35-fold to 70-fold) risk.
ETIOLOGY
The exact cause of CD is unknown, although several causative factors
have been suggested. Genetic factors and transmissible infection are
considered as the most likely possibilities, in as much as both are
observed in many patients with CD. For example, the HLA B27 gene is
known to predispose to the development of inflammatory bowel disease.
Some investigators have indicated that nonsteroidal anti-inflammatory
drugs, toxins, and infections may be associated with the development of
CD. Because lymphangiectasia and enlarged bowel lymph nodes are
frequently found in CD, obstruction of the lymphatic system also has
been suggested as a possible factor. Smoking has been recognized as one
of the strongest risk factors for the development of CD. Interestingly,
no specific dietary habits or dietary ingredients have been implicated
consistently with development of CD.
PATHOLOGY
In CD, the involved intestinal wall is thickened with edema (extra
fluid), fibrosis, and lymphatic dilation. Small ulcers may appear in the
inner lining of the intestine, producing a cobblestone appearance which
can be seen during endoscopic evaluation of the GI tract. Deep
ulcerations may penetrate through the bowel wall, and can produce sinus
tracts, fistulas (connections of the intestines to either the skin or
another organ such as the bladder), and abscesses in the abdomen. When
examining the intestines under a microscope, pathologists can often
identify characteristic findings, such as granulomas. Frequently the
surgeon can estimate the extent of gross involvement with CD at the time
of operation based on the degree of intestinal thickening or the extent
of fat surrounding the intestine (Fig 1).
NATURAL HISTORY
Although CD typically affects young adults, it has been recognized
increasingly in children, who often have severe symptoms of the disease.
In a review of more than 600 cases at the Mayo Clinic, 14% of patients
had symptoms before age 15 years. Early complaints include weight loss
(90%), abdominal pain (70%), diarrhea (67%), and fever (25%).
Extraintestinal manifestations including arthritis also may become
apparent early in the course of the disease. Due to the non-specific
nature of symptoms of CD, the d iagnosis is often quite delayed.
Children with CD usually have a chronic course, although some children have alternating remissions and relapses. Perianal ulcers, abscesses, and fistulas are frequent in children with CD of the colon, and perianal lesions are often the first manifestation of CD. Inflammation of the distal small intestine and proximal colon is the most common form of CD in children. Bloody diarrhea is common with CD of the colon.
Many extraintestinal manifestations occur with CD as well as ulcerative colitis, and include growth retardation, weight loss, joint pains, skin lesions (erythema nodosum, pyoderma gangrenosum), sclerosing cholangitis (liver disease), gallstones, kidney stones, uveitis (eye inflammation), and anemia (low blood count).
PHYSICAL FEATURES AND EVALUATION
Children with CD often have growth retardation and are underweight for
age. Perianal ulcers or sinuses are typical in children with colorectal
disease. The ulcers are often painful, and the perineal muscles may
become damaged.
Laboratory findings in children with CD usually indicate anemia (low blood count), elevation of the erythrocyte sedimentation rate, low level of albumin, and prolonged prothrombin time. Vitamin B 12 absorption may be abnormal if there is extensive distal ileal involvement. Stool cultures are usually negative for pathogenic bacteria and parasites.
Endoscopic evaluation of the upper and lower GI tracts is helpful to both make an initial diagnosis as well as assess for disease severity during the disease course. During the endoscopy, a gastroenterologist can make assessments of the GI tract both by visual exam as well as by biopsy of the intestinal wall.
Radiographic evaluation of the GI tract is essential for the management of CD. The bowel of CD has typical appearances when examined with either a computerized tomography (CT scan) or barium contrast study. These scans can reveal abnormalities of any site of the GI tract from the esophagus to the rectum, but they chiefly occur in the distal ileum and colon. The small bowel mucosa may appear thickened or distorted. Mucosal ulcerations, luminal narrowing, intestinal fistulas, sinus tracts, or intra-abdominal abscesses may be present. Long-term disease can result in strictures (obstructions), particularly in the small intestine.
CD can result in several intestinal complications, including obstruction, bleeding, perforation, or fistulas. Fistulas (connections between the intestine and other structures) should be suspected when a flare-up of symptoms is associated with the development of persistent and localized abdominal tenderness. The fistula often is located between the terminal ileum and right colon or sigmoid colon. Fistulas from the intestine to the bladder or urethra occasionally occur. Urinary tract infection in a patient with CD suggests a fistula from the intestine; and pneumaturia (air in the urine) or fecaluria (stool in the urine) suggests this complication.
Free perforation of the intestine into the abdomen is uncommon, but can happen during an acute exacerbation of CD. Rarely is steroid therapy implicated as a major factor in the development of a perforation. Adenocarcinoma of the intestine is an uncommon complication of CD, is less frequent than with chronic ulcerative colitis, and is rare in children. Nonetheless, the incidence of colorectal carcinoma is approximately 20 times greater in patients with CD than in the general population. Your gastroenterologist can help devise a long-term surveillance plan to screen for the development of cancer.
MEDICAL MANAGEMENT
Because CD is a chronic inflammatory condition, medical therapy does not
cure the disease, but is directed at reducing the inflammation and
providing symptomatic relief. Many children with mild CD can be managed
for long periods with relatively simple dietary modifications, including
high-calorie, high-protein, low-roughage foods. Milk is eliminated for
children with milk intolerance. In patients with fatty diarrhea, a
low-fat diet with added medium-chain triglycerides is recommended.
Between-meal nutritious snacks are encouraged to minimize any
nutritional risks.
Steroids (prednisone) are generally used early in the disease course for CD. Some studies have indicated that budesonide, a glucocorticosteroid almost as effective as prednisone, but without the steroid-associated side effects, may be equally effective. Steroids also are given to acutely ill hospitalized children to treat severe intestinal flares or extraintestinal manifestations, such as arthralgias and skin lesions unresponsive to other measures. Topical mesalamine (Rowasa) as an enema or suppository may be helpful in patients with anorectal CD. Sulfasalazine (Azulfidine) or mesalamine is often used as initial treatment for CD, although they tend to be less effective than for ulcerative colitis. Certain antibiotics, such as metronidazole and ciprofloxacin, have helped to relieve symptoms.
For the severely affected child, other immunosuppressant medications, such as azathioprine (Imuran), 6-mercaptopurine, or cyclosporine have been used as complimentary drugs. Cyclosporine suppresses cell-mediated immunity by interfering with interleukin-2 synthesis and release, and has been administered to patients unresponsive to other therapy. Long-term use of cyclosporine may produce serious side effects, such as kidney toxicity and increased susceptibility to infections. Tacrolimus (Prograf), another immunosuppressive drug, has been shown to have a beneficial effect in the management of chronic CD.
The observation of increased levels of tumor necrosis factor in CD has prompted the use of certain anti–tumor necrosis factor medications, such as infliximab (Remicade) and etanercept, particularly for the treatment of perianal disease or fistulas. These agents are generally reserved for children with CD unresponsive to other medications. The use of these agents involves several risks, and should be discussed with your child’s gastroenterologist.
Following surgery for CD, several medications can improve a child’s quality of life. Cholestyramine resin may be beneficial in treating diarrhea after ileal resection. Antispasmodics such as Lomotil may be helpful in alleviating diarrhea and abdominal cramps. Long-term narcotics should be avoided because of the addictive potential. Similarly, long-term use of nonsteroidal anti-inflammatory drugs should rarely be used because of the potential for exacerbating intestinal inflammation.
Hospitalization for children with CD is advised when symptoms become severe and there is progressive nutritional deficiency despite therapy on an ambulatory basis. Parenteral alimentation and bowel rest may produce improvement in many patients. The ready accessibility of a physician on whom the family can rely does much to help manage children with severe disease.
OPERATIVE THERAPY
Surgery is indicated for children with severe complications of CD,
including intestinal obstruction, growth retardation, medical
intractability, fistulas, perirectal fistulas or abscess, intestinal
bleeding, abdominal pain, or abdominal abscess. A judicious combination
of complete bowel rest with total parenteral nutrition has helped to
reduce the need for surgery. However, if these measures fail, surgery is
often required, and generally results in marked clinical improvement. In
many cases of chronic CD, surgical resection of affected intestine may
make it feasible to reduce the dose or discontinue medications.
The most common indication for surgery is intestinal obstruction or intractable pain, particularly for children with ileocecal disease. Resection of the grossly involved segment of the ileum with primary intestinal reconstruction is often performed for children with disease limited to a small portion of the small intestine. (Figure 1)
|
Figure 1. Segment of small intestine following resection for Chron’s disease associated with intestinal obstruction. Note severe intestinal inflammation, with thickened bowel loops and “creeping fat” from the mesentery over the bowel. |
Strictureplasty (reconstruction of a stricture without bowel resection) is reserved for patients with localized obstruction associated with extensive small bowel disease, although this technique is generally not required in children. In some cases, children will present for what is thought to be appendicitis, and are found at surgery to have CD of the distal ileum.
Protracted diarrhea for children with colonic disease can lead to the need for surgery. Fistulas from the bowel to either the skin or bladder can lead to the need for surgery in other children. Anal fistulas, abscesses, or fissures are often the earliest manifestation of colorectal CD. For these children, there is a limited role for surgery, but there is a great need for meticulous perianal care. Nitroglycerin 0.2% has been used locally with some success. Perianal disease may be simple, as with a fissure or ulcer, or more complex, with an abscess or a fistula that may be simple or more extensive and occasionally lead to a rectovaginal fistula. In cases of severe colorectal disease, either a diverting ileostomy (exteriorization of the small intestine) or proctocolectomy (removal of the colon and rectum) may be required. In other cases, drainage of perianal sinuses, fistulas, or abscesses can be required.
Parenteral nutrition has been used routinely in the perioperative period in children undergoing surgery. Other medical management, including corticosteroids, sulfasalazine, mesalamine, is continued during the preoperative period as well.
Inflammatory disease of the small intestine recurs in approximately 25-40% of children requiring surgery for ileocecal disease over a 5-10 year period. Recurrence is even more common in children with colorectal disease, and some may require further operation. In most patients, recurrent disease can be managed successfully without further surgery, using a combination of medications, bowel rest with partial or total parenteral nutrition, or antibiotics for varying periods.
In the cases of severe intraabdominal infection, it may not be safe to perform a primary bowel reconstruction, and an ileostomy or colostomy may be required. Skip areas of involved small intestine are usually not resected, unless they cause obstruction or other symptoms. Colectomy with ileosigmoid anastomosis may be performed in children with mild rectal involvement who do not have perianal sinuses or fistulas, although recurrent inflammation in the ileum requiring subsequent operation is common. The endorectal ileal pull-through procedure is generally not performed because of the high frequency of recurrent disease in the distal ileum, although this option should be discussed with your child’s surgeon. Enterovesicle fistulas are treated by resection of the diseased intestine with simultaneous closure of the bladder.
Risks of surgical resection for CD are similar to other abdominal procedures, and include a small risk of infection or bleeding, as well as postoperative bowel obstruction. Death following surgery for CD is extremely rare. Particular risks of resection of an inflammatory mass involving the distal ileum and cecum include damage to the right ureter (kidney drainage).
Operation for recurrent CD is necessary in approximately 30% of patients who have had a previous resection. The success rate of the second operation is more than 50%. Simple debridement or adequate drainage is treatment of perianal wounds and chronic infection in many patients.
SUMMARY
Despite the high incidence of recurrent disease after operation in children with CD, there is a gratifying response with relief of major symptoms in many patients. Most gain weight and are free of pain and extraintestinal symptoms for extended periods. When necessary, additional resections can be performed, and the patient’s condition is likely to improve again. The goal of a one-time curative operation for CD is rarely achievable, however. For most children with CD, treatment includes many years of medical therapy with occasional operations for the treatment of specific complications, with the goal of preserving as much length of intestine as feasible. Most of these patients experience fewer, milder symptoms and require less vigorous therapy as they progress into adulthood.
Articles and graphics adapted from O'Neill: Principles of Pediatric Surgery. © 2003, Elsevier.