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We describe a female patient with Crohn's disease who presented with longstanding gingival swelling. She went into remission after surgery, but had labial involvement while in remission. A review of the literature is presented with special reference to the clinical characteristics of orofacial Crohn's disease and Melkersson-Rosenthal syndrome, which can mimic such presentation of Crohn's disease.

Two patients with Crohn's disease who developed squamous cell carcinoma in chronic sinus tracts are reported. Because nonhealing perineal sinus tracts or fistulas are relatively common in patients with Crohn's disease, the diagnosis of carcinoma may not be considered until the malignancy is advanced. Because of the potential for a surgical and oncologic "cure," early diagnosis may improve the prognosis. Malignancy should be considered in patients with nonhealing perineal sinuses who develop severe continuous perianal pain. Diagnosis is best made by needle aspiration cytology and sinus tract biopsy. Treatment should include wide excision of the sinus tract, surrounding soft tissue, and lymph nodes, followed by chemotherapy and radiotherapy in selected cases.

Sixty-six young patients (average age, 17.2 years) underwent intestinal operations for Crohn's disease during a 14-year period at the UCLA Hospital. Nine had undergone previous appendectomy for suspected appendicitis, and in seven subsequent enteric fistulae and/or obstruction involving the cecum developed. None of the appendices were acutely inflamed. During the same period, a total of 125 children with Crohn's disease underwent medical or surgical care, and in none did acute appendicitis develop. Of the 66 patients under 22 years old who required intestinal resection, 60 had removal of the cecum. In a child with ileal Crohn's disease, rarely should a normal appendix be removed because of the high likelihood of complications and the low incidence of subsequent appendicitis.

During the past 3 yr, 17 patients with chronic ulcerative colitis and 6 with Crohn's disease who had severe rectal and colonic involvement underwent excision of the rectal mucosa without removal of the rectal muscle in combination with total colectomy and cutaneous ileostomy as a 1- or 2-stage procedure. This operative technique has cured each of the patients of their primary colonic and rectal disease and has obviated many of the unpleasant complications that often occur after total proctectomy, such as impotence, prolonged perineal drainage, and bladder dysfunction. The operation has the further advantages of lower operative blood loss, shorter operative time, and earlier safe ambulation. On the basis of the favorable experience with mucosal proctectomy, sphincterotomy, and perineal drainage in 23 patients, none of whom experienced major complications, we believe that this operation warrants further clinical trial in patients with inflammatory bowel disease involving the rectum, which is refractory to medical therapy. Total proctectomy might eventually find scant application in patients with inflammatory bowel disease.

Celiac sprue and Crohn's disease have very rarely been documented in the same patient. We report a case involving an adolescent male with known Crohn's disease who had extreme wasting, growth retardation, intractable diarrhea, and delay in development of secondary sexual characteristics. Peroral jejunal biopsy showed a nonspecific flat mucosal lesion compatible with celiac sprue. An 8.0-kg weight increase occurred within 4 weeks of institution of a gluten-free diet. Jejunal rebiopsy documented significant improvement with a mild to moderate villous abnormality. After 2 years on a gluten-free diet, a rebiopsy of the jejunum documented only a mild villous abnormality.

Seventeen pediatric patients, ages 9.25--20.5 yr, were placed on a program of home parenteral nutrition (HPN) for severe, symptomatic Crohn's disease. Prior therapy with sulfasalazine in 14, adrenocorticosteroids in 12, inpatient total parenteral nutrition in 7, and/or surgical resections in 6 failed to suppress disease activity. Remission was attained in 12 of the 17 after one course of HPN alone. Four patients had surgical procedures and 1 required steroids in addition to HPN. Remissions have been maintained in 4 of those 12 for a mean duration of 315 days after discontinuation of HPN. Of the 8 who relapsed after a mean duration of 68 days, second courses of HPN were undertaken in 7 and third courses in 2. All 17 had a marked improvement in disease symptoms while receiving HPN in addition to gaining weight sufficient to place them at a higher percentile on standard growth charts. Ten patients demonstrated "catch-up" growth and 4 others increased their height appropriately. A chromium 51-labeled albumin stool collection of greater than 1% in 5 of 7 patients at the completion of a HPN course correlated with relapse within 4 mo. Serial radiographic contrast studies and erythrocyte sedimentation rates were not predictive of prolonged remissions. Home parenteral nutrition complications were minimal, with only one episode of sepsis per 5.8 catheter experience years. It is concluded that HPN is a safe and effective means of inducing remissions and providing optimal nutritional support in pediatric patients with severe Crohn's disease. Patients with less than adequate response to standard medical management should be considered candidates for this therapeutic modality.

Twin brothers whose monozygosity was demonstrated by analysis of dermatoglyphic patterns, chromosome banding, HLA typing, and gene markers developed Crohn's disease within 8 mo of each other. Both had involvement of the terminal ileum, colon, and rectum, as well as arthritis. However, one had severe esophagitis and recurrent pancreatitis, and the other had severe arthritis and oral mucosal involvement. Both twins had manifestations controlled by parenteral nutrition. This report describes the similarities and differences in the complicated course of Crohn's disease in identical twins and demonstrates that while the development of Crohn's disease may be genetically influenced the site of gastrointestinal involvement is affected by other factors. This experience does not support previously held concepts of the role of genetics in the clinical manifestations of Crohn's disease.

Ulcerative colitis differs from Crohn's colitis in several ways. In ulcerative colitis the disease is limited to the mucosa and, occasionally, the submucosa; Crohn's colitis may involve all layers of the large intestine. Ulcerative colitis almost always begins in the rectum, is diffuse, and spreads proximally. Crohn's colitis may spare the rectum and has a patchy distribution. Perianal fistulas and ulcers are rare in ulcerative colitis but are common in Crohn's colitis. Granulomas and giant cells are not found in ulcerative colitis but are seen in the majority of patients with Crohn's colitis. Colonic and extraintestinal symptoms in the two illnesses may be indistinguishable but growth failure is far more severe in Crohn's colitis and may precede intestinal symptoms by months to years. Cancer of the colon is a risk in patients with either ulcerative or Crohn's colitis but is far more common in the former. It is important to distinguish between ulcerative colitis and Crohn's colitis because response to treatment and prognosis are different. Although neither condition can be cured by medical management, patients with ulcerative colitis may respond more frequently. Unfortunately, in the pediatric age range most cases of ulcerative and Crohn's colitis may be classified as moderate to severe. Fortunately for patients with ulcerative colitis, total colectomy with ileostomy will result in cure of illness. Patients with Crohn's colitis who require surgery may obtain remission of symptoms, but the disease is likely to recur in the small intestine.