Neuromuscular and vascular hamartoma as an unusual cause of small bowel obstruction.

INTRODUCTION: Neuromuscular and vascular hamartoma (NMVH) is a rare, controversial lesion of the intestine, with only 23 cases reported in the English literature since its initial description in 1982. PRESENTATION OF CASE: A 59year old female suffering from longstanding Crohn's disease with chronic stricture presented with symptoms of small bowel obstruction. Contrast studies demonstrated massive dilatation of the proximal small bowel. Laparotomy identified a 5cm long stenotic segment of ileum, with grossly distended jejunum and ileum proximally. Pathology determined the stricture's aetiology as a neuromuscular and vascular hamartoma of the small intestine. DISCUSSION: NMVH is a benign lesion of hamartomatous origin. Its very existence is questionable due to histological similarities with several reactive pathologies, such as Crohn's and diaphragm diseases. CONCLUSION: NMVH could be confused with a spectrum of chronic inflammatory bowel conditions, but this report establishes it as a distinct cause of chronic bowel obstruction.

Early studies on the safety and efficacy of thalidomide for symptomatic inflammatory bowel disease.

BACKGROUND AND AIM: Thalidomide is clinically effective in the treatment of graft versus host disease in bone marrow transplantation and aphthous ulceration in HIV infection. It appears to exert a selective effect on tumor necrosis factor-alpha (TNF-alpha) production. Tumor necrosis factor-alpha is implicated in the pathogenesis of inflammatory bowel disease (IBD). The aim of this study was to assess the efficacy and safety of thalidomide in symptomatic IBD. METHODS: Eleven patients (nine males, mean age 33 years, range 20-77 years) with chronic inflammatory bowel disease (six Crohn's disease (CD), four ulcerative colitis (UC), one indeterminate colitis (IC)) who were symptomatic despite standard medical therapy were administered a daily dose of thalidomide for 12 weeks in an open-labeled protocol. Their response was assessed by using clinical, colonoscopic, histological, and immunological methods. RESULTS: Two patients withdrew at 3 weeks because of mood disturbances. Of the remaining nine patients, eight (five CD, two UC and one IC) had a marked clinical response, while one patient with CD had no response. The mean stool frequency decreased from 4.3 to 2.3 per day (P = 0.0012), and the stool consistency increased from 2.1 to 1.2 (P = 0.02). The mean Crohn's Disease Activity Index decreased from 117 to 48 (P = 0.0008). Endoscopic inflammatory and histological grade, C-reactive protein and erythrocyte sedimentation rate (ESR) all decreased significantly (P = 0.011, P = 0.03, P = 0.023 and P = 0.044, respectively). However, the serum TNF-alpha levels did not change. Side-effects included mild sedation, xerostomia and skin dryness in all, constipation in three, and minor abnormalities in nerve conduction in one patient. CONCLUSION: These data strongly suggest that thalidomide is an effective short-term treatment for symptomatic IBD.