Chronic intestinal pseudo-obstruction due to lymphocytic leiomyositis: is there a place for immunomodulatory therapy?

There is a rare cause of chronic intestinal pseudo-obstruction (CIPO) characterised by a lymphocytic infiltrate in the muscle of the intestine, which is called idiopathic lymphocytic leiomyositis. Few cases have been reported and prognosis is very poor. We present a case with a comparatively benign evolution, showing good response to immunosuppressive therapy. The patient was a healthy 16 year old female who presented with a crisis of postprandial bloating followed by diarrhoea and vomiting. During the following months she lost 10 kg in weight and any attempt at oral feeding resulted in severe abdominal distension and vomiting. Therefore, total parenteral nutrition was finally prescribed. Plain abdominal film and small bowel follow through indicated huge dilatation of the small intestine with air fluid levels. Gastroscopy and colonoscopy were normal, as were mucosal biopsies. Human immunodeficiency virus, hepatitis …