1/4. pneumatosis cystoides intestinalis, four cases of a rare disease.pneumatosis cystoides intestinalis (PCI) is a disease in which small gas-filled cysts appear in the intestinal wall. Four cases presented here demonstrate the diversity of the associated diseases. In two of the patients constipation probably played a role; in the third patient decreased colonic motility, elevated intestinal pressure and increased mucosal permeability in the context of enteritis treated with codeine was the underlying problem; in the fourth high protein feeding and bowel ischaemia was diagnosed. Various aetiologies are presented in the literature. There is no specific history and physical or laboratory findings do not help to diagnose PCI. Plain abdominal film, ultrasound, computer tomography, magnetic resonance imaging, barium contrast studies and/or endoscopy may be necessary for diagnosis. Therapy is based on enhancing partial oxygen pressure in the bowel wall. PCI usually runs a benign course.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/4. pneumatosis cystoides intestinalis with systemic sclerosis, limited type resulting in a poor prognosis.pneumatosis cystoides intestinalis (PCI) is a rare disease characterized by the presence of multilocular intramural clusters of gas in the alimentary tract and has been considered to have a favorable response to conservative treatment. We describe the first case of limited type of systemic sclerosis (SSc) with PCI. A 74-year-old Japanese woman presented with a 4-month history of an unhealed cutaneous ulcer on the right third finger, along with sclerodactyly of bilateral hands. Proximal skin sclerosis was absent. The patient reported acute abdominal pain, and a diagnosis of PCI was established on plain radiography. The patient died of multiple organ failure 5 months after the development of PCI. PCI is rarely complicated with SSc, and all cases previously reported were associated with diffuse SSc. Because PCI is one of the poor prognostic factors of SSc, we should recognize the presence of this condition even in patients with limited cutaneous involvement.- - - - - - - - - - ranking = 0.25keywords = rare disease (Clic here for more details about this article) |
3/4. Pneumo-retroperitoneum associated with pneumatosis cystoides intestinals.pneumatosis cystoides intestinalis, a rare disease characterised by gas cysts involving portions of the intestinal tract, is obscure in its etiology and intriguing in its presentation. Amongst several theories a new concept suggest that retroperitoneal gas is a probable source of origin. Experimental studies in animals have demonstrated that air can track down the mediastinum into the retroperitoneum and thereby into the gut wall from an alveolar rupture. A case of pneumo-retroperitoneum in a patient with pneumatosis cystoides intestinalis is now reported.- - - - - - - - - - ranking = 0.25keywords = rare disease (Clic here for more details about this article) |
4/4. pneumatosis cystoides intestinalis confined to the small intestine treated with hyperbaric oxygen.pneumatosis cystoides intestinalis is a rare disease characterized by the presence of multiple intramural gas-filled cysts in the gastrointestinal tract. The etiology remains unknown, but the disease can present with profound disturbances of bowel function. We report the successful management of pneumatosis cystoides intestinalis of the small intestine with the use of hyperbaric oxygen.- - - - - - - - - - ranking = 0.25keywords = rare disease (Clic here for more details about this article) |