Chronic intestinal pseudo-obstruction with pneumatosis cystoides intestinalis in a patient with systemic sclerosis

Abstract

Rationale: Chronic intestinal pseudo-obstruction (CIPO) and pneumatosis cystoides intestinalis (PCI) are rare abdominal diseases and the pathological mechanisms have not been fully elucidated. Systemic sclerosis (SSc), which is characterized by the progressive sclerotic changes of skin and internal organs, is a refractory collagen disease and is frequently associated with digestive disorders including CIPO. Patient concerns: A 68-year-old woman who has been well managed for SSc over the long term, who presented with abdominal fullness for the first time. Diagnoses: Abdominal X-ray and computed tomography (CT) images showed PCI with pneumoperitoneum findings. Based on the diagnosis of CIPO, we evaluated the intestinal peristalsis of the patient by using cine magnetic resonance imaging (MRI). Interventions: Oral medications of 15 g/d of Daikenchuto, 750 mg/d of Metronidazole and Sodium Picosulfate were started for improving the bowel peristaltic movement and decreasing intestinal gas production. Outcomes: A great improvement of CIPO and PCI by multidrug therapy without any surgical treatments for such an unusual case. Lessons: This case indicates that SSc can be accompanied with not only CIPO but also PCI as digestive disorders and that cine MRI, which is a definitely beneficial imaging modality, can intelligibly visualize the peristalsis of the intestines and lead to successful medical control by noninvasive treatment.

Pneumatosis cystoides intestinalis in dermatomyositis: a case series report and literature review

“…These changes can lead to decreased digestive function, leading to gastroparesis or constipation. Excessive bacterial growth and intestinal expansion can cause increased pressure within the lumen, which leads to gas entering the intestinal wall in this condition ( 23 , 24 ). The second reason is intestinal vasculitis.…”

Pneumatosis cystoides intestinalis revealed by an occlusive syndrome: A case report

“…When there is a suspicion of Pneumatosis cystoides intestinalis on imaging, a thorough clinical and paraclinical investigation should be carried out to assess the severity of the damage. This allows the early detection of acute ischemic lesions which are true medical and surgical emergencies [1,2,[5][6][7][8]. www.ijcripathology.com…”

“…A first theory, called "mechanical," is based on the principle that intraluminal hyperpressure secondary to digestive obstruction will cause submucosal dissection from the digestive lumen. The mechanical theory largely explains the association of digestive disorders with cystic fibrosis of the intestine [1][2][3][4][5][6][7][8].…”

Management of pneumatosis cystoides intestinalis with pneumoperitoneum: 5-years systematic review

“…Chemotherapeutics and antiangiogenic monoclonal antibodies appear to increase the intestinal capillary permeability, allowing the passage of air from the lumen into the intestinal wall 35,36 . Chronic constipation may promote bacterial overgrowth, leading to increased intraluminal hydrogen production and resulting in leakage into the intestinal wall, according to the biomechanical theory 3,37 . Conversely, alpha-glucosidase inhibitors reduce carbohydrate absorption, which will be fermented by the gut microbiota, resulting in gas production and peristaltic dysfunction.…”