Dermatomyositis- Related Intestinal Dysmotility

Dermatomyositis (DM) is an inflammatory myopathy characterized by proximal muscle weakness and pathognomonic skin lesions. A 69-year-old woman with a recent diagnosis of DM one month prior, treated with corticosteroids and immunomodulators, presented to our inpatient rehabilitation with worsening dysphagia and constipation. At the time of our evaluation, physical examination was notable for erythematous papules over the MCPs, PIPs, elbows, and knees as well as a violaceous rash on the face. Muscle strength was diminished bilaterally with proximal distribution being affected greater than distal. Laboratory studies were notable for CK level 31 IU/ L, ANA by immunofluorescence of 1:80, and aldolase 4 u/L. The eleven-antibody myositis panel was negative. Skeletal muscle biopsy of the left thigh showed partially treated acquired inflammatory myopathy with perifascicular atrophy. During hospitalization, she was found to have pulmonary embolism. She received enoxaparin 1 mg/kg subcutaneous BID. Soon after, she developed rectal bleeding. Colonoscopy showed a stercoral ulcer caused by chronic constipation. While dysphagia is common, being present in 25-50% of patients with DM, lower gastrointestinal problems involving the small and large intestine are rare and typically present as a late manifestation of the disease. Decreased peristalsis in the large colon can lead to constipation, impaction, and subsequent mucosal ulceration, and pressure necrosis induced by fecaloma formation. Although rare, our case highlights the importance of recognizing gastrointestinal complications that dermatomyositis can cause and the effects that those complications have on morbidity and mortality.

Gastroschisis Complicated by Colonic Atresia

Introduction: Gastroschisis with colonic atresia is a rare association. Due to its rarity and variation in presentation, no standardized surgical treatment option exists. Complicated gastroschisis is associated with a higher morbidity and mortality than intestinal atresia or gastroschisis alone. Methods: This is a case report of a neonate with congenital gastroschisis. On day of life 1, a silo was placed. On day of life 4, the upper portion of silo contents appeared more congested with dusky discoloration and the patient was oliguric. She was taken to the operating room for exploration. Results: The patient required resection of the terminal ileum and an atretic portion of the cecum due to necrosis. Four days later, primary closure of the gastroschisis defect was performed with creation of an end ileostomy. Elective ileostomy takedown was performed 5 months later. She returned to the operating room for anastomotic revision and gastrostomy tube placement for intestinal dysmotility and prolonged ileus. Tube feedings were discontinued 3 months post-operatively. Conclusions: Gastroschisis with intestinal atresia is associated with increased hospital length of stay, longer duration of parenteral nutrition, more severe intestinal dysfunction, increased surgical complications, and higher mortality than gastroschisis or intestinal atresia alone. With associated colonic atresia, loss of a significant length of bowel due to necrosis, including the ileocecal region, is almost unavoidable. An already compromised blood supply to the atretic bowel is further impaired by a tight abdominal wall defect and postnatal increases in gravitational force. Complicated gastroschisis remains a complex surgical challenge requiring further discussion.

Assessment of Whole Gut Motility in Adolescents Using the Wireless Motility Capsule Test

Functional gastrointestinal (GI) disorders are often associated with intestinal dysmotility representing a diagnostic challenge. A relatively new method is the wireless motility capsule (WMC) test, which continuously measures pH, pressure, temperature and regional transit times as it passes through the GI tract. In adults, the WMC test was approved for use in the diagnosis of gastroparesis and constipation by assessing GI transit and contractility. We performed the WMC test in nine adolescent patients aged 12–17 years with functional GI symptoms from July 2017 until February 2019. Abnormal transit times were detected in four patients. Three patients showed abnormal transit times of the upper GI tract, in two cases contractility analysis revealed gastroparesis, in one patient abnormal colonic transit was detected. Conclusion: The WMC test is a minimally invasive procedure with potential to expand future diagnostic opportunities for paediatric patients with functional GI disorders and suspected motility disturbances.

The Alpha-Lipoic Acid Improves Survival and Prevents Irinotecan-Induced Inflammation and Intestinal Dysmotility in Mice

Irinotecan, an anticancer drug, induces diarrhea and intestinal inflammation, resulting in an increase in the cost of care and in treatment delays. In this study, we investigated whether alpha-lipoic acid (α-LA) could improve irinotecan-mediated intestinal inflammation, diarrhea and dysmotility. Intestinal mucositis was induced by irinotecan injection (75 mg/kg, i.p., for 4 days) in Swiss mice. α-LA (50, 100 or 200 mg/kg, gavage) was administered daily 1 h before the injection of irinotecan. Duodenum tissues were obtained for inflammation and proliferation analysis. The outcomes: diarrhea, intestinal dysmotility, weight body loss and survival were evaluated. Compared with the control condition, irinotecan diminished (p < 0.05) intestinal villus height, caused a loss of crypt integrity and intense inflammatory cell infiltration, increased myeloperoxidase (MPO), IL-6 and IL-1β levels and decreased reduced glutathione (GSH) levels in duodenum segments and increased gastric retention and decreased liquid retention in the medial intestinal segment, resulting in increased intestinal transit, severe diarrhea and reduced survival (approximately 72%). Furthermore, α-LA (200 mg/kg) pretreatment ameliorated (p < 0.05) these irinotecan-induced effects. Our findings show that α-LA reduced irinotecan-induced inflammation, intestinal dysmotility and diarrhea, resulting in improved survival. α-LA may be a useful therapeutic agent for the treatment of gut dysmotility in patients with intestinal mucositis associated with irinotecan treatment.

The management of adult patients with severe chronic small intestinal dysmotility

Adult patients with severe chronic small intestinal dysmotility are not uncommon and can be difficult to manage. This guideline gives an outline of how to make the diagnosis. It discusses factors which contribute to or cause a picture of severe chronic intestinal dysmotility (eg, obstruction, functional gastrointestinal disorders, drugs, psychosocial issues and malnutrition). It gives management guidelines for patients with an enteric myopathy or neuropathy including the use of enteral and parenteral nutrition.

Small intestinal dysmotility in cirrhotic patients: correlation with severity of liver disease and cirrhosis-associated complications

Introduction Altered small intestinal motility has been observed in various manometry studies in patients with cirrhosis. Since small bowel manometry is available only in a few centers, interpretation of dysmotility in cirrhosis is controversial. Patients and Methods In this study, both fasting and postprandial manometric tracings of 24-hour antroduodenojejunal manometries were analyzed using both visual analysis and computer-aided analysis. Results In 34 patients (83 %), the mean migrating motor complex (MMC) cycle length was different compared with healthy controls. Phase II was prolonged in 27 patients (66 %), while phase I showed a reduced duration in 23 (56 %) and in phase III in 13 individuals (32 %). We also observed special motor patterns, e. g., migrating clustered contractions (MCCs) or retrograde clustered contractions (RCCs), which were present during fasting (69 %) and postprandial (92 %) motility, while none of the healthy controls showed any special motor patterns. Special motor patterns showed a significant correlation with the severity of cirrhosis (Child-Score; p > 0.05) and the existence of ascites (p < 0.05). Discussion This study in a large cohort of patients with cirrhosis by using 24-hour, solid state portable manometry showed in most individuals disturbances of cyclic fasting motility. Special motor patterns like RCCs during fasting and postprandial motility could be observed exclusively in the cirrhosis group, showing a significant correlation with severity of cirrhosis and the occurence of associated complications.