Belly dancer's dyskinesia or diaphragmatic flutter (DF) is a rare condition characterized by repetitive involuntary contractions of the diaphragm. Also known as diaphragmatic myoclonus (DM), this disorder can manifest with involuntary movement of the abdominal wall and contraction of accessory respiratory muscles or respiratory myoclonus. Because of its variable presentation, diagnosis can often be difficult and delayed. This phenomenon is thought to be secondary to abnormal excitation of the phrenic nerve, via the central nervous system or along the nerve. Another possible mechanism is the irritation of the diaphragm itself. Diagnosis can be made with ultrasound, thoracic videofluoroscopy, or electromyography (EMG). Different pharmacologic and surgical therapies have been used in the past, but overall, there are no specific guidelines regarding treatment. In this report, we present a case of DF in a young female patient.
INTRODUCTION: Gangliocytic paragangliomas are a rare subclass of neuroendocrine tumors that usually appear in the periampullary region of duodenum. They are generally benign, although may have potential for metastases depending on size and extension. They most commonly present with bleeding or abdominal pain, but cases with obstructive jaundice and pancreatitis have also been reported. They are often misdiagnosed as GISTs or other neoplasms, but with histologic examination, an accurate diagnosis can be reached. However, intraoperative diagnosis with FNA and frozen section analysis are often inaccurate and could affect surgical decision. CASE DESCRIPTION/METHODS: 62-year-old male with past medical history of diverticulitis, internal hemorrhoids, and iron deficiency anemia presented with syncope and gastrointestinal bleeding. During evaluation with colonoscopy, a mass was found in the rectum, which was later confirmed to be an adenocarcinoma. An FDG-avid duodenal segment was incidentally found while undergoing workup with PET-scan. He had neoadjuvant chemoradiation, with resection of rectal cancer and temporal ileostomy. Biopsy of duodenum with endoscopic ultrasound revealed normal duodenal mucosa on 2 separate occasions. Ultimately, the duodenal tumor was excised via exploratory laparotomy. Intraoperative frozen biopsy of mass revealed a GIST and a Whipple procedure was performed due to proximity to ampulla. Final pathology report revealed a gangliocytic paraganglioma, after staining positive with synaptophysin (as well as S-100 and neuron specific enolase) and negative for antibody to CD117, which is a sensitive marker for GISTs. DISCUSSION: Duodenal gangliocytic paragangliomas remain to be rare entities. There is yet no clear consensus in regards to appropriate workup, treatment or follow-up, especially in patients with metastases or concurrent adenocarcinoma. It is uncertain if coexistence with a rectal adenocarcinoma (as seen in this case) was coincidental or due to similar pathogenesis. Diagnosis with FNA and frozen section biopsies can be useful, but are often inaccurate. The success largely relies on the operator and clinical suspicion, but it is especially hard when there are no symptoms or relevant history. The recommended management is margin-free resection endoscopically, with local surgical excision or Whipple procedure. Studies have shown that some molecular markers can predict malignant potential. Yet, whether they are useful for management or surveillance has not been determined.
INTRODUCTION: There is limited evidence available regarding the trends of hospitalization and in-hospital outcomes amongst various gastrointestinal malignancies. Our study measured the trends of hospitalization and the difference related to in-hospital outcomes, such as inpatient length of stay (LOS), cost and mortality in patients with various gastrointestinal malignancies. METHODS: The National Inpatient Sample (NIS) dataset was queried from 2009 to 2014 to identify all patients with a multilevel diagnosis of gastrointestinal neoplasms including cancer of the esophagus, cancer of the stomach, cancer of the colon, cancer of rectum/anus, cancer of the liver and intrahepatic bile duct and cancer of the pancreas. Categorical and continuous variables were tested using Chi-square test and Student t-test respectively. RESULTS: We identified a total of 1,995,281 patients with a diagnosis of neoplasm from 2009 to 2014. Among them, 15,276 (0.8%) had cancer of the esophagus, 28,018 (1.4%) had cancer of the stomach, 114,917 (5.8%) had cancer of the colon, 49,690 (2.5%) had cancer of the rectum and anus, 26,565 (1.3%) had cancer of liver and intrahepatic bile duct, 44,749 (2.2%) had cancer of the pancreas. We found that colon cancer had the highest inpatient mortality rate of 5.8% while esophageal cancer had the lowest at 0.8%. The mean LOS for cancer of the stomach was the longest (≈9.49 days) while the cost of care was highest for esophageal cancers (26752.84$). More importantly, the trend of hospitalization among all the major subtypes of gastrointestinal malignancies was significantly unchanged over the period of 5 years. CONCLUSION: Gastrointestinal malignancies in the United States represent a significant burden of disease. Our study provides further evidence that there is a significant burden of cost, hospitalization, as well as mortality in this cohort of patients. Given the alarming increase in the incidence of gastrointestinal malignancies and related deaths, such data may have profound implications regarding future demographics to focus research, screening or preventative measures accordingly.
INTRODUCTION: Inclusion body myositis (IBM) is a chronic progressive myopathy characterized by dysphagia in 10% to 73% of patients with one-year mortality of 31% in patients with symptoms of dysphagia. We present a case of a patient with IBM esophagitis causing severe weight loss and complete loss of ability to swallow with a discussion of potential management modalities. CASE DESCRIPTION/METHODS: The patient was a 77-year-old male with a past medical history of inclusion body myositis diagnosed at age of 57 and muscular dystrophy who presented with progressive dysphagia for several years and a significant weight loss of 40 pounds over last 8 months. He initially was able to tolerate small bites, however, he was not able to tolerate solids and his diet was modified to a mechanical soft diet. Prior to the hospital admission, the patient had an outpatient modified barium study (MBS) which revealed penetration and aspiration with thin and nectar thick liquids. An endoscopy was attempted for percutaneous gastrostomy tube placement but was unsuccessful due to the inability to pass through the upper esophageal sphincter. He subsequently underwent a fluoroscopy-guided percutaneous gastrostomy tube placement by an interventional radiologist. DISCUSSION: IBM is a chronic progressive inflammatory myopathy. Associated dysphagia can result in nutritional deficits, weight loss, aspiration pneumonia and decreased quality of life. The underlying mechanism for dysphagia is related to inadequate pharyngeal contraction, poor relaxation of the cricopharyngeal muscle, and reduced hyolaryngeal elevation. The management of dysphagia in IBM is challenging due to its steroid resistance nature. Multiple treatment modalities are used to treat dysphagia secondary to IBM, including conservative measures, intravenous immunoglobulin therapy, and balloon. Surgical intervention with cricopharyngeal myotomy has been suggested when conservative therapies fail or when severe weight loss and malnutrition are apparent. The later appears to be effective especially when hypertonicity of the cricopharyngeal muscle and impaired relaxation are present. A retrospective study of 26 patients with IBM has shown higher mortality in patients who required PEG placement compared to the ones who underwent cricopharyngeal myotomy. Per-oral endoscopic myotomy (POEM) showed success for spastic esophageal disorders such as diffuse esophageal spasm. At the meantime, no studies have implied the procedure for IBM esophagitis giving a great room for future studies.
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