Coronary Artery Dissection and Myocarditis Caused by Eosinophilic Granulomatosis with Polyangiitis (EGPA): A Case Report.

Eosinophilic granulomatosis with polyangiitis (EGPA) also referred to as Churg-Strauss syndrome is a rare vasculitis of the small to medium vessels. We present a rare case of acute coronary artery dissection brought on by EGPA, which generally has a poor prognosis. A 41-year-old male with history of bronchial asthma presented to the emergency room with a 2-week history of dyspnea, cough with clear phlegm, and fever. For the past eight months he had experienced episodes with similar symptoms relieved by steroids. CT chest showed bilateral upper lobe patchy opacities with extensive workup for infectious etiology being negative. He had peripheral eosinophilia with sinusitis. He had acute coronary syndrome and Coronary angiogram showed Right coronary artery dissection. After making a diagnosis of EGPA based on American college of Rheumatology criteria, he was successfully treated with high dose immunosuppression. Coronary artery dissection is a fatal and uncommon complication of EGPA which is usually diagnosed postmortem. Early recognition of this condition ante mortem and aggressive treatment can be lifesaving as demonstrated in our case.

Duodenal Adenocarcinoma in a patient with Lynch Syndrome. A Case Report and Facts Related to Small Intestine Cancer.

Diagnosing small bowel cancer has been challenging due to its unusual presentation and inaccessibility on endoscopy. A 41-year-old male with a history of irritable bowel syndrome underwent esophagogastroduodenoscopy (EGD) for worsening fatigue and lightheadedness despite iron supplements therapy for low hemoglobin. Initial upper endoscopy showed esophagitis and non-bleeding duodenal bulb ulcer with exudate. Endoscopic ultrasound (EUS) with fine-needle aspiration was done due to persistent concern of malignancy and demonstrated moderately differentiated adenocarcinoma in the second portion of the duodenum. Endoscopic ultrasound with fine-needle aspiration may be a superior approach to diagnosing duodenal carcinoma than EGD alone. Small bowel cancer can be a part of the tumor spectrum of Lynch syndrome. Duodenal adenocarcinomas present at a late stage and portend a poor prognosis. We present a case of duodenal adenocarcinoma in an otherwise healthy individual emphasizing the importance of malignancy in the differential and genetic counseling in individuals with the family risk factor.

Acquiring the Diagnosis of an Acquired Tracheoesophageal Fistula with EtCO2: A Case Discussion with Review of the Literature.

Acquired tracheoesophageal fistula (TEF) is a rare complication of esophageal or lung cancer. A 57-year-old male presented with complaints of vomiting, cough, 20 lb weight loss, and progressive dysphagia. Early laryngoscopy and CT chest showed a normal pharynx with an irregular thickness of the thoracic esophagus. The upper gastrointestinal endoscopy (UGIE) and upper endoscopic ultrasound (EUS) revealed a hypoechoic mass evolving as complete obstruction. During the procedure, minimal CO2 was used for insufflation; however, when attempts were made to traverse the obstruction, capnography revealed an end-tidal CO2 (EtCO2) estimating 90 mmHg indicating possible TEF. This case depicts the use of capnography during UGIE in diagnosing an acquired TEF.

Esophageal Submucosal Injection of Capsaicin but Not Acid Induces Symptoms in Normal Subjects.

BACKGROUND/AIMS: Transient receptor potential vanilloid-1 (TRPV1) is a candidate for mediating acid-induced symptoms in the esophagus. We conducted studies to determine if the presence of acid in the mucosa/submucosa and direct activation of TRPV1 by capsaicin elicited symptoms in normal healthy subjects. We also studied the presence of TRPV1 receptors in the esophagus. METHODS: Unsedated endoscopy was performed on healthy subjects with no symptoms. Using a sclerotherapy needle, normal saline (pH 2.0-7.5) was injected into the mucosa/submucosa, 5 cm above the Z line. In a separate group of healthy subjects, injection of capsaicin and vehicle was also studied. Quality of symptoms was reported using the McGill Pain Questionnaire, and symptom intensity using the visual analogue scale (VAS). Immunohistochemistry was performed on 8 surgical esophagus specimens using TRPV1 antibody. RESULTS: Acid injection either did not elicit or elicited mild symptoms in subjects at all pH solutions. Capsaicin but not the vehicle elicited severe heartburn/chest pain in all subjects. Mean VAS for capsaicin was 91 ± 3 and symptoms lasted for 25 ± 1 minutes. Immunohistochemistry revealed a linear TRPV1 staining pattern between the epithelial layer and the submucosa that extended into the papillae. Eighty-five percent of papillae stained positive for TRPV1 with a mean 1.1 positive papillae per high-powered field. CONCLUSIONS: The mechanism of acid-induced heartburn and chest pain is not the simple interaction of hydrogen ions with afferents located in the esophageal mucosa and submucosa. TRPV1 receptors are present in the lamina propria and their activation induces heartburn and chest pain.

Reversal of asynchrony between circular and longitudinal muscle contraction in nutcracker esophagus by atropine.

BACKGROUND & AIMS: Patients with high-amplitude esophageal contractions (nutcracker esophagus [NCE]) show asynchrony of circular muscle (CM) and longitudinal muscle (LM) contraction during peristalsis. The goal of our study was to determine if this asynchrony is related to an increase in the cholinergic receptor activity. METHODS: High-frequency intraluminal ultrasound images and pressures of the esophagus were recorded simultaneously in 10 normal subjects and 10 patients with NCE. Recordings were obtained at 2 cm above the lower esophageal sphincter under 2 study conditions in normal subjects (before and after 80 microgm/kg of edrophonium), and under 3 study conditions in the NCE patients (control, 5 microgm and 10 microgm/kg of atropine). RESULTS: In normal subjects, edrophonium induced an increase in the CM and LM contraction amplitude, an increase in the contraction duration, and asynchrony of LM and CM contraction during peristalsis. On the other hand, increased contraction amplitude, duration, and asynchrony of LM and CM contraction observed at the baseline in the NCE patients were reversed by atropine in a dose-dependent fashion. CONCLUSIONS: These data prove that the esophageal motor abnormalities in patients with nutcracker esophagus, including asynchrony of CM and LM contraction, are related to a hypercholinergic state.

A unique longitudinal muscle contraction pattern associated with transient lower esophageal sphincter relaxation.

BACKGROUND & AIMS: Contraction of the longitudinal muscle of the esophagus may play a role in the relaxation and opening of the lower esophageal sphincter (LES). The goal of our study was to determine the pattern and precise temporal correlation between local longitudinal muscle contraction (LMC) of the esophagus during peristalsis and transient LES relaxation (TLESR). METHODS: Esophageal pressures and high-frequency intraluminal ultrasound imaging of the esophagus were recorded in 24 healthy subjects during swallow-induced peristalsis and spontaneous TLESR. Intraluminal multiple impedance recordings were obtained to determine the relationship between "common cavity pressure" and gastroesophageal reflux (GER). RESULTS: During swallow-induced peristalsis, there is simultaneous contraction of circular and longitudinal muscles of the esophagus. On the other hand, TLESR is associated with a distinct pattern of LMC in the esophagus that has the following characteristics: (1) it is restricted to the distal esophagus; (2) it begins before the onset of TLESR and spreads in a retrograde manner; (3) it is generally stronger than the swallow-induced contraction; and (4) it is sustained during the entire duration of TLESR. The increase in esophageal pressure during TLESR is temporally correlated with the contraction of the LM of the distal esophagus, rather than with the impedance recorded GER. CONCLUSIONS: We propose that the LMC of the distal esophagus may play an important role in the relaxation of LES and induction of GER.

Cholinergic stimulation induces asynchrony between the circular and longitudinal muscle contraction during esophageal peristalsis.

In healthy subjects, a close temporal correlation exists between contractions of the circular muscle (CM) and longitudinal muscle (LM) layers of the esophagus. Patients with nutcracker esophagus show disassociation between the peak of contractions of the CM and LM layers and the peak of contraction 1-3 s apart (Jung HY, Puckett JL, Bhalla V, Rojas-Feria M, Bhargava V, Liu J, Mittal RK. Gastroenterology 128: 1179-1186, 2005). The purpose of the present study was to evaluate the effect of acetylcholinesterase inhibitor (edrophonium) and acetylcholine receptor antagonist (atropine) on human esophageal peristalsis in normal subjects. High-frequency intraluminal ultrasound imaging and manometry were performed simultaneously during swallow-induced peristalsis in ten normal subjects. Standardized 5-ml water swallows were recorded 2 cm above the lower esophageal sphincter under three study conditions: control, edrophonium (80 microg/kg iv), and atropine (10 microg/kg iv). A close temporal correlation exists between the peak pressure and peak wall thickness during the control period. The mean time lag between the peak LM and peak CM contraction was 0.03 s. After edrophonium administration, the mean contraction amplitude increased from 101 +/- 9 mmHg to 150 +/- 20 mmHg (P < 0.05) and mean peak muscle thickness increased from 3.0 +/- 0.2 mm to 3.6 +/- 0.3 mm (P < 0.01), and duration of both CM and LM contractions were also increased. Furthermore, the mean time difference between the peak LM and CM was increased to 1.1 s, (ranging 0.2 to 3.4 s) (P < 0.0001). We conclude that cholinomimetic agent induces discoordination between the two muscle layers of the esophagus.