[Fever and dysphagia of a young woman]

We report the case of a 39-year old patient with septicemia treated for pharyngitis with antibiotics since a few days. She wasn't able to swallow her antibiotics anymore because of dysphagia. Radiologic examination revealed pulmonary infiltrates and Vena iugularis interna-thrombosis. These findings and anamnesis led to the diagnosis of Lemierre syndrome inspite of lacking detection of bacteria. After changing the antibiotic therapy and start of anticoagulation further course of illness was favorable. The long duration of hospitalization was indepted to high morbidity typically seen in Lemierre syndrome.

Electroanatomic reconstruction of the left atrium, pulmonary veins, and esophagus compared with the "true anatomy" on multislice computed tomography in patients undergoing catheter ablation of atrial fibrillation

BACKGROUND: Current concepts of catheter ablation for atrial fibrillation (AF) commonly use three-dimensional (3D) reconstructions of the left atrium (LA) for orientation, catheter navigation, and ablation line placement. OBJECTIVES: The purpose of this study was to compare the 3D electroanatomic reconstruction (Carto) of the LA, pulmonary veins (PVs), and esophagus with the true anatomy displayed on multislice computed tomography (CT). METHODS: In this prospective study, 100 patients undergoing AF catheter ablation underwent contrast-enhanced spiral CT scan with barium swallow and subsequent multiplanar and 3D reconstructions. Using Carto, circumferential plus linear LA lesions were placed. The esophagus was tagged and integrated into the Carto map. RESULTS: Compared with the true anatomy on CT, the electroanatomic reconstruction accurately displayed the true distance between the lower PVs; the distances between left upper PV, left lower PV, right lower PV, and center of the esophagus; the longitudinal diameter of the encircling line around the funnel of the left PVs; and the length of the mitral isthmus line. Only the distances between the upper PVs, the distance between the right upper PV and esophagus, and the diameter of the right encircling line were significantly shorter on the electroanatomic reconstructions. Furthermore, electroanatomic tagging of the esophagus reliably visualized the true anatomic relationship to the LA. On multiple tagging and repeated CT scans, the LA and esophagus showed a stable anatomic relationship, without relevant sideward shifting of the esophagus. CONCLUSION: Electroanatomic reconstruction can display with high accuracy the true 3D anatomy of the LA and PVs in most of the regions of interest for AF catheter ablation. In addition, Carto was able to visualize the true anatomic relationship between the esophagus and LA. Both structures showed a stable anatomic relationship on Carto and CT without relevant sideward shifting of the esophagus.

Dysphagia due to triple A syndrome: successful treatment of achalasia by balloon dilatation

Triple A syndrome is a rare autosomal recessive inherited disorder which is characterized by alacrima, adrenal insufficiency, and achalasia. We report on a 14-year old girl with dysphagia, regurgitation, and vomiting since 5 years. At the age of five years an Addison crisis was diagnosed and cortisone substitution was initiated. In addition, the patient had episodes of conjunctivitis. Severe esophagitis and candida infection were diagnosed by esophago-gastro-duodenoscopy and treated with omeprazole and fluconazole. The esophageal barium swallow was typical for achalasia. Medical treatment of achalasia with oral nifedipine resulted only in a partial and temporal improvement. But after seven balloon dilatations dysphagia and nocturnal coughing improved clearly and a remarkable gain of weight could be seen. Direct sequencing showed a homozygous nonsense mutation in exon 11 of the AAAS gene leading to truncation at position 342 of the 546 amino acid protein. CONCLUSION: Triple A syndrome has to be considered in patients with dysphagia. In our patient, the absence of tears since birth followed by adrenal insufficiency were early signs of the triple A syndrome. Balloon dilatation of the esophago-gastric junction is an effective treatment, which can avoid surgical interventions.

Swallowing Activity Assessed by Ambulatory Impedance-pH Monitoring Predicts Awake and Asleep Periods at Night

INTRODUCTION: Voluntary muscle activity, including swallowing, decreases during the night. The association between nocturnal awakenings and swallowing activity is under-researched with limited information on the frequency of swallows during awake and asleep periods. AIM: The aim of this study was to assess nocturnal swallowing activity and identify a cut-off predicting awake and asleep periods. METHODS: Patients undergoing impedance-pH monitoring as part of GERD work-up were asked to wear a wrist activity detecting device (Actigraph(®)) at night. Swallowing activity was quantified by analysing impedance changes in the proximal esophagus. Awake and asleep periods were determined using a validated scoring system (Sadeh algorithm). Receiver operating characteristics (ROC) analyses were performed to determine sensitivity, specificity and accuracy of swallowing frequency to identify awake and asleep periods. RESULTS: Data from 76 patients (28 male, 48 female; mean age 56 ± 15 years) were included in the analysis. The ROC analysis found that 0.33 sw/min (i.e. one swallow every 3 min) had the optimal sensitivity (78 %) and specificity (76 %) to differentiate awake from asleep periods. A swallowing frequency of 0.25 sw/min (i.e. one swallow every 4 min) was 93 % sensitive and 57 % specific to identify awake periods. A swallowing frequency of 1 sw/min was 20 % sensitive but 96 % specific in identifying awake periods. Impedance-pH monitoring detects differences in swallowing activity during awake and asleep periods. Swallowing frequency noticed during ambulatory impedance-pH monitoring can predict the state of consciousness during nocturnal periods

Association Between Baseline Impedance Values and Response Proton Pump Inhibitorsin Patients With Heartburn

BACKGROUND & AIMS: Esophageal impedance measurements have been proposed to indicate the status of the esophageal mucosa, and might be used to study the roles of the impaired mucosal integrity and increased acid sensitivity in patients with heartburn. We compared baseline impedance levels among patients with heartburn who did and did not respond to proton pump inhibitor (PPI) therapy, along with the pathophysiological characteristics of functional heartburn (FH). METHODS: In a case-control study, we collected data from January to December 203 on patients with heartburn and normal findings from endoscopy who were not receiving PPI therapy and underwent impedance pH testing at hospitals in Italy. Patients with negative test results were placed on an 8-week course of PPI therapy (84 patients received esomeprazole and 36 patients received pantoprazole). Patients with more than 50% symptom improvement were classified as FH/PPI responders and patients with less than 50% symptom improvement were classified as FH/PPI nonresponders. Patients with hypersensitive esophagus and healthy volunteers served as controls. In all patients and controls, we measured acid exposure time, number of refluxes, baseline impedance, and swallow-induced peristaltic wave indices. RESULTS: FH/PPI responders had higher acid exposure times, numbers of reflux events, and acid refluxes compared with FH/PPI nonresponders (P < .05). Patients with hypersensitive esophagus had mean acid exposure times and numbers of reflux events similar to those of FH/PPI responders. Baseline impedance levels were lower in FH/PPI responders and patients with hypersensitive esophagus, compared with FH/PPI nonresponders and healthy volunteers (P < .001). Swallow-induced peristaltic wave indices were similar between FH/PPI responders and patients with hypersensitive esophagus. CONCLUSIONS: Patients with FH who respond to PPI therapy have impedance pH features similar to those of patients with hypersensitive esophagus. Baseline impedance measurements might allow for identification of patients who respond to PPIs but would be classified as having FH based on conventional impedance-pH measurements.

Manually calculated oesophageal bolus clearance time increases in parallel with reflux severity at impedance-pH monitoring.

BACKGROUND Oesophageal clearance has been scarcely studied. AIMS Oesophageal clearance in endoscopy-negative heartburn was assessed to detect differences in bolus clearance time among patients sub-grouped according to impedance-pH findings. METHODS In 118 consecutive endoscopy-negative heartburn patients impedance-pH monitoring was performed off-therapy. Acid exposure time, number of refluxes, baseline impedance, post-reflux swallow-induced peristaltic wave index and both automated and manual bolus clearance time were calculated. Patients were sub-grouped into pH/impedance positive (abnormal acid exposure and/or number of refluxes) and pH/impedance negative (normal acid exposure and number of refluxes), the former further subdivided on the basis of abnormal/normal acid exposure time (pH+/-) and abnormal/normal number of refluxes (impedance+/-). RESULTS Poor correlation (r=0.35) between automated and manual bolus clearance time was found. Manual bolus clearance time progressively decreased from pH+/impedance+ (42.6s), pH+/impedance- (27.1s), pH-/impedance+ (17.8s) to pH-/impedance- (10.8s). There was an inverse correlation between manual bolus clearance time and both baseline impedance and post-reflux swallow-induced peristaltic wave index, and a direct correlation between manual bolus clearance and acid exposure time. A manual bolus clearance time value of 14.8s had an accuracy of 93% to differentiate pH/impedance positive from pH/impedance negative patients. CONCLUSIONS When manually measured, bolus clearance time reflects reflux severity, confirming the pathophysiological relevance of oesophageal clearance in reflux disease.