Gender is a determinative factor in the initial clinical presentation of eosinophilic esophagitis.

Eosinophilic esophagitis (EoE) is a chronic, immune-mediated disease resulting in symptoms of esophageal dysmotility. Abnormalities include dysphagia, food impaction and reflux. Although men appear to comprise a majority of the EoE population, few studies have directly assessed gender-associated clinical differences. The aim of this study is to identify the effect of gender on the initial clinical presentation of adult-onset EoE patients. We reviewed our electronic medical record database from January 2008 to December 2011 for adults diagnosed with EoE per the 2011 updated consensus guidelines. Patient demographics, presenting symptoms, endoscopy findings and complications were recorded. Proportions were compared using chi-squared analysis, and means were compared using the Student's t-test. A total of 162 patients met the inclusion criteria and 71 (44%) were women. Women were more likely to report chest pain (P = 0.03) and heartburn (P = 0.06), whereas men more commonly reported dysphagia (P = 0.04) and a history of food impaction (P = 0.05). Endoscopic findings were similar between groups. No patients suffered esophageal perforations. These data suggest that men report more fibrostenotic symptoms and women report more inflammatory symptoms at the time of diagnosis. There was no difference in endoscopic findings between genders. This is one of the only reviews comparing differences in clinical presentation, endoscopic findings and complications between gender for EoE. The current recommended guidelines state that any patient with symptoms of esophageal dysfunction should be biopsied for EoE. Our findings support biopsying patients with typical and atypical symptoms of dysmotility including heartburn and chest pain.

A case-control study of the pathology of oesophageal disease in systemic sclerosis (scleroderma).

BACKGROUND: Atrophy of the smooth muscle layers of the muscularis propria characterises oesophageal involvement in systemic sclerosis (scleroderma). The aetiology of this atrophy and of the resultant oesophageal dysfunction is unknown. OBJECTIVES: To examine oesophageal tissue for evidence of fibrosis, vascular disease, inflammatory reactions and neural abnormalities to determine the possible causes of this disease process. METHODS: A case-control survey was conducted using oesophageal tissue from 74 scleroderma cases and 74 age, race and sex-matched controls from our autopsy files. Histological evidence of oesophageal muscle atrophy was correlated with the degree of vascular changes, inflammatory infiltration, fibrosis, abnormalities of the myenteric plexus and reduction of interstitial cells of Cajal (ICC) using a predesigned semiquantitative descriptive method. RESULTS: Smooth-muscle atrophy was found in 94% of scleroderma cases, and in 5% of controls (p<0.001). Atrophy was evident in the circular smooth muscle in 93% of cases, and in the longitudinal smooth muscle in 66% of cases. Intimal proliferation of arterioles was found in 38% of cases and in 5% of controls (p<0.001), but was not associated with smooth-muscle atrophy (p = 0.29). Despite these vascular changes, there was no evidence of compromised perfusion, such as findings suggestive of acute ischaemic necroses. Minimal cellular infiltrates were seen in the myenteric plexus in 82% of cases and in 92% of controls (p = 0.091). ICC were found in fewer numbers in areas of atrophic smooth muscle compared with adjacent normal smooth muscle in selected scleroderma cases. CONCLUSION: The pathological findings of oesophageal lesions in scleroderma seem inconsistent with either an ischaemic or an inflammatory process. The loss of circular and longitudinal smooth muscle in the distal scleroderma oesophagus may represent loss of normal neural function followed by secondary tissue atrophy, or may be a primary smooth muscle lesion.

Esophageal lichen planus: case report and review of the literature.

Involvement of the esophagus by lichen planus is a rarely reported condition. The histologic features of esophageal lichen planus, which may differ from those of cutaneous disease, have only rarely been illustrated. We describe a 58-year-old woman with skin and oral lichen planus who presented with dysphagia and an esophageal stricture that were ultimately diagnosed as esophageal lichen planus. Multiple esophageal biopsies demonstrated a lichenoid, T cell-rich lymphocytic infiltrate, along with degeneration of the basal epithelium and Civatte bodies. Correct diagnosis of esophageal lichen planus is critical because of its prognostic and therapeutic distinction from other more common causes of esophagitis and stricture formation.

Computers in endoscopy.

Computers have taken over the endoscopy unit, as they have the rest of society. In 1980, most endoscopy units had no computers at all. Today, however, the average hospital endoscopy unit in the United States is equipped with many computers. Computers have revolutionized performance and teaching techniques of endoscopy. Changes in communication of findings and management of the endoscopy unit that are occurring are attributed to the use of computers. This article discusses videoendoscopy, advances in electronic technology, and the impact of the digital image.

Can restrictions on reimbursement for anti-ulcer drugs decrease Medicaid pharmacy costs without increasing hospitalizations?

OBJECTIVE: To examine the impact of a policy restricting reimbursement for Medicaid anti-ulcer drugs on anti-ulcer drug use and peptic-related hospitalizations. DATA SOURCES/STUDY SETTING: In addition to U.S. Census Bureau data, all of the following from Florida: Medicaid anti-ulcer drug claims data, 1989-1993; Medicaid eligibility data, 1989-1993; and acute care nonfederal hospital discharge abstract data (Medicaid and non-Medicaid), 1989-1993. STUDY DESIGN: In this observational study, a Poisson multiple regression model was used to compare changes, after policy implementation, in Medicaid reimbursement for prescription anti-ulcer drugs as well as hospitalization rates between pre- and post-implementation periods in Medicaid versus non-Medicaid patients hospitalized with peptic ulcer disease. PRINCIPAL FINDINGS: Following policy implementation, the rate of Medicaid reimbursement for anti-ulcer drugs decreased 33 percent (p < .001). No associated increase occurred in the rate of Medicaid peptic-related hospitalizations. CONCLUSIONS: Florida's policy restricting Medicaid reimbursement for anti-ulcer drugs was associated with a substantial reduction in outpatient anti-ulcer drug utilization without any significant increase in the rate of hospitalization for peptic-related conditions.

Cyclic vomiting: association with multiple homeostatic abnormalities and response to ketorolac.

Cyclic vomiting is a rare syndrome that over the years has variously been ascribed to psychogenic causes, sensory seizures, abdominal migraine, and more recently, to mechanical or electrical disturbances in gastric physiology. We describe the case of a 65-year-old white diabetic female with a 10-yr history of recurrent episodes of nausea and vomiting, occurring every 10-12 days and lasting approximately 1-3 days at a time. These episodes were accompanied by edema, mild temperature elevations, and remarkable elevations in blood pressure. In between these episodes, the patient remained asymptomatic. Initial screening tests were also negative except for moderate gastroparesis. However, antral motility was found to be normal, as was an electrogastrogram. Detailed neurological and psychiatric evaluations were negative. Trials of erythromycin, metoclopramide, naloxone, ondansetron, and amitryptiline were unsuccessful. Serial endocrinological testing revealed that an episode of vomiting was always preceded by an abnormal elevation in at least one of the following: serum adrenocorticotropic hormone, serum cortisol, or urinary cortisol. In the midst of an episode, all three values were exceedingly high (e.g., > 10-fold increases in 24-hr urinary cortisol levels). Fluctuations of a milder degree, though still abnormally high, were also noted in between cycles at times when the patient was completely asymptomatic. High-dose dexamethasone suppressed these hormonal surges completely but not the clinical symptoms, which continued undisturbed. The patient was finally given a trial of intramuscular ketorolac during one of her episodes, which produced prompt and sustained relief. During the next few weeks, she was given this drug each time her symptoms commenced, and each time it appeared that her cycle had been aborted. She has since been able to terminate her episodes promptly and completely by self-administration of ketorolac. We speculate that her syndrome is caused by a poorly characterized disorder of endogenous prostaglandin release, resulting not only in derangements in the hypothalamic pituitary system but also in nausea and vomiting.

Botulinum toxin for achalasia: long-term outcome and predictors of response.

BACKGROUND & AIMS: Botulinum toxin injection into the lower esophageal sphincter of patients with achalasia results in effective short-term relief of symptoms. The aims of this study were to examine the long-term outcome of these patients and to determine the predictors of response to this therapy. METHODS: Thirty-one patients with achalasia treated with botulinum toxin were followed up prospectively for a median duration of 890 days. RESULTS: Twenty-eight patients improved initially, but only 20 patients had sustained improvement beyond 3 months; the latter patients were classified as responders. The response rate was greater in patients older than 50 years of age (82% vs. 43% in younger patients; P = 0.03) and in patients with vigorous achalasia (100% vs. 52% with classic achalasia; P = 0.03). Duration of illness, previous dilation, and baseline radiological characteristics did not influence outcome. Nineteen responders eventually had relapse after a median duration of 468 days (range, 153 - 840 days). Fifteen of these patients received a second injection with satisfactory results obtained in the majority of patients. CONCLUSIONS: Botulinum toxin is an effective treatment for achalasia in about two thirds of patients, with a duration of response averaging 1.3 years. Age and type of achalasia seem to be important predictors of response.

Intrasphincteric botulinum toxin for the treatment of achalasia.

BACKGROUND: Achalasia is a disorder of swallowing in which the lower esophageal sphincter fails to relax. We report the use of botulinum toxin, a paralytic agent, for the treatment of this condition. METHODS: In a double-blind trial, 21 patients with achalasia received either 80 units of botulinum toxin or placebo, injected endoscopically into the lower esophageal sphincter. One week later, the response to treatment was assessed on the basis of changes in the symptom scores (measured on a scale from 0 to 9), pharyngoesophagograms, and results of esophageal manometric and scintigraphic studies. Patients who received placebo initially were subsequently treated with botulinum toxin. After six months, esophageal scintigraphy was repeated. RESULTS: One week after treatment, the mean decrease in the symptom score was 5.4 points for the patients treated with botulinum toxin and 0.5 point for the placebo group (P = 0.001). The mean decrease in the pressure of the lower esophageal sphincter was 33 percent in the treatment group, as compared with a mean increase of 12 percent in the placebo group (P = 0.02), and the mean increase in the width of the opening of the lower esophageal sphincter was 204 percent in the treatment group, as compared with a mean decrease of 14 percent in the placebo group (P = 0.02). Nineteen of the 21 patients treated with botulinum toxin had symptomatic improvement initially; after six months 14 patients were still in remission. This improvement was accompanied by a decrease in esophageal retention that was sustained at six months (46 percent, as compared with a pretreatment value of 77 percent; P = 0.04). There were no serious adverse effects. CONCLUSIONS: Injection of botulinum toxin into the lower esophageal sphincter is an effective, safe, and simple method of treatment for achalasia, with results that are sustained for several months.

Effects of intrasphincteric botulinum toxin on the lower esophageal sphincter in piglets.

BACKGROUND: The toxin of Clostridium botulinum (BoTx) inhibits the release of acetylcholine from nerve terminals and causes paralysis of skeletal muscle. The present study examined the hypothesis that BoTx may have a similar effect on gastrointestinal smooth muscle. METHODS: Baseline lower esophageal sphincter (LES) pressures were obtained in five piglets, and normal saline was injected endoscopically into the LES. One week later, LES pressure was measured again, followed by injection of BoTx into the LES. After another week, LES pressure was measured again. RESULTS: Compared with a baseline LES pressure of 8.2 +/- 1.5 mm Hg, LES pressure decreased to 3.2 +/- 1.0 mm Hg after BoTx injection, a reduction of about 60% (P < 0.01). By contrast, LES pressure did not change significantly after normal saline injection. The animals showed no evidence of toxicity. Data from other experiments showed that after injection with toxin, the LES responds normally to bethanechol and pentagastrin but displays a paradoxical response to edrophonium and cholecystokinin. CONCLUSIONS: BoTx is a potent inhibitor of resting LES tone. Its relatively specific anticholinergic effect may help clarify the role of cholinergic and noncholinergic pathways in the regulation of gastrointestinal sphincters.

Medical treatment of esophageal motility disorders.

Swallowing is a complex mechanism that is based on the coordinated interplay of tongue, pharynx, and esophagus. Disturbances of this interplay or disorders of one or several of these components lead to dysphagia, non-cardiac chest pain, or regurgitation. The major esophageal motility disorders include achalasia, diffuse esophageal spasm, hypercontractile esophagus ("nutcracker esophagus"), and hypocontractile esophagus ("scleroderma esophagus"). Other esophageal diseases such as hypopharyngeal (Zenker's) diverticula or gastroesophageal reflux disease also may be sequelae of primary esophageal motility disorder. Finally, a substantial group of patients referred for evaluation of possible esophageal motor disorders have milder degrees of dysmotility--referred to as nonspecific esophageal motor disorder--that are of unclear clinical significance. Medical treatment of esophageal motility disorders involves the uses of agents that either reduce (anti-cholinergic agents, nitrates, calcium antagonists) or enhance (prokinetic agents) esophageal contractility. Despite the beneficial effect of the various drugs on esophageal motility parameters, the clinical benefit of medical treatment is often disappointing. From clinical and epidemiological studies there is some evidence for a "psychological" component in the pathogenesis or perception of esophageal symptoms. Further understanding of esophageal pathophysiology, as well as development of new receptor selective drugs, might increase our chances of successful treatment of esophageal motility disorders.

Vagal reflexes referred from the upper aerodigestive tract: an infrequently recognized cause of common cardiorespiratory responses.

OBJECTIVE: To review the physiologic basis for normal and abnormal vagal reflexes arising from the pharynx, larynx, and esophagus, as well as the relevance of vagal reflexes to the pathogenesis of such clinically common cardiorespiratory responses as bradycardia, tachycardia, dysrhythmia, coronary angiospasm, bronchospasm, laryngospasm, prolonged apnea, and singultus (hiccups). DATA SOURCES: Pertinent articles and reviews were identified through a MEDLINE search (April 1966 to October 1991). Older studies and others not identified in the MEDLINE search were found through a manual search of the bibliographies of the retrieved articles. STUDY SELECTION: Experimental studies in both humans and animals, as well as case series and single case reports, were selected for evaluation and citation. In instances where a similar phenomenon was described in multiple independent reports, only studies that provided a novel finding or interpretation were cited. More authoritative book chapters and peer-reviewed summaries were also cited in support of commonly accepted principles. DATA EXTRACTION AND SYNTHESIS: Most of the clinical data are derived from case reports and small case series and are therefore anecdotal; equal weight was given to all such studies. Reports of conflicting observations or interpretations were clearly identified and were cited without exception. CONCLUSIONS: Stimulation of the upper aerodigestive tract can lead to clinically significant cardiorespiratory responses. Although the prevalence of and risk factors for such responses have not been established, we suggest that a pharyngeal, a laryngeal, or an esophageal source for abnormal cardiorespiratory responses be sought whenever a detailed clinical evaluation fails to reveal a cause, particularly when there are concurrent symptoms or signs of upper aerodigestive tract disease, such as dysphagia or gastroesophageal reflux.

Swallowing dysfunction in the postpolio syndrome: a cinefluorographic study.

Twenty patients with a remote history of poliomyelitis and recent or progressive dysphagia were evaluated with cinefluorography. Radiographic abnormalities were present in the pharynx in varying degrees in all but one of the patients. Findings included atrophy of the prevertebral soft tissues, unilateral or bilateral weakness of the tongue or soft palate, paresis or paralysis of the pharyngeal constrictor muscle, incomplete or absent epiglottic tilt, poor laryngeal elevation, poor laryngeal closure with laryngeal penetration, aspiration (often without a cough), and luminal narrowing at the cricopharyngeal level. Other structural lesions included a Zenker diverticulum in one patient, bilateral pharyngeal pouches in five, and a unilateral pouch in one. Additional structural lesions contributing to dysphagia were found in two other patients, including a focal stricture in the cervical esophagus in one patient and two stenotic rings in the distal esophagus in another. In four patients (one of whom had the Zenker diverticulum), the inferior constrictor muscle contracted forcibly above a prominent cricopharyngeus muscle, perhaps contributing to the formation of the diverticulum. It is important to examine postpolio patients with dysphagia carefully with dynamic imaging to assess the severity of decompensation and to detect other lesions that may be treatable. The information derived can be used to guide management.

Caustic ingestion injuries of the upper aerodigestive tract.

Few reports have described in detail the injuries that occur to the oral cavity, pharynx, and larynx following caustic ingestion. The role of dynamic radiographic studies to delineate the extent of damage has been minimized. In-depth radiographic analysis of such cases has not, to our knowledge, been previously reported. In order to examine the injuries and functional abnormalities of these sites following caustic ingestion, the records of The Johns Hopkins Swallowing Center were reviewed. Five patients were identified as having significant upper aerodigestive tract caustic injuries. All patients had dysphagia, epiglottis injuries, and incomplete laryngeal protection with aspiration. Four of five had sustained some degree of esophageal stenosis. Also noted were pharyngeal muscle dysfunction, nasopharyngeal regurgitation, tongue fixation, and hypopharyngeal stenosis. Roentgenographic findings are described and illustrated. The multidisciplinary approach to the management and rehabilitation of these patients is discussed.

Gastric antral vascular ectasia ("watermelon stomach"): radiologic findings.

Radiologic findings in a patient with gastric antral vascular ectasia are described on computed tomographic scans, upper gastrointestinal series, and specimen radiographs. Findings include prominent, scalloped antral folds radiating to the pylorus and thickening of the gastric antrum. Pathognomonic red vascular folds, likened to stripes on a watermelon, can be seen endoscopically.