Esophageal leiomyomatosis in a woman with a history of vulvar leiomyoma and Barrett's esophagus: a case report and review of the literature.

BACKGROUND: The diagnosis and treatment of esophageal pathology remains a challenge despite advances in preoperative endoscopy, radiographic staging, and perioperative care. CASE REPORT: In this article, we present an interesting case of esophageal leiomyomatosis in a woman with a history of vulvar leiomyoma and Barrett's esophagus. This paper represents the first reported simultaneous occurrence of these three pathologic entities in the English literature. CONCLUSIONS: The clinical presentation and characteristic pathologic findings in patients with esophageal leiomyomatosis are reviewed. Diagnostic and therapeutic approaches to esophageal masses are discussed including the indications for esophageal resection.

Lower esophageal sphincter relaxation characteristics using a sleeve sensor in clinical manometry.

OBJECTIVE: We undertook this study to determine the characteristics of swallow-induced lower esophageal sphincter (LES) relaxation in the setting of clinical manometry using a standardized methodology. METHODS: We reviewed 170 manometric recordings performed using a perfused manometric assembly with a sleeve sensor and a computer polygraph. Patients were categorized as patient controls, gastroesophageal reflux disease (GERD), diffuse esophageal spasm (DES), or achalasia. Tracing were semiautomatically analyzed for basal LES pressure, LES pressure during deglutitive relaxation (relaxation LES pressure), duration of LES relaxation, timing of LES relaxation, and the success rate of primary peristalsis. RESULTS: Forty-six patient controls, 93 with GERD, five with DES, and 26 with achalasia were identified. GERD and achalasia patients had lower or higher basal LES pressures than patient controls, respectively. Compared with patient controls, achalasia patients had higher relaxation LES pressures, lower percent LES relaxation, and shorter durations of LES relaxation. The best single measure for distinguishing achalasia was the relaxation LES pressure; using the 95th percentile value of patient controls (12 mm Hg) as the upper limit of normal, its sensitivity and positive predictive value for the diagnosis of achalasia were 92% and 88%, respectively. Coupled with the finding of aperistalsis, a relaxation LES pressure > or = 10 mm Hg achieved 100% sensitivity and positive predictive value among these patients. CONCLUSIONS: Sleeve sensor recording is a practical method for clinical manometry that reliably records LES relaxation characteristics and is amenable to both a standardized manometry protocol and a semiautomated analysis routine. Relaxation LES pressure has a high diagnostic value for achalasia.

Clinical applications of esophageal manometry and pH monitoring.

In summary, GERD patients are usually well managed using a careful medical history, endoscopy, and empirical trials of antireflux medications. Extended esophageal pH monitoring is unnecessary in most patients but can be of considerable value in managing patients with typical or atypical symptoms who are refractory to standard therapy for GERD. Furthermore, the test can be useful in documenting abnormal reflux in an individual without esophagitis being evaluated for antireflux surgery. The test is done with compact, portable data loggers, miniature pH electrodes, and computerized data analysis. The pH electrode should be positioned 5 cm above the manometrically defined upper limit of the LES, and patients should undergo the test on an unrestricted diet. In terms of data analysis, the total percentage time of pH < 4 provides as much information as any other scheme of quantifying esophageal acid exposure, but symptom association is essential when evaluating atypical or sporadic symptoms. Enthusiasm for 24-h pH monitoring must, however, be tempered with an analysis of its proven clinical utility in patient management with its utility rightfully compared with that of an empirical trial of anti-reflux therapy. Ambulatory pH monitoring is probably most useful in examining patients without typical reflux symptoms or patients who have either partially or completely failed a trial of anti-reflux therapy. To date, there have not been any prospective, controlled clinical trials evaluating these uses. Suggested clinical indications for ambulatory pH monitoring are listed in Table 5 (53).

Esophageal bolus transit imaged by ultrafast computerized tomography.

BACKGROUND & AIMS: Conventional transit imaging techniques detect only the solid or liquid component of a swallowed bolus within the esophagus. This study aimed to dynamically image the composition, distribution, and propulsion of esophageal contents during swallowing. METHODS: Multiple adjacent cross-sectional images of the esophagus were obtained in 7 subjects using ultrafast computerized tomography. Images from two 10-mL swallows were synchronized and analyzed for bolus distribution and propulsion, cross-sectional area, intraluminal volume, and intraluminal content. RESULTS: Both liquid and air were swallowed, with the relative distribution varying among levels. Within the ampulla, air occupied 71% of the luminal cross-sectional area. Air was propelled ahead of the liquid bolus at 17 cm/s compared with 7 cm/s for fluid (P < 0.01) and accumulation within the ampulla. Mean bolus velocity was slower through the ampulla. A variable (8-32 mL) volume of air was ingested during swallowing. CONCLUSIONS: Ultrafast computerized tomography studies during transit of a swallowed bolus through the esophagus showed substantial aerophagia and partial bolus separation with air preceding fluid. The ampulla exhibited greater distention that the tubular esophagus because the bolus accumulated at this level before transfer across the hiatus.

The endoscopic management of esophageal strictures.

Endoscopic dilation for symptomatic esophageal strictures is an effective and easily performed procedure in the palliation of benign as well as malignant esophageal strictures. This article describes the advantages and disadvantages of the various types of esophageal dilators as well as the techniques used in dilation. Precautions, complications, long-term outcome, and the special circumstances related to difficult dilations also are discussed.

Esophageal dysphagia.

This paper is a review of esophageal anatomy, physiology and pathophysiology. The diagnosis and therapy of benign and malignant esophageal strictures are discussed including the specifics of esophageal dilation and tumor ablation procedures. The diagnosis and therapy of esophagitis in the immunocompromised (HIV, chemotherapy, transplant recipient) host is discussed. The pathophysiology and treatment of achalasia and esophageal spasm are reviewed. Finally, current concepts of the pathophysiology and therapy of gastroesophageal reflux disease. Emphasis is placed on the dual sphincter theory of gastroesophageal junction competence and the need for maintenance anti-secretory therapy.

Shape, volume, and content of the deglutitive pharyngeal chamber imaged by ultrafast computerized tomography.

BACKGROUND: Conventional radiographic techniques image only the silhouettes of the deglutitive pharyngeal chamber. This study aimed to accurately image the horizontal plane shape and content of the pharynx during swallowing. METHODS: Dynamic computerized tomography images of the pharynx were obtained at the rate of 17 per second during swallowing. Multiple adjacent levels were imaged in eight subjects and a single level was scanned in four subjects during swallows of varied volume. Images were analyzed for area, volume, and the bolus fraction of the deglutitive pharyngeal chamber. RESULTS: The deglutitive chamber enlarged to approximately 24 mL (during tongue loading) compared with a preswallow pharyngeal volume averaging 15 mL. Throughout the 10 mL swallows, the bolus occupied less than 30% of the lumen regardless of axial level. The bolus fraction of the deglutitive chamber increased with swallow volume, as did the dimensions of the upper esophageal sphincter and the bolus velocity through the upper esophageal sphincter. CONCLUSIONS: The deglutitive pharyngeal chamber was typically approximately 15 mL > the bolus volume, implying that an obligatory 15 mL of air was swallowed under these test conditions. Most swallowed air originated as air trapped within the pharynx and larynx as the oropharynx was sealed from above and below.

Deglutitive tongue action: volume accommodation and bolus propulsion.

BACKGROUND: Swallow function is best analyzed in components because discrete component failure may be compensated for with devised maneuvers, postures, or biofeedback techniques. The present investigation examined normal deglutitive tongue function. METHODS: Biplane videofluoroscopy synchronized with intraluminal manometry was performed in eight volunteers. Tongue surface motion was characterized as centripetal or centrifugal along seven equiangular rays emanating from the tongue center during 1-, 5-, 10-, and 20-mL swallows. RESULTS: The tongue perimeter remained in contact with the alveolar ridge while the central groove exhibited centripetal and subsequent centrifugal motion that, in conjunction with the pharyngeal walls, created an oropharyngeal propulsive chamber and then expelled that chamber's contents into the hypopharynx. Intrabolus propulsive pressure was generated when the initially expansive propulsive chamber volume contracted to the test bolus volume. Because pharyngeal chamber action cycle timing was relatively constant among bolus volumes, vigorous expulsion occurred with large volumes but relatively delayed, sluggish expulsion occurred with smaller volumes. CONCLUSIONS: Deglutitive tongue functions include bolus containment, volume accommodation, and the major contributor to bolus propulsion.

Pharyngeal clearance during swallowing: a combined manometric and videofluoroscopic study.

The deglutitive pharyngeal contraction was analyzed using simultaneous videofluoroscopic and manometric studies of eight volunteers. Anterior, posterior, and longitudinal movements of the pharyngeal surfaces, relative to the cervical vertebrae, were measured during swallows of 5 and 10 mL of liquid barium. Profound pharyngeal shortening during bolus transit through the pharynx eliminated access to the larynx and elevated the upper esophageal sphincter to within 1.5 cm of the retrolingual pharynx. Bolus head movement through the pharynx preceded the propagated pharyngeal contraction and registered manometrically as a slight intrabolus pressure before the major pressure complex. Contraction in the horizontal plane began after bolus head transit and culminated with stripping of the bolus tail through the pharynx. Prolonged upper sphincter opening with the larger-volume swallows resulted from a delayed onset rather than altered propagation of the horizontal pharyngeal contraction. It is concluded that the propagated pharyngeal contraction facilitates pharyngeal clearance but has a minimal role in the process of bolus propulsion during swallowing. The propagated contraction works in concert with profound pharyngeal shortening to minimize hypopharyngeal residue after a swallow.

Gastrointestinal manifestations of epidermolysis bullosa. A study of 101 patients.

One hundred one patients with EB were evaluated by a combination of prospective and retrospective review, and analyzed regarding the nature, incidence, and prevalence of their gastrointestinal (GI) manifestations. Involvement of the GI tract is a well-known extracutaneous manifestation of dystrophic EB, but it also occurred in more than one-half and one-third, respectively, of those with junctional and simplex EB. Most of the serious consequences, such as esophageal strictures and microstomia, occurred in recessive dystrophic EB but were also seen, although infrequently, in the junctional and simplex forms. The majority of patients with dysphagia had an esophageal stricture, and the cervical esophagus was the most common location. The onset of dysphagia generally occurred in the first decade of life, in patients much younger than previously recognized. Diagnostic endoscopy did not reveal lesions which could not have been detected radiographically. Lower GI complaints were common, especially constipation and perianal blistering, and affected all types of EB. These complaints contributed substantially to management problems but they did not correlate with colonic pathology and appeared to reflect anal or perianal disease.

Aging and the esophagus: common pathologic conditions and their effect upon swallowing in the geriatric population.

Dysphagia in the elderly is most often oropharyngeal or hypopharyngeal in location and neuropathologic in etiology. Aging itself, although causing demonstrable structural and functional changes in the esophagus, does not cause any clinically relevant esophageal dysphagia. A variety of pathologic conditions seen in the geriatric population affect the esophagus and can alter esophageal function, resulting in symptomatic dysphagia. Accurate diagnosis requires a thorough evaluation performed in an unhurried fashion, often with the assistance of ancillary personnel. Treatment of these esophageal disorders is multidisciplinary and may involve dietary manipulations, the administration of medications, therapeutic endoscopic procedures, and occasionally surgery.

Viral hepatitis. The new ABC's.

Hepatitis may be caused by hepatitis A virus, hepatitis B virus, hepatitis C virus (classic non-A non-B viral hepatitis), hepatitis D virus (delta agent), and hepatitis E virus (epidemic non-A non-B viral hepatitis). Cytomegalovirus, Epstein-Barr virus, and herpes simplex virus may also occasionally cause hepatitis. Some forms of hepatitis carry the risks of chronic infection, cirrhosis, or hepatocellular carcinoma. Treatment options for viral hepatitis are limited and, in many cases, still under investigation. Prophylaxis is available for many forms of hepatitis and should be offered to those at risk.

Circadian periodicity of the time of onset of acute lower gastrointestinal bleeding.

We investigated the chronobiological parameters of acute gastrointestinal (GI) bleeding in 51 patients able to time-specify the onset of bleeding within 30 min of occurrence. Bleeding was determined to be either from the upper or lower GI tract. The upper GI bleeding group consisted of 32 patients (22 male, 10 female) who bled from peptic ulcer disease (16), Mallory-Weiss tear (4), gastritis (3), esophageal varices (3), gastric neoplasm (2), Dieulafoy's lesion (1), and unknown (3). The lower GI bleeding group consisted of 19 patients (9 male, 10 female) who bled from diverticulosis coli (5), hemorrhoids (2), arteriovenous malformations (2), colonic polyps (2), cecal ulcer (1), antibiotic-associated colitis (1), and unknown (6). Rhythmicity was evaluated by inferential statistics. The time of onset of lower GI bleeding (34 episodes) displayed significant circadian periodicity (p = 0.014) with its peak at 1100 h. Single cosinor analysis revealed: MESOR-1.42 (95% CI = 0.86- 1.97); amplitude = 1.22 (0.44-1.99); phase angle = -165.12 (-201.66 -128.58). Upper GI bleeding (42 episodes) displayed no circadian periodicity (p = 0.46). When both upper and lower GI bleeding were evaluated together, no circadian rhythm was evident (p = 0.07). We conclude that there is a circadian periodicity in the time of onset of only acute lower GI bleeding with its peak at 1100 h. The pacemaker of this periodicity remains unknown.