Remission of recurrent gastrointestinal bleeding after septal reduction therapy in patients with hypertrophic obstructive cardiomyopathy-associated acquired von Willebrand syndrome.

BACKGROUND: Gastrointestinal hemorrhage is considered to be a severe complication of von Willebrand disease. The optimal therapy for acquired von Willebrand syndrome and severe gastrointestinal bleeding with hypertrophic cardiomyopathy is undefined. PATIENTS/METHODS: Seventy-seven patients (median age, 67 years; interquartile range [IQR], 56-75 years; 49% women) with hypertrophic cardiomyopathy underwent von Willebrand factor multimer testing and acquisition of bleeding history. Bleeding was detected in 27 (36%) (median age, 74 years; IQR 66-76 years; 74% women), 20 with gastrointestinal bleeding, including 11 women with transfusion dependence. In these 11 women, the median duration of transfusion dependency was 36 months (IQR 18-44 months), and the median number of transfusions required was 25 (IQR 20-38). Two patients had undergone bowel resection for bleeding, one of them twice. Seven patients showed angiodysplasia, and the remainder had no endoscopic lesion. Bleeding recurred after bowel surgery or endoscopic intervention and medical therapy for hypertrophic cardiomyopathy in 10 of 11 patients. Two patients had septal myectomy, and six patients underwent alcohol septal ablation. With the exception of one patient in whom a significant gradient persisted after septal ablation, after the periprocedural period, patients after septal reduction therapy remained free of recurrent bleeding and need for transfusions. CONCLUSION: Acquired von Willebrand syndrome is common in hypertrophic cardiomyopathy. Gastrointestinal bleeding often recurs after endoscopic therapy, but may be relieved by structural cardiac repair.

Shear stress-associated acquired von Willebrand syndrome in patients with mitral regurgitation.

BACKGROUND: Mitral valve regurgitation is associated with an acquired hemostatic defect. OBJECTIVE: We sought to assess the prevalence and severity of acquired von Willebrand syndrome in patients with native valve mitral regurgitation (MR). PATIENTS/METHODS: Fifty-three patients were prospectively observed with bleeding questionnaires and laboratory tests when undergoing an echocardiographic assessment of MR. In patients referred for mitral valve surgery, testing was repeated postoperatively. RESULTS: Echocardiography identified 13 patients with mild MR, 14 with moderate MR, and 26 with severe MR. Among patients with mild, moderate or severe MR, loss of the highest molecular weight von Willebrand factor (VWF) multimers occurred in 8%, 64%, and 85%, respectively, median platelet function analyzer collagen ADP closure times (PFA-CADPs) were 84 s (interquartile range [IQR] 73-96 s), 156 s (IQR 104-181 s), and 190 s (IQR 157-279 s), respectively, and the ratios of VWF latex activity to antigen were 0.92 (IQR 0.83-0.97), 0.85 (IQR 0.76-0.89), and 0.79 (IQR 0.75-0.82), respectively (all P < 0.001). Nine patients reported clinically significant bleeding, and seven had intestinal angiodysplasia and transfusion-dependent gastrointestinal bleeding (Heyde syndrome), with the median number of transfusions required being 20 (IQR 10-33; range 4-50). In patients who underwent mitral valve repair (n = 13) or replacement (n = 7), all measures of VWF function reported above improved significantly. CONCLUSION: The high-shear environment of moderate to severe MR is sufficient to produce prevalent perturbations in VWF activity. Acquired von Willebrand syndrome may occur in this setting, and appears to be reversible with mitral valve surgery.

Successful oesophageal pH monitoring with a catheter-free system.

BACKGROUND: Traditional catheter-based oesophageal pH testing is limited by patient discomfort and the tendency for patients to alter their diet and activities during the study. A catheter-free pH monitoring system (Bravo) designed to avoid these problems has recently become available, but the advantages and limitations of this device have not been fully explored. AIM: To report our initial experience with this new technology. METHODS: The records of consecutive patients undergoing Bravo pH monitoring were reviewed. The squamo-columnar junction was localized endoscopically and the pH capsule was placed 6 cm above this junction. All patients were re-endoscoped immediately following placement to document mucosal attachment. Patients were monitored for 24-48 h and then returned the radiotelemetry recording device. Data were subsequently downloaded to a personal computer. RESULTS: Sixty studies were performed over an 11-month period. In seven of the 60 (12%), the probe did not attach properly, but in six of these a replacement probe was prepared and deployed without difficulty. In one case, the probe could not be attached after two attempts and the procedure was abandoned. During one procedure, the probe was attached to the mucosa at a point 9 cm from the squamo-columnar junction, but a positive test result was obtained. In two cases, the data were not initially retrievable from the recorder, but in one case the manufacturer was able to retrieve the data overnight. Finally, two patients were away from the data recorder for extended periods, resulting in a loss of data, in two cases, but there was sufficient information for interpretation in both studies. Therefore, adequate diagnostic data were obtained in 58 of the 60 (97%) studies. CONCLUSIONS: Catheter-free pH testing is a major advance in patient convenience and comfort. The technical difficulties associated with this new technology are minimal and appear to be no more frequent than those seen with catheter-based systems.

Lichen planus involving the esophagus.

Lichen planus is a common mucocutaneus disorder that rarely involves the esophagus. We report two challenging cases presenting with recurrent dysphagia originally suspected due to gastroesophageal reflux. Subsequent evaluation revealed peculiar endoscopic findings of desquamative esophagitis leading to the diagnosis of lichen planus of the esophagus. This disorder should be considered in middle age or elderly women presenting with unexplained dysphagia or odynophagia. In this paper we review the available literature on the subject and summarize every case reported to date.

Metastasis of head and neck carcinoma to the site of percutaneous endoscopic gastrostomy: case report and literature review.

BACKGROUND: Patients with head and neck cancer often need a percutaneous endoscopic gastrostomy to provide adequate nutrition because of inability to swallow after tumor radiation therapy. However, metastasis of the original tumor to the gastrostomy exit site may occur. METHODS: We describe the case of a 61-year-old man with stage III (T2 N1) squamous cell carcinoma of the tongue in whom a PEG tube was placed to circumvent anticipated difficulties in swallowing after radiation therapy. We also compare this case with similar cases in the literature. RESULTS: Soreness and erythema near the gastrostomy site reported by the patient were diagnosed as cellulitis, and two courses of antibiotic treatment were prescribed. However, a biopsy showed that the original squamous cell carcinoma had metastasized to the gastrostomy exit site. The "pull" method of tube placement had been used in this patient and in all 19 cases of metastasis reported in the literature. CONCLUSIONS: Metastatic cancer should be considered in patients with head and neck cancer who have unexplained skin changes at the gastrostomy site. Our experience with this case and review of the literature indicate that, in patients with head and neck cancer, "pull" procedures for placement of gastrostomy tubes may induce metastasis by direct implantation of tumor cells because of contact between the gastrostomy tube and tumor cells. Methods of tube insertion that avoid such contact are preferred.

Manometry of the upper esophageal sphincter and pharynx is not useful in unselected patients referred for esophageal testing.

OBJECTIVE: We sought to determine whether routinely performing upper esophageal sphincter/pharyngeal (UES/P) manometry in patients referred for esophageal testing alters clinical practice. METHODS: Clinical history along with radiography and manometry studies were reviewed in 470 patients referred for manometry. A total of 435 (92.6%) were found to have adequate evaluation of the UES/P and were included in the study. RESULTS: Of 435 studies, 80 (17.7%) had one or more UES/P abnormalities. Indications for these 80 studies were dysphagia in 30 cases (37.5%), chest pain in 26 (32.5%), both in four (5.0%), and other in 20 (25%). There were 85 manometric abnormalities in the 80 studies: hypertensive UES in 16 cases (18.8%), incomplete UES relaxation in 38 (44.7%), hypertensive pharynx in seven (8.2%), hypotensive pharynx in 15 (17.6%), and abnormal coordination in nine (10.6%). A total of 41 patients had a barium study, which was normal in 11 cases (26%), confirmed the problem in 16 (40%), and suggested another problem in 14 (34%). In 17 patients (21%), there was clinical suspicion for an oropharyngeal disorder before manometry, whereas in 58 cases it was an unexpected finding. Of the 58 patients with an unexpected finding, therapy based on this finding was offered in six (three esophageal dilations, two dietary changes, and one swallowing therapy). If patients with concomitant esophageal body disorders are excluded, in only three patients an unexpected UES finding led to change in management. CONCLUSIONS: An unexpected UES/P finding resulted in a change in therapy in only six of the manometries. Barium esophagogram was diagnostic of the oropharyngeal abnormality in nearly two-thirds of the patients in whom it was clinically suspected. These data, along with limited therapeutic options, suggest limited usefulness for routine UES/P manometry.

Challenging problems presenting as constipation.

Patients who seek medical care for constipation present challenges which may involve communication problems, difficulties in diagnosis, lack of optimal therapy, uncorrectable underlying disorders, or psychiatric issues which complicated management. This review describes some of these challenges, and presents management approaches that may increase the likelihood of satisfactory treatment outcomes. Situations which are reviewed include the diagnostic evaluation of the new patient with constipation; syndromes of uncertain pathophysiology including irritable bowel syndrome, slow transit constipation, and pelvic floor dysfunction; constipation associated with neurologic disorders including spinal cord injury, Parkinson's disease, and multiple sclerosis; and psychiatric issues which complicate the management of constipation, including recognition of associated psychiatric diseases, unusual attitudes toward bowel function, eating disorders, and referral for psychiatric care.

A restricted 'spotlight' of attention in visual object recognition.

We describe a patient (NJ), with a progressive visual disturbance, who showed an impairment in identifying larger visually-presented objects relative to their smaller counterparts. NJ showed this size effect for line drawings of objects, words and single letters. When presented with large letters comprised of smaller letters and asked to give speeded identification responses to either the large or small letters, NJ was grossly impaired at identifying the large letter. Additionally, when presented with a context meant to bias responding to either the large or small letter, NJ showed faster and more accurate responding in the small direction, but not in the large direction. We interpret these results as indicative of an impaired 'spotlight' of attention, which is deployed across the visual array, and is necessary for providing the selective visual attention responsible for the integration of visual features.

Breast cancer evaluation and follow-up: a survey of The Ohio Chapter of The American College of Surgeons.

Preoperative evaluation and postoperative follow-up of breast cancer patients vary considerably. Recent literature suggests that routine surveillance studies for breast cancer patients can be reduced without compromising the outcome. The Ohio State Chapter of The American College of Surgeons Committee on Cancer sponsored a survey of its general surgeon fellows to determine their practice philosophies regarding these issues. The questions centered around breast cancer screening, evaluation and treatment, and follow-up. The fellows were also questioned as to their opinions regarding practice parameters and whether the State Chapter should take a role in this area. Of the 764 surveys sent out, 34.2% were appropriate for evaluation. For breast cancer screening, 96.1% believe yearly mammography is important. Newly diagnosed breast cancer patients are generally evaluated with history and physical exam, chest X-ray, complete blood cell count, and liver function tests. Bone scans are used by 38.6% of surgeons. Most patients with positive lymph nodes see a medical oncologist. About half of primary breast cancer treatment (44.7%) is by breast preservation. Essentially all surgeons follow their patients after treatment for breast cancer surveillance. Essentially all surgeons feel that physical exam and mammograms are important for post-treatment follow-up. Complete blood cell count, liver function studies, and chest X-rays are used less commonly but still by more than half of the surgeons. 44.4% of the surgeons have found difficulty with third-party payers covering the costs of surveillance studies. 87.7% of surgeons surveyed felt the State Chapter should become involved in establishing clinical guidelines or practice parameters.

Maintenance of symptomatic remission in patients with Crohn's disease.

Crohn's disease is a chronic inflammatory intestinal disorder characterized in most patients by repeated episodes of diminished and exacerbated symptoms. Recent controlled trials demonstrated that oral preparations of 5-aminosalicylic acid decrease recurrence rates by approximately 40% when administered long-term to patients with quiescent Crohn's disease. Orally administered corticosteroids, sulfasalazine, metronidazole, azathioprine, and cyclosporine have not proved of benefit in the prevention of recurrences of Crohn's disease. Nonetheless, corticosteroids, metronidazole, and azathioprine can control chronically active disease. Methotrexate may have some benefit in the treatment of active Crohn's disease, but its role in maintenance of remission has not been investigated. Elimination diets seem to prolong periods of symptomatic remission. Further studies are needed to define subgroups of patients who are most likely to benefit from preventive therapy.

Nitric oxide mediates inhibitory nerve input in human and canine jejunum.

BACKGROUND: Nitric oxide (NO) may be an inhibitory neurotransmitter in the intestinal muscle. The present study examined its role in human and canine jejunum. METHODS: Mechanical and intracellular electrical activity were recorded simultaneously from the circular muscle layer. RESULTS: In the human jejunum, nerve stimulation inhibited mechanical activity and evoked an inhibitory junction potential that consisted of an initial fast hyperpolarization followed by a late sustained hyperpolarization. NO inhibited mechanical activity and evoked a dose-dependent hyperpolarization that mimicked the late hyperpolarization. In the canine jejunum, nerve stimulation inhibited mechanical activity and evoked an inhibitory junction potential that consisted of only a fast hyperpolarization. NG-Monomethyl-L-arginine and NG-nitro-L-arginine attenuated nerve-mediated inhibition of mechanical activity in both species. However, the effect of the synthase inhibitors on inhibitory junction potentials differed in the two species. In canine jejunum, both inhibitors reduced the amplitude of the initial fast hyperpolarization. In human jejunum, both inhibitors reduced only the late sustained hyperpolarization. CONCLUSIONS: NO mediates neural inhibition in circular muscle of both human and canine jejunum through different mechanisms.