Obstructive jaundice due to isolated tuberculosis of the pancreas: endoscopic treatment instead of surgery?

The optimal treatment for relieving biliary obstruction due to isolated pancreatic tuberculosis has not so far been defined, and most previously reported patients were treated surgically. We describe a 17-year-old, immunocompetent girl who was admitted with obstructive jaundice caused by a tuberculous mass in the head of the pancreas. Antituberculous therapy alone failed to alleviate the jaundice, and she was therefore treated by stent insertion and, subsequently, balloon dilation of the common bile duct stricture. At follow-up 5 years later, magnetic resonance cholangiopancreatography showed no evidence of stricture in the common bile duct.

Nutcracker oesophagus: a double-blind, placebo-controlled, cross-over study of the effects of lansoprazole.

BACKGROUND: Nutcracker oesophagus is characterized by high-amplitude oesophageal contractions. Recent data have shown a high prevalence of gastro-oesophageal acid reflux in patients with nutcracker oesophagus and, in open-label trials, patients seemed to benefit from acid suppression. Therefore, it has been suggested that non-cardiac chest pain in patients with nutcracker oesophagus may be related to reflux rather than to the motor abnormality itself. AIMS: To investigate the effect of intensive acid-suppressive treatment on chest pain in patients with nutcracker oesophagus. METHODS: Nineteen patients with nutcracker oesophagus received lansoprazole or placebo in a double-blind, randomized, cross-over study. RESULTS: Significant reductions in pain intensity (P < 0.006) and pain duration (P < 0.05) were registered during the study. The magnitude of symptom relief achieved with lansoprazole did not differ significantly from that achieved with placebo. The motility pattern did not change during the study. CONCLUSIONS: This study does not prove that acid-suppressive therapy is effective for pain relief in nutcracker oesophagus. As the amelioration of pain was not accompanied by any change in the nutcracker oesophagus pattern, it is unlikely that the high-amplitude oesophageal contractions are the cause of pain. Thus, the possible role of acid in the pathophysiology of pain in nutcracker oesophagus needs further study.

Recurrent pancreatitis as a late complication of endoscopic sphincterotomy for common bile duct stones: diagnosis and therapy.

Although long-term complications of endoscopic sphincterotomy (ES) have often been reported, the possible effects of the procedure on the pancreatic duct orifice remain virtually unknown. Three women patients are described who developed attacks of recurrent pancreatitis at 2, 7, and 27 months after ES for bile duct stones. The attacks were apparently caused by a partial stenosis of the pancreatic orifice, arising from post-procedure fibrosis and scarring. The patients were evaluated and treated endoscopically. All three patients benefited from endoscopic therapy; two became symptom-free and one improved. Symptoms recurred in the three patients, after 26, 41, and 23 months. In one patient, re-stenosis was documented and repeat sphincterotomy resulted in complete relief of symptoms during the ensuing 5 months. In the remaining two patients, the symptoms were mild and no further intervention was needed. Concerning complications, one patient suffered a moderately severe pancreatitis without sequelae. Recurrent pancreatitis may emerge as a late complication after ES for common bile duct stones. It is probably causally related to stenosis of the pancreatic orifice brought about by fibrosis and scarring, which may exceptionally develop after the procedure. Endoscopic therapy should probably be considered in the first instance, but the optimal treatment for this condition remains to be determined.

Diagnosis of nutcracker esophagus, segmental or diffuse hypertensive patterns, and clinical characteristics.

Different criteria have been used for the diagnosis of nutcracker esophagus, but the clinical importance of this is largely unknown. To this end, 45 patients with hypertensive esophageal peristalsis were identified from 572 esophageal investigations, and thereafter explored with regard to diagnostic criteria and clinical background data. The numbers of patients classified as having nutcracker esophagus with different criteria were 25 (by Richter criteria), 39 (by Achem criteria), and 45 (by Gothenburg criteria). In comparison with patients with segmental hypertensive peristalsis, patients with diffuse hypertensive peristalsis were more often referred for chest pain (P < 0.05), less often for reflux symptoms (P < 0.05), and tended to have arterial hypertension less often (P < 0.08). In conclusion, the choice of criteria for the diagnosis of nutcracker hypertensive peristalsis has prominent effects on the number of patients receiving this diagnosis. Moreover, a diffuse nutcracker peristaltic pattern appears more strongly linked to chest pain.

Longer term outcome of steroid refractory ulcerative colitis treated with intravenous cyclosporine without subsequent oral cyclosporine maintenance therapy.

BACKGROUND AND AIMS: Intravenous cyclosporine (Cy) is increasingly used in patients with severe ulcerative colitis who fail to respond to corticosteroids. However, in spite of subsequent oral Cy maintenance therapy almost one-half of the initial responders need colectomy within a year. In light of the drug's limited efficacy and potential toxicity use of oral long-term Cy can be questioned. PATIENTS AND METHODS: Nineteen patients with steroid refractory severe ulcerative colitis were treated intravenously with Cy. RESULTS: Of the 19 patients 14 (76%) achieved remission. Six of the patients (46%) remained in remission for 12-61 months. Eight patients experienced one to four flares during the year after treatment. However, except for one patient who needed another course of intravenous Cy, all responded to corticosteroids. The duration of remission since the last flare in these patients (five received azathioprin) was 10-36 months. None of the patients needed colectomy because of symptoms. CONCLUSION: These preliminary data suggest that a course of intravenous Cy can turn corticosteroid-refractory ulcerative colitis to corticosteroids responsive. The outcome of patients not receiving oral Cy maintenance therapy appears to be satisfactory. Azathioprin maintenance therapy can probably be reserved for select patients.

Gastroesophageal acid reflux in patients with nutcracker esophagus.

BACKGROUND: Hypertensive esophageal contraction, called nutcracker esophagus (NE), is the most common motility abnormality associated with cardiac-like chest pain. However, its significance for the development of symptoms has been a matter of controversy for decades, and recently it has been suggested that NE might represent a primarily acid-related esophageal disorder. The frequency of acid-related esophageal dysfunction is studied in an unselected group of patients with NE. METHODS: During the period March 1993 to June 1998, 572 consecutive patients underwent esophageal manometry and 24-h pH monitoring. RESULTS: A motility pattern consistent with NE was found in 45 subjects referred because of chest pain (n = 35), reflux dyspepsia only (n = 8) or epigastric pain (n = 2). Acid-related esophageal dysfunction was noted in 30 (70%) of the NE patients; abnormal acid exposure time (n = 21), esophagitis (n = 2) or positive symptom index (n = 7). In addition, an increased number of reflux episodes were found in another three subjects. NE was more prevalent in subjects referred for chest pain than in those referred for other symptoms (14.3% versus 4.5%; P < 0.0001) and, conversely, 78% of the patients with NE were referred because of chest pain. CONCLUSIONS: Various aspects of acid-related esophageal dysfunction occur frequently in patients with NE, suggesting that acid may play a role in the development of symptoms in NE. Nonetheless, given its association with chest pain, NE could be a marker of a subgroup of patients with acid reflux, distinct from other reflux patients.

Role of immunosuppressive therapy in refractory sprue-like disease.

Patients with malabsorption and histological findings consistent with celiac disease, who are unresponsive to gluten free diet, and in whom other causes of flat mucosa have been excluded, are considered to suffer from so called unclassified or refractory sprue. Although the true nature of this condition needs to be further elucidated, it is known to represent a difficult therapeutical problem with potentially fatal course. Herein, we report a patient with refractory sprue-like disease who after failing to respond to corticosteroids and TPN was in a critical condition. He responded promptly to cyclosporine and made a remarkable recovery. In contrast to previous reports, the cyclosporine treatment in this patient was pursued only for 1 month, whereupon the patient turned responsive to steroids. Subsequent treatment with azathioprine allowed corticosteroids to be reduced to a low maintenance dose and eventually all drugs could be discontinued without reappearance of symptoms. Cyclosporine therapy might be lifesaving in occasional patients with refractory sprue-like disease and it may result in reversal of steroid resistance. Moreover, azathioprine seems to have a steroid sparing effect in this setting. Short term immunosuppressive treatment may have an advantage of lower risk for drug related side effects.

Esophageal dysfunction in syndrome X.

Syndrome X is defined as anginal chest pain accompanied by objective signs of ischemia on exercise testing or myocardial scintigraphy, but with angiographically "normal" coronary arteries. The etiology of this enticing syndrome is still not known. Besides myocardial ischemia, esophageal dysfunction and visceral hypersensitivity may play a role in the development of pain. The purpose of this study was to study esophageal function and visceral sensitivity in patients with syndrome X. Twenty consecutive patients with the diagnosis of syndrome X were investigated with esophageal manometry and a 24-hour pH recording. Visceral esophageal sensitivity was explored by balloon distention of the distal esophagus, as well as by instillation of acid. Twelve patients (67% of the 18 evaluated) had some abnormality on 24-hour pH monitoring; 2 had abnormal global acid exposure time (pH <4) and 7 had symptoms coincidental with episodes of pH <4. Seven patients (35%) had esophageal dysmotility including 5 with the "nutcracker" esophagus. Esophageal hypersensitivity to acid (n = 9) or distention (n = 13) was seen in 14 of the 20 patients. Eleven patients received acid suppressive therapy that resulted in amelioration of chest pain in 8 (73%). Thus, results suggest that esophageal hypersensitivity rather than gross functional abnormality is an important factor for the development of chest pain in patients with syndrome X, and that acid in the context of a hypersensitive esophagus is the main culprit. Acid suppression may ameliorate pain in a substantial proportion of patients.

Esophageal visceral pain sensitivity: effects of TENS and correlation with manometric findings.

Increased esophageal visceral sensitivity has been suggested to be an important factor in the development of esophageal chest pain. Transcutaneous electrical nerve stimulation (TENS) has been found effective in the treatment of visceral heart pain in severe angina pectoris, but its effect on esophageal pain perception is not known. In this study, we used the method of graded intraesophageal balloon distension to study the effects of TENS on esophageal motility and pain sensitivity. In addition, we explored the relationship between manometric findings and esophageal susceptibility to pain. TENS reduced symptoms during balloon distension significantly and decreased peristaltic velocity. Increased visceral perception was positively correlated to the amplitude and duration of the esophageal peristalsis. This study suggests a correlation between increased peristaltic waves and visceral perception in the esophagus. TENS appears to reduce esophageal pain sensitivity and thus may be a useful treatment for noncardiac chest pain of esophageal origin.

Endoscopic bile duct stent placement as a predictor of outcome following endoscopic sphincterotomy in patients with suspected sphincter of Oddi dysfunction.

OBJECTIVES: To determine whether symptomatic improvement following placement of endoscopic stent across the biliary sphincter could predict the longer-term clinical outcome after endoscopic sphincterotomy (ES). METHODS: Twenty-three post-cholecystectomy patients with suspected sphincter of Oddi dysfunction underwent, sequentially, sphincter of Oddi manometry, endoscopic stent placement, ES, and follow-up for a further 6-12 months. RESULTS: Eight (35%) patients either did not respond (n = 5), did not tolerate the stent (n = 1) or relapsed during stenting (n = 2). Only the patient who did not tolerate the stent from the outset (12%) improved after ES. Of five patients who responded to stenting and had ES within 8 weeks, only two (40%) remained asymptomatic. In contrast, of 10 patients who were pain-free during 12-14 weeks of stenting, nine (90%) continued to be asymptomatic after ES. All seven patients with an elevated sphincter of Oddi pressure responded to stenting and six benefited from sphincter ablation. Five (31%) of 16 patients who had normal sphincter pressure and had improvement after 12-14 weeks of stenting remained free from pain following ES. ES resulted in long-term freedom from pain in 12 of the 23 patients: six of the seven patients with elevated sphincter of Oddi pressure and six of the 16 subjects with normal manometry (P < 0.05). CONCLUSION: Freedom from symptoms during at least 12 weeks of stenting predicted a favourable outcome after ES, irrespective of sphincter of Oddi pressure. Patients who failed to improve or showed improvement only with short-term stenting were less likely to benefit.

Post-cholecystectomy patients with "objective signs" of partial bile outflow obstruction: clinical characteristics, sphincter of Oddi manometry findings, and results of therapy.

Seventeen patients, 15 women and 2 men with upper abdominal pain categorized to biliary group I suspected sphincter of Oddi dysfunction, underwent thorough clinical evaluation including ERCP and endoscopic sphincter of Oddi manometry. All the patients were subsequently treated by endoscopic (n = 15) or surgical (n = 2) sphincterotomy. The mean age of patients studied was 62 years. They all had cholecystectomy several years previously (median, 18 years). At ERCP all patients showed a large common bile duct dilation (mean diameter, 18 mm) and delayed contrast drainage greater than 45 minutes. Sphincter of Oddi manometry, although abnormal in most patients, revealed normal sphincter of Oddi pressure in six patients (35%). Nonetheless, sphincterotomy resulted in relief of symptoms in all (mean follow-up, 28 months; range, 3 to 46 months), thus suggesting that their pain was indeed caused by the disordered sphincter of Oddi. These findings support the previous assumption that in symptomatic post-cholecystectomy patients the triad of elevated liver function tests, dilated common bile duct, and delayed contrast drainage at endoscopic retrograde cholangiopancreatography indicates a definitive sphincter of Oddi abnormality. The normal sphincter of Oddi pressures in many of these patients are probably due to a different, noncompliant type of sphincter of Oddi dysfunction. Because these patients invariably benefit from sphincterotomy, sphincter of Oddi manometry in this category is not only unnecessary, but it may also be misleading.

Effect of midazolam on sphincter of Oddi motility.

Midazolam is a more recent benzodiazepine used for sedation during endoscopic procedures, including sphincter of Oddi (SO) manometry. However, the effect of this drug on the human SO has so far not been studied. In this paper we explored the effect of midazolam on human SO motility by means of endoscopic manometry. Twelve patients with suspected SO dysfunction were investigated. We found that in patients with normal manometry findings, midazolam had no effect on the sphincter motility. In contrast, in all patients with elevated SO pressure (SOP), as well as in one of the three patients with borderline SO tone, midazolam (2.5 mg i.v.) produced a relaxatory effect. Due to this effect the final readings were affected in three out of five patients, i.e. the abnormally elevated SOP decreased to a borderline level in two and in the remaining patient the borderline level in two and in the remaining patient the borderline SOP decreased to normal. In one other patient with a markedly elevated SOP the additional injection of 2.5 mg of midazolam caused a further decrease in SOP to a borderline level. We conclude that midazolam, due to its relaxatory effect on SO, appears to be a less suitable sedative for SO manometry.

Endoscopic treatment of anastomotic strictures in Crohn's disease.

The results of endoscopic balloon dilatation in 27 patients with anastomotic strictures caused by Crohn's disease are reported. All patients had obstructive symptoms not responding to corticosteroids and of such a degree that resection or strictureplasty was considered necessary. The patients were followed up for 7 to 38 months after their first dilatation. Eighteen patients were more or less free from obstructive symptoms while the results were less favourable in the remaining nine patients. The median time after the latest surgical intervention was seven years in the group with a successful outcome and 1.6 years in the group with a poor outcome, which may indicate that some patients have a more aggressive form of Crohn's disease where balloon dilatation is only of moderate value. However, all patients experienced at least a temporary effect, so that balloon dilatation can be considered for use in situations where it is desirable to postpone surgery.

Prevalence of primary sclerosing cholangitis in patients with ulcerative colitis.

All patients greater than or equal to 16 years old with a diagnosis of ulcerative colitis were identified in five well-defined catchment areas, representing 12.7% of the Swedish population. Exactly 1500 patients were retrieved, giving a point prevalence of 170/10(5) inhabitants. It was possible to obtain liver function test results less than 2 years old in 94% of the patients and to obtain endoscopic retrograde cholangiographic results in 65 of the 72 patients with abnormal serum alkaline phosphatase values. Primary sclerosing cholangitis was diagnosed in 55 of the patients (3.7%). The prevalence of the disease was 5.5% in patients with substantial colitis and 0.5% in patients with distal colitis. There was a marked male predominance in cholangitis patients compared with colitis patients without cholangitis. Ninety-five percent of the patients with cholangitis had substantial colitis, which was more than the 62% of patients without cholangitis who had colitis. Female patients with cholangitis were older than male patients at the time of diagnosis of both cholangitis and colitis, which contrasted to the equal age at diagnosis of colitis in male and female patients without cholangitis.