[Slowly regressing acute pandysautonomia associated with esophageal achalasia: a case report]. / Pandysautonomie aigue lentement regressive associee a une achalasie de l'oesophage: a propos d'un cas.

Acute pandysautonomia is a rare acute autonomic neuropathy that mainly affects young women. We report a case of idiopathic acute pandysautonomia associated with an esophageal achalasia in a 30-year-old woman. The clinical features were inaugural dysphagia followed by signs of parasympathetic failure of the entire digestive tract, bladder and pupils. Twenty-four hours of electrocardiographic recording showed involvement of sympathetic adrenergic nerves. Esophageal achalasia was patent on esophageal manometry. Upper digestive tract motility was first involved and then extended to the entire digestive tract with intestinal obstruction, which required emergency ileostomy. Recovery of autonomic functions was slow. After 16 months, dysphagia and gut paresis improved and digestive continuity was restored. In case of subacute intestinal pseudo-obstruction associated with autonomic dysfunction, acute pandysautonomia should be suspected. In our report, the association with esophageal achalasia is uncommon.

[Long-term evolution of achalasia of the lower esophageal sphincter and intestinal paraneoplasic pseudo-obstruction revealing small cell lung carcinoma]. / Evolution à long terme d'une achalasie du sphincter inférieur de l'esophage et d'une pseudo-obstruction intestinale paranéoplasiques révélatrices d'un carcinome pulmonaire à petites cellules.

We report a case of a small cell carcinoma of the lung revealed by chronic intestinal pseudo-obstruction associated with achalasia of the lower esophageal sphincter. Tumoral remission was achieved for more than 21 months after chemoradiotherapy but this did not prevent the paraneoplasic syndrome from persisting and medical treatment was not successful in treating the intestinal pseudo-obstruction or the dysphagia, which was not improved by esophageal dilation.

Tioguanine in patients with Crohn's disease intolerant or resistant to azathioprine/mercaptopurine.

BACKGROUND: Tioguanine (TG) is an antimetabolite which may be regarded as an alternative to azathioprine (AZA)/mercaptopurine (MP) in patients with inflammatory bowel diseases. AIMS: : To evaluate the tolerance and efficacy of TG in patients with Crohn's disease, intolerant or resistant to AZA/MP. METHODS: An open prospective study was made on Crohn's disease patients treated with TG. Intolerance to AZA/MP was defined as a reaction occurring within 1 month after introduction of AZA/MP, including pancreatitis, abdominal pain, fever, arthralgia, myalgia, cutaneous rash, fatigue, alopecia, hepatitis and digestive intolerance. Resistance to AZA/MP was defined as the persistence of activity after at least 3 months of AZA/MP therapy. RESULTS: Forty-nine Crohn's disease patients (36 women, 13 men; intolerance: n = 39; resistance: n= 10) were treated with TG (20 mg/day). Clinical pancreatitis did not recur under TG. Five patients (10%) had to stop TG due to intolerant reactions observed 13-21 days after TG was started. No haematological side-effects were observed under TG. The probability of clinical remission without corticosteroids or infliximab at 6 and 12 months was 46% and 79%, respectively, in the 40 patients with active disease at baseline. The probability of clinical relapse during maintenance TG therapy at 6 and 12 months was 29% and 53%, respectively, in the 28 patients in remission at baseline or who had achieved remission on TG. CONCLUSIONS: TG is a possible alternative treatment in Crohn's disease patients, intolerant (especially for pancreatitis) or resistant to AZA/MP.

Evolution of clinical and radiological features at diagnosis of achalasia during a 19-year period in central France.

OBJECTIVES: Symptoms of achalasia are often misinterpreted, resulting in delayed diagnosis. The aims of our study were (1) to estimate the evolution of clinical and radiological features of a large population of achalasic patients between two successive periods; and (2) to determine the influence of symptoms on diagnostic delay. METHODS: Between 1980 and 1998, all achalasia patients treated in our unit were assessed at the time of manometric diagnosis for clinical and radiological features. These data were compared between two successive periods (1980-1994 and 1994-1998). Then, a correlation between the diagnostic delay, clinical and radiological data and symptoms was investigated. RESULTS: Three hundred and forty-five consecutive achalasia patients were assessed (mean age at diagnosis, 56 years; mean diagnostic delay, 5.7 years). The duration of the disease was correlated with the oesophageal diameter (P = 0.0001). Dysphagia, chest pain and heartburn were more frequent in young patients (respectively, P = 0.003, 0.0001 and 0.001). Women had 1.7 times the risk of men for suffering of chest pain (95% CI, 1.1 -2.6) and 2.2 times the risk for heartburn (95% CI, 1.2-4.0). Pulmonary involvement was more frequent when the oesophagus was dilated (P = 0.0002), and 3.4 times more frequent when associated with regurgitations (95% CI, 1.3-8.9). The oesophageal diameter was significantly smaller (38 vs 48 mm) in the last period, but we have not observed any significant shortening of the diagnostic delay. No symptoms influenced the diagnostic delay. CONCLUSIONS: Despite a smaller oesophageal diameter at the time of diagnosis, during the period 1994-1998, diagnostic delay was not reduced. No clinical features associated with late diagnoses could be identified.

Risk factors for immediate complications after progressive pneumatic dilation for achalasia.

OBJECTIVE: The aim of this retrospective study was to determine the risk factors of early complications after progressive pneumatic dilation for achalasia. METHODS: Five hundred four dilations were performed in 237 consecutive achalasic patients between 1980 and 1994 with the same technique: low-pressure pneumatic dilation every other day with balloons of increasing diameter until success criteria were obtained (depending on manometric examination and esophageal x-ray transit performed 24 h after each dilation). Clinical, radiographical, endoscopical, and manometrical data as technical procedure characteristics for patients with perforations or other early complications were compared with those without complications. RESULTS: We observed 15 complications (6% of patients): 7 perforations (3%), 3 asymptomatic esophageal mucosal tears, 4 esophageal hematomas, and 1 fever. Perforations occurred in 6 of 7 patients during the first dilation. The mean age was 68.5 yr in the group with complications versus 56.4 yr for the remainder (p < 0.05). Two deaths occurred in patients older than 90 yr. Instability of the balloon during dilations was noted in 8 of 15 cases of complications versus 57 of 222 patients (p < 0.05). No other data differed significantly. CONCLUSIONS: This study showed a low prevalence of early complications using this progressive technique. Patients with hiatal hernia, esophageal diverticulum, or vigorous achalasia may safely undergo progressive pneumatic dilation. Only patients older than 90 yr should be referred for progressive pneumatic dilation with caution. Most of perforations arose during the first dilation, but there was no predictive pretherapeutic factor of perforation.

Esophageal swallowing phase assessed by audiosignal recording: relationship with manometry in gastroesophageal reflux disease patients.

The acoustic technique has been used for pharyngeal exploration but to date no such technique has been devised to assess esophageal motility. The aim of this study was to demonstrate that displacement through the esophagus can be quantified using this method in healthy subjects and in patients with gastroesophageal reflux. Concurrent manometric and acoustic recordings were also performed in the patients. Fifteen controls (38.5 +/- 13 years old) and 10 patients (34.9 +/- 6 years old) were included. All were recorded during wet and dry swallow sequences with microphones placed below the cricoid cartilage and on the xiphoid appendix. Standard manometry was performed for lower esophageal sphincter (LES) exploration. For the acoustic technique, the frequency of xiphoid signals (FX), esophageal transit time (ETT), duration of xiphoid sound (SD), and for the manometric study, the duration of LES relaxation (RD) were recorded and mean values were calculated (FXm), (ETTm), (SDm), (RDm). FXm for wet (94 vs 81.6%) and dry swallows (86 vs 66.6%) decreased in patients. ETTm was significantly higher (P < 0.01) for wet than for dry swallows (5.6 +/- 0.9 vs 5.2 +/- 1.2 sec) for controls but not for patients. ETTm was significantly higher for patients for wet (7.2 +/- 2.1 sec) and for dry swallows (6.5 +/- 2.3 sec) than for controls and SDm was lower. Xiphoid sound appeared in the second half of LES relaxation. Our noninvasive acoustic technique is simple and reproducible. It is well correlated with manometry, and it allows characterization of the displacement of the bolus through the esophagus and the LES. The technique could be used alone to determine appropriate pharmacological and surgical treatments for esophageal motility disorders.

[Hepatic artery ligation for liver metastasis of a acinar cell carcinoma of the pancreas revealed by a nodular panniculitis]. / Ligature de l'artère hépatique pour métastases hépatiques d'un carcinome à cellules acineuses du pancréas révélé par une panniculite nodulaire.

We report the case of a 67-year-old-man presenting with pancreatic acinar cell carcinoma revealed by dermatological manifestations of cytosteatonecrosis and treated by hepatic artery ligation. The pancreatic etiology of these lesions was suspected due to hyperlipasemia, and was confirmed by abdominal computerized tomography showing a pancreatic tumor and multiple liver nodules, and by histological examination of one of these lesions. Because of symptomatic treatment failure, rapid impairment of patient's general condition, and by analogy with the treatment of hepatic metastases of neuroendocrine tumors, hepatic artery ligation was performed. Lipasemia decreased markedly and symptoms disappeared for 45 days. Hepatic artery obstruction may be used for emergency treatment of secreting liver metastases.

[Low-grade gastric MALT lymphoma and helicobacter heilmannii (Gastrospirillum hominis]. / Lymphome gastrique de bas grade du MALT et Helicobacter heilmannii (Gastrospirillum hominis).

We report the fourth case of low-grade gastric MALT lymphoma associated to Helicobacter heilmannii. This spiral organism may be present in the gastric mucosa of animals where it is non pathogenic while, in humans, its presence is always associated with chronic gastritis. In this case, Helicobacter heilmannii was observed in the absence of Helicobacter pylori. Regression of endoscopic and histological lesions after Helicobacter heilmannii eradication suggests its role in gastric lymphoma. This observation underlines the need for searching for Helicobacter heilmannii by careful histological examination, in the absence of Helicobacter pylori, and the importance of its eradication in the treatment of gastric lymphoma.

[Myotomy for esophageal muscular hypertrophy with eosinophil infiltration of the esophagus associated with toxocariasis revealed by esophageal motor disorder]. / Myotomie pour hypertrophie musculaire avec infiltration à éosinophiles de l'oesophage associée à une toxocarose et révélée par un trouble moteur oesophagien.

We report the case of a 61-year-old-man with an eosinophilic esophagitis with esophageal motor disorder associated with toxocariasis. He complained of non cardiac chest pain and had eosinophilia leading to the detection of Toxocara canis infection. Pain persisted despite treatment of toxocariasis. Basal manometry was normal but ambulatory 24-hour manometry-pHmetry showed diffuse esophageal spasm. Ultrasonography showed a thickening of the esophageal musculature in the two inferior thirds of the esophagus. After failure of treatment with sodium cromoglycate steroids and esophageal dilatation, calcium antagonists were partially effective. A long esophageal myotomy was performed permiting the disappearance of symptoms. The histological examination of a side myotomy biopsy showed an eosinophilic infiltration of the esophageal muscle layer. This observation leads to discuss the possible relation between toxocariasis, the esophageal motor disorder and the eosinophilic infiltration of the esophageal muscle layer.

[Complete remission of a mesenteric fibromatosis after taking sulindac]. / Régression complète d'une fibromatose mésentérique après prise de sulindac.

We report the case of a 22-year-old-man having a familial adenomatous polyposis coli treated by total colectomy with ileo-rectal anastomosis. Two years after the operation, an asymptomatic mesenteric fibromatosis appeared which was nonresectable due to mesenteric vessels infiltration. Nine years later, sulindac therapy was started for residual polyps in the rectal stump. This treatment was taken intermittently, during periods of 1 to 8 months, for 6 years. After 4 years of treatment, the tumor was no longer palpable. Four years after sulindac discontinuation, the patient was operated on for suspicion of intestinal adhesion. The mesenteric fibromatosis had completely disappeared and mesenteric vessels were free. This complete macroscopic regression of a desmoid tumor after sulindac therapy emphasizes again the interest of this treatment for mesenteric fibromatosis.

Achalasia: outcome of patients treated with intrasphincteric injection of botulinum toxin.

BACKGROUND: To evaluate the safety and clinical efficacy of botulinum toxin (BT) in patients with achalasia followed up for six months. METHODS: Fifty five symptomatic patients with manometrically proven achalasia were included in a multicentre prospective trial. Before and two weeks and two months after intrasphincteric injection of BT, symptoms of dysphagia, regurgitation, and chest pain were scored on a 0-3 scale, and lower oesophageal sphincter pressure (LOSP) was assessed. The symptom score was determined again at six months, clinical improvement being defined by < or = 3, relapse by > 3, and failure as a relapse after two injections or loss to follow up. RESULTS: Except for transient chest or epigastric pain (22%), no side effects were observed. There was a significant decrease in LOSP after treatment. Symptom scores were significantly improved at two weeks (2.0 (SD 1.6)), two months (1.7 (1.8)), and six months (1.9 (2.0)) compared with pretreatment values (5.1 (1.8), p < 0.001). At six months, 33 patients had clinical improvement (27 after one injection), 17 were considered failures, and five had just relapsed. Although there was a trend for age (older patients being more responsive), age, sex, prior duration of symptoms, initial symptom score, weight loss, LOSP, magnitude of oesophageal contractions, vigorous or non-vigorous achalasia, previous dilatations, and radiological features were not predictive of results. CONCLUSIONS: This multicentre series confirms that intrasphincteric injection of BT is a safe procedure, resulting in clinical improvement in 60% of patients with achalasia at six months. The therapeutic role of BT in achalasia needs further evaluation with regard to other alternatives.