Abdominal lymphomas, convulsive seizure and coma: a case of successfully treated, advanced Whipple's disease with cerebral involvement

Whipple's disease is a rare, generalized inflammatory disorder due to the recently described bacterium Tropheryma whippelii. We report an unusual, successfully treated case of a 32-year-old woman, who presented with a 25 month history of large abdominal lymphomas, polyserositis and cachexia. The diagnosis of Whipple's disease was confirmed by duodenoscopy, lymph node and duodenal histology and polymerase chain reaction analysis of biopsy material and cerebrospinal fluid. A prolonged convulsive seizure with a subsequent 5 day period of coma were interpreted as signs of cerebral involvement. Under antibiotic treatment with trimethoprim-sulfamethoxazole (co-trimoxazole) the patient recovered completely, CT scans showed a complete regression of abdominal lymphomas. The therapy was continued over 18 months without the occurrence of a relapse.     

A newly recognized entity: intraductal "oncocytic" papillary neoplasm of the pancreas

Intraductal papillary-mucinous tumors of the pancreas are increasingly recognized, and their characteristic endoscopic and radiological features are well reported in the literature in recent years. Oncocytic features in these tumors are uncommon and unrecognized. Intraductal oncocytic papillary neoplasm is a distinct pancreatic tumor and is a recently recognized entity. We report a case of a 69-yr-old patient who presented with symptoms mimicking pancreatitis, resulting in delay in the diagnosis of her pancreatic tumor. She underwent a successful Whipple's procedure and subsequently has remained well. The resected specimen showed an intraductal oncocytic papillary-mucinous neoplasm. The entity is new and the literature information is inadequate at present to judge the biological behavior of this tumor. We discuss this recently recognized entity.     

Duodenal duplication in adults. Laparoscopic treatment

The authors report a case of duodenal duplication in a adult. This lesion is rare. The clinical course is dominated by a risk of cancer. These lesions should be recognized to avoid a mutilating excision procedure in favour of a conservative procedure. The originality of this case is the laparoscopic approach which confirmed the diagnosis by the elective implantation on the first part of the duodenum. This approach, by mobilisation of the duodenum, allows excision by a very short laparotomy.     

Modified duodenum-preserving resection of the head of the pancreas

A modified procedure of duodenum-preserving resection of the head of the pancreas was used in treating three patients with benign lesions in the head of the pancreas. Compared with Beger's procedure, the modified procedure was simpler, and pancreaticojejunostomy on duodenal side was not necessary because of less remaining pancreas attached to the conjunction of the pancreatobiliary duct. The patients, recovered and ate food from 4 to 6 days after operation without symptoms of duodenal obstruction. The patient's digestive function was normal, and the pre- and postoperative pancreatic endocrine function was almost identical. This procedure can be used as the first-choice surgical procedure for benign lesions in the head of the pancreas. Problems in how to protect blood supply of the duodenum were also discussed.     

Transgastric endoluminal laparoscopic resection of a duodenal polyp

Transgastric endoluminal surgery is a laparoscopic procedure used to treat gastric lesions such as polyps and carcinoma in situ. This approach can also be used to treat proximal duodenal problems such as bleeding and polyps. Our case was a young patient with bleeding from a benign duodenal polyp, localized 3 cm from the papilla of Vater. Two trocars were introduced by laparoscopy into the stomach. Guided by the flexible gastroscope, the polyp (a 6-cm Brunner's benign hamartoma) was resected by means of the endostaplers. Transgastric endoluminal surgery can be used to extirpate benign lesions in the proximal duodenum. The use of a flexible gastroscope can be very useful because of its adaptability to the anatomy.     

PCR analysis of T. whippelii DNA in a case of Whipple's disease: effect of antibiotics and correlation with histology

A 58-yr-old man developed severe weight loss, arthralgias, and diarrhea. Endoscopic examination of the stomach and duodenum revealed thickened folds of duodenal mucosa. Biopsy of the gastric mucosa was negative, whereas duodenal biopsy revealed blunted epithelial villi and PAS-positive foamy macrophages within the lamina propria. Bacilli typical of those associated with Whipple's disease were found by electron microscopy. The diagnosis was confirmed by polymerase chain reaction (PCR) assay, which detected a portion of the 16S ribosomal RNA gene sequence corresponding to the Whipple bacillus (Tropheryma whippelii) in duodenum, stomach, and liver biopsies before therapy. T. whippelii DNA was eliminated from all tissues tested within 3 months of starting antibiotic treatment, but the histological improvement lagged behind the clinical and molecular evidence of improvement.     

Comparative effects of enteric-coated pancreatin microsphere therapy after conventional and pylorus-preserving pancreatoduodenectomy

BACKGROUND: A comparative study was performed between patients with exocrine pancreatic insufficiency after conventional pancreatoduodenectomy (Whipple's procedure) and pylorus-preserving pancreatoduodenectomy (PPPD). In these patients the pharmacodynamics of 2-mm enteric-coated pancreatin microspheres (ECPMs) and their gastric transit time in relation to that of a solid meal were investigated. The efficacy of ECPM preparations may differ after Whipple's procedure compared with PPPD, because the latter procedure does not include gastrectomy. METHODS: Gastric transit was assessed by double-isotope scintigraphy. A pancake meal was labelled with 99mTc. ECPMs were cold-labelled with 170Er and neutron activated shortly before ingestion to enable imaging with a gamma camera. Intraluminal pancreatic enzyme activity was assessed during a 6-h period with two indirect tests: the cholesteryl [14C]octanoate breath test and the N-benzoyl-L-tyrosyl-p-aminobenzoic acid-p-aminosalicylic acid (NBT-PABA-PAS) test. RESULTS: In patients who had Whipple's procedure, the gastric transit time of ECPMs and of the pancake meal was not significantly different. The outcome of the indirect pancreatic function tests during enzyme supplementation was comparable, and not significantly different, from that in healthy volunteers. In patients who had PPPD, however, the gastric transit time of microspheres was greatly delayed compared with that of the pancake meal (P < 0.05). Improvement in the outcome of the indirect pancreatic function tests during enzyme supplementation was much less and remained well below that of healthy volunteers (P < 0.05). CONCLUSION: In cases of exocrine pancreatic insufficiency after Whipple's procedure, 2-mm ECPM treatment adequately restores pancreatic enzyme activity. Following PPPD, however, ECPM treatment is often ineffective because the microspheres are retained in the stomach. In these patients, use of conventional powdered pancreatin enzyme preparations may improve the efficacy of treatment.     

Whipple's disease: results of long-term follow-up

AIM: To analyze clinical, diagnostic and therapeutic aspects of Whipple's disease. MATERIALS AND METHODS: Diagnostic and therapeutic data are available for 7 patients registered in 1990-1997. The diagnosis was made using intestinoscope SIF-10L ("Olympus"). Biopsies were obtained from the jejunal, duodenal and gastric mucosa. The patients received tetracycline, erythromycin, biceptol, on demand--prednisolone. RESULTS: Whipple's disease was diagnosed 6 years on the average following the first clinical symptoms. In most patients the intestinal symptoms were preceded or accompanied by such extraintestinal symptoms as enlargement of the lymph nodes, lowering of hemoglobin, hypoproteinemia, ESR increase to 40-60 mm/h. To study biopsies from the distal duodenum is the only measure needed for diagnosis of Whipple's disease. In untreated patients PAS-positive macrophages are detectable also in gastric body mucosa. The immediate treatment outcome is favorable. The recurrence was observed only in one patient who had given up taking tetracycline. CONCLUSION: Long-term antibacterial therapy brings the recovery. Primary disorder of the cellular immunity responsible for the disease onset necessitates long-term follow-up of the patients. Control biopsy should be examined once a year.     

Whipple's syndrome (uveitis, B27-negative spondylarthropathy, meningitis, and lymphadenopathy) associated with Arthrobacter sp. infection

OBJECTIVE: To report an unusual case of Whipple's disease, including uveitis, seronegative spondylarthropathy, meningitis, and lymphadenopathy, associated with an Arthrobacter sp. infection. DESIGN: Interventional case report. PATIENT AND INTERVENTION: A 60-year-old white man presenting with severe chronic uveitis and systemic inflammatory manifestations was treated efficiently for Whipple's disease after histopathologic analysis of vitreous and inguinal adenopathy biopsy specimens. The authors performed a retrospective, laboratory-based evaluation of stored tissue specimens. MEASUREMENTS: Molecular analysis based on 16S ribosomal RNA gene amplification was applied to pretreatment biopsy specimens of inguinal lymph node to identify a causative bacterial agent. RESULTS: Tropheryma whippelii genome was not detected in these specimens. However, an amplification product was obtained after the first polymerase chain reaction run and subsequently was sequenced. It corresponded to an Arthrobacter sp., a gram-positive agent presenting diagnostic patterns and therapeutic management similar to those of Whipple's disease caused by T. whippelii. CONCLUSION: The absence of T. whippelii identification by molecular amplification during a clinically and histologically oriented Whipple's syndrome should not rule out the diagnosis. Arthrobacter infection may represent a new bacterial etiology of systemic inflammatory disorders involving the eye and associated with periodic acid-Schiff-positive inclusions.     

Carcinoid tumor in the retroperitoneal space

Authors present a rare case of the pancreatic carcinoid. Diagnosis was based on clinical examination, ultrasonography, CT, and histopathological evaluation. The patient was operated on twice: (Whipple's procedure with the right hemicolectomy and then Roux-Y anastomosis). During follow-up the liver metastases appeared. The patient was treated with chemotherapy. She died 19 month after operation because of neoplastic dissemination.     

A case report of resected primary duodenal carcinoma associated with early gastric cancer and cumulative results at 21 institutions in Japan

A case of primary duodenal carcinoma simultaneously associated with an early gastric cancer is reported. A 72-year-old woman complaining of appetite loss and nausea was admitted in June 1988. Endoscopic examination showed an ulcerative lesion in the angle of the stomach and a Borrmann type 2 tumor in the bulb of the duodenum. Both lesions were revealed to be adenocarcinomas by histological examination of obtained biopsy specimens. Synchronous carcinoma was diagnosed and pancreatoduodenectomy and lymph node dissection were performed. The primary tumor of the duodenum was histologically a moderately differentiated adenocarcinoma, and the gastric cancer was a tumor limited to the mucosa. Metastasis was recognized in a regional lymph node (No. 14A). There has been no recurrence during the 4-year postoperative follow-up period. This result suggests that pancreatoduodenectomy with systematic regional lymph node dissection can greatly contribute to prolonging the survival of patients with advanced duodenal cancer. This case is very rare, in that curative operation was performed for a primary duodenal carcinoma simultaneously associated with an early gastric cancer.     

Severe pulmonary hypertension reversed by antibiotics in a patient with Whipple's disease

The case is described of a 58 year old man with systemic Whipple's disease with pericardial and pleural effusions and severe pulmonary hypertension. After three months of antibiotic treatment there was a complete resolution, not only of the symptoms known to be associated with Whipple's disease (diarrhoea, arthralgia, pericardial and pleural effusions), but also of pulmonary hypertension.     

Nonsteroidal anti-inflammatory drug induced duodenal web

Duodenal webs represent an unusual cause of intestinal obstruction in adults. These anomalies are generally considered to be congenital in origin and usually present in infancy. However, they occasionally become symptomatic in adulthood. In these cases, because of the delay in symptoms, the etiology of duodenal webs in adults is uncertain. Gastrointestinal webs in adults have also been reported in the small intestine and colon. It is generally accepted that these lesions are an acquired defect related to long term nonsteroidal anti-inflammatory drug (NSAID) use. We report a patient with a history of long term NSAID use who presented with symptoms of gastric outlet obstruction due to the presence of a duodenal web.     

Symptomatic cardiomyopathy as a presentation in Whipple's disease

A patient presenting with congestive cardiac failure and anaemia underwent investigation which led to the diagnosis of Whipple's disease, associated with dilated cardiomyopathy. Conventional antibiotic therapy for Whipple's disease resulted in resolution of the traditional features of Whipple's disease and a marked improvement in the patient's heart failure.     

Villous tumors of the duodenum

Villous tumors of the duodenum are extremely rare, only 41 cases having been described in the world literature. Modes of presentation were: gastrointestinal bleeding (11 cases); obstructive jaundice (9 cases); duodenal obstruction (10 cases); vague abdominal discomfort (8 cases) and as an incidental finding on barium study of the upper gastrointestinal tract (2 cases) or at autopsy (1 case). Twelve of the 42 cases were associated with invasive adenocarcinoma. These were confined to patients over 50 years of age. Benign tumors should be treated with local excision while in those harboring adenocarcinoma pancreaticoduodenectomy is the treatment of choice. Endoscopy and biopsy should assume a major role in attempting to obtain an accurate preoperative diagnosis. Caution is advised in that the superficial portions of the tumor may appear benign while deeper portions may contain invasive adenocarcinoma. An additional case with a bizarre presentation is described and the literature reviewed.     

Musculocutaneous nerve palsy following traumatic spinal cord injury

Whipple's disease is a multisystem bacterial disease usually characterized by malabsorption, diarrhea and polyarthritis. Ocular manifestations include uveitis, vitreitis, retinis, myositis, papilledema and optic atrophy. We report a case of a chronic bilateral vitreitis in a 63 year-old man who had been treated for a Whipple's disease with gastrointestinal involvement 30 years before. The jejunal biopsy was negative but the polymerase-chain-reaction (PCR) revealed the presence of Trophyrema Whippelii in the vitreous prelevement of both eye. This new, original strategy of PCR is specific and more sensitive than histological diagnosis.     

Spondylodiscitis caused by Tropheryma whippelii

We describe the first case of spondylodiscitis caused by Tropheryma whippelii in which this so far unculturable organism was shown to be present at the site of infection in a patient without significant gastrointestinal symptoms. The methods used included broad-range PCR amplification with universal primers complementary to constant sequences of the gene coding for 16S rRNA, direct sequencing of the amplified fragment, and comparison of the sequence determined with those deposited in sequence databases. In addition to demonstrating the presence of this organism in the affected vertebral body, we found in our patient that the specific PCR is more sensitive than histology for detecting Whipple's bacilli in bowel biopsy specimens. Because histology of small bowel biopsies from the duodenum were-in contrast to PCR from the same site-not diagnostic for Whipple's disease in our patient, we recommend PCR whenever Whipple's disease has to be excluded.     

Duodenum-preserving resection of the pancreatic head for mucinous ductal ectasia without overt carcinoma

BACKGROUND/AIMS: The clinical characteristics of mucinous ductal ectasia (MDE) of the pancreas without overt carcinoma have not been clarified. To clarify MDE and assess the optimal treatment procedure, including the technique of duodenum-preserving resection of the pancreatic head (DpRPH), we studied four patients. METHODOLOGY: Our patients consisted of three men and one woman, with a mean age of 71 years. The patients underwent DpRPH (n=3) or the pylorus-preserving Whipple procedure (PpW) (n=1). Clinicopathological features, postoperative pancreatic function, and technique to preserve duodenal blood flow were studied. RESULTS: All patients had intraductal mucin-hypersecretion and multilocular cysts lined by hyperplastic epithelium. The lesions were located in the uncinate process (n=3) or head-body (n=1) of the pancreas. DpRPH totally removed the lesions in the uncinate process. Of the three patients receiving DpRPH, dusky duodenum and a postoperative duodenal ulcer developed in two whose gastroduodenal arteries (GDA) were divided, but did not develop in one with undivided GDA. Postoperative glucose tolerance test and peptide para-aminobenzoic acid test after DpRPH showed better values than those after PpW. All patients are alive and well 22 to 40 months after surgery. CONCLUSIONS: DpRPH is a new standard for MDE. During DpRPH, preservation of the GDA and the superior portion of the pancreatic head is recommended to maintain an adequate duodenal blood flow.     

Sociogenic stress and rodent reproduction

Between 1993 and 1995, we operated on 18 patients for split lesions of the peroneal brevis tendon associated with chronic ankle instability. Five patients were competitive athletes, seven were recreational athletes, and six were persons. Symptoms developed in three phases: ankle sprain, chronic instability, and posterolateral pain. The mean delay between sprain and posterolateral pain was 6 years. At the time of surgery the main complaint was retromalleolar pain in nine patients, pain and instability in eight patients, and instability only in one patient. Diagnosis of tendinous lesions was based on clinical examination in three cases, preoperative magnetic resonance imaging in eight cases, preoperative tenography in one case, and surgical exploration in six cases. The lesion was localized at the tip of the lateral malleolus and was visible only after opening the peroneal retinaculum. In three cases an accessory peroneal muscle was present. A Chrisman-Snook procedure was performed in 13 cases and a simple tendinous repair in 5 cases. The split lesion of the peroneus brevis tendon may be the result of chronic ankle laxity. This lesion needs a specific surgical treatment and the peroneal tendon must be checked in case of surgical procedure for ankle laxity. After ligamentous repair, residual pain can be due to a neglected peroneus brevis tear.     

Changes in the duodenal mucosa in Whipple's disease

A case of Whipple's disease is described in a male patient of 43 who has been previously examined in different hospitals for six years. The diagnosis was established after surgery and histological examination of the duodenum biopsy. Infiltration of the tunica propria of the duodenum with PAS-positive macrophages was observed. Therapy with rondomycin in combination with GUMET-P produced a response but the recurrence took place after therapy. A new operation was followed by peritonitis and pneumonia which terminated in the patient's death.