Whipple's disease infection surgical treatment: presentation of a rare case and literature review.

The Whipple' Disease (W.D.) is a very rare disease with an incidence of 1 per 1.000.000 inhabitants; it is a systemic infection that may mimic a wide spectrum of clinical disorders, which may have a fatal outcome and affects mainly male 40-50 years old. The infective agent is an actinomycete, Tropheryma Whipplei (T.W.) that was isolated 100 years after first description by Wipple, and identified in macrophages of mucosa of the small intestine by biopsy which is characterized by periodic acid-Schiff-positive, products of the inner membrane of his polysaccharide bacterial cell wall. The multisystemic clinical manifestations evolve rapidly towards an organic decay characterized by weight loss, malabsorption, diarrhea, polyathralgia, opthalmoplegia, neuro-psychiatric disorders and sometimes associated to endocarditis. Early antibiotic treatment with trimethoprim and sulfometathaxazole reduces the fatal evolution of the disease. The authors present a rare experience about a female subject in which the clinical gastrointestinal signs were preceded by neuro-psychiatric disorders, and evolved into obstruction and intestinal perforation which required an emergency surgery with temporary ileostomy, recanalized only after adequate medical treatment with a full dose of antibiotic and resolution of clinical disease for the high risks of fistulae for the edema and lymphadenopathy of mucosa. The diagnosis was histologically examined by intestinal biopsy performed during surgery, which showed PAS-positive histiocytes, while PRC polymerase RNA was negative, which confirms the high sensibility of PAS positive and low specificity of RNA polymerase for T.W.

Skull metastasis of ampulla of Vater adenocarcinoma 5 years after Whipple operation: case report and literature review.

Skull metastases can occur with nearly all types of tumor. They frequently are asymptomatic, causing local swelling, which is usually painless and rarely leads to neurological dysfunction. Carcinoma of the ampulla of Vater is an uncommon tumor accounting for approximately 0.2% of all gastrointestinal malignancies, with an estimated incidence of less than 6 cases per 1,000,000 population per year. We report about an extremely rare case of a 54-year-old female patient with a right frontal skull metastases of an ampulla of Vater adenocarcinoma 5 years after pylorus-preserving pancreaticoduodenectomy. Literature review revealed only one published case of cranial bone metastases of carcinoma of the ampulla of Vater. To the best of our knowledge this is the first case report of skull metastases of ampullary adenocarcinoma after a symptom-free interval of 5 years after R0 resection of the primary tumor, and the second published case that involves the skull.

Isolated Whipple disease of the brain resembling a tumour.

INTRODUCTION: Isolated Whipple disease of the central nervous system is a rare occurrence. Migratory arthralgias and gastrointestinal problems, including malabsorption, abdominal pain, diarrhea, and weight loss, are common presenting symptoms. DISCUSSION: For those patients with systemic signs and symptoms of Whipple disease, 6% to 43% will have clinically manifested CNS involvement that may include alterations in personality, ataxia, and dementia. We report our experience with a patient, who was successfully treated for Whipple disease 12 years prior to presentation and had a magnetic resonance image of the brain that revealed two solitary lesions resembling a tumor upon presentation.

Whipple Disease Presenting as Cystic Brain Tumor: Case Report and Review of the Literature.

Although neurological features are commonly encountered in Whipple"s disease (WD), presentation with purely neurological patterns is uncommon. Exclusive confinement to the central nervous system (CNS) is extremely rare. In these cases, the development of an isolated cerebral mass is exceptional. In the present paper, the authors describe a case of a 68-year-old man who presented with partial seizures. The neurological examination was normal. The imaging showed a cystic lesion. This tumor-like lesion was removed by performing frontal craniotomy. A histopathological investigation revealed the presence of numerous perivascular foamy histiocytes infiltrating the brain parenchyma. The majority of these histiocytes showed Periodic acid-schiff (PAS)-positive intense staining, which is distinctive feature of cerebral WD. The diagnosis was confirmed by polymerase chain reaction (PCR) analysis of cerebrospinal fluid. There were no gastrointestinal symptoms and no PAS inclusions in intestinal mucosa. The patient received Ceftriaxone intravenously followed by oral trimethoprime-sulfamethoxazole (TMP-SMZ) for 12 months and recovered well. This case illustrates atypical WD, confined exclusively to the central nervous system.

Smooth muscle distribution in the extrahepatic bile duct: histologic and immunohistochemical studies of 122 cases.

The distribution of smooth muscle fibers in the extrahepatic bile duct (EBD) wall is not well characterized. We analyzed 101 consecutive Whipple's operation specimens and 21 autopsy specimens for the pattern of smooth muscle distribution in EBD using the Masson-trichrome stain and the desmin immunohistochemical stain. The patterns were categorized as continuous, interrupted, scattered, and no muscle layer. EBDs were divided into lower, middle, and upper portions, and the distribution pattern of smooth muscle fibers was analyzed separately in each portion. Because most surgically resected specimens contained the middle and lower EBDs with only a portion of the upper EBD, only the length of the middle and lower EBDs (common bile duct, CBD) was measured. The mean length of CBD in surgically resected specimens was 6.4 +/- 1.4 cm (men, 6.6 +/- 1.3 cm; women, 6.1 +/- 1.5 cm). The mean length of CBD in autopsy specimens was 6.8 +/- 1.0 cm. The predominant patterns of the lower third of the EBD were interrupted (49%) and continuous (43%). The predominant patterns of the middle third of the EBD were scattered (63%) and interrupted (23%). Those of the upper third of the EBD were no muscle fiber (58%) and scattered (39%). In conclusion, different patterns of smooth muscle distribution were observed in different portions of the EBD. Because scattered muscle fibers or no muscle fibers were the main features of the upper third of the EBD, understanding of this pattern may be helpful for assessment of the depth of invasion or staging of carcinoma of the upper third of the EBD.

[Mesenteric lipodystrophy]. / Lipodistrofia de mesenterio.

This case report describes the clinic and pathologic findings of an inflammatory pseudotumor of the mesentery in a 43 years old male. Previous to the hospital admission he had several episodes of intestinal occlusion and an abdominal mass. Computed tomography and mesenteric angiography were very helpful. Laboratory tests were unspecific, and the final diagnosis was obtained after surgery and histologic study of the lesion. We review published literature on inflammatory pseudotumors.

[Changes in the duodenal mucosa in Whipple's disease]. / Izmeneniia slizistoi obolochki dvenadtsatiperstnoi kishki pri bolezni Uipla.

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.

Tropheryma whipplei endocarditis without gastrointestinal involvement.

Whipple's disease, caused by the bacterium Tropheryma whipplei, is a rare chronic multi-system illness commonly affecting the gastrointestinal (GI) tract and presenting with a triad of diarrhoea, weight loss and malabsorption. While 20-55% of patients with a diagnosis of Whipple's disease have clinically evident cardiac manifestations, the initial presentation with isolated valvular disease, without any GI symptoms, is rare. Whereas cardiac involvement usually involves a single valve, cases of double-valve involvement are extremely rare. We report the case of a patient with T. whipplei native aortic and mitral valvular endocarditis, without GI involvement, who presented with the new-onset cardiac failure and ventricular arrhythmias, which required urgent double-valve replacement. This case report is accompanied by a review of the relevant literature.

Whipple's disease confined to the central nervous system.

We report a 49-year-old woman with a left parietal lesion, shown on CT and MRI as an isolated ring-enhancing mass. The diagnosis of cerebral Whipple's disease was made by brain biopsy; there were no gastrointestinal symptoms nor periodic-acid Schiff-positive inclusions in the jejunal mucosa. This case illustrates atypical Whipple's disease, confined exclusively to the central nervous system.