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.

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.

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.

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.

Pancreaticoyeyunostomía ducto-seromuscular término-lateral en la operación whipple: experiencia en 27 casos / Seromuscular end pancreaticojejunostomy-duct-side in the whipple operation: experience with 27 cases

Presentar la experiencia con la anastomosis ductoserosa termino-lateral en la pancreaticoyeyunostomía del Whipple, en el Servicio de Cirugía General del Instituto Autónomo Hospital Universitario de Los Andes, Mérida-Venezuela. Estudio prospectivo, descriptivo en pacientes sometidos a duodenopancreatectomía por patología maligna a nivel pancreático y región periampular entre los años 2003 a 2007. En todos los casos se realizó preservación pilórica con anastomosis ducto-serosa termino-lateral del remanente pancreático, con colocación de stent intraductal, excepto en un caso donde se realizó la técnica invaginante término-terminal. Se evaluaron las complicaciones postoperatorias inherentes a la técnica quirúrgica, haciendo especial énfasis en la fístula pancreática. Un total de 27 pacientes durante el período 2003 a 2007 fueron sometidos a duodenopancreatectomía cefálica en forma electiva por etiología maligna tales como: ampuloma II casos, colangiocarcinoma distal 2 casos, cáncer de páncreas 12 casos, tumor neuroendocrino 1 caso, adenoma velloso malignizado de colédoco 1 caso. Las edades estuvieron comprendidas entre 42-81 años con promedio de 61 años, predominantemente del sexo femenino en una proporción 4:1. Entre las complicaciones posquirúrgicas relacionadas con la técnica encontramos 4 casos (14.8 por ciento) dado por: 2 fístulas pancreáticas (una de ellas por la ligadura accidental del stent intraductal), 1 dehiscencia de la duodenopancreatectomía, siendo la causa de muerte, y finalmente un caso de atonía gástrica manejado médicamente en forma satisfactoria. La anastomosis ducto-serosa en el manejo del remanente pancreático del Whipple constituye una técnica de fácil ejecución y con buenos resultados, ya que ofrece menos morbimortalidad.

Duodenopancreatectomía cefálica en tumores periampulares. Disección de la arteria mesentérica superior como abordaje inicial. Descripción de la técnica y evaluación de nuestra experiencia inicial / Cephalic duodenopancreatectomy in periampullary tumours. Dissection of the superior mesenteric artery as aninitial approach. Description of the technique and an assessment of our initial experience

Introducción. La duodenopancreatectomía cefálica (DPC) con abordaje inicial de la arteria mesentérica superior (AMS) ha sido descrita como una técnica útil para reducir las pérdidas de sangre y evitar una intervención inútil si hay afectación arterial. Objetivos. Analizar los resultados de dos modificaciones recientes en la técnica quirúrgica de la DPC introducidas en nuestro grupo: disección primaria de la AMS y la gastroenterostomía antecólica. Pacientes y método. Se dividió a los pacientes en 2 grupos, según hayan recibido o no disección inicial de la AMS. También se analizaron los resultados según el tipo de reconstrucción gástrica. Se comparan los resultados perioperatorios y a largo plazo. Resultados. La mortalidad general fue del 5% sin diferencias entre la DPC con abordaje inicial de la AMS y la técnica convencional. La tasa de transfusión (p < 0,001), el volumen transfundido (p = 0,001) y la incidencia general de complicaciones fue menor (p = 0,01) en el grupo con disección de la AMS. La estancia postoperatoria también fue significativamente menor (p # 0,001). A pesar de que la afectación ganglionar fue más frecuente en los pacientes operados con abordaje inicial de la AMS (p = 0,001), la tasa de recidiva fue la misma que con la técnica convencional. Dentro del grupo con disección inicial de la AMS, aquellos con reconstrucción antecólica presentaron con menor frecuencia retraso en el vaciamiento gástrico (p = 0,008). Conclusiones. La DPC con abordaje inicial de la AMS es una técnica segura. La transfusión, las complicaciones y la estancia hospitalaria son mejores. Cuando se asocia a reconstrucción duodenoyeyunal antecólica, los retrasos de vaciamiento gástrico son menos frecuentes (AU)

Introduction. Pancreatoduodenectomy (PD) with initial dissection of the superior mesenteric artery (SMA) has been described as a useful technical variant to reduce blood loss and to avoid an unnecessary intervention in those cases with arterial involvement. Objectives. To analyse the results of two recent technical modifications of PD introduced by our group: initial dissection of SMA and antecolic gastroenterostomy. Patients and method. Patients were divided into two groups: with and without initial dissection of the SMA. The results were also analysed according to the type of gastric reconstruction. Perioperative and long-term results are compared. Results. The overall mortality was 5%, with no significant differences between the initial SMA dissection and conventional PD. The transfusion rate (p < 0.001), the volume of blood products transfused (p = 0.001), and the overall complication rate were lower (p = 0.01) in the initial SMA dissection group. Also the postoperative hospital stay was significantly lower (p # 0.001). Despite a higher frequency of lymph node involvement in patients treated with initial SMA dissection (p = 0.001), the recurrence rate was similar between both groups. Among patients with initial SMA dissection, those who received antecolic reconstruction had a lower rate of delayed gastric emptying (p = 0.008). Conclusions. Initial SMA dissection PD is a safe technique. The transfusion rate, morbidity and postoperative hospital stay are better when compared with conventional CPD. When an antecolic duodenal-jejunal reconstruction is associated, delayed gastric emptying cases are less frequent (AU)

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.

[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.

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.

Lipodistrofia de mesenterio / Mesenteric lipodystrophy

Se realiza la presentación clinico-patológica de un pseudotumor inflamatorio del mesenterio en un masculino de 43 años. Acudió al hospital con un tumor abdominal de 2 meses de evolución, el cual había condicionado cuadros de obstrucción intestinal. De los estudios de gabinete realizados, la tomografía axial computada y la angiografía mesentérica fueron de gran utilidad. Los exámenes de laboratorio fueron inespecíficos, y el diagnóstico de certeza se obtuvo mediante cirugía y estudio histológico de lesión. Se realizó la revisión bibliográfica de otros casos previos reportados

[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.

Pancreaticogastrostomía en una pancreatoduodenectomía con preservación del piloro: reporte de un caso / Pancreaticogastrostomy in a pancreatoduodenectomy with prevention of pyloric: presentation a case

La alta morbimortalidad asociada a la Operación de Whipple estandar se ha reducido en los últimos años, sin embargo, aún observan un gran número de complicaciones relacionadas con el muñon pancreático, tales como fugas de la anastomosis pancreatoyeyunal y disfunción endo y exocrina; otra serie de complicaciones se ven en relación a la gastrectomía como son el dumping, diarrea, alteraciones en la absorción y la úlcera marginal. La pancreaticogastrostomía acompañada a una pancreatoduodenectomía con preservación del píloto se presenta como una combinación excelente para reducir tales complicaciones. Nosotros presentamos el caso de un paciente masculino de 57 años de edad al cual se le realizó el procedimiento; posterior a ello, se realizó una valoración endoscópica y radiológica encontrando una mucosa de características normales y una función pilórica y vaciamiento gástrico adecuado. El quimismo gástrico resultó hiper-secretante, posiblemente debido a la alcalinización del antro gástrico por la secreción pancreática. La evaluación endocrina del páncreas determinado por una curva de tolerancia glucosada fue normal al igual que la evaluación exocrina a través del Test de Pancreolauryl