Treatment Of Post-Operative Chylothorax: The Role Of Intranodal Lymphangiography And Thoracic Duct Disruption.

Thoracic duct embolization has been increasingly adopted as a first-line therapy of chylothorax and this procedure includes lipiodol lymphangiography, thoracic duct access and embolization. Lymphangiography itself has a therapeutic role, with volume-dependent success rates of 37%-97% and even a reported 100% success rate in outputs of < 500 mL/day. We present a clinical case of a 48-years-old man diagnosed with esophageal squamous cell carcinoma, who underwent esophagectomy and presented with post-operative high-output (> 1L/day) chylothorax; thoracic duct embolization was proposed. Even though thoracic duct access and embolization were not achieved due to technical and anatomical factors, lipiodol lymphangiography and possibly thoracic duct maceration (after several punctures/attempts) contributed to the clinical success of the procedure, and this chylothorax with output values superior to those reported in the literature resolved within three days. As such, the therapeutic role of intranodal lymphangiography and thoracic duct disruption should be taken into account.

Prognostic factors for traumatic bowel injuries: killing time.

BACKGROUND: Intestinal rupture/perforation after abdominal trauma is a rare complication, but it is related to significant morbidity and mortality. Our objective is to identify determinants of prognosis in patients surgically treated for a bowel injury (small bowel and colon) after abdominal trauma. METHODS: The present study is a retrospective analysis of 102 patients seen at our hospital during a 10-year period in whom laparotomy for traumatic bowel injury was performed. Predictors for morbidity and mortality were assessed in univariate and multivariate analysis models. RESULTS: Mean age at diagnosis was 40 years, and most patients were male. There was a slight preponderance of blunt abdominal trauma, and the most frequent mechanism of injury was motor vehicle accident. In 75% of patients there was a small bowel lesion, and the colon was affected in 47%. There was no statistical relation between stoma construction and mortality, but an increase in morbidity was ultimately dependent on the severity of the underlying trauma. The univariate determinants of mortality were the new injury severity score (NISS) and American Society of Anesthesiologists (ASA) scores, the presence of blunt trauma and multiple intestinal or extra-abdominal lesions, and the elapsed time to surgery. The occurrence of postoperative complications was related to all these factors, as well as to tachycardia, hypotension, and bleeding. In multivariate analysis ASA score (p = 0.015), NISS (p = 0.002), time to surgery (p = 0.007), and presence of colonic lesions (p = 0.02) were identified as independent prognostic factors for postoperative morbidity. CONCLUSIONS: The only modifiable determinant of morbidity seems to be the time to surgery. Only an expeditious evaluation and diagnosis and prompt surgical intervention can improve the prognosis of these patients.

Primary cutaneous B-cell lymphoma arising from an inguinal hernioplasty scar.

The primary cutaneous B-cell lymphoma (PCBCL) is a rare neoplasm. It is believed that antigenic stimulation and chronic inflammation can be the basis of pathogenesis. Here, we report a case that to our knowledge is the first patient with a presentation of a PCBCL arising from a surgical scar, in particular, an inguinal hernioplasty. The case reminds us of the importance of raising clinical suspicion for malignant neoplasms in surgical scars, in particular, after mesh placement. MeSH terms: 'lymphoma, large b-cell, diffuse', 'skin' and 'cicatrix'.

Clear cell change in colonic polyps.

In this study, the pathogenesis of clear cell change in colorectal epithelial lesions was studied. A total of 4 cases of clear cell change (1 hyperplasic polyp and 3 adenomas) were characterized using histochemistry, immunohistochemistry, and electron microscopy. All lesions developed in the left colon. In all, 1 adenoma with clear cell dysplastic glands progressed to adenocarcinoma without clear cell change. Clear cell cytoplasmatic vacuoles were negative for glycogen and mucins (MUC 2, MUC 5AC). Ki-67 LI in clear cell adenoma components was lower than in common adenoma components of the same dysplasia grades (while p53 and beta-catenin were similarly expressed). Ultrastructural features of clear cell change showed features of lipid-like material. Clear cell change is found in hyperplastic and neoplastic lesions of the colon and is not due to the accumulation of glycogen or mucins. A degenerative nature of clear cell change is suggested by the demonstration of lipid-like material in the vacuoles of clear cells.

The interleukin-8-251*T/*A polymorphism is not associated with risk for gastric carcinoma development in a Portuguese population.

It has been demonstrated that polymorphisms within inflammation-related genes are associated with risk of gastric carcinoma in Helicobacter pylori-infected individuals. Recently, several studies have reported conflicting results regarding the association between the interleukin (IL)8-251*T/*A polymorphism and risk of gastric carcinoma. In this study, we performed a case-control analysis, including 693 controls, 187 chronic gastritis cases and 333 gastric carcinoma cases, to determine the association between the IL8-251 polymorphism and risk of chronic gastritis and gastric carcinoma in the northern Portugal population. We found no significant association between the IL8-251 polymorphism and increased risk of chronic gastritis or gastric carcinoma, in agreement with that reported in other populations of white origin. The retrospective analysis of published data shows that the association between the IL8-251 polymorphism and risk of gastric carcinoma tends to be reproducible in populations of Asian origin. The estimated effect of the polymorphism under analysis was not significantly different in subgroups of gastric carcinoma cases defined by histologic type and anatomic site of the tumours, and by sex and age of the participants. In conclusion our results indicate that although the IL8-251 polymorphism might be a relevant host susceptibility factor for gastric carcinoma development, this association is likely to be ethnic-specific.

Pseudomyxoma peritonei presenting as irreducible epigastric hernia.

Abdominal wall hernia is a common and usually straightforward pathology presenting in surgery clinics. On occasion, the surgeon is faced with unexpected findings requiring difficult intraoperative decision. We present a case of pseudomyxoma peritonei incidentally found during surgery for epigastric hernia. The patient complained of a long lasting epigastric hernia with recent onset pain and growth. Surgery was limited to laparoscopic incisional biopsy of mucinous peritoneal deposit, confirming the diagnosis and suggesting an appendiceal origin. The patient was subsequently referred to a specialized peritoneal cancer unit for definitive treatment which consisted of cytoreductive surgery plus hyperthermic intraperitoneal chemotherapy, which can be compromised by previous organ resection. This case highlights the importance of maintaining a high level of suspicion before unusual clinical courses of common pathology.

Metastatic melanoma: An unusual cause of gastrointestinal bleeding and intussusception-A case report.

INTRODUCTION: Malignant melanoma is responsible for 1-3% of all malignancies being the gastrointestinal tract one of the most common metastatic locations. PRESENTATION OF CASE: We present the case of a 71-year old male with previous history of cutaneous melanoma. Seven years later, while being studied due to suspected small bowel bleeding, he developed small bowel obstruction after being submitted to double balloon enteroscopy. Further investigation revealed small bowel intussusception. He was taken up for emergency laparotomy that confirmed ileal intussusception secondary to an intraluminal mass. We performed segmental enterectomy with primary anastomosis. Histology confirmed intestinal melanoma metastasis. DISCUSSION: Malignant melanoma frequently spreads to the gastrointestinal tract. The presentation is mainly asymptomatic and the diagnosis is often made only after complications develop. Small bowel intussusception and gastrointestinal bleeding are unusual presentations of metastatic melanoma, with few cases reported. Surgical resection not only provides symptom control but also positively affects prognosis. CONCLUSION: Although usually asymptomatic, melanoma metastasis should always be considered in a patient with intestinal symptoms or chronic anemia and personal history of melanoma. Surgical resection is the treatment of choice, leading to an increase in overall survival.

Primary breast lymphoma: A mimic of inflammatory breast cancer.

INTRODUCTION: Primary breast lymphoma (PBL) is a rare disease accounting for 0.04-0.5% of all breast malignancies. PRESENTATION OF CASE: This article reports a clinical case of a 81-year-old woman with a history of right breast erythema and edema associated to breast pain, with over two weeks duration. Physical examination demonstrated mammary asymmetry and inflammatory signs with orange peel skin. She had the right upper limb swollen and palpable axillar and supraclavicular lymph nodes. The principal suspicion was an inflammatory breast cancer. Imaging was unspecific. Core needle biopsy of an axillary lymph node and of the breast mass identified morphological and immunophenotypic features consistent with diagnosis of primary non-Hodgkin DLBCL. Patient was guided by Hematology. DISCUSSION: The clinical and imaging findings in breast lymphoma can mimic those of breast carcinoma. Sometimes, PBL presentation is suggestive of Inflammatory breast cancer. Diagnosis depends on adequate tissue sampling for histology examination and immunophenotyping. The therapeutic management of PBL is controversial and is not clearly established, but many studies support that it is not a surgical disease and can be treated successfully with combined chemotherapy and radiotherapy. CONCLUSION: Breast lymphoma must be considered in the differential diagnosis of a breast lump, even in the presence of cutaneous inflammatory changes. PBL can be treated successfully with combined therapy.

Heterotopic mesenteric and abdominal wall ossification - Two case reports in one institution.

INTRODUCTION: Heterotopic ossification occurs when bone develops in tissues which usually don't undergo ossification. Heterotopic mesenteric ossification, also known as intra-abdominal myositis ossificans, is a rare and benign form of ossification, usually related with previous abdominal surgery or trauma. PRESENTATION OF CASES: We report two cases of heterotopic ossification both after multiple abdominal surgeries, with intraoperative findings of mesenteric and abdominal wall ossification. Histopathology revealed metaplastic bone deposition, without evidence of atypia or malignancy. DISCUSSION: This rare entity shares clinical and pathological characteristics of myositis ossificans. It is important to consider the differential diagnosis with sarcomas. In the cases described, the patients were proposed for elective surgery and this pathology was as an incidental finding. CONCLUSION: The simultaneous presence of mesenteric and abdominal wall ossification in both patients makes these cases even rarer.

Jejunoileal diverticulosis, a rare cause of ileal perforation - Case report.

INTRODUCTION: Jejunoileal diverticulosis (JID) is a rare condition associated with nonspecific symptoms, consisting of acquired false diverticula. It frequently co-exists with colonic diverticulosis. Diagnosis is usually made incidentally or after complications. These include hemorrhage, obstruction and diverticulitis, with or without perforation. PRESENTATION OF CASE: 81-year-old man presented with a painful abdominal mass in the right lower quadrant (RLQ), diffuse abdominal discomfort and fever. Abdominal examination confirmed a well-defined mass in the RLQ without rebound tenderness. Laboratory analysis revealed elevated inflammatory markers and CT scan showed a cavitated lesion with an air-fluid level in the RLQ, without evidence of intraperitoneal free air or fluid. Admitted for conservative treatment, failure to improve led to laparotomy on the 6th day of hospitalization, with identification of jejunoileal diverticulosis complicated with diverticulitis and walled-off perforation. We performed segmental enterectomy. DISCUSSION: The incidence of JID is estimated at 0.2-7% and it is usually diagnosed in the sixth/seventh decade of life. From a diagnostic perspective, JID is a challenging disorder, without reliable diagnostic tests. Diverticulitis is the most common complication. Perforation generally causes only localized peritonitis, as involved diverticula are often walled off by the surrounding mesentery. In selected cases, medical therapy may suffice. For all other patients prompt laparotomy with segmental intestinal resection is the treatment of choice. CONCLUSION: JID remains under diagnosed. When it presents as an acute complication it may require immediate surgical intervention. In an elderly person, especially with known gastrointestinal diverticulosis, one must have a high index of suspicion for perforation.

Role of pathology in the identification of hereditary diffuse gastric cancer: report of a Portuguese family.

Mutations in E-cadherin gene are the underlying genetic defect in approximately one-third of the hereditary diffuse gastric cancer (HDGC) families described to date. Positive family history of diffuse gastric cancer and early age of onset of gastric tumours are the clinical criteria currently used to qualify for HDGC. In the present study, we describe a Portuguese family with HDGC that was selected for CDH1 mutation screening after histological observation of the gastrectomy specimen of one member, who died at the age of 23 years from widely invasive diffuse gastric carcinoma. The detection in the surgical specimen of tiny foci of intramucosal diffuse carcinoma as well as in situ carcinoma lesions and pagetoid spread of signet ring cells raised the hypothesis of HDGC, which was confirmed by pedigree analysis of the family and detection of CDH1 germline mutation. We conclude that there are morphological hints that may help in the identification of HDGC.

Unusual presentation of a Meckel's diverticulum: A case report.

INTRODUCTION: Meckel's diverticulum (MD) is the most common congenital malformation of the gastrointestinal tract. Intestinal obstruction is the lead presenting symptom in the adult population due to multiple causes (intussusception, incarceration, adhesions, strictures and torsion). Our patient had a complicated MD with an unique combination of risk factors and findings. PRESENTATION OF CASE: We report an unusual case of an 18-year-old patient presenting with acute small bowel obstruction for several days, who developed focal peritoneal signs on right lower quadrant. On laparotomy, findings included a necrotic giant MD and a small bowel volvulus around a fibrous band that attached MD to the umbilicus. Segmental enterectomy with primary anastomosis was performed. DISCUSSION: Axial torsion and gangrene of MD is the rarest complication. Its pre-operative diagnosis remains elusive as it can be clinically indistinguishable from other intra-abdominal inflammatory conditions. The correct diagnosis of complicated MD before surgery is often difficult because this condition can mimic other acute abdominal pathologies. There are several risk factors that can point to an accurate and early diagnosis, especially when combined with the appropriate imaging techniques, such as computed tomography with oral and intravenous contrast. CONCLUSION: This complication remains underdiagnosed, often with delayed surgical intervention and sub-optimal treatment that leads to significant morbidity and mortality.

E-Cadherin (CDH1) and p53 rather than SMAD4 and Caspase-10 germline mutations contribute to genetic predisposition in Portuguese gastric cancer patients.

Approximately 30% of all hereditary diffuse gastric cancer (HDGC) families carry CDH1 germline mutations. The other two thirds remain genetically unexplained and are probably caused by alterations in other genes. Using polymerase chain reaction (PCR)/single-strand conformation polymorphism (SSCP)/sequencing, we screened 32 Portuguese families with a history of gastric cancer and 23 patients with early onset gastric cancer for CDH1 germline mutations. In probands negative for CDH1 mutations, we screened genes involved in hereditary cancer syndromes in which gastric cancer may be one of the component tumours, namely p53 (Li-Fraumeni Syndrome) and hMLH1 and hMSH2 (HNPCC). We also screened in these patients for mutations in Caspase-10, a gene inactivated in sporadic gastric cancer, and SMAD4, a gene whose inactivation in mice is associated with signet-ring cell carcinoma of the stomach. One of the families fulfilling the HDGC criteria harboured a CDH1 germline mutation, and one of the families with incomplete criteria harboured a p53 germline mutation. No mutations were identified in hMLH1 and hMSH2, and only sequence variants were found in SMAD4 and Caspase-10. The present work reports for the first time CDH1 germline mutations in Portuguese gastric cancer families, and highlights the need for p53 mutation screening in families lacking CDH1 germline mutations, in a country with one of the highest incidences of gastric cancer in the world. No evidence was found for a role of germline mutations in SMAD4 and Caspase-10 in families lacking CDH1 mutations.

Acute diverticulitis in younger patients: any rationale for a different approach?

AIM: To compare the natural history and course of acute diverticulitis in a younger age group with an older population and to evaluate whether younger patients should be managed differently. METHODS: This study was a retrospective review of 157 patients treated with acute diverticulitis between January 1, 2004 and December 31, 2007. Diverticulitis was stratified according to the Hinchey classification. Patients were divided into 2 populations: group A ≤ 50 years (n = 31); group B > 50 years (n = 126). Mean patient follow-up was 15 mo. RESULTS: The median age was 60 years. A significantly higher proportion of patients in group B presented with complicated diverticulitis (36.5% vs 12.9%, P = 0.01). Recurrence was more frequent in group A (25.8% vs 11.1%, P = 0.03) and the mean time-to-recurrence was shorter (12 mo vs 28 mo, P = 0.26). The most severe recurrent episodes of acute diverticulitis were classified as Hinchey stage I and none of the patients required emergency surgery. In multivariate analysis, only age (P = 0.024) was identified as an independent prognostic factor for recurrence. CONCLUSION: Based on the results of this study, the authors recommend that diverticulitis management should be based on the severity of the disease and not on the age of the patient.