HELLP syndrome: understanding and management of a pregnancy-specific disease.

HELLP, a syndrome characterised by haemolysis, elevated liver enzymes and low platelets, a variant of pre-eclampsia/eclampsia, is a multisystemic disorder with an incidence of up to 0.9% in all pregnancies and occurs in about one-quarter of pre-eclamptic patients. Most obstetricians presume that HELLP derives from an autoimmune reaction, leading to a materno- fetal imbalance, with accompanying aggregation of platelets, endothelial malfunction along with inborn errors of fatty acid oxidative metabolism. HELLP is characterised by high mortality and morbidity rates, leading to possibly life-threatening complications regarding both the mother and the fetus. Delivery is indicated if HELLP syndrome occurs after 34 gestational weeks or the fetal and/or maternal conditions deteriorate. Vaginal delivery is preferable. Standard corticosteroid treatment is of uncertain clinical value in maternal HELLP syndrome. Future observations and research results may shed more light on improving our understanding of the aspects of HELLP syndrome.

Gene numerical imbalances in cytological specimens based on fluorescence/chromogenic in situ hybridization analysis.

Design and development of novel targeted therapeutic strategies is an innovation in handling patients with solid malignancies including breast, colon, lung, head & neck or even pancreatic and hepatocellular carcinoma. For a long time, immunohistocytochemistry (IHC/ICC) has been performed as a routine method in almost all labs for evaluating protein expression. Modern molecular approaches show that identification of specific structural and numerical imbalances regarding genes involved in signal transduction pathways provide important data to the oncologists. Alterations in molecules such as epidermal growth factor receptor (EGFR), HER2/neu, PTEN or Topoisomerase IIa affect the response rates to specific chemotherapeutic agents modifying also patients' prognostic rates. In situ hybridization (ISH) techniques based on fluorescence and chromogenic variants (FISH/CISH) or silver in situ hybridization (SISH) are applicable in both tissue and cell substrates. Concerning cytological specimens, FISH/CISH analysis appears to be a fast and very accurate method in estimating gene/chromosome ratios. In this paper, we sought to evaluate the usefulness of FISH/ CISH analysis in cytological specimens, describing also the advantages and disadvantages of these methods from the technical point of view.

Small bowel intussusception and concurrent jejunal polyp with neoplastic transformation: a new diagnosis of Peutz-Jeghers syndrome.

Peutz-Jeghers syndrome (PJS) is a rare hereditary disease characterised by hyperpigmentation of the oral mucosa and gastrointestinal hamartomatous polyps. We report a case of a 27-year-old man who presented with a 5-day history of epigastric pain and rectal bleeding. Computed tomography suggested small bowel obstruction secondary to ileocolic intussusception and an incidental polyp in the mid jejunum. The patient underwent exploratory laparotomy during which right hemicolectomy and small bowel resection were performed. Histology from surgical specimens revealed Peutz-Jeghers polyps, one of which had low-grade dysplasia. This case emphasises that although rare, adults with PJS can present with intussusception. Also illustrated is the extremely rare possibility of concurrent polyps occurring in different parts of the bowel with neoplastic transformation. Intussusception is a challenge to diagnose because the presentation is often non-specific. Clinical history-taking and physical examination along with prompt axial imaging is important for the diagnosis. Careful examination of the bowel and polypectomy during laparotomy may prevent neoplastic transformation and short bowel syndrome.

Intestinal neurofibromatosis and small-bowel adenocarcinoma: a single case study.

OBJECTIVE: Patients with Von Recklinghausen's disease (neurofibromatosis type 1) are at increased risk of developing various tumours. However, the coexistence of neurofibromatosis with small-bowel adenocarcinoma is exceedingly rare. We present an uncommon case of neurofibromatosis type 1, involving the small bowel in a 73-year-old man, who was admitted to our department with signs of acute abdomen. At laparotomy, multiple mesenteric and intramural nodules were seen in the distal ileum. These nodules obstructed ileal lumen, while the intestine wall was perforated in one point. A wide resection of the affected ileum together with all visible nodules in the adjacent mesentery was performed. Histology revealed neurofibromatosis type 1 with malignant transformation to small-bowel adenocarcinoma. The patient had no additional therapy. In a follow-up of 2 years, the patient is very well and there was no recurrence of the disease. We suggest that adenocarcinoma of small bowel should be considered in the evaluation of acute abdominal pain in neurofibromatosis patients.

TNP-assisted fascial closure in a patient with acute abdomen and abdominal compartment syndrome.

Topical negative pressure was applied to prevent abdominal compartment syndrome in a patient following surgery for an acute abdomen. It delayed fascial closure, protected the underlying bowel and facilitated abdominal re-entry.

"Falling down": a retroperitoneal catastropheC.

Hemoperitoneum due to ruptured retroperitoneal varices is an extremely rare condition and a poor prognostic sign with a catastrophic and life-threatening situation. Early recognition affords appropriate management and urgent surgical intervention in order to favor the survival rate. In this case report we accurately describe the complex clinical course of a 56-year old woman with retroperitoneal varices, who few months earlier had a chest trauma with multiple left lower rib fractures and 10 years earlier she underwent to ovarian hyperstimulation for an ovulation induction. She was taken to the emergency room for a fainting episode with signs of a clear hemodinamic shock without a present history of trauma. The intricacy of this case was mostly due to the choice of the correct management, where the damage control resuscitation turned out to have an important role.

Enhancing the emergency general surgical service: an example of the aggregation of marginal gains.

INTRODUCTION: We aimed to enhance the emergency general surgical service in our high-volume centre in order to reduce four-hour target breaches, to expedite senior decision making and to avoid unnecessary admissions. MATERIALS AND METHODS: The aggregation of marginal gains theory was applied. A dual consultant on-call system was established by the incremental employment of five emergency general surgeons with a specialist interest in colorectal or oesophagogastric surgery. A surgical ambulatory care unit, which combines consultant-led clinical review with dedicated next-day radiology slots, and a dedicated working week half-day gastrointestinal urgent theatre session were instituted to facilitate ambulatory care pathways. RESULTS: The presence of two consultant surgeons being on call during weekday working hours decreased the four-hour target breaches and allowed consultant presence in the surgical ambulatory care clinic and the gastrointestinal urgent theatre list. Of 1371 surgical ambulatory care clinic appointments within 30 months, 1135 (82.7%) avoided a hospital admission, corresponding to savings of £309,752 . The coordinated functioning of the surgical ambulatory care clinic and the gastrointestinal urgent theatre list resulted in statistically significantly reduced hospital stays for patients operated for abscess drainage (gastrointestinal urgent theatre median 11 hours (interquartile range 3, 38) compared with emergency median 31 hours (interquartile range 24, 53), P < 0.001) or diagnostic laparoscopy/appendicectomy (gastrointestinal urgent theatre median 52 hours (interquartile range 41, 71) compared with emergency median 61 hours (interquartile range 43, 99), P = 0.005). Overnight surgery was reduced with only surgery that was absolutely necessary occurring out of hours. CONCLUSION: The expansion of the 'traditional' on-call surgical team, the establishment of the surgical ambulatory care clinic and the gastrointestinal urgent theatre list led to marginal gains with a reduction in unnecessary inpatient stays, expedited decision making and improved financial efficiency.

Large intraparotid facial nerve schwannoma: case report and review of the literature.

Here is reported an extremely rare case of a large intraparotid facial nerve schwannoma in a 32-year-old female who presented with a parotid mass. There had been a long clinical course and sudden onset of facial weakness. Diagnostic evaluation and surgical management are discussed along with a brief review of the literature.

[Pancreatic fistula after left pancreatectomy. Risk factors analysis on 68 patients]. / La fistola pancreatica dopo pancreasectomia sinistra. Analisi dei fattori di rischio su 68 casi.

AIM: The aim of this study was to identify risk factors related to pancreatic fistula after left pancreatectomy, considering the difference between the use of mechanical suture and the manual suture to close the pancreatic stump. METHODS: Sixty-eight patients, undergoing left pancreatectomy, were included in this study during a 10-year period. Eight possible risk factors related to pancreatic fistula were examined, such as demographic data (age and sex), pathology (pancreatic and extrapancreatic), technical characteristics (stump closure, concomitant splenectomy, additional procedures), texture of pancreatic parenchyma, octreotide therapy. RESULTS: Fourty-one patients (60%) underwent left pancreatectomy for primary pancreatic disease and 27 (40%) for extrapancreatic malignancy. Postoperative mortality and morbidity rates were 1.5% and 35%, respectively. Fourteen patients (20%) developed pancreatic fistula: 4 of them were classified as Grade A, 9 as Grade B and only one as Grade C. Three factors have been significantly associated to the incidence of pancreatic fistula: none prophylactic octreotide therapy, spleen preserving and soft pancreatic texture. It's still unclear the influence of pancreatic stump closure (stapler vs hand closure) in the onset of pancreatic fistula. CONCLUSION: In this study the incidence of pancreatic fistula after left pancreatectomy has been 20%. This rate is lower for patients with fibrotic pancreatic tissue, concomitant splenectomy and postoperative prophylactic octreotide therapy.

Abdominal wall endometriosis: report of two cases.

Endometriosis is the abnormal existence of functional uterine mucosal tissue outside the uterus. It is a usual disorder of women in reproductive age, which is mainly located in the female genital tract. We report the cases of two women with endometriosis of the abdominal wall; the first one in the rectus abdominis muscle and the second one in the surgical scar of previous caesarian incision. The diagnosis was made by the histopathological analysis of the surgical specimens.

Intraductal papillary mucinous neoplasms of the pancreas.

BACKGROUND/AIMS: Intraductal papillary mucinous neoplasms (IPMNs) are neoplasms of the pancreatic duct epithelium characterized by intraductal papillary growth and thick mucin secretion. Quantities of mucin fill the main and/or branches of pancreatic ducts and cause ductal dilatation. This review encompasses IPMNs, including symptoms, diagnosis, management, and prognosis. METHODS: A Pubmed database search was performed. All abstracts were reviewed and all articles in which cases of IPMNs could be identified were further scrutinized. Further references were extracted by cross-referencing. RESULTS: Only one-third of all patients are symptomatic. According to the site of involvement, IPMNs are classified into three types: main duct type, branch duct type, and combined type. Most branch type IPMNs are benign, while the other two types are frequently malignant. The presence of large mural nodules increases the possibility of malignancy in all types. Presence of a large branch type IPMN and marked dilatation of the main duct indicate the existence of adenoma at least. Synchronous or metachronous malignancies may be developed in various organs. Endoscopic retrograde cholangiopancreatography, endoscopic ultrasonography, and intraductal ultrasonography clearly demonstrate ductal dilatation and mural nodules, while magnetic resonance pancreatography best visualizes the entire outline of IPMNs. CONCLUSIONS: Prognosis is excellent after complete resection of benign and non-invasive malignant IPMNs. The extent of pancreatic resection and the intraoperative management of resection margins remain controversial. Total pancreatectomy should be reserved for patients with resectable but extensive IPMNs involving the whole pancreas; its benefits, however, must be balanced against operative and postoperative risks. Regular monitoring for disease recurrence is important after surgery.

[Late complication after colon self-expandable metal stent placement: a case report]. / Complicanza tardiva dopo posizionamento colico di stent metallico autoespandibile: caso clinico.

Treatment of acute colorectal malignant obstruction, by using self-expandable metallic stents is useful for both palliative and decompressive therapy before the final surgical treatment. In this case, the patient may be benefit from a period of medical optimization prior to undergoing planned surgical resection by a colorectal surgeon. This is a minimally invasive procedure, relatively safe, which obviates the need for colostomy for evacuation relieving physical and psychological burden and contributing the improvement of quality of life. Furthermore, this method also has the advantage of being cost-effective. The previous experience in the benign biliary stenosis allowed the extension of using the metallic stents also for the treatment of benign colorectal diseases (diverticular occlusion, anastomotic strictures, colonic endometriosis). Complications of colon self-expandable metallic stents placement may occur during the procedure and soon after placement (early complications) or, rarely, late after insertion (late complications). These include bleeding, re-obstruction, pain, tenesmus, stent migration, and perforation. The authors report a case of an 81 year-old woman with inoperable rectal carcinoma with liver metastasis who underwent palliative treatment of self-expanding metallic stent endoscopic placement. One month later, the patient presented with acute abdomen at Accidents and Emergencies Department. The diagnosis was a late rectosigmoid junction perforation by stent placement.

Simultaneous deregulation of p16 and cyclin D1 genes in pancreatic ductal adenocarcinoma: a combined immunohistochemistry and image analysis study based on tissue microarrays.

PURPOSE: Deregulation of cell cycle control molecules, such as cyclins and their inhibitors, is a crucial event in the carcinogenetic process. Our aim was to identify potential correlations between p16 and cyclin D1 expression in pancreatic ductal adenocarcinoma (PDAC) that affect the biological behavior of this neoplasm. MATERIALS AND METHODS: Using tissue microarray (TMA) technology, 50 paraffin-embedded tissue samples of histologically confirmed primary PDACs were cored twice and re-embedded to the final recipient block. Immunohistochemistry (IHC) was performed using monoclonal anti-p16 and anti-cyclin D1 antibodies. Protein expression levels were determined by performing computerized image analysis (CIA; estimation of Nuclear Labeling Index-NLI). SPSS (chi square test and interrater Cohen's kappa) was used for statistical analysis. RESULTS: Cyclin D1 overexpression was observed in 24/50 (48%) of the examined carcinomas, whereas p16 loss or reduced expression was detected in 40/50 (80%) cases. Statistical significance was noted when correlating grade to cyclin D1 (p=0.038), stage to p16 (p=0.012) and also to cyclin D1 (p=0.011). Interestingly, combined protein alterations (p16 loss and cyclin D1 overexpression) were observed in 23/50 (46%) cases associated with advanced stage (p=0.019). Overall combined expression of the two molecules demonstrated a significantly low value (kappa=0.012; 95% confidence interval-CI: 0.010-0.014). CONCLUSION: A significant proportion of PDACs is characterized by simultaneous protein alterations regarding p16 and cyclin D1 genes. This mechanism of genetic deregulation in cell cycle potentially explains in part the aggressive phenotype of this neoplasm.

[Indications and outcomes of surgical management of chronic pancreatitis: literature review]. / Indicazioni e risultati del trattamento chirurgico nella pancreatite cronica: revisione della letteratura.

Surgical management of chronic pancreatitis remains a challenge for surgeons. Last decades, the improvement of knowledge regarding to pathophysiology of chronic pancreatitis, improved results of major pancreatic resections, and new diagnostic techniques in clinical practice resulted in significant changes in the surgical approach of this condition. Intractable pain, suspicion of malignancy, and involvement of adjacent organs are the main indications for surgery, while the improvement of patient's quality of life is the main purpose of surgical treatment. The surgical approach to chronic pancreatitis should be individualized based on pancreatic anatomy, pain characteristics, exocrine and endocrine function, and medical co-morbidity. The surgical treatment approach usually involves pancreatic duct drainage procedures and resectional procedures including longitudinal pancreatojejunostomy, pancreatoduodenectomy, pylorus-preserving pancreatoduodenectomy, distal pancreatectomy, total pancreatectomy, duodenum-preserving pancreatic head resection (Beger's procedure), and local resection of the pancreatic head with longitudinal pancreatojejunostomy (Frey's procedure). Recently, non-pancreatic and endoscopic management of pain have also been described (splancnicectomy). Surgical procedures provide long-term pain relief, improve the patients? quality of life with preservation of endocrine and exocrine pancreatic function, and are associated with low mortality and morbidity rates. However, new studies are needed to determine which procedure is safe and effective for the surgical management of patients with chronic pancreatitis.

Lichtenstein tension-free repair of inguinal hernia.

Recurrences have been a significant problem following hernia repair. The purpose of this study was to present our experience of Lichtenstein tension-free repair of inguinal hernia. In this retrospective study, 223 inguinal hernia repairs were performed between September 2000 and August 2003 in 203 patients, using a polypropylene mesh. The main outcome measure was early and late complications and especially recurrences. There were 189 males (93.1%) and 14 females (6.9%). Inguinal hernia was indirect in 70% of cases (n=156), direct in 25% (n=56), and of the mixed type in 5% (n=11). Bilateral inguinal hernia was found in 20 patients (9.8%). 210 (94.2%) of hernias were de novo, while 13 (5.8%) were recurrences. The mean patients age was 54.3 years (range, 32-71 years). The follow-up was completed in 160 patients (78.8%) by clinical examination. The median follow-up period was 3.0 years (range, 1-5 years). Seroma and postoperative neuralgia were observed in one and 5 patients respectively. There was only one recurrence (0.4%) four years later. Lichtenstein tension-free mesh repair of inguinal hernia is a simple and safe method, with no significant early and late morbidity and achieved a method with no recurrence during the follow-up period.

Upper gastrointestinal bleeding due to primary aortoenteric fistula.

Primary aortoenteric fistula (PAEF) is a rare but clinically important cause of catastrophic gastrointestinal bleeding. "Herald bleeding" is a characteristic symptom which refers to specific case of upper gastrointestinal bleeding that stop temporarily spontaneously and then proceeds to massive bleeding. We present the case report of a 55-year-old male with PAEF who was admitted due to upper gastrointestinal bleeding. Endoscopic studies were unremarkable and patient underwent exploratory laparotomy. The postoperative course was uneventful. A high index of suspicion, early diagnosis and prompt appropriate surgical intervention are crucial for survival of patient with PAEF. Gastrointestinal bleeding combined with a negative endoscopy suggests PAEF. Endovascular operation is an attractive treatment option.

Constipation-predominant irritable bowel syndrome associated to hyperprolactinemia.

Irritable bowel syndrome (IBS) is considered to be a physical disorder that mainly affects the bowel and is clinically characterized by lower abdominal pain or discomfort, diarrhea, constipation (or alternating diarrhea/constipation), gas, bloating, and nausea. According to recent studies, it appears that there is an association with increased prolactin levels in patients suffering from IBS. We report a rare case of regression of IBS symptoms (constipation type) in a 16-year-old female adolescent after receiving cabergoline for treating hyperprolactinemia due to pituitary macroadenoma. Our hypothesis is that increased prolactin levels, for instance due to a pituitary adenoma, may suppress prolactin-releasing peptide release and lead to a reverse feedback interaction, consequently resulting in oversecretion of cholecystokinin, inducing the development of IBS.

A very simple technique to repair Grynfeltt-Lesshaft hernia.

PURPOSE: A very simple technique to repair a superior lumbar hernia is described. The location of this type of hernia, also known as the Grynfeltt-Lesshaft hernia, is defined by a triangle placed in the lumbar region. METHOD: An unusual case of a 67-year-old woman with a superior lumbar hernia is reported. The diagnosis was made by physical examination. RESULTS: The defect of the posterior abdominal wall was repaired with a polypropylene dart mesh. The patient had no evidence of recurrence at 11 months follow up. CONCLUSIONS: The surgical approach described in this paper is simple and easy to perform, and its result is comparable with other techniques that are much more sophisticated. No cases on the use of dart mesh to repair Grynfeltt-Lesshaft hernia have been reported by surgical journals indexed in PubMed.

Spontaneous rupture of splenic artery aneurysm in pregnancy: a case report.

Splenic artery aneurysms (SAA) occur predominantly in women, and the majority of them are asymptomatic until rupture. Over half of those that rupture occur during pregnancy. Spontaneously ruptured SAA during pregnancy is always a life-threatening surgical entity for both the mother and the fetus. We report the case of a 29-year-old woman at 34 weeks' gestation with spontaneous rupture of SAA who underwent emergency exploratory laparotomy and splenectomy. This case illustrates the need to consider ruptured SAA as part of important differential diagnosis in haemodynamically unstable pregnant women.

Thyroid microcarcinoma during thyroidectomy.

INTRODUCTION: We aimed to retrospectively assess the prevalence of microcarcinoma in thyroidectomy specimens from a Greek population and the role of histopathology in determining management of these patients. METHODS: We used histopathological reports of thyroidectomies performed in a Greek general district hospital. The samples consisted of 191 thyroidectomies performed between January 1997 and July 2001. The female:male ratio was approximately 2:1 and the follow-up period was 327 weeks. RESULTS: There were 29 microcarcinomas (15.2 percent) with a female:male ratio of 6:1. The prevalence rate in cases with Hashimoto's thyroiditis was significantly higher compared to cases with other benign thyroid pathology (26.8 percent versus 11.9 percent, p-value equals 0.02). Eight microcarcinomas (27.6 percent) were multifocal. The histological type was that of papillary tumour in ten cases (34.5 percent) and follicular in 18 cases (62.1 percent). There were no deaths, recurrences or metastases during the follow-up period. CONCLUSION: Our results suggest that incidental microcarcinomas are low-risk tumours that do not require routine further intervention. The latter may be necessary for tumours with poor differentiation or for non-incidental microcarcinomas.