Advances in diagnosis,treatment and palliation of cholangiocarcinoma:1990-2009

Several advances in diagnosis,treatment and palliation of cholangiocarcinoma(CC)have occurred in the last decades.A multidisciplinary approach to this disease is therefore recommended.CC is a relatively rare tumor and the main risk factors are:chronic inflammation, genetic predisposition and congenital abnormalities of the biliary tree.While the incidence of intra-hepatic CC is increasing,the incidence of extra-hepatic CC is trending down.The only curative treatment for CC is surgical resection with negative margins.Liver transplantation has been proposed only for selected patients with hilar CC that cannot be resected who have no metastatic disease after a period of neoadjuvant chemo-radiation therapy.Magnetic resonance imaging/magnetic resonance cholangiopancreatography,positron emission tomography scan,endoscopic ultrasound and computed tomography scans are the most frequently used modalities for diagnosis and tumor staging.Adjuvant therapy,palliative chemotherapy and radiotherapy have been relatively ineffective for inoperable CC.For most of these patients biliary stenting provides effective palliation.Photodynamic therapy is an emerging palliative treatment that seems to provide pain relief,improve biliary patency and increase survival. The clinical utility of other emerging therapies such as transarterial chemoembolization,hepatic arterial chemoinfusion and high intensity intraductal ultrasound needs further study.

Abdominal neurenteric cyst

Neurenteric cysts are extremely rare congenital anomalies, often presenting in the first 5 years of life, and are caused by an incomplete separation of the notochord from the foregut during the third week of embryogenesis. They are frequently accompanied with spinal or gastrointestinal abnormalities, but the latter may be absent in adults. Although usually located in the thorax, neurenteric cysts may be found along the entire spine. We present a 24-year-old woman admitted for epigastric pain, nausea, vomiting, low grade fever and leucocytosis. She underwent cystgastrostomy for a Ioculated cyst of the distal pancreas at the age of 4 years, which recurred when she was at the age of 11 years. Ultrasound and computer tomograghy （CT） scan revealed a 16 cmx 15 cm cystic mass in the body and tail of pancreas, with a 6-7 mm thickened wall. Laboratory data and chest X-ray were normal and spinal radiographs did not show any structural abnormalities. The patient underwent a complete cyst excision, and after an uneventful recovery, remained symptom-free without recurrence during the 5-year follow-up. The cyst was found to contain 1200 mL of pale viscous fluid. It was covered by a primitive singlelayered cuboidal epithelium, along with specialized antral glandular parenchyma and hypoplastic primitive gastric mucosa. Focal glandular groups resembling those of the body of the stomach were also seen. In addition, ciliary respiratory epithelium, foci of squamous metaplasia and mucinous glands were present. The wall of the cyst contained a muscular layer, neuroglial tissue with plexogenic nerve fascicles, Paccini corpuscle-like structures, hyperplastic neuroganglionar elements and occasional psammomatous bodies, as well as fibroblast-like areas of surrounding stroma. Cartilagenous tissue was not found in any part of the cyst. Immunohistochemistry confirmed the presence of neurogenic elements marked by S-100, GFAP, NF and NSE. The gastric epithelium showed mostly CK7 and EMA immunoexpression, and the respiratory epithelium revealed a CK8 and CK18 immunoprofile without CK 10/13 positive elements, though neither CEA or AFP positive cells were found. To our knowledge, this is the first reported case of an abdominally located neurenteric cyst with no associated spinal anomalies.

Megacolon in adulthood after surgical treatment of Hirschsprung's disease in early childhood

Hirschsprung＇s disease （HD） is a disorder associated with congenital malformation of the enteric nervous system with segmental aganglionosis. Prevailing therapy includes a resection of the affected part of the bowel, However, patients often do not obtain complete functional improvement after surgical treatment. We present the case of a 25-year-old woman who had surgical treatment of lid in early childhood. After that procedure she had clinical features of constipation for years in the end, passing of stool once a week, requiring laxatives and enemas. We diagnosed an incomplete resection of the aganglionic bowel via rectal biopsy and resected the remaining aganglionic segment. Two months after surgery the patient＇s bowel function improved to a frequency of 1-4 stools per day. We conclude that regular follow-up is required to identify lid patients with persistent alterations of bowel function after surgery. In patients presenting with constipation, recognition of a remaining aganglionic segment or other alterations of the enteric nervous system should be aimed at in an early stage. 2005 The WJG Press and Elsevier Inc. All rights reserved

Heart-shaped anastomosis for Hirschsprung's disease: Operative technique and long-term follow-up

AIM: To study the long-term therapeutic effect of 'heartshaped' anastomosis for Hirschsprung's disease.METHODS: From January 1986 to October 1997, we performed one-stage 'heart-shaped' anastomosis for 193 patients with Hirschsprung's disease (HD). One hundred and fiftytwo patients were followed up patients (follow-up rate 79%).The operative outcome and postoperative complications were retrospectively analyzed.RESULTS: Early complications included urine retention in 2patients, enteritis in 10, anastomotic stricture in 1, and intestinal obstruction in 2. No infection of abdominal cavity or wound and anastomotic leakage or death occurred in any patients. Late complications were present in 22 cases,including adhesive intestinal obstruction in 2, longer anal in 5, incision hernia in 2, enteritis in 6, occasional stool stains in 7 and 6 related with improper diet. No constipation or incontinence occurred in any patient.CONCLUSION: The early and late postoperative complication rates were 7.8% and 11.4% respectively in our 'heartshaped anastomosis' procedure. 'Heart-shaped'anastomosis procedure for Hirschsprung's disease provides a better therapeutic effect compared to classic procedures.

Duplicated appendix complicated by appendiceal cancer

A 37-year old male presented with an acute abdomen suggestive of an appendiceal perforation.Urgent laparotomy showed a duplicated appendix with one of the lumens involved with appendicitis and a focal periappendicular abscess while the other lumen had a localized appendiceal cancer.Recognition of congenital intestinal duplications in adults is important to avoid serious clinical consequences.

PERITONEAL DIALYSIS AFTER REPAIR OF CONGENITAL HEART DISEASE IN CHILDREN

Objective. To investigate the effect of peritoneal dialysis on fluid balance and outcome in childrenwho receive cardiac operation.Methods. From July to Dec. 2000, 12(2.08%) patients of 576 consecutive children who underwentheart operation required peritoneal dialysis because of acute renal failure. The mean age of these 12 pa-tients was (2.9±2.0) years (range, 5 months～7 years) and the mean body weight was (12±3) kg (range,7.4～18.5 kg ).Results. The interval between the operation and the initiation of peritoneal dialysis was (21.2±11.4)hours (4.4～42 hours). The duration of peritoneal dialysis was (6.3±4.8) days (0.47～15 days). Mortality inthese 12 patients was 25%. Fluid removed by peritoneal dialysis was(34.7±17.8) ml@kg1@day-1@ Asymop-tomatic hypokalemia, thrombocytopenia and hyperglycemia were frequent complications, which were easilymanaged. Hemodynamics and pulmonary function improved during the study period.Conclusion. The early initiation of peritoneal dialysis is an effective and safe method totreat acute renal failure after cardiac operation in children.

Congenital bronchoesophageal fistula in adults

AIM: To study the clinical characteristics, diagnosis and surgical treatment of congenital bronchoesophageal fi stulae in adults. METHODS: Eleven adult cases of congenital bronchoesophageal fistula diagnosed and treated in our hospital between May 1990 and August 2010 were reviewed. Its clinical presentations, diagnostic methods, anatomic type, treatment, and follow-up were recorded. RESULTS: Of the chief clinical presentations, nonspecific cough and sputum were found in 10 (90.9%), recurrent bouts of cough after drinking liquid food in 6 (54.6%), hemoptysis in 6 (54.6%), low fever in 4 (36.4%), and chest pain in 3 (27.3%) of the 11 cases, respectively. The duration of symptoms before diagnosis ranged 5-36.5 years. The diagnosis of congenital bronchoesophageal fistulae was established in 9 patients by barium esophagography, in 1 patient by esophagoscopy and in 1 patient by bronchoscopy, respectively. The congenital bronchoesophageal fistulae communicated with a segmental bronchus, a main bronchus, and an intermediate bronchus in 8, 2 and 1 patients, respectively.The treatment of congenital bronchoesophageal fistulae involved excision of the fistula in 10 patients or division and suturing in 1 patient. The associated lung lesion was removed in all patients. No long-term sequelae were found during the postoperative follow-up except in 1 patient with bronchial fistula who accepted reoperation before recovery. CONCLUSION: Congenital bronchoesophageal fistula is rare in adults. Its most useful diagnostic method is esophagography. It must be treated surgically as soon as the diagnosis is established.

Surgical treatment of three cases of Cantrell's syndrome

This ten-year retrospective study was designed to examine the morbidity and mortality of three cases of Cantrell＇s syndrome between 1998 and 2008. The three patients showed different degrees of Cantrell＇s pentalogy including abdominal ectopia cordis, thoracic-abdominal ectopia cordis and left ventricular diverticulum. Of the three, the 5-month-old boy suffering from complicated congenital heart disease with abdominal ectopia cordis received a successful single stage repair and reconstruction of the abdominal wall. The 33-week-old premature girl with thoracic-abdominal ectopia cordis underwent two stage correction of tetraology of Fallot. The 4-year-old girl underwent ectomy of left ventricular diverticulum and thoracoabdominal wall repair. Twenty-four to thirty-five months follow-up were satisfactory. We hold that two-stage repair are technically feasible for Cantrell＇s syndrome, especially for those with complex congenital heart diseases. Post-operative ventilatory support and multiple post-operative care should be prolonged. Malnutrition, infection and arrhytbmia are central problems in medical care and surgery should be considered if there was progressive heart failure or hemodynamic instability.

REVIEW OF RIGHT VENTRICULAR FAILURE:GENERAL CONSIDERATION

Right ventricular failure(RVF)is a complicated syndrome with multiple etiologies.RVF relates to pulmonary hypertension,left ventricle failure,and congenital heart diseases.The origin of its pathway is based on pathological gene expression and concomitant diseases.Diagnosis of RVF is a serious problem for clinicians,but none of the criteria in current clinical practice provides uncontaminated information on either systolic or diastolic function.Perioperative assessment and bedside monitoring of right ventricle function have to be revised and widely used.Right ventricle function in transplant patients demands different evaluation using biomarkers or/and autopsy.Treatment of RVF has surgical and non-surgical approaches;both are still in development and need further clarification.

后手【老聂论衡】

问题是，这样的“先手”，其使用的价值如何？其付出的代价又如何？ 在棋中，并不是任何时候都要争先手，都要去挑战。

Comparative study on cerebral injury after open heart surgery in patients with congenital and rheumatic heart disease

Objective: To comparatively study the different effects of open heart surgery on brain tissues of patients with congenital and rheumatic heart disease. Methods: Forty patients with congenital heart disease (CHD, CHD group, n=20) or rheumatic heart disease (RHD, RHD group, n=20) underwent on-pump (cardiopulmonary bypass, CPB) heart-beating open heart surgery. Blood samples before CPB, and 20 minutes, 1 hour, 24 hours and 7 days after CPB were collected, and the levels of neuron-specific enolase (NSE) and protein S-100b in the plasma were determined with enzyme-linked immunoadsorbent assay (ELISA), respectively. All the patients were examined with electroencephalogram (EEG) before and 1 week after operation. The changes of NSE, S-100b and EEG compared to verify the difference of postoperative cerebral injury between CHD cases and RHD cases. Results: The plasma level of S-100b increased significantly 20 minutes after CPB and was still higher than the preoperative level at 24 hours after operation in both groups (P< 0.01). The plasma level of NSE increased more significantly in the CHD group than in the RHD group 20 minutes after CPB and it returned to the normal level 24 hours after CPB in the CHD group but remained at a high level in the RHD group (P< 0.01). The levels of NSE and S-100b returned to the normal levels on the 7th day after CPB. Abnormal EEG was found in 75% of the patients in the CHD group and 60% in the RHD group. Conclusions: On-pump heart-beating open heart surgery can cause certain cerebral injury in the patients with CHD or RHD. The injury was more severe and recovered more quickly in the CHD group than in the RHD group.