Management of renal hypertension in children with Takayasu's arteritis using renal autografting or allograft transplantation in selected circumstances and total lymphoid irradiation.

In a decade from 1980, 11 children aged 3 to 11 years presented with Takayasu's arteritis (TA). All were severely hypertensive. Operative correction was offered to 10 of 11 children presenting with renovascular hypertension (RVH) including cardiac failure alone in 1 and both renal and cardiac failure in 8, a result of TA involving renal arteries by stenosis or occlusion. Nine patients had renal autotransplantation to an heterotopic site in the pelvis. Seven of 12 kidneys were salvaged by autotransplant with relief of RVH. Renal artery stenosis was successfully corrected by this procedure in 5 patients. Autotransplantation failed in 4 patients, 1 of whom subsequently had a successful allograft transplant. One patient was treated primarily by cadaver allograft transplantation. One patient whose autotransplant failed had a functioning contralateral kidney and is well with controlled RVH. One patient died prior to any treatment. Patient survival improved with the use of total lymphoid irradiation in the most recent 7 patients.     

Heart- and heart-lung transplantation in children

Since January 1987, 16 prepubertal children have undergone heart (13) or heart-lung (3) transplantation. Immunosuppression included cyclosporine and azathioprine and excluded steroids except in case of rejection. The indications for heart transplantation were hypoplastic left heart syndrome (4 infants, mean age = 2 months), congenital heart disease (4 patients, mean age = 5.7 years) and cardiomyopathy (5 patients, mean age = 2.8 years). There were 4 early deaths (acute graft failure in 2, pulmonary hypertension in 1, infection in 1) and 1 late death (heart failure at 3 months). The 8 survivors had a mean follow-up of 12 months (range 1-19 months). Late complications were minimal. There were 4 episodes of rejection in 2 patients. There was no infection, normal somatic growth and no systemic hypertension. Renal function remained within normal limits although mild-to-moderate tubulointerstitial lesions were found in 4 renal biopsies. Three children (9-11 years old) underwent heart-lung transplantation. The early postoperative course was difficult with 6 episodes of rejection and 5 infections. One patient died at 3 months from infectious complications. One child has a complete rehabilitation 8 months posttransplantation. The last patient is clinically well at 7 months but has a residual tracheal stenosis. The long-term fate of these children, and particularly the long-term effects of cyclosporine therapy are unknown. Heart and heart-lung transplantation remain under investigation but may be reasonable approaches for infants and children with end-stage cardiac and/or pulmonary disease.     

Preoperative chemotherapy in 'unresectable' hepatoblastoma

Chemotherapy was used to treat 11 children with hepatoblastoma that was judged to be unresectable because of tumor tissue in both lobes of the liver (eight patients) or because of size of the primary tumor (three patients). Three with bilobar involvement also had metastatic disease. Adriamycin was used in all patients. In nine, it was used in combination with cisplatin. A combination of other agents was used in four of these children. After two to six cycles (mean, 4 cycles), eight primary tumors exhibited marked response with greater than 50% reduction in size. Metastases disappeared in two patients. Complete resection of residual tumor was attempted in eight cases, and was successful in seven. One patient died at the time of surgery during an extended right hepatectomy. Two children had anaplastic hepatoblastomas that did not respond to chemotherapy, and the children died. One responder with giant cell hepatitis died from a severe coagulopathy and bleeding during chemotherapy before surgery. With preoperative chemotherapy, seven of 11 children with "unresectable" hepatoblastoma are now alive without disease 4 to 42 months following successful resection.     

Liver transplantation for arteriohepatic dysplasia (Alagille's syndrome)

Thirteen out of 268 children (<18 years old) underwent hepatic transplantation (OLT) for end-stage liver disease (ESLD) associated with arteriohepatic dysplasia (AHD). Seven children are alive and well with normal liver function. Six children died, four within 11 days of the operation and the other two at 4 and 10 months after the OLT. Vascular complications with associated septicemia were responsible for the deaths of three children. Two died of heart failure and circulatory collapse, secondary to pulmonary hypertension and congenital heart disease. The remaining patient died of overwhelming sepsis not associated with technical complications. Seven patients had a portoenterostomy or portocholecystostomy early in life; five of these died after the OLT. Severe cardiovascular abnormalities in some of our patients suggest that complete hemodynamic monitoring with invasive studies should be performed in all patients with AHD, especially in cases of documented hypertrophy of the right ventricle. The improved quality of life in our surviving patients confirms the validity of OLT as a treatment of choice in cases of ESLD due to AHD.     

肝移植治疗终末期肝泡型包虫病

目的 探讨肝移植治疗终末期肝泡型包虫病的临床应用价值.方法 回顾性分析2000年12月至2010年8月新疆医科大学第一附属医院收治的8例行肝移植治疗终末期肝泡型包虫病患者的临床资料,观察患者手术时间、无肝期、术中红细胞悬液输注量、术后并发症等指标.结果 8例患者中位手术时间为635 min(490～760 min),中位无肝期为66 min(44～240 min),术中红细胞悬液输注量中位数为20U(4～40 U).1例行抢救性经典原位肝移植的患者因严重的肝性脑病、肾功能衰竭和凝血功能障碍于术后1 d死亡,其余7例患者手术成功.术后中位随访时间6个月(3～29个月).3例患者分别于术后3、5、6个月因胆源性脓毒血症、胆道铸型、急性排斥反应等并发症死亡.1例患者术后发生胆管吻合口狭窄,行胆肠吻合后痊愈;1例患者左肺转移灶明显缩小和稳定;1例患者术后发生胆管吻合口漏经治疗后痊愈;1例行左半健侧肝脏自体移植的患者,健康生存.结论 终末期肝泡型包虫病是肝移植的适应证.低剂量免疫抑制剂和术后长期服用抗包虫药物是预防术后肝泡型包虫病复发和转移的关键.自体肝移植术后因无需免疫抑制剂治疗,是治疗终末期肝泡型包虫病的理想术式.     

肝肾联合移植15例报道

目的探讨肝肾联合移植的适应证和疗效。方法对2001年2月至2003年12月施行肝肾联合移植术的15例患者进行了随访。15例中,乙型肝炎后肝硬化合并肝肾综合征8例、合并尿毒症2例、合并糖尿病肾病1例;多囊肝和多囊肾2例;Caroli病合并多囊肾1例;酒精性肝硬化合并尿毒症1例。对肝肾联合移植患者的手术方式,围手术期并发症,术后急、慢性排斥反应和乙型肝炎复发情况及随访结果进行了分析。结果15例肝肾联合移植术后移植物功能均恢复良好,6个月和1年生存率为100%。1例术前有严重营养不良者,术后给与48d的呼吸机支持后康复。术后创面出血和消化道出血各1例,经非手术治疗后治愈。胆道吻合口狭窄1例,用内镜下球囊扩张术治愈。1例术后2周发生急性移植肝排斥反应,给予激素冲击治疗后得到控制。1例术后30个月时因停用拉米夫定后乙型肝炎复发死于移植肝功能丧失。结论肝肾联合移植是终末期肝病合并慢性肾功能衰竭或肾功能损害的安全有效方法。对乙型肝炎患者术后尽早应用拉米夫定和乙型肝炎病毒免疫球蛋白预防肝炎复发。     

Cerebrovascular occlusive disease in children: A survey

Summary Eight cases of stroke in children treated with surgery are reported. Four of the patients had Moya-moya disease and presented with TIA. They were treated by surgically creating collaterals through the STA-MCA bypass, encephalomyosyangiosis and encephalomyoarteriosynangiosis operations. Three patients with traumatic or idiopathic carotid artery stenosis or occlusion presented with TIA and were treated with STA-MCA bypass. One patient with idiopathic carotid artery stenosis presented with a complaint of a continuous swishing noise in the ear and had a STA-MCA bypass followed by carotid artery ligation. Although stroke in children is usually secondary to systemic disease, there is a group of children with surgically treatable disease who need to be identified and treated. The potential benefit of surgery may depend on the promptness with which a definitive diagnosis is reached.     

The Pierce-Donachy ventricular assist device as a bridge to cardiac transplantation

The Pierce-Donachy ventricular assist device (VAD) was used as an attempted bridge to orthotopic cardiac transplantation in 12 patients aged 13 to 55 years. Ischemic (4 patients), dilated (4 patients), acute viral (1 patient), postpartum (1 patient), and hypertrophic cardiomyopathy (1 patient), along with a failed transplant (1 patient), were the causative factors of end-stage cardiomyopathy in these patients. All patients were candidates for orthotopic cardiac transplantation but sustained refractory cardiogenic shock (cardiac index less than 2 L/min/m2). Left VADs were placed in all patients; 7 also required right VADs. Four patients died of hemorrhagic complications less than 24 hours after VAD insertion. Ventricular assist device stabilization was successful in 8 patients and support ranged from eight hours to 64 days. Seven patients successfully underwent orthotopic cardiac transplantation. One died postoperatively of hemorrhagic complications, 6 were discharged from the hospital, and 1 patient died at 3 months of cytomegalovirus infection. Five patients are long-term survivors. The Pierce-Donachy VAD is an effective means for supporting critically ill patients with end-stage cardiomyopathy and cardiogenic shock before orthotopic cardiac transplantation. Death is related to hemorrhagic, rather than infectious or thromboembolic, complications. Patients successfully stabilized with the VAD can undergo orthotopic cardiac transplantation with acceptable mortality and morbidity rates.     

Treatment options for severe cardiac amyloidosis: heart transplantation combined with chemotherapy and stem cell transplantation for patients with AL-amyloidosis and heart and liver transplantation for patients with ATTR-amyloidosis.

OBJECTIVE: Cardiac amyloidosis (CA) is associated with a poor prognosis and a survival rate of less than 30% 2 years after clinical manifestation. Considered as a semi-malignant disease, CA is often a contraindication for HTx; however, depending on the type of CA, there are excellent treatment regimes that can be combined with HTx. In AL-amyloidosis, chemotherapy and stem cell transplantation are necessary and in TTR-amyloidosis, where the liver is the source of the pathologic protein, liver transplantation is recommended after HTx. METHODS AND RESULTS: More than 60 patients with AL-amyloidosis and more than 25 patients with ATTR-amyloidosis have been investigated at our centre. Eighteen patients showed signs of end-stage heart failure. Four patients died within 1 month after listing for HTx. Seven patients with AL (mean age 41.8 years) and five patients with ATTR-amyloidosis (mean age 42.6 years) were successfully transplanted with an actual survival rate of 91.6%. One patient died 8 months after HTx due to infection. Five AL patients received chemotherapy and SCT and one ATTR patient was liver transplanted. Three AL patients showed complete remission of amyloidosis. CONCLUSIONS: Cardiac amyloidosis is a potentially curative disease after HTx when combined with either chemotherapy and SCT or LiverTx depending on the type of the amyloidosis. Due to the natural course of the disease, urgent HTx after cardiac manifestation is mandatory. With this approach, excellent survival rates and even remission of the underlying disease is possible.     

Rescue cardiac transplantation for early failure of the Fontan-type circulation in children

OBJECTIVE: Early failure of the Fontan-type circulation is a potentially fatal complication. We review our experience with cardiac transplantation in children presenting with end-stage heart failure in this scenario. METHODS: We performed a retrospective review. Between 1985 and 2003, 6 children aged less than 16 years were referred for cardiac transplantation. The indication for cardiac transplantation was end-stage cardiac failure early after the completion of the Fontan-type operation. RESULTS: All 6 patients listed for transplantation underwent cardiac transplantation; the median interval to transplantation from the operation was 36 days (range, 6-180 days). Four patients had undergone the Fontan procedure, and 2 had one-and-a-half-ventricle repair. All 6 patients were ventilated and inotrope dependent, with varying degrees of multiorgan dysfunction. One patient was bridged to transplantation with extracorporeal membrane oxygenation. The median age at transplantation was 7.1 years (range, 3-12.5 years), and weight was 18.9 kg (range, 11-35 kg). One patient died on the operating table (graft failure and hemorrhage). In 5 survivors the median intensive care unit stay was 10 days (range, 8-61 days). On follow-up of 6 to 81 months, there have been no subsequent deaths, and the quality of life in survivors is good. CONCLUSION: Rescue cardiac transplantation in the setting of an early failure of the Fontan-type circulation and end-stage cardiac failure is an effective treatment option and can be performed with acceptable early mortality and encouraging short-term to medium-term results.     

Transjugular intrahepatic portosystemic shunt after adult liver transplantation: experience in eight patients.

BACKGROUND: Transjugular intrahepatic portosystemic shunting (TIPS) has become an effective treatment for the complications of portal hypertension. We assessed the feasibility and outcome of TIPS in liver transplant recipients. METHODS: During the period from December 1992 to January 1998, eight adults presenting recurrent hepatitis C virus (five patients) and hepatitis B virus (one patient) infection, veno-occlusive disease (one patient), and secondary biliary cirrhosis (one patient) had TIPS because of refractory ascites (five patients), bleeding esophageal varices (one patient), refractory hepatic hydrothorax (one patient), retransplantation (two patients), and redo-biliary surgery (one patient). RESULTS: In two patients, the procedure was difficult due to cavo-caval implantation. Ascites, hydrothorax, and variceal bleeding were controlled in all patients. Moderate to severe encephalopathy developed in four patients; two patients had worsening of their existing encephalopathy. Three of five patients treated with cyclosporine needed a drastic dose reduction due to the development of severe side effects. No long-term survivor developed shunt stenosis or occlusion. Two patients did moderately well at 6 and 14 months, respectively; the former died due to chronic rejection while waiting for a retransplantation. Three did well at 14, 36, and 28 months, respectively; the latter patient died of liver failure 32 months after TIPS. One jaundiced patient died after 1.5 months due to necrotic pancreatitis. Two patients died after 4 and 8.5 months, respectively, due to liver failure; the latter was doing well until 7 months after TIPS. CONCLUSIONS: TIPS is feasible in transplant recipients in cases of decompensated allograft cirrhosis, of allograft veno-occlusive disease or when retransplantation or redo-biliary surgery are scheduled in the presence of portal hypertension. At transplantation, the surgeon should keep in mind the eventuality of a later TIPS procedure. Close immunosuppression monitoring is warranted because modified metabolization of cyclosporine (and probably tacrolimus) may cause serious side effects.     

The risk of stroke in patients with asymptomatic carotid stenosis undergoing cardiac surgery: a follow-up study

During a 7-year period, 4047 patients underwent a battery of noninvasive carotid tests before cardiac surgery. Two thirds of the patients with abnormal studies underwent carotid angiography. One hundred fifty-three patients (3.8%) had significant carotid disease, narrowing the luminal diameter by greater than 50%. The incidence of transient ischemic attack or cerebrovascular accident following cardiac surgery was 1.9% in those patients with no carotid disease and 9.2% in those patients with carotid lesions. The incidence of transient ischemic attack or cerebrovascular accident in 32 patients with inoperable (occluded) carotid vessels was 15.6% and in 121 patients with operable (stenotic) lesions was 7.4%. In the group of patients with stenosis, 57 patients underwent carotid operation with an 8.8% incidence of neurologic deficit. During the last 1 1/2 years, no patient with asymptomatic carotid stenosis underwent simultaneous carotid and coronary surgery. Four of 64 patients with combined lesions but no carotid surgery (6.3%) had a neurologic deficit, one of which was severe and permanent. The highest incidence of neurologic dysfunction occurred in patients with unilateral occlusions and contralateral stenosis. Four of 12 patients in this group had a deficit (three of seven patients underwent operation; one of five did not), one of which was permanent. The operative mortality rate after cardiac surgery was three times higher in patients with carotid disease than in those patients with normal carotid arteries. Combined carotid and coronary surgery is currently reserved for patients with neurologic symptoms and severe cardiac disease.     

Diaphragmatic repair of two cases of hepatic hydrothorax using video-assisted thoracoscopic surgery

Hepatic hydrothorax is defined as the presence of a significant pleural effusion that develops in a patient with cirrhosis of the liver who does not have underlying cardiac or pulmonary disease. There are few published case reports dealing with hepatic hydrothorax treated surgically because patients with hepatic hydrothorax have end-stage liver disease. Recently, we treated two patients with refractory hepatic hydrothorax by directly suturing the diaphragmatic defects during video-assisted thoracoscopic surgery (VATS). During surgery, the diaphragmatic defects were identified using abdominal insufflation of saline with indocyanine green or carbon dioxide. After suture closure using fibrin glue, both right pleural effusions were improved. The patients’ postoperative courses were uneventful, and they did not require a drainage tube when they were discharged.     

Management of congenital tracheal stenosis

Objectives: Congenital tracheal stenosis is a rare disease. Various methods for treatment exist but there is still much debate as to the appropriate surgical procedure. We present our surgical experiences of patch tracheoplasty and slide tracheoplasty as viable methods for the treatment of congenital tracheal stenosis. Methods: From 1994 to 2002, 13 patients were diagnosed with congenital tracheal stenosis. Eight patients (7 symptomatic and 1 asymptomatic) had their stenosis corrected, three by means of pericardial patch tracheoplasty, four by slide tracheoplasty, and one by resection and anastomosis. Concomitant operations were performed on six patients to treat congenital cardiovascular disease. Five patients showing no significant symptoms did not undergo tracheal surgery and received only cardiac procedures. A retrospective review of the hospital course, complications, and long-term results was conducted. Results: Among the patch tracheoplasty group, every patient suffered from granulation tissue formation. One patient died of respiratory acidosis and one was hospitalized due to recurrent granulation tissue, which required frequent bronchoscopy. The third patient from this group is free of all symptoms. Among the slide tracheoplasty group, one patient died of anastomosis disruption. The three remaining patients are alive and well. The one patient who received resection and anastomosis is alive without symptoms. Conclusions: Surgical repair of long-segment congenital tracheal stenosis exhibited high mortality and morbidity rates. Every patient that underwent pericardial patch tracheoplasty suffered from troublesome granulation tissue. As slide tracheoplasty provided relatively good results in the short and mid-term follow-up periods, it seems to be a preferred method for the treatment of long-segment congenital tracheal stenosis.     

Management and outcome of major bile duct injuries after laparoscopic cholecystectomy: From therapeutic endoscopy to liver transplantation

Laparoscopic cholecystectomy is associated with a higher rate of bile duct injuries than an open cholecystectomy. The annual incidence of bile duct injuries has remained almost constant and these injuries tend to be more serious, making demands on the method of repair. We wanted to report the management and outcome of major bile duct injuries after laparoscopic cholecystectomy in patients referred to a hepatobiliary and liver transplantation unit. Eighteen patients (14 women), with a median age of 53.5 years were referred to the liver surgery unit with a major bile duct injury after laparoscopic cholecystectomy. The injury was identified after a median of 3 days (range, 0 to 25 days) after operation and the median time interval to referral was 79 days (0 to 2270 days). Fourteen patients had undergone surgery before referral. By the time of referral, four patients had developed end-stage cirrhosis, necessitating liver transplantation. Three of them had undergone bilioenteric drainage operations at the referring institute. Of the remaining 14 patients, three were managed by therapeutic endoscopic procedures. Ten patients were managed with Roux-en-Y hepaticojejunostomy. One died of septic complications before the repair. A median time for hospitalization in our unit was 33 days (range, 10 to 164 days). At present, 16 patients are alive. One patient died of Kaposi's sarcoma 7 months after liver transplantation. A long interval between bile duct injury and referral was associated with the development of end-stage liver disease. Surgery of biliary lesions is demanding, and surgical experience with multidisciplinary approach, including therapeutic endoscopy and liver transplantation, is necessary for successful outcome. (Liver Transpl 2002;8:1036-1043.)     

Orthotopic liver transplantation for incurable alveolar echinococcosis: report of five cases from west China

Alveolar echinococcosis of the liver, caused by the larval stage of Echinococcus multilocularis, has the characteristics of a slow-growing liver cancer. The aim of the present work was to report a series of patients who received orthotopic liver transplantation (OLT) for life-threatening disease. Our article summarizes the medical history, diagnosis, treatment, and prognosis of five patients who received OLT between 2001 and 2002. Most patients had a long history of symptomatic disease (iterative cholangitis, obstructive jaundice) and repeated abdominal surgery. One patient died during the hospitalization mostly related to bacterial infection and multiple organ failure. Another accidental death happened 3 months later from heart failure. Three patients are alive in good condition verifying that OLT is a feasible option for these end-stage cases. In general, combination therapy-chemotherapy, interventional therapy, radical surgery or/and OLT at an early stage-is proposed in advanced cases of which OLT has clearly played a vital role. Despite major technical difficulties, OLT for incurable disease is feasible. Specific management is needed to improve the results: earlier decision for OLT in symptomatic disease, routine pre- and post-transplant therapy, reduced immunosuppression, and regular follow-up.     

胰肾联合移植改善糖尿病继发病变的初步研究

目的探讨胰肾联合移植术对糖尿病并发肾功能衰竭患者继发病变的改善情况。方法回顾性分析本院糖尿病并发肾功能衰竭患者接受胰肾联合移植术前及术后2年的临床资料。结果 8例胰肾联合移植患者除1例因脑血管意外放弃治疗外,余7例患者术后恢复顺利。平均随访时间23.3(1～56)个月。与术前比较,尿蛋白明显改善(P0.05);视网膜病变、心脏功能等指标改善差异无统计学意义。结论胰肾联合移植术可改善糖尿病并发肾功能衰竭患者的糖尿病肾病。     

Non-transplant cardiac surgery for end-stage dilated cardiomyopathy in small children.

Between October 1997 and December 2000, a total of 7 pediatric patients with end-stage dilated cardiomyopathy (DCM) were referred to our service for heart transplantation; non-transplant cardiac surgery was offered as a biologic bridge. Two patients died before surgery and the in-hospital surgical mortality rate was 50%: 75% in 4 patients who received emergency surgery and 0% in 2 patients who received urgent surgery. Non-transplant cardiac surgery improved clinical outcome and acted as a biologic bridge, instead of a mechanical bridge, to heart transplantation in small children with DCM and severe heart failure.     

Cardiac Surgery in Patients with Human Immunodeficiency Virus Infection: Indications and Results

Ten patients with human immunodeficiency virus (HIV) infections underwent cardiac surgery using cardiopulmonary bypass. All were in Centers for Disease Control (CDC) group II. The cardiac involvement was either urgent or severely symptomatic in all cases. One patient died due to acquired immunodeficiency syndrome (AIDS) unrelated cause. No complications were encountered in this series. Eight of the nine survivors were available for follow-up. Three of these eight patients progressed to AIDS (CDC group IV) and subsequently died. Five patients are alive and in CDC group II. Prognosis of the HIV infection and the natural history of the cardiac disease are the two main elements to be considered whenever cardiac surgery is required.     

Vascular complications after living donor liver transplantation: a Brazilian, single-center experience

Background

In living donor liver transplantation (LDLT), vascular complications are more frequently seen than in deceased donor transplantation. Early arterial, portal vein, or hepatic vein thromboses are complications that can lead to graft loss and patient death. The aim of this study was to assess the incidence, treatment, and outcome of vascular complications after LDLT in a single Brazilian center.

Methods

Between December 2001 and December 2010, we performed 130 LDLT. Sixty-four recipients were children (27 weighing <10 kg).

Results

Nine recipients had vascular complications. Hepatic artery thrombosis (HAT) occurred in 4 (3.1%), portal vein thrombosis (PVT) in 3 (2.3%), and hepatic vein thrombosis (HVT) and hepatic arterial stenosis (HAS) in 1 (0.8%) patient each. Complications were identified by Doppler and confirmed by angiography or angiotomography. Patients with HAT were listed for retransplantation. One died before retransplant. Two children were submitted to retransplantation; one is still alive, with neurologic sequelae. One adult with HAT was retransplanted with a deceased donor graft and is doing well 58 months after surgery. Two patients with PVT died as a consequence of graft malfunction. In the other case, portal vein arterialization was performed, but patient died 11 months posttransplant. HVT was detected after cardiac reanimation and was treated with an endovascular stent. This patient died 3 months after LDLT. HAS was diagnosed after liver abscess development and was successfully treated by endovascular angioplasty. No recurrence was observed after 22 months. Follow-up ranged from 9 to 117 months.

Conclusion

Pediatric patients are more prone to develop vascular complications after LDLT. Long-term survival was statistically lower for recipients with vascular complications (33.3% vs 77.7%; P = .008).