Manoel Carlos Prieto Velhote

The Endoscopic and Surgical Management of Portal Hypertension in Children: Analysis of 123 Cases

Since 1973, 178 children with portal hypertension (PH) have been seen at Instituto da Criança of the University of São Paulo Medical School. Fifty-five of these children were excluded from this analysis for various reasons, including no treatment required, death before treatment, or incomplete data. From the remaining 123 children with esophageal varices, only 96 (76.1%) of them had at least one episode of upper gastrointestinal hemorrhage. Eighty-eight children were submitted to injection sclerotherapy; 26 treated prophylactically, and 62 for treatment of previous bleeding. Eleven (42.3%) children from the prophylactic group bled from esophageal varices during the treatment. They were all successfully managed thereafter. Satisfactory results were achieved in 53 (85.4%) children in the therapeutic group. Twenty-eight (45.1%) children had at least one episode of bleeding after beginning of sclerotherapy, 19 of whom eventually had successful control of the variceal bleeding. From 1973 to 1984, distal splenorenal shunt (DSS) was the procedure of choice for the treatment of bleeding esophageal varices. Forty-two children have undergone DSS during this period. Only one child was shunted prophylactically. Since 1985, injection sclerotherapy has been the first choice for the treatment and only seven children with sclerotherapy failure have since been treated by DSS. Characteristically these children had very similar splenoportographic pattern with huge esophageal and gastric varices and deviation of portal vein blood flow toward the left gastric vein.(ABSTRACT TRUNCATED AT 250 WORDS)

Digestive Adaptation with Intestinal Reserve: A Neuroendocrine-Based Operation for Morbid Obesity

Background: Mechanical obstacles to food ingestion, nutrient-excluded segments and malabsorption are common strategies of bariatric surgery which are a potential cause of symptoms or complications. We describe an operation Digestive Adaptation with Intestinal Reserve (DAIR) that does not utilize these tools, aiming fundamentally at neuroendocrine changes. Methods: The operation includes sleeve gastrectomy, omentectomy and enterectomy, maintaining the initial 40 cm of jejunum and final 260 cm of ileum (keeping the bowel length at the lower limit for adaptation to normal). Jejunum is laterally anastomosed to ileum 80 cm proximal to the cecum. A gastroileostomy creates a transit bipartition (ileum and proximal bowel in transit). 55 patients are presented whose follow-up is >12 months (12-34 months). Fasting ghrelin and resistin, and postprandial GLP-1 and PYY were measured. Results: Mean BMI reduction was 4.8, 9.5, 15.4 and 20.1 kg/m2 respectively at 1, 3, 6 and 12 months. Patients have early satiety and major improvement in pre-surgical co-morbidities, especially diabetes and hypertension. GLP-1 and PYY response to food ingestion were enhanced; fasting ghrelin and resistin were significantly reduced (P<0.05). Radiographic studies show nutrient transit through the pylorus and through the gastroileostomy. Early surgical complications (2 in 55 patients) resolved without sequelae. There were no signals of malabsorption, no deaths, and most patients present no symptoms at all. Conclusions: DAIR amplifies postprandial neuroendocrine response and provokes intense weight loss. DAIR reduces production of ghrelin and resistin and enables more nutrients to be absorbed distally enhancing GLP-1 and PYY secretion. Diabetes improved significantly without duodenal exclusion.

Hepatic venous reconstruction in pediatric living‐related donor liver transplantation – Experience of a single center

Abstract:u2002 In pediatric patients submitted to living related liver transplantation, hepatic venous reconstruction is critical because of the diameter of the hepatic veins and the potential risk of twisting of the graft over the line of the anastomosis. The aim of the present study is to present our experience in hepatic venous reconstruction performed in pediatric living related donor liver transplantation. Fifty‐four consecutive transplants were performed and two methods were utilized for the reconstruction of the hepatic vein: direct anastomosis of the orifice of the donor left or left and middle hepatic veins and the common orifice of the recipient left and middle hepatic veins (group 1–26 cases), and wide triangular anastomosis after creating a wide triangular orifice in the recipient inferior vena cava at the confluence of all the hepatic veins with an additional longitudinal incision in the inferior angle of the orifice (group 2–28 cases). In group 1, eight patients were excluded because of graft problems in the early postoperative period and five among the remaining 18 patients (27.7%) presented stricture at the site of the hepatic vein anastomosis. All these patients had to be submitted to two or three sessions of balloon dilatations of the anastomoses and in four of them a metal stent had to be placed. The liver histopathological changes were completely reversed by the placement of the stent. Among the 28 patients of the group 2, none of them presented hepatic vein stenosis (pu2003=u20030.01). The results of the present series lead to the conclusion that hepatic venous reconstruction in pediatric living donor liver transplantation must be preferentially performed by using a wide triangulation on the recipient inferior vena cava, including the orifices of the three hepatic veins. In cases of stenosis, the endovascular dilatation is the treatment of choice followed by stent placement in cases of recurrence.

Sleeve gastrectomy with transit bipartition: a potent intervention for metabolic syndrome and obesity.

Objective: To present 5-year results of sleeve gastrectomy (SG) with transit bipartition (TB) as a metabolic intervention for obesity. Background: Recent data suggest that high glycemic index foods may lead to a hormonally hyperactive proximal gut and a hypoactivate distal gut, which are linked to metabolic syndrome. TB was designed to counterbalance these effects. Methods: A total of 1020 obese patients with body mass index (BMI) ranging from 33 to 72 Kg/m2 underwent SG and TB (SG + TB). TB creates a gastroileal anastomosis in the antrum after the SG; nutrient transit is maintained in the duodenum, avoiding blind loops and minimizing malabsorption. The stomach retains 2 outflow pathways. A lateral enteroanastomosis connects both segments at 80 cm proximal to the cecum. Results: Adequate follow-up data were collected in 59.1% of patients from 4 months to 5 years. The average percent of excess BMI loss was 91%, 94%, 85%, 78%, and 74% in the first, second, third, fourth, and fifth year, respectively. Patients experienced early satiety and major improvement in presurgical comorbidities, including diabetes (86% in remission), following surgery. Two deaths occurred (0.2%). Other surgical complications occurred in 6% of patients. Signs of malabsorption were rare. Conclusions: SG + TB is a simple procedure that results in rapid weight loss and remission or major improvement of comorbidities. Strictly aiming at physiological correction, TB avoids prostheses, narrow anastomoses, excluded segments, and malabsorption. Weight and comorbidities are much improved. Diabetes is improved without duodenal exclusion. TB is an excellent complement to an SG.

Mycophenolate mofetil promotes prolonged improvement of renal dysfunction after pediatric liver transplantation: experience of a single center.

Abstract:u2002 Few studies have evaluated the long‐term use of MMF in liver transplanted children with renal dysfunction. The aim of this study is to report the experience of a pediatric transplantation center on the efficacy and security of long‐term use of a MMF immunosuppressant protocol with reduced doses of CNIs in stable liver transplanted children with renal dysfunction secondary to prolonged use of CsA or Tac. Between 1988 and 2003, 191 children underwent OLT and 11 patients developed renal dysfunction secondary to CNIs toxicity as evaluated by biochemical renal function parameters. The interval between liver transplantation and the introduction of the protocol varied from one to 12u2003yr. Renal function was evaluated by biochemical parameters in five phases: immediately prior to MMF administration; 3, 6, 12 and 24u2003months after the introduction of MMF. Among the patients, nine of them (82%) showed improvement of renal function parameters in comparison with the pretreatment values. The two patients that did not show any improvement were patients in whom the interval of time between OLT and the introduction of MMF was longer. All parameters of liver function remained unchanged. No episodes of acute or chronic rejection or increases in infection rates during the period were detected. Two patients developed transitory diarrhea and leukopenia that were reverted with reduction of MMF dosage. In conclusion, in liver transplanted pediatric patients with CNI‐induced chronic renal dysfunction, the administration of MMF in addition to reduced doses of CNIs promotes long‐term improvement in renal function parameters with no additional risks.

A NOTES modification of the transanal pull-through.

Transanal endorectal pull-through (TAEPT) surgery is primarily performed for rectosigmoid aganglionosis, generally with excellent results. There is evidence that overstretching the anus and tension traction in the sigmoid during the procedure could impair the final continence of the patient. Many researchers suggest the use of small umbilical or laparoscopic access to aid in colon mobilization, thus preventing excessive handling within the anal canal. We assumed that transabdominal mobilization of the sigmoid could be prevented by utilizing the NOTES (natural orifices transluminal endoscopic surgery) technique. We performed a TAEPT with NOTES access of the sigmoid vascular pedicle, keeping the surgery exclusively transanal, which prevented scars in the abdomen and minimized the stretching of perineal structures.

Twenty seven-year experience with sternal cleft repair

Congenital sternal cleft is a rare disease, and primary repair in the neonatal period is its best management. In 1998 we presented three surgical techniques for sternal cleft correction, but since 1999 we have elected one of them as our procedure of choice. Our latest results are now presented. It is a review of 15 patients operated from October 1979 to December 2007. Surgical repair consisted of 3 sliding chondrotomies, 10 reconstructions based on a posterior sternal wall, 1 reconstruction combined to Ravitch operation for pectus excavatum and 1 associated with total repair of Cantrells pentalogy. Data concerning epidemiological features, surgical reconstruction, aesthetic results, postoperative major complications, mortality and hospital stay, were collected from hospital charts. Follow-up ranged from 4 months to 27 years. All patients submitted to surgical correction had a good aesthetic and functional result. Neither postoperative mortality nor major complication was observed. Two patients had subcutaneous fluid collection that prolonged the drainage duration. The mean hospital stay was 6 days. In conclusion, reconstructing sternal cleft with a posterior periosteal flap from sternal bars and chondral graft is an effective option with good aesthetic and long-term functional results.

Living Related Donor Liver Transplantation in Children

OBJECTIVEnThe objective of this study was to report our experience with pediatric orthotopic liver transplantation (OLT) with living related donors.nnnMETHODSnWe performed a retrospective chart analysis of 121 living related donor liver transplantations (LRDLT) from June 1998 to June 2010.nnnRESULTSnIndications were biliary atresia (BA; n = 81), primary sclerosing cholangitis (n = 5), α-1 antitrypsin deficiency (n = 4); cholestasis (n = 9), fulminant hepatic failure (n = 8), autoimmune hepatitis (n = 2), Alagille syndrome (n = 4), hepatoblastoma (n = 3), tyrosinemia (n = 2), and congenital hepatic fibrosis (n = 3). The age of the recipients ranged from 7-174 months (median, 22) and the weights ranged from 6-58 kg (median, 10). Forty-nine children (40.5%) weighed ≤10 kg. The grafts included the left lateral segment (n = 108), the left lobe (n = 12), and the right lobe (n = 1). The donors included 71 mothers, 45 fathers, 2 uncles, 1 grandmother, 1 grandfather, and 1 sister with a median age of 29 years (range, 16-53 ys) and a median weight of 68 kg (range, 47-106). Sixteen patients (12.9%) required retransplantation, most commonly due to hepatic artery thrombosis (HAT; n = 13; 10.7%). The other complications were biliary stenosis (n = 25; 20.6%), portal vein thrombosis (PVT; n = 11; 9.1%), portal vein stenosis (n = 5; 4.1%), hepatic vein stenosis (n = 6; 4.9%), and lymphoproliferative disorders (n = 8; 6.6%). The ultimate survival rate of recipients was 90.3% after 1 year and 75.8% after 3 years. Causes of early death within 1 month were HAT (n = 6), PVT (n = 2), severe graft dysfunction (n = 1), sepsis (n = 1), and intraoperative death in children with acute liver failure (n = 2). Causes of late deaths included lymphoproliferative disease (n = 3), chronic rejection (n = 2), biliary complications (n = 3), and recurrent disease (n = 3; hepatoblastoma and primary sclerosing cholangitis).nnnCONCLUSIONSnDespite the heightened possibility of complications (mainly vascular), LRDLT represented a good alternative to transplantation from cadaveric donors in pediatric populations. It was associated with a high survival ratio.

Sirolimus in pediatric liver transplantation: a single-center experience.

BACKGROUND AND AIMSnLiver transplantation (OLT) in children has seen significant improvements in recent years. Long-term immunosuppressive strategies have focused on avoiding the risks of long-term immunosuppression, particularly nephrotoxicity, de novo malignancy and late infections. Since its introduction in renal transplantation in 1999, sirolimus (SRL) has been used by an increasing number of liver transplant centers. The aim of this study was to review the experience using SRL in pediatric liver transplant recipients at a single center.nnnMETHODSnBetween 1989 and 2006, 318 children underwent OLT including 13 who were converted to SRL therapy because of tacrolimus-related side effects. The indications were posttransplant lymphoproliferative disease (PTLD; n = 11), nephrotoxicity (n = 1), and de novo autoimmune hepatitis (n = 1). One patient with PTLD previously concurrently displayed chronic rejection. SRL dosages ranged between 0.4 and 5 mg/d. The median duration of follow-up was 18 months.nnnRESULTSnPTLD recurred in 1 patient. There were no episodes of acute rejection. One child developed hyperlipidemia that resolved with diet and medication.nnnCONCLUSIONSnConversion from tacrolimus to SRL in selected pediatric liver transplant recipients is safe. Children with PTLD may benefit from immunosuppression with SRL after liver transplantation.

Successful treatment of de novo autoimmune hepatitis and cirrhosis after pediatric liver transplantation.

Abstract: Over a 15‐yr period of observation, among the 205 children who underwent liver transplantations, one of them developed a particular type of late graft dysfunction with clinical and histological similarity to autoimmune hepatitis. The patient had α1‐antitrypsin deficiency and did not previously have autoimmune hepatitis or any other autoimmune disease before transplantation. Infectious and surgical complications were excluded. After repeated episodes of unexplained fluctuations of liver function tests and liver biopsies demonstrating reactive or a biliary pattern, without any corresponding alteration of percutaneous cholangiography, a liver‐biopsy sample taken 4u2003yr after the transplant showed active chronic hepatitis progressing to cirrhosis, portal lymphocyte aggregates, and a large number of plasma cells. At that time, autoantibodies (gastric parietal cell antibody, liver–kidney microsomal antibody, and anti‐hepatic cytosol) were positive and serum IgG levels were high. Based on these findings of autoimmune disease, a diagnosis of ‘de novo autoimmune hepatitis’ was made. The treatment consisted of reducing the dose of cyclosporine, reintroduction of corticosteroids, and addition of mycophenolate mofetil. After 19 months of treatment, a new liver‐biopsy sample showed marked reduction of portal and lobular inflammatory infiltrate, with regression of fibrosis and of the architectural disruption. At that time, serum autoantibodies became negative. The last liver‐biopsy sample showed inactive cirrhosis and disappearance of interface hepatitis and of plasma cell infiltrate. Presently, 9u2003yr after the transplantation, the patient is doing well, with normal liver function tests and no evidence of cirrhosis. Her immunosuppressive therapy consists of tacrolimus, mycophenolate mofetil, and prednisolone. In conclusion, the present case demonstrates that de novo autoimmune hepatitis can appear in liver‐transplant patients despite appropriate anti‐rejection immunosuppression, and triple therapy with tacrolimus, mycophenolate mofetil, and prednisolone could sustain the graft and prevent retransplantation.