Which is the best technique for hepatic venous reconstruction in pediatric living-donor liver transplantation? Experience from a single center.

BACKGROUND/PURPOSE: The introduction of the piggyback technique for reconstruction of the liver outflow in reduced-size liver transplants for pediatric patients has increased the incidence of hepatic venous outflow block (HVOB). Here, we proposed a new technique for hepatic venous reconstruction in pediatric living-donor liver transplantation. METHODS: Three techniques were used: direct anastomosis of the orifice of the donor hepatic veins and the orifice of the recipient hepatic veins (group 1); triangular anastomosis after creating a wide triangular orifice in the recipient inferior vena cava at the confluence of all the hepatic veins (group 2); and a new technique, which is a wide longitudinal anastomosis performed at the anterior wall of the inferior vena cava (group 3). RESULTS: In groups 1 and 2, the incidences of HVOB were 27.7% and 5.7%, respectively. In group 3, no patient presented HVOB (P = .001). No difference was noted between groups 2 and 3. CONCLUSIONS: Hepatic venous reconstruction in pediatric living-donor liver transplantation must be preferentially performed by using a wide longitudinal incision at the anterior wall of the recipient inferior vena cava. As an alternative technique, triangulation of the recipient inferior vena cava, including the orifices of the 3 hepatic veins, may be used.

Posterior reversible encephalopathy syndrome after liver transplantation in children: a rare complication related to calcineurin inhibitor effects.

PRES is a neuroclinical and radiological syndrome that results from treatment with calcineurin inhibitor immunosuppressives. Severe hypertension is commonly present, but some patients may be normotensive. We report herein two children who received liver transplants, as treatment for biliary atresia in the first case and for Alagille's syndrome in the second one. In the early postoperative, both patients presented hypertension and seizures. In both cases, the image findings suggested the diagnosis of PRES. The CT scan showed alterations in the posterior area of the brain, and brain MRI demonstrated parietal and occipital areas of high signal intensity. Both children were treated by switching the immunosuppressive regimen and controlling arterial blood pressure. They displayed full recuperation without any neurologic sequelae. Probably, the pathophysiology of PRES results from sparse sympathetic innervation of the vertebrobasilar circulation, which is responsible for supplying blood to the posterior areas of the brain. In conclusion, all liver-transplanted children who present with neurological symptoms PRES should be considered in the differential diagnosis, although this is a rare complication. As treatment, we recommend rigorous control of arterial blood pressure and switching the immunosuppressive regimen.

Early diagnostic and endoscopic dilatation for the treatment of acquired upper airway stenosis after intubation in children.

BACKGROUND: Upper airway stenosis (UAS) after endotracheal intubation is a common problem in children. Most literature refers to a surgical treatment for these lesions. Laryngotracheal reconstruction and cricotracheal resection are used for low- and high-grade stenosis, but decannulation is not always possible immediately after surgery. PURPOSE: The aim of this study was to verify the feasibility and results of endoscopic dilatations for treatment of subglottic stenosis. METHOD: The study encompassed a 12-year retrospective analysis of patients treated for UAS in a tertiary center. All children were symptomatic at the time of the endoscopic diagnosis. The stenosis was graded according to the Myer-Cotton criteria. Endoscopic dilatation was initiated immediately after the diagnosis. Children with grade IV stenosis underwent surgery. RESULTS: Children with tracheal stenosis and no involvement of the subglottic area did not respond to endoscopic dilatations and underwent surgery. There were 45 children with grade I stenosis, 12 with grade II, 7 with grade III, and 4 with grade IV lesions. Patients with grade I, II, and III stenosis were all treated by endoscopic dilatations alone and were decannulated when asymptomatic. The average time for decannulation was 18.90 +/- 26.07 months for grade I, 32.5 +/- 27.08 months for grade II, and 27.57 +/- 20.60 months for grade III stenosis (P < .01, for grade II vs grade III). CONCLUSION: Grade I, II, and III subglottic stenoses can be safely managed by early endoscopic dilatations with a high rate of success and low rate of morbidity but require a significantly long period of treatment.

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

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 12 yr. Renal function was evaluated by biochemical parameters in five phases: immediately prior to MMF administration; 3, 6, 12 and 24 months 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.

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

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 alpha1-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 4 yr 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, 9 yr 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.

An alternative method of arterial reconstruction in pediatric living donor liver transplantation with the recipient right gastroepiploic artery.

The classical method for arterial reconstruction in pediatric living donor liver transplantation using left lateral segment consists of end-to-end anastomosis between the donor left hepatic artery and the recipient right hepatic artery. In the present case, an intra-operative hepatic artery thrombosis occurred because of extensive intima wall dissection of the recipient hepatic artery. The patient was a 6-yr-old boy with fulminant hepatic failure, who underwent living donor partial liver transplantation with left lateral segment from his father. The graft was irrigated by a left hepatic artery and an accessory left hepatic artery from gastric artery, both arteries with diameter of <2 mm. These arteries were anastomosed to the recipient right and left hepatic arteries, respectively. Before performing the bile duct reconstruction it was noted that these anastomoses were occluded by clots of blood. An extensive subintimal dissection of the recipient hepatic artery was the cause of this problem. The creation of a new anastomosis by using a more proximal part of this artery without subintimal dissection was judged impossible. Then, the right gastroepiploic artery was mobilized and an anastomosis was performed with the donor left hepatic artery in an end-to-end fashion. Arterial blood flow to the graft was established successfully and the patient's postoperative recovery was excellent. Fifteen days after the transplantation, an angiotomography demonstrated a good hepatic arterial blood flow. The patient is now alive and well, 4 months after the transplantation. In conclusion, the method of hepatic graft arterialization described here is an important option for patients who undergo living donor or split liver transplantation.

Hepatic venous reconstruction in pediatric living-related donor liver transplantation--experience of a single center.

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 (p = 0.01). 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.

[Prognostic factors and survival in neonates with congenital diaphragmatic hernia]. / Fatores prognósticos e sobrevida em recém-nascidos com hérnia diafragmática congênita.

OBJECTIVE: To evaluate the prognostic factors importance to survival in neonatal period of newborns with congenital diaphragmatic hernia treated at Hospital das Clínicas, School of Medicine of Universidade de São Paulo, and to compare the outcome with data published in medical literature. So that the results of this study might allow updating family counseling and guiding changes in clinical management of our department. METHODS: Retrospective study of 27 consecutive newborns with congenital diaphragmatic hernia admitted to the Pediatric Surgery Department of Instituto da Criança, School of Medicine of Universidade de São Paulo, from April 1991 to January 2002, and statistical comparison with medical literature metanalysis data. RESULTS: Of 27 patients, 15 were born at our institution and 12 were admitted by transference after birth. Twelve (44%) have had congenital diaphragmatic hernia diagnosed prenatally and 23 (85%) were full-term newborns. Most patients presented early respiratory distress and needed intubation at delivery room. Six newborns presented criteria for indication of extracorporeal membrane oxygenation. Twenty patients (74%) were submitted to operative repair and seven (26%) died without the minimal clinical stabilization necessary for surgical procedure (five of these patients reached criteria for indication of extracorporeal membrane oxygenation). The postoperative mortality was 25% (5/20). The overall survival of neonatal period was 56% (15/27). The survival of patients that were born at our hospital was 33% (4/12), and the survival of the newborns admitted by transference was 73% (11/15). Severe respiratory distress, early indication to mechanical ventilation and severe hypoxemia (post-ductal pO2 < 100 mmHg despite all efforts) were identified as predictors of bad outcome with statistical significance. CONCLUSION: Our high mortality rate of newborns with congenital diaphragmatic hernia is statistically similar to that described in international publications. In the group of non-responsive patients to standard treatment available, the use of extracorporeal membrane oxygenation should be able to reduce mortality. The impact of this therapeutical strategy in the overall survival depends on other factors that were not analyzed in the present study. Family counseling of patients' parents on congenital diaphragmatic hernia in our department may follow the same patterns referred in world medical literature.

Anéis vasculares na infância: dignóstico e tratamento / Vascular rings in childhood: diagnosis and treatment

Objetivo: apresentar a experiência do Serviço de Cirurgia Pediátrica do Instituto da Criança do HCFMUSP no diagnóstico e tratamento de crianças com anomalias do arco aórtico e definir a importância dos exames complementares para o diagnóstico. Método: estudo retrospectivo de 22 crianças com diagnóstico de compressão traqueoesofágica por anel vascular tratadas no Instituto da Criança, no período de 1985 a 2000, investigando-se dados clínicos pré e pós-operatórios, exames complementares e evolução.Resultados: a anomalia vascular mais freqüente foi artéria inominada direita anômala (10 casos), seguido de duplo arco aórtico (7 casos) e arco aórtico à direita (5 casos). Os sintomas predominantes foram respiratórios (86por cento) e de início precoce (76por cento desde o período neonatal). Entretanto, o diagnóstico definitivo na maioria dos casos (60por cento) só foi estabelecido após l ano de vida. O exame mais importante para o diagnóstico foi o esofagograma. A correção de todas anomalias foi realizada por toracotomia póstero-lateral esquerda. Não ocorreram complicações cirúrgicas. A evolução foi pior nos casos operados mais tardiamente. Todas as crianças permanece- ram sintomáticas por até 6 meses, apesar de significativa melhora no pós-operatório. Conclusão: o diagnóstico de anel vascular deve ser investigado nas crianças com sintomas respiratórios de início precoce e nas "chiadoras'. de difícil controle. O diagnóstico pode ser realizado de forma simples através do esofagograma. Os demais exames de imagem acrescentam poucas informações e são dispensáveis na maioria dos casos. Os sintomas respiratórios podem persistir com menor intensidade por períodos variáveis no pós-operatório

[Vascular rings in childhood: diagnosis and treatment]. / Anéis vasculares na infância: diagnóstico e tratamento.

OBJECTIVE: To present the study carried out by the Pediatric Surgery Department of Instituto da Criança at the Medical School of Universidade de São Paulo regarding the diagnosis and treatment of children with aortic arch abnormalities and to define the role of complementary exams for diagnosis. METHODS: Retrospective study of 22 patients with diagnosis of tracheoesophageal compression treated at Instituto da Criança from 1985 to 2000, analyzing pre- and postoperative clinical data, diagnostic exams and outcome. RESULTS: The most frequent diagnosis was right aberrant innominate artery (10 cases), followed by double aortic arch (7 cases) and right aortic arch (5 cases). Respiratory symptoms (86%) and early manifestation (76% since the neonatal period) were predominant. Nevertheless, most cases (60%) had the definitive diagnosis established only after 1 year of life. The most relevant examination for the diagnosis was the esophagogram. The correction of all the anomalies was carried out through left postero-lateral thoracotomy. There were no surgical complications. The outcome was worse in patients with delayed treatment. All children remained symptomatic for up to 6 months, although they had significant improvement in the postoperative period. CONCLUSIONS: The diagnosis of vascular rings should be considered in children with early respiratory symptoms and in the wheezing baby with difficult control. The diagnosis may be established just through the esophagogram. Other image studies add few information and they are unnecessary in most cases. Less severe symptoms may persist for variable periods.