Centrilobular necrosis as a manifestation of venous outflow block in pediatric malnourished liver transplant recipients--case reports.

CLN is a frequent histological finding in biopsies after pediatric: LT, and its pathogenesis has not yet been fully clarified and has different causes. Among the vascular causes, VOB is sometimes difficult to diagnose, especially when technical variants such as split-liver, reduced-liver, or living-related LT are utilized. Three liver-transplanted malnourished children (ages 12, 20, and 28 months) developed altered LFTs and post-operative ascites with right pleural effusion (two cases) and jaundice (one case). Doppler ultrasound examinations were normal and liver biopsies showed CLN interpreted as severe ACR. There were no responses to the medical treatment. Additional investigation with CT angiography suggested obstructed hepatic vein drainage, which was confirmed by interventional radiology and angioplasty of the anastomosis between the hepatic vein and the inferior vena cava, with clinical and histological resolution. It is concluded that in malnourished children undergoing LT with technical variations, in which the occurrence of severe ACR is usually less common because of the severity of the patient condition, the finding of CLN should raise the possibility of VOB, so that excessive immunosuppression and its consequences can be avoided.

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.

[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.