MARCOS MARQUES DA SILVA

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Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina - Médico

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Agora exibindo 1 - 10 de 23
  • article 0 Citação(ões) na Scopus
    Omphalocele and biliary atresia: chance or causality. A case report
    (2022) ROSA, Julia Amim; PINTO, Ana Maria Rossignolli; BIGIO, Juliana Zoboli Del; LIMA, Larissa Barbosa; SILVA, Marcos Marques da; MANO, Rafaela Braga Cabrera; FALCAO, Mario Cicero
    To relate omphalocele and biliary atresia and investigate possible embryological correlations that justify the simultaneous occurrence. A female preterm newborn diagnosed as omphalocele; cesarean delivery, weight 2,500g, 46 XX karyotype. Initially, the newborn remained fasting and on parenteral nutrition, and enteral diet was introduced later, with good acceptance. On the 12th day of life, the newborn presented direct hyperbilirubinemia, increased levels of liver enzymes and fecal acholia, with a presumptive diagnosis of biliary atresia. However, the ultrasound was inconclusive, due to anatomical changes resulting from omphalocele. A surgical approach was chosen on the 37th day of life aiming to confirm diagnosis of biliary atresia and to repair omphalocele. During the surgical procedure, structural alterations compatible with biliary atresia were observed, later confirmed by pathological examination; a hepatoportoenterostomy was performed and the omphalocele was corrected. She evolved well in the postoperative period, with a decrease in direct bilirubin and liver enzymes, as well as resolution of fecal acholia, and was discharged in good clinical condition. This is a bizarre and extremely rare association, but the prognosis may be good when an early diagnosis is made and surgery performed, besides support and clinical management to prevent complications in the perioperative period. Although the pathogenesis of the diseases has not been fully defined yet, there is, to date, no direct relation between them. The association between omphalocele and biliary atresia is extremely uncommon, with only two published cases.
  • article 6 Citação(ões) na Scopus
    Living donor liver transplantation in children: Should the adult donor be operated on by an adult or pediatric surgeon? Experience of a single pediatric center
    (2014) ANDRADE, Wagner de Castro; VELHOTE, Manoel Carlos Prieto; AYOUB, Ali Ahman; SILVA, Marcos Marques; GIBELLI, Nelson Elias M.; TANNURI, Ana Cristina A.; SANTOS, Maria Merces; PINHO-APEZZATO, Maria Lucia; BARROS, Fabio de; MOREIRA, Daniel Rangel; MIYATANI, Helena T.; PEREIRA, Raimundo Renato; TANNURI, Uenis
    Background/Purpose: Living donor liver transplantation has become a cornerstone for the treatment of children with end-stage hepatic dysfunction, especially within populations or countries with low rates of organ utilization from deceased donors. The objective is to report our experience with 185 living donors operated on by a team pediatric surgeons in a tertiary center for pediatric liver transplantation. Methods: Retrospective analysis of medical records of donors of hepatic grafts for transplant undergoing surgery between June 1998 and March 2013. Results: Over the last 14 years, 185 liver transplants were performed in pediatric recipients of grafts from living donors. Among the donors, 166 left lateral segments (89.7%), 18 left lobes without the caudate lobe (9.7%) and 1 right lobe (0.5%) were harvested. The donor age ranged from 16 to 53 years, and the weight ranged from 47 to 106 kg. In 10 donors, an additional graft of the donor inferior mesenteric vein was harvested to substitute for a hypoplastic recipient portal vein. The transfusion of blood products was required in 15 donors (8.1%). The mean hospital stay was 5 days. No deaths occurred, but complications were identified in 23 patients (12.4%): 9 patients experienced abdominal pain and severe gastrointestinal symptoms and 3 patients required reoperations. Eight donors presented with minor bile leaks that were treated conservatively, and 3 patients developed extra-peritoneal infections (1 wound collection, 1 phlebitis and 1 pneumonia). Eight grafts (4.3%) showed primary dysfunction resulting in recipient death (3 cases of fulminant hepatitis, 1 patient with metabolic disease, 1 patient with Alagille syndrome and 3 cases of biliary atresia in infants under 1 year old). There was no relation between donor complications and primary graft dysfunction (P = 0.6). Conclusions: Living donor transplantation is safe for the donor and presents a low morbidity. The donor surgery may be performed by a team of trained pediatric surgeons.
  • article 37 Citação(ões) na Scopus
    Living Related Donor Liver Transplantation in Children
    (2011) TANNURI, A. C. A.; GIBELLI, N. E. M.; RICARDI, L. R. S.; SANTOS, M. M.; MAKSOUD-FILHO, J. G.; PINHO-APEZZATO, M. L.; SILVA, M. M.; VELHOTE, M. C. P.; AYOUB, A. A. R.; ANDRADE, W. C.; LEAL, A. J.; MIYATANI, H. T.; TANNURI, U.
    Objective. The objective of this study was to report our experience with pediatric orthotopic liver transplantation (OLT) with living related donors. Methods. We performed a retrospective chart analysis of 121 living related donor liver transplantations (LRDLT) from June 1998 to June 2010. Results. Indications were biliary atresia (BA; n = 81), primary sclerosing cholangitis (n = 5), alpha-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). Conclusions. Despite 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.
  • article 15 Citação(ões) na Scopus
    Rex Shunt for Acute Portal Vein Thrombosis After Pediatric Liver Transplantation in Children With Biliary Atresia
    (2011) GIBELLI, N. E. M.; TANNURI, A. C. A.; TANNURI, U.; SANTOS, M. M.; PINHO-APEZZATO, M. L.; MAKSOUD-FILHO, J. G.; VELHOTE, M. C. P.; AYOUB, A. A. R.; SILVA, M. M.; ANDRADE, W. C.
    Background/Purpose. Posttransplantation portal vein thrombosis (PVT) can have severe health consequences, and portal hypertension and other consequences of the long-term privation of portal inflow to the graft may be hazardous, especially in young children. The Rex shunt has been used successfully to treat PVT patients since 1998. In 2007, we started to perform this surgery in patients with idiopathic PVT and late posttransplantation PVT. Herein we have reported our experience with this technique in acute posttransplantation PVT. Methods. Three patients of ages 12, 15, and 18 months underwent cadaveric (n = 1) or living donor (n = 2) orthotopic liver transplantation (OLT). All patients had biliary atresia with portal vein hypoplasia; they developed acute PVT on the first postoperative day. They underwent a mesenteric-portal surgical shunt (Rex shunt) using a left internal jugular vein autograft (n = 2) or cadaveric iliac vein graft (n = 1) on the first postoperative day. Results. The 8-month follow-up has confirmed shunt patency by postoperative Doppler ultrasound. There have been no biliary complications to date. Conclusions. The mesenteric-portal shunt (Rex shunt) using an autograft of the left internal jugular or a cadaveric vein graft should be considered for children with acute PVT after OLT. These children usually have small portal veins; reanastomosis is often unsuccessful. In addition, this technique has the advantage to avoid manipulation of the hepatic hilum and biliary anastomosis. Although this study was based on a limited experience, we concluded that this technique is feasible, with great benefits to and low risks for these patients.
  • article 11 Citação(ões) na Scopus
    Longitudinal assessment of lung area measurements by two-dimensional ultrasound in fetuses with isolated left-sided congenital diaphragmatic hernia
    (2015) RUANO, R.; BRITTO, I. S. W.; SANGI-HAGHPEYKAR, H.; BUSSAMRA, L. C. S.; SILVA, M. M. Da; BELFORT, M. A.; DETER, R. L.; LEE, W.; TANNURI, U.; ZUGAIB, M.
    ObjectiveTo evaluate lung growth in healthy fetuses and those with congenital diaphragmatic hernia (CDH) using two-dimensional (2D) ultrasound. MethodsFetal right lung measurements obtained by 2D ultrasound between 19 and 37 weeks' gestation were evaluated longitudinally in 66 healthy fetuses and 52 fetuses with isolated left-sided CDH. Right lung areas were determined by the tracing' and longest-diameters' methods and, subsequently, lung area-to-head circumference ratios (LHRs) were calculated. Functions fitted to these size parameters with respect to gestational age were evaluated for three sets of group-wise comparisons: (1) healthy vs CDH fetuses; (2) different degrees of severity of CDH; and (3) CDH fetuses that survived vs those that died by 6 months postpartum. ResultsThere was a significantly slower increase in right lung areas and LHRs with advancing gestational age in CDH fetuses than in healthy individuals (P < 0.05). Compared to those with milder forms of CDH, lung areas and LHRs of fetuses with more severe forms displayed a smaller increase (P < 0.05) and LHRs of fetuses with severe CDH did not increase during pregnancy (P > 0.05). Individuals who died postpartum did not show any increase in LHR (P > 0.05) throughout gestation. ConclusionsThe right lung area and LHR, calculated using either the longest-diameters or tracing method, display reduced growth rates during gestation in cases of isolated left-sided CDH as compared with healthy fetuses. The growth curve characteristics of fetal lung areas and LHRs may be useful for predicting neonatal mortality.
  • article 110 Citação(ões) na Scopus
    Prediction and probability of neonatal outcome in isolated congenital diaphragmatic hernia using multiple ultrasound parameters
    (2012) RUANO, R.; TAKASHI, E.; SILVA, M. M. da; CAMPOS, J. A. D. B.; TANNURI, U.; ZUGAIB, M.
    Objectives To evaluate the accuracy and probabilities of different fetal ultrasound parameters to predict neonatal outcome in isolated congenital diaphragmatic hernia (CDH). Methods Between January 2004 and December 2010, we evaluated prospectively 108 fetuses with isolated CDH (82 left-sided and 26 right-sided). The following parameters were evaluated: gestational age at diagnosis, side of the diaphragmatic defect, presence of polyhydramnios, presence of liver herniated into the fetal thorax (liver-up), lung-to-head ratio (LHR) and observed/expected LHR (o/e-LHR), observed/expected contralateral and total fetal lung volume (o/e-ContFLV and o/e-TotFLV) ratios, ultrasonographic fetal lung volume/fetal weight ratio (US-FLW), observed/expected contralateral and main pulmonary artery diameter (o/e-ContPA and o/eMPA) ratios and the contralateral vascularization index (Cont-VI). The outcomes were neonatal death and severe postnatal pulmonary arterial hypertension (PAH). Results Neonatal mortality was 64.8% (70/108). Severe PAH was diagnosed in 68 (63.0%) cases, of which 63 died neonatally (92.6%) (P < 0.001). Gestational age at diagnosis, side of the defect and polyhydramnios were not associated with poor outcome (P > 0.05). LHR, o/eLHR, liver-up, o/e-ContFLV, o/e-TotFLV, US-FLW, o/eContPA, o/e-MPA and Cont-VI were associated with both neonatal death and severe postnatal PAH (P < 0.001). Receiver-operating characteristics curves indicated that measuring total lung volumes (o/e-TotFLV and US-FLW) was more accurate than was considering only the contralateral lung sizes (LHR, o/e-LHR and o/e-ContFLV; P < 0.05), and Cont-VI was the most accurate ultrasound parameter to predict neonatal death and severe PAH (P < 0.001). Conclusions Evaluating total lung volumes is more accurate than is measuring only the contralateral lung size. Evaluating pulmonary vascularization (Cont-VI) is the most accurate predictor of neonatal outcome. Estimating the probability of survival and severe PAH allows classification of cases according to prognosis.
  • conferenceObject
    REX SHUNT FOR ACUTE PORTAL VEIN THROMBOSIS AFTER PEDIATRIC LIVER TRANSPLANT IN CHILDREN WITH BILIARY ATRESIA
    (2013) GIBELLI, Nelson Elias Mendes; ANDRADE, Wagner de Castro; VELHOTE, Manoel Carlos Prieto; AYOUB, Ali Abdul Rahman; SILVA, Marcos Marques da; PINHO-APEZZATO, Maria Lucia de; TANNURI, Ana Cristina Aoun; BARROS, Fabio de; RICARDI, Luis Roberto Schlaich; MOREIRA, Daniel de Albuquerque Rangel; MIYATANI, Helena Thie; PEREIRA, Paulo Renato Alencar; TANNURI, Uenis
    BACKGROUND/PURPOSE: zPost transplant portal vein thrombosis (PVT)can be extremely disastrous, and portal hypertension and other consequences of the long term privation of portal inflow to the graft may be hazardous, especially in the very young children. Since 1998, Rex shunt has been used successfully to treat these patients. In 2007 we started to perform this surgery in patients with idiopathic PVT and late post transplant PVT. We report our experience with this technique in acute post transplant PVT. METHODS: Case report of six patients (age–12–18 months) submitted to cadaveric (1) and living donor (5) liver transplant (LT). All patients had biliary atresia with portal vein hipoplasia and developed acute portal vein thrombosis (PVT) in the first post-operative day. They were submitted to a mesenteric-portal surgical shunt (Rex shunt) with left internal jugular vein autograft (5) and cadaveric iliac vein graft (1) in the first post-operative day. RESULTS: Current follow-up of 12 months. Postoperative Doppler ultrasounds confirmed shunt patency. There were no biliary complications until now. CONCLUSION: The mesenteric-portal shunt (Rex shunt) with left internal jugular vein autograft should be considered in children with acute PVT after liver transplantation. These children usually have small portal veins, and reanastomosis is often unsuccessful. In addition, this technique has the advantage that we do not manipulate the biliary anastomosis and the hepatic hilum, thus avoiding biliary complications. Although this is an initial experience, we conclude that this technique is feasible, with great benefits for these patients and with low risks.
  • bookPart
    Diagnóstico pré-natal de malformações congênitas
    (2020) SILVA, Marcos Marques da; STEFANI, Luciana Francine Bocchi de; BERNARDES, Lisandra Stein
  • bookPart
    Hérnia diafragmática congênita
    (2020) CECCON, Maria Esther Jurfest Rivero; SILVA, Marcos Marques da
  • article 52 Citação(ões) na Scopus
    Early fetoscopic tracheal occlusion for extremely severe pulmonary hypoplasia in isolated congenital diaphragmatic hernia: preliminary results
    (2013) RUANO, R.; PEIRO, J. L.; SILVA, M. M. da; CAMPOS, J. A. D. B.; CARRERAS, E.; TANNURI, U.; ZUGAIB, M.
    Objective To evaluate the effect of early fetoscopic tracheal occlusion (FETO) (22-24 weeks' gestation) on pulmonary response and neonatal survival in cases of extremely severe isolated congenital diaphragmatic hernia (CDH). Methods This was a multicenter study involving fetuses with extremely severe CDH (lung-to-head ratio < 0.70, liver herniation into the thoracic cavity and no other detectable anomalies). Between August 2010 and December 2011, eight fetuses underwent early FETO. Data were compared with nine fetuses that underwent standard FETO and 10 without fetoscopic procedure from January 2006 to July 2010. FETO was performed under maternal epidural anesthesia, supplemented with fetal intramuscular anesthesia. Fetal lung size and vascularity were evaluated by ultrasound before and every 2 weeks after FETO. Postnatal therapy was equivalent for both treated fetuses and controls. Primary outcome was infant survival to 180 days and secondary outcome was fetal pulmonary response. Results Maternal and fetal demographic characteristics and obstetric complications were similar in the three groups (P > 0.05). Infant survival rate was significantly higher in the early FETO group (62.5%) compared with the standard group (11.1%) and with controls (0%) (P < 0.01). Early FETO resulted in a significant improvement in fetal lung size and pulmonary vascularity when compared with standard FETO (P < 0.01). Conclusions Early FETO may improve infant survival by further increases of lung size and pulmonary vascularity in cases with extremely severe pulmonary hypoplasia in isolated CDH. This study supports formal testing of the hypothesis with a randomized controlled trial.