M. M. Silva

Prognostic factors associated with congenital cystic adenomatoid malformation of the lung

This study presents 18 cases of prenatally diagnosed congenital cystic adenomatoid malformation (CCAM) to identify potential factors that could predict prognosis. Comparisons of prenatal parameters were made between fetuses that survived and those that died perinatally. It was found that microcystic lesion, bilateral lung involvement and hydrops were each highly correlated with poor prognosis, while neither polyhydramnios nor mediastinal shift was significantly associated with had outcome. Fetal interventions were indicated only in two of the surviving cases: a thoracocentesis and a cysto‐amniotic shunt. A therapeutic amniocentesis was performed in one case of polyhydramnios. The diagnosis of CCAM was histologically confirmed in all cases by necropsy or by postnatal lobectomy. Copyright

Comparison between fetal endoscopic tracheal occlusion using a 1.0-mm fetoscope and prenatal expectant management in severe congenital diaphragmatic hernia.

Objectives: To evaluate if fetal endoscopic tracheal occlusion (FETO) for severe congenital diaphragmatic hernia (CDH) using a 1.0-mm fetoscope improves neonatal outcome. Method: Between January 2006 and December 2008, a controlled study was conducted at a single center in which FETO was proposed for fetuses with severe isolated CDH (lung-to-head ratio <1.0) and liver herniation to the thoracic cavity but no other detectable anomalies at diagnosis (<26 weeks). FETO was performed under maternal epidural and fetal intramuscular anesthesia, guided by ultrasonography and 1.0-mm fetoscope between 26 and 30 weeks. All cases submitted to FETO were delivered by ex utero intrapartum therapy procedure. Postnatal therapy was the same for both treated fetuses and controls. The primary outcome was neonatal survival (up to 28 days after birth). Results: A total of 35 women met the inclusion criteria, and in 17 of them, fetal intervention was intended. However, in 1 case, it was not possible to insert the balloon inside the fetal trachea because of placental bleeding. FETO was therefore successfully performed in 16 fetuses with severe CDH. Eighteen cases received no prenatal intervention and served as the control group. Mean gestational age at diagnosis was similar in both groups (p > 0.05). Delivery occurred at 35.6 (range: 28–38) weeks in the FETO group and at 37.5 (range: 31–40) weeks (p = 0.18) among controls. Nine of 17 (52.9%) infants in the FETO group and 1 of 18 (5.6%) in the control group survived (p < 0.01). Severe pulmonary arterial hypertension was present in 8/17 (47.1%) infants from the FETO group and in 16/18 (88.9%) controls (p = 0.01). Conclusion: The present study shows that FETO using a 1.0-mm fetoscope is feasible and may improve neonatal outcome in severe CDH.

The outcome of newborns with abdominal wall defects according to the method of abdominal closure: the experience of a single center

Recent reports suggest that the technique of abdominal closure in neonates with anterior abdominal wall defects (AWD) correlates with the outcome. The aim of this study is to analyze factors related to mortality and morbidity, according to the technique of abdominal closure of these neonates. Retrospective analysis of charts from 76 consecutive neonates with AWD treated in a single institution. They were divided according to the type of abdominal wall closure: group I: primary closure, group II: silo followed by primary closure and group III: silo followed by polypropylene mesh. Outcome was analyzed separately for neonates with gastroschisis and omphalocele. There were 13 deaths (17.1%). Mortality for neonates with isolated defects was 9.6%. Mortality rate was similar in all groups for either neonates with gastroschisis or omphalocele. Postoperative complications were not significantly different among groups except for a prolonged time of hospitalization in group III. Mortality rate is not correlated with the type of abdominal closure. Neonates with primary closure or with other methods of abdominal wall closure had similar rate of postoperative complications. Neonates with mesh closure of the abdomen have prolonged hospitalization. The use of a polypropylene mesh is a good alternative for neonates whose primary closure or closure after silo placement is not possible.

Living Related Donor Liver Transplantation in Children

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), 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). 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.

Bowel dilation as a predictor of adverse outcome in isolated fetal gastroschisis.

This study was designed to evaluate bowel diameter as a predictor of adverse outcome in isolated fetal gastroschisis.

Percutaneous laser ablation under ultrasound guidance for fetal hyperechogenic microcystic lung lesions with hydrops: a single center cohort and a literature review

To evaluate the perinatal outcomes in hydropic fetuses with congenital microcystic pulmonary lesions that underwent percutaneous, invasive, laser therapy.

Multiple clinical presentations of lymphoproliferative disorders in pediatric liver transplant recipients: a single-center experience.

Posttransplantation lymphoproliferative disorder (PTLD) is a serious complication following solid organ transplantation that has been linked to Epstein-Barr virus (EBV) infection. The aim of this article was to describe a single-center experience with the multiplicity of clinical presentations of PTLD. Among 350 liver transplantations performed in 303 children, 13 survivor children displayed a histological diagnosis of PTLD (13/242 survivors; 5.4%). The age at diagnosis ranged from 12 to 258 months (median, 47), and the time from transplantation ranged from 1 to 84 months (median, 13). Ten of these children (76.9%) were EBV-naïve prior to transplantation. Fever was present in all cases. The clinical signs at presentation were anemia (92.3%), diarrhea and vomiting (69.2%), recurrent upper airway infections (38.4%), Waldeyer ring lymphoid tissue hypertrophy (23.0%), abdominal mass lesions (30.7%), massive cervical and mediastinal adenopathy (15.3%), or gastrointestinal and respiratory symptoms (30.7%). One child developed fulminant hepatic allograft failure secondary to graft involvement by PTLD. Polymorphic PTLD was diagnosed in 6 patients; 7 had the diagnosis of lymphoma. Treatment consisted of stopping immunosuppression as well as starting intravenous gancyclovir and anti-CD20 monoclonal antibody therapy. The mortality rate was 53.8%. The clinical presentation of PTLD varied from fever of unknown origin to fulminant hepatic failure. The other symptoms that may be linked to the diagnosis of PTLD are pancytopenia, tonsil and adenoid hypertrophy, cervical or mediastinal lymph node enlargement, as well as abdominal masses. Despite numerous advances, the optimal treatment approach for PTLD is not completely known and the mortality rate is still high.

Quantitative Lung Index, Contralateral Lung Area, or Lung-to-Head Ratio to Predict the Neonatal Outcome in Isolated Congenital Diaphragmatic Hernia?

To estimate the accuracy of the quantitative lung index and contralateral lung area for prediction of the neonatal outcome in isolated congenital diaphragmatic hernia in comparison to other available prediction models.

Orthotopic Liver Transplantation in Biliary Atresia: A Single-Center Experience

INTRODUCTION Biliary atresia (BA) is the leading indication for orthotopic liver transplantation (OLT) among children. However, there are technical difficulties, including the limited dimensions of anatomical structures, hypoplasia and/or thrombosis of the portal vein and previous portoenterostomy procedures. OBJECTIVE The objective of this study was to present our experience of 239 children with BA who underwent OLT between September 1989 and June 2010 compared with OLT performed for other causes. METHODS We performed a retrospective analysis of patient charts and analysis of complications and survival. RESULTS BA was the most common indication for OLT (207/409; 50.6%). The median age of subjects was 26 months (range, 7-192). Their median weight was 11 kg (range, 5-63) with 110 children (53.1%) weighing ≤10 kg. We performed 126 transplantations from cadaveric donors (60.8%) and 81 from living-related donors (LRD) (39.2%). Retransplantation was required for 31 recipients (14.9%), primarily due to hepatic artery thrombosis (HAT; 64.5%). Other complications included the following: portal vein thrombosis (PVT; 13.0%), biliary stenosis and/or fistula (22.2%), bowel perforation (7.0%), and posttransplantation lymphoproliferative disorder (PTLD; 5.3%). Among the cases of OLT for other causes, the median age of recipients was 81 months (range, 11-17 years), which was higher than that for children with BA. Retransplantation was required in 3.5% of these patients (P < .05), mostly due to HAT. The incidences of PVT, bowel perforation, and PTLD were significantly lower (P < .05). There was no significant difference between biliary complications in the 2 groups. The overall survival rates at 1 versus 5 years were 79.7% versus 68.1% for BA, and 81.2% versus 75.7% for other causes, respectively. CONCLUSIONS Children who undergo OLT for BA are younger than those engrafted for other causes, displaying a higher risk of complications and retransplantations.

Rex Shunt for Acute Portal Vein Thrombosis After Pediatric Liver Transplantation in Children With Biliary Atresia

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.