Long-term results of percutaneous bilioenteric anastomotic stricture treatment in liver-transplanted children.

The purpose of this study was to evaluate the mid- and long-term results of percutaneous transhepatic cholangiography (PTC) and biliary drainage in children with isolated bilioenteric anastomotic stenosis (BAS) after pediatric liver transplantation. Sixty-four children underwent PTC from March 1993 to May 2008. Nineteen cholangiograms were normal; 10 showed intrahepatic biliary stenosis and BAS, and 35 showed isolated BAS. Cadaveric grafts were used in 19 and living donor grafts in 16 patients. Four patients received a whole liver, and 31 patients received a left lobe or left lateral segment. Roux-en-Y hepaticojejunostomy was performed in all patients. Indication for PTC was based on clinical, laboratory, and histopathologic findings. In patients with isolated BAS, dilation and biliary catheter placement, with changes every 2 months, were performed. Patients were separated into 4 groups according to number of treatment sessions required. The drainage catheter was removed if cholangiogram showed no significant residual stenosis and normal biliary emptying time after a minimum of 6 months. The relationship between risk factors (recipient's weight <10 kg, previous exposure to Cytomegalovirus, donor-recipient sex and weight relations, autoimmune disease as indication for transplantion, previous Kasai's surgery, use of reduced liver grafts, chronic or acute rejection occurrence) and treatment was evaluated. Before PTC, fever was observed in 46%, biliary dilation in 23%, increased bilirubin in 57%, and increased gamma-glutamyltransferase (GGT) in 100% of patients. In the group with BAS, 24 of 35 (69%) patients had histopathologic findings of cholestasis as did 9 of 19 (47%) patients in the group with normal PTC. Of the 35 patients, 23 (65.7%) needed 1 (group I), 7 needed 2 (group II), 4 needed 3 (group III), and 1 needed 4 treatment sessions (group IV). The best results were observed after 1 treatment session, and the mean duration of catheter placement and replacement was 10 months. The primary patency rate was 61.2%, and the recurrence rate was 34.3% (group I). Seven patients (7 of 35; 20%) had their stricture treated with a second treatment session (group II). The average drainage time in group II was 24 months. During a period >20 months, 4 patients (4 of 35; 11.4%) required 1 additional treatment session (group III), and 1 patient (1 of 35; 2.9%) had a catheter placed at the end of the study period (group IV). Drainage time in group I was significantly shorter than those in groups II, III, and IV (p < 0.05). There was no statistically significant relation between therapeutic response and the selected risk factors (p > 0.05). The majority of complications, such as catheter displacement and leakage, were classified as minor; however, 2 patients (5.7%) with hemobilia were noted. Complications increased according to the need for reintervention. In conclusion, balloon dilation and percutaneous drainage placement is safe and effective, and it has long-term patency for children with BAS after liver transplantation. Because of prolonged treatment time, reintervention may be necessary, thereby increasing the complication rate. Balloon dilation and percutaneous drainage placement should be considered as the first treatment option because of its minimally invasive nature.

To shunt or not to shunt a pulmonary adenomatoid cystic malformation after 33 weeks of gestation: a case report.

CONTEXT: Macrocystic adenomatoid malformation of the lung can cause severe mediastinal shift, hydrops and polyhydramnios, thereby increasing the risk of perinatal deaths. After 33 weeks of gestation, repeated puncturing of the cyst is recommended. We present a case in which a cyst-amniotic shunt was placed instead of performing this procedure. CASE REPORT: A cyst-amniotic shunt was placed at 33 weeks of gestation because of a large macrocystic adenomatoid malformation of the lung associated with severe mediastinal shift and polyhydramnios. Although it was confirmed that the catheter was in the correct place, the cyst increased in size again two weeks later, associated with repetition of polyhydramnios. It was postulated that the catheter was blocked, and we chose to place another catheter instead of performing repeated punctures. The cystic volume, polyhydramnios and mediastinal shift regressed progressively. At 38.5 weeks, a 3,310/g male infant was delivered without presenting any respiratory distress. The infant underwent thoracotomy on the 15th day of life. Thus, in the present study, we discuss the possibility of placing a cyst-amniotic shunt instead of performing repeated cystic punctures, even at a gestational age close to full term.

To shunt or not to shunt a pulmonary adenomatoid cystic malformation after 33 weeks of gestation: a case report / Derivar ou não uma malformação adenomatóide cística do pulmão após 33 semanas de gestação: relato de caso

CONTEXT: Macrocystic adenomatoid malformation of the lung can cause severe mediastinal shift, hydrops and polyhydramnios, thereby increasing the risk of perinatal deaths. After 33 weeks of gestation, repeated puncturing of the cyst is recommended. We present a case in which a cyst-amniotic shunt was placed instead of performing this procedure. CASE REPORT: A cyst-amniotic shunt was placed at 33 weeks of gestation because of a large macrocystic adenomatoid malformation of the lung associated with severe mediastinal shift and polyhydramnios. Although it was confirmed that the catheter was in the correct place, the cyst increased in size again two weeks later, associated with repetition of polyhydramnios. It was postulated that the catheter was blocked, and we chose to place another catheter instead of performing repeated punctures. The cystic volume, polyhydramnios and mediastinal shift regressed progressively. At 38.5 weeks, a 3,310/g male infant was delivered without presenting any respiratory distress. The infant underwent thoracotomy on the 15th day of life. Thus, in the present study, we discuss the possibility of placing a cyst-amniotic shunt instead of performing repeated cystic punctures, even at a gestational age close to full term.

CONTEXTO: A malformação adenomatóide do pulmão tipo macrocística pode causar compressão mediastinal grave, hidropisia e polihidrâmnio aumentando a chance de óbito perinatal. Após a 33ª semana de gestação, recomenda-se realizar punções repetidas do cisto. Apresentamos um caso em que um dreno cístico-amniótico foi colocado e as punções foram evitadas. RELATO DE CASO: Um dreno cístico-amniótico foi colocado na 33ª semana de gestação devido a grande malfomação adenomatóide cística do pulmão associada a desvio de mediastino grave e polihidrâmnio. Apesar de o cateter ter sido identificado no local correto, o cisto voltou a crescer duas semanas após, repetindo o polihidrâmnio. Postulou-se que o cateter estava obstruído e optamos por colocar um novo cateter ao invés de realizar punções repetidas desse cisto. Foi observada regressão progressiva do volume do cisto, do polihidrâmnio e do desvio de mediastino. Na 38ª semana e meia, um menino de 3.310 g nasceu sem apresentar dificuldade respiratória, sendo submetido a toracotomia no 15º dia de vida. Portanto, no presente estudo, discute-se sobre a possibilidade de colocação do dreno cístico-amniótico no lugar de realizar punções repetidas do cisto mesmo em idades gestacionais próximas do termo.

Intimal dissection of the hepatic artery after thrombectomy as a cause of graft loss in pediatric living-related liver transplantation.

HAT is the main cause of graft loss in pediatric living-related LTx. Revascularization of the graft by thrombectomy and re-anastomosis has been reported to be effective for graft salvage in cases of HAT and should be attempted when potential donors are not available for emergency re-transplantation. Immediate complications secondary to revascularization attempts in cases of HAT are not described. Late complications are mainly related to biliary tree ischemia. We report a case of child who experienced intimal hepatic artery dissection, which extended into intra-hepatic branches of the artery after a thrombectomy with a Fogarty balloon catheter in an attempt to restore arterial flow after HAT. This complication led to acute deterioration of the graft and the need for emergency re-transplantation.

Which is better for esophageal substitution in children, esophagocoloplasty or gastric transposition? A 27-year experience of a single center.

BACKGROUND/PURPOSE: Esophagocoloplasty and gastric transposition are 2 major methods of esophageal substitution in children. The purpose of this study is to review the authors' experience with these 2 techniques and compare the complications of these operations to determine whether 1 method emerges superior to the other. METHODS: A total of 149 children underwent surgery: 115 children underwent esophagocoloplasty, and 34 children underwent gastric transposition. Most patients (113-75.8%) had long-gap esophageal atresia. The operative technique of esophagocoloplasty consisted of the interposition of the transverse colon maintained by a double vascular pedicle based on the left colic vessels and the marginal paracolic arcade. Gastric transposition was performed according to classical technique. The transposition of colon and stomach was performed using blunt mediastinal dissection in all patients without thoracotomy. Complications and mortality of the 2 groups of patients were compared. These complications were classified as minor (cervical anastomosis leak, abdominal evisceration, diarrhea, strictures, and reflux to the interposed viscera) and major (necrosis of transposed viscera, dehiscence of an intra-abdominal or intrapleural suture, torsion of transposed viscera, delayed gastric emptying requiring reoperation, and cologastric anastomosis stricture). RESULTS: There were 2 graft necrosis, 1 (0.8%) in the esophagocoloplasty group and another (2.9%) in the gastric transposition group. Patients who underwent esophagocoloplasty experienced a greater incidence of minor complications (P = .001) and less major complications in comparison with the gastric transposition group (P = .001). All minor complications were treatable and had no consequences. No difference was noted between the 2 groups with regard to the mortality rate (0.9% and 5.9%, respectively, P > .05). CONCLUSIONS: Esophagocoloplasty and gastric transposition are satisfactory means of esophageal substitution in children. Considering the incidence of major postoperative complications, esophagocoloplasty must be the first choice for esophageal replacement in children.

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.

Endovascular treatment of acute portal vein thrombosis after liver transplantation in a child.

Although operative techniques in hepatic transplantation have reduced the time and mortality on waiting lists, the rate of vascular complications associated with these techniques has increased. Stenosis or thrombosis of the portal vein is an infrequent complication, and if present, surgical treatment is considered the traditional management. This article describes a case of acute portal vein thrombosis after liver transplantation from a living donor to a child managed by percutaneous techniques.

The second and third right posterior intercostal veins: an alternate route for central venous access with an implantable port in children.

BACKGROUND/PURPOSE: Some children dependent on total parenteral nutrition for long periods have no more axillary, internal jugular, external jugular, saphenous, and femoral veins available for cannulation. In such patients, the central venous system can still be accessed via the azygos vein by placing an implantable port catheter through one of the right posterior intercostal veins. This is the first known description of such procedure. METHODS: We report the use of the second and third right intercostal veins for placement of the catheter by right intrapleural thoracotomy in 2 pediatric patients with short gut syndrome. RESULTS: Recovery from the thoracotomy was uncomplicated, and the patients could receive complete intravenous nutritive mixtures immediately after the insertion of the catheter. Both patients remain dependent on total parenteral nutrition and are awaiting an intestinal transplantation. CONCLUSION: The knowledge of alternate routes to obtain central venous access for prolonged parenteral nutrition is critically important, and the azygos system can be used when more accessible veins are unavailable.

Endovascular treatment of stenosis between hepatic vein and inferior vena cava following liver transplantation in a child: a case report.

The liver transplantation technique advances have allowed the endovascular treatment of stenosis between hepatic vein and inferior vena cava, and this has become an established and widely acceptable method for the treatment of patients with end-stage liver disease. However, in spite of the advances in the surgical technique of liver transplantation there is relatively still a high incidence of postoperative complications, especially those related to vascular complications. One technical variant of orthotopic liver transplantation is the piggyback technique with conservation of the recipient vena cava, which is anastomosed to the graft hepatic veins. As a consequence of the increased number of liver transplants in children, there is a higher demand for endovascular treatment of vascular stenosis, such as those at the level of the hepatic veins. This leads to more consistent experience of endovascular treatment of the surgical vascular complications following liver transplantation. This article describes the case of a child submitted to liver transplantation with reduced graft (left lateral segment) who presented stenosis of the anastomosis between the hepatic vein and IVC 6 months later which was successfully treated by PTA.

Alterations of enteric nerve plexus in experimental gastroschisis: is there a delay in the maturation?

BACKGROUND/PURPOSE: After surgical correction of gastroschisis, intestinal transitory hypoperistalsis usually occurs. Long-term parenteral nutrition often is necessary leading to a higher morbidity associated with this malformation. The etiology of this transitory intestinal hypomotility is unknown. It may be caused by a reversible inflammatory process in the intestinal wall or other causes, including an alteration of the maturation of intestinal neural plexus, because the disturbance disappears spontaneously after a variable period. The aim of this work was to study the neuronal cells of the myenteric plexus of the fetal intestine in experimental gastroschisis. The main hypothesis was that the transitory intestinal dismotility seen in gastroschisis could be secondary to alteration in the maturation of the enteric nervous plexus. METHODS: Twenty-seven time-mated rabbits, on gestational day 25, were submitted to a midline laparotomy; the gravid bicornuate uterus was exposed and opened, and the more distal fetuses relative to the vaginal opening had the abdominal wall opened by a small incision to produce gastroschisis (n = 29). The fetuses not submitted to gastroschisis were used as controls (n = 12). The amniotic fluid was carefully aspirated from the opened uterus and saved for later repositions. On gestational day 30, the does were again submitted to general anesthesia, and the fetuses were delivered by cesarean section. The fetal intestine was removed, the adjacent mesentery excised, and intestinal specimens were harvested for histologic studies. The specimens were stained for acetyl-cholinesterase activity (AChE) to assess the maturity of the nervous enteric cells and for lactate dehydrogenase (LDH) that identify specifically immature nervous cells. The histologic sections stained by LDH were submitted to histomorphometric analysis of the nervous cells through an image system analysis (Kontron 300). The results were submitted to statistical analyses (P <.05). RESULTS: Macroscopic alterations of the fetal gastroschisis intestine are similar to the human findings: shortening of the intestine, intestinal wall thickening, and a hypertrophied muscular layer. In the gastroschisis group, histologic AChE activity was decreased in comparison with control intestines. The histomorphometric assessment in slices stained with LDH, which identify immature nervous cells, showed that the neuronal intestinal cells of the gastroschisis group were significantly smaller and more numerous relative to the control group. CONCLUSIONS: There were significant differences in the nervous plexus of the intestine of fetuses with gastroschisis relative to the controls. The observed morphologic changes may be caused by alteration in the maturation of the intestinal neuronal in gastroschisis. This alteration may explain the transitory intestinal hypomotility observed in infants after surgical correction of gastroschisis.

An exclusively intraabdominal distal esophageal segment prevents primary delayed anastomosis in children with pure esophageal atresia.

PURPOSE: The aim of this study was to evaluate the outcome of children with pure esophageal atresia (EA) managed by the strategy of delayed primary anastomosis and to find parameters to identify which of these children will have satisfactory esophageal growth to permit a successful primary esophageal anastomosis. METHODS: Sixteen children with pure EA were treated by delayed anastomosis strategy from June 1992 to May 2001 at the Instituto da Criança-University of São Paulo Medical School. The treatment consisted of creating an early feeding gastrostomy, continuous upper pouch suction, and periodic radiologic/endoscopic assessments of the gap between the superior and inferior esophageal pouches. The first assessment was done 4 weeks after the initial gastrostomy. Subsequent assessments were done, when necessary, 4 to 10 weeks after the first one. Children with the gap between the pouches corresponding to 2 vertebral bodies or less were considered for primary repair. RESULTS: The waiting period ranged from 8 to 14 weeks. Five children were considered for primary repair (31.3%). All of them had an end-to-end esophageal anastomosis performed without tension. Eleven children (68.7%) did not show significant esophageal growth after the waiting period and were not considered for primary esophageal anastomosis. Eight of them (50%) showed an entirely intraabdominal distal pouch at the first assessment. CONCLUSIONS: (1) Infants with an exclusively intraabdominal distal pouch at the first evaluation will not reach sufficient elongation of the pouches and should be considered early as a candidate for esophageal replacement. (2) Combined radiologic/endoscopic assessment and the measurement of the gap in terms of vertebral bodies is a practical and reliable method to predict the feasibility of primary anastomosis. (3) Using these criteria, primary anastomosis could be accomplished in less than a third of cases.

Transjugular intrahepatic portosystemic shunt in a child with Budd-Chiari syndrome: technical modification and extended followup.

An 8-year-old girl with Budd-Chiari syndrome presented with upper gastrointestinal hemorrhage and ascites. TIPS to decompress the portal hypertension was performed by puncturing the portal vein directly from the inferior vena cava. The shunt remains patent after 3 years without requiring secondary intervention.

Ressonância magnética: seu valor no tratamento da incontinência fecal / Magnetic resonance imaging: it`s role in the fecal incontinence treatment

Dezessete pacientes portadores de anomalias pelve-perineais e incontinência fecal foram estudados por meio da ressonância magnética (RM), para definir possível correçäo cirúrgica da incontinência fecal. 14 eram portadores de malformaçäo ano-retal, sendo 13 pós-abaixamento de cólon por várias técnicas e um após ano-retoplastia sagital posterior (ARPSP), - 2 eram portadores de trauma perineal e 1, portador de síndrome de Fournier. 2 eram controles normais. Maior ênfase foi dada à musculatura pelve-perineal (músculo elevador, complexo muscular perineal e fibras parassagitais), e sua relaçäo com o canal reto-anal, anatomia sacral e do filum terminal. Em 5 pacientes foi indicado tratamento cirúrgico (ARPSP), por apresentarem : 1) musculatura pelve-perineal bem desenvolvida; 2) canal ano-retal fora de seu posicionamento adequado; 3) anatomia sacral normal. 12 pacientes continuaram com tratamento clínico, pois näo se enquadravam nesses critérios, e aqueles que foram operados mostram acentuada melhora da continência fecal. Concluiu-se que a RM pode definir as estruturas anatômicas que permitem indicar o tratamento cirúrgico, com melhora acentuada da continência fecal

Diluíçåo de concentrado de hemácias em soluçåo fisiológica a 70ºC nåo causa hemólise / Dilution of red blood cell concenntrate with normal sline at 70ºC does not result in hemolysis

Heating of cold stored blood before transfusion is required to avoid hypothermia during massive transfusion. The objective of this study was to verify the efficacy and safety of a new technique of heating red blood cell concentrates (RBCC) through dilution in normal saline (NS) at 70ºC. In glass tubes, 5 ml of RBCC at ambient temperature or refrigerated were diluted in 5 ml NS at three different temperatures: ambient, 70ºC or 95ºC. The resulting temperature was recorded. Free hemoglobin (Hb) and plasma potassium (K+) were determined and compared among the different dilutions. The dilution of refrigerated RBCC in NS at ambient temperature was considered as control, with a resulting temperature of 18.5 +- 0.4ºC, free Hb of 15.2 +- 3.0 mg/dL, aqnd K+ of 5.0 +- 0,5 mEq/L. Normal Saline at 70ºC resulted in a temperature of 29.4+- 0.7ºC, without any increase in free Hb and K+. However, Normal Saline at 95ºC induced hemolysis with an increase in free Hb to 2142.2 +- 242.1 mg/dL (p<0,05) and in K+ to 6.6 +- 2.3 mEq/L. Therefore, NS at 70ºC did not cause hemolysis. This technique is simple, fast, inexpensive, and it can be used in catastrophes