Antenatal prognostic factor of fetal echogenic bowel.

OBJECTIVE: The aim of this study was to identify antenatal prognostic factors of neonatal outcomes in cases of fetal echogenic bowel (FEB). STUDY DESIGN: A retrospective study in three tertiary referral centers including fetal echogenic bowel over a 10-year period (from January 2003 to December 2013). The echogenicity of the fetal bowel was graded from 1 to 3, according to Slotnick's definition. Associated echographic findings such as bowel dilations, gallbladder abnormalities, calcifications, extra-abdominal abnormalities, intrauterine growth restriction (IUGR) and a decrease in amniotic fluid volume, if present were also recorded. This was followed by the FEB's sonographic evolution. The sonographic evolution was considered favorable if it was stable or decreasing and unfavorable if the echogenicity of the bowel increased or if additional sonographic findings appeared. Neonates had a pediatric examination in the delivery room and upon discharge from the maternity hospital. An outcome was considered good in the case of on-term delivery of a newborn with normal clinical examination and meconium elimination. RESULTS: Complete pregnancy outcome data were available for 409 pregnancies. 338 newborns had uneventful outcomes (82.6%). Antenatal exploration diagnosed 4 cases of aneuploidy (1 case of trisomy 13, 1 case of trisomy 18 and 2 cases of triploidies), 16 cases of congenital infections, 9 cases of cystic fibrosis and 11 cases of bowel abnormalities. After a multivariate analysis, we discovered the sonographic grade of the echogenic bowel was not a prognostic factor of neonatal outcome. The isolated fetal echogenic bowel had a 6.6-fold increase chance of uneventful outcomes (adjusted odd ratio (aOR) 6.6, 95% CI 3-14.4). Notably, favorable sonographic evolution (aOR 8.1, 95% CI 4.1-16) and late gestational age at the time of the diagnosis (aOR 1.17, 95% CI 1.07-1.27) are independent, good prognostic factors of good neonatal outcomes. None of the 180 fetuses with isolated fetal echogenic bowel and favorable sonographic evolution had adverse outcomes. Among these, 4 cases (0.98%) of aneuploïdy, 17 cases (4.2%) of congenital infections and 9 cases (2.2%) of cystic fibroses were also diagnosed. No cases of Down syndrome (DS) were reported. CONCLUSION: Our study shows that the grade should not be considered a prognostic factor of neonatal outcomes. Our data suggests the need to reevaluate the concept of systematic amniocentesis. Sonographic evolution of fetal bowel is an independent, strong prognostic factor for good neonatal outcomes. It also better defines the FEB prognostic.

Laparoscopic Resection of Pancreatic Tumors in Children: Results of a Multicentric Survey.

AIM: This study aimed to report the results of a multicentric survey about laparoscopic treatment of pancreatic tumors in children. MATERIALS AND METHODS: The data of patients operated using minimally invasive surgery (MIS) for a pancreatic tumor in 5 International centers of Pediatric Surgery in the last 5 years were retrospectively reviewed. We recorded data relating to the clinical presentation, diagnostic evaluation, surgical technique, and outcome. RESULTS: Fifteen patients (average age 2.2 years) were identified. The most common symptoms at presentation were related to the hypoglycemic hyperinsulinism, followed by abdominal pain and vomiting. Tumor types were insulinoma (n = 4), congenital hyperinsulinism of infancy (CHI) diffuse type (n = 3), CHI focal type (n = 3), solid pseudopapillary tumor (n = 2), and cystic malformation (n = 3). The diagnostic assessment was completed using ultrasound associated with computed tomography (CT) scan in all centers; 18FDOPA positron emission tomography in combination with CT was adopted in 2 centers. The MIS procedures performed were as follows: tumor enucleation (n = 4), distal pancreatectomy (n = 8), subtotal pancreatectomy (n = 2), and pancreatico-jejunostomy (n = 1). Average operative time was 110 minutes. As for postoperative complications, we recorded 1 persistent hypoglycemia, requiring redo-surgery (IIIb Clavien-Dindo) and 1 thrombosis of splenic vein, not requiring any treatment (I Clavien-Dindo). CONCLUSIONS: Laparoscopic resection can be considered a safe and effective treatment with minimal morbidity and excellent outcomes for most pediatric pancreatic tumors. Suspension of the stomach with a transparietal stitch and use of new hemostatic devices as Starion TLS3 or Ligasure are key factors for the success of the procedure. A long-term follow-up is mandatory in these patients to evaluate postoperative complications and long-term outcome.

Recurrence of Solid Pseudopapillary Neoplasms of the Pancreas: Results of a Nationwide Study of Risk Factors and Treatment Modalities.

BACKGROUND: Solid pseudopapillary neoplasms of the pancreas (SPPN) can relapse very late, but little is known about risk factors for recurrence and optimal treatment. We aimed to identify risk factors for recurrence and to analyze treatment modalities in all French pediatric cases of SPPN over the past 20 years. MATERIAL AND METHODS: Data were collected from pediatric oncologists and surgeons, and also from adult pancreatic surgeons in order to identify late recurrences. RESULTS: Fifty-one patients (41 girls) were identified. Median age at diagnosis was 13.1 years [8.7-17.9]. Abdominal pain was the commonest presenting symptom (32/49, 65%). The tumor was located in the pancreatic head in 24 patients (47%). Preoperative biopsy or cytology was performed in 14 cases (28%). All patients were operated with a median of 23 days [0-163] after diagnosis. The rate of postoperative morbidity was 29%. With a median follow-up of 65 months [0.3-221], the overall and event-free survival was 100% and 71%, respectively. Seven patients (13.7%) relapsed with a median of 43 months [33-94] after initial surgery. Six were treated surgically, either alone (n = 3) or with perioperative chemotherapy (n = 2) or hyperthermic intraperitoneal chemotherapy (n = 1). One patient in whom further treatment was not feasible was still alive at last news. Risk factors for recurrence were positive surgical margins (P = 0.03) and age less than 13.5 years at diagnosis (P = 0.03). CONCLUSIONS: SPPN recurrence in this pediatric series was a rare and late event that did not undermine overall survival. Complete surgical removal of recurrent tumors appears to be the best option.

Laparoscopic cystojejunostomy for type I cystic biliary atresia in children.

PURPOSE: The use of laparoscopy in the treatment of biliary atresia (BA) is still debated. We report our strategy using laparoscopy in type I cystic BA. MATERIALS AND METHODS: We reviewed the records of patients treated for BA from 2002-2013. When the diagnosis was suspected, an ultrasound was performed. If it showed a cyst > 5 mm in the hilum with no patent gallbladder, we performed an initial explorative laparoscopy. In the case of a patent biliary tree above the cyst, a laparoscopic cystojejunostomy was performed. In cases of absent communication (type III), conversion and portoenterostomy were performed. Pre and postoperative data and overall survival rate with the native liver were reviewed. RESULTS: Forty-four children were treated for BA. Six presented with a cystic form diagnosed by US. Three children had type I BA; three had type III BA. No postoperative complications were noted. Median follow-up was 62.2 months (22.7-93.5). One patient died of a cardiac malformation. The five remaining patients are alive with their native liver. Of the 38 treated for noncystic BA, 16 were transplanted. CONCLUSION: We confirmed the prognosis of cystic BA, which is less severe than noncystic BA. Our strategy using laparoscopy allowed for the confirmation and qualification of the type of BA. In type I, complete treatment by laparoscopy has been performed safely.

Congenital diaphragmatic hernia: does gestational age at diagnosis matter when evaluating morbidity and mortality?

OBJECTIVE: The objective of the investigation was to study the relationship between gestational age at diagnosis and mortality and morbidity in fetuses with an isolated congenital diaphragmatic hernia. STUDY DESIGN: Between January 2008 and November 2013, 377 live births with isolated congenital diaphragmatic hernia diagnosed antenatally at a known gestational age were recorded in the database of the French National Center for Rare Diseases. The primary outcome studied was mortality estimated at 28 days and at 6 months. The secondary outcome was morbidity evaluated by pulmonary arterial hypertension at 48 hours, oxygen therapy dependence at 28 days, oral disorders, enteral feeding, and prosthetic patch repair. Analyses were adjusted for the main factors of congenital diaphragmatic hernia severity (side of the hernia, thoracic herniation of the liver, gestational age at birth, lung-to-head ratio, and prenatal treatment by tracheal occlusion. RESULTS: Mortality rates at 28 days decreased significantly (P < .001) when gestational age at diagnosis increased: 61.1%, 39.2%, and 10.4% for a diagnosis in the first, second, and third trimester, respectively. Adjusted odds ratios were 3.12 [95% confidence interval, 1.86-5.25] and 0.35 [95% confidence interval, 0.18-0.66] for a diagnosis in the first and third trimesters, respectively, compared with a diagnosis in the second trimester. Similarly, morbidity decreased significantly when gestational age at diagnosis increased, and the trend remained significant after adjustment for the main factors of congenital diaphragmatic hernia severity (P < .001). CONCLUSION: Gestational age at diagnosis is an independent predictor of postnatal prognosis for children presenting an isolated congenital diaphragmatic hernia and should be taken into account when estimating postnatal morbidity and mortality.

Congenital pulmonary airway malformation and sequestration: Two standpoints for a single condition.

UNLABELLED: In adults, congenital pulmonary malformations are candidates for surgery due to symptoms. A pre-natal diagnosis is simple and effective, and allows an early thoracoscopic surgical treatment. A retrospective study was performed to assess management in two different populations of adults and children to define the best strategy. SUBJECTS AND METHODS: Pulmonary malformations followed at the University Hospital from 2000 to 2012 were reviewed. Clinical history, malformation site, duration of hospitalisation, complications and pathology examinations were collected. RESULTS: A total of 52 cases (33 children, 19 adults) were identified. In children, 28 asymptomatic cases were diagnosed pre-natally and 5 during the neonatal period due to infections. Surgery was performed on the children between the ages of 2 and 6 months. Nineteen adults underwent surgery, 16 because of symptoms and 3 adults for anomalies mimicking tumours. The mean age within the adult group was 42.5 years. In children, there was one thoracotomy and 32 thoracoscopies, with 7 conversions for difficult exposure, dissection of vascular pedicles, bleeding or bronchial injury. In the adults, there were 15 thoracotomies and 4 thoracoscopies, with one conversion. Post-operative complications in the adults were twice as frequent than in children. The mean time of the children's hospitalisation was 7.75 days versus 7.16 days for the adults. Pathological examinations showed in the children: 7 sequestrations, 18 congenital cystic pulmonary malformations (CPAM), 8 CPAM associated sequestrations; in adults: 16 sequestrations, 3 intra-pulmonary cysts. CONCLUSION: Early thoracoscopic surgery allows pulmonary parenchyma conservation with pulmonary development, reduces respiratory and infectious complications, eliminates a false positive cancer diagnosis later in life and decreases risks of thoracic parietal deformation.

Results from the French National Esophageal Atresia register: one-year outcome.

BACKGROUND: The aim of the present national prospective population-based study was to assess the early morbidity of esophageal atresia (EA). METHODS: All 38 multidisciplinary French centers that care for patients with EA returned a specific questionnaire about the 1-year outcome for each patient. This information was centralized, checked, and entered into a database. RESULTS: From the total population of 307 EA patients born in 2008 and 2009, data about the 1-year outcome were obtained from 301 (98%) patients, of whom 4% were lost to follow-up and 5% died. Medical complications occurred in 34% of the patients: anastomotic leaks (8%), recurrent tracheoesophageal fistula (4%), and anastomotic stenosis (22%); all of the latter group needed dilation (median, 2 dilations/patient). A new hospitalization was required for 59% of patients (2.5 hospitalizations/patient) for digestive (52%) or respiratory (48%) reasons. Twelve percent of patients required antireflux surgery at a median age of 164 days (range, 33-398 days), and 1% underwent an aortopexy for severe tracheomalacia. The weight/age Z-score was -0.8 (range, -5.5 to 3.7 months) at 12 months. Fifteen percent of patients were undernourished at 12 months of age, whereas 37% presented with respiratory symptoms and 15% had dysphagia at the last follow-up. Significant independent factors associated with medical complications were anastomotic esophageal tension (p = .0009) and presence of a gastrostomy (p = .0002); exclusive oral feeding at discharge was associated with a decreased risk of complications (p = .007). CONCLUSIONS: Digestive and respiratory morbidities remain frequent during the first year of life and are associated with difficult anastomosis and lack of full oral feeding.

Early thoracoscopic resection of an atypical upper extralobar sequestration.

We report a very rare case of complex upper extralobar pulmonary sequestration, detected during pregnancy and resected early because the diagnosis was uncertain (possibility of a teratoma). At 2 months, the upper left pulmonary lesion was completely removed by thoracoscopy. Pathology concluded an extralobar sequestration associated with a duplication cyst of mixed bronchogenic and esophageal type. The postoperative course was uneventful. The early thoracoscopic approach in this type of antenatally diagnosed lesion was a good option.

Putative criteria for predicting spontaneous regression of prenatally diagnosed thoracoabdominal cystic lesions.

PURPOSE: Cystic lesions are common findings during prenatal ultrasonography but their prenatal and postnatal prognosis is difficult to establish because of some regress spontaneously. The purpose of this study was to identify putative criteria to predict regression of partially or completely cystic lesions detected by prenatal ultrasound. METHODS: Prenatal ultrasound features of thoracic or abdominal cystic lesions were retrospectively analyzed. Ovarian and urological lesions were not included in this study. RESULTS: A total of 57 cystic lesions were studied. Of the 57 lesion, 36 lesions including 10 abdominal (43.5%) and 26 thoracic (76.5%) lesions required surgical resection. Of the 57 lesions, 10 persistent lesions after birth were only monitored. Eleven lesions including eight abdominal (34.7%) and three thoracic (8.8%) lesions regressed prenatally (p = 0.02). Regressing abdominal lesions consistently presented as solitary lesions with a homogenous aspect. Only one abdominal lesion showed a multilobulated aspect. Two regressing thoracic lesions were purely cystic and one lesion presented a heterogeneous aspect. CONCLUSION: Regression of cystic lesions detected by prenatal ultrasound scan was more likely for lesions in abdominal (mainly adrenal or splenic lesions) than thoracic locations. The likelihood of regression was highest for purely cystic abdominal lesions.

Extrahepatic vitelline vein aneurysm: prenatal diagnosis and follow up.

Umbilical vein varix is a well-described prenatal anomaly in which the prognosis remains unclear. We describe a very rare venous malformation that mimicked an umbilical vein varix consisting of a persistent vitelline vein. From 2003 to 2010, three patients were referred starting at 20 weeks gestation to our prenatal centers for an umbilical vein varix diagnosis. Fetal follow up was unremarkable, with the exception of the dilated vein size (mean: 35 mm at 33 weeks gestation). After birth, the three children presented with thrombosis from the aneurysmal sac to the portal trunk. All the children underwent surgical thrombectomy and resection of the aneurysmal sac after birth. Operative findings showed no umbilical vein but an abnormal dilated and thrombosed vein coming from the umbilicus to the portal vein following the right vitelline vein trajectory. One child was treated with systemic heparin. Median follow up is 5.6 years. Currently, one patient has a normal portal flow. The other two have persistent portal vein thrombosis with portal cavernoma and portal hypertension. This malformation is rare and should be considered in cases of early diagnosed umbilical vein varix whose diameter is greater than 20mm. We advocate an early surgical thrombectomy with heparinization to prevent portal vein thrombosis.

Congenital diaphragmatic hernia: an evaluation of risk factors for failure of thoracoscopic primary repair in neonates.

PURPOSE: Publications aiming to prove the feasibility and safety of thoracoscopic CDH-repair in neonates grow in numbers. Some teams use selection criteria, but none have proven statistical evidence. The aim of this study is to detect risk factors for failure of thoracoscopic primary closure of CDH in neonates. METHODS: In 8 centers performing minimal access surgery (MAS), complete prenatal, postnatal, and operative data were evaluated for a retrospective study concerning patients with thoracoscopic congenital diaphragmatic hernia (CDH) repair. Most of the selection criteria and risk factors mentioned in the literature were analyzed. Two groups were defined: Group A - neonates who tolerated thoracoscopic primary repair, and Group B - neonates who required conversion or presented with major complications after thoracoscopic repair. Univariate and multivariate logistic regressions were used to compare these two groups. RESULTS: From 2006 to 2010, thoracoscopy was performed in 40 neonates: Group A consisting of 28 neonates, and Group B 9 patients. Three patients were excluded because of insufficient data or major associated malformations. Significant statistical differences were found in Group B for postnatal PaCO2 >60 mmHg, need of iNO during postnatal stabilization, intrathoracic position of the stomach, pulmonary hypertension signs on the postnatal cardiac ultrasound, and preoperative OI >3.0. On multivariate analysis, only an OI >3.0 was significantly associated with conversion or major post-operative complication of thoracoscopic primary repair. CONCLUSION: CDH can be safely repaired in the neonatal period by thoracoscopy. The limiting factor for thoracoscopic CDH repair is PPHN. The best preoperative indicator for PPHN is OI. Prospective studies are nonetheless necessary to prove the effectiveness of using these risk factors as selection criteria to help design surgical management protocols for neonates presenting CDH.

Management of choledochal cyst: Evolution with antenatal diagnosis and laparoscopic approach.

BACKGROUND/AIM: Laparoscopic excision of a choledochal cyst (CC) with hepaticojejunostomy gained a wide acceptance in the treatment of children even in neonatal period. Although, the use of prenatal diagnostic techniques causes a significant increase in antenatal diagnosis of CC, the time of surgical intervention during infancy is still controversial. A retrospective study was performed to evaluate the results of laparoscopic management of CC with special emphasis on antenatal diagnosis and treatment, and to compare the results with open procedure. MATERIALS AND METHODS: The patients who were diagnosed with choledochal cyst and underwent either open or laparoscopic hepaticojejunostomy in two centres, hopital d'enfant de La Timone from Marseille and hopital Robert Debre from Paris between November 2000 and December 2009 were included in the study. The data obtained from medical reports were evaluated for sex, time of antenatal diagnosis, age at time of operation, operative time, time of postoperation. RESULTS: A total of 19 hepaticojejunostomy were performed, including 10 open procedures (group A), and 9 laparoscopic procedures (group B, 4 were diagnosed prenatally, without conversion to open procedure). There were 3 boys and 16 girls, ranging in age from 2 weeks to 16 years. Patients in the group A were older than patients in the group B. The mean hospital stay and time to return of bowel fuction was longer in the group B. there were 60% of pre-operative complications in group A versus 22% in group B. There was one postoperative complications in group B (biliary leakage nedeed redo surgery). No significant differences were found between different parameters except for operative time (288.56 min in the group B versus 206 min in the group A. (p = 0.041)). CONCLUSIONS: Our initial experience indicates that the laparoscopic approach in infancy is technically feasible, safe, and effective, with a low morbidity and a comparable outcome to the open approach. Therefore, we propose a laparoscopic approach for antenatally diagnosed CC as early as possible, before the onset of complications.

Evolution of congenital malformations of the umbilical-portal-hepatic venous system.

OBJECTIVE: The objective of this study is to describe the evolution of 8 cases of congenital malformations of the umbilical-portal-hepatic venous system diagnosed before the first month of life. MATERIALS AND METHODS: All cases of congenital malformation of the portal and hepatic venous system diagnosed prenatally or during the first month of life in our institution were systematically reviewed since November 2000. Clinical features, imaging, and anatomical findings were reviewed, focusing primarily on clinical and radiologic evolution. RESULTS: Eight cases of congenital malformation of the umbilical-portal-hepatic venous system were studied. Fifty percent of these malformations were diagnosed prenatally. We report 4 portosystemic shunts. Three involuted spontaneously, and the fourth one required surgical treatment. We report a variation of the usual anatomy of portal and hepatic veins that remained asymptomatic, an aneurysmal dilatation of a vitelline vein causing portal vein thrombosis that needed prompt surgical treatment with good result, a complex portal and hepatic venous malformation treated operatively, and a persistent right umbilical vein that remained asymptomatic. CONCLUSION: Prenatal diagnosis of malformations of the umbilical-portal-hepatic venous network is uncommon. Little is known about the postnatal prognosis. Clinical, biologic, and radiologic follow-up by ultrasonography is essential to distinguish pathologic situations from normal anatomical variants.

Bronchogenic cyst: best time for surgery?

BACKGROUND: Bronchogenic cyst (BC) is a benign congenital mediastinal tumor whose natural course remains unclear. In adults, most BCs are removed by thoracotomy after complications. Currently, prenatal diagnosis is generally feasible and allows an early thoracoscopic intervention. The purpose of this retrospective study was to ascertain the best time for the operation. METHODS: Reviewed were 36 patients (11 children, 25 adults) with a BC managed from 2000 to 2011. Clinical history, cyst size, duration of hospitalization, preoperative and postoperative complications, and detection of inflammatory elements were compared (Student t tests) between pediatric and adult patients. RESULTS: In the pediatric group, diagnosis was made prenatally in 7 patients, during the neonatal period in 2, and later in 2. Nine were asymptomatic. In the adult group, 20 patients were treated for complications. Thoracotomy was performed in 2 children and thoracoscopy in 9 (no conversion). A thoracoscopic operation was performed in 9 adults (2 conversions), and 17 adults required additional procedures (4 pericardial and 9 lung resections, 3 bronchial, and 1 esophageal sutures). The average length of hospitalization was 4.45 days for children (3.33 days in the thoracoscopic subgroup) and 8 days for adults. Mean maximal cyst diameter was 2.2 cm in children and 6.5 cm in adults (p < 0.10). Pathologic study revealed inflammatory reaction in 2 children (18%) vs 21 adults (84%; p < 0.001). CONCLUSIONS: Early surgical resection of BCs provides better conservation of pulmonary parenchyma, a lower incidence of inflammatory lesions, and a reduced rate of complications, and should be proposed after prenatal diagnosis, between the 6th and 12th month of life.

Outcome of alimentary tract duplications operated on by minimally invasive surgery: a retrospective multicenter study by the GECI (Groupe d'Etude en Coeliochirurgie Infantile).

BACKGROUND: Alimentary tract duplications (ATD) are a rare cause of intestinal obstruction in childhood. There are many case reports but few series about laparoscopy or thoracoscopy for ATD. The aim of our study was to report the outcome of minimally invasive surgery (MIS) for ATD. METHODS: This was a retrospective multicenter study from the GECI (Groupe d'Etude en Coeliochirurgie Infantile). We reviewed the charts of 114 patients operated on by MIS for ATD from 1994 to 2009. RESULTS: Sixty-two patients (54 %) had a prenatal diagnosis. Forty-nine patients (43 %) were symptomatic before surgery: 33 of those patients (63 %) with postnatal diagnosis compared to 16 (25 %) with prenatal diagnosis (P < 0.01). In this last group, the median age at onset of symptoms was 16 days (range = 0-972). One hundred and two patients had laparoscopy (esophageal to rectal duplications) and 12 patients had thoracoscopy for esophageal duplications. The mean operative time was 90 min (range = 82-98). There were 32 (28 %) resection anastomoses, 55 (48 %) enucleations, and 27 (24 %) unroofings. The conversion rate was 32 %, and in a multivariate analysis, it was significantly higher, up to 41 % for patients weighing <10 kg (P < 0.01). Ten patients (8 %) had unintentional perioperative opening of the digestive tract during the dissection. Eight patients had nine postoperative complications, including six small bowel obstructions. The median length of hospital stay was 4 days (range = 1-21) without conversion and 6 days (range = 1-27) with conversion (P = 0.01). The median follow-up was 3 months (range = 1-120). Eighteen of the 27 patients who underwent partial surgery had an ultrasound examination during follow-up. Five (18 %) of them had macroscopic residue. CONCLUSION: This study showed that MIS for ATD is feasible with a low rate of complications. Patients with prenatal diagnosis should have prompt surgery to prevent symptoms, despite a high rate of conversion in small infants.

Major bile duct injuries during cholecystectomy in children: conservative laparoscopic approach is possible.

Major bile duct injury is an inherent complication in cases of both open and laparoscopic cholecystectomies. In case of choledochal lesion, conservative treatment or internal derivation by a Roux-en-Y can be proposed. We report the case of a 5-year-old boy referred to our center for an iatrogenic choledochal ligation after open cholecystectomy (performed 20 d before) for asymptomatic gallbladder stone. We performed a laparoscopic conservative treatment with a consistent good result 5 years after the procedure.

[The pseudopapillary tumor of the pancreas (or Frantz's tumor)]. / Tumeur cystopapillaire du pancréas (tumeur de Frantz) chez l'adolescent et l'adulte jeune.

The pseudopapillary tumor of the pancreas (or Frantz's tumor) is a rare exocrine pancreatic tumor with a low degree of malignancy. It occurs more frequently among women between 20 and 40 years of age and in the Asian population. This tumor is rarer in the pediatric population. The symptoms are subtle, the most striking being pain and an abdominal mass. Pathologically, the tumor is usually well circumscribed with regions of necrosis, hemorrhage and cystic degeneration. A thick, fibrous capsule is often present. The low grade of malignancy of this tumor with a fibrous capsule led to perform a surgical resection. The localization and local invasion determine the surgical technique. Despite its potential for local infiltration and metastatic disease (up to 15% confined often to the liver), the prognosis is favorable after a surgical resection with correct margins. Long follow-up is necessary to detect a possible recurrence, even late.

Nitric oxide activity through guanylate cyclase and phosphodiesterase modulation is impaired in fetal lambs with congenital diaphragmatic hernia.

BACKGROUND: Congenital diaphragmatic hernia (CDH) is associated with pulmonary hypertension and death. Administration of nitric oxide (NO) alone remains ineffective in CDH cases. We investigated in near full-term lambs with and without CDH the role of guanylate cyclase (GC), the enzyme activated by NO in increasing cyclic 3'-5'-guanylosine monophosphate, and the role of phosphodiesterase (PDE) 5, the enzyme-degrading cyclic 3'-5'-guanylosine monophosphate. METHODS: Congenital diaphragmatic hernia was surgically created in fetal lambs at 85 days of gestation. Pulmonary hemodynamics were assessed by means of pressure and blood flow catheters (135 days). In vitro, we tested drugs on rings of isolated pulmonary vessels. RESULTS: In vivo, sodium nitroprusside, a direct NO donor, and methyl-2(4-aminophenyl)-1,2-dihydro-1-oxo-7-(2-pyridinylmethoxy)-4-(3,4,5 trimethoxyphenyl)-3-isoquinoline carboxylate sulfate (T-1032) and Zaprinast, both PDE 5 blockers, reduced pulmonary vascular resistance in CDH and non-CDH animals. The activation of GC by sodium nitroprusside and the inhibition of PDE 5 by T-1032 were less effective in CDH animals. In vitro, the stimulation of GC by 3(5'hydroxymethyl-2'furyl)-1-benzyl indazole (YC-1) (a benzyl indazole derivative) and the inhibition of PDE 5 by T-1032 were less effective in pulmonary vascular rings from CDH animals. The YC-1-induced vasodilation in rings from CDH animals was higher when associated with the PDE 5 inhibitor T-1032. CONCLUSIONS: Guanylate cyclase and PDE 5 play a role in controlling pulmonary vascular tone in fetal lambs with or without CDH. Both enzymes seem to be impaired in fetal lambs with CDH.

Acute effect of a dual ETA-ETB receptor antagonist on pulmonary arterial vasculature in preterm lamb fetuses with surgically induced diaphragmatic hernia.

PURPOSE: To study the effects of tezosentan, a dual ETA and ETB receptor antagonist on the cardiopulmonary profile in a fetal lamb model of CDH in utero. METHODS: A diaphragmatic hernia was surgically created at day 75 of gestation. During 45 min of tezosentan perfusion (1 mg/kg), hemodynamic parameters (pulmonary and aortic pressures, left pulmonary and aortic flows, left auricle pressure, heart rate) were measured at day 135 of gestation. Age-matched fetal lambs served as control animals. Secondarily, parietal tension of vessels rings of pulmonary arteries was assessed in organ baths under increasing concentration of tezosentan. RESULTS: In CDH group, under perfusion of tezosentan, pulmonary artery pressure decreased from 45.8 ± 4.1 to 37.6 ± 5.9 mmHg (P < 0.05). Pulmonary artery flow and pulmonary vascular resistance remained constant. In control group, pulmonary artery flow increased from 153.9 ± 15.8 to 233.4 ± 26 ml/min (P < 0.05). Pulmonary artery pressure did not vary. Subsequently calculated pulmonary vascular resistance decreased. In organ bath, no significant relaxation was observed. CONCLUSION: In this fetal lamb model of CDH, tezosentan decreased pulmonary artery pressure but did not modify pulmonary blood flow. Endothelin may play a role in the regulation of pulmonary vascular tone in utero.

Thoracoscopy in the management of congenital lung diseases in infancy.

BACKGROUND: This study aimed to compare the results of thoracoscopic surgery for congenital lung diseases between infants younger than 6 months and those older than 6 months at the time of surgery in terms of operation duration, surgical complications, chest tube duration, and hospital stay. METHODS: The charts of 30 thoracoscopic resections for congenital lung diseases were retrospectively reviewed. This study compared 17 children younger than 6 months (mean, 3.94 months; range, 0.37-5.7 months; group 1) with 13 children older than 6 months (mean, 12.05 months; range, 6.2-24.63 months; group 2) at the time of surgery. The median follow-up period was 9 months (range, 1-41 months). RESULTS: Lobectomy was performed in 27 cases, bilobectomy in 1 case, and nonanatomic excision in 2 cases. The mean operating time for group 1 (176±54 min) was similar to that for group 2 (160±46 min). The difference is not significant. The mean duration of chest tube drainage was similar in the two groups (4.4 days; range, 1-9 days for group 1 vs. 4.1 days; range, 3-8 days for group 2). The complications included 1 major and 10 minor complications, with no statistically significant difference between the two groups. Three surgical procedures in each group were converted. The hospital stay was not statistically different between the two groups (8 days; range, 3-20 days for group 1 vs. 6 days; range, 4-10 days for group 2). CONCLUSIONS: The study findings showed no statistically significant difference between the two groups in terms of operation time, complication rate, conversion rate, or hospital stay. Lobectomy can be safely and successfully performed by thoracoscopy even for children younger than 6 months.