Congenital lung malformations: correlation between prenatal and postnatal imaging and pathological findings

Aim: Congenital lung malformations are a common finding during prenatal ultrasonography (US). Investigations were completed by means of prenatal MRI and postnatal computed tomographic (CT) scan. The purpose of this study was to compare these prenatal findings with postnatal findings and pathological findings after surgical resection.Materials and methods: Prenatal examinations and postnatal CTscan results of congenital malformations were compared with pathological findings.Results: From 2007 to 2013, 39 prenatally diagnosed congenital lung malformations were resected: 18 congenital cystic adenomatoid malformation, eight pulmonary sequestration, five bronchogenic cyst, one bronchial atresia and six complex lesions. Correlation between imaging and diagnosis was as follows: congenital cystic adenomatoid malformation was seen in 17/17 patients using postnatal CT, in 10/15 patients using prenatal MRI and in 17/18 patients using prenatal US. Correlation between imaging and diagnosis was as follows: bronchogenic cyst was seen in 3/5, 3/5 and 3/5 patients, pulmonary sequestration was seen in 7/9, 5/9 and 4/9 patients, and complex lesion was seen in 4/5, 3/6, and 2/6 patients using postnatal CT, prenatal MRI, and prenatal US,  respectively. Overall, 32/37 cases were diagnosed by means of postnatal CT, 21/36 cases were diagnosed by means of prenatal MRI and 26/39 cases were diagnosed by means of prenatal US.Conclusion: Discordance between imaging data and definitive diagnosis is not rare. Our results suggest that postnatal CT scan is the most sensible and specific examination. Before birth, US seems better compared with MRI for description of the lesion. MRI seems to be useful in case of complex lesions and pulmonary sequestration.Keywords: congenital lung malformation, imaging, prenata

Atrésie de l'oesophage (quelle qualité de vie à long terme ?)

AIX-MARSEILLE2-BU Méd/Odontol. (130552103) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

ETUDE DE LA FIABILITE DES TESTS RAPIDES POUR LE DIAGNOSTIC DES INFECTIONS URINAIRES DANS LE SERVICE DES URGENCES PEDIATRIQUES DE LA TIMONE

AIX-MARSEILLE2-BU Méd/Odontol. (130552103) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Bronchogenic cyst: Clinical course from antenatal diagnosis to postnatal thoracoscopic resection

Purpose: The purpose of this study was to describe an approach to surgical management of bronchogenic cysts based on the natural course observed from the time of antenatal screening to surgical resection in patients treated at our institution and reported in the literature. Materials and Methods: We retrospectively reviewed the clinical features of all children presenting bronchogenic cyst diagnosed antenatally from 2007 to 2010. A total of six children were included. Results: Antenatal diagnosis was accurate in 62.5% of cases. In the first year of life, the size of the cyst remained stable in four patients, doubled in one, and increased 30% within six months in one. The indication for surgery was emphysema of the left bronchus in two patients and rapid growth in two patients. One patient is still awaiting surgery. Conclusion: Bronchogenic cysts grow slowly in the first months of life, but growth is exponential even in the absence of complications. We recommend complete resection before the age of two years to prevent infectious complications and facilitate surgery

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

Neurostimulation-Guided Anal Intrasphincteric Botulinum Toxin Injection in Children with Hirschsprungʼs Disease

International audienceIn Hirschsprung disease (HD), despite successful surgical treatment, 50% of children experience long-term functional gastrointestinal problems, particularly chronic functional obstructive symptoms. We report our experience regarding clinical effects of neurostimulation-guided anal intrasphincteric botulinum toxin (BT) injections on postoperative obstructive symptoms attributed to a nonrelaxing anal sphincter complex in HD patients

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

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

Phasic hemodynamics and reverse blood flows in the aortic isthmus and pulmonary arteries of preterm lambs with pulmonary vascular dysfunction

Time-domain representations of the fetal aortopulmonary circulation were carried out in lamb fetuses to study hemodynamic consequences of congenital diaphragmatic hernia (CDH) and the effects of endothelin-receptor antagonist tezosentan (3 mg/45 min). From the isthmic aortic and left pulmonary artery (PA) flows (Q) and isthmic aortic, PA, and left auricle pressures (P) on day 135 in 10 controls and 7 CDH fetuses (28 ewes), discrete-triggered P and Q waveforms were modelized as Pt and Qt functions to obtain basic hemodynamic profiles, pulsatile waves [P, Q, and entry impedance (Ze)], and P and Q hysteresis loops. In the controls, blood propelling energy was accounted for by biventricular ejection flow waves (kinetic energy) with low Ze and by flow-driven pressure waves (potential energy) with low Ze. Weak fetal pulmonary perfusion was ensured by reflux (reverse flows) from PA branches to the ductus anteriosus and aortic isthmus as reverse flows. Endothelin-receptor antagonist blockade using tezosentan slightly increased the forward flow but largely increased diastolic backward flow with a diminished left auricle pre- and postloading. In CHD fetuses, the static component overrode phasic flows that were detrimental to reverse flows and the direction of the diastolic isthmic flow changed to forward during the diastole period. Decreased cardiac output, flattened pressure waves, and increased forward Ze promoted backward flow to the detriment of forward flow (especially during diastole). Additionally, the intrapulmonary arteriovenous shunting was ineffective. The slowing of cardiac output, the dampening of energetic pressure waves and pulsatility, and the heightening of phasic impedances contributed to the lowering of aortopulmonary blood flows. We speculate that reverse pulmonary flow is a physiological requirement to protect the fetal pulmonary circulation from the prominent right ventricular stream and to enhance blood flow to the fetal heart and brain