P de Lagausie

Prenatal management of gastroschisis: the place of the amnioexchange procedure

Gastroschisis is a malformation of the anterior abdominal wall that consists of a right paraumbilical defect with bowel loops bathed in the amniotic fluid. The survival rate is now greater than 90% and the prognosis relies mainly on morbidity attributable to bowel dysfunction. Recent research has examined gastrointestinal waste present in amniotic fluid that induces bowel toxicity and an inflammatory process. The amnioexchange procedure (changing the amniotic fluid regularly) involves a new therapeutic approach: reducing bowel injuries in the fetuses. This article shows that there is an inflammatory reaction in human gastroschisis and in the authors model, and that the clinical and biological data plead for the practice of amnioexchange in human beings. A randomized, controlled study is now needed.

Prenatal diagnosis and evaluation of defect length in esophageal atresia using direct and indirect (tracheal print) signs

The prenatal diagnosis of esophageal atresia is challenging. The length of the defect of the esophageal atretic portion is one of the parameters affecting outcome and prenatal evaluation of this length has not, to our knowledge, been described previously. We report on seven fetuses assessed prospectively which were suspected to have esophageal atresia. Targeted ultrasound examination of both fetal cervical and thoracic structures was performed in each case in order to assess prenatally the atretic portion. The length of the defect was assessed both directly, by visualizing the interruption of the hyperechoic lines representing the walls of the esophagus in a mid‐sagittal view (n = 4), and indirectly, by means of the ‘tracheal print’ (n = 5). Both methods were used in three cases. Prenatal results were compared with postnatal or postmortem findings. The prenatal diagnosis of esophageal atresia was made correctly in six of the seven cases and in all of these there was concordance between prenatal and postnatal estimates of the esophageal defect lengths. Direct or indirect sonographic assessment of the esophagus in cases of suspected prenatal esophageal atresia improves the specificity of its diagnosis and aids prenatal evaluation. Copyright

Invaginations intestinales itératives et mucocèle appendiculaire chez un enfant atteint de mucoviscidose

Resume Les manifestations digestives de la mucoviscidose, en dehors de linsuffisance pancreatique externe et de lileus meconial, sont rares et de gravite variable. Observation Un enfant souffrant de mucoviscidose a, a lâge de 4,5 ans, une invagination ileocolique. Sa recidive justifie une appendicectomie, qui permet le diagnostic de mucocele appendiculaire. Neanmoins, les episodes dinvaginations se reproduisent et necessitent une resection ileocolique droite, 2 ans plus tard, emportant le moignon appendiculaire obstrue. Conclusion La mucocele appendiculaire peut etre responsable dinvagination ileocolique. Lexerese dune collerette c˦cale lors de l appendicectomie pourrait etre utile pour eviter que la mucocele ne se reforme a partir du moignon appendiculaire.

La hernie diaphragmatique congénitale. I. Simple defect du diaphragme ou pathologie du mésenchyme pulmonaire

Resume Decrit pour la premiere fois en 1848 par Bochdalek, la hernie diaphragmatique congenitale est plus que jamais un sujet dactualite. Comment se fait-il que la mortalite dun simple defect diaphragmatique avoisine encore 50 % en 1997 et ce en depit des progres continus en reanimation neonatale ? Si un certain nombre de problemes restent non resolus, les nombreux travaux experimentaux des 30 dernieres annees ont permis de remettre en question certains dogmes et de mieux comprendre la physiopathologie de la hernie diaphragmatique congenitale. Cet article passe en revue les recentes acquisitions concernant letiologie, la pathogenie et la physiopathologie de cette malformation complexe.

Crosse aortique gauche — aorte descendante droite — ligament artériel droit (aorte encerclante). Une malformation rare des arcs aortiques

Resume Les anomalies des arcs aortiques susceptibles de comprimer laxe tracheobronchique sont bien connues. Cette observation montre linteret de limagerie par resonance magnetique (IRM) pour le diagnostic et rappelle que les troubles respiratoires chez le nourrisson doivent faire evoquer ces anomalies. Observation Les auteurs rapportent une malformation tres rare des arcs aortiques a type daorte encerclante avec crosse aortique gauche, aorte descendante droite et ligament arteriel droit. Elle sest revelee par des troubles respiratoires iteratifs dus a la compression de laxe tracheobronchique par le ligament arteriel. Le traitement a ete chirurgical, par section du ligament permettant de lever la compression. Le diagnostic de telles anomalies est habituellement etabli sur les cliches du transit œsogastroduodenal (TOGD), la fibroscopie tracheobronchique, langiographie et lIRM. Conclusion Dans le cas present, l IRM sest revelee superieure a l angiographie. La section du ligament arteriel est parfois insuffisante si lagent de compression est represente par laorte. Le decroisement aortique, intervention plus complexe, represente alors lalternative chirurgicale.

OC209: Amniotic fluid exchange in gastroschisis

history. When abnormalities were detected CDTVU was repeated after 4–6 weeks, if the abnormality persisted, tumor markers, CT and laparoscopy were done. The mean age of the patients was 47 ± 12 year ranging from 16 to 88 years. Results: From 22.013 CDTVU performed 35 were diagnosed as suspicious of malignancy. Twenty nine of these patients have been surgically treated, in two the repeated CDTVU was normal and 4 were lost from follow-up. Nineteen of the surgically treated women had malignant lesions hystologically confirmed, 15 of them were tumors in stage I, 3 in stage III and one was a metastatic melanoma. In 10 patients benign lesions were diagnosed (3 serous cysts, 2 mucinous cysts, 2 endometriotic cysts, 1 cystadenofibroma, 1 cystic teratoma and 1 hydrosalpinx with paraovarian cyst). Conclusion: Although consensus about the benefits of using CDTVU as a screening procedure are not conclusive, our results suggest that it can help in diagnose of ovarian cancer in early stages.