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Objective: To evaluate the complementarity between prenatal ultrasound, computed tomography, and MRI scans for fetuses with Binder phenotype., Methods: We carried out a retrospective study from January 1, 2009, to June 30, 2018, of fetuses with Binder phenotype. Prenatal ultrasound (US) data were collected. A systematic survey of the entire skeleton was performed to look for associated abnormalities such as calcifications, brachytelephalangy, and spinal stenosis. Parents were systematically offered fetal skeletal computed tomography (CT)., Results: Thirteen cases were included. Two cases of perinatal respiratory distress (18%) were observed. Chondrodysplasia punctata was diagnosed from the presence of calcifications, especially of the proximal femoral epiphyses and tarsal bones, in five cases (38%) by US and in 10 cases (83%) by CT. Calcifications of the hyoid bone were detected by CT in three cases (25%) one of which had respiratory distress. Polyhydramnios was associated with the Binder phenotype in four cases (30%) one of which had respiratory distress. One single fetus had combined polyhydramnios and laryngeal calcifications, and he suffered from perinatal respiratory distress., Conclusion: An antenatal diagnosis of Binder phenotype is often associated with chondrodysplasia punctata. We recommend the use of fetal CT as a complement to US in this condition., (© 2019 John Wiley & Sons, Ltd.)

The field and applications of postmortem imaging are exponentially growing. Its potential to identify the cause of death in trauma and ballistic cases is now properly documented, as well as its use in drug mule identification. In pediatric and perinatal practice, large significant series are less available, except for MRI and central nervous system analysis where scientific evidence is now robust. In this review, after a short historical review and analysis of current problems and challenges, we will try to depict the way we see the future of this subspecialty of postmortem cross-sectional imaging, including all specific situations: terminations of pregnancy, intrauterine death, sudden unexpected infant death and identification issues.

Background: It has been described that both the colon and distal ileum present with a physiological hypersignal on T1-weighted sequences during the second and third trimesters of pregnancy because of their protein-rich meconium content, it was unclear whether the normal characteristics that have been described on fetal MRI can be applied to gastrointestinal (GI) obstructions., Objective: To analyse the localisation value of T1 hypersignal within dilated bowel loops in fetuses with gastrointestinal tract obstruction., Materials and Methods: A retrospective 4-year multicentre study analysing cases of fetal GI obstruction in which MRI demonstrated T1 hypersignal content in the dilated loops. Data collected included gestational age (GA) at diagnosis, bowel appearance on US, CFTR gene mutations and amniotic levels of gastrointestinal enzymes. The suggested prenatal diagnosis was eventually compared to postnatal imaging and surgery., Results: Eleven patients were included. The median GA at US diagnosis was 23 weeks (range 13-32). In eight cases there was a single dilated loop, while several segments were affected in three. The median GA at MRI was 29 weeks (range 23-35). One case presented with cystic fibrosis mutations. Final prenatally suspected diagnoses were distal ileal atresia or colon in nine cases and proximal atresia in two. Postnatal findings were proximal jejunal atresia in nine cases and meconium ileus in two. In five cases the surgical findings demonstrated short bowel syndrome., Conclusion: In cases of fetal occlusion, T1 hypersignal should not be considered as a sign of distal ileal or colonic occlusion. The obstruction may be proximal, implying a risk of small bowel syndrome, which requires adequate parental counselling.

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. [ABSTRACT FROM AUTHOR]

Abstract: 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. [Copyright &y& Elsevier]

Intralobar sequestration (ILS) associated with congenital cystic adenomatoid malformation (CCAM) is a well-known entity. This hybrid form has many considerations for an appropriate management. This report aims to analyze pitfalls in the diagnosis, treatment, and thoracoscopic procedure pertaining to this association. Between May 2004 and September 2007, 23 fetal lung lesions were diagnosed prenatally and managed at the authors’ institution. Seven of these lesions were the hybrid form of ILS-CCAM. The records for these lesions were reviewed retrospectively. In all seven fetuses, prenatal ultrasound diagnosed congenital lung abnormalities. In three cases, fetal magnetic resonance imaging (MRI) demonstrated a macrocystic lesion supplied by an aberrant vessel. In all cases, the intrauterine evolution remained uncomplicated. All the newborns were asymptomatic at birth. Chest computed tomography (CT) performed at the age of 1 month showed the aberrant vessel in five cases. A regression of the lesion was noted in one patient and that of the aberrant vessel in another patient. Elective thoracoscopic lobectomy of the affected lobe was performed for six patients. The average age at the time of intervention was 5 months. At this writing, one patient is under observation due to resolution of the lesion. The operating time ranged from 80 to 160 min. One conversion to thoracotomy was required. All surgically treated patients had an uneventful hospital course. Histopathologic examination confirmed the association of intralobar sequestration with Stocker type 2 CCAM in five cases and with CCAM type 1 in one case. Prenatal diagnosis of the ILS-CCAM association was possible, as was resolution of one or both components of this lesion. Preoperative identification of the aberrant vessel is important for prevention of operative morbidity. This should be controlled before the lobectomy is begun. [ABSTRACT FROM AUTHOR]

Abstract: 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 20weeks 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: 35mm at 33weeks 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 5.6years. 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. [Copyright &y& Elsevier]