Nadia Belarbi

Ileal involvement is age dependent in pediatric Crohn's disease.

Background: Lymphoid follicles (LFs) have been suggested to play a role at the early stage of Crohns disease (CD) lesions. In the small bowel, LFs are grouped, forming Peyers patches, which develop early in fetal life, grow in size and number until puberty, and undergo involution. In contrast, colonic LFs are isolated and undergo little change during life. As a result, if LFs play a role in the occurrence of CD lesions, the distribution of ileal and colonic lesions is expected to be altered in small children. Methods: Medical records of 2 independent French (n = 136) and Swedish (n = 55) cohorts of consecutive pediatric CD were reviewed. Disease sites and age of onset were recorded, and the age‐dependent probability to develop ileal lesions was computed. The CARD15/NOD2 genotype was also analyzed when available (n = 99). Results: The curves of disease occurrence were significantly different in case of CD with or without ileal lesions (P < 0.0001). At the age of 8 years, the probability (95% confidence interval) of small bowel involvement was 0.19 (0.07‐0.39). It increased until 16 years of age to 0.61 (0.54‐0.68). It was slightly higher in patients carrying 1 or more CARD15/NOD2 mutations [0.75 (0.55‐0.89)] than in wild‐type patients [0.46 (0.34‐0.58)]. CARD15 mutations also influenced the age of onset of ileal disease (P < 0.02). Conclusions: In children, ileal CD lesions are delayed compared with colonic lesions. This observation is in agreement with the previously proposed hypothesis of a pathophysiological role of Peyers patches in ileal CD. The rarity of small bowel lesions should be a warning to be cautious when classifying chronic colitis in small children.

Ultrasonography of Crohn disease in children.

US is increasingly performed in Crohn disease (CD) in children as a first line imaging modality. It reduces the use of other more invasive examinations such as endoscopy, CT or contrast enema. We describe bowel ultrasonography technique, normal bowel appearances on US and pathological patterns in CD. We discuss the current role and limitations of bowel US in CD in children including diagnosis, extent of disease, assessment of disease activity, follow-up and detection of complications. The diagnostic accuracy of US is discussed according to the literature and compared to other imaging modalities. US is currently used for screening in children with the suspicion of inflammatory bowel disease (IBD) with a good negative predictive value. In follow-up, US has a role in monitoring medical treatment by evaluating disease activity, extent of disease and for detecting complications.

Laparoscopic distal pancreatectomy for Frantz’s tumor in a child

Solid pseudopapillary tumor of the pancreas is a rare pathologic entity. Although the role of laparoscopy in surgery of the pancreas is still controversial, laparoscopic distal pancreatectomy has been reported with good results in adults. We report a laparoscopic spleen-preserving distal pancreatectomy in a 9-year-old boy who presented with a low-grade malignant tumor. Needle biopsy was impossible. A laparoscopic spleenpreserving distal pancreatectomy was performed. We used four trocars, and the operative time was 240 min. Conversion to open surgery was not necessary. The boy’s postoperative recovery was uneventful, and he was discharged on the 6th day. CT-scan control at 6 months was normal. This case shows that even in advanced surgical cases, such as spleen-preserving distal pancreatectomy, laparoscopic procedures can be done safely, within a reasonable operative time, in children.

Intrahepatic foreign body laparoscopic extraction

An 11-month-old boy presented with an intrahepatic foreign body after ingestion of a sewing needle. Surgical management using an exclusive laparoscopic extraction was successful.

Mowat–Wilson syndrome in a fetus with antenatal diagnosis of short corpus callosum: Advocacy for standard autopsy

Mowat-Wilson syndrome (MWS) is a genetic disease caused by heterozygous mutations or deletions of the ZEB2 gene rarely diagnosed prenatally and with little fetal description reported. It is mainly characterized by moderate-to-severe intellectual disability, epilepsy, facial dysmorphism and various malformations including Hirschsprung disease and corpus callosum anomalies. Here we report a fetal case of MWS well described, suspected at standard autopsy. The association of a corpus callosum hypoplasia with a histological Hirschsprung disease and a typical facial gestalt allowed the guiding of genetic testing. Classical fetopathological examination still keeps indications in cases of syndromic association in the era of virtual autopsy.

Trigonocephaly and valproate: a case report and review of literature.

Morgane Valentin1, Guillaume Ducarme1*, Carine Yver1, Edith Vuillard2, Nadia Belarbi3, Dominique Renier4 and Dominique Luton1 1Department of Obstetrics and Gynecology. Hôpital Beaujon, AP-HP, Université Paris 7, Clichy, France 2Department of Prenatal Diagnosis. Hôpital Robert Debré, AP-HP, Université Paris 7, Paris, France 3Department of Pediatric Radiology. Hôpital Robert Debré, AP-HP, Université Paris 7, Paris, France 4Department of Pediatric Neurosurgery. Hôpital Necker-Enfants Malades, AP-HP, Université 5, Paris, France

Prenatal diagnosis of Pierre Robin Sequence: accuracy and ability to predict phenotype and functional severity

To assess the outcome of fetuses who had sonographic features suggestive of Pierre Robin Sequence (PRS).

Apparent diffusion coefficient measurements of the fetal brain during the third trimester of pregnancy: how reliable are they in clinical practice?

The objective of this study was to determine the reproducibility, the inter‐hemispheric difference and the reference apparent diffusion coefficient (ADC) values of the fetal brain according to gestational age.

Prenatal pelvic MRI: additional clues for assessment of urogenital obstructive anomalies.

OBJECTIVE Ultrasound prenatal evaluation of pelvic cystic mass can be challenging. After having ruled out a cloaca anterior to a large hydrocolpos, it is important to differentiate between combined urogenital anomalies such as urogenital sinus and isolated genital anomalies. PATIENTS AND METHODS We reviewed the charts of 13 women referred for a third trimester pelvic MRI for cystic pelvic mass discovered in second trimester ultrasound. We evaluated MRI compared with postnatal surgical findings in order to determine clues for improving prenatal diagnoses. RESULTS MRI excluded the diagnosis of cloacal malformation in nine cases with no false negative. Once a cloaca is ruled out, a different signal between the bladder and the hydrocolpos on T2 sequences is in favor of an isolated genital obstruction. In contrast, in case of urogenital sinus, the vagina is filled with a mixture of genital secretions and urine, which gives it an MRI signal similar to the bladder on T2 sequences. CONCLUSION Third trimester fetal MRI is an essential exam for characterization of pelvic cystic mass diagnosed by ultrasound. This exam appears valuable for invalidating the diagnosis of cloacal malformation and for differentiating between isolated genital obstruction and urogenital sinus.

Is fetal cerebral MRI worthwhile in antenatally diagnosed isolated cleft lip with or without palate

This study aims to evaluate the use of fetal brain magnetic resonance imaging (MRI) following an antenatal sonographic diagnosis of isolated cleft lip with or without cleft palate (CL/P).