Hydronephrosis caused by kidney malrotation.

Hydronephrosis associated with kidney malrotation can be a surgical challenge. We present the case of a 3.5 y.o.-boy presenting with left pyelo-ureteric obstruction caused by kidney hyperrotation (270°) resulting in recurring urinary tract infection. After complete radiological work-up, we opted for a primary laparoscopic ureterocalicostomy, which allowed for complete resolution of the pelvic dilatation. Kidney malrotation can present with a wide variation in anatomic features. Radiological work-up is the cornerstone of surgical strategy planning. Laparoscopic ureterocalicostomy is a useful primary option in unusual anatomical situations

Challenges in the Diagnosis and Management of Acquired Nontraumatic Urethral Strictures in Boys in Yaoundé, Cameroon

Introduction. Urethral strictures in boys denote narrowing of the urethra which can be congenital or acquired. In case of acquired strictures, the etiology is iatrogenic or traumatic and rarely infectious or inflammatory. The aim of this study was to highlight the diagnostic and therapeutic difficulties of acquired nontraumatic urethral strictures in boys in Yaoundé, Cameroon. Methodology. The authors report five cases of nontraumatic urethral strictures managed at the Pediatric Surgery Department of the YGOPH over a two-year period (November 2012–November 2014). In order to confirm the diagnosis of urethral stricture, all patients were assessed with both cystourethrography and urethrocystoscopy. Results. In all the cases the urethra was inflammatory with either a single or multiple strictures. The surgical management included internal urethrotomy (n=1), urethral dilatation (n=1), vesicostomy (n=2), and urethral catheterization (n=3). With a median follow-up of 8.2 months (4–16 months) all patients remained symptoms-free. Conclusion. The authors report the difficulties encountered in the diagnosis and management of nontraumatic urethral strictures in boys at a tertiary hospital in Yaoundé, Cameroon. The existence of an inflammatory etiology of urethral strictures in boys deserves to be considered

HSPG-Deficient Zebrafish Uncovers Dental Aspect of Multiple Osteochondromas

Multiple Osteochondromas (MO; previously known as multiple hereditary exostosis) is an autosomal dominant genetic condition that is characterized by the formation of cartilaginous bone tumours (osteochondromas) at multiple sites in the skeleton, secondary bursa formation and impingement of nerves, tendons and vessels, bone curving, and short stature. MO is also known to be associated with arthritis, general pain, scarring and occasional malignant transformation of osteochondroma into secondary peripheral chondrosarcoma. MO patients present additional complains but the relevance of those in relation to the syndromal background needs validation. Mutations in two enzymes that are required during heparan sulphate synthesis (EXT1 or EXT2) are known to cause MO. Previously, we have used zebrafish which harbour mutations in ext2 as a model for MO and shown that ext2−/− fish have skeletal defects that resemble those seen in osteochondromas. Here we analyse dental defects present in ext2−/− fish. Histological analysis reveals that ext2−/− fish have very severe defects associated with the formation and the morphology of teeth. At 5 days post fertilization 100% of ext2−/− fish have a single tooth at the end of the 5th pharyngeal arch, whereas wild-type fish develop three teeth, located in the middle of the pharyngeal arch. ext2−/− teeth have abnormal morphology (they were shorter and thicker than in the WT) and patchy ossification at the tooth base. Deformities such as split crowns and enamel lesions were found in 20% of ext2+/− adults. The tooth morphology in ext2−/− was partially rescued by FGF8 administered locally (bead implants). Our findings from zebrafish model were validated in a dental survey that was conducted with assistance of the MHE Research Foundation. The presence of the malformed and/or displaced teeth with abnormal enamel was declared by half of the respondents indicating that MO might indeed be also associated with dental problems

Evolutionary Trends of the Pharyngeal Dentition in Cypriniformes (Actinopterygii: Ostariophysi)

International audienceBACKGROUND: The fish order Cypriniformes is one of the most diverse ray-finned fish groups in the world with more than 3000 recognized species. Cypriniformes are characterized by a striking distribution of their dentition: namely the absence of oral teeth and presence of pharyngeal teeth on the last gill arch (fifth ceratobranchial). Despite this limited localisation, the diversity of tooth patterns in Cypriniformes is astonishing. Here we provide a further description of this diversity using X-ray microtomography and we map the resulting dental characters on a phylogenetic tree to explore evolutionary trends. RESULTS: We performed a pilot survey of dental formulae and individual tooth shapes in 34 adult species of Cypriniformes by X-ray microtomography (using either conventional X-ray machine, or synchrotron microtomography when necessary) or by dissecting. By mapping morphological results in a phylogenetic tree, it emerges that the two super-families Cobitoidea and Cyprinoidea have followed two distinct evolutionary pathways. Furthermore, our analysis supports the hypothesis of a three-row dentition as ancestral for Cyprinoidea and a general trend in tooth row reduction in most derived lineages. Yet, this general scheme must be considered with caution as several events of tooth row gain and loss have occurred during evolutionary history of Cyprinoidea. SIGNIFICANCE: Dentition diversity in Cypriniformes constitutes an excellent model to study the evolution of complex morphological structures. This morphological survey clearly advocates for extending the use of X-ray microtomography to study tooth morphology in Cypriniformes. Yet, our survey also underlines that improved knowledge of Cypriniformes life traits, such as feeding habits, is required as current knowledge is not sufficient to conclude on the link between diet and dental morphology

Diagnostic et prise en charge de la torsion testiculaire chez l’enfant [Diagnosis and management of testicular torsion in children]

Testicular torsion is a true urologic emergency with a bimodal age presentation : the perinatal testicular torsion presenting with a hard, tender or non-tender scrotal mass usually with underlying dark discoloration of the skin and the peripubertal testicular torsion presenting with severe acute testicular pain, vomiting and frequently pain irradiation in the inguinal area, a high riding-horizontally positioned testis and absence of the cremasteric reflex. Obtaining a US Doppler must not add unnecessary delay to the urgent surgical treatment which entails detorsion and bilateral orchidopexy. We do not recommend manual detorsion as around 30 % of testis rotate externally. Up to 50 % of patients presenting with a testicular torsion suffered beforehand from intermittent testicular pain episodes