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Final ID: Poster #: CR-01 (R)

A diffuse form of neurofibromatosis of the bladder in a new born: radiological presentation of a case

Purpose or Case Report: Objective: to report an unsual presentation of neurofibromatosis involving the genito-urinary tract. Bladder involvement was the initial presentation of a Von Recklinghausen disease in a new born for whom the family disease was initially unknown by the medical staff.
Methods & Materials: We report an exceptional case of a new born girl who presented with antenatal left hydronephrosis on ultrasound at 36 weeks LMP ( last menstrual period). Post natal US and MRI performed the first month of life revealed a diffuse nodular bladder thickening, progressing on the follow up examinations and showing trigone involvement with bilateral uretero- hydronephrosis. Cystoscopy revealed a nodular thickening of the bladder wall, hiding both ureteral ostia. Bilateral nephrostomy and secondarily left ureterostomy have been performed. Bladder biopsy diagnosed a plexiform neurinoma.
Results: At 6 month old, the child does not suffer from secondary localization but shows delayed growth. She is medically treated by a drug on trial. Surgical indication has not been decides yet. Retrospectively, family research revealed that many members are suffering from neurofibromatosis ( grand father, father, 2 brothers, uncle and aunt)
Conclusions: Unique teaching point: in neonatal period the first diagnosis to raise when there is a nodular thickening of the bladder wall is rhabdomyosarcoma. Plexiform neurofibroma is a very rare diferential diagnosis . The incidence of Von Recklinghausen is 1 in 3000 living births, howeverurinary manifestations are rare, and exceptional in neonatal period.
Session Info:

Electronic Exhibits - Case Report (Radiographer)

Fetal Imaging / Neonatal

Radiographer Scientific Exhibits - Case Reports

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Significance of Bladder Debris on Ultrasound in Pediatric Outpatients

Nowariak Meagan, Kruc Rebecca, Ames Jeff, Murati Michael, Hoggard Eric, Dietz Kelly

More than just Urachal Remnants. Demystifying Radiologic Characterization of Urachal Anomalies

Hodes Aaron, Armenta Paul, Levin Terry

More abstracts from these authors:
Vitamin K deficiency with intracranial hemorrhage: let’s check the gallbladder

Biscaye Stephanie, Albertario Marco, Guesmi Myriam, Leloutre Beatrice, Occelli Aurelie, Boyer Corinne

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