Introduction:
Nephronophthisis (NPHP) is an autosomal recessive disease that affects pediatric population between the first and the second decade. The kidney effect is characterized by a chronic tubulointerstitial nephritis leading to end stage renal disease. Many syndromes could be associated with NPHP including: cerebellar ataxia (Joubert syndrome), retinitis pigmentosa (Senior-Løken syndrome), mental retardation, cardiac malformation, situs invertus and many others (12).
For these patients, congenital vascular abnormalities are diagnosed fortuitously during a central vein catheter placement. The procedure could present unusual difficulties as a guide wire misplacement, vein perforation or catheter dysfunction.
We reported a case of a 13 years child in ESRD affected by NPHP with dextrocardia, left superior vena cava (LSVC) and left azygos vein where the diagnostic of theses anatomical abnormalities was done by fluoroscopy with contrast injection after encountering unusual difficulties to perform a hemodialysis catheter.