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.