Discussion:
Three type of NPHP were described : juvenile or type 1 NPHP which the symptoms start between 4 and 6 years and lead the patient to ESRD at 13 years or later, infantile NPHP or type 2 which lead the patient to ESRD before 2 years; and adolescent NPHP or type 3 where ESRD occurred at mean age of 19 years (13). Extrarenal syndromes are associated with NPHP in 10 to 20 %, including: cerebellar ataxia (Joubert syndrome), retinitis pigmentosa (Senior-Løken syndrome), mental retardation, cardiac malformation, situs invertus and many others (1). NPHP with situs invertus or congenital heart abnormalities occur mostly in infantile patients. The most common congenital heart defect in this setting is ventricular septal defect (12).
Patients with genetic disorders are potentially more susceptible to present organs or vascular abnormalities compared to general population (11). LSVC occurs in 0.3% to 0.5% of the general population and in 3% to 5% of the patients with congenital heart disease, (6-7). The development of the left anterior cardinal vein occurs a complete regression of the right SVC (7-11). Most commonly the LSVC allows blood to reach the right atrium through the coronary sinus. LSVC is usually asymptomatic and does not require treatment unless accompanied by other cardiac anomalies (6-7-8).
If central veins abnormalities do not rule out, central vein procedures could lead to serious complications (2). Ultrasonography and fluoroscopy have shown fewer complications and fewer catheters tip malposition compared to procedures done by blinded fashion (3-4-5). Peter .G and col described surgically placed left-sided subclavian CVC in a patient with a left-sided superior vena cava which caused a hemothorax; subsequently, an interventional radiologist placed a CVC in the left internal jugular vein under fluoroscopy (2).
Our patient presented two major difficulties: central vein abnormality which is: LSVC and, a thrombosed left jugular vein with thrombosed left brachiocephalic vein secondary to repeated short term catheter placement.
For these patients, ultrasound guidance for vein puncture and fluoroscopic guidance for the accurate position of the catheter should be mandatory to avoid major complications or CVC misplacement. (9-10-11).