ESPN 50th Annual Meeting

ESPN 2017


 
CO-EXISTANCE OF INTERRUPTED INFERIOR VENA CAVA SYNDROME AND AUTOSOMAL RECESSIVE POLYCYSTIC KIDNEY DISEASE: CASE REPORT
OZDE NISA TURKKAN 1 DENIZ OZBAY 1 ZEYNEP YURUK YILDIRIM 1 ZUHAL BAYRAMOGLU 2 ALEV YILMAZ 1 AHMET NAYIR 1

1- ISTANBUL UNIVERSITY ISTANBUL FACULTY OF MEDICINE PEDIATRIC NEPHROLOGY DEPARTMENT
2- ISTANBUL UNIVERSITY ISTANBUL FACULTY OF MEDICINE PEDIATRIC RADIOLOGY DEPARTMENT
 
Introduction:

 Interrupted Inferior Vena Cava Syndrome (IVCS) is a rare vascular anomaly.   Although it usually presents with other congenital anomalies, there is no report about its co-existing with autosomal recessive polycystic kidney disease (ARPKD).

Material and methods:

 Here we present a 4-month-old girl with ARPKD and IVCS.

Results:

 Four month-old female patient presented to our clinic due to vomiting which started in the neonatal period but intensified gradually.  History of small for gestational age and consanguineous marriage were noted. Growth retardation, tachycardia (140/min), 2/6 systolic murmur, severe hypertension and end-stage renal disease were detected in her physical and laboratory examination.  
Interrupted inferior vena cava, which continued as a dilated azygos vein to the superior vena cava was detected on abdominal ultrasonography. Left isomerism and polysplenia were found as the component of IVCS. The liver parenchymal echogenicity had risen in accordance with congenital hepatic fibrosis. Bilateral grade 2 hyperechogenicity and multiple ubiquitous millimetric anechoic cysts were seen in renal parenchyma.  Echocardiography revealed left ventricle wall and intraventricular septum hypertrophy as a result of chronic hypertension. Cranial ultrasound was normal. Peritoneal dialysis and the relevant medical therapy for end stage renal disease and hypertension has been arranged.

Conclusions:

 Interrupted inferior Vena Cava Syndrome may be co-exist with ARPKD. Patients with ARPKD should be evaluated in terms of other congenital anomalies.