Rare Case of a Kidney and Inferior Vena Cava Abnormalities With
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Clinics and Practice 2018; volume 8:1079 Rare case of a kidney and inferior vena cava Case Report Correspondence: Timir K. Paul, Division of Cardiology, Department of Medicine, East abnormalities with extensive A 27-year-old male with no significant Tennessee State University, 329 N State of lower extremity deep vein past medical history was admitted with Franklin Rd, Johnson City, TN 37604, USA. right lower extremity pain and swelling that Tel.: 423.979.4100 - Fax: 423.979.4134. thrombosis in a young started a week ago. Patient first noted E-mail: [email protected] healthy male swelling in the right ankle that was associ- ated with pain in the calf and thigh. Pain Key words: Deep vein thrombosis; Inferior vena cava hypoplasia; Renal hypoplasia. was described as progressively worsening Muhammad Khalid,1 1 1 with tightness and heaviness in the affected Contributions: MK wrote the initial manu- Manisha Nukavarapu, Rupal Shah, leg that was exacerbated with ambulation. 2 script draft and literature review; MN and RS Timir K. Paul Patient endorsed recent short duration of reviewed the initial draft with editions and TK 1Department of Medicine; 2Division of immobility involving a 4 hour road trip but did final revision and any edition as needed. Cardiology, Department of Medicine, denied any recent surgery, prior history of thrombosis, weight changes, new bone pain, Conflict of interest: the authors declare no East Tennessee State University, potential conflict of interest. Johnson City, TN, USA or history of easy bruising or bleeding. No known history of any congenital abnormal- Funding: none. ities, intra uterine growth restriction, oligo- hydramnios and no pregnancy related com- Received for publication: 6 May 2018. plications was reported. Revision received: 24 September 2018. Abstract Vitals on admission included blood Accepted for publication: 25 September 2018. pressure B.P 133/98 mm Hg, pulse rate of Kidney and inferior vena cava (IVC) This work is licensed under a Creative abnormalities with extensive deep vein 100 beats per minutes, respiratory rate 18 Commons Attribution NonCommercial 4.0 thrombosis (DVT) is a very rare cause of per minute, temperature of 97.8 F, and nor- Licenseonly (CC BY-NC 4.0). DVT and has a diverse clinical presentation. mal oxygen saturation on room air. Physical Computed tomography (CT) angiography is examination was significant for right lower ©Copyright M. Khalid et al., 2018 extremity swelling most prominent at the Licensee PAGEPress, Italy the gold standard for diagnosis and treat- thigh with mild erythema and calf tender- Clinics and Practice 2018; 8:1079 ment including thrombectomy, thromboly- ness. Laboratory showed normal bloodusedoi:10.4081/cp.2018.1079 sis and systemic anticoagulation. We pres- counts, liver and kidney functions. ent a rare case of active young healthy male Patient initially received full dose of admitted with acute onset of right lower Lovenox that was switched to low molecu- extremity pain and swelling who was found lar weight heparin infusion. CT scan of anticoagulation. Patient was found to have to have extensive DVT on doppler ultra- chest, abdomen and pelvis was obtained to extensive thrombosis of the pelvic veins on sound. CT abdomen showed extensive clot evaluate potential etiology of thrombosis angiogram. Based on CT scan and burden involving right common femoral and extent of DVT. CT scan ruled out pul- angiogram findings, our patients has con- vein extending into internal and external monary embolus and revealed an extensive genital anomaly of inferior vena cava with iliac veins associated with IVC hypoplasia DVT of the right common femoral vein, left no intrahepatic portion of the IVC, and and hypoplastic left kidney. Patient under- common iliac vein, left internal and external hepatic veins drain into the azygous venous went urgent thrombectomy, catheter direct- iliac veins. There was an incidental finding system which continues into the superior ed thrombolysis and was discharged home of congenital inferior vena cava (IVC) vena cava which can sometimes mimic with in stable condition on oral anticoagulation. anomaly, with no intrahepatic portion of chronic inferior vena cava occlusion with inferior vena cava, and a hypoplastic left venous thrombosis usually associated with kidney (Figure 1A-C). Further evaluation of significant venous collaterals. The patient Non-commercialthe right lower extremity with doppler ultra- was subsequently treated with direct throm- Introduction sound revealed acute thrombus occluding bolysis, mechanical thrombectomy and bal- the common femoral, profunda femoral and loon angioplasty (Figure 3A-C). Post Kidney and inferior vena cava abnor- popliteal veins (Figure 1D). Left lower thrombolysis catheter showed contrast flow malities with leg thrombosis (KILT) syn- extremity doppler study was negative for from left iliac vein to right iliac vein and drome is most commonly seen as an inci- DVT. CT scan showed continuation of azy- lumbar vein with no inferior vena cava seen dentally finding on imaging studies espe- gous vein into the superior vena cava via (Figure 3D). Oncology recommended life- cially in a young population. It is a rare azygous arch which has been highlighted in long anticoagulation because of unpro- cause of deep vein thrombosis with very detail in (Figure 2A-E). There was no sig- voked DVT, extent of thrombosis with mul- few case reports in literature so far. We nificant venous collaterals found on CT tiple risk factors including IVC abnormali- present a case of 27-year-old young healthy scan after discussion with the radiologist. ties. Patient was started on rivaroxaban on discharge and instructed to use elastic male admitted with right leg pain who was Hypercoagulable work up was ordered. stockings, and to avoid excessive exercise, found to have extensive deep vein thrombo- Factor V Leiden, prothrombin mutation and immobilization and smoking. sis (DVT) of right lower extremity extend- anticardiolpin antibody were negative. ing into the common femoral vein, pelvic Testing for protein C, protein S, antithrom- and abdominal veins with kidney and infe- bin III, lupus anticoagulant was deferred rior vena cava abnormalities. because of difficulty of interpretation of Discussion these results in the setting of an acute venous thromboembolism and systemic KILT syndrome was first reported by [page 95] [Clinics and Practice 2018; 8:1079] Case Report Van Veen et al.1 It is a triad of kidney and IVC abnormalities and leg thrombosis. Only few case report studies have been reported in the literature. Inferior vena cava hypoplasia is an uncommon congenital cause of ilio-femoral DVT especially in young patients, accounting for 5% cases of DVT under 30 years old2 and having a prevalence rate of % in the general popula- tion.3 IVC embryogenesis is very complex and involves confluence of three sets of the fetal veins, the posterior cardinal, sub cardi- nal, and supracardinal veins. There are four different parts to the IVC, which are hepat- ic, supra renal, renal and infra renal. The possible etiology of IVC atresia/hypoplasia is felt to be due to intrauterine or perinatal thrombosis of IVC, resulting in occlusion and disappearance or hypoplasia of IVC.4 The most common congenital anomalies are duplication of IVC, IVC continued with intra thoracic azygous vein, left sided IVC, IVC agenesis and retro aortic left renal vein. IVC abnormalities have a reported preva- lence of 0.07-8.5% in the general popula- only tion. IVC hypoplasia/agenesis usually Figure 1. (A-C) Computed tomography scan of the abdomen showed absent intra-hepat- results in the extensive collateral formation ic portion of IVC and confluence of hepatic veins, hypoplastic left kidney and absent both hepatic and renal portions of the inferior vena cava (arrows). (D) Doppler ultra- which results in the slow venous flow from sound clot in the deep veins of right leg (arrow). the lower extremity ended up with the increased venous pressure, venous stasis use and recurrent DVT.4 Agenesia of IVC has already been reported in the literature to be associated with extensive DVT.5 Duciu et al.6 first reported a case of KILT syndrome in sib- lings. DVT can be either provoked or unprovoked. Factors that are involved in provoked DVT include recent surgery, pro- long immobilization, trauma, pregnancy, obesity, smoking, recent travel, lupus anti- coagulant, use of oral contraceptive pills and malignancy. If there are no obvious pre- disposing factors present, then DVT is most likely unprovoked which can be due to the absence of protein C, Protein S, antithrom- bin III, factor V/prothrombin geneNon-commercial mutation or congenital IVC anomalies. Most of the patients are asymptomatic prior to diagnosis and, if symptomatic, clinical presentation can be diverse including lower limb pain and swelling, loin pain, hematuria, back pain, and lower extremity ulcers.7 Pulmonary embolus is rarely seen with KILT syndrome, because the embolus is usually trapped in the azygous vein, which prevents propagation to the pulmonary cir- culation. The typical presentation of KILT syn- drome are in male patients presenting with extensive or bilateral DVT in the setting of physical exertion and associated with phys- ical exertion. Shaline et al. reported a case Figure 2. Computed tomography scan demonstrates azygous continuation from the of IVC atresia presenting with DVT associ- hepatic veins into the superior vena cava as continuation of the hepatic veins (arrows). ated in the setting of physical exertion.8 The [Clinics and Practice 2018; 8:1079] [page 96] Case Report DVT commonly involves distal IVC, com- have also been reported as seen in our different diagnostic modalities that may be mon, internal and external iliac and femoral patient.10 used to diagnose DVT. CT venography is veins. Our patient was physically very Usually, patients with unexplained or the gold standard to diagnose DVT but it active and CT scan showed DVT of the unproved DVT required hypercoaguable has been replaced by regular ultrasound due right common femoral vein through com- work up which could be the possible expla- to invasive in nature.