Living Donor Liver Transplantation in the Absence of Inferior Vena Cava: A Case Report S. Hatipoglu, A. Olmez, D. Ozgor, C. Kayaalp, and S. Yilmaz ABSTRACT Because of difficulties in the supply of cadaveric organs, of living donor liver transplantations are performed in increasing numbers. Congenital hepatic fibrosis associated with fibrosis and atrophy of the inferior vena cava were present in a potential recipient of living donor liver transplantation. This case report documented living donor liver transplantation as a treatment modality for a patient with absence of the inferior vena cava due to chronic liver failure. IVER transplantation is the only therapeutic option for chronic liver failure affording patients extended survival with an excellent quality of life.
CASE REPORT A 21-year-old man was admitted with chronic liver failure due to progressive congenital hepatic fibrosis with an absence of the inferior vena cava. A prior diagnosis of congenital hepatic fibrosis had been established by gastroenterologists 5 years prior. The patient’s general condition was progressively deteriorating requiring intensive care unit care. On admission, the laboratory values were poor with a Model for End-stage Liver Disease (MELD) score of 21 and a Child-Pugh score of C-11. Computed tomographic (CT) images of the thoracic and abdominal regions showed the absence of a retrohepatic inferior vena cava, with pronounced dilatation of the paravertebral venous plexus as well as of the cutaneous, iliac, hemiazygos, and azygous veins (Fig 1). The right lobe living donor was the 47-year-old mother. At laparotomy, the liver was fibrotic, hard nodular with cirrhosis. During surgery we observed dilated paraesophageal, retroperitoneal, paravertebral, and perigastric varicose veins. During dissection of the retrohepatic area, we observed the retrohepatic inferior vena cava to be fibrotic and thrombosed (Fig 2), and removed it after total hepatectomy. The caudal inferior vena cava was dissected to the renal veins for an anastomosis. There was no blood flow from suprarenal inferior vena caval stump. During the clamping of the total inferior vena cava the collateral circulation maintained venous flow from the lower body. The splanchnic organs did not show anomalies of venous drainage; urine output was normal and there was no hemodynamic instability. The donor right lobe was implanted with a right hepatic vein end-to-side anastomosis to the suprahepatic inferior vena cava (Figs 2 and 3). The portal vein and the right hepatic bile duct were joined end-to-end to the recipient structures. Right liver graft implantation was performed uneventfully without a retrohepatic vena cava replacement (Fig 3). He was discharged after 18 days in the hospital. The patient is alive and well at 5 months postoperatively.
In Turkey, organs from deceased donors remain scarce. Thus, potential liver transplant recipients who do not have a living donor may die while awaiting a cadaveric donor. Since the first successful operation in 1989, living donor liver transplantation has become an important choice of treatment for patients with end-stage liver diseases. Living donor liver transplantation was expanded to adult patients in 1994, but applicability has been limited by the risk of small-for-size grafts. Finally, procurement of a right lobe graft from a living donor has been developed as an option to prevent the problem of graft size among older pediatric and adult patients. The normal inferior vena cava is composed of four segments: hepatic, suprarenal, renal, and infrarenal. Malformations or anomalies of thoracic or abdominal veins are not uncommon, whereas interruptions of the inferior vena cava with azygos or hemiazygos continuation have a reported prevalence of ⬃0.5%.1 The Absence of an inferior vena cava is an uncommon but well-recognized anomaly. Anomalies of the inferior vena cava have been described more frequently (0.6% to 2%) than other cardiovascular defects, and less frequently among otherwise healthy individuals. Various anomalies of the inferior vena cava have been described including complete or partial absence or presence of bilateral inferior vena cavae.2 From the Department of General Surgery and Liver Transplantation Unit, School of Medicine, Inonu University, Malatya, Turkey. Address reprint requests to Sinan Hatipoglu, MD, Assistant Professor, Department of General Surgery and Liver Transplantation Unit, School of Medicine, Inonu University, Malatya 44000, Turkey. E-mail: [email protected]
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0041-1345/–see front matter http://dx.doi.org/10.1016/j.transproceed.2012.05.053
Transplantation Proceedings, 44, 1761–1763 (2012)
HATIPOGLU, OLMEZ, OZGOR ET AL
Fig 1. Multirow detector computed tomographic venous angiography indicated the absence of the perihepatic inferior vena cava, whereas the venous blood is drained into the superior vena cava via the vena azygos and other venous collaterals.
In adults, changes in the inferior vena cava have been reported as inccidental findings during radiologic examinations or laparotomy. Patent paraumbilical and subcutaneous abdominal veins are found frequently as collaterals in patients with portal hypertension namely due to liver cir-
rhosis. Regarding the vascular anomaly, the most important adjuncts for diagnosis are obtained by venous angiography and thoracoabdominal CT-scan that with intravenous contrast enhancement is the method of choice to diagnose this venous anomaly.
Fig 2. Retrohepatic inferior vena cava was fibrotic and thrombosed (white arrows), as were the suprahepatic inferior vena cava (long black arrow) and portal vein (short black arrow).
LIVING DONOR LIVER TRANSPLANTATION
Fig 3. Multirow detector computed tomographic venous angiography at the 8th postoperative day. Right graft hepatic vein were joined end-to-side to the suprahepatic inferior vena cava (black arrow).
Thrombosis of the inferior vena cava is a rare condition that can result from various underlying diseases, including hypercoagulability, tumor obstruction interruption by filter or surgery, trauma, inflammation or infection as well as idiopathic causes.3 Inferior vena cavae occlusion due to benign causes poses a different clinical challenge; maintenance of inferior vena cavae patency and symptom relief must be extended over many years. The importance of recognizing this disease is, mainly, to avoid unnecessary surgical interventions. There are reports of even inadvertent ligatures of venous collaterals leading to patient death.4,5 Endoluminal recanalization and stent placement in a chronically occluded inferior vena cava without chronic liver diseases show good intermediate-term outcomes and should be considered among symptomatic patient who do not have an adequate alternative therapy.3 In our case, the patient was asymptomatic during his childhood and thus did not undergo a radiographic examination. So, there is controversy as to whether absence of the inferior vena cava was a true embryonic anomaly or the result of peri- or postnatal inferior venacavae thrombosis due to congenital hepatic fibrosis causing regression and disappearance of a once present inferior vena cava. The
only cases reported in the literature have been due to congenital absence of the inferior vena cava. If the patient is hemodynamically stable, there is neither congestion in the lower extremity nor anomalous venous drainage from the splanchnic organs. Urine output was normal throughout the inferior venae caval clamping with vena caval occlusion due to sufficient venous collateral circulation. Liver graft implantation was performed uneventfully without retrohepatic vena cava replacement or a need to provide continuity of the inferior vena cava. REFERENCES 1. Guenther J, Marrouche N, Ruef J: Ablation of atrial flutter by the femoral approach in the absence of inferior vena cava. Europace 9:1073, 2007 2. Iqbal J, Nagaraju E: Congenital absence of inferior vena cava and thrombosis: a case report. J Med Case Reports 2:46, 2008 3. Razavi MK, Hansch EC, Kee ST, et al: Chronically occluded inferior venae cavae: endovascular treatment. Radiology 214:133, 2000 4. Effler DB, Greer AE, Sifers EC: Anomaly of the vena cava inferior; report of fatality after ligation. JAMA 146:1321, 1951 5. Sandercoe GD, Brooke-Cowden GL: Developmental anomaly of the inferior vena cava. ANZ J Surg 73:356, 2003