The presence of intrahepatic vascular and ductal variants in the liver of a suitable donor should not be considered a contraindication to living liver donation. When a suitable living donor is available, a good understanding of such anomalies and meticulous surgical technique make living donor liver transplantation one of the best therapeutic options, especially when recipients are disadvantaged by their weight range in terms of long waiting times for cadaveric donation.
We present a case of a complex donor anatomy during a right lobe living donor transplant performed to treat a patient with hepatic epithelioid hemangioendothelioma. This is a rare, low-grade vascular malignancy whose therapeutic algorithm is far from standardized.[1, 2] As demonstrated by different authors,[3-6] liver transplantation is a valuable treatment for hepatic epithelioid hemangioendothelioma, even in cases of extrahepatic metastasis. In our case, the 26-year-old donor and the 26-year-old recipient were twins (brother and sister, respectively). The recipient presented with obstructive jaundice and abdominal pain. The computed tomography scan showed multiple, large, central, and peripheral liver lesions suspected to be hemangioendotheliomas. She was stented via endoscopic retrograde cholangiopancreatography, and the histological examination confirmed the radiological diagnosis. An attempt at removing the stent 6 months after its positioning failed because of multiple intrahepatic strictures. She deteriorated with progressive jaundice and weight loss (44 kg) and had a body mass index of 16 kg/m2 before transplantation. Her Model for End-Stage Liver Disease score, though not representative of her deteriorating clinical condition, had progressed from 7 at listing to 14 (UK End-Stage Liver Disease score = 50) just before transplantation. Her twin brother, a fit and healthy male weighing 68 kg (body mass index = 23 kg/m2), came forward as a possible donor. He was blood group–identical (O Rh+) and had excellent liver function. However, his liver had several anatomical anomalies. The portal vein had a type 4 variation: a segment VIII portal vein branch (6.2 mm in diameter) originating from the distal main left portal vein (Fig. 1). A large vein (7 mm in diameter), draining segment VII, joined the inferior vena cava just inferiorly to the main right hepatic vein; another vein (4 mm in diameter) was identified as an accessory low inferior right hepatic vein. An intraoperative cholangiogram showed a right posterior bile duct draining into the main left bile duct (Fig. 2). The artery to segment VIII arose from the right system, so there was no concern about arterial ischemia for this segment after graft removal. On the back table, the graft was prepared as follows:
- The segment VIII portal branch was detached from the left portal branch origin and reconstructed with the right portal vein into a single vein orifice, which was elongated with a fresh cadaveric, ABO-identical iliac vein interpositional graft.
- Venoplasty between the right hepatic vein and the vein draining segment VII was first performed, and this was followed by quilt plasty using an ABO-identical, cadaveric iliac vein sutured around the common venous orifice.
- The stumps of the branches of the middle hepatic vein draining segments V and VIII (V5 and V8), were reconstructed with a fresh cadaveric, ABO-identical iliac artery.
Implantation was performed through the creation of an extended midline orifice in the recipient's inferior vena cava, which accommodated the venoplasty patch of the graft. The reconstructed right portal vein and the right hepatic artery were regularly anastomosed with 6/0 and 7/0 Prolene sutures, respectively. The 2 ducts were drained with a Roux-en-Y loop because the extent of the tumor in the recipient precluded the use of the recipient's ducts. Postoperatively, the donor presented with a right pleural effusion of no clinical relevance. He was discharged 6 days after surgery and was free from vascular or biliary complications at 4 months' follow-up. The recipient immediately had excellent liver function, and regular Doppler ultrasound examinations showed patency of the reconstructed vessels, which was confirmed with a computed tomography scan (Figs. 3 and 4) at discharge 11 days after transplantation.