This patient was a 42-year-old previously healthy white man with a one-year history of alcoholic/hepatitis C cirrhosis complicated by refractory ascites, encephalopathy, spur cell anemia, and hepatorenal syndrome. He had already been listed for liver transplantation with a model for end-stage liver disease score of 39 when a liver became available as part of a directed donation from the patient’s recently deceased aunt. The donor liver was recovered from a 64-year-old white woman who met brain death criteria after an acute, spontaneous subarachnoid hemorrhage. Before her vascular event, her medical history was significant for Ehlers-Danlos syndrome (EDS), multiple hip dislocations, and dysplasia of bilateral acetabula and femurs. Biopsy of the liver allograft showed little fat and normal parenchyma. Liver function tests were normal at the time of recovery. After organ recovery, several small tears in Glisson’s capsule were observed; nevertheless, the liver was judged of sufficient quality to proceed with transplantation. The recipient underwent hepatic transplantation using the standard piggyback technique. After removal of the vascular clamps, the allograft began to bleed freely from the aforementioned capsular avulsions. Packing these areas caused additional dissection of the parenchyma and further bleeding; topical procoagulants were ineffective. It was deemed that further attempts at hemostasis were becoming counterproductive. The right upper quadrant was packed and the patient was closed with the anticipation of a second look the next day. Overnight, he developed an abdominal compartment syndrome and returned to the operating room for exploration and repacking and further attempts at hemostasis, including a range of procoagulants and the creation of a Vicryl mesh liver wrap. On postoperative day two, the patient returned to the operating room for continued bleeding and the judgment was made that the liver hemorrhage was nonrecoverable. A graft hepatectomy with portocaval anastomosis was performed and the patient was emergently relisted for transplantation. Approximately 24 hr later, a normal-appearing liver from a 43-year-old female donor became available and he was retransplanted. There were no further bleeding complications, but he required a third transplant six days later when the second liver failed secondary to primary nonfunction. His third and final transplant functioned well. He was discharged to a rehabilitation facility in good condition and continues to do well with normal liver and kidney function 1 yr after discharge. EDS is a heterogeneous group of heritable disorders of connective tissue characterized by skin hyperextensibility, joint hypermobility, and tissue fragility. Vascular EDS, also known as EDS type IV or Sack-Barabas syndrome, is caused by a defect in the COL3A1 gene, which codes for type III collagen (1). Vascular EDS is the only form of EDS associated with a high risk of early death resulting from arterial or visceral ruptures (2, 3). Although rare in comparison to vascular catastrophes, spontaneous rupture of the liver in a patient with EDS type IV has been reported (4). On one previous occasion, spontaneous rupture of a transplanted liver from an EDS donor occurred in a similar fashion as the case described in this report (5). Attempts to identify documentation of the donor’s EDS subtype were unsuccessful, but it is reasonable to suspect type IV EDS in her case. It is unknown how many successful transplants have taken place using organs from patients with EDS; however, the authors strongly encourage caution in accepting organs for transplant from a donor with a known connective tissue disease. Brian R. Swenson Hilary A. Sanfey Timothy L. Pruett Department of Surgery Division of Transplantation University of Virginia Health System Charlottesville, VA
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Oct 1, 2009
Adriana Prada Ruiz + 4
Open Access