Living donor liver transplantation for biliary atresia complicated by situs inversus: Technical highlights

Authors


Abstract

Living-donor liver transplantation (LDLT) has become an established technique to treat children with end-stage liver disease. Biliary atresia (BA), one of the most common indications for liver transplantation in children, can be associated with situs inversus (SI). In the past, the presence of SI has been considered to be an absolute contraindication for liver transplantation because of the technical difficulties. Recently, some reports of successful diseased-donor liver transplantation in patients with BA complicated by SI have been published; however, few reports of that with LDLT exist. The technical difficulties involved with LDLT for such cases have not been described. Herein, we present 4 successful cases of LDLT for BA with SI. Complex anomalies associated with SI, such as a hepatic artery arising from the supraceliac aorta, a preduodenal portal vein, and absence of the retrohepatic inferior vena cava, increase the technical difficulties involved with the operation. Additional caution is required in LDLT because a living-donor graft has short vessels and the availability of vascular grafts from the donor is limited. In conclusion, LDLT for BA complicated by SI can be managed successfully with technical modifications and scrupulous attention. This series represents the largest reported group of patients with BA complicated by SI who underwent a successful LDLT procedure. (Liver Transpl 2005;11:1444–1447.)

Situs inversus (SI) is a condition characterized by a mirror image orientation of the abdominal and thoracic viscera relative to the midline. It includes one or more of the following: polysplenia, intestinal nonrotation, preduodenal portal vein, aberrant hepatic arterial supply, and absence of the retrohepatic inferior vena cava. SI is a rare anomaly, with a frequency reported to be between 0.002% and 0.1%.1 Interestingly, association with biliary atresia (BA) occurs in up to 28% of children with SI.2

Although BA is one of the most common indications for liver transplantation, patients with BA complicated by SI have been considered highly questionable candidates because of the technical difficulties.3 However, several reports of successful diseased-donor liver transplantation (DDLT) in patients with BA complicated by SI have been published recently.4–6

Living-donor liver transplantation (LDLT) has become a standard option for pediatric patients. However, there are few reports of a successful use of a living-donor graft for patients with BA and SI. Herein, we present 4 cases of LDLT performed for BA complicated by SI and discuss the necessary operative management, especially technical highlights, for an SI recipient undergoing such liver transplantation.

Abbreviations

LDLT, living-donor liver transplantation; BA, biliary atresia; SI, situs inversus; DDLT, diseased-donor liver transplantation; HA, hepatic artery; PV, portal vein; IVC, inferior vena cava; LUQ, left upper quadrant; HV, hepatic vein; V2, vein from segment 2; V3, vein from segment 3.

Patients and Methods

Between June 1990 and June 2004, 1,000 LDLT procedures were performed at Kyoto University, of which 613 were performed on children (younger than 18 years). For all 1,000 LDLT procedures, 415 were for BA and 4 were for BA with SI. All candidates previously underwent a Kasai operation. The diagnosis of SI was established at presentation using radiography and confirmed during surgical exploration, which was performed prior to LDLT.

The entire operative procedure has been described elsewhere.7 For the donor operation, a left lateral segment graft was used for three cases. After isolation of the left hepatic artery, hepatic duct, and portal branch in the donor, a hepatic parenchyma of the medial segment was transected 5 mm to the right of the falciform ligament without blood inflow occlusion or graft manipulation. A reduced monosegmental graft method, which was recently introduced for small infants to mitigate the problem of large-for-size graft, was used for one case. Briefly, in the recipient operation, following isolation of the hepatic artery (HA) and portal vein (PV), the liver was dissected from the inferior vena cava (IVC) by ligation and dissection of the short hepatic veins without IVC clamping. After dissection and closure of the right hepatic vein, a total hepatectomy was completed after side-clamping of the IVC to maintain caval blood flow. The liver graft was implanted into the left upper quadrant (LUQ). For vascular reconstruction, we usually use 5-0 Prolene (continuous) for the hepatic vein (HV), 6-0 Prolene (continuous) for the PV, and 8-0 Prolene (interrupted) for the HA. For biliary reconstruction, a choledochojejunostomy with a Roux-en-Y anastomosis was performed in all patients. Full-layer abdominal closure was performed in each. Posttransplant immunosuppression consisted of tacrolimus and low-dose steroids.7

Results

Patient profiles during the pre- and posttransplant periods as well as those during the operation are summarized in Tables 1 and 2.

Table 1. Patient Profiles During the Pre- and Posttransplant Periods
Patient No.AgeWeight (kg)Pretransplant Operative NumbersAnatomical AbnormalitiesGRWR (%)DonorBlood Type CombinationGraft TypeComplicationsICU StayHospital StayFollow-up Period
  1. Abbreviations: GRWR, graft-to-recipient weight ratio; IVC, inferior vena cava; PV, portal vein; PHA, proper hepatic artery; S2, segment 2; S3, segment 3.

15 yr155Polysplenia1.56FatherIdenticalS2+3PV stenosis5 days77 days10 yr 10 months, alive
    Absence of IVC        
    Preduodenal PV        
    PHA directly from aorta        
22 yr10.52Polysplenia2.28MotherIdenticalS2+3Small intestinal perforation4 days153 days3 yr 11 months, alive
    Malrotation    PV stenosis   
    Left-sided IVC        
    Preduodenal PV        
    PHA directly from aorta        
    Dextrocardia        
31 yr 6 months7.21Absence of IVC3.1MotherIdenticalS2+3None5 days44 days3 yr 11 months, alive
49 months3.63Malrotation3.27MotherIdenticalReduced monosegmentNone22 days141 days6 months, alive
Table 2. Patient Profiles During the Operation
Patient No.Arterial AnastomosisHepatic Vein AnastomosisPortal Vein AnastomosisWarm Ischemic Time*Operation Time
DonorRecipientDonorRecipientDonorRecipient
  • Abbreviations: LHA, left hepatic artery; PHA, proper hepatic artery; RHA, right hepatic artery; V2, hepatic vein from segment 2; V3, hepatic vein from segment 3; LPV, left portal vein. All bile duct reconstruction procedures were performed using a Roux-en-Y choledochojejunostomy.

  • *

    Warm ischemic time was defined as the time from implantation of the liver graft to reperfusion of the portal flow.

1LHAPHALHVHepatic venous cloacaLPVPV trunk with interposed graft56 min14 hr 35 min
2LHAPHAV2Anatomical RHVLPVPV trunk with interposed graft50 min16 hr 22 min
   V3Anatomical LHV+MHV    
3LHARHALHVHepatic venous cloacaLPVPV trunk with branch patch method35 min10 hr 30 min
4LHAPHALHVHepatic venous cloacaLPVPV trunk50 min9 hr 36 min

Case 1

A boy underwent a Kasai operation 43 days after birth, which was followed by revision of the hepatic hilum, because of persistent cholangitis. Liver transplantation was scheduled for the deteriorating liver function as well as variceal bleeding at the age of 5 years old. After a hepatectomy, the liver was transplanted in the usual manner, and an interposed graft taken from the inferior mesenteric vein of his father was used for PV reconstruction. The posttransplant course was uneventful. Yearly follow-up revealed PV stenosis 9 years after transplantation, which was shown to be a complete obstruction with portography.

Case 2

A 2-year 4-month-old girl presented with progressive hepatic failure for LDLT. She had a history of BA and underwent a Kasai operation at the age of 2 months. At that time, the patient was noted to have SI with dextrocardia, polysplenia, intestinal malrotation, and a preduodenal portal vein. A left lateral segment graft from her mother was placed in an LUQ. The graft had two independent veins from segments 2 (V2) and 3 (V3), thus two anastomoses were made individually between graft V2 and the common anastomotic stump of the middle and left hepatic veins of the recipient, and between graft V3 and the right hepatic vein of the recipient. An interposed graft taken from the ovarian vein of the donor was used for PV reconstruction. The immediate postoperative course was complicated by a small intestinal perforation, whereas the later course was further complicated by PV stenosis two years after transplantation.

Case 3

A 1-year 6-month-old girl with a history of BA presented with progressive hepatic failure. The patient had undergone a Kasai operation at the age of 8 months, at which time she was noted to have SI. LDLT was performed using a lateral segment graft from her mother. After the hepatectomy, the liver was transplanted in the usual manner, and a branch patch method was used for PV reconstruction. The posttransplant course was uneventful.

Case 4

A girl underwent a duodenocolonic dissociation (LADD) procedure and a duodenoduodenostomy for malrotation of the intestine and duodenal atresia at the age of 9 days, at which time she was noted to have SI and dextrocardia. At the age of 7 months, liver dysfunction and jaundice were pointed out, and she underwent exteriorization of the gall bladder, which was followed by a Kasai operation, because of persistent jaundice. Liver transplantation was scheduled for the deteriorating liver function at the age of 9 months. LDLT was performed using a reduced monosegmental graft from her mother. After a hepatectomy, the graft was placed in an LUQ. The retrohepatic IVC was absent, and the HV drained directly through the diaphragm into the right atrium. There was a discrepancy in diameter between the graft LHV and recipient HV, therefore, the anastomosis was performed end-to-end using an interrupted suture with 5-0 Prolene. Other reconstruction was performed in the usual manner. Her intensive care unit stay was extended by a respiratory disorder; however, there were no major complications during the postoperative course.

Discussion

Anatomical variations, especially vascular anomalies, increase the technical difficulties of liver transplantation and previously resulted in a high rate of mortality. Therefore, the technical aspects of performing liver transplantation in the present group of patients was more challenging than simply overcoming the problem of a mirror-image anatomy of the liver. Raynor et al.4 was the first to report a successful DDLT procedure for a patient with SI, and later Farmer et al.5 reported the results of DDLT for BA with SI in 6 patients. They concluded that the presence of SI requires technical modifications but does not significantly change the outcome.

There are only three reports of LDLT for these patients so far, despite the fact that the procedure has become a standard option for patients with end-stage liver disease.6, 8, 9 The technical difficulties of performing LDLT for BA with SI have not been fully elucidated.

In the LDLT for the patient with an absence of the retrohepatic IVC, the direction and diameter of the graft HV and the recipient suprahepatic cava are important factors for successful HV reconstruction. We added two modifications to the usual end-to-end anastomosis between the donor HV and recipient suprahepatic cava: (1) to prevent torsion and keep the direction of the HV anastomosis, the falciform ligament was fixed to the abdominal wall, and Doppler ultrasonography was used for real-time evaluation of the graft blood supply at each stage of the abdominal closure; and (2) to overcome the size discrepancy of anastomotic orifices, an interrupted suture method was used, which was the first time for us.

A preduodenal PV, which was the predominant anomaly in patients with SI, can be hazardous unless properly recognized during a hepatectomy. Most recent reports have noted that if a preduodenal PV is sufficiently mobilized and not injured, the anomaly rarely interferes with a standard end-to-end anastomosis.4, 5, 8 In LDLT, PV reconstruction was also successfully performed with an end-to-end anastomosis, and preduodenal reconstruction was feasible and effective. Reconstruction of the PV in preduodenal position makes the PV straight and prevents kinking.10 Since the graft from a living donor has a short PV and the recipient PV is quite sclerotic and small in BA cases, due to cholangitis or previous multiple laparotomies, vascular grafts for the PV reconstruction might be useful to overcome the problems. In addition, use of an interrupted suture for PV reconstruction in cases presenting a diameter-size discrepancy may contribute to preventing postoperative stenosis.

An aberrant HA supply is one of the most frequent anatomic variations seen in children with SI. Mattei et al.6 found an aberrant HA supply, which included replaced and accessory HA branches, in 35% of patients with SI who had undergone liver transplantation. The majority of the variations reported were an HA originating from the superior mesenteric artery, a left HA originating from the left gastric artery, and a right HA originating from the celiac artery.3, 6, 8 In our series, a proper HA was found arising directly from the supraceliac aorta in 3 of the 4 cases, however, we found only a single report of a patient with the same type of HA anomaly.5 In LDLT, it is important to prevent damage when recipient HA dissection is performed during the hepatectomy because the artery of the hepatic graft is often short.

In LDLT for children, a lateral segment graft or left lobe graft is generally used.7 However, in small infants, implantation of left lateral segment grafts can be a problem because of the large-for-size graft. To relieve the problem of large-for-size grafts in small infants, monosegmental LDLT and reduced monosegmental LDLT were recently performed by our group.10 The indication for using these techniques was infants with an estimated graft-to-recipient weight ratio of greater than 4.0%. During those procedures, the segmental graft was safely placed in the left subphrenic space, and a suitable orientation was obtained for anastomosis of the hilar vessels. In these cases, a partial liver graft from a living-donor graft or split liver graft appears to be an optimal choice.

The present series represents the largest reported group of patients with BA complicated by SI who underwent successful LDLT. Complex anomalies associated with SI increase the technical difficulties of the operation. Additional caution is required when performing LDLT because a living-donor graft has short vessels and the availability of vascular grafts from the donor is limited. However, LDLT for BA complicated by SI can be managed successfully with technical modifications and scrupulous attention.

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