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Abstract

  1. Top of page
  2. Abstract
  3. PATIENTS AND METHODS
  4. RESULTS
  5. DISCUSSION
  6. ACKNOWLEDGMENT
  7. REFERENCES

The vascular anomalies encountered in patients with biliary atresia associated with polysplenia syndrome and situs inversus (SI) demand technical refinements when liver transplantation is being performed. The available data describing the technique used in living donor liver transplantation (LDLT) in this population are limited; the short vascular stumps of the donor's liver can impart additional technical difficulties during vascular reconstruction. Here we describe our experience with 9 children with biliary atresia and SI who underwent LDLT. In our series, the retrohepatic vena cava was absent for 7 patients, 7 had a preduodenal portal vein (PV), and 4 had a variant arterial anatomy. The donor's left hepatic vein was anastomosed to the confluence of the recipient's 3 hepatic veins in 7 patients. Vascular grafts were used for PV reconstruction in 3 cases. A left lateral segment graft was used in all but 1 patient who needed a graft reduction. All grafts were placed in the upper left abdomen. There were no vascular complications after transplantation. All patients were alive and well at a median follow-up of 55 months. In conclusion, LDLT can be successfully performed in pediatric patients with SI. Complex vascular anomalies associated with the use of partial liver grafts obtained from living donors are not associated with an increased occurrence of vascular complications. Liver Transpl 19:431–436, 2013. © 2013 AASLD.

Abbreviations
CHA

common hepatic artery

HA

hepatic artery

IJV

internal jugular vein

IMV

inferior mesenteric vein

IVC

inferior vena cava

LDLT

living donor liver transplantation

LGA

left gastric artery

LGC

lesser gastric curvature

LHA

left hepatic artery

LHV

left hepatic vein

LLS

left lateral segment

PHA

proper hepatic artery

PV

portal vein

RHV

right hepatic vein

RHVC

retrohepatic vena cava

SI

situs inversus

SMA

superior mesenteric artery

SMVJ

splenomesenteric vein junction.

Situs inversus (SI) is a rare condition characterized by the mirror-image orientation of the abdominal and/or thoracic viscera with respect to the midline, and it occurs in less than 0.005% of the general population.[1] Despite its rarity, biliary atresia may occur in up to 28% of infants born with SI, and most are candidates for liver transplantation.[2] This population of patients often presents with polysplenia syndrome, which includes a variety of anatomical anomalies, including cardiac disease, intestinal malrotation, a preduodenal portal vein (PV), an absence of the retrohepatic inferior vena cava (IVC), and an aberrant arterial supply to the liver.[3]

The indication of liver replacement in patients with end-stage liver disease and SI was questionable in the past, mainly because of the technical difficulties due to vascular anomalies and graft positioning. However, several reports have demonstrated the feasibility of liver transplantation with deceased donor grafts in these patients.[2, 4] Because of the shortage of pediatric deceased donors, the majority of liver transplants performed in young pediatric patients are performed with partial liver grafts, including grafts from living donors. Except for a case series of 4 patients published by the Kyoto group,[5] there are only anecdotal reports about living donor liver transplantation (LDLT) in recipients with SI. Here we present the largest series of patients with biliary atresia and SI who underwent LDLT, and we compare the operative technical aspects and complications with the available literature.

PATIENTS AND METHODS

  1. Top of page
  2. Abstract
  3. PATIENTS AND METHODS
  4. RESULTS
  5. DISCUSSION
  6. ACKNOWLEDGMENT
  7. REFERENCES

A retrospective review of all pediatric patients undergoing LDLT at Sirio-Libanes Hospital/A. C. Camargo Hospital (São Paulo, Brazil) was performed. Medical records were obtained so that we could characterize the clinical course and complications after liver transplantation and assess the preoperative and perioperative characteristics of the recipients. A single person was responsible for collecting the data from the charts and entering them into the computer database. This study was approved by the hospital's ethics committee.

The voluntary intent of the donor was first assessed, and informed consent was mandatory. In this study, all living donors were related to the recipients, and all underwent a psychological evaluation in order to rule out any psychological disturbances. The preoperative evaluation of the candidates and the operative technique used for the donor hepatectomy [left lateral segment (LLS)] have been described elsewhere.[6] After dissection of the vascular inflow to the liver, parenchymal transection was accomplished with a Cavitron ultrasound surgical aspirator (Cavitron, Stanford, CA) 0.5 to 1.0 cm to the right of the falciform ligament. The blood supply to each graft was kept intact until the recipient was ready for implantation to minimize the cold ischemia time. Intraoperative cholecystectomy and cholangiography were performed in order to determine the position at which to cut the left biliary tree. After the completion of the hepatectomy, the graft was flushed with histidine tryptophan ketoglutarate solution at 4°C and was prepared for implantation.

The preoperative evaluation of these patients with SI and biliary atresia was not different from the preoperative evaluation of patients with biliary atresia alone. The SI diagnosis was obtained after a clinical examination or an abdominal ultrasound investigation or from the surgical reports of children who previously underwent laparotomy. Standard procedures, including blood tests, upper gastrointestinal endoscopy, Doppler ultrasound examinations, and multiprofessional evaluations, were followed; during Doppler ultrasound examinations, the PV diameter was observed so that we could predict the use of vascular grafts during PV reconstruction (usually in patients with a PV diameter < 5 mm). The recipient hepatectomy did not differ from that for patients with situs solitus, and this included the positioning of the surgical team. After the recipient operation, a Doppler ultrasound scan was routinely performed on postoperative day 1 to evaluate vascular patency. Immunosuppression was based on tacrolimus (Prograf) and steroids. For the first month, the tacrolimus target level was 8 to 10 ng/mL. Patients were weaned from steroids after the third postoperative month in all cases. Because of the arterial anastomoses made with a microvascular technique, all patients with platelet counts higher than 50.000 platelets per microliter received an antiplatelet agent (dipyridamole) during the first 3 postoperative months.

RESULTS

  1. Top of page
  2. Abstract
  3. PATIENTS AND METHODS
  4. RESULTS
  5. DISCUSSION
  6. ACKNOWLEDGMENT
  7. REFERENCES

From October 1995 to October 2012, 493 LDLT procedures (477 primary transplants) were performed in patients less than 18 years of age at Sirio-Libanes Hospital/A. C. Camargo Hospital. We were able to identify 9 patients with SI who underwent LDLT, and all did so because of biliary atresia.

The recipients were children (6 males and 3 females) with ages ranging from 6 to 22 months (median = 11 months). The patients' weights ranged from 5.8 to 15 kg (median = 8.6 kg). Five patients had previously undergone the Kasai operation. In the other 4 patients, the diagnosis of SI was obtained via patient examinations and radiological tests.

As for the vascular anatomy, the retrohepatic vena cava (RHVC) was absent for 7 of the 9 patients, and another patient presented with a hypoplastic RHVC. The PV was in a preduodenal position in 7 of the 9 patients. A variant arterial anatomy was seen in 4 recipients; in 1 of these recipients, the common hepatic artery (CHA) arose directly from the aorta. For another patient, the main hepatic artery (HA) completely originated from the left gastric artery (LGA; Fig. 1A). Associated anatomical anomalies are summarized in Table 1.

image

Figure 1. (A) Vascular anomalies in a pediatric recipient with SI: preduodenal PV and CHA arising from the LGA at the LGC. (B) Reduced LLS graft in a recipient with SI and a very small abdominal cavity.

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Table 1. Patients With SI and End-Stage Liver Disease Undergoing LDLT: Clinical Characteristics and Outcomes
CaseSex/Age (Months)Graft TypeGraft-to-Recipient Weight Ratio (%)Anatomical AnomaliesHepatic Vein AnastomosisPV AnastomosisLength of Stay (Days)Intensive Care Unit Stay (Days)Follow-Up (Months)Complications/Outcomes
1Female/15LLS3.5Absent RHVCHepatic vein confluencePV trunk112110Alive and well
Preduodenal PV
Polysplenia
2Female/11LLS4.1Hypoplastic RHVCHepatic vein confluencePV trunk932583Respiratory complications in a recent postoperative period; alive and well
Preduodenal PV
Polysplenia
3Male/14LLS1.6Absent RHVCHepatic vein confluencePV trunk81078Intraoperative portal thrombosis (anastomosis redone); alive and well
Preduodenal PV
LHA from LGA
4Male/14LLS4.1Absent RHVCSuprahepatic IVCSMVJ vascular graft: donor IMV121262Intraoperative portal thrombosis (anastomosis redone); alive and well
Sclerotic preduodenal PV
Polysplenia
5Male/11LLS5.6Absent RHVCHepatic vein confluencePV trunk171755Reoperation due to bile leakage from cut surface; late biliary stenosis treated with percutaneous ductoplasty; alive and well
Preduodenal PV
Polysplenia
Dextrocardia
CHA from LGA
6Male/6LLS2.7PolyspleniaRetrohepatic IVCSMVJ vascular graft: recipient IJV151538Alive and well
PV thrombosis
7Male/7Reduced LLS4.3 (after graft reduction)Absent RHVCHepatic vein confluencePV trunk421536Alive and well
Preduodenal PV
Polysplenia
CHA from aorta
8Female/9LLS4.2Absent RHVCHepatic vein confluencePV trunk20426Small bowel perforation; alive and well
Polysplenia
LHA from LGA
9Male/22LLS2Absent RHVCHepatic vein confluenceSMVJ vascular graft: cryopreserved iliac artery2352Bile leakage from the cut surface (resolved); alive and well
Polysplenia
Sclerotic preduodenal PV
Dextrocardia

Eight patients received an LLS graft. In 1 case, a reduced LLS was used (patient's weight = 5.8 kg, graft weight before reduction = 350 g, graft-to-recipient weight ratio before reduction = 6.03%; Fig. 1B). All liver grafts were placed in the upper left abdomen, and a stay suture between the liver graft's falciform ligament and the diaphragm was needed in only 1 case (a recipient weighing 15 kg with a large abdominal cavity due to ascites). The donor's left hepatic vein (LHV) was anastomosed to the confluence of the recipient's 3 hepatic veins in 7 of the 9 patients; in the remaining 2 patients, because of caliber mismatches, the anastomoses were performed directly in the IVC (the retrohepatic IVC in one patient and the suprahepatic IVC in the other patient because of the absence of the RHVC).

Three patients required the use of vascular grafts during PV anastomosis (1 recipient with PV thrombosis and 2 with sclerotic preduodenal PVs). Vascular grafts were chosen according to the calibers and availability. All vascular grafts [n = 3: donor's inferior mesenteric vein (IMV), recipient's internal jugular vein (IJV), and cryopreserved iliac artery from a deceased donor] were interposed between the recipient's splenomesenteric vein junction (SMVJ) and the donor's left PV. In the remaining 6 patients, PV reconstruction was performed between the left PV of the graft and the PV trunk of the recipient, usually below the PV bifurcation, with continuous 7-0 Prolene sutures and growth factor.

All arterial anastomoses were performed with microvascular techniques, and no changes in the anastomosis technique were necessary because of arterial anatomical anomalies. The position of the liver graft did not bring difficulties to biliary reconstruction either. For the posterior wall of the bilioenteric anastomosis, a parachute technique with continuous 7-0 Prolene sutures was used, and for the anterior wall, interrupted 7-0 polydioxanone sutures were used without a stent.

As for postoperative complications, relaparotomy was necessary in 2 patients: one with bile leakage from the cut surface and sepsis, and another with a small bowel perforation. During follow-up, 1 patient presented with a stricture of the bilioenteric anastomosis and was successfully treated with percutaneous stenting. Despite vascular anomalies, none of the patients in this series developed vascular complications during follow-up. In the first 2 years, routine Doppler ultrasound examinations were performed every 6 months in order to ensure vascular patency.

The median values for intraoperative packed red blood cell transfusions, cold ischemia times, and warm ischemia times were 187.5 mL (range = 90-354 mL), 63.1 minutes (range = 20-145 minutes), and 39.5 minutes (range = 29-59 minutes), respectively. These values were similar to previously published data from our group.[7] Follow-up data are summarized in Table 1.

DISCUSSION

  1. Top of page
  2. Abstract
  3. PATIENTS AND METHODS
  4. RESULTS
  5. DISCUSSION
  6. ACKNOWLEDGMENT
  7. REFERENCES

The complex vascular anomalies observed in patients with SI and biliary atresia/polysplenia syndrome increase the technical difficulty of the operation, but today this condition is no longer a contraindication for liver transplantation. In contrast to whole liver transplantation in recipients with SI, in which the size and depth of the right lobe are responsible for the difficult accommodation of the liver in the abdominal cavity, LLS graft positioning is usually not a concern (previously published cases of pediatric patients with SI undergoing LDLT are summarized in Table 2). In most of our cases, even large grafts were accommodated without additional measures such as graft reduction or delayed abdominal closure. In 1 case, graft reduction was necessary because of the combination of a small abdominal cavity and a large LLS in a very young recipient (age = 7 months). All liver grafts were positioned in the left upper quadrant, which allowed good orientation for hepatic vein reconstruction.

Table 2. Literature Review of Pediatric Patients With SI and End-Stage Liver Disease Undergoing LDLT: Demographics and Outcomes
StudySex/Age (Months)GraftAnatomical AnomaliesHepatic Vein AnastomosisPV AnastomosisGraft FixationFollow-Up
Mattei et al.[4] (1998)Female/36LLSAbsent RHVCHepatic vein confluencePV trunk18 months
Preduodenal PV
Malrotation
Maggard et al.[8] (1999)Female/11LLSAbsent RHVCHepatic vein confluencePV trunkYes8 months
Preduodenal PV
Polysplenia
PHA from SMA
LHA from LGA
Sugawara et al.[9] (2001)Male/35LLSDextrocardiaHepatic vein confluencePV trunk20 months
Lee et al.[10] (2004)Male/4LLSAbsent RHVCHepatic vein confluencePV trunkYes24 months
Polysplenia
Malrotation
Matsubara et al.[5] (2005)Male/60LLSAbsent RHVCHepatic vein confluencePV trunk with interposed graft: donor IMVYes10 years
Preduodenal PV
Polysplenia
PHA from aorta
Matsubara et al.[5] (2005)Female/28LLSPreduodenal PVHepatic vein from segment 2 with LHV and MHV Hepatic vein from segment 3 with RHVPV trunk with interposed graft: donor ovarian veinYes4 years
Polysplenia
Dextrocardia
Malrotation
PHA from aorta
Matsubara et al.[5] (2005)Female/18LLSAbsent RHVCHepatic vein confluencePV trunkYes4 years
Matsubara et al.[5] (2005)Female/9Reduced monosegmentAbsent RHVCHepatic vein confluencePV trunkYes6 months
Malrotation
Duodenal atresia
Sanada et al.[11] (2008)Male/7LLSAbsent RHVCSuprahepatic IVCSMVJ vascular graft: donor splenic vein6 months
Sclerotic PV
Kasahara et al.[12] (2009)Female/16Reduced LLSAbsent RHVCRight atriumShunt vessel9 months
Absent PV
Polysplenia
Malrotation

Despite the high incidence of anatomical anomalies of the IVC (for 8 patients, the RHVC was absent or hypoplastic), hepatic vein reconstruction was performed without technical modifications in 7 patients; in these cases, the donor's LHV was anastomosed to the confluence of the recipient's 3 hepatic veins with continuous 6-0 Prolene sutures. In 1 patient, a common cloaca with the recipient's hepatic veins could not be accomplished because of the abnormal distance between the 3 hepatic veins; in that case, the recipient's hepatic veins were closed, and the graft was implanted directly to the RHVC. In another case, a patient with an absent RHVC had a hepatic vein confluence with a small diameter, and it was necessary to make an anastomosis between the donor's LHV and the recipient's suprahepatic vena cava. In a case series of 4 patients with SI who underwent LDLT, Matsubara et al.[5] recommended that the anastomosis between the donor's hepatic vein and the recipient's hepatic veins should be performed with interrupted sutures in order to overcome the size discrepancy of the anastomotic orifices. Additionally, the authors sutured the falciform ligament of each liver graft in the recipient's abdominal wall to prevent torsion of the anastomosis. In similar cases, we decided to construct the anastomosis directly on the vena cava to ensure a satisfactory blood outflow. With the exception of 1 case, we did not need to use stay sutures between the liver graft and the abdominal wall; however, our patients were markedly younger than the patients reported by the Kyoto group[5] (11 versus 23 months, respectively). In small cavities, after graft implantation, there is no additional space to allow liver mobility or anastomosis torsion.

PV anomalies similarly did not justify considerable changes in the surgical technique during graft implantation. As well described in previous publications,[2, 4] additional care is mandatory during the recipient hepatectomy because early recognition of a preduodenal PV is important for avoiding a severe vascular injury. A preduodenal position for the PV did not compromise an end-to-end anastomosis with the donor's left PV, and the anastomoses were performed with continuous 7-0 Prolene sutures and growth factor. In 3 patients, the use of vascular conduits was necessary for PV anastomoses because of PV thrombosis in 1 recipient and a significant caliber reduction of the PV in 2 patients. However, we believe that the intraluminal PV alterations were probably related to the inflammatory process caused by biliary atresia and that there was no direct correlation with SI.

During LDLT, arterial anomalies justify special attention when hilar dissection is being performed during the recipient operation because the artery of the liver graft is normally short. In all of the cases presented in this series, graft arterialization was performed with a microvascular technique, and no cases of HA thrombosis were observed during follow-up.

Overall, LDLT can be successfully performed in pediatric patients with biliary atresia and SI. Complex vascular anomalies on the recipient side, which are associated with the use of partial liver grafts (short vascular stumps), are not associated with an increased occurrence of vascular complications. Vascular grafts should be available for portal reconstruction. Adequate graft positioning can be achieved when partial livers obtained from living donors are used.

ACKNOWLEDGMENT

  1. Top of page
  2. Abstract
  3. PATIENTS AND METHODS
  4. RESULTS
  5. DISCUSSION
  6. ACKNOWLEDGMENT
  7. REFERENCES

The authors thank Maria Luisa Roncoroni Seda for her helpful suggestions and Ms. Claudia Marfil Romero (liver transplant nurse coordinator, A. C. Camargo Hospital) and Ms. Rosemeire Aparecida da Silva for their help with the patient data acquisition.

REFERENCES

  1. Top of page
  2. Abstract
  3. PATIENTS AND METHODS
  4. RESULTS
  5. DISCUSSION
  6. ACKNOWLEDGMENT
  7. REFERENCES