Pediatr Transplantation 2015: 19: E56–E61
© 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd
Pediatric Transplantation DOI: 10.1111/petr.12437
Better innovate than compromise: A novel hepatic outflow reconstruction technique in pediatric living donor liver transplantation Cherian PT, Mishra AK, Bangaari A, Kota V, Sathyanarayanan M, Raya R, Rela M. (2015) Better innovate than compromise: A novel hepatic outflow reconstruction technique in pediatric living donor liver transplantation. Pediatr Transplant, 19: E56–E61. DOI: 10.1111/ petr.12437.
P. Thomas Cherian1,3, Ashish K. Mishra1,2, Ashish Bangaari4, Venugopal Kota3, Mohan Sathyanarayanan5, Ravichandra Raya5 and Mohamed Rela1,3,6 1
Abstract: Pediatric LDLT using donors with unfavorable vascular anatomy is challenging in terms of donor safety, and complexity of reconstruction in the recipient. We describe an innovative technique of hepatic venous outflow reconstruction involving the recipient RHV, in the presence of a rudimentary RHV in the donor. The postoperative course of the donor and recipient was uneventful with satisfactory venous outflow in both. This technique avoided the use of prosthetic material, an important consideration given the recipient age and requirement for growth. This shows that donors previously considered unsuitable for donation can be utilized safely as long as principles of vascular anastomosis are adhered to. Moreover, it highlights that innovation is sometimes necessary to avoid compromise in donor safety.
Department of HPB and Liver Transplantation, Global Hospitals, Hyderabad, Telangana, India, 2 Department of Surgical Gastro and Liver Transplant, Global Hospitals, Hyderabad, Telangana, India, 3Institute of Liver Disease & Transplantation, Global Health City, Chennai, Tamil Nadu, India, 4 Department of Liver Transplant Anaesthesia, MIOT Hospital, Chennai, Tamil Nadu, India, 5Department of Anaesthesiology and Critical Care, Global Hospitals, Hyderabad, Telangana, India, 6Insitute of Liver Studies, King’s College Hospital, London, UK Key words: live donor safety – hepatic outflow obstruction – innovative technique – acute liver failure – graft survival – pediatric liver transplantation P. Thomas Cherian, Department of HPB Surgery & Liver Transplantation, Global Hospital, 6-1-1070 Lakdi-ka-Pool, Hyderabad, Telangana 500004, India Tel.: +91 97 0477 7700 Fax: +91 40 2324 4455 E-mail: [email protected] Accepted for publication 9 January 2015
Pediatric LDLT using donors with unfavorable vascular anatomy is challenging in terms of donor safety, and recipient reconstruction. A novel technique in venous outflow is described wherein the recipient RHV is utilized as an in situ vein patch to allow retention of the full outflow of the residual liver in the donor. The technique allowed the use of the only available donor who Abbreviations: ACLF, acute-on-chronic liver failure; ALF, acute liver failure; CT, computerized tomography; GRWR, graft–recipient weight ratio; HV, hepatic vein; HVOO, hepatic venous outflow obstruction; INR, internationalized normal ratio; IVC, inferior vena cava; LDLT, living donor liver transplantation; LL, left lobe; LLS, left lateral segment; MHV, middle hepatic vein; PELD, pediatric endstage liver disease; POD, postoperative day; RHV, right hepatic vein; RI, resistive index; SFS, small for size.
E56
might have been considered unsuitable using conventional reconstruction methods, without the use of prosthetic material or compromising donor safety. Case description
A 10-yr-old boy (20 kg/113 cm) with suspected Wilson’s disease (PELD score 23) was referred to us for an LDLT. Presenting complaints included stunted growth, jaundice, itching, and abdominal distension. Investigations revealed a low hemoglobin (8 g/dL) and high serum bilirubin (10.3 mg/dL; INR = 1.8, aspartate aminotransferase = 429 IU/L, serum ceruloplasmin = 55 mg/dL, and creatinine = 0.5 mg/dL). Urinary copper levels were raised, Kaiser–Fisher rings were detected, and an autoimmune work-up was
Innovative technique for hepatic outflow
negative. Liver biopsy confirmed excessive copper staining suggestive of Wilson’s disease. Despite the chronic history, shortly after transplant assessment, his condition deteriorated with significant ascites, increased bilirubin (23.1 mg/ dL), and worsening coagulopathy (INR = 3.2). Frequent large volume paracentesis was then required to control severe respiratory distress, in view of which urgent transplantation was deemed necessary, which in India almost always requires a living related donor (cadaveric organ rate of 0.05–0.08/million population) (1). On evaluation of the family, only one donor – patient’s mother (body mass index 20) – was found suitable to a certain extent and willing for donation (father – wrong blood group; no adult siblings; no willing relatives). Donor liver protocol CT scan showed a nonsteatotic liver (Liver Attenuation Index +12), an estimated total volume of 803 cc and right and LL volumes of 560 and 243 cc, respectively. Hepatic venous anatomy revealed a rudimentary RHV, with most of the right sided segments VIII, VI, and V draining into the dominant MHV. However, a large part of this drainage was through a 1.2-cm segment VIII (S-VIII) vein opening into the MHV about 1.5 cm from its junction with the IVC, which was draining additionally a part of segment VII (Fig. 1). Two accessory right inferior HVs of size 3.3 and 2.3 mm were also present, possibly draining
Fig. 1. CT reconstruction: HV of donor, showing a rudimentary RHV and a large S-VIII vein opening into a dominant MHV about 2 cm from its junction.
S-VI. Arterial and portal venous anatomy was conventional. The volume of the LLS was 142 cc (estimated GRWR of 0.71), which might have been inadequate in a child. Moreover, given the poor recipient physiological reserve and high PELD score, such an SFS graft we felt might reduce the child’s ability to tolerate postoperative stress/complications. Hence, a decision was made to use the whole LL. However, this decision raised a potential issue, as conventionally the MHV is taken with an LL graft. Given the above hepatic venous anatomy, we felt ligating both the S-V and SVIII veins (required if we are to take the MHV with the graft) would lead to severe congestion of the entire right anterior sector and part of segment VII, raising the possibility of a “functional” SFS syndrome and/or segmental necrosis, increasing donor risk. On the other hand, with a dominant MHV draining quite a large aspect of S-IV, we felt an LL without the MHV would jeopardize the recipient. The multiple, individual S-IV HVs draining into the MHV were small caliber, precluding bench reconstruction direct onto the cava, which might have allowed an LL implantation without the MHV. A right posterior section graft was not an option with the rudimentary RHV and abovementioned anatomy, as the graft venous drainage might be compromised at the level of the caval anastomosis and S-VI outflow. Given the urgency of the situation, a decision was finally made to utilize an unconventional technique of reconstructing the venous outflow in the recipient, which would enable the use of the whole LL without compromising donor safety. It was decided to procure the LL with the MHV taken with the graft only up to and excluding the MHV aspect that was draining the segment VIII vein, followed by the utilization of the recipient’s RHV to reconstruct the missing 1.5-cm segment of MHV in the LL graft (estimated GRWR was 1.15). Donor operation proceeded with mobilization of LL and parenchymal transection to the right of MHV. The large S-V vein draining into MHV was ligated, causing approximately an 8 9 7 cm area of congestion in segment V. The drainage of segment VIII was preserved as discussed above, with a short part of MHV left intact with the S-VIII vein attached, to drain into the IVC. Thus, the MHV was divided superior to the S-VIII vein opening and the LL graft removed with the distal 1.5 cm of MHV missing (Fig. 2). The final graft weight was 288 g. Recipient hepatectomy was completed as per conventional E57
Cherian et al.
Fig. 2. (a) The transection technique in the donor – zigzag across the MHV. (b) The end result in the donor post-removal of the LL graft. The distal end of the S-VIII vein is created by suturing shut the two sides of the defect caused by removal of the MHV above the S-VIII vein insertion.
technique. However, at the terminal stage of the hepatectomy before the division of the RHV (after the right portal vein was clamped), we dissected into the recipient (cirrhotic) liver to deliver a 3-cm length of the RHV out of the liver parenchyma, which was then clamped and divided. Following this, the combined cuffs of MHV and LHV were separately clamped and hepatectomy was completed. At implantation, a large Satinsky clamp was applied across the IVC above the HV to allow the creation of a single orifice for the caval outflow anastomosis. To achieve this, the long length of RHV procured from the recipient liver was opened out medially to create a patch, which was utilized to bridge the gap in the lateral aspect of the graft (donor) MHV, and create a single outflow orifice along with the recipient MHV and LHV stumps (Fig. 3). Finally, the remnant graft MHV and LHV was anastomosed to this combined cuff of recipient RHV, MHV, and LHV with a continuous posterior layer and interrupted anterior layer using polypropylene 5’0 sutures (Fig. 4). Moreover, as in this particular LL graft, the left and middle HVs were joining the IVC almost separately; we feared a risk of LHV outflow compromise due to the unconventional nature of the caval anastomosis. Therefore, the intervening venous septum was removed at backbench via a V-shaped venotomy (into the liver parenchyma), and a subsequent everting venoplasty bringing the two venous outflow tracts together into a single open orifice (Fig. 4). A simple or classic technique of hepatic outflow reconstruction with a E58
Fig. 3. Intra-operative picture: Single orifice created by opening out of the long RHV, and how it was used to bridge a gap in the donor (graft) MHV.
simple venoplasty alone would have been difficult in the current case owing to the length of the gap that was required to be bridged and even if achieved would have left the graft very skewed (in lie), making hilar inflow reconstruction much more difficult and angulated. The portal venous and left hepatic arterial anastomoses were done with continuous 6’0 and interrupted polypropylene 8’0 sutures, respectively. Intra-operative Doppler showed excellent arterial (RI of 0.67, peak hepatic arterial flow 42 cm/s) and portal venous inflows (portal flow of 67 cm/s). The two HVs showed excellent triphasic flow all the way to the periphery, suggesting a good outflow tract.
Innovative technique for hepatic outflow
Fig. 4. (a) The reconstruction technique in the recipient wherein the native RHV is brought across to bridge the 2cm gap in the graft MHV. Note the small venoplasty at the junction of the middle and left HV. (b) The state of recipient HVs in the anhepatic phase and the planned opening out of the native RHV (which was extracted from the explant before final cross-clamping).
Postoperative donor Doppler revealed normal remnant right lobe with a patent RHV, S-VIII vein, right portal vein, and right hepatic artery flows. She was discharged on POD six in a stable ambulatory condition. The recipient postoperative Doppler also showed excellent arterial, portal, and venous flows and RI of 0.62. He received overnight ventilator support with sedation and was extubated the next day. He was transferred to step-down intensive care unit on POD three with gradual normalization of laboratory and clinical parameters. He was discharged on POD 21 without incident. His last follow-up nine months post-surgery showed good graft function on blood investigations, and triphasic flow in the reconstructed HVs on Doppler ultrasound. Discussion
Liver failure can develop as ALF (in the absence of any pre-existing liver disease), acute decompensation of chronic liver disease (ACLF), or a chronic decompensation in end-stage liver disease (2). Patients with ACLF especially with multi-organ involvement are well known to suffer high mortality (3). Although hepatic encephalopathy is an hallmark of acute decompensation, some exceptional circumstances such as progressive hyperbilirubinemia, sepsis, or rapid-onset (type I) hepatorenal syndrome can lead to similar mortality rates despite the absence of hepatic
encephalopathy. In the current case, the decompensation manifested with severe hyperbilirubinemia and high volume recurrent hydrothorax. Liver transplantation remains the only definitive proven therapy to improve survival in such patients, who do not improve with supportive measures. In countries with a healthy deceased donor rate and a well-developed system of organ allocation, recipients with ALF can avail of organs via super-urgent listing criteria. However, in countries wherein cadaveric donors are few, such patients who require emergent transplantation have a disadvantage and thereby high mortality. Given the deceased donation rate of
© 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd
Pediatric Transplantation DOI: 10.1111/petr.12437
Better innovate than compromise: A novel hepatic outflow reconstruction technique in pediatric living donor liver transplantation Cherian PT, Mishra AK, Bangaari A, Kota V, Sathyanarayanan M, Raya R, Rela M. (2015) Better innovate than compromise: A novel hepatic outflow reconstruction technique in pediatric living donor liver transplantation. Pediatr Transplant, 19: E56–E61. DOI: 10.1111/ petr.12437.
P. Thomas Cherian1,3, Ashish K. Mishra1,2, Ashish Bangaari4, Venugopal Kota3, Mohan Sathyanarayanan5, Ravichandra Raya5 and Mohamed Rela1,3,6 1
Abstract: Pediatric LDLT using donors with unfavorable vascular anatomy is challenging in terms of donor safety, and complexity of reconstruction in the recipient. We describe an innovative technique of hepatic venous outflow reconstruction involving the recipient RHV, in the presence of a rudimentary RHV in the donor. The postoperative course of the donor and recipient was uneventful with satisfactory venous outflow in both. This technique avoided the use of prosthetic material, an important consideration given the recipient age and requirement for growth. This shows that donors previously considered unsuitable for donation can be utilized safely as long as principles of vascular anastomosis are adhered to. Moreover, it highlights that innovation is sometimes necessary to avoid compromise in donor safety.
Department of HPB and Liver Transplantation, Global Hospitals, Hyderabad, Telangana, India, 2 Department of Surgical Gastro and Liver Transplant, Global Hospitals, Hyderabad, Telangana, India, 3Institute of Liver Disease & Transplantation, Global Health City, Chennai, Tamil Nadu, India, 4 Department of Liver Transplant Anaesthesia, MIOT Hospital, Chennai, Tamil Nadu, India, 5Department of Anaesthesiology and Critical Care, Global Hospitals, Hyderabad, Telangana, India, 6Insitute of Liver Studies, King’s College Hospital, London, UK Key words: live donor safety – hepatic outflow obstruction – innovative technique – acute liver failure – graft survival – pediatric liver transplantation P. Thomas Cherian, Department of HPB Surgery & Liver Transplantation, Global Hospital, 6-1-1070 Lakdi-ka-Pool, Hyderabad, Telangana 500004, India Tel.: +91 97 0477 7700 Fax: +91 40 2324 4455 E-mail: [email protected] Accepted for publication 9 January 2015
Pediatric LDLT using donors with unfavorable vascular anatomy is challenging in terms of donor safety, and recipient reconstruction. A novel technique in venous outflow is described wherein the recipient RHV is utilized as an in situ vein patch to allow retention of the full outflow of the residual liver in the donor. The technique allowed the use of the only available donor who Abbreviations: ACLF, acute-on-chronic liver failure; ALF, acute liver failure; CT, computerized tomography; GRWR, graft–recipient weight ratio; HV, hepatic vein; HVOO, hepatic venous outflow obstruction; INR, internationalized normal ratio; IVC, inferior vena cava; LDLT, living donor liver transplantation; LL, left lobe; LLS, left lateral segment; MHV, middle hepatic vein; PELD, pediatric endstage liver disease; POD, postoperative day; RHV, right hepatic vein; RI, resistive index; SFS, small for size.
E56
might have been considered unsuitable using conventional reconstruction methods, without the use of prosthetic material or compromising donor safety. Case description
A 10-yr-old boy (20 kg/113 cm) with suspected Wilson’s disease (PELD score 23) was referred to us for an LDLT. Presenting complaints included stunted growth, jaundice, itching, and abdominal distension. Investigations revealed a low hemoglobin (8 g/dL) and high serum bilirubin (10.3 mg/dL; INR = 1.8, aspartate aminotransferase = 429 IU/L, serum ceruloplasmin = 55 mg/dL, and creatinine = 0.5 mg/dL). Urinary copper levels were raised, Kaiser–Fisher rings were detected, and an autoimmune work-up was
Innovative technique for hepatic outflow
negative. Liver biopsy confirmed excessive copper staining suggestive of Wilson’s disease. Despite the chronic history, shortly after transplant assessment, his condition deteriorated with significant ascites, increased bilirubin (23.1 mg/ dL), and worsening coagulopathy (INR = 3.2). Frequent large volume paracentesis was then required to control severe respiratory distress, in view of which urgent transplantation was deemed necessary, which in India almost always requires a living related donor (cadaveric organ rate of 0.05–0.08/million population) (1). On evaluation of the family, only one donor – patient’s mother (body mass index 20) – was found suitable to a certain extent and willing for donation (father – wrong blood group; no adult siblings; no willing relatives). Donor liver protocol CT scan showed a nonsteatotic liver (Liver Attenuation Index +12), an estimated total volume of 803 cc and right and LL volumes of 560 and 243 cc, respectively. Hepatic venous anatomy revealed a rudimentary RHV, with most of the right sided segments VIII, VI, and V draining into the dominant MHV. However, a large part of this drainage was through a 1.2-cm segment VIII (S-VIII) vein opening into the MHV about 1.5 cm from its junction with the IVC, which was draining additionally a part of segment VII (Fig. 1). Two accessory right inferior HVs of size 3.3 and 2.3 mm were also present, possibly draining
Fig. 1. CT reconstruction: HV of donor, showing a rudimentary RHV and a large S-VIII vein opening into a dominant MHV about 2 cm from its junction.
S-VI. Arterial and portal venous anatomy was conventional. The volume of the LLS was 142 cc (estimated GRWR of 0.71), which might have been inadequate in a child. Moreover, given the poor recipient physiological reserve and high PELD score, such an SFS graft we felt might reduce the child’s ability to tolerate postoperative stress/complications. Hence, a decision was made to use the whole LL. However, this decision raised a potential issue, as conventionally the MHV is taken with an LL graft. Given the above hepatic venous anatomy, we felt ligating both the S-V and SVIII veins (required if we are to take the MHV with the graft) would lead to severe congestion of the entire right anterior sector and part of segment VII, raising the possibility of a “functional” SFS syndrome and/or segmental necrosis, increasing donor risk. On the other hand, with a dominant MHV draining quite a large aspect of S-IV, we felt an LL without the MHV would jeopardize the recipient. The multiple, individual S-IV HVs draining into the MHV were small caliber, precluding bench reconstruction direct onto the cava, which might have allowed an LL implantation without the MHV. A right posterior section graft was not an option with the rudimentary RHV and abovementioned anatomy, as the graft venous drainage might be compromised at the level of the caval anastomosis and S-VI outflow. Given the urgency of the situation, a decision was finally made to utilize an unconventional technique of reconstructing the venous outflow in the recipient, which would enable the use of the whole LL without compromising donor safety. It was decided to procure the LL with the MHV taken with the graft only up to and excluding the MHV aspect that was draining the segment VIII vein, followed by the utilization of the recipient’s RHV to reconstruct the missing 1.5-cm segment of MHV in the LL graft (estimated GRWR was 1.15). Donor operation proceeded with mobilization of LL and parenchymal transection to the right of MHV. The large S-V vein draining into MHV was ligated, causing approximately an 8 9 7 cm area of congestion in segment V. The drainage of segment VIII was preserved as discussed above, with a short part of MHV left intact with the S-VIII vein attached, to drain into the IVC. Thus, the MHV was divided superior to the S-VIII vein opening and the LL graft removed with the distal 1.5 cm of MHV missing (Fig. 2). The final graft weight was 288 g. Recipient hepatectomy was completed as per conventional E57
Cherian et al.
Fig. 2. (a) The transection technique in the donor – zigzag across the MHV. (b) The end result in the donor post-removal of the LL graft. The distal end of the S-VIII vein is created by suturing shut the two sides of the defect caused by removal of the MHV above the S-VIII vein insertion.
technique. However, at the terminal stage of the hepatectomy before the division of the RHV (after the right portal vein was clamped), we dissected into the recipient (cirrhotic) liver to deliver a 3-cm length of the RHV out of the liver parenchyma, which was then clamped and divided. Following this, the combined cuffs of MHV and LHV were separately clamped and hepatectomy was completed. At implantation, a large Satinsky clamp was applied across the IVC above the HV to allow the creation of a single orifice for the caval outflow anastomosis. To achieve this, the long length of RHV procured from the recipient liver was opened out medially to create a patch, which was utilized to bridge the gap in the lateral aspect of the graft (donor) MHV, and create a single outflow orifice along with the recipient MHV and LHV stumps (Fig. 3). Finally, the remnant graft MHV and LHV was anastomosed to this combined cuff of recipient RHV, MHV, and LHV with a continuous posterior layer and interrupted anterior layer using polypropylene 5’0 sutures (Fig. 4). Moreover, as in this particular LL graft, the left and middle HVs were joining the IVC almost separately; we feared a risk of LHV outflow compromise due to the unconventional nature of the caval anastomosis. Therefore, the intervening venous septum was removed at backbench via a V-shaped venotomy (into the liver parenchyma), and a subsequent everting venoplasty bringing the two venous outflow tracts together into a single open orifice (Fig. 4). A simple or classic technique of hepatic outflow reconstruction with a E58
Fig. 3. Intra-operative picture: Single orifice created by opening out of the long RHV, and how it was used to bridge a gap in the donor (graft) MHV.
simple venoplasty alone would have been difficult in the current case owing to the length of the gap that was required to be bridged and even if achieved would have left the graft very skewed (in lie), making hilar inflow reconstruction much more difficult and angulated. The portal venous and left hepatic arterial anastomoses were done with continuous 6’0 and interrupted polypropylene 8’0 sutures, respectively. Intra-operative Doppler showed excellent arterial (RI of 0.67, peak hepatic arterial flow 42 cm/s) and portal venous inflows (portal flow of 67 cm/s). The two HVs showed excellent triphasic flow all the way to the periphery, suggesting a good outflow tract.
Innovative technique for hepatic outflow
Fig. 4. (a) The reconstruction technique in the recipient wherein the native RHV is brought across to bridge the 2cm gap in the graft MHV. Note the small venoplasty at the junction of the middle and left HV. (b) The state of recipient HVs in the anhepatic phase and the planned opening out of the native RHV (which was extracted from the explant before final cross-clamping).
Postoperative donor Doppler revealed normal remnant right lobe with a patent RHV, S-VIII vein, right portal vein, and right hepatic artery flows. She was discharged on POD six in a stable ambulatory condition. The recipient postoperative Doppler also showed excellent arterial, portal, and venous flows and RI of 0.62. He received overnight ventilator support with sedation and was extubated the next day. He was transferred to step-down intensive care unit on POD three with gradual normalization of laboratory and clinical parameters. He was discharged on POD 21 without incident. His last follow-up nine months post-surgery showed good graft function on blood investigations, and triphasic flow in the reconstructed HVs on Doppler ultrasound. Discussion
Liver failure can develop as ALF (in the absence of any pre-existing liver disease), acute decompensation of chronic liver disease (ACLF), or a chronic decompensation in end-stage liver disease (2). Patients with ACLF especially with multi-organ involvement are well known to suffer high mortality (3). Although hepatic encephalopathy is an hallmark of acute decompensation, some exceptional circumstances such as progressive hyperbilirubinemia, sepsis, or rapid-onset (type I) hepatorenal syndrome can lead to similar mortality rates despite the absence of hepatic
encephalopathy. In the current case, the decompensation manifested with severe hyperbilirubinemia and high volume recurrent hydrothorax. Liver transplantation remains the only definitive proven therapy to improve survival in such patients, who do not improve with supportive measures. In countries with a healthy deceased donor rate and a well-developed system of organ allocation, recipients with ALF can avail of organs via super-urgent listing criteria. However, in countries wherein cadaveric donors are few, such patients who require emergent transplantation have a disadvantage and thereby high mortality. Given the deceased donation rate of
