Auxiliary Partial Orthotopic Liver Transplantation for Selected Noncirrhotic Metabolic Liver Disease

Abstract

Auxiliary partial orthotopic liver transplantation (APOLT) in selected noncirrhotic metabolic liver diseases (NCMLDs) is a viable alternative to orthotopic liver transplantation (OLT) as it supplements the function of the native liver with the missing functional protein. APOLT for NCMLD is not universally accepted due to concerns of increased technical complications and longterm graft atrophy. Review of a prospectively collected database of all pediatric patients (age ≤16 years) who underwent liver transplantation for NCMLD from August 2009 up to June 2017 was performed. Patients were divided into 2 groups: group 1 underwent APOLT and group 2 underwent OLT. In total, 18 OLTs and 12 APOLTs were performed for NCMLDs during the study period. There was no significant difference in the age and weight of the recipients in both groups. All APOLT patients needed intraoperative portal flow modulation. Intraoperative peak and end of surgery lactate were significantly higher in the OLT group, and cold ischemia time was longer in the APOLT group. There were no differences in postoperative liver function tests apart from higher peak international normalized ratio in the OLT group. The incidence of postoperative complications, duration of hospital stay, and 1‐ and 5‐year survivals were similar in both groups. In conclusion, we present the largest series of APOLT for NCMLD. APOLT is a safe and effective alternative to OLT and may even be better than OLT due to lesser physiological stress and the smoother postoperative period for selected patients with NCMLD.

Liver Transplantation in Children With Propionic Acidemia: Medium‐Term Outcomes

“…The clinical success of this procedure demonstrates that a sufficient amount of enzymes is replaced and provides similar benefits to a whole liver replacement. The advantage is that if gene therapy becomes available in the future, these children could still be potential candidates …”

Minimally Invasive Treatment of Metabolic Decompensation Due to Portal Steal in Auxiliary Liver Transplantation

“…With improving results, APOLT has been shown to be a safe and effective alternative to OLT, and may even be better than OLT due to lesser physiological stress and a smoother postoperative period for selected patients with NCMLD. (5,9) In the early postoperative period, the normally functioning native liver reduces the systemic effect of graft dysfunction, which gives the patient and graft an opportunity to recover. This is particularly advantageous in children with PA where graft dysfunction after OLT can precipitate severe metabolic stress and decompensation.…”

“…This is particularly advantageous in children with PA where graft dysfunction after OLT can precipitate severe metabolic stress and decompensation. (5,9) Portal steal can be a major issue in APOLT for NCMLD. (5,(7)(8)(9) It increases the risk of graft atrophy and recurrence of the original symptoms of the disease.…”

“…The graft was implanted, and portal flow modulation by graded hemiportal banding was performed using the technique described by us elsewhere. (9,10) Bile duct anastomosis was by a Roux-en-Y hepaticojejunostomy.…”

Auxiliary Partial Orthotopic Liver Transplantation for Noncirrhotic Metabolic Liver Disease: Reigniting Interest in an Old but New Technique

“…In the current issue of Liver Transplantation , Rammohan et al present their experience of APOLT for NCMLD in 12 recipients . This report comes from a center highly experienced in performing liver transplantation with technical variant grafts.…”