Association between Kasai portoenterostomy at low caseload centres and transplant complications in children with biliary atresia.

BACKGROUND: Kasai portoenterostomy (KPE) is the preferred treatment for biliary atresia (BA) patients. It has been shown that the center caseload of KPE impacts on native liver survival. We aimed to define the impact of KPE caseload on complications at the time of liver transplantation (LT). METHODS: Retrospective data collection of LT for BA performed in our tertiary center between 2010 and 2018. The patients were grouped according to the caseload of the center that performed KPE: Group A (≥5 KPE/year) and Group B (<5 KPE/year). We analyzed total transplant time (TTT), hepatectomy time, amount of plasma and red blood cell (RBC) transfusions, occurrence of bowel perforations at LT. RESULTS: Among 115 patients, Group A (n 44) and Group B (n 71) were comparable for age, sex, PELD score, TTT. The groups differed for: median hepatectomy time (57 min, IQR = 50-67; vs 65, IQR 55-89, p = 0.045); RBC transfusions (95 ml, IQR 0-250; vs 200 ml, IQR 70-500, p = 0.017); bowel perforations (0/44 vs 15/71, p = 0.001). One-year graft loss in Group A vs Group B was 1/44 vs 7/71 (p = 0.239), whereas deaths were 0/44 vs 5/71 respectively (p = 0.183); 5/15 patients who had a perforation eventually lost the graft. CONCLUSIONS: This study found an association between KPE performed in low caseload center and the incidence of complications at LT. These patients tend to have a worse outcome. The centralization of KPE to referral center represents an advantage at the time of LT. MINI ABSTRACT: We studied the impact of Kasai portoenterostomy (KPE) caseload on complications at the time of liver transplantation (LT), in 115 patients. We found an association between KPE performed in low caseload center and increased bowel perforations and blood transfusions. We suggest to centralize to experienced center all children requiring KPE.

Total Oesophagogastric Dissociation in Neurologically Impaired Children: 18 Years' Experience and Long-term Follow-up.

OBJECTIVES: Total oesophagogastric dissociation (TOGD) is an alternative antireflux surgery for neurologically impaired children because of a 16% to 38% fundoplication failure rate. This study evaluates TOGD's feasibility and its long-term efficacy both as a Primary and as a "Rescue" procedure after failed fundoplication. METHODS: Thirty patients (18 boys) who underwent TOGD between 2000 and 2018 in 2 Italian Centres were retrospectively reviewed. Twenty-three were Primary procedures and 7 were "Rescue" ones. Inclusion criteria were severe neurodisability, intractable gastroesophageal reflux, and dysphagia. RESULTS: Preoperatively, all children had regurgitation, vomiting or retching, and 93% had unsafe swallowing and aspiration, with recurrent chest infections/aspiration pneumonia. Median relative weight was 77% (48%--118%). All patients were taking antireflux medication before surgery. Median age at TOGD was 6.48 years (0.69--22.18). Median follow-up was 3.5 years (0.6-17.7). No recurrence of gastroesophageal reflux (GER) and vomiting was recorded. The number of chest infections and length of hospital stay showed a significative decrease (P value <0.0001 for both), whereas median relative weight reached 101% (P value 0.002). Parents'/caregivers' perception of outcome showed a significative improvement. Six patients (20%) experienced early complications and 3 required surgical intervention. Three late complications (10%) also required surgery. There was no surgery-related mortality. CONCLUSION: TOGD is an effective procedure with an acceptably low complication rate for children with severe neurological impairment and is followed by a major improvement in general health and quality of life for children and families. There was no substantial difference in outcome between Primary and "Rescue" procedures.