Objectives: A proportion of children with chronic liver disease have severe portal hypertension (PH) and a preserved synthetic and biliary function. In our institution these children have been managed with transjugular intrahepatic portosystemic shunts (TIPS). We aimed to evaluate the long-term patency of TIPS placed in pediatric patients with PH. Methods: Retrospective study of children who underwent TIPS in the last 15 years. We compared patients with cirrhotic PH to those with noncirrhotic PH, and all with an historical cohort of children who underwent a surgical portosystemic shunt. Kaplan-Meier analysis measured long-term shunt patency. Results: Twenty-nine patients were recorded (cirrhotic PH = 11, noncirrhotic PH  = 18, mean age 10.3 years[±4.3], mean weight 36.7 kg [±20.1], mean pediatric end-stage liver disease score 4.1 [±7.1]); in 5 TIPS was placed after split liver transplantation. Indication for TIPS was variceal bleeding in 18, refractory ascites in 11. Primary patency rates at 6 months and at 1, 2, and 4 years were 91%, 83%, 60%, and 46%, respectively. At last follow-up (mean of 2.8 years [±2.4, range 0.1–8.1 years]) secondary patency (after radiological revision) was 100%. The patency rate of the historical cohort of patients who underwent a surgical portosystemic shunt was 26 of 31 (82%) at a median follow-up of 12.5 years (1.6–25.8). Conclusion: TIPS appears to have a high mid-term patency rate, especially if monitored and revised. Its high clinical success rate, along with a minimally invasive approach, suggests that in this setting TIPS should not be regarded only as a bridge to liver transplantation.

Children with EHPVO have a high rate of bleeding episodes early in life. A stepwise approach comprising of medical, endoscopic, and surgical options provided excellent survival and bleeding control in this population.

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.

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