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.

Long-term Outcome of Transjugular Intrahepatic Portosystemic Shunt in Children With Portal Hypertension.

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.

Results of a stepwise approach to extrahepatic portal vein obstruction in children.

BACKGROUND: The management of extrahepatic portal vein obstruction (EHPVO) in children is controversial. We report our experience with a prospective evaluation of a stepwise protocol based on severity of portal hypertension and feasibility of mesoportal bypass (MPB). METHODS: After diagnosis, children with EHPVO underwent surveillance endoscopies and received nonselective ß-blockers (NSBBs) or endoscopic variceal obliteration (EVO) when large varices were detected. In patients who failed NSBBs and EVO, we considered MPB as first-line and shunts or transjugular intrahepatic portosystemic shunt (TIPS) as second-line options. RESULTS: Sixty-five children, median age 12.5 (range 1.6-25.8), whose age at diagnosis was 3.5 (0.2-17.5) years, were referred to our unit. Forty-three (66%) had a neonatal illness, 36 (55%) an umbilical vein catheterisation. Thirty-two (49%) presented with bleeding at a median age of 3.8 years (0.5-15.5); during an 8.4-year follow-up period (1-16), 43 (66%) had a bleeding episode, 52 (80%) were started on NSBBs, 55 (85%) required EVO, and 33 (51%) required surgery or TIPS. The Rex recessus was patent in 24 of 54 (44%), negatively affected by a history of umbilical catheterisation (P = 0.01). Thirty-four (53%) patients underwent a major procedure: MPB (13), proximal splenorenal (13), distal splenorenal (2), mesocaval shunt (3), TIPS (2), and OLT (1). At the last follow-up, 2 patients died, 53 of 57 (93%) are alive with bleeding control, 27 of 33 (82%) have a patent conduit. CONCLUSIONS: 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.

Successful bleeding control by a combined conventional surgical approach and video-assisted surgery: a case report.

The use of central venous catheters (CVCs) nowadays is a routine practice in the treatment of severely acute-diseased children. However, the procedure still carries a risk of morbidity, and severe complications are reported. When respiratory and/or hemodynamic instability develop after the procedure, prompt patient evaluation to exclude iatrogenic damage is mandatory, regardless of the primary patient condition. If a vascular injury related to CVC placement procedure is detected, the availability of an interventional radiologist and/or any surgical facilities plays an important role in the management of this life-threatening complication. We report the case of a 12-year-old boy hospitalized in the Pediatric Intensive Care Unit of our hospital for a severe motorveicle accident, who, about 30 minutes from the percutaneous CVC placement, developed tachycardia, hypoxemia, and hypotension. A chest X-ray confirmed the right positioning of the catheter, the presence also of a large left hemothorax. Interventional radiology took place, but it failed to stop the bleeding. Urgent anterolateral thoracotomy was performed while the patient was kept in a supine position because of a cervical spine luxation. During surgery, bleeding was found coming from the thoracic dome and because of a tear next to the left subclavian artery. Access to that area was technically difficult; after blood and clots were removed, multiple attempts to obtain the hemostasis failed, and definitive control of the hemorrhage was achieved only by video-assisted thoracic surgery (VATS). The postoperative period was uneventful. In this study, the authors discuss the management of this kind of complication and the value of a combined surgical approach (conventional, with a minimal access surgery procedure such as VATS) in the treatment of thoracic vascular injuries related to the insertion of a percutaneous CVC. To the best of our experience, this is the first time in which this combination of procedures has been reported in the literature.