Background: Intestinal failure (IF) is the reduction in functioning gut mass below the minimal level necessary for adequate digestion and absorption of nutrients and fluids for weight maintenance in adults or for growth in children. There is a paucity of epidemiologic data on pediatric IF. The purpose of this study was to determine the prevalence, incidence, regional distribution and underlying diagnosis of pediatric chronic IF (CIF) requiring home parenteral nutrition (HPN) in Italy. Methods: Local investigators were selected in 19 Italian centers either of reference for pediatric HPN or having pediatric gastroenterologists or surgeons on staff and already collaborating with the Italian Society for Pediatric Gastroenterology, Hepatology and Nutrition with regard to IF. Data requested in this survey for children at home on Parenteral Nutrition (PN) on 1 December 2016 included patient initials, year of birth, gender, family’s place of residence and underlying diagnosis determining IF. Results: We recorded 145 CIF patients on HPN aged ≤19 years. The overall prevalence was 14.12/million inhabitants (95% CI: 9.20–18.93); the overall incidence was 1.41/million inhabitant years (95% CI: 0.53–2.20). Conclusion: Our survey provides new epidemiological data on pediatric CIF in Italy; these data may be quantitatively useful in developing IF care strategy plans in all developed countries.

Hepatoblastoma with tumour thrombi extending into inferior-vena-cava and right atrium are often unresectable with an extremely poor prognosis. The surgical approach is technically challenging and might require major liver resection with vascular reconstruction and extracorporeal circulation. However, which is the best surgical technique is yet unclear. A 11-months-old boy was referred for a right hepatic lobe mass(90×78mm) suspicious of hepatoblastoma with tumoral thrombi extending into the inferior-vena-cava and the right atrium, bilateral lung lesions and serum alpha-fetoprotein level of 50.795IU/mL. After 8 months of chemotherapy (SIOPEL 2004-high-risk-Protocol), the lung lesions were no longer clearly visible and the hepatoblastoma size decreased to 61×64mm. Thus, ante situm liver resection was planned: after hepatic parenchymal transection, hypothermic cardiopulmonary bypass was started and en bloc resection of the extended-right hepatic lobe, the retro/suprahepatic cava and the tumoral trombi was performed with concomitant cold perfusion of the remnant liver. The inferior-vena-cava was replaced with an aortic graft from a blood-group compatible cadaveric donor. The post-operative course was uneventful and after 8 months of follow-up the child has normal liver function and an alpha-fetoprotein level and is free of disease recurrence with patent vascular graft. We report for the first time a case of ante situ liver resection and inferior-vena-cava replacement associated with hypothermic cardiopulmonary bypass in a child with hepatoblastoma. Herein, we extensively review the literature for hepatoblastoma with thumoral thrombi and we describe the technical aspects of ante situm approach, which is a realistic option in otherwise unresectable hepatoblastoma.

Primary ovarian torsion (POT) affects a morphologically normal ovary without associated adnexal lesions and that carries a high recurrence risk. The role of oophoropexy in preventing POT recurrence remains unclear. To evaluate the effectiveness of oophoropexy in reducing recurrence rates after the first episode of POT. A systematic review and meta-analysis was conducted following PRISMA 2020 guidelines. Retrospective studies including girls <18 years undergoing surgery for POT were considered. The primary outcome was torsion recurrence. Study quality was assessed using the MINORS tool. Twelve studies (209 patients; mean age 7.8 ± 4.1 years) met inclusion criteria. Detorsion alone was performed in 168 patients (80.4 %) and detorsion + oophoropexy in 41 patients (19.6 %). Recurrence occurred in 17.3 % of patients after ovarian detorsion alone versus 4.9 % after detorsion + oophoropexy. Three comparative studies (106 patients) were included in the meta-analysis. Pooled analysis showed a trend toward reduction in recurrence for oophoropexy (OR 0.54, 95 % CI 0.17-1.72, p = 0.15, I² = 0 %). Ultrasonography demonstrated preserved ovarian vascularization and trophism in most patients. POT carries a substantial risk of recurrence. Oophoropexy may reduce recurrence compared with detorsion alone without negatively affecting ovarian trophism, although statistical significance was not reached. At present, oophoropexy cannot yet be recommended as routine after a first episode of POT, but it appears to hold promise as a strategy to reduce recurrence.