Long-term outcome of pancreatic function following oncological surgery in children:Institutional experience and review of the literature

BACKGROUND Pancreatic neoplasms are uncommon in children and in most cases they are benign or have low malignant potential.Pancreatoblastoma and solid pseudopapillary tumor are the most frequent types in early and late childhood,respectively.Complete resection,although burdened by severe complications,is the only curative treatment for these diseases.Pancreatic surgery may result in impaired exocrine and endocrine pancreatic function.However,limited data are available on the long-term pediatric pancreatic function following surgical resection.AIM To investigate endocrine and exocrine pancreatic function and growth after oncological pancreatic surgery in a pediatric series.METHODS A retrospective analysis of all pediatric patients who underwent surgery for pancreatic neoplasm in our Institution from January 31,2002 to the present was performed.Endocrine and exocrine insufficiency,auxological and fat-soluble vitamin status(A,D,E and clotting tests)were assessed at diagnosis and at every follow-up visit.Exocrine insufficiency was defined as steatorrhea with fecal elastase-1<200μg/g stool,while endocrine insufficiency was identified as onset of Diabetes or Impaired Glucose Tolerance.Growth was evaluated based on body mass index(BMI)z-score trend.RESULTS Sixteen patients(12 girls and 4 boys,mean age 10.7±5.3 years),were included.Nine patients(56%)had a neoplasm in the pancreatic head,4 in the body/tail,2 in the tail and 1 in the body.Histological findings were as follows:Solid pseudopapillary tumor in 10 patients(62.5%),insulinoma in 2 patients,neuroendocrine tumor in 2 patients and acinar cell carcinoma in 2 patients.The most frequent surgery was pancreaticoduodenectomy(50%).Exocrine failure occurred in 4 patients(25%)and endocrine failure in 2 patients(12.5%).Exocrine insufficiency occurred early(within 6 mo after surgery)and endocrine insufficiency later(8 and 10 years after surgery).Mean BMI z-score was 0.36±1.1 at diagnosis and 0.27±0.95 at the last assessment.Vitamin D was insufficient(<30 ng/mL)in 8 of the 16 patients during the follow-up period.Vitamins A,E and clotting test were into the normal ranges in all patients.CONCLUSION Careful and long-term monitoring should follow any pancreatic surgery,to recognize and promptly treat exocrine and endocrine pancreatic insufficiency,which can occur after surgery.

Combined endovascular-surgical treatment for complex congenital intrahepatic arterioportal fistula: A case report and review of the literature

BACKGROUND Congenital intrahepatic arterioportal fistula(IAPF) is a rare vascular malformation in infants that causes severe portal hypertension(PH) with poor prognosis if untreated. Currently, radiological embolisation is considered the first-line therapy for simple IAPF; however, it might be not resolutive for complex hepatic vascular lesions. When endovascular embolization is not sufficient to completely obliterate the IAPF, surgical intervention is needed, but it has been associated with severe morbidity and mortality in small children.Furthermore, indications are not defined.CASE SUMMARY We present the first case of a 6-month-old girl with trisomy 21 affected by a complex congenital IAFP, which caused severe PH, successfully treated with an endovascular-surgical hybrid procedure. The novel technique comprised a multistep endovascular embolisation, including a superselective transarterial embolisation of the afferent vessels and a direct transhepatic embolisation of the dilated portal vein segment, combined with selective surgical ligation of the arterial branches that supply the fistula, which were too small to be embolised.The complex IAPF was also associated with severe cholestasis and intra/extrahepatic biliary tree dilatation, which was successfully treated by a temporary biliary drainage. At 24-mo follow-up, the hybrid endovascularsurgical procedure achieved complete occlusion of the complex IAPF and resolution of the PH. A comprehensive review of the literature on congenital IAPF management, focussed on alternative treatment strategies, is also reported.CONCLUSION The combined radiological-surgical approach is a safe and effective treatment option for complex IAPF and avoids major invasive surgery.