robotic-central-pancreatectomy-with-roux-en-y-pancreaticojejunostomy

Robotic Central Pancreatectomy with Roux-en-Y Pancreaticojejunostomy

Central pancreatectomy is a parenchyma-sparing alternative to distal pancreatectomy in patients with a benign or low-grade malignant tumor in the body of ...

Emma Children's Hospital

Choledochal malformations (CMs) are increasingly diagnosed antenatally. There is a dilemma between early surgery to prevent CM-related symptoms and postponing surgery to reduce complications. We aimed to identify the optimal timing of surgery in asymptomatic neonates with antenatally diagnosed CM and to identify predictors for development of symptoms.

The timing of surgery of antenatally diagnosed choledochal malformations: A descriptive analysis of a 26-year nationwide cohort.

A choledochal malformation (CM) is a rare entity, especially in the Western world. We aimed to determine the incidence of CM in the Netherlands and the outcome of surgery for CM in childhood.

Choledochal Malformation in Children: Lessons Learned from a Dutch National Study.

ABCC8 and KCJN11 mutations cause the most severe diazoxide-resistant forms of congenital hyperinsulinism (CHI). Somatostatin analogues are considered as second-line treatment in diazoxide-unresponsive cases. Current treatment protocols include the first-generation somatostatin analogue octreotide while pasireotide, a second-generation somatostatin analogue, may be more effective in reducing insulin secretion. Herein we report the first off-label use of pasireotide in a boy with severe therapy resistant form of CHI due to a homozygous ABCC8 mutation. After partial pancreatectomy, hyperinsulinism persisted and in an attempt to prevent further surgery, off-label treatment with pasireotide was started. Short-acting pasireotide treatment caused high blood glucose levels shortly after the injections. Long-acting pasireotide treatment resulted in more stable glycemic control. No side effects (e.g. central adrenal insufficiency) were noticed during a two-month treatment period. Because of recurrent hypoglycemia despite a rather high carbohydrate intake the boy finally underwent a near-total pancreatectomy at the age of 11 months. In conclusion, pasireotide treatment slightly improved glycemic control without side effects in a boy with severe CHI. However, the effect of pasireotide was not sufficient to prevent near-total pancreatectomy in this case of severe CHI.

Article	Year
The timing of surgery of antenatally diagnosed choledochal malformations: A descriptive analysis of a 26-year nationwide cohort. Journal of pediatric surgery\| PubMed ID: 28318597	2017
Choledochal Malformation in Children: Lessons Learned from a Dutch National Study. World journal of surgery\| PubMed ID: 28589237	2017
First report on the use of pasireotide in a case of severe congenital hyperinsulinism due to a homozygous ABCC8 mutation. Annals of pediatric endocrinology & metabolism\| PubMed ID: 33971706	2021

Matthijs W.N. Oomen

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