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Congenital hyperinsulinism and surgical outcome in a single tertiary center in Brazil
Raphael Del Roio Liberatore1, Isabella Christina Mazzaro Monteiro1, Flavio de Oliveira Pileggi2
1Universidade de São Paulo, Faculdade de Medicina de Ribeirão Preto, Divisão de Endocrinologia Pediátrica e Departamento de Puericultura e Pediatria, Ribeirão Preto, SP, Brazil.
Insights
Congenital hyperinsulinism (CHI) is a rare genetic disorder causing hypoglycemia. Surgical management in a tertiary center resulted in 100% survival, with minimally invasive surgery showing shorter hospital stays.
Area of Science:
- Pediatric Endocrinology
- Surgical Gastroenterology
- Genetics
Background:
- Congenital hyperinsulinism (CHI) is a genetic disorder of pancreatic beta-cell dysfunction leading to persistent hypoglycemia in infants.
- Severe cases unresponsive to medical management often necessitate surgical intervention, typically near-total pancreatectomy.
Purpose of the Study:
- To evaluate clinical and surgical outcomes impacting survival in infants diagnosed with congenital hyperinsulinism.
- To assess the effectiveness of different surgical approaches in managing CHI.
Main Methods:
- Retrospective cohort study conducted at a single university tertiary care center.
- Analysis of demographic, clinical, laboratory, and surgical data from CHI patients.
- Comparison of outcomes between open and minimally invasive surgery (MIS).
Main Results:
- A cohort of infants with CHI presented with hypoglycemia shortly after birth, requiring timely surgical intervention.
- Minimally invasive surgery (MIS) was associated with a significantly shorter length of hospital stay compared to open procedures (p < 0.05).
- Overall survival rate in the studied cohort was 100%.
Conclusions:
- Congenital hyperinsulinism requires management in a multidisciplinary, tertiary care setting due to its complexity.
- Surgical intervention, particularly MIS, offers favorable outcomes for CHI patients.
- Laparoscopic approaches are recommended as the preferred surgical option for managing CHI.
Objective:
Congenital hyperinsulinism (CHI) is a heterogeneous genetic disease characterized by increased insulin secretion and causes persistent hypoglycemia in neonates and infants due to dysregulation of insulin secretion by pancreatic β cells. Babies with severe hypoglycemia and for whom medical treatment has been ineffective usually require surgical treatment with near-total pancreatectomy. To evaluate the clinical and surgical aspects affecting survival outcomes in babies diagnosed with CHI in a single tertiary care center.
Methods:
Retrospective Cohort study involving a single university tertiary center for the treatment of CHI. The authors study the demographics, clinical, laboratory, and surgical outcomes of this casuistic.
Results:
61 % were female, 39 % male, Birth weight: 3576 g (±313); Age of onset of symptoms: from the 2nd hour of life to 28 days; Time between diagnosis and surgery ranged between 10 and 60 days; Medical clinical treatment, all patients received glucose solution with a continuous glucose infusion and diazoxide. 81 % of the patients used corticosteroids, 77 %. thiazide, 72 % octreotide, 27 % nifedipine; Neurological sequelae during development and growth: 54 % had some degree of delay in neuropsychomotor development, 27 % obesity. Surgery was performed open in 6 and 12 minimally invasive surgery (MIS).
Histopathology:
2 focal and 16 diffuse, Length of stay (days) was lower in MIS (p < 0.05). Survival was 100 %.
Conclusions:
CHI is a rare and difficult-to-manage tumor that must be performed in a multidisciplinary and tertiary center. Most surgical results are good and the laparoscopic approach to disease has been the best choice for patients.
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