Surgery in congenital hyperinsulinism-tips and tricks not only for surgeons. A practical guide

Area of Science:

• Pediatric Surgery
• Endocrinology
• Genetics

Background:

• Congenital hyperinsulinism (CHI) presents in focal, diffuse, and atypical forms.
• Each CHI subtype necessitates distinct surgical approaches and techniques.
• Accurate diagnosis and subtype classification are crucial for effective management.

Purpose of the Study:

• To outline the specific surgical strategies for each form of congenital hyperinsulinism (CHI).
• To emphasize the importance of tailored surgical techniques based on CHI subtype.
• To highlight the challenges and uncertainties in surgical management of diffuse and atypical CHI.

Main Methods:

• Positron emission tomography/computed tomography (PET/CT)-guided local resection for focal CHI.
• Conservative management as the primary approach for diffuse CHI.
• Careful surgical decision-making in atypical CHI cases to avoid overtreatment.

Main Results:

• Focal CHI resection preserves healthy pancreatic tissue by targeting the lesion precisely.
• Diffuse CHI management prioritizes medical therapy; surgery is a last resort for persistent hypoglycemia.
• The optimal extent of resection for diffuse CHI to cure hyperinsulinism without causing diabetes remains unknown, leading to unpredictable outcomes.
• Atypical CHI requires meticulous surgical judgment to prevent unnecessary extensive resections.

Conclusions:

• Surgical management of CHI must be individualized based on focal, diffuse, or atypical presentation.
• Preserving pancreatic function is a key consideration, especially in focal and diffuse CHI.
• Further research is needed to define the precise surgical indications and extent for diffuse CHI to balance efficacy and complication risks.