Pancreatectomy (removal of a portion or nearly all of the pancreas) is usually a treatment of last resort when the simpler medical measures fail to provide prolonged normal blood sugar levels. For many decades, the most common surgical procedure was removal of about 95% of the pancreas. This cured some infants but not all, and some needed second procedures to remove even the last remnants. Insulin-dependent diabetes mellitus commonly develops, though in many cases it occurs many years after the pancreatectomy.

In the early 1990s it was discovered that a sizeable minority of cases of Kir and SUR mutations were focal, involving overproduction of insulin by only a portion of the pancreas. These cases can be cured by removing much less of the pancreas, resulting in excellent outcomes with no long-term problems. The initial pancreatectomy became a prolonged process of repeated microscopic examination of small pieces removed from various parts of the pancreas while the infant was under anesthesia in the operating room, with an experienced surgeon and pathologist attempting to identify a focal region of overactive cells which could be completely removed.

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