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Abstract

Background

The authors have reported a newly recognized hypoglyacemic disorder, non-insulinoma pancreatogenous hypoglycaemia syndrome (NIPHS), which is characterized by postprandial hyperinsulinaemic hypoglycaemia, negative 72-h fast, negative transabdominal and intraoperative ultrasonography, spiral computed tomography and coeliac axis angiography, positive selective arterial calcium stimulation test (SACST), absence of insulinoma but presence of islet hypertrophy and nesidioblastosis, and relief of symptoms from gradient-guided partial pancreatectomy. Experience with the next five patients with this disorder (6–10; 1998–1999) compared with the original five (1–5; 1996–1998) is reported.

Results

Wide age range, male predominance, postprandial occurrence of symptoms, negative radiological localization, nesidioblastosis/islet hypertrophy, but no insulinoma characterized all ten cases. Response to SACST indicated widespread islet hyperfunction in patients 1 and 5; limitation to distribution of the SPA and GDA in patients 3, 4, 6 and 8, to distribution of the SPA in patients 2, 7 and 9, and to distribution of the SMA in patient 10. Except for patient 5, pancreatic resection to the left of the SMV was performed when only the SPA was positive on the SACST. Patients 5 and 8 have had recurrence of symptoms; they resolved within 3 months for patient 5.

Patient12345678910
  • *

    Superior mesenteric artery (SMA) supplied the whole pancreas. SPA, splenic artery; GDA, gastroduodenal artery; SMV, superior mesenteric vein; pc, postprandial hyperinsulinaemic hypoglycaemia; R, right of; L, left of

SexMMMMFMMFMF
Age (years)37167272786429432828
Symptomspcpcpcpcpcpcpcpcpcpc
SACST positive in
 SPA++++ ++++ 
 GDA+ ++ + +  
 SMA+   +*    +
Surgical resection in relation to SMVRLRRLRLRLR

Conclusion

NIPHS can usually be treated successfully with gradient-guided partial pancreatectomy. © 2000 British Journal of Surgery Society Ltd