New Approach to Surgery for Some Diffuse Patients

A surgeon in Germany, Dr. Winfried Barthlen, has successfully treated patients with diffuse congenital hyperinsulinism (HI) by removing only 40% of the pancreas rather than 95% or more, which has been the standard for some time. By removing only 40 percent of the pancreas, Dr. Barthlen is hopeful that his patients will not develop diabetes. These results will be published very soon but Dr. Barthlen was kind enough to give us a sneak preview of his results prior to publication.

Dr. Barthlen of Griefswald, Germany, explains that the patients who have successfully undergone these 40% partial pancreatectomies are part of a sub-set of patients who have diffuse HI. He writes that they have the “so-called atypical segmental mosaic congenital hyperinsulinism. This means that one or few local acculumations of pathological islets are side by side with almost normal pancreatic tissue” within the same pancreas. Using a PET-CT, he is able to locate where these “hot spots” on the pancreas are likely to be. Dr. Barthlen only operates on patients that cannot be well managed with medication.

During surgery, Dr. Barthlen performs biopsies to locate the cells. Only then does he surgically remove the diseased cells, leaving the healthy ones intact. Dr. Barthlen believes that if 60% of the pancreas remains, the HI patients will not become diabetic in puberty. Dr. Barthlen adds that “in most cases the surgery can be performed by laparoscopy so that scars are minimal.” Dr. Barthlen explains that this type of surgery would not be effective for patients who have pathological cells throughout their pancreas but he does believe that further research will show that there are a fair number of patients with atypical segmental mosaic congenital hyperisulinism.

In February, 2011, Dr. Barthlen was Guest Editor, along with Dr. Pascale De Lonlay, a member of the Congenital Hyperinsulinism International Advisory Group, of the periodical, “Seminars in Pediatric Surgery.” The entire issue was devoted to HI and many members of our Advisory Group published in the periodical. We have linked it to the website. There is a wealth of material there.

In addition to serving as Director of Pediatric Surgery in Greifswald, Dr. Barthlen is a professor of Surgery. He has been working in the field of HI since 2002 when he was at the University Hospital in Berlin. We will invite Dr. Barthlen to present at the Congenital Hyperinsulinism International Family Conference which will take place in Philadelphia, Pennsylvania March 17 and 18th, 2012. I hope he can make it!

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