Center for hyperinsulinism Greifswald

 

Greifswald pediatric surgery is the surgical partner for COACH (Collaborative Alliance on Congenital Hyperinsulinism). This is a Germany-wide network of specialists dedicated to hyperinsulinism:

Here you can find all the most up-to-date information about hyperinsulinism. Please feel free to email one of our experts directly (it just takes a mouse click) and you will receive an answer in short order.

 

Philosophy of treating hyperinsulinism with surgery

  • Every operation invades a child´s bodily integrity. It is only justified - evenin legal terms - if the benefits to be gained far outweigh the risks. This weighing up of pros and cons is based on the physician´s accumulated knowledge and experience and on the ethical maxim: treat every child as if it were your own. 

 

In the case of hyperinsulinism this means:

 

 

Focal Form:

  • Surgical removal of the focus is always indicated here, regardless of where it is situated on the pancreas. Solely the small focus, which usually ranges in size from 5 to 12 mm, secretes excess insulin. If it is removed completely, the child is cured. The entire remaining pancreas is completely healthy and remains intact. Often, a focus will have small offshoots and microsatellites in the immediate vicinity; seldom is it very large (several cm), very rarely are there two foci. Thanks to PET-CT, where to find the focus is known down to the millimeter before the operation. Intraoperative ultrasound and laser optical coherence tomography, which can reveal the extent of the focus and its branches, are also deployed during the operation.  Multiple, precise tissue examinations during every stage of the operation and tightly coordinated feedback from pathology ensure that only diseased tissues are removed. But the removal must be absolutely total: if focus parts as small as the head of a pin remain they can prevent a cure. At the same time, we try not to touch the pancreatic and the bile ducts, which we succeed in doing in most cases. However, if a duct system runs right through the focus, opening it is unavoidable. Reconstructive procedures used in adult surgery for draining off digestive juices permit normal eating and quality of life later on. Except for foci that are located in the pancreatic head, these operations today are minimally invasive, being done laparoscopically. They leave practically no accretions behind in the abdominal cavity and only minimal scars on the skin. The children recover very quickly from the operation and are already active again on the 2nd day. These operations usually last several hours, since all focal tissues must be sought out and carefully removed under continual control by pathology. Thanks to modern child anesthesia, this no longer presents a problem today.

 

Diffuse Form:

  • Until early this century, from 95% to 98% of the pancreas was removed in these cases, often already during the child´s first months of life. Diagnostic and medication capabilities were not as good as they are today. The hypoglycemia could be overcome by these resections, but at a high price: as we now know, most of the children operated on developed diabetes mellitus by puberty at the latest, which is very difficult to treat. Hence today, management of the diffuse form, in which all pancreatic island cells uniformly produce insulin to excess, is predominantly based on drug therapy. Operations are only indicated here if, despite optimal care, severe hypoglycemias occur over and over again or the medication´s side effects are not tolerated. In Greifswald, no longer is almost the entire pancreas removed even then, since a marked improvement in the blood glucose situation can be attained with just a limited resection. Restraint is indicated here today.

 

Atypical Forms:

  • We discovered only recently that the old breakdown between focal and diffuse does in all likelihood not correspond to reality. It turns out that there are atypical mosaic forms in which different regions of the pancreas are affected differently: areas with a high density of sick cells alternate with areas in which the island cells appear scarcely changed or even are quite healthy.
    Clues to the presence of such "leopard patterns" can be found with PET-CT with appropriate techniques and experience. But this requires close collaboration among nuclear medicine specialists, radiologists, pediatric endocrinologists, pediatric surgeons and pathologists. Professor Barthlen personally attends every PET-CT examination, since especially during the contrast medium´s flow, that is to say, while the PET-CT images are being acquired dynamically, a great deal of important information is to be obtained that can potentially be decisive for a successful operation.

    Such detailed differentiation is so important because an option exists when it comes to surgically treating the atypical mosaic form: a marked improvement in the blood glucose situation can be obtained with sparing surgical removal of only the most highly diseased areas. Even complete cures have been described.

     

     

    Our Strategy for Pediatric Surgery:

  • In cases where an adequate quality of life cannot be achieved with medications and where constant glucose fluctuations lead to increasing concern for the child´s well-being, the following course of action may be offered:
    We will gladly consult with your by phone or email. You can contact us anytime. We will assist with problems related to billing the medical insurance provider and arranging transportation. We facilitate an appointment for the PET-CT in the Diagnostic-Therapeutic Center at Frankfurter Tor. The child and its parents are housed 2 days in the Spandau Forest Hospital (Managing Director: PD Dr. Frank Jochum). Dr. Stephan-Matthias Reyle-Hahn, an experienced pediatric anesthesiologist, oversees the anesthesia. Professor Barthlen is present in person during the entire PET-CT examination.
    Already by evening of the examination day we will be able to tell you if surgical treatment options exist for your child and what they are. In discussing every detail with you, we will lay out for you in an open and Frank manner the pros and cons, the risks and odds involved in going ahead. We, together with Professor Dr. Klaus Mohnike of the Magdeburg Pediatric Uniclinic, have at our disposal the most modern menu of medicinal and surgical alternatives for treating hyperinsulinism, developed in large part by us (please see the publications list). We consider it to be very important to inform the parents accurately so that they can give their "informed consent", for it is the parents who must or will handle the situation at home with the child afterwards.
    If a surgical intervention holds promise, the next step is to take tiny tissue samples with a laparoscope from several places on the pancreas and to examine them immediately. If all island cells are uniformly affected in all areas, a purely diffuse form exists. This puts it into the domain of treatment with medication means we will resort to drug therapy, and the operation is ended. The scars left from the laparoscopy are minute; surgical complications, such as bleeding or accretions, are extremely rare, given appropriate technique and experience. Parents come away certain that there is no surgical treatment option holding the promise of success, which makes it easier for many to live with the constant blood glucose testing and the medication´s side effects.
    If, on the other hand, areas with high and low densities of pathological island cells are detected on the pancreas, an atypical mosaic form exists. While still under the same anesthesia, next only the most conspicuous area of the pancreas is removed. The largest part of the gland remains as is. In most cases this, too, can be accomplished with minimal invasiveness, i.e. laparoscopically. The success of such a limited removal of pancreatic tissue in the case of atypical mosaic forms however cannot be predicted with certainty: we have no way of knowing at present how the island cells in the remaining pancreas will behave. Preliminary experiences, however, are encouraging: there are children who do exhibit completely stable blood glucose levels and no longer require any kind of medication. Diabetes later on during puberty is not to be expected for them, since their pancreas was left intact for the most part.

    To date unfortunately there is no method available anywhere in the world other than surgical biopsy for differentiating the purely diffuse from the atypical forms. COACH, through molecular biological and genetic experiments, is intensively working on identifying prognostic markers that will let us predict the success of surgical intervention in cases of diffuse and atypical forms of congenital hyperinsulinism.

    Options for treating hyperinsulinism using medication or surgery are undergoing change. Treatment must be individualized; in the pediatric surgery center we adapt it to fit the child.