>> OUR DAUGHTER CAROLINE WAS BORN ON JUNE 13TH,
AT CENTRAL BAPTIST HOSPITAL IN LEXINGTON,
KENTUCKY.
SHE WAS PRONOUNCED HEALTHY AND WE WERE DISCHARGED FROM
THE HOSPITAL AND GETTING READY TO GO HOME WHEN A VERY
ASTUTE NURSE NOTICED AND THOUGHT THAT CAROLINE
SEEMED COLD AND A LITTLE BIT LETHARGIC.
>> WE WERE WORRIED ABOUT THE USUAL THINGS THAT YOU WORRY
ABOUT, YOU KNOW, YOU WANT THEM TO BE HEALTHY AND YOU
WANT THEM TO HAVE 10 FINGERS AND 10 TOES,
BUT WE HAD NEVER HEARD ABOUT HYPERINSULINISM.
WE HAD BEEN VERY WELL PREPARED AND WELL BRIEFED
THE DAY OF THE SURGERY.
DR. ADZICK HAD TALKED WITH US THE DAY BEFORE.
WE FELT LIKE WE UNDERSTOOD WHAT WAS GOING TO BE
HAPPENING.
>> A SURGICAL APPROACH AT THE BEGINNING IS THE SAME
WITH EITHER FOCAL DISEASE OR DIFFUSE DISEASE.
A LAPAROTOMY IS PERFORMED IN A BABY WHO'S UNDER GENERAL
ANESTHESIA BY ONE OF OUR EXPERT PEDIATRIC
ANESTHESIOLOGISTS.
A SIDEWAYS WOUND IS MADE ABOVE THE LEVEL OF THE BELLY
BUTTON AND THE PANCREAS IS EXPOSED.
THE PANCREAS HAS A HEAD, A NECK,
A BODY AND A TAIL.
I EXAMINE THE PANCREAS USING MAGNIFYING GLASSES THAT
MAGNIFY THINGS BY FACTOR OF FOUR.
AND SOMETIMES THERE ARE LITTLE VISUAL HINTS AS TO
WHERE A FOCAL LESION MIGHT BE.
I ALSO PALPATE THE PANCREAS BECAUSE FOCAL LESIONS TEND
TO BE A BIT FIRMER THAN SURROUNDING NORMAL
PANCREATIC TISSUE.
IF THERE ARE NO ABNORMAL FINDINGS ON INSPECTION
OR PALPATION, I DO THREE BIOPSIES.
ONE FROM THE HEAD, ONE FROM THE BODY
AND ONE FROM THE TAIL OF THE PANCREAS.
>> WHEN YOU HAVE A FOCAL LESION,
THE REST OF YOUR PANCREAS IS NORMAL.
WHEN THERE'S DIFFUSE DISEASE,
ALL THE CELLS, ALL THE BETA CELLS IN THE PANCREAS,
ARE ABNORMAL.
SO THERE WILL BE MULTIPLE BIOPSIES TAKEN JUST
TO BE SURE.
>> IT'S ONLY BY PATHOLOGICAL EXAMINATION WE CAN RECOGNIZE
WHAT PORTION OF THE PANCREAS IS EXACTLY ABNORMAL.
AND THE SURGEON WILL, IN TURN, BE ABLE TO REMOVE
THE ABNORMALITY.
>> WHERE THE ARROW IS, THESE ARE THE ISLETS, OK.
SO THIS IS NORMAL.
AND THIS IS ABNORMAL.
>> THE INTERACTION BETWEEN THE SURGEON AND THE PATHOLOGIST
IN THE OPERATING ROOM IS CRUCIAL.
BECAUSE ONE WANTS ACCURATE INFORMATION GIVEN
TO THE PATHOLOGIST.
ONE WANTS THE FROZEN SECTION DIAGNOSES TO BE PROMPT
BECAUSE THE BABY IS ANESTHETIZED.
AND ACTUALLY WE HAVE THINGS ARRANGED IN THE OPERATING
ROOM SUCH THAT THERE'S A LARGE SCREEN ONTO WHICH
THE PATHOLOGIC SECTIONS CAN BE PROJECTED FROM PATHOLOGY AN
ENTIRE FLOOR AWAY SO THAT WE CAN IN REAL TIME DISCUSS
WHAT THE FINDINGS ARE.
>> FOR BABIES WITH FOCAL DISEASE IF THERE IS NOT
A VISIBLE OR PALPABLE HINT AS TO WHERE THE FOCAL LESION
IS, NOW WITH PREOPERATIVE PET SCANNING,
I KNOW WHERE TO ZERO IN.
FOCAL LESIONS CAN BE AS SMALL AS TWO OR THREE
MILLIMETERS IN SIZE SO SOMETIMES IT CAN BE TRICKY,
EVEN WITH THE PREOPERATIVE INFORMATION, TO FIND IT.
A FOCAL LESION IN THE TAIL OR THE BODY OR THE NECK OF
THE PANCREAS IS STRAIGHT FORWARD TO EXCISE AND MAY
INVOLVE TAKING OFF THE END OF THE PANCREAS ALONG WITH
THE FOCAL LESION OR JUST THE FOCAL LESION ITSELF IF IT'S
NOT CLOSE TO THE MAIN PANCREATIC DUCT WHICH IS
SORT OF THE PIPELINE THROUGH WHICH PANCREATIC SECRETIONS
RUN.
AND WE DON'T WANT TO CAUSE AN INJURY TO THAT DURING
THE OPERATION.
FOCAL LESIONS IN THE HEAD OF THE PANCREAS ARE MORE
COMPLEX BECAUSE THERE'S ALL THIS IMPORTANT TRAFFIC THAT
GOES THROUGH OR AROUND THE PANCREATIC HEAD.
THE PORTAL VEIN IS BEHIND IT.
THE SUPERIOR MESENTERIC ARTERY IS BEHIND IT.
THE COMMON BILE DUCT PASSES THROUGH IT.
THE ARTERIAL BLOOD SUPPLY TO THE DUODENUM IS SHARED.
SO IT'S A VERY DELICATE, VERY FINE DISSECTION AND THE WAY
THINGS WORK, ALL THE DRAINAGE OF THE PANCREATIC
SECRETION AND ENZYMES OCCURS FROM THE TAIL TOWARD THE
HEAD AND TO THE DUODENUM.
SO IF YOU TAKE A FOCAL LESION OF THE HEAD OUT
AND HAVE TO TAKE OUT THE PANCREATIC DUCT IN THAT
PORTION, THEN WE'VE LEARNED THAT IT'S IMPORTANT TO SAVE
THE BODY AND THE TAIL OF THE PANCREAS.
BRING UP A LOOP OF BOWEL, THE MEDICAL TERM
IS ROUX-EN-Y LOOP, SO THAT THOSE PANCREATIC SECRETIONS
CAN DRAIN INTO THE INTESTINE WHERE THEY BELONG.
AND SO WE PRESERVE THE NORMAL ISLET CELLS AND THAT
NORMAL PORTION OF THE PANCREAS.
>> OUR SURGEON HAS DONE OVER 400 PANCREATECTOMIES SO HE
HAS THE MOST EXPERIENCE IN THE WORLD.
>> IT'S A WONDERFUL FEELING TO LEAVE THE OPERATING ROOM
HAVING DONE SOMETHING THAT WILL HAVE A HUGE IMPACT ON
A CHILD'S LIFE AND ON THAT FAMILY.
YOU READY FOR SOME GOOD NEWS?
>> I'M READY FOR SOME GOOD NEWS.
SHE HAD INDEED HAD A FOCAL LESION THAT WAS IN THE TAIL
OF HER PANCREAS, WHICH WAS THE MOST UNLIKELY PLACE FOR
IT TO BE, AND IT'S THE BEST PLACE TO HAVE A LESION
BECAUSE IT'S NOT ATTACHED TO ANYTHING ELSE.
I REMEMBER HIM SAYING THAT THERE WAS EVERY REASON
TO BELIEVE THAT CAROLINE WOULD BE CURED.
>> APPROXIMATELY HALF OF THE CHILDREN THAT REQUIRE
SURGERY HAVE THIS FOCAL FORM OF HYPERINSULINISM.
THE OTHER HALF HAVE DIFFUSE HYPERINSULINISM.
>> THOSE THAT HAVE DIFFUSE DISEASE,
WE CAN REDUCE THE AMOUNT OF PANCREAS BY SURGERY,
BUT WE CAN'T CURE IT COMPLETELY.
>> IF THERE ARE ABNORMAL FINDINGS CONSISTENT WITH
DIFFUSE DISEASE ON EACH OF THE THREE BIOPSIES,
THEN A NEAR TOTAL PANCREATECTOMY IS DONE,
SAVING ONLY A TINY NUB IN THE PANCREAS BETWEEN
THE DUODENUM AND THE COMMON BILE DUCT.
THIS HOPEFULLY WILL HELP IN THE MANAGEMENT
OF THE DISEASE IN TERMS OF PREVENTION OF BRAIN
THREATENING HYPOGLYCEMIA, BUT IT'S REALLY NOT A CURE.
SOME BABIES DO QUITE WELL AND DON'T NEED MUCH
IN THE WAY OF ADDITIONAL MEDICAL THERAPY.
OTHERS CAN HAVE RECURRENT HYPOGLYCEMIA AND NEED
ADDITIONAL MEDICAL THERAPY AND THERE'S A THIRD GROUP
THAT DEVELOPS INSULIN- DEPENDENT DIABETES.