>> FOR A SMALL- TO MODERATE-SIZE OMPHALOCELES AS LONG AS
THE BABY'S TESTING IS REASSURING AND THERE'S NO OBSTETRICAL
CONTRAINDICATIONS, THOSE ARE BABIES THAT ACTUALLY DO BETTER
WITH A VAGINAL DELIVERY.
BABIES WITH GIANT OMPHALOCELES OR OMPHALOCELES THAT CONTAIN
LIVER ARE A WHOLE DIFFERENT ISSUE.
>> IT IS IMPORTANT IN OUR VIEW FOR FETUSES THAT HAVE A LARGE OR
GIANT OMPHALOCELE IN WHICH THERE IS A PORTION OR MAJORITY OF THE
LIVER IN THE OMPHALOCELE SAC TO HAVE A PLANNED DELIVERY
BY CESAREAN SECTION
BECAUSE OF THE RISK OF DYSTOCIA OR DIFFICULTY IN THE DELIVERY
WITH A GIANT OMPHALOCELE, OR BECAUSE THE RISK OF DAMAGE TO
THE LIVER DURING THE VAGINAL DELIVERY.
>> CHARLIE WAS DELIVERED BY C-SECTION AND IMMEDIATELY RUSHED
OVER HERE TO CHILDREN'S HOSPITAL AND PUT IN THE NEONATAL I.C.U.
>> IN BABIES WITH OMPHALOCELES THEIR BOWEL AND LIVER
ARE COVERED WITH A SAC.
AND THAT SAC IS CRITICAL.
>> IF THE OMPHALOCELE SAC RUPTURES, THEN THE COMPLICATION
RISK AND THE RISK OF DEATH SKYROCKETS.
>> SO IT'S ONE OF OUR TOP CONCERNS WHEN THE BABY IS BORN
THAT SOMEONE'S SOLE RESPONSIBILITY IS MANAGEMENT OF
THAT OMPHALOCELE SAC WHILE SOMEONE ELSE'S RESPONSIBILITY IS
MANAGEMENT OF THE AIRWAY.
>> A LOT OF TIMES INFANTS WITH A GIANT OMPHALOCELE WILL HAVE
SMALL LUNGS AND REQUIRE ASSISTANCE RIGHT FROM THE MOMENT
THEY'RE DELIVERED.
>> CHARLIE WAS PUT IMMEDIATELY ON A VENTILATOR AND WAS ON
A VENTILATOR FOR ALMOST TWO MONTHS.
>> FOR AN OMPHALOCELE WE USUALLY WRAP WITH A STERILE DRESSING THE
ENTIRE OMPHALOCELE AND THEN BRING THE BABY TO THE INTENSIVE
CARE NURSERY.
>> JUST GOING TO THE N.I.C.U. FOR THE FIRST TIME WAS VERY
SCARY BECAUSE YOU SAW ALL THESE VERY, VERY SICK BABIES
AND REALIZED THAT THAT WOULD BE YOU.
>> ONCE THE BABY IS STABILIZED, THE ISSUE IS HOW IS THE
OMPHALOCELE GOING TO BE CLOSED.
>> BABIES WITH SMALL OMPHALOCELES OFTEN ARE ABLE
TO GO TO THE O.R. AND HAVE WHAT'S KNOWN AS A PRIMARY
REPAIR, WHICH MEANS THEY GO TO THE O.R. ONE TIME AND
ARE ABLE TO HAVE IT CLOSED RIGHT THEN AND THERE.
>> THEN THERE ARE INFANTS WITH GIANT OMPHALOCELES THAT CONTAIN
DIFFERENT COMPONENTS OF BOWEL AND LIVER.
AND THOSE INFANTS ARE A LITTLE MORE COMPLICATED.
>> THERE'S NOT MUCH ABDOMINAL CAPACITY BECAUSE VIRTUALLY
EVERYTHING IS OUT PARTICULARLY THE LIVER.
THE MAJORITY OF THE LIVER IS IN THE OMPHALOCELE SAC.
SO THE ISSUE THERE IS HOW YOU CAN GRADUALLY REDUCE THE
CONTENTS OF THE OMPHALOCELE SAC BACK INTO THE ABDOMEN AND
EVENTUALLY CLOSE IT WITHOUT CAUSING HARM TO THE BABY.
>> YOU COULD IMAGINE TRYING TO SLOWLY REDUCE ALL OF THOSE
ORGANS BACK IN.
WELL, AS YOU DO THAT WHAT DOES IT DO?
IT PUSHES THE DIAPHRAGM UP.
IF YOU HAVE A SMALL CHEST CAVITY AND SMALL LUNGS,
ANY KIND OF DISTURBANCE CAN JUST PUSH THE KID,
YOU KNOW, OVER THE EDGE AND TO A MAJOR RESPIRATORY CRISIS.
>> WE USE A TECHNIQUE WHICH INVOLVES BRINGING THE BABY TO
THE OPERATING ROOM, FIND THE FASCIA OR THE STRENGTH
LAYER, TWO BANDS OF MUSCLE ALONG EACH SIDE OF THE OMPHALOCELE
DEFECT.
THEN SEWING A TEFLON-COATED MESH SHEET ON EACH SIDE TO THE FASCIA
SO YOU HAVE TWO SHEETS COMING UP.
AND THEN YOU SEW THE SHEETS TOGETHER AND YOU LEAVE
THE OMPHALOCELE SAC INTACT.
THEN USUALLY ON AN EVERY- OTHER-DAY BASIS,
YOU CAN THEN CRIMP DOWN ON THESE TWO SHEETS AND SEW THEM BACK
TOGETHER SO YOU EVENTUALLY CLOSE THE BABY ALL THE WAY.
>> DURING THAT TIME WE HAVE TO FOLLOW THEM VERY CLOSELY TO MAKE
SURE THAT WITH THE CHALLENGES OF CLOSING THE ABDOMINAL WALL
DEFECT, WE DON'T INTERFERE OR HAVE ANY SETBACKS WITH THEIR
PULMONARY PERFORMANCE.
>> WE WATCH THE PRESSURES THAT IT TAKES TO FILL THEIR LUNGS
WITH AIR.
WE EXPECT THAT THOSE PRESSURES WILL BE HIGH INITIALLY POSTOP.
AND ONCE THEY BEGIN TO COME DOWN,
THEN THAT TELLS US THAT THE BABY'S READY TO GO BACK
TO THE O.R.
>> HE WENT THROUGH SEVEN DIFFERENT SURGERIES IN THE
COURSE OF THREE WEEKS.
IT SEEMED LIKE IT WAS EVERY OTHER DAY.
>> AND SO THIS CAN TAKE PLACE OVER A PERIOD OF A WEEK OR
LONGER WHERE THEY GO TO THE O.R. EVERY OTHER DAY,
EVERY THIRD DAY UNTIL FINAL CLOSURE.