Midface Distraction Osteogenesis (1st Phase)

One week after his initial surgery to install the rigid external distraction (RED) device, Henry is home from the hospital and settling in.  The surgery itself was intense and for most of the week it wholly overshadowed the attachment of the (RED) device to his skull. It was a variation on a procedure often used for moving the upper jaw and mid-face area forward to treat serious upper jaw misalignment with the lower jaw.

Called a Le Fort I osteotomy, it basically cuts the bone to separate the upper jaw along a common horizontal fracture line where the bone is weakest just below the nose. Vertical cuts on either side of the upper jaw complete the separation. In Henry's case, a relatively large curved plate was installed in an additional step to span the open gap above his upper gum line where previous procedures had inserted grafted bone.  This will add stability to the comparatively soft bone-grafted bridge behind his upper lip.

Three surgeons, each taking turns, worked from within Henry's mouth and behind his upper lip to avoid leaving external scars. The procedure took about six hours since with so much hardware already installed in his mouth there was very little room in which to work and extreme care was needed to avoid cutting into several important facial nerves located in the same vicinity, nerves that were often exposed during the operation.

The expected three-day stay was challenging and we needed to push very hard to be released. Generally they let hospital patients go once they can be disconnected from IV liquids after handling a similar liquid intake on their own.  Similarly, patients also need to be able to expel liquids on their own and be able to handle non-IV medications.

During this surgery it was apparently very difficult to keep an enormous amount of blood from draining from Henry's mouth into his stomach, and more than could be adequately suctioned out as things were wrapping up in the operating room.  Beforehand, this was a child who had vomited maybe two or three times ever.  After the surgery he would almost immediately vomit up fairly large amounts of foamy blood and mucus each time we gave him small amounts of clear liquid and this continued well into the second day, which really set back his recovery time.  He safely turned a corner and was taking liquids by that second evening, but it was difficult to convince the resident doctor on call that things were going satisfactorily on day three, despite the advice of our attending nurses who agreed with us.

Henry really wanted to go home.  Eventually, we were able to record that he was drinking the equivalent of about a cup of liquid per hour, even still attached to IV fluids, and we were finally released on the evening of day three, returning home just in time for Thanksgiving the next day.

Appearance
Photos we had seen beforehand of patients wearing the rigid external distraction (RED) device tended to otherwise show relatively mild effects.  These photos must have been taken weeks after the initial osteotomy surgery, which produces a lot of painful-looking swelling and continual drainage of a mixture of blood and mucus from the nostrils, both lasting more than a week.  This drainage is just part of the healing process.  The patient's lips may also be very tender and sore from the stretching that takes place during the surgery. 

Liquid Diet
Post-surgery we were asked to keep Henry on a liquid diet for at least a week before gradually also adding in very soft foods.   This was a challenge for him given that his stomach had already been made a little sensitive and that the large number of medications (pain, antibiotics, etc.) we needed to give him were also all in liquid form.

The RED Device
The rigid external distraction (RED) device attached to Henry's skull turned out to be a different model than the one we had seen as a example, but it might be an improvement.  It attaches to his head with four bolts on each side instead of three and along more of a curved line.  The result seems very stable, which had been a concern.  It is obviously unconformable but so far he is handling it very well.  Home care requires daily cleaning and changing the dressings around these bolts.

We returned to the hospital five days after the initial surgery for the first of the daily turns of two front screws on the device, which will slowly extend the middle portion of his face outward.  Our medical team adjusted the time frame for this process after the surgery.  Basically, they would like us to make the turns twice each day for a little more than two weeks and then they'll review the progress.  Four days along Henry doesn't feel anything from the turns yet, and doesn't seem to mind the routine.  We've been told that he will very likely begin to feel some discomfort around the second week or so.  One can see that his upper jaw has already visibly begun to move forward.

Once this mid-face extension process is complete, it seems the options for what to do next have now been reversed.  As of today Plan A would keep the device attached for about 60 additional days, hoping for supporting bone to grow.  Plan B would remove the device sometime just before Christmas in another surgery that would embed plates and more grafted bone to secure the outward extension.  We'll see.

Other than general discomfort, the primary challenges posed by the device seem to be achieving a good head position for sleeping, which we've addressed by tilting the front portion of his mattress upward instead of using pillows, and that the device makes it extremely difficult to drink from a cup, which we've addressed by collecting a battery of syringes in a variety of sizes that Henry largely uses himself.

Henry has handled all of this by absorbing each challenge and then assembling the will to overcome it, and at this he is very practiced. But it’s clearly been very hard. He’s a remarkable kid.