Updates

Craniofacial Program in Austin About C3DO

Counterclockwise Craniofacial Distraction Osteogenesis (C3DO) is a powerful surgical procedure to expand the entire upper airway in children with tracheostomies. 

Families from around the country travel to our Craniofacial Destination Program in Texas Children’s North Austin Campus for the C3DO procedure. The surgery was first described by one of our surgeons, Dr. Richard Hopper, and our craniofacial orthodontist, Dr. Hitesh Kapadia, who have the most experience in the country performing this technique. The procedure has been successful in treating complex upper airway obstruction and removing a tracheostomy in conditions such as Treacher Collins syndrome and Craniofacial Microsomia when other techniques like mandible distraction have failed.

Overview

The C3DO procedure starts with a full day surgery to separate the bones of the face so that they can slowly be moved into their correct position after surgery using a device attached to the skull (halo distractor) and two attached to the lower jaw (mandible distractors). The power of the C3DO is that it rotates the entire upper and lower face forwards and upwards to decompress the entire upper airway obstruction. Drs. Hopper and Kapadia have published research showing how the C3DO results in airway improvements that were not possible with other techniques.

Counterclockwise Craniofacial Distraction Osteogenesis - PubMed (nih.gov) 

Counterclockwise Craniofacial Distraction Osteogenesis for Tracheostomy-Dependent Children with Treacher Collins Syndrome - PubMed (nih.gov)

The C3DO is done is three stages.

  • The first is the surgery which lasts a full day, followed by one week in hospital.
  • The family is taught how to activate (turn) the devices starting five days after surgery.
  • After discharge from hospital, our team will need to see the patient every week for four to five weeks to adjust the devices and create the desired rotation of the face.

During this time the jaws are wired shut so that everything moves together. Patients will require a feeding tube during this turning phase and use their tracheostomy to breathe.

Once a patient’s face is in the correct position and the airway is decompressed, the patient is brought back to the operating room for a very brief procedure to release the jaw wires and let the patient open and close their mouth. The devices remain in place to stabilize the jaws as they heal. The patient and family can return home during the 3-month healing phase, and then return to the Craniofacial Destination Clinic for removal of the devices and airway assessment in preparation for decannulation of the tracheostomy.

Frequently Asked Questions

Our contact information, general information about the procedure, plus preoperative, surgery and postoperative details are given below.

Call Us:

  • Craniofacial Coordinator: 737-229-3473
  • Nurse Line: 737-229-3517 (select option 3)
    Available Monday to Friday, 8 AM to 5 PM CT

Email Us: atxncplasticsgroup@texaschildrens.org

During your first consultation, our treatment coordinator will work with you to transfer your child’s past medical records for review. These include things such as CT scans, sleep studies, dental images and airway evaluations. From these records our team will be able to determine if your child has a subcranial rotation deformity and could benefit from C3DO. If they appear to be a candidate, we will arrange an up-to-date airway camera evaluation in the operating room (sleep endoscopy) by an experienced otolaryngology team, either in your home institution or in our North Austin Campus. If your child tolerates capping of their tracheostomy, a recent capped sleep study will also be needed.

A side view x-ray of a child who benefited from the C3DO procedure. The black lines show where the facial bones should be located. The C3DO procedure rotates the bones of the entire face into this new position to open the airway. 

C3DO is only recommended for severe upper airway compression; therefore, almost all our patients already have a tracheostomy in place. Occasionally a patient had their tracheostomy removed earlier but developed severe obstruction later that needs the C3DO. In these cases, we recommend that the tracheostomy be put back in for a safe airway during the C3DO treatment.

The youngest we recommend undergoing C3DO is seven years. In our experience, the recommended age range is seven to 11 years old, but we have successfully done C3DO on teenagers and young adults.

We have been doing the C3DO procedure for more than 10 years. To date over 90 percent of patients undergoing C3DO have had their tracheostomy removed, and none have had their tracheostomy replaced. Although we prefer that a patient have C3DO as their first jaw surgery, many of our patients have had multiple previous surgeries at other institutions, such as mandible distraction osteogenesis or choanal atresia surgery, and then have been referred to us for the C3DO procedure.

  • Some patients who need a C3DO procedure to help their airway are also missing the joints of their lower jaw (temporomandibular joints, TMJ). In these cases, your surgeon will recommend to reconstruct the joints with bone and cartilage grafts taken from one rib on each side of the chest (costo-chondral grafts).
  • This surgery is usually performed at age five to six years of age to allow healing before the C3DO. It takes around six hours and is followed by two to three nights in hospital. The rib grafts are placed through the same jawline neck scars that will be used for the C3DO.
  • A recent CT scan is needed at least four weeks before this procedure for computer surgery planning.
  • Recovery after this surgery is quick. Your child will be on a soft diet for six weeks but can open and close their mouth immediately. They can return to most pre-surgery activities but need to avoid ball games, energetic play or running for 6 weeks to allow the bone to heal.

Computer planning of a rib graft surgery to replace a child’s missing lower jaw joints before they will be ready for the C3DO procedure. This planning lets the surgeon avoid the teeth (black arrow) and the nerve inside the bone (white arrow).

  • Initial consultation in person or via telemedicine
  • Pre-surgical in-person visit at the Craniofacial Destination Program clinic in our North Austin Campus four to six weeks before C3DO
  • C3DO surgery
  • Two nights in the Intensive Care Unit
  • Three to five nights in the hospital inpatient ward
  • Four to five week turning phase with weekly clinic visits at our clinic
  • Jaw wiring removal as day surgery
  • Three month healing phase at home
  • Device and splint removal surgery with cheek bone reconstruction if needed
  • Post-C3DO sleep study in evaluation for removal of tracheostomy

The Halo device (red arrows) slowly rotates the face forward and upward. The two mandible distraction devices (blue arrows) makes the lower jaw longer. The jaws are wired shut during turning to make sure they move at the same time. The surgeon removes these wires once the turning is finished. 

Once your child’s C3DO surgery is scheduled, we will arrange a visit at our clinic four to six weeks before surgery. During this visit your child will meet with the Anesthesiology team (PASS clinic), have blood test drawn, and have a CT scan that your surgeon will use to do detailed computer planning of the surgery. Our craniofacial orthodontist will also take a digital scan of your child’s upper teeth so they can build the custom acrylic splint used to attach to the halo distraction device. In some cases, we can work with experienced craniofacial teams closer to your home to get the CT scan and intraoral scan, but an in-person pre-surgery visit will still be required.

Illustration of the custom acrylic splint being secured by wires to the upper jaw during surgery

The surgery takes a full day in the operating room. Approximately half of the time is taken to make the cuts in the bone, and the rest is to apply the wires and three distraction devices.

The C3DO procedure uses three incisions. The first is a zig-zag scar that runs over the top of the scalp from temple to temple (coronal incision). The other two are below both the angle of the lower jaw where it meets the neck and are just over one inch long. All incisions are closed with dissolving sutures and there are no dressings that need to be changed.

Yes, a blood transfusion is required to keep your child’s blood levels safe during the surgery. Blood work will be drawn before surgery to prepare and you will sign a separate consent form.

  • After surgery, your child will be taken to the CT scanner for a post-surgery image, and then taken to the Pediatric Intensive Care Unit (PICU). We will reunite you with your child as soon as possible after the PICU team has assumed care.
  • It can be emotional to see your child for the first time in the PICU since they will have the distraction devices in place and a number of tubes to help with giving fluids and nutrition. They will be kept very sedated for 24 hours to keep them comfortable. Swelling of the face will be the most 24 hours after surgery and will last two to three days before the swelling quickly goes down. Your child will be transferred to the hospital inpatient ward after two to three days in the PICU. Total length of time in the hospital is five to seven days.
  • You will be able to stay with your child throughout the hospital say. We recommend you take time to rest outside of the hospital while your child is in the PICU or alternate with another caregiver. It is important you are not sleep-deprived when your child is moved to the ward since you will be more involved in their care after that and will start to learn to turn the distraction devices.
  • During the time in the PICU, your child will receive pain medicine through their intravenous lines and will be kept sedated for their comfort. After 24 hours they will be allowed to be more awake and we will start to transition their pain medications to acetaminophen (Tylenol), ibuprofen and a prescription pain medicine called oxycodone. These are the three medications your child will continue after they are discharged from hospital, but the oxycodone is only needed for a few days. Acetaminophen and ibuprofen are given as needed during the turning phase of distraction but are rarely needed during the healing phase.
  • Parents have reported that they found turning the devices a lot easier than they had anticipated. The small screwdrivers used to turn the device have arrows to show the director of turning and are easy to use after a few tries. We will provide you with a small calendar to record each turn that is done in the morning and again in the evening.
  • Your child should not feel pain when you turn the devices. The devices move the face very slowly at a rate of 1 to 2 millimeters (0.04 to 0.08 inches) per day. Early in the turning phase the screwdrivers are easy to turn, and your child usually does not feel any movement. Towards the end of the one month turning phase the muscles can start to stretch, and your child may feel an ache after turning that is like the feeling after having orthodontic braces adjusted. This is usually managed with over-the-counter acetaminophen and ibuprofen.
  • Although families usually mentally prepare themselves for the surgery and hospital stay, we will also help you prepare for the turning phase. Even though the turning of the devices does not cause pain, your child’s face is slowly and steadily moving forward over weeks, and therefore it does not have time to fully heal until the turning phase is complete. Families who have undergone C3DO describe the turning phase as tiring.
  • Your child will need your emotional support during this phase and encouragement that what they are feeling is temporary and that they will feel better after the turning is over. We have child life and child psychology teams available to you during this time if needed.
  • Turning of the devices often causes drooling and extra saliva production. This stops quickly after the healing phase begins.
  • After your child is discharged from the hospital, we will need to see them in our clinic once a week during the entire turning phase. During these visits we will take an X-ray to track the movement of your child’s face and the change in their airway shadow. Your surgeon will also need to make some minor adjustments to the devices during this period. These are done with special screwdrivers that change the angle of movement to create the desired rotation of the face.
  • Once the turning phase is completed, your child will return to the operating room for a brief 30-minute procedure to release the wires keeping their jaws together and to apply some stabilizing bars to the mandible distractors for the healing phase. We do not need to see your child in clinic during the healing phase, but will keep in touch through messaging and video visits.
  • Families who live out-of-state will usually arrange to have temporary lodging in Austin for five weeks to complete the surgery, turning phase and wire removal procedure. At that point your child can return home either on the plane or by car.
  • During the healing phase at home, your child will want return to many of their pre-surgery activities. Since the devices are no longer being turned, their bones start to heal quickly and their face quickly starts to feel more normal to them. They just need to avoid activities that would put their devices at risk, such as ball games, energetic play or running. If your child was experiencing drooling during the turning phase, it usually quickly stops during the healing phase.
  • Since the devices used for the C3DO are outside your child’s skin, they are easy to remove in 30-45 minutes. The removal does not create post-surgery pain, and if no other surgery is performed at the same time, you can return home the same day, or fly home the next morning if living out of state.
  • In certain cases of Treacher Collins syndrome, the cheek bones have not formed at birth. If this is the case with your child, your surgeon will usually recommend that the surgery to create new cheek bones be done at the same surgery to remove the devices. This surgery helps stabilize the new position of the upper jaw and improves eye protection. This cheek bone surgery takes a full day in the operating room and two to three nights admission before returning home.
  • Removal of a tracheostomy cannot be rushed and needs a number of steps before confirming that it is safe to proceed. Our otolaryngology airway specialists will be involved in this decision. After the C3DO your child will need a post-treatment capped sleep study. This can be done either here at the North Austin Campus or closer to home.
  • In addition, our airway specialists will repeat the sleep endoscopy (camera view of the airway) at the time of device removal and confirm that the airway has expanded enough by comparing to the pre-C3DO sleep endoscopy results. In some cases, soft tissue problems such as tonsils or a “floppy” epiglottis (a valve that covers the windpipe) needs to be treated after C3DO but before the tracheostomy can be removed.
  • Once our team has confirmed it is safe to remove the tracheostomy, we recommend it is removed during an overnight stay at the hospital so we can observe your child closely. The tracheostomy stoma (skin hole) needs to completely heal before your child can do activities such as swimming.
  • After successful and safe removal of your child’s tracheostomy, we expect your child to be able to enjoy activities they couldn’t do before such as swimming.
  • We also recommend you follow with your primary care physician to make sure your child is gaining weight and growing appropriately. We continue to follow your child annually, or occasionally every two years, after C3DO in coordination with your local doctors.
  • We need to make sure your child does not develop signs of sleep apnea as they grow. It is not uncommon for teenagers to benefit from positive pressure support (CPAP or biPAP) of their airways when sleeping. This will be determined by any new symptoms (snoring, sleepiness, restless sleep, lack of weight gain, etc.) and would be checked with a repeat sleep study.
  • C3DO is a surgery designed to improve your child’s airway. All children who had C3DO will still need a period of orthodontic treatment (braces) in their teenage years, followed by orthognathic (jaw) surgery. This surgery will align their bite and further improve their airway when they have finished growing (age 16 for females, age 18 for males). This final jaw surgery does not involve devices or distraction, but instead uses small titanium plates and a splint to hold the jaws in place to heal for six weeks. Time in hospital is usually two to three days. Although this surgery is relatively common in teenagers with bite problems, jaw surgery in patients who have undergone C3DO should have the surgery done by an experienced craniofacial team. Our craniofacial orthodontist can treat your child at our North Austin Campus if you live close by or we can coordinate care with an orthodontist closer to home if you live at a distance from the hospital.