Craniofacial Surgery: A Center for Treating Complex Craniofacial Anomalies
Multiple pediatric subspecialists combine algorithmic care, the latest technology and science to achieve the best outcomes.
In the Cleft & Craniofacial Center, 3-D models take the guesswork out for pediatric surgeons Rick Redett, Amir Dorafshar and Anand Kumar.
Pediatric craniofacial surgeon Anand Kumar's heart goes out to newborns with Pierre Robin syndrome, a congenital condition marked by a severely small mandible and a cleft soft palate, putting them at high risk of airway obstruction, as the tongue frequently falls to the back of the throat. Also, the typical small opening in the roof of the mouth may cause choking or regurgitation of liquids through the nose. Needless to say, the condition brings breathing and feeding difficulties, too. These patients, stresses Kumar, need prompt multidisciplinary care.
For newborns in the Johns Hopkins Children's Center's neonatal intensive care unit (NICU), appropriate consultation most often happens at or soon after birth. But in the past, some referrals from other NICUs that do not have craniofacial specialists have been misdirected and/or delayed through miscommunication on both ends. Who should the patient with these anomalies see—ENT, genetics, plastics, pulmonary? In some cases, even the neonatal physician-on-call, often a resident on a three-month rotation in the NICU, was uncertain where to send the patient and opted for a subspecialist he or she knew.
To ensure appropriate and expeditious referrals in these cases, Kumar and colleagues led the development of a condition-specific algorithmic care model for craniofacial patients. When a referring hospital describes a newborn with a suspected small jaw and cleft palate, for instance, the NICU physician-on-call searches such signs and symptoms on the algorithm and sends one email that connects with all of the appropriate subspecialists. Everyone in the call pool who has signed off on the protocol for this condition, Kumar explains, is assigned the case on a rotating basis.
"I like the algorithm because it touches on many specialties and is team based not person based," says Kumar. "Referrers like it too—they're calling back with new patients because they felt we did a nice job the first time around getting their patient in and appropriately treated. They're telling us algorithmic care is improving our outcomes and patient satisfaction."
The other piece of that equation, of course, is the Johns Hopkin Children's Center's new Cleft and Craniofacial Center, where team members diagnose, treat and study anomalies like Pierre Robin syndrome. Relying on the latest technology, including low-dose CT scanners interfaced with 3-D cameras, they have enhanced their diagnostic capability to virtually plan surgical procedures and provide the most appropriate and safest care. Using a dental cone beam CT, for example, they're able to produce precise 3-D images of a patient's teeth, soft tissues, nerve paths and bone at lower radiation exposure compared to conventional CT.
"No dental cast models are needed; we're going pure digital in scanning teeth," says Kumar. "And the images are 99 percent accurate."
Craniofacial specialists like Kumar are also scientists focused on advancing care —Kumar's research includes cranial bone regeneration — and they constantly look for and develop innovative therapies for conditions like neonatal airway obstruction, which comes with Pierre Robin syndrome. Traditional treatments for the disorder involve the use of breathing and feeding tubes and bone grafting procedures, which pose risks, among others, of blood loss, tissue damage, and potential tooth bud damage. Following the center's distraction osteogenesis protocol, Kumar and colleagues reduce these risks by manipulating the jawbone and inserting a device to gradually move it forward, bringing the obstructing tongue with it from the back of the throat. The device is initially adjusted, followed by a three-month healing period, and then removed to allow the patient to heal hardware-free.
"By moving bones slowly over time, the technique avoids soft tissue damage and promotes bone formation with no permanent hardware," says Kumar.
Nevertheless, Kumar adds, the latest technology and novel therapies mean little without a unified multidisciplinary team and a seamless way for patients to find their way to the Cleft and Craniofacial Center. "The technology is very futuristic and accurate, but the real story is that we have finally found a way to implement it in a collegial fashion through our algorithm," he says. "When someone calls, we say 'No problem,' and activate the protocol."
In addition to Kumar, Cleft and Craniofacial Center team members include plastic and reconstructive surgeons Rick Redett and Amir Dorafshar, head and neck surgeons David Tunkel and Jonathan Walsh, and neonatologists Chris Golden and Janine Bullard. The new center also works hand-in-hand with pediatric anesthesia, its new pediatric pre-anesthesia clinic and difficult-airway response team led by Nick Dalesio. For more information, visit the Cleft and Craniofacial Center website.