Ariel Henley writes movingly about her experience here with Crouzon syndrome, a congenital craniofacial anomaly. In craniofacial surgery, the goal is to create more normal appearance, that doesn’t trigger comments in passing and most important, harmonizes how “normal” the patient feels inside, with their outer appearance. It’s hard work and tremendously rewarding.
Effy Redman penned a lovely essay about her experience with Mobius syndrome. In this congenital deformity, the nerves that supply the muscles in the face don’t develop properly, so children have little to no expression. A “social smile” develops by around 6 weeks of age, so very quickly there can be a disconnect from the baby’s feelings, and what others perceive, limiting and changing communication. Currently we have to wait for children to be big enough to have microsurgery, to transplant a muscle and connect its supplying nerve to one of the functioning muscles used for chewing, to create a smile; and it takes two surgeries, one for each side. Depending on the child’s size, this can be around 5-7 years of age. We often talk about restoring smiles with cleft lip repair (“Operation Smile”, “Smile Train”) but this is a rarer and more challenging condition to treat, though it is similarly rewarding!
We carry the EarWell ear molding device, and it was just profiled on NPR! Between one-fifth and one-third of infants are born with prominent or misfolded ears. Immediately after birth, the cartilage is very flexible, thanks to circulating estrogen left over from pregnancy. The ears are especially malleable. Some misfolded ears unfold on their own shortly after the child is born, but if the issue does not resolve itself within a week, early intervention is advisable. There is a very specific window of time in which treatment with the EarWell device is appropriate: before the child is one month of age. If you miss this window, ear correction will have to wait until the child is old enough to undergo surgery (around age 5 or 6 ). Having surgery is a much more invasive option, with issues of recovery, cost and potential discomfort to consider. What is the EarWell Infant Ear Correction System? The EarWell is a custom-fit plastic molding device. It resembles a “cradle” that adheres around the ear, with retractors that shape the ear’s structure. A special shell fits over the ear and holds all of the components in place. The EarWell device should be worn for six […]
In the United States, approximately one of every 700 babies is born with a cleft lip and/or cleft palate, a condition created when tissue in the baby’s upper lip or the roof of the mouth does not join together completely during pregnancy and leaves an opening. Despite unique health challenges, those born with cleft and craniofacial conditions can lead fulfilling, successful, and accomplished lives. Clefts are usually repaired surgically in the first year of life, though many children require additional surgeries and treatments through adolescence to correct challenges to breathing, eating, or speech development. Individuals born with cleft lip or palate often need specialized dental or orthodontic care throughout their lives as well. I really enjoy taking care of children born with facial differences, it’s very rewarding. Even though it’s common to think that facial surgery is all cosmetic, having a facial difference can really change how you interact with the world, so reconstructing the “interface” you have with the world can be very positive. This essay details one view on facial discrimination. Here’s a great list of national and regional groups if you want to donate or help support in other ways!
At the end of last week I traveled down to Palm Springs, where the annual American Cleft association and American Craniofacial Society meetings were held. It was great to see mentors and colleagues, and also hear how other centers handle their cleft and craniofacial cases. We all face similar challenges in optimizing care, so it’s great to be able to discuss and collaborate. There is a surprising amount of controversy on how to best treat different conditions, so we need more research, but it’s difficult when there are so few cases. We do agree on the desired outcomes though: well-adjusted young adults who interact with their peers without drawing much attention to their scars. Until next year!
What a cool prosthetic hand! This is a great article on what 3D printing can accomplish to help children with severe hand anomalies – or who suffered devastating losses through trauma: The children are like Ethan Brown, 8, who was born with two fingers missing on his left hand. Now he wears a Cyborg Beast in black and red, his school colors. “It looks even cooler than the picture, ” he said. “It looks like Ironman or Spider-Man.” He was once teased for his disability, said his mother, Melina Brown, of Opelika, Ala., who now volunteers for E-nable. “Now he’s different in a cool way, and the other kids say they want a new hand, too.” We plastic surgeons can transplant toes for thumbs, and reconfigure tendons and remaining remnants to improve function, but there are still some problems that we can’t overcome. I love that these bright, “superhero” hands are also a low-cost solution, and make the kids stand out in a positive way. Kudos!
Most people have heard of, or seen, a cleft lip. There are much rarer facial clefts, however, where large segments of the soft tissues and bone are involved, leading to missing tissue or bone, or redundant tissue where the two parts of the face didn’t come together as close as they normally should. These clefts were first described and classified by Paul Tessier, the father of craniofacial surgery, who trained my mentor Dr. Kawamoto (they co-authored the original publication on these clefts). Dr. Tessier pioneered moving the facial bones, including the eye sockets and forehead bone which also houses the brain; he showed that it could be done safely and make patients with severe deformities look normal again. Now with 3D printers and skull models, we can plan these kinds of surgery in more detail before heading in to the operating room. Dr. Tessier didn’t even have CT scans – just xrays—but he could visualize his plan just from his exam, and improvise in the operating room because of his experience. There is a nice article and video (above) in the New York Times this week about how the craniofacial surgeon at Boston Children’s Hospital planned a Tessier no. 4 cleft repair […]
A nice essay was just published in the New Yorker by a man suffering from facial palsy (his diagnosis is Bell’s palsy), which left him with residual weakness on one side of his face. It’s a cliché that you don’t appreciate something til its gone, but in the case of one-sided facial palsy, one half of your face is a constant reminder of what you used to have: symmetry at rest, facial expressions, blinking to keep your eye moist, and resting tension in your lip muscles to keep you from drooling. Bell’s palsy in particular usually resolves, and so most of the facial functions return, but if they don’t, or you have some permanent injury to the facial nerve (through trauma or after tumor resection), or you’re born without functioning facial nerves (hemifacial microsomia, Moebius syndrome) there are ongoing problems. The details and options for these kinds of reconstructions are too varied and extensive to go into here, but I’m glad my training allows me to help in these situations. One of the last cases I did as resident involved transferring a muscle and nerve from the inner thigh to a little girl’s cheek, so we could restore her smile. It […]