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!
After the recent win of deaf contestant Nyle DiMarco on America’s Next Top Model, another woman with Treacher Collins syndrome (a craniofacial anomaly) wrote about her experience auditioning for the show. I applaud her honesty in describing her challenges and frustration with the standards of beauty. “We live in a world of difference, a world that so far, has not often been represented in the modeling and entertainment industries. This prejudice carries over to the professional world, where people with facial disorders want to be accepted and looked at based on our own merits. . . . I remember how the other contestants derided DiMarco because of his deafness, because he lived in a world of silence and was different, because, as they said, he would never fit into the high-stakes world he so aspired to join. With tears in my eyes, I heard his name called and watched his face light up in disbelief and overwhelming happiness when the American Sign Language interpreter translated the announcement of his win.” Treacher Collins is a rare craniofacial syndrome affecting the orbits, midface, ears, and lower jaw. Even though she had several surgeries which are typically recommended for reconstruction, she still […]
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 […]
I’ve always enjoyed old movies and recently I caught one I hadn’t seen before: A Woman’s Face, starring Joan Crawford. In the film, Crawford plays a woman who was severely scarred on one side of her face as a child, and grows up extremely self-conscious of the scar. She runs an inn, and blackmails on the side – and it’s implied that her facial scar has scarred her character as well, leading her to malicious crimes. I won’t spoil it for you here, but suffice it to say that she meets a plastic surgeon, who after twelve (!) surgeries is able to restore her facial symmetry and she appears to have no scars (this is Hollywood after all – in real life there is no scar-less surgery!). And as a result, she feels able to leave her life of crime behind. Of course, I don’t think that one’s appearance determines destiny, or that scarring or deformity is associated with crime. I do think, however, that this film dramatizes the effect that self-consciousness of a facial deformity can affect all of one’s interactions; and the converse idea, which is that others make assumptions about a person’s character based on their appearance. […]
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!
This story came out of the UK recently – and I would like to make clear that punching a patient is not an appropriate or effective approach to fixing a cheekbone fracture! Surgeon who punched patient is fired Of course most patients sustain these injuries through some kind of trauma, but reproducing that impact doesn’t fix the fracture or help anyone. Some of my patients joke (I think?!) that they imagine that I have to hit them again to put the bones back in place, but I promise that is not what happens. There is more swelling again after the repair, but that’s from my gaining access to the fractures, not from repeat trauma! Stay safe this Cinco de Mayo!
My patients who are Golden State Warriors fans, know that I really only follow one player, one team: Jeremy Lin, and whatever team he is on. Sadly, I know, now it is the Lakers. (Let’s not talk any more about it.) I came across the news of the Oklahoma Thunder point guard Russell Westbrook’s injury when I saw him playing with his face mask on: Russell Westbrook was hit in the face by his teammates’s knee during a game on February 27th. He had a zygomatic arch fracture, which is the cheekbone – and it was repaired the following day. I see these fractures occasionally – the repair is usually pretty straightforward, but if it is complex and comminuted (with multiple fragments) sometimes I need to make an incision across the top of the head, from ear to ear, to reduce and fix it properly. As with all facial fractures, we avoid making visible incisions on the face, so at least the scalp incision is hidden by the hair. He got the VIP treatment by having it repaired the following day – if there is a lot of swelling, I wait a few days until it improves, because it’s […]
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 […]