Johanna’s Story – Surgery to Correct Goldenhar Syndrome

As a surgeon with extensive experience working with children, I’m often approached by non-profit organizations to provide pro bono surgery for children in need. I do dozens of these surgeries a year, especially through my involvement with Operation Hope, where I travel around the world with other physicians, providing surgeries to children in desperate circumstances. Surgeries like cleft palate repair have the power to change these children’s lives. I’m honored to be a part of that.

But every now and then, I have a case that’s so special, it really stands out… a case like Johanna. Johanna is a patient who came to me through an organization called Healing the Children, which opened its chapter in Louisville this year with Johanna’s case.

Johanna is from Quito, Ecuador, and has Goldenhar Syndrome. This rather rare syndrome causes a number of problems with the head and face for these children, and little Johanna, only six years old and a petite 35 pounds, has the full syndrome. When she arrived at my office this fall, she had:

• Only a flap of skin where her ears were supposed to be, and no discernible ear canal
• A lateral cleft palate
• Deformities of her jawbone that made her chin uneven
• Limited hearing, an undetermined amount of structure in her inner ear, and one corner of the mouth higher than the other

Johanna is the youngest of five children in a loving home in Ecuador. But with her father working in construction, and her mother working as a housekeeper, they just did not have the funds for the multiple surgeries required. So, through Steve Diamond at Healing the Children, they came to us and another team of specialists in Louisville. Rob and Marta McKinnon of Goshen, Ky. agreed to serve as her foster family in Louisville, to shepherd her through her surgery.

Johanna is a little marvel. Even though she is so small and in a completely foreign place, she handles the whole process with maturity far beyond her years. Her foster family seems genuinely thrilled to have her around. They report she has no trouble blending in, getting used to new things, or making her feelings known through an advanced kind of charades. We’re not sure how much she can hear us, but she responds well to signals, and seems to follow the conversation as I discuss the surgical procedure with her foster family.

My job in this process was to recreate her ears, so that Dr. Gadre, a neurotologist at the Heuser Hearing Institute, can then work on options such as hearing aids or cochlear implants to restore her full hearing. Unfortunately, we won’t be able to address all Johanna’s problems on this trip, as her visa only runs through Christmas. But we can create the framework for her ears and, we hope, her new life.

Step one of the surgery was to remove the existing flap of skin for her ear. Then we created a zigzag incision on her scalp, so we could gain access to the parietal fascial flap of skin in her scalp. To be specific, this is a thin layer of skin underneath the scalp. We placed a plastic, artificial framework for her ear, which will serve as the cartilage in the finished ear. Then we pulled the artery as well as the skin from her scalp over her ear, and attached it to the framework. We then grafted skin from her neck and groin to create the skin covering the ear, and attached the adjoining nerves and blood vessels. When we were finished, the ear looked rather undefined. But as it heals, the skin shrinks down over the framework and creates an attractive ear.

Because Johanna is so little, we had to choose certain surgical alternatives. For instance, we had to use the artificial ear, as opposed to creating a framework from her ribs, because of her size. But with the assistance of Dr. Gordon Tobin and the plastic surgery residents from the University of Louisville, both surgeries for her ears went well, with no complications.

I also credit this surgery’s success to Johanna herself, and the loving support of her foster family, as well as her family in Ecuador. As scary as this procedure can be for a small child, she never cried or refused any of our instruction. Her foster family reported that she even handled the difficulties that come with recovery with grace and determination. I am happy to report that she now has full hearing in one ear and a beautiful reconstruction. I think we all can take a lesson from Johanna, and many of the children we see in our practice. Life is full of challenges. It’s how you face them that determines the outcome. Johanna has taken only the first step of her journey. She will still need surgery to repair her jaw, her cleft palate, and possibly her hearing in the other ear. We wish her well.