After an ultrasound, x-ray and CT scan, Liam was diagnosed with bicoronal craniosynostosis at one month old. We didn’t know his craniosynostosis was caused by Muenke Syndrome until Liam was six months old. Muenke Syndrome is characterized by craniosynostosis (the premature fusion of one or more skull sutures), hearing loss and developmental delays. Soon after the diagnosis, we met with our pediatric plastic surgeon and neurosurgeon to discuss how they were going to correct Liam’s craniosynostosis. The biggest concern with any craniosynostosis diagnosis is the lack of room for a child’s brain to grow.
On December 21st, 2021, Liam had Posterior Vault Distraction surgery. The procedure involved separating the back half of Liam’s skull from the front half and attaching a mechanism (distractors) that separate the two halves over time. We spent the night in the PICU and another four days in the hospital after this surgery. Once we left the hospital, it was my husband’s and my responsibility to turn the distractors morning and night to push Liam’s skull back. We did this for forty days. During these forty days, Liam developed four severe infections. Our pediatric plastic surgeon finally removed the dangly parts of distractors, but the rest of the mechanism remained attached to his skull until April 19, 2022.
On April 19, Liam had another surgery to remove the distractors. During the procedure, the dura layer (another protective layer under the skull) was accidentally cut. We spent the night in the PICU to make sure there was no spinal fluid leakage from the dura tear and then returned home the next day. An hour after returning home, Liam became listless, his temperature spiked to 103.2, his head became extremely swollen and he wasn’t eating. We traveled two hours back to the hospital where we stayed another two nights. Just days after being released from the hospital Liam’s head became severely infected again. A week and a half later, we are still trying to get the infection under control.
Liam might only be eleven months old (he will be a year old on May 14th), but his bravery and strength are admirable. In his short time on earth, he’s been through more than most people have in their entire lives. We’ve been to at least one doctor’s appointment a week since he was born. He’s been poked, prodded, tested and scanned more times than we can count. What has made it even more difficult is that he doesn’t understand any of it. He was terrified to move for the four months he had the distractors in. He also slept in a baby swing during this time because he couldn’t lay on the sides of his head. The numerous infections caused a lot of pain and discomfort. The medications made his stomach upset. Despite all of this, Liam never lost his joy. To this day, people always comment on his infectious little smile (it really is the best)!
Liam is now one year old and is continuing to struggle with healing from the removal of the distractors. The left side is as good as we could expect; it's healed and the scars are big but clean. The right side is a
hot mess. The front exit point of the distractor never healed and we had to go back to the surgeon and get it cauterized. Now it's a large (size of a US Quarter) black scab. We're waiting to see if it starts to heal. The distractor was attached to the skull in the back of his head, and this site has formed a very large pocket of fluid. The pocket is supposed to be absorbed gradually over the 6-8 weeks following the surgery, but Liam's is growing and moving forward gradually. It's now moved to the incision site and has started draining lightly. It doesn't appear to be infected, but it doesn't appear to be going away. We're watching it closely, expecting it to burst soon based on the movement and growth rate. If it moves forward much more the pocket will be directly under the ear-to-ear incision created when the distractors were installed. This is a very weak point in his scalp and won't handle the pressure of the protuding pocket well.
Liam's incisions on the right side of his head refuse to heal. The surgeon says that the tissue is too inflamed to create new skin cells and wants us to take him to the hospital either weekly or every two weeks to have a short surgery to debride or remove the hardened buildup over the incision wounds then cauterize the tissue underneath. We have a second opinion scheduled with Johns Hopkins All Children's hospital, and pending any further advice we're going to proceed with a plan to try to get his poor little head to heal. Topical medications like hydrocortisone which would reduce the inflamation are preventing any healing, so he's stuck in a terrible cycle where the wounds won't heal on their own.
We still have at least one more surgery in the foreseeable future and maybe more down the road, but we have faith that all of this will only be a small part in Liam’s story. God has big plans for our little man.
We're extremely grateful for the support that we've received from friends, family, and our extended Facebook, Twitter and Instagram communities. Thank you all so much for the gifts, words of encouragement, support, and prayers. Please know that this support means everything to us right now. The support groups we would traditionally rely on are mostly unavailable to us due to distance and Covid 19. We have no family that lives close.
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