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Endoscopic Management of Sagittal Craniosynostosis

Heather Hicks noticed the odd shape of her son Robert Noack’s head as soon as he was born. “His head was long and narrow, shaped kind of like a peanut,” she says. “At the time I was the only one who was concerned.” 

Hicks and her family are residents of Willis, Texas, a small community about an hour north of Houston. In a fortunate twist of fate, Robert contracted respiratory syncytial virus at the age of two weeks, and was admitted to a downtown hospital where he was in intensive care for nearly a month. “I was still super concerned about the shape of his head, and finally one of his doctors recognized that he had craniosynostosis.”

The physician referred her to pediatric neurosurgeon Manish N. Shah, M.D., and pediatric plastic and craniofacial surgeon Matthew Greives, M.D., who work together as part of the renowned Texas Cleft-Craniofacial Team at Children’s Memorial Hermann Hospital and McGovern Medical School at UTHealth. Both hold faculty positions in the medical school’s department of Pediatric Surgery – Dr. Shah in the division of Pediatric Neurosurgery and Dr. Greives in the division of Pediatric Plastic and Craniofacial Surgery. The two surgeons have a track record of success collaborating on craniosynostosis cases.

A Rare Condition

A rare condition that occurs in 1 in 2,500 babies born in the United States, craniosynostosis changes the growth pattern of the skull by premature fusion of its fibrous sutures. Robert is among the 40 percent to 60 percent of cases in which the sagittal suture fuses earlier than normal. Thanks to his mother’s persistence and a physician’s awareness of the signs of craniosynostosis, he was diagnosed well before the age of three months, early enough to qualify for endoscopic surgery.

“When certain types of craniosynostosis are diagnosed early, we can make small incisions at the front and back of the skull, visualize the fused sutures using the endoscope and remove a strip of skull bone to reopen the space between the skull plates,” Dr. Shah says. “The procedure takes about 20 minutes, which means the child spends less time under anesthesia, with less bleeding and swelling than with open surgery. Most kids spend one night in the hospital.”

The traditional open surgery is done on infants older than five months. A large incision is made over the top of the head, from just above one ear to just above the other. After loosening the tissue covering the bone and exposing the skull, the surgeons remove a strip of bone where the affected sutures connect. In some cases, larger pieces of bone also must be removed, reshaped and replaced. The open surgery can take from three to six hours, and most children who undergo the procedure require a blood transfusion.

A Less Invasive Surgery

Robert had the less-invasive endoscopic surgery between three and four months of age, when his skull was still pliable. No transfusion was necessary. Although the endoscopic procedure has an overall 5 percent transfusion rate, to date, Dr. Shah and Dr. Greives have a 0 percent transfusion rate for all cases. When Robert’s hair grew back, his scars were unnoticeable.

“The Texas Cleft-Craniofacial Team has one of the longest histories treating craniosynostosis, and the addition of the newer endoscopic technique keeps our institution on the cutting edge,” Dr. Greives says. “We work closely with the helmet therapist to reduce the length of time the child spends in the helmet. They do laser scans before, during and after helmeting to follow the child’s progress, and we work with them to make adjustments as necessary. We try to make it as easy on parents as possible, and those who have gone through the process with us have done well.”

Dr. Shah emphasizes the importance of early diagnosis of craniosynostosis. “Our endoscopic technique is excellent but can only be done if parents and pediatricians recognize the condition early,” he says. “If a child is past four months of age, the bone is too thick to use the endoscope.”

Robert wore two different helmets over a period of six months to help reshape his head, the first one painted to look like a leather aviator hat complete with goggles.

“I’m so glad I kept after it and finally found doctors who could help,” Heather Hicks says. “As a mom, I worry about everything that happens to my children. To avoid having us drive to Houston, Dr. Greives offered to answer any questions I had by email. I sent photos, and he responded back within an hour. They both did an amazing job.”