CRANIOSYNOSTOSIS REPAIR: FIXING BABY'S HEAD SHAPE
From Washington University Physicians, posted November 3, 2010, written by Mary Jo Blackwood, RN, MPH
Most babies’ skulls develop normally, but in roughly 1 out of every 2,500 births, the sutures (or growth plates) between the bones of the skull fuse prematurely, a condition called craniosynostosis. When that happens, the brain and skull cannot grow normally, putting extra pressure on the developing brain and deforming the face and skull.
For the last few decades, the standard for treating this condition has been open correction, where the scalp is incised and the bones are cut and remodeled into a more normal shape.
A newer technique, endoscopic craniosynostosis repair, offers an alternative to the traditional approach with the benefits of a shorter procedure, quicker recovery and minimal hospital stay. It has now been successfully performed on over 70 patients at St. Louis Children’s Hospital.
Both types of repair are handled by a dedicated surgical team consisting of a neurosurgeon and a plastic surgeon, to make sure the surgery relieves pressure on the brain and achieves an excellent aesthetic result. Washington University pediatric neurosurgeon, Dr. Mathew D. Smyth (seen in photo at right) and Dr. Albert Woo the section chief for pediatric plastic surgery perform the procedures.
Says Dr. Smyth, “The most common form of this condition involves the sagittal suture, which runs front-to-back across the stop of the skull. When that is fused, the baby develops a long skinny head and bulging forehead. Other sutures may be involved, singly or together, including the coronal that runs ear to ear, the metopic, or forehead suture; and the lambdoid suture in the back. We can use either procedure on any of these, depending on the age of the baby.”
Smyth says they get referrals from a multi-state area and even from other countries; and have the only program in the region that uses the endoscopic method. St. Louis Children’s Hospital is also the busiest center in the region for management of these problems. “We do one or two open or endoscopic craniosynostosis repairs a week.”
Dr. Woo explains that the team’s ultimate goal is to take a child with a very abnormal appearance and the health risks of increased pressure on the brain, headaches and eye injury; bringing that child to a normal appearance with the expectation of a normal life.
Open Craniosynostosis Repair
Now most commonly performed at the medical center on babies of age six months and older, the open method involves an incision across the top of the head from ear to ear. During the operation, the deformed bone is removed, reshaped or replaced, and anchored with dissolving plates and screws. The surgery can take from three to eight hours, depending on the severity of the deformity and location of the fused sutures. It usually requires a blood transfusion and a three to five-day hospital stay. This surgery, however, is done once and all the remolding is done at the time of the procedure. Woo says most children will never need additional surgery; and because they are young, they replace any missing bone quickly and heal rapidly.
Endoscopic Craniosynostosis Repair
In the endoscopic method, a much smaller surgery is performed. Only one or two incisions on the top of the head of about one inch each are made in the scalp, allowing several small segments of bone to be removed to relieve the pressure on the skull. Surgically, it is an easier procedure for the baby, requiring only about 1.5 hours of surgical time or less, without blood transfusions, and in most cases only an overnight stay. The discomfort and swelling also are dramatically decreased.
However, during the endoscopic repair, there is no direct surgical remodeling of the skull. That is accomplished by the use of a specially designed helmet that the baby wears for up to nine months. By adjusting the helmet frequently as the head grows, the skull is gradually molded into its correct shape.
Dr. Woo explains: “The endoscopic approach works best early when the baby’s skull is not completely calcified and is more malleable. We typically perform these procedures around three months of age, in order to get an optimal result.”
The Key is the Helmet
As Smyth explains, the open procedure is harder on the baby while the endoscopic approach is harder on the parents because of the frequent helmet fittings. “We like them to come back here if at all possible because the babies need multiple fittings as they grow; and their heads grow rapidly in the first year.”
He says about three to four days after surgery, when swelling has gone down, they do a laser three-dimensional scan of the head to get precise helmet dimensions. Making the helmet takes another few days. The baby wears it up to the age of one, coming in initially every two weeks for adjustments, and then once a month.
Another key to the success of the program is nurse practitioner Sybill Naidoo who does the helmet fittings and adjustments. If patients live a long distance from the medical center, she also coordinates with a remote orthotist to make the corrections. Baby’s first birthday has double cause for celebration. The candles are lit and the helmet comes off—of a child with a beautifully molded head, primed for a bright future.