INFORMATION PROVIDED BY: Rady Children's Hospital's
SAN DIEGO - The ongoing success achieved by Rady Children's Hospital and Health Center of San Diego's Craniofacial/Neurological Services joint program is testimony to the advanced technologies available there, the use of innovative products, procedures and surgical techniques, and a first-rate surgical facility.
But most importantly, says Surgical Director and Chief of Craniofacial Surgery Steven R. Cohen, M.D., it's a unique team approach by a skilled and compassionate group of surgeons that sets them at the forefront of this highly specialized area of medicine.
"We have a superb team with some of the most highly skilled specialists in the West taking care of a variety of medical challenges related to skull deformities," explained Dr. Cohen. "When craniofacial patients come through these doors, we have the advantage of passing them through an entire team for diagnostics and treatment. Instead of treating just one focus at a time, we can spend six to eight hours on an operation with three different teams doing their respective jobs. And when those young patients are done, they've undergone what might take another center a year to accomplish."
In addition to Dr. Cohen, who performs plastic and reconstructive surgery on craniofacial patients, the core team of specialists include Cohen's partner, Associate Surgical Director and Associate Chief of Craniofacial Surgery, Ralph E. Holmes, M.D.; Director of Oculoplastics Surgery, Don Kikkawa, M.D.; Director of Oral and Maxillofacial Surgery, Lester Machado, M.D.; Chief of Pediatric Neurosurgery, Hal Meltzer, M.D.; Associate Chief of Pediatric Neurosurgery, Michael Levy, M.D.; Anthony Magit, M.D., Pediatric Otolaryngologist; and Medical Director of Craniofacial Services and Genetics, Marilyn Jones, M.D.
This multi-disciplined team also includes specialists in ophthalmology, otolaryngology, pulmonology, speech pathology, orthodontics, pediatric dentistry, psychosocial services and nursing.
The Craniofacial/Neurosurgical team at Children's has experienced particular success performing a delicate procedure to correct a condition called craniosynostosis, which occurs at birth when the open areas that separate the seven bone plates in the skull, also known as sutures, become fused.
When a suture closes too soon, the skull can grow in an abnormal manner, changing the shape of the skull, face, eye sockets and jaw. Craniosynostosis, which occurs in about 1 in every 2,000 births, may cause damage to the growing brain because there isn't enough room for it to grow inside the skull. It can lead to developmental delay, brain damage, and vision problems.
The most common type of synostosis is sagittal, in which the head becomes elongated, narrowed and takes on a boat shape, a condition called scaphocephaly. Surgeons on the team are now utilizing a revolutionary and innovative surgical approach, developed by Drs. Cohen, Holmes and Meltzer, in which very small incisions are made in the head and specially designed optic devices called endoscopes are used to magnify and visualize the underlying anatomy.
By operating endoscopically on children as early as possible, many within their first year of life, the team has produced results comparable to more traditional cranial vault reshaping (a procedure that releases the fused suture and places cuts in the side bones of the skull to help reshape it) but with additional benefits as well. Those benefits include smaller, less invasive incisions, less blood loss and consequently fewer blood transfusions, shorter operating times, less time under anesthesia, and faster healing and recovery.
"For certain groups of patients with craniosynostosis, 70% of the children, if caught before the age of four months, can be operated on endoscopically," said Dr. Hal Meltzer, a pediatric neurosurgeon on the team. "This means they are having a surgery that can take at the shortest 30 minutes and at the longest two hours versus six to eight hours elsewhere. We believe that 90% should be done endoscopically because of the tremendous benefits associated with it."
Before the endoscopic technique was introduced, Meltzer explained, surgeons made ear-to-ear incisions across the top of the head when performing craniosynostosis procedures. The incisions resulted in substantial blood loss for the patient that often required blood transfusions, severe swelling and lengthy recovery times. Parents sometimes needed the assistance of social workers to help the family cope with the trauma. The far less invasive endoscopic technique is now being embraced by both physicians and families alike because it eliminates the need for noticeably long incisions and the resulting negative side effects associated with them.
Taking advantage of advances in technologies and materials, Drs. Cohen and Holmes have also become pioneers in the use of biodegradable devices for cranio-maxillofacial surgery. The doctors have worked closely with leading medical device manufacturers to bioengineer new devices that dissolve into, and are absorbed by, the body. Instead of metal plates, for example, the doctors are now using these lighter and dissoluble devices in endoscopic craniofacial surgery to reduce the need for post-operative therapy that often required the use of a helmet to help re-contour the shape of the skull.
Cohen is also an internationally renowned expert in midface distraction and has developed an FDA approved invention and technique that requires dividing the skull into two sections and implanting special plates beneath the skin to reattach the sections. In time, the plates are pulled apart with the use of bioresorbable expansion screws, allowing new bone to grow into the gap between the plates and reshape the skull.
Working as a team, the plastic surgeons, neurosurgeons and pediatric specialists at Children's are achieving the best possible outcomes for patients with complex skull deformities.
"This is a very special team," Cohen said, "and I don't think there is anything like it available anywhere in the Western region with this kind of working relationship. We're also located in a lovely city with a wonderful climate, and one that is very kid and family friendly, which is a big plus for patients and their families while preparing and recovering from surgery. For those who are faced with these challenges, we want to present San Diego and this magnificent program as the jewels that they are."
Founded in 1954, San Diego Children's Hospital and Health Center is the region's only designated pediatric trauma center and the only area hospital dedicated solely to pediatric care. In addition to caring for children at its main campus in Kearny Mesa, Children's has 15 neighborhood centers offering primary care and specialized services. Children's is also active in numerous community outreach programs, including health education, early intervention and counseling, child abuse prevention and child safety issues.
Endoscopic & Minimally-Invasive Treatment for Craniosynostosis
Treatment of Craniosynostosis: A Team Approach
With the newer endoscopic techniques, the earlier the surgery, the better the outcome. We recommend immediate referral for any patient with a serious head deformity secondary to craniosynostosis to determine if the patient qualifies for the endoscope or minimally invasive approach.
Endoscopic and Minimally Invasive Craniofacial Surgery
An innovative surgical procedure for craniofacial surgery is available at Children's Hospital, San Diego. Surgeons make only small incisions and use specially designed optic devices called endoscopes. The endoscope is an instrument with extremely sophisticated optics that permits magnified visualization of the patient's anatomy. It has been used in general surgery and patients generally have faster recovery times, smaller incisions and less blood loss.
Before (Pre-Op) After Endoscopic Correction (2 weeks Post-Op) After Band Therapy www.chsd.org/body.cfm?id=1918 (5 months Post-Op)
4 month-old boy with Metopic Synostosis - Before and after cranial reshaping
Minimally invasive, endoscopically assisted strip craniectomy has been successful for early treatment of craniosynostosis. Physicians have reported excellent results for patients with sagittal synostosis when they use an endoscopic technique and combined strip synostectomy with postoperative helmet molding. By operating as early as possible, our group along with others have produced comparable results to standard cranial vault reshaping with less blood loss, shorter operating times and earlier discharge from the hospital.
Before After
2 month-old girl with Sagittal Synostosis- Before and 3 months after Endoscopic Correction
Traditional vs. Endoscopic correction:
Minimally Invasive Approach " Small incisions will be made in the scalp within the hair and sometimes along the crease of the upper eyelids " Using a small lighted endoscope, the operation will be projected onto a T.V. screen " Resorbable devices may be used for bone stabilization " Post-operative helmet or band will be prescribed in many cases to "fine tune" the shape of the skull " The helmet may be needed up to 3 months " Your child will be fitted with a helmet
http://www.chsd.org/ 10 to 14 days after the operation
Dr. Steven R. Cohen, Dr. Ralph E. Holmes and Dr. Hal S. Meltzer have developed a new technique that eliminates or shortens the need for postoperative helmet or band therapy. Our surgeons now use an improved endoscopic technique that permits more definitive head shape changes and immediate reconstruction of the skull deformity. By utilizing extensive cranial osteotomies and wedge ostectomies together with rigid fixation using bioresorbable devices, immediate cranial reconstruction can be achieved in selected craniosynostosis patients who undergo the endoscopic-assisted technique.
Conditions Treated With The Endoscopic-Assisted Approach:
Endoscopic approach for metopic synostosis - small incisions allow access. The bones are cut with endoscopic guidance and small biodegradable plates are used for stabilization.
Dr. Cohen and Dr. Holmes are pioneers in the use of biodegradable devices in cranio-maxillofacial surgery. They have worked closely with leading bioresorbable device makers to develop numerous new techniques that use fixation devices that eventually dissolve and are absorbed by the body. Instead of metal plates, biodegradable devices are also being used in endoscopic craniofacial surgery to reduce the need for postoperative helmet or band therapy http://www.chsd.org/body.cfm?id=1918
Summary
Most major craniofacial teams have individualized their operative approach to obtain the best aesthetic outcomes and lowest reoperation rates. Total cranial vault reconstruction is a major operative procedure not without morbidity.
A new technique combining an endoscopic-assisted approach with postoperative helmet molding for treatment of sagittal synostosis has had excellent results. Disadvantages of this approach are the prolonged need for postoperative helmet molding and limitations when it comes to treating severe variations in scaphocephaly.
We have developed an operative approach that incorporates extensive corrective osteotomies and ostectomies with immediate correction with or without specially designed bioresorbable plates and screws. Our results are encouraging and we now treat selected patients with all types of single suture craniosynostoses including unicoronal and metopic with the endoscopic minimally invasive approach. With more experience it may be possible to eliminate the need for postoperative helmets in patients with sagittal synostosis as well as in the correction of other types of single suture craniosynostoses.
Craniosynostosis
What is Craniosynostosis?
"Craniosynostosis" is a term used to describe the premature fusion of one or more of the skull's sutures before the brain is fully grown.
Background
The infant skull consists of five plates of bone held together by clear, fibrous areas called sutures -see adjacent diagram ». The sutures should remain open as long as the brain continues to grow, enabling the skull to expand and properly accommodate the brain's growth.
When a suture closes, a predictable abnormality of head shape occurs. In certain children, changes around the eyes and face may occur because of fusion of the sutures in the cranial base. When this occurs, the child often has an associated craniofacial syndrome that must be diagnosed by a skilled geneticist.
Treatment
The treatment of craniosynostosis is surgical. Most cases are treated because of the resulting severe aesthetic deformities. However, a small percentage of cases are associated with increases in intracranial pressure due to changes in head shape, configuration and volume.
Types of Craniosynostosis
Sagittal Craniosynostosis The most common type of single suture fusion. The head becomes elongated and narrowed and takes on the shape of a boat, scaphocephaly.
Metopic Craniosynostosis The forehead portion of the skull becomes triangular in shape and the eyes become closer together (trigonocephaly).
Coronal Craniosynostosis When one coronal suture is fused, the orbit is pulled back and upward, while the opposite side grows down and forward to compensate. If both coronal sutures are involved, the entire forehead along with the orbital rims above the eyes are drawn backward (brachycephaly). Sometimes the head appears tall (turricephaly).
Lambdoid Craniosynostosis The head becomes trapezoidal in shape. This is the rarest of the craniosynostoses, accounting for only about 4 percent of cases.
Frontal deformation of right forehead/orbit in position-related deformity
Other Pediatric Skull Deformities
Position-Related Head Deformities
Position-related (deformational) deformities are a common abnormality of the skull. Positional skull deformities have dramatically increased following the recommendation by the American Academy of Pediatrics that all infants sleep on their backs to prevent sudden infant death syndrome (SIDS).
Torticollis
Prolonged abnormal positioning is believed to lead to a pressure deformation of the skull. The back of the head is usually flat on one side, and the ear and forehead on the same side are rotated forward. Generally, these deformities are easily differentiated from craniosynostosis by a thorough examination.
Unicoronal craniosynostosis Position-related sleep deformity
A CAT scan is necessary in some cases to differentiate the disorder from others requiring surgery. Craniofacial surgery is necessary in only a few patients with especially pronounced deformities not covered by the hair. For children with severe deformations around the forehead and eyes, craniofacial surgery may also be necessary. This surgery is similar to that performed for craniosynostosis.
Cephalohematoma
Calcified cephalohematoma
Cephalohematoma means a bruise of the skull. Occasionally, a difficult delivery or an early trauma causes bruising and bleeding in a specific site of the skull. This may become infiltrated by calcium and produce a prominence. Most cephalohematomas do not require treatment. However, when they are especially large and disfiguring, the neurosurgeon, occasionally working with the craniofacial plastic surgeon, will recontour the skull. This is relatively simple to do.
Benign Subdural Hyrogromas of Infancy
Benign subdural hygromas
Benign subdural hygromas of infancy are a relatively common occurrence associated with prominence of the forehead and rapid head growth in early infancy. A CAT scan of the skull reveals extra fluid outside the brain, but underneath its covering membrane (dura), leading to skull changes. Most of these patients only need follow up, as the head deformity is self-correcting. They occasionally have developmental delay initially, as the large head slows acquisition of motor skills. They invariably catch up between one-andone- half and three years of age.
Dermoids
Dermoid cyst
Dermoids are small congenital cysts sometimes with sinus tracts. Often found over the eyebrows, dermoids can occur anywhere on the skull and forehead. When they occur in the midline, a MRI or CT scan is necessary to rule out intracranial extension. Treatment is generally simple and sometimes may be carried out with endoscopic techniques. If intracranial extension is found, a combined neurosurgical and craniofacial plastic surgical approach may be necessary.
Enchephaloceles
Pre-operative encephalocele Post-operative encephalocele
Encephaloceles are conditions where the underlying brain with its coverings protrudes through a defect in the skull. This usually occurs in the midline of the skull, in the area between the forehead and nose or in the back of the skull. When located in the back of the skull, they are often associated with neurological problems. Usually these are dramatic deformities diagnosed immediately after birth. Occasionally a small encephalocele in the nasal and forehead region will go undetected for a time. Diagnosis requires a CAT scan, and treatment is carried out by the neurosurgeon working with the craniofacial plastic surgeon.
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