July 12, 1999 LOS ANGELES (July 8, 1999) -- While the potentially life-saving benefits of the "Back to Sleep" campaign -- launched nationally in 1994 to reduce the risk of Sudden Infant Death Syndrome (SIDS) -- are indisputable, the admonition to place babies on their backs during sleep has resulted in an epidemic of plagiocephaly, or "misshapen head," in infants, according to John M. Graham, M.D., Sc.D., director of Cedars-Sinai Medical Center's Craniofacial Clinic.
Over the last five years, the incidence of nonsynostotic positional plagiocephaly has jumped fivefold: from an estimated 1 in 300 live births to 1 in 60 births today. Though easily treatable, plagiocephaly and accompanying torticollis, a shortened or tightened muscle on one side of the neck, may go unrecognized by parents and health care practitioners alike.
Misshapen heads are often secondary to the muscular torticollis, which causes the baby to tilt his or her head toward the tight side and turn away, resulting in a preferred resting position. As a consequence, the infant's normal, rapid rate of head growth coupled with a consistent resting position can lead to significant asymmetry of the head shape.
"When infants sleep in one position, there is consistent pressure on their soft and forming skull, which can result in deformation of the head," explained Dr. Graham, who estimates the clinic handles 250 to 300 visits a year for this condition. "Unfortunately, many care providers are unaware of the symptoms and inappropriately reassure parents that the child will grow out of it. Left untreated, torticollis and plagiocephaly can result in permanent distortion of the head as well as persistent facial asymmetry."
The result is often needless worry, he added. "It's really a very simple problem that needs to be more clearly recognized by the medical community and public." Muscular torticollis is usually caused by the limited room in the uterus for the baby during late gestation, and it is even more common with multiple gestation (twins or triplets). Torticollis can be difficult to detect at birth or in young infancy because the neck is relatively short. One noticeable symptom is a persistent head turn, and parents frequently report a preferred resting position with sleep. After age six months, the neck begins to lengthen, and tilting of the head toward the tight side may become more evident, suggesting the need for neck physical therapy.
Culver City residents George and Kelly Athanasas know first-hand the concern and confusion this condition can cause. For several months the anxious new parents observed a number of troubling symptoms in baby Niko. A lump on the side of his neck was initially diagnosed as a swollen gland, though there was no response to treatment. Niko wasn't turning his head either, and the right back side grew increasingly prominent while the back left became flattened. Kelly also recognized a noticeable tilt to Niko's bottom gum line, and that the baby's ears seemed out of alignment.
These are some of the telltale signs of torticollis and plagiocephaly, according to Dr. Graham, and parents are often the first to recognize them. The condition, which does not affect normal brain growth and function, may include the following physical characteristics:
* the back of the head is flattened on the same side that the forehead is more prominent
* the ear on the side of the occipital flattening (at the back of head) can be larger and advanced forward, compared to the other ear
* the jaw can be asymmetric, with an upward slant on the same side as the shortened muscle
* facial asymmetry, with one cheek appearing more full and one side of the forehead appearing more prominent than on the other side
After visiting two pediatricians, the increasingly concerned Athanasases were referred to Cedars-Sinai's Craniofacial Clinic.
"When I called the clinic, I described Niko's symptoms to the nurse, and I immediately felt reassured that the staff could help us," said Kelly, who brought the then seven-month-old baby in for a comprehensive evaluation by Dr. Graham. Niko was subsequently diagnosed with torticollis-plagiocephaly deformation sequence and began a prescribed regimen of neck massage and stretching. He was also fitted with a custom-made "helmet" -- worn virtually around the clock for four months -- to help reform his head and reposition his features.
Treatment of the condition includes neck physical therapy to correct the muscular torticollis along with repositioning techniques and/or helmet therapy to correct the head shape. Complete resolution of the shortened muscle takes four to six months of regular neck-stretching and head turning exercises, which are performed by the parents. The sleeping position must also be altered to train the baby not to lie on the flattened part of the head, allowing this area to fill in properly. This can be accomplished by using an infant positioning device and placing favorite toys and stimulating objects on the baby's non-preferred side to encourage him or her to remain in the prescribed position.
If significant head asymmetry is present at six months, helmet therapy is necessary to correct the head shape.
"The correction of head asymmetry with helmet therapy is based on head growth -- the normal rate of growth slows at around one year of age, so we have a window of opportunity when the baby is between six months and one year old," Dr. Graham stated.
In Niko's case, his plagiocephaly was already quite pronounced so helmet therapy began simultaneously with the neck stretching exercises and repositioning techniques. The corrective helmet is designed to "remold" the head to a natural, oval shape, and therapy usually continues for four months. Babies quickly adapt to wearing the helmet, which is ventilated with holes to allow its use in Southern California's warm climate.
"Within the first few weeks of wearing the helmet, I called the doctor's office and said, 'Niko's cured, he's cured!,' " Kelly remembered. "They told me it would take longer, but I was amazed at the progress."
Every few weeks, Niko returned to the clinic for evaluation or to the orthotics specialist for adjustments to his helmet. Despite Kelly's concern that his features might always be "a little crooked," she watched with excitement as his ears and gums shifted into proper alignment, and Niko's head began to round normally. "He's great now, and his head is gorgeous," she said with relief. "Every parent wants their baby to be perfect, and I was so afraid he'd be deformed."
Tony and Susan Avallon, also of Culver City, faced similar concerns when twins Perris and Quinn displayed signs of the condition. At four months, both babies had noticeable flattening behind the right ear, while the left back sides of their heads appeared more prominent. Susan also noted that their right ears were larger.
"It was scary, because the problem was clearly getting worse, and no one could explain why," said Susan.
A pediatrician told the anxious parents that it was "nothing to worry about," and a neurosurgeon wanted to fit the twins with $7,000 "headbands." Neither mentioned torticollis and plagiocephaly, which Susan first heard about at her Twins Club meeting. That chance conversation led to a referral to Dr. Graham, who told them that torticollis is more frequent in twins and triplets because they have even less room in the uterus in late gestation.
"He was really so great, so thorough," she recalled. "He went over everything and spent hours with us."
The twins, then five months old, embarked on neck physical therapy and repositioning techniques as their first course of treatment. While Quinn responded well, it was determined that Perris required the helmet therapy to correct her degree of plagiocephaly. At six months, she was fitted with a helmet, which she wore day and night. Her mother explained to curious onlookers that the headwear was like "braces on your teeth," but designed to round the head into shape. Susan was impressed with the dramatic -- and speedy -- improvement she witnessed in Perris' head shape.
"I'd never heard of this condition; my pediatrician had never heard of it," Susan added. "This is a dramatic example of the importance of a second opinion. I'm really happy now, and I'm so glad I found Dr. Graham." Director of Clinical Genetics and Dysmorphology at Cedars-Sinai Medical Center, Dr. Graham is also a professor of pediatrics at the University of California-Los Angeles School of Medicine. He is a graduate of Johns Hopkins University and The Medical University of South Carolina, and also earned a Doctor of Science degree in Communicative Disorders from Johns Hopkins School of Hygiene and Public Health.
Dr. Graham, who has authored more than 200 publications on medical genetics and birth defects, is president of the Society of Craniofacial Genetics, and a member of the American Cleft Palate/Craniofacial Association, American Society of Human Genetics, Society for Pediatric Research and American Pediatric Society. In addition to his work with plagiocephaly and torticollis, he runs a diagnostic and treatment center for children with developmental delays or birth defects at Cedars-Sinai Medical Center.
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