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Monday, April 12, 1999
Copyright © Las Vegas Review-Journal
REVERSING NATURE
By surgically correcting a condition that causes the heads of babies to bulge out, doctors bring parents peace of mind.
By Tanya Flanagan
Review-Journal
Still in a daze from being sedated, Diane Morales lay in a hospital bed, straining through a mental fog to see her newborn son whom a nurse held before her.
What should have been one of the happiest moments in her life became a nightmare.
Questions flooded her mind. Her husband, Carlos, also was bewildered as he stared at their child. They looked at the doctors, at each other, and at their son, whose head was shaped like a football.
What happened and why?
"I just looked at him and thought immediately, `Something is wrong with my baby.' " Diane Morales recalled.
Their son, Christopher, now 10, had scaphocephaly, a form of craniosynostosis, a disorder that results in an abnormal skull and head shape. There are more than 80 types of craniosynostoses, congenital anomalies characterized by premature closure of one or more fibrous joints or sutures between the bones in the skull before the brain completes its growth.
Sutures usually hold a baby's skull cap together and stay open until the brain develops. When the joints close prematurely, the brain continues to grow and expands wherever it can, causing parts of the head to bulge out and take on odd shapes.
Craniosynostosis occurs in one in every 3,000 newborns, according to Dr. Matthew Rosenberg, a Las Vegas plastic surgeon who has treated more than 50 children with the condition since he began practicing in the valley in 1992. Scaphocephaly occurs in one in 6,000 live births, he said.
Scaphocephaly, involving a single suture, is the most common, representing more than 50 percent of all cases, Rosenberg said. Research on the condition dates to 1851, with surgical procedures being done as early as 1891.
Children show symptoms at birth or shortly after, said Dr. Colleen Morris, clinical geneticist with the University of Nevada, Reno's School of Medicine.
As recently as five years ago, little was known about the causes of craniosynostosis, Morris said. Today, known causes relate to the fetus's positioning in the womb and occasionally how a newborn's head is placed when a parent lays it down to nap.
"Genetic mutations can run in families or can occur for the first time in a (single) pregnancy," Morris said. "It (a mutation) happens in cell division, and 3 percent to 4 percent of all children are born with a birth defect that might be something minor like having an extra toe or something serious like craniosynostosis or having a cleft palate. Even Down syndrome is included in that 3 (percent) to 4 percent risk."
Doctors told the couple that Christopher's malady was an isolated incident that would not recur should they have more children. They believed it until their daughter, Deandra, was born four years after her brother with the same condition. Two years ago, they had a third child, Elizabeth, who was born without indications of the condition but developed signs of craniosynostosis by her first birthday.
The children's birth defects baffle the couple.
"We don't understand how that happened because she had a daughter before we married and I had a son, and nothing was wrong with either of them," Carlos Morales said.
Deandra's diagnosis was the most difficult to accept, Diane Morales said. "With my daughter, it was a lot harder because they (doctors) told me I wasn't going to have another child like this," she said. "Her forehead was pushed out, her temples were indented really bad, and there was a lump on top. Her head was oblong, fat and round at the cheeks, and then the top was narrow and pointy, and one eye was higher than the other."
The couple were living in New York when Christopher was born. They moved to Las Vegas when he was 6 months old. While traveling, they were in an automobile accident that left Diane Morales disabled from a neck injury. While living in New York, both Diane and Carlos Morales worked for an emergency medical transport company. She was an emergency medical technician and he was a supervisor and paramedic.
Now Diane Morales, 34, lives in the family's northeast Las Vegas home with the couple's four children. The oldest, Stephanie Joyce, 13, was born with no complications. The medical care, hospital bills and other related costs have strained the couple's 13-year marriage.
Carlos Morales, 48, does not live with the family but works as a security guard for a local company. He provides financial support to his family and spends time with the children.
"We love our kids, and we are trying to work everything out," Diane Morales said.
The surgical procedures and follow-up therapy done to correct both children's deformities have been the most difficult part of the ordeal for the parents. Christopher's surgery was done in New York and lasted 6 1/2 hours. His sister's was performed by Rosenberg and a team of Las Vegas physicians in 1993 and took 2 1/2 hours. Both had a strip of bone roughly an inch wide removed from the center of their heads -- from the front to the back.
In such surgeries, bone is always removed, and occasionally repositioned. The amount of removal depends on the number of sutures that close prematurely, Rosenberg said.
The different kinds of craniosynostosis are defined based on the number and location of sutures that close, he said. Surgery relieves pressure on the brain caused by the fused bones. Relieving the pressure lets the brain grow to its normal size and the head grow to a normal shape.
Rosenberg did not treat Elizabeth or Christopher, but he said the fact that siblings were born with cranial disorders alarmed him, so he referred the family to Morris for a genetic evaluation in October 1997. Morris said her office sent the family an information packet and requested photos of relatives, but she never received a response.
Diane Morales said she and her husband saw a genetics counselor shortly after Deandra's 1993 surgery, but they learned nothing from the experience.
"They said it is genetic, but they can't tell us if it's two of his genes or two of my genes or one of his and one of mine," she said. "Also they couldn't tell me if my children are carrying the gene. They can't tell us if my children will have children with this, and that is more frustrating than anything else because when they grow up, if they ask me are they going to have kids like this, I don't have an answer. I know how hard it was to go through it with my children, and they will have to make a decision on their own. I wish there were answers that I could give them."
Morris said research is helping to answer such questions.
"In the last five years, there has been a lot of research with this, and now several different genes have been identified that show they cause craniosynostosis," Morris said.
In a family such as the Moraleses, both parents would be examined, and one parent likely would have the gene.
"In most craniosynostosis cases, it is one gene, and genes come in pairs, and that means one in the pair isn't working. This also means that sometimes you can have one parent who has such mild effects they don't even know they have the gene" until is shows up more profoundly in their child.
Morris said numerous genes exist that are linked to craniosynostosis. A patient's symptoms or the number of sutures involved determines which genes a specialist would examine for mutations.
Today the condition has been reversed in the Morales children, and the scars and baldness created by the surgery barely show.
"Nobody (really) notices," says Christopher, who spends about 10 minutes each morning strategically combing his dark brown hair to cover the bald patches.
Deandra also suffers from baldness. She has a scar that extends across the top of her head between each ear, which looks like a barber cut a part in her hair. No hair grows where the scar is, so she wears ponytails.
In addition to the scarring, the children had learning disabilities. Clark County School District teachers told the Moraleses that both their children were developmentally delayed and suffered from poor motor skills. At early ages, both had trouble writing their names, sequencing numbers and objects, and catching a ball, Diane Morales said.
The setbacks weren't permanent for Christopher. When he first started school, he lagged behind his peers in an early childhood development class. Now he is part of a fourth-grade gifted students program at Mountain View Elementary School.
Deandra also began school in the same type of program. She still has difficulty writing her name but should be able to move into a regular first-grade class next year, her mother said.
Elizabeth, now 2, did not have surgery. She was diagnosed with mild craniosynostosis by a neurosurgeon but is scheduled to see Rosenberg for a second opinion in May, Diane Morales said.
Rosenberg said he favors forgoing surgery "if the child is beyond one year of age unless there is evidence of increased cranial pressure or the head is severely misshaped" and as long as any prematurely closed sutures in a child's head don't place pressure on the brain. Pressure can cause brain damage.
When Elizabeth was conceived, her parents nervously awaited her birth.
"We just waited to see what her head was going to be shaped like. It was tense," Diane Morales said, and at birth, "The eyes were closed because she was swollen. I thought I was going to die when I saw her."
Eventually her head took on a normal roundness, and Diane Morales was hopeful the pattern had been broken. At a year old, a large bulge appeared on Elizabeth's head just behind her forehead. A CAT scan revealed craniosynostosis. Her curly brown hair covers the defect.
"The important thing is that my children are beautiful now," Diane Morales said. "It has been hard on everyone, but the doctors were wonderful, and we are getting through this.
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Carlos Morales, 48, and his wife Diane, 34, at their northeast Las Vegas home with their children, from left, Deandra, 5, Christopher, 10, and Elizabeth, 2. Each child was born with craniosynostosis.
Photo by Jim Laurie.
Deandra Morales in 1993 when she was 3 months old, before plastic surgeon Matthew Rosenberg fixed her skull abnormalities. She was born with scaphocephaly craniosynostosis, a condition that causes the head to take on an odd shape.
Deandra after the surgery.
Photos courtesty of Dr. Matthew Rosenberg
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