Georgia Health Sciences University. Georgiasm

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1 1828 Georgiasm A monthly newsletter from MCGHealth Children s Medical Center and GHSU Department of Pediatrics Volume II Issue 6 mcghealth.org/kids facebook.com/mcghealthkids twitter.com/mcghealthkids

2 Georgiasm Letter from the Chairman This month s feature story profiles toddler Dakota Maass, who was born with craniosynostosis, a congenital defect that causes one or more of the sutures the connections between individual skull bones to close and harden earlier than normal. The result is not only an abnormally shaped head, but also restricted skull growth and increased pressure that can cause vision problems and impede intellectual development. Craniosynostosis is one of the many craniofacial abnormalities that we re able to correct with surgery at MCGHealth Children s Medical Center. We offer the best in total care for children like Dakota with congenital abnormalities and disfiguring injuries. Our superior team of pediatric experts including Dr. Jack Yu, a fellowship-trained pediatric plastic and craniofacial surgeon, and neurosurgeon Dr. Dion Macomson, both of whom cared for Dakota perform a full range of surgeries that eliminate the impact of these conditions on a child s basic health and functioning, as well as on their social life and self-esteem. Our pediatric plastic and craniofacial surgery program offers comprehensive solutions for all types of congenital and traumatic deformities in children including hydrocephalus, tethered cord and myelomeningoceles, cleft lips and palates, hemifacial microsomia (a condition in which one side of the face is larger than the other), Treacher Collins Syndrome, Pierre Robin Sequence, Nager Syndrome and many others. Ongoing research at by experts such as Dr. Yu, is aimed at finding answers to why conditions like craniosynostosis occur, and ways to prevent or control them (see article Researchers Want to Know How Bone Regulates Growth ). Chiefs Corner Our year as chief residents has been a challenging and delightful learning experience. One of our final duties is to pass on everything we have learned over the past year to the rising chief residents. We are very fortunate to have two fantastic people filling our positions. We are pleased to introduce Dr. Ketarah Robinson and Dr. Lee Johnson, chief residents for the Department of Pediatrics for the academic school year. Dr. Robinson is from Williamston, North Carolina and graduated from East Carolina University. She plans to join a private practice upon completing her year as chief. Dr. Lee Johnson is from West Point, Georgia and graduated from the Medical College of Georgia. He also plans to join a private practice after serving as chief resident. Both are excellent physicians and dedicated people. We know they will be wonderful chief residents. Hail to the new chiefs! Joshua Smith, MD Co-Chief Resident Nancy Wood, MD Co-Chief Resident It s a great privilege to work alongside superb specialists and their teams of dedicated professionals at our children s hospital. The ultimate measure of success in serving little Dakotas and their families is how they credit every member of our seamless team! Sincerely, Bernard L. Maria, MD/MBA Pediatrician-in-Chief, MCGHealth Children s Medical Center Ellington Charles Hawes Professor Chairman, Department of Pediatrics 1

3 1828 From Behind the Desk When our patients need surgery, it is important for us to remember that they are still children and need subspecialty care that addresses their unique needs. Our pediatric surgery subspecialty provides an excellent mix of both pediatric and surgical expertise, and also partners with other subspecialty providers when the need arises. One such example involves patients requiring critical care post-operatively. Pediatric surgeons work with intensivists in the neonatal or pediatric intensive-care units to provide the highest quality care to their patients. Another patient population that benefits from the collaboration of pediatric surgery and other subspecialties, are those requiring a lifesaving therapy known as ECMO, or Extra-Corporeal Membrane Oxygenation. These patients are very fragile and require the teamwork of many different specialists. ECMO is a therapy designed to provide temporary circulatory and respiratory support for patients whose systems are too sick to manage on their own. It is not uncommon for an additional therapy to be utilized to provide dialysis for these patients at times. When this occurs, surgeons, intensivists and nephrologists all team together to coordinate and manage the care for these very ill children. Pediatric surgeons also work side by side with other disciplines to plan the care of children pre-operatively. They orchestrate a monthly multidisciplinary conference with colleagues in pathology, radiology and pediatric gastroenterology to prepare for complicated cases. The demand we see for pediatric surgery is growing each year due to the outstanding care provided by our surgeons. When the Children s Medical Center moved into its new building ten years ago, the plan was to be able to support 2,300 surgeries per year. Last year, our team of surgeons completed over 6,000 pediatric surgeries and we continue to grow, improving the care of children. James Mumford, MHSA, FACHE Interim VP of Children s Hospital Administration Administrative Director, Pediatrics Kimberly D. Basso, RN, MSN Interim VP Pediatric Patient Care Services mcghealth.org/kids 2

4 Georgiasm Feature Story Surgeons Correct Misshapen Skull to Allow for Normal Brain Growth When Dakota Maass was born, her head was misshaped, much like a conehead, says her mom. Similar to the creatures in Alien, says Kelly Maass. The doctors thought it was because of complications in the birth canal, and that it would grow out normally within a few months, says Kelly. This was the Waynesboro resident s eighth child the third she d delivered at home and she sensed something wasn t right. In Dakota s case, the abnormal shape was growing more instead of less pronounced. After a consult with MCGHealth Children s Medical Center s pediatric neurosurgeon Dr. Samuel Macomson, Kelly and her husband Jason Maass learned their now 14-month old toddler had craniosynostosis. Often babies are born with misshapen heads, most of which round out after a few months, notes Dr. Jack Yu, pediatric plastic surgeon at MCGHealth Children s Medical Center. Infant brains are like a big bag of Jello that pushes down on their new, somewhat soft skulls, he explains. This can cause one side of the head to be flatter, which the baby then tends to favor, worsening the condition. The majority of these self-correct as the baby gets older. It s important to differentiate between an abnormal shape due to positioning a condition that will improve to one that s not. If in doubt, consult with a specialist. A congenital condition in which the bony plates of the skull close together too early, craniosynostosis causes problems with normal brain and skull growth. Often an abnormally shaped head is the only sign something s wrong. Dakota had sagittal synostosis, the most common form of craniosynostosis, in which the middle bone connection that runs down the middle of the skull closes too early. We don t know why this happens, explains Dr. Macomson. Her skull had fused almost entirely before she was born, and could have really put a lot of pressure on her brain, recalls Kelly. The doctors were surprised she didn t have brain damage because of the skull hardening. The treatment for craniosynostosis is reconstructive surgery to reconfigure the bony plates of the skull, allowing for normal bone and brain growth. 3

5 1828 It was a real blow when they told us she needed surgery, says Kelly. A slew of CAT scans and MRIs helped determine Dakota s bone structure and whether or not there was abnormal pressure on her brain. We received support from the hospital throughout the entire process. They answered every question I presented by phone or . I did a lot of research; I wanted to know all the details. It was all glued back together with screws made of a polymer derived from cornstarch, which eventually is broken down by the body, allowing the skull to continue to expand as Dakota grows. Immediately after surgery, her parents could see her head was round, even wrapped in bandages. It was rough seeing her post-surgery, recalls Kelly. She s a thrill-seeker; she likes to climb, jump, and be thrown, so the hardest part was seeing her pale and listless, with a blank look on her face. Dakota was out of the pediatric intensive care unit in a day and a half, though she faced more recovery time. She didn t sleep, eat or smile, just cried and stared. She also became really afraid of the nurses and doctors. But everybody at the hospital was fantastic, continues Kelly. They understood her fear and worked with me, allowed me to do things for her, like take her vitals while they stood by. The children s hospital really held our chins above water, because this was such a terrible thing to go through, says Kelly. I can t say enough about the hospital; I almost miss that we won t be there regularly to see all the familiar faces. And except for not wanting to be around strangers in scrubs, laughs Kelly, Dakota is doing beautifully and her head is now perfectly round! ww On the day of the surgery, she says she went on autopilot. Everybody that was going to be in the operating room came in to introduce themselves and asked if we had any questions, says Kelly. I anticipated being more nervous, but I wasn t I attribute that to everyone being so attentive. Together Dr. Macomson and Dr. Yu performed the surgery, which they do roughly two times a month. Making a rounded zigzag cut, Dr. Yu reformed the bone on Dakota s skull into strips, removing the hardened and fused bone so new bone could continue to grow along with her. This type of incision minimizes the appearance of the scar by allowing the hair to fall and overlap it in a natural way, he explains. In the swimming pool, you don t want it to be obvious. Jack Yu, DMD, MD, MS, ED Chief, Section of Plastic Surgery Director of Craniofacial Center S. Dion Macomson, MD Assistant Director, Residency Program Assistant Professor, Department of Neurosurgery mcghealth.org/kids 4

6 Georgiasm Health First Back to Sleep May Lead to Benign, Temporary Flattening of the Head In 1992, after careful consideration of the data linking prone sleeping position (face down, on the abdomen) to sudden infant death syndrome (SIDS), the American Academy of Pediatrics recommended that infants be placed on their backs for sleep. The incidence of SIDS declined by more than 40 percent, but as a consequence, the number of infants with flattening of the occiput (the back of the head), due to prolonged sleep time on the back, increased. This condition, also known as benign deformational plagiocephaly or oblique head, can also be due to the infant s position before birth in the uterus, especially when there is crowding due to multiple infants. The incidence in infants may be as high as 5 to 48 percent of all babies and tends to be higher in non-breastfed infants. It may be difficult to differentiate this benign, self-limited condition from craniosynostosis, in which one of the sutures of the head closes prematurely, before growth is complete. With plagiocephaly, there may be a shift of the ear forward on the side of the flattened occiput, along with a shift in the forehead and cheek, which gives the appearance of an enlarged head. To prevent this condition from developing, parents should be encouraged to give the baby some tummy time on a firm surface, while the baby is awake and parents are alert and watchful. The baby should always sleep on its back, but the position of the head can be alternated between facing to the right and to the left. The infant should not spend prolonged time in a car seat, except when riding in a vehicle. If flattening of the back of the head begins to develop, the baby s health care provider should instruct the parents to do neck exercises on the infant with each diaper change and to move the infant s crib so that the infant s preferred view will be away from the flattened side of the head. If there is an associated torticollis, or shortening of the muscles of the neck on one side, the infant may need physical therapy. Rarely, an infant may need referral for a skull-molding helmet for persistent or unresponsive plagiocephaly. These helmets are most helpful between the ages of four and twelve months. After a year of age, they are unlikely to have benefit. Surgery is needed in only the most severe forms of plagiocephaly. The normal course of events is improvement over time, as the child develops better head control and begins to sit upright, crawl and spend less time on his or her back. A recent study showed that most affected children have normal shaped heads by 3 to 5 years of age. Alice Little Caldwell, MD Assistant Professor of Pediatrics 5

7 1828 News from the Child Health Discovery Institute Researchers Seek Answers to Bone Growth Regulation Researchers at are hoping to understand a secret language: the one bone cells use to talk to each other. What is that language converting the physical pull of the bone stretching to grow, to cells knowing it s being pulled to grow? he asks. It s the foundation that underpins the ability to self-regulate and antagonistic coupling, the necessary steps in tissue homeostasis. His team is using animal models to test an enzymatic fractal process, Ets-2 Phosphorylation. The Ets-2 study looks at the smaller components of bone homeostasis. We re chasing down signal processing by following what happens to the molecules in animal models when the skull stretches to grow, says Dr. Yu. If researchers can figure that out, he says, the idea is that they can then determine a way to fool a bone into thinking it needs to grow. If we can understand how bone regulates itself, explains Dr. Yu. We may be able to apply that knowledge to help with conditions such as osteoporosis. If the brain grows half an inch, the skull bone grows just exactly that much, explains Dr. Jack Yu, a pediatric plastic surgeon who specializes in craniofacial surgery and has been leading bone skull studies for 20 years. We re trying to determine how the cells know when and how much to grow. Jack Yu, DMD, MD, MS, ED Chief, Section of Plastic Surgery Director of Craniofacial Center In order to determine how things go wrong with bone growth, such as with craniosynostosis (in which bony plates of the head fuse together abnormally), the team is looking at what s in place when things go right. How does brain growth dictate bone growth, no more and no less? says Dr. Yu. It s a beautiful orchestration of corresponding reactions. It s a simple, elegant yet highly effective process. Dr. Yu and his team have published an article in the journal, Plastic and Reconstructive Surgery, on their research on mechanotransduction the secret language that involves the conversion of physical signals to biochemical signals. mcghealth.org/kids 6

8 Georgiasm 1828 Children s Miracle Network Celebration Telethon A True Celebration For Mia Walker, it was a snakebite; for Mark Gregory, chronic lung disease; Margaret Beckum, cancer, and Alyha Williams, scleroderma. These children hail from rural Springfield, South Carolina stretching down to coastal Savannah. They re separated by circumstances and geography, but their illnesses led them to the same place MCGHealth Children s Medical Center. They shared their patient experiences during the 26th annual Children s Miracle Network Hospitals Celebration, which was broadcast live from the lobby of MCGHealth Children s Medical Center on Sunday, June 5 on WRDW-TV Channel 12. This special event celebrated the funds raised by sponsors and donors to benefit programs and services at MCGHealth Children s Medical Center, the area s only children s hospital. The broadcast included video tours of the hospital, conversations with patients, donors and staff and recognition of committed sponsors. The 2011 Children s Miracle Network Celebration announced the grand total of $857,893 in pledges raised to support the programs and services of the MCGHealth Children s Medical Center. For more information or to make a donation, call , or visit mcghealth.org/giving. The MCGHealth Children s Medical Center has been a beneficiary of the Children s Miracle Network since Money raised helps fund the many resources needed to enhance and maintain the quality of care children receive. Editor-in-Chief: Bernard L. Maria, MD, MBA Associate Editor: Alice Little Caldwell, MD Editorial Assistants: Liz Stockstill (MCGHealth Marketing Department) Jordan Freeman and Jessica Munday (Trio Solutions Inc.) Feature Writer: Mary Sue Lawrence (Trio Solutions Inc.) Contributing Writers: Joshua Smith, MD; Nancy Wood, MD; James Mumford, MHSA, FACHE; Kimberly D. Basso, RN, MSN; and Liz Stockstill, MCGHealth Marketing specialist Publishers: Trio Solutions Inc. mcghealth.org/kids 7