Ask the Doctor: Positional Plagiocephaly

We have been working with the team at Texas Children's Hospital to bring you some FAQs as part of our effort to provide relevant, timely, and much-needed medical information to you.  We believe that helping patients and their families find and access the right craniofacial team is an essential service that we provide. Today's FAQs concern babies with plagiocephaly who wear a helmet.

From the doctor:
Our patient families have helped generate Frequently Asked Questions about cranial orthotic helmets. Some babies are in helmets for positional plagiocephaly, while others are in helmets after endoscopic craniosynostosis surgery. We prepared some typical answers to helmet FAQ's. Many families have worked with Jim Brookshier CPO LPO (orthotist), who has been taking care of children's helmet and head shape needs in Texas for over 10 years. Jim has partnered up with me to write these FAQ sheets. Of course, this information cannot substitute for regular visits with your orthotist and your physician to avoid problems and to work towards the best outcomes possible.

Sandi Lam MD MBA
Pediatric Neurosurgeon
Texas Children's Hospital Craniofacial Surgery Team

See the PDF here.

From The Bench: Minority and Public Insurance Status: Delay to Treatment

By Francis Smith

Minority and Public Insurance Status: Is There a Delay to Alveolar Bone Grafting Surgery?

from The Cleft Palate-Craniofacial Journal, Volume 54, Issue 1, January 2017.

A new article in The Cleft Palate-Craniofacial Journal illustrates the inequality of access of children to critical cleft lip/palate surgery due to minority status.

There are certain ages or life stages at which repair of cleft lip and palate and subsequent follow-up procedures need to be done to ensure good oral and dental function and health, according to published guidelines . Primary cleft repair takes place within the first 12-18 months of life, and follow-up surgeries should be performed according to the stages of development of the palate and other oral structures. A cleft in the lip and/or palate can result in a gap in the alveolus, or bony structure that forms the upper dental arch. One critical surgery is alveolar bone grafting (ABG) surgery, in which bone is taken from the pelvis (specifically, from the iliac crest) and put into the alveolar gap resulting from the cleft, to close the gap. ABG surgery is typically performed at age 8 or 9, during transition from the deciduous dentition ("baby teeth") to permanent dentition ("adult teeth"). This surgery is necessary in order to stabilize the dental arch, provide bony support for permanent teeth and allow for complete oral rehabilitation going forth. Without this surgery, or if it is delayed, complete repair of the cleft is impossible, the dental arch is unstable and cannot support permanent teeth, and oral rehabilitation is compromised.

Unfortunately, children in minority racial groups (particularly black and Hispanic) and who are on public medical insurance (such as Medicaid and state Title V plans) have less access to health care than those in non-minority groups and insured privately. This includes children with cleft lip and/or palate. Prior research has found such a delay in cleft palate repair and follow-up surgeries in children in minority groups and who are on public insurance plans. 

Researchers at the Children's Hospital of Philadelphia (CHOP) did a 13-year retrospective chart study of 233 patients who underwent ABG surgery at CHOP to determine when the children had the surgery. The researchers included only children with nonsyndromic clefts (not part of any syndrome) who had prior preliminary cleft palate repair at CHOP and stayed on with the same team over the years for the ABG surgery; those who left the team before ABG were excluded. Also, this study is the experience of one institution, and not necessary reflective of the general experience among other hospitals. On average, the age at which ABG was done was 8 years. However, Caucasian and Asian children underwent ABG approximately one year earlier than their African-American and Hispanic peers. Further, the researchers found that most of the patients examined in the study who had ABG surgery were white males as well as on Asian children who were adopted into white families and got timely cleft care once they entered the healthcare system. In this study, insurance status played no significant role--the type of insurance did not affect the timing of surgery, and less than 20% of patients were on public insurance.

From their results, the team concluded that there are significant delays and disparities in timely ABG surgery among black and Hispanic children compared to their white and Asian peers. This demonstrates a need to increase minority access to good healthcare and treatment for cleft lip and palate in a timely fashion.

Texas Children's shares Craniosynostosis Helmet FAQs

Today on the blog, we are expanding our "Ask the Doctor" series!

We have been working with the team at Texas Children's Hospital to bring you some FAQs as part of our effort to provide relevant, timely, and much-needed medical information to you.  We believe that helping patients and their families find and access the right craniofacial team is an essential service that we provide. Today's FAQs concern babies who wear helmets after craniosynostosis surgery and next week, we'll post the FAQs for plagiocephaly helmet wear.

From the doctor:
Our patient families have helped generate Frequently Asked Questions about cranial orthotic helmets. Some babies are in helmets for positional plagiocephaly, while others are in helmets after endoscopic craniosynostosis surgery. We prepared some typical answers to helmet FAQ's. Many families have worked with Jim Brookshier CPO LPO (orthotist), who has been taking care of children's helmet and head shape needs in Texas for over 10 years. Jim has partnered up with me to write these FAQ sheets. Of course, this information cannot substitute for regular visits with your orthotist and your physician to avoid problems and to work towards the best outcomes possible.

Sandi Lam MD MBA
Pediatric Neurosurgeon
Texas Children's Hospital Craniofacial Surgery Team

See the PDF here.

Media Monday: Barrow Neurological Institute Takes Stand Against Bullying

Time for a Media Monday post that will warm your heart and truly inspire you. Watch this video from the Barrow Neurological Institute and read below for the article about the project.



SOURCE: http://sports.yahoo.com/news/barrow-neurological-institute-takes-stand-192900746.html

Barrow Neurological Institute Takes Stand Against Bullying

Produced by the Barrow Cleft and Craniofacial Center, the two-minute video features patients with craniofacial differences, including cleft lip and palate. The video’s message is simple: There is beauty and “wonder” in everyone.

From birth on, children with craniofacial disorders encounter a multitude of challenges, including special feeding during infancy, multiple surgeries and dental procedures, speech therapy, and other intensive care.

But for many of them, accepting themselves and being accepted by others remains a challenge.

“The piece that makes or breaks our patients in life is acceptance,” says Patricia Glick, DMD, a member of Barrow’s craniofacial team. “It is an important part of their lives—to connect with others and be accepted.”

The Barrow medical professionals believe that children who see and learn about craniofacial disorders are less likely to stare, ask cruel questions, taunt or reject peers with facial or skull differences, and are more likely to be accepting.

“I would like our patients to be able to feel like just another face in the crowd,” says Lori Takeuchi, Program Coordinator at Barrow.

Nearly 7,000 babies with cleft lip and palate are born in the U.S. each year. The Barrow Cleft and Craniofacial Center, supported by the Inzalaco family, is among the largest, most comprehensive craniofacial programs in the nation.

Awareness of craniofacial disorders is growing. The Children’s Craniofacial Association is sponsoring the 12th Annual Craniofacial Acceptance Month in September, andWonder, a movie based on the best-selling novel by R.J. Palacio about a child with a severe craniofacial disorder, will debut in theaters in 2017.

Schools reading Wonder can contact Barrow at 602.406.3560 to learn more about educational anti-bullying sessions for accepting children with facial differences.

About Barrow Neurological Institute

Barrow Neurological Institute at Dignity Health St. Joseph’s Hospital and Medical Center in Phoenix is internationally recognized as a leader in neurosurgery, neurology, neurological research and patient care. Learn more by visiting barrowneuro.org.

Watch and share the video.
Read about how Barrow is interacting with Wonder.

View source version on businesswire.com: 

http://www.businesswire.com/news/home/20160914006282/en/

Happy #CAM2016 from Dr. Ricalde & Team!

Happy Craniofacial Acceptance Month 

from Dr. Pat Ricalde's team at the Florida Craniofacial Institute! We love their fun and creative video!

Thank you all for helping us celebrate!

Did you know? Dr. Ricalde is also having a golf tournament this coming February and they have pledged to send one family to the CCA Retreat from their fundraising efforts! We are thrilled to have the support. Learn more about the golf event in Tampa here.

Craniofacial Acceptance Month Kicks Off!

CRANIOFACIAL ACCEPTANCE MONTH IS HERE!

We have several special videos for you this month... but, we're kicking off the special wishes from the team in Dallas at Medical City Hospital. Thank you team for your special wish! Stay tuned, friends. CCA will be updating social media and the blog daily.




You can also create your own "Happy #CAM2016" video that CCA will share! Simply record your video (less than 30 seconds, please!) and make sure to tag CCA and share with us.

While you're at it ... why not make it a dance?! Check out Sydney's "Dance for Acceptance" video challenge and get your friends involved. #DanceForCraniofacialAcceptance

CL&P Medical Research Survey

The Family Experiences Survey for Florida Families of Children with Orofacial Clefts 

The Florida Birth Defects Surveillance Program is pleased to announce that we have developed a survey to help families collectively share their experiences in accessing important resources, services, and supports for children birth to 18-years-old born with cleft lip and/or palate. The results of this survey will be used to develop recommendations for improving systems of care here in Florida. We would be very grateful if you could help us reach as many participants as possible.

Please distribute this survey to any parent with a child under the age of 18 who are born with orofacial clefts in Florida. This anonymous survey should take no longer than 30 minutes to complete, and will be distributed throughout the state. Only one survey per family should be completed.
The survey is available online for Florida residents at  https://usfhealth.az1.qualtrics.com/SE/?SID=SV_blJ6lNvxGvIxlpr

A Spanish version of the survey is currently under development.

Sincerely,

For further information, please contact: Jennifer Marshall, PhD, CPH                    Russell S. Kirby, PhD, MS, FACE Research Assistant Professor                  Distinguished Professor and Marrell Endowed Chair 813-396-2672                                            813-396-2347 email: [email protected]            email: [email protected]

From the Bench with Francis

Today we have an entry from one of our longtime CCA members, Dr. Francis Smith. He recently returned from a research trip and shares this update with us. We are looking forward to the culmination of these interviews he is conducting. Thanks, Francis!

From the Bench with Francis

by Dr. Francis Smith

In the middle of March this year, I attended an international craniofacial research conference in Ventura, CA, called the Gordon Research Conference on Craniofacial Morphogenesis and Tissue Regeneration. Part of a larger series of Gordon Research Conferences, this one is held biannually, alternating between Ventura, CA, and Il Ciocco, Italy (hence, the next one, in 2018, will be in Italy). As a researcher, since I was in graduate (PhD) school years ago, I have gone to many scientific conferences in the US and abroad to present my research. Often I get to share not only my research, but also my unique connection to my research.

In advance of the conference, I made a poster about my ongoing public engagement initiatives and strategies for public outreach and fostering collaboration between researchers and craniofacial doctors.

Our conference was held at a resort hotel (designed by the late Frank Lloyd Wright’s architectural firm) with plenty of conference space and we took all our meals together in the spacious restaurant overlooking the Ventura Harbor.

On the day and a half before the actual conference, there was a Gordon Research Seminar for postdoctoral fellows (like me) and graduate students in which we presented our research to our peers, both oral and poster. I presented my poster on public engagement strategies and reconnected with old colleagues (and met new ones) from round the world.

On the day the conference itself began, I was happy to see many of my old mentors from London, San Francisco, and Calgary come in, as well as others I had met in the past through connections I made at previous conferences and other events. Throughout the rest of the week, I met many new colleagues (it is vitally important in my field to make as many connections as possible—it’s called networking) from across the nation as well as round the world.

Our days were filled with talks from eminent researchers about the latest advances in research concerning the genetics and molecular biology of embryonic development of the craniofacial complex, as well as craniofacial malformations (including some talks on Treacher Collins syndrome; they’re always finding some new culprit gene for it or something new about the etiology of it). 

Midday and afternoon were lunch and free time. I went to downtown Ventura a couple of times just to browse the bookstores and antique malls. I picked up an excellent condition 1946 textbook on oral surgery and walked around the entire harbor and marina, and once waded into the ocean. Later each afternoon there was a poster session (with posters all set up and people discussing them), then dinner, and a full slate of more talks into the evening, followed by social time with a bar on hand.

I presented about the need for us – craniofacial scientists – to reach out and educate the public (as well as government, craniofacial charities, and scientific organizations) about craniofacial anomalies and research. I also recruited more craniofacial scientists and professors in attendance to contact me about being interviewed in person (on video) in future; once I have collected enough interviews, I will make them available to the public and families of children with craniofacial disorders in order to educate them on the potential benefits of our research. 

I also stressed the need for ecumenical cross-communication between researchers and craniofacial doctors, so we could learn from each other and gain perspective from each other, through possible strategies such as attending each other’s conferences and having a comprehensive meeting one day for both sides of the field of craniofacial medicine, as a sort of Vatican II for the craniofacial field. Craniofacial doctors could learn a bit of the science underlying the craniofacial defects they treat, and we researchers could gain the medical perspective on craniofacial disorders from the doctors. I also mentioned the need to lobby the government about craniofacial issues and reach out to scientific societies and craniofacial organizations in order to disseminate our research findings. I also discussed my past and ongoing strategies whereby I have been a mentor to the craniofacial community, as well as reaching out to the public through my past talks around the world and my news media interviews.

All in all, this conference was a great way to reconnect with old colleagues, meet new ones, and spread the word about craniofacial public engagement.

From the Bench with Francis

Today we have an entry from one of our longtime CCA members, Dr. Francis Smith. He recently returned from a research trip and shares this update with us. We are looking forward to the culmination of these interviews he is conducting. Thanks, Francis!

In January I traveled to the United Kingdom for work related to my craniofacial research. I was already familiar with London, having studied at King’s College London (for a degree in biomedical and craniofacial sciences) from 2004-2007. It was good to revisit my alma mater for the first time in 9 years—it was 9 years too long! After all, London was my first home away from home (and my first move away from home and family), and it was the cradle of my career in craniofacial research.

Shortly after I moved to Denver last summer to begin my current research position at the University of Colorado, my supervising professor, my former mentors from the past, and I came up with the idea for me to start interviewing my former professors and colleagues (in both the US and UK) about their craniofacial research and its potential impact and benefits for families of children with craniofacial anomalies as well as the public. These interviews would be videotaped, collected, edited, and ultimately compiled into a publicly accessible online video program as an educational resource.

So, with the help of my current supervising professor and our lab’s grant manager/travel arranger, I made plans to travel to the United Kingdom in mid-January 2016. I would spend a week in London, give some talks and do an interview of my former professors at King’s College London, then spend three days in Dundee, Scotland, to interview another colleague at the University of Dundee Dental School. I eagerly counted down the months, then the weeks, then the days, to my trip; I was excited to get back to London after so long.

After a nonstop overnight flight from Denver to London, I spent my first day reestablishing my bearings and exploring long-familiar parts of the city. The next day, I got right to work. I visited my alma mater (the 18^th century Guy’s Hospital campus of King’s College London) and met up with a bunch of my former anatomy lecturers and reconnected with my craniofacial professors and colleagues (and met new ones) in the department’s laboratories on floor 27 overlooking the Thames. I gave a lecture to my craniofacial and anatomy colleagues recapping what research I have done over the years since I graduated from King’s in 2007, including my work on the effects of hypoxia (low oxygen) in early developing chick embryos. Over the next few days I sat in on other lectures in the craniofacial department. The most important thing I did, however, during that week, was that I interviewed three of my colleagues (while being videotaped) about their research, what advances they have made, and what impact these advances could make on the public and families with craniofacial conditions. We also discussed strategies for education and outreach to the public, as well as how researchers and craniofacial doctors could communicate and work with each other (and ways to promote integration between them) and how we could stimulate global cooperation amongst researchers.

The following week, I traveled north to Dundee, Scotland, to meet a newer friend/colleague who was a professor at the University of Dundee Dental School who also did craniofacial research. Over dinner one evening, we discussed at length our research and strategies for public outreach and advocacy for craniofacial research and awareness, as well as strategies for research-clinical interaction and global research collaboration. The following day, I interviewed him like I did my London colleagues.

All too soon, it was time to head back home to Denver. Revisiting London only strengthened my desire to return there one day long term if my career provides the opportunity. These interviews are only the first two of a series of interviews I intend to collect (from both US and UK colleagues) and ultimately compile into a publicly accessible educational video resource. I look forward to one day making this available as a valuable resource for families with craniofacial conditions as well as the public.

Ask the Doctor: What about the Vertex Bulge?

CCA posts a monthly blog series called, "Ask the Doctor." You can submit your questions to Annie Reeves ([email protected]) and we will ask Dr. Earl Gage of Kids' Plastic Surgery in St. Louis to answer them. Thanks Dr. Gage for helping spread information and resources for our cranio families! Today's question is about the sagittal bump.

Ask the Doctor

Earl Gage, MD

Kids Plastic Surgery, MercyChildren’s Hospital – St Louis, MO

Question: On several forums, we have heard of post-op sagittal craniosynostosis children who develop a vertex bulge (more commonly referred to as a "sagittal bump"). Some doctors seem to indicate that this alone indicates the need for a repeat surgery, whereas other doctors adopt a wait and see approach. Is the vertex bulge always a sign of intracranial pressure if the child is otherwise developing well? What could be the other causes of the bulge/bump?

Answer:  Small bumps on the skull or other contour irregularities are common after cranial surgeries for craniosynostosis. A large vertex bulge occurring after sagittal syostosis correction, however, is rare. In fact, I have only been able to find one report of this phenomenon from a craniofacial team in Oxford, England¹.

In their 2008 series, Marucci and colleagues reviewed 89 patients with isolated sagittal synostosis treated with strip craniectomy. Their patients had the surgical procedure between 5-7 months of age. None of the children had helmet therapy after their procedure as is commonly done after similar procedures here in the United States. Seven of their patients developed a prominent vertex bulge in the months following surgery. All seven of these patients were re-imaged with MRI after the bulge was noted. In 5 patients, there was evidence for elevated intracranial pressure by exam or by MRI, and formal intracranial pressure measurements were done. Pressure was elevated in 4 of these patients. Three patients with elevated pressure and 2 with normal pressure were also found to have fusion of additional cranial sutures on the MRI scans. Genetic testing was done in all children with a vertex bulge, and abnormalities in the fibroblast growth factor receptor gene were found in 2 patients, both of whom also had elevated pressure and additional coronal suture fusion. All seven of the patients ended up having secondary open cranial remodeling procedures, and all but one had resolution of the bulge with open surgery.

While I can’t say with certainty, I suspect that the recommendation for additional surgery in children with a vertex bulge is based, at least in part, on this study. In my opinion, the occurrence of a prominent vertex bulge after strip craniectomy raises at least two important questions. 

First, was the original diagnosis of isolated sagittal synostosis correct? Sometimes, multiple suture craniosynostosis can present atypically and may be misdiagnosed initially, even by experienced surgical teams. When a vertex bulge occurs, it should raise a flag that additional testing for FGFR-associated and other syndromic craniosynostoses should be considered. 

Second, is strip craniectomy really adequate treatment for sagittal synostosis, or is the decompression with these smaller procedures inadequate? Emerging data on endoscopic assisted strip craniotomies seem to suggest that strip craniectomy with post-operative helmet therapy can be expected yield acceptable results when performed by an experienced team, when families are compliant with post-surgery helmet regimens and when the helmet orthotist is good at their trade. The shorter operative times, shorter hospital stays and decreased likelihood of blood transfusions associated with these smaller procedures as compared to open cranial vault procedures are also appealing. However, every craniofacial team should make honest, periodic assessments of their results for head shape, need for re-operation, occurrence of complications and incidence of developmental issues to be certain that their results are as good as those achieved with open cranial remodeling procedures which remain the gold-standard treatment.

So what does this mean if your child has a vertex bulge after strip craniectomy? I would recommend repeat imaging (CT or MRI) as well as a repeat eye exam be done to look for evidence of elevated intracranial pressure and to rule out multisuture craniosynostosis. Where there is evidence of elevated pressure by imaging or eye exam, formal intracranial pressure measurement should be discussed with your neurosurgeon. In addition, your pediatrician or a developmental pediatrics specialist should also monitor your child to be sure that your child’s development is progressing as expected. Your team should also strongly consider genetic testing for fibroblast growth factor receptor gene abnormalities and possibly other genes associated with craniosynostosis if these tests were not previously done. Your craniofacial surgeon should also regularly monitor your child’s head growth and follow your child for headaches, vision complaints or other symptoms of elevated pressure like nausea and vomiting.  If additional sutures are fused, there is deterioration of the head shape or there is evidence of elevated intracranial pressure, repeat surgery should be considered.

However, if all of these evaluations are normal and the vertex bulge is mild, then taking a wait-and-see approach is appropriate, deferring the decision on repeat surgery until your child is older and becomes concerned enough about the head shape to want another surgery.

¹ Marucci DD, Johnston CP, Anslow P, Jayamohan J, Richards PG, Wilkie AO, Wall SA. Implications of vertex bulge following modified strip cranioectomy for sagittal synostosis. Plastic and Reconstructive Surgery. 2008 Jul; 122(1):217-24.