New tool for craniosynostosis treatment highlights Neuroscience Institute’s push to advance care

For pediatric neurosurgeon, Dr. Suresh Magge and his colleagues in the CHOC Neuroscience Institute, Christmas this year arrived in late June.

That’s when a 3D camera system was installed in the neurosurgery clinic that will significantly advance CHOC’s mission to treat craniosynostosis, says Dr. Magge, PSF neurosurgery division chief for CHOC and co-medical director of the Neuroscience Institute.

Craniosynostosis, which affects 1 in every 2,000 infants, causes an infant’s skull to fuse early, creating an irregular skull shape, and can lead to increased pressure on the brain as a child matures. This can lead to headaches, vision problems, and cognitive issues. 

The 3D motion-capture camera can, in seconds, capture a comprehensive array of images that will allow CHOC neurosurgeons to better analyze and measure in detail a child’s head. This, in turn, will allow them to enhance research in craniosynostosis and design the best possible treatments.

Dr. Suresh Magge, neurosurgery medical director
Dr. Suresh Magge, neurosurgery medical director

“This really makes a difference,” says Dr. Magge, noting that traditional 2D photos and measurements “only go so far.”

“This new camera allows us to get data quickly and safely,” Dr. Magge says.

CHOC recently had its first craniosynostosis patient imaged by the 3D camera.

A push to greatness

The new camera is a critical step in Dr. Magge’s push to advance the path of the Neuroscience Institute in becoming a world-class destination for neurological care.

Dr. Magge was recruited to CHOC last October after an 11-year tenure at Children’s National Hospital in Washington, D.C., where he started the medical center’s neurosurgery fellowship training program and was the director of medical student education in pediatric neurosurgery.

During his time at Children’s National, Dr. Magge started the region’s first minimally invasive craniosynostosis program. He has brought this surgery to CHOC as well.

The neurosurgery division also includes Dr. Michael Muhonen, Dr. William Loudon, and Dr. Joffre Olaya.

For the last several decades, the go-to surgery to treat craniosynostosis has been an open surgical correction called calvarial reconstruction. For this surgery, doctors must wait until the child is 6-12 months of age and perform a large surgery that involves opening the scalp, taking apart the entire skull, then putting it back together.

“It’s a good surgery, and most kids do well, but we have newer techniques that are less invasive,” Dr. Magge says. The open calvarial reconstruction surgery generally takes 4-6 hours and requires a hospital stay of 3-5 days, as well as a blood transfusion during surgery. 

Unlocking the skull

The minimally invasive procedure Dr. Magge learned during his fellowship at Boston Children’s Hospital involves using an endoscope with a camera attached to its tip.

After making one or two small incisions, Dr. Magge goes under the scalp and then under the skull, using the endoscope to separate the skull from the underlying tissue. He then cuts out a piece of bone — 1 to 2 centimeters in width – to “unlock” the skull.

This surgery only takes about an hour, and most children don’t need a blood transfusion and can go home the next day. After surgery, they wear a molding helmet that helps to reshape the skull. This minimally invasive surgery is generally done by 3-4 months of age (earlier than the open surgery).

“The data shows this surgery works very well,” says Dr. Magge, who has given many presentations and written multiple papers about this procedure.

The aesthetic results have been shown to be excellent in many papers, Dr. Magge says. What still needs to be verified by research are the long-term cognitive outcomes of patients after either the open or minimally invasive surgery.

To that end, Dr. Magge launched a study about two years ago involving patients from multiple hospitals that looks at children five years after surgery. Dr. Magge plans to enroll patients from CHOC in the study.

He estimates the study will be completed in about two years. 

Working with plastic surgeon Dr. Raj Vyas, Dr. Magge says CHOC offers comprehensive cranio-facial services. 

Dr. Raj Vyas
Dr. Raj Vyas

“To be comprehensive,” he says, “you have to offer traditional surgery as well as minimally invasive surgery.” 

Part of the funding for the new 3D camera came from a CSO grant established by CHOC Vice President for Research and Chief Scientific Officer Dr. Terence Sanger, a physician, engineer, and computational neuroscientist who also is vice chair of research for pediatrics at the UCI School of Medicine.

Dr. Terence Sanger
Dr. Terence Sanger

The 3D camera arrives as significant infrastructure changes are underway at the Neuroscience Institute: CHOC recently opened its new state-of-the-art outpatient center, establishing a clinical hub for caregivers to serve patients and families in a centralized location. Additionally, plans are underway to expand the hospital’s inpatient neuroscience unit.

“CHOC has been very supportive of the Neuroscience Institute,” Dr. Magge says. “I’m very excited.”

Learn more about the CHOC Neuroscience Institute.

CHOC intraventricular hemorrhage and hydrocephalus patient accepted to medical school

Twenty-four years ago, Dr. Michael Muhonen, who had started his pediatric neurological practice at CHOC only a year earlier, treated a baby born with what essentially was a traumatic brain injury.

The infant boy, born at 23 weeks and weighing 3 pounds, suffered an intraventricular hemorrhage – bleeding inside and around the ventricles, the spaces in the brain containing the cerebrospinal fluid.

It was a grade-four bleed – the worst. Dr. Muhonen gave the boy an 80-percent chance of having some form of serious neurological dysfunction such as cerebral palsy. The newborn also had hydrocephalus, which required a shunt to be implanted in his head to drain excess fluid to his abdominal cavity.

That baby, Eric Rhee, is now 24.

Recently, over coffee, Eric talked about his plans this year: He’s moving to Bethlehem, Penn., to attend the Temple/St. Luke’s School of Medicine.

“Wow, I would never have predicted this,” Dr. Muhonen says, with a wide smile. “I’ve seen many grade-four bleeds in infants, but I don’t recall any who have succeeded to the degree that Eric has.”

Eric, who since summer 2019 has been working as a medical scribe at CHOC and, with Dr. Muhonen, on a research paper on shunts like the one that will stay in his body for the rest of his life, is a bit surprised himself.

Of being accepted into medical school, Eric says: “I just want to be consistent, reliable, and efficient at what I do, learning from superiors who spent years upon decades refining their craft.”

Dr. Muhonen attributes his remarkable recovery, in part, to the high-quality neonatal care that Eric received during his three-month stay in CHOC’s NICU as an infant.

“The odds were extremely stacked against him,” Dr. Muhonen says. “He’s unique. It speaks to the kind of person he is that he’s been able to graduate from high school and UC Berkeley with honors and go on to medical school. I’m humbled that I could be a minor part of his journey.”

Dr. Michael Muhonen

Shunts like the one Eric has for his hydrocephalus typically get replaced every five years due to corrosion and other issues. Eric had his replaced only once when he was a child, and it wasn’t until December 2016 when he had to have it replaced again. Dr. Muhonen, who has seen Eric regularly over the years, consulted with neurosurgeons at a hospital near UC Berkeley before the decision was made to replace the entire shunt.

After graduating from UC Berkeley in 2019, Eric started working at CHOC while studying for the MCAT.

“One of the things I liked about CHOC growing up, I always felt like I was at home,” Eric says. “Even though I felt like I was in a very vulnerable place, I was always at ease.

“They’re kind and really good at what they do,” he says of CHOC clinical and related staff. “I want to be like that, too. Everyone at CHOC is a master of their own craft and essential to accomplishing a bigger objective. Every person is important.”

Eric took the MCAT in January 2020 and found out in December that he got accepted into Temple/St. Luke’s School of Medicine.

Dr. Muhonen has no doubts Eric will make a great doctor.

“It will be a physician like Eric who will make a great discovery,” he says. “Instead of relying on a shunt to treat hydrocephalus, maybe he’ll make a discovery to obviate the need for a shunt and have the brain internally drain water on its own somehow.”

Says Eric: “I just want to be trustworthy and dependable while making meaningful connections and having an impact on others.”

Learn more about CHOC’s Neuroscience Institute.

Neuroscience leaders discuss innovations and their successes, and the need for much more work to be done

Two of CHOC’s leading pediatric neurosurgeons recently shared their insights on how innovation is helping to close the gap between clinical needs and the availability of pediatric devices, but how there is much more work to be done to get critically ill kids the treatments they need.

The webinar, “From Clinical Insight to Commercialization: Innovations That Can Transform Pediatric Healthcare,” featured Dr. Suresh N. Magge, CHOC CS Neurosurgery Division Chief, and co-director of CHOC’s Neuroscience Institute, and Dr. Michael G. Muhonen, the institute’s previous co-director.

Hosting the “OC LIFe (Lifesciences Innovators Forum)” on April 28, 2021 was Dr. Terence Sanger, a physician, engineer, and computational neuroscientist and vice president, chief scientific officer at CHOC, and vice chair of research for pediatrics at the UCI School of Medicine.

“As innovators, we should never be satisfied,” said Dr. Sanger, who specializes in movement disorders and who helped pioneer deep brain stimulation, which has yielded positive outcomes. “An innovative and collaborative approach is required so that pediatric patients can have access to the fit-for-purpose devices they need.”

Brain tumor treatments

Drs. Magge and Muhonen took turns discussing new neurosurgical technologies and opportunities for interventions.

Dr. Magge focused on new technology that has been used to treat brain tumors, which are a different breed compared to adult brain tumors. More often, Dr. Magge said, pediatric brain tumors are of a lower grade and can be treated.

“Many kids have gone on to live good lives thanks to innovation, research, and applying the technologies we have,” Dr. Magge said.

In one example, he detailed how microsurgical techniques have greatly aided in the removal of a craniopharyngioma, a benign tumor that usually arises in the base of the brain near the pituitary gland that can be dangerous or life threatening if not treated.

“If you can get the tumor out,” Dr. Magge said, “you can cure the patient. But it’s challenging because it’s in a deep part of the brain.” 

During the procedure, the neurosurgeon must locate some of the natural divides of the brain and separate them out to get to the tumor. Microsurgery allows the neurosurgeon to work between very narrow areas.

With a technology known as surgical navigation, neurosurgeons can pinpoint exactly where they are in the brain and get to very specific areas. Another technology is a powerful microscope that magnifies small areas of the brain. In addition, ultrasound and MRI within the operating room can tell you in surgery if there is any tumor left. 

“This is all thanks to innovation and technology that we are incorporating in surgery,” Dr. Magge said.

Dr. Magge then discussed medulloblastomas, one of the most common types of tumors neurosurgeons see in kids. Such large tumors grow in the lower back part of the brain — the cerebellum, which is involved in muscle coordination, balance, and movement.

Thirty years ago, Dr. Magge said, kids with medulloblastomas received high doses of radiation that left a lot of them with severe cognitive and hormonal deficits.

The treatment for medulloblastomas had evolved so that less radiation is used in the treatment. In addition, in the last decade, researchers have discovered that these tumors differ significantly based on their genetic makeup.

“These tumors have multiple genetic subtypes, and we can target them genetically with different types of treatments,” Dr. Magge explained.

He said innovation also has led to advances in the treatment of diffuse intrinsic pontine gliomas (DIGP), highly aggressive and difficult-to-treat brain tumors that grow in an area of the brainstem that controls many of the body’s most vital functions such as breathing, blood pressure, and heart rate.

The prognosis for DIPGs remains very poor because they are considered non-resectable tumors – ones that cannot be removed with surgery. Life expectancy is eight to 12 months after diagnosis.

“This is one of the toughest diagnoses we have to give to families because of the lack of good treatment options,” Dr. Magge said.

For years, biopsies were ruled out because they could cause significant side effects, and neurosurgeons saw no point in performing them since there were no treatments. Without biopsies, the tumor tissue could not be studied in a lab for potentially effective treatments. 

Technology has changed this is the last 10 years, Dr. Magge said, thanks to stereotactically guided needles that allow neurosurgeons to perform DIPG biopsies safely.

“We at CHOC and other pediatric hospitals have shown we can do this safely with minimum morbidity,” said Dr. Magge, who has participated in a large clinical trial regarding DIPG biopsies.

“With this technology, we can get tissue and genetically sequence these tumors and find out if there are certain mutations that are particularly amenable to certain treatments,” Dr. Magge said of this precision-medicine approach. 

“These are small steps along the path,” he added. “We have by no means found all the answers. We have so much farther to go, but I think we’re on the right track.” 

Closing the gap

Dr. Muhonen recalled one of the first patients he saw when he came to Orange County in 1995: a young girl with severe spasms in her legs. She couldn’t walk without assistance.

“We had to do something innovative,” Dr. Muhonen said.

He had injected baclofen, a muscle relaxer and antispasmodic agent, into the spinal column of an adult the year before, but never in a child. After receiving approval to do so, he implanted a device that allowed long-term injection of baclofen in the girl’s spinal cord. Six months later, she was able to walk and even run on her own.

In another example of innovation, Dr. Muhonen worked for five years on helping to develop a wireless sensor to measure pressure in the brain. The FDA approved the device for adults, but has yet to for children.

Most companies get medical devices approved for adults because it’s easier, because there’s a larger patient population, and there’s more money to be made. 

“The bulk of challenges associated with developing and accelerating pediatric medical devices is market-driven,” Dr. Muhonen said. “We want children to get the best possible care available, but the relative market size is small compared to adults, which is one reason some device makers avoid it.”

One of Dr. Muhonen’s chief interests is treating hydrocephalus, the buildup of fluid in the ventricles deep within the brain.

Innovation in this area has been a long time coming, he said, since the invention in the early 1950s of a shunt that drained fluid from the brain into the abdominal cavity. Many problems can occur with the shunt, such as spontaneously twisting up into a knot due to a child’s movement or calcifying and breaking apart after being in the body for a long time. Kids who received a shunt typically face more than 10 surgeries, Dr. Muhonen said.

“The holy grail for pediatric neurosurgeons is, can we create a ‘smart shunt?’” Dr. Muhonen said.

An ideal shunt, he said, could be programmed to drain a specific amount of water and measure pressure.

Dr. Muhonen said a derivative from cone snails is inspiring research into a new generation of painkillers for adults, but has yet to be approved for testing on kids.

Impediments to innovation

Dr. Sanger asked Drs. Magge and Muhonen about impediments to pediatric innovation. Ethically, he posited, shouldn’t new devices and other innovations be tested in adults first? 

“I don’t think there are any easy answers to this,” Dr. Magge said. “It’s difficult. You don’t just do a biopsy on a tumor that might help kids in the future. If you perform surgery on a child, there has to be some potential benefit to that child.”

Dr. Muhonen said children are the most vulnerable of society and thus are the worthiest of innovations in healthcare. 

Dr. Magge said he and others at CHOC have been looking at ways to inject dyes to paint brain tumors to more easily distinguish them from healthy brain tissue.

“Sometimes the tumor is obvious, sometimes it’s more challenging,” he said.  While dye injections have been used in adults, it is less commonly used in children.

Dr. Sanger mentioned “big effect sizes” resulting from innovation in pediatric medicine. 

“We’re used to the idea of statistical research involving a lot of patients,” he said. “But this is a different type of research. You take someone who has never walked before and now they’re running. You take someone who is going to die of a brain tumor and now they’re not. These are very big effect sizes.” 

“There are good reasons for the regulations we have,” Dr. Magge said. “That being said, that doesn’t mean we can’t innovate. And there are mechanisms for us to do that, and to do it safely.

“Our first motto is, ‘Do no harm,’” Dr. Magge continued. “I always tell residents to do the right thing and treat each patient as if they were your own child. Doing the right thing means asking the right questions. ‘How can we do this better?’ You can always learn from everything you do. At the end of every procedure, you critique it. You’re constantly learning. That’s what I always encourage.” 

Dr. Sanger closed the session by noting that clinical evidence should ideally be reflective of the spectrum of pediatric patients and the developmental differences that can impact the use and effectiveness of medical devices.

“This is a collaborative effort,” he added. “CHOC is working closely with the FDA’s new System of Hospitals for Innovation in Pediatrics – Medical Devices (SHIP-MD) Program, our academic partners, industry, entrepreneurs and the investor community to close the gaps. Also, we are now practicing medicine in a world immersed with data. Advances in computing and health information technology have given rise to new sources and types of biomedical data. Clinicians know real-world data will continue to emerge as a source of clinical evidence.”

The Presenting Sponsor of the webinar, “From Clinical Insight to Commercialization: Innovations That Can Transform Pediatric Healthcare,”  was Biocom California, which connects life science organizations to each other so they can collaborate and work smarter together. The CHOC Research Institute co-sponsored the hour-plus session.

The webinar was presented in partnership with SBDC @ UCI Beall Applied Innovation, a resource for any high-technology, high-growth, scalable venture from the community or the UCI ecosystem that needs help with business planning, business development and funding-readiness.

Learn more about CHOC’s Neuroscience Institute.

Innovating Toward a Cure: Smart Shunt Technology for Hydrocephalus

Shunts for hydrocephalus require an average of about 10 revisions during the patient’s lifetime. New innovations at CHOC aim to revolutionize shunt design to give patients more control in the management of this condition.

For the past few years, Dr. Michael Muhonen, pediatric neurosurgeon at CHOC, has been developing and refining a “smart shunt,” which allows families of children with hydrocephalus to measure intracranial pressure at home or determine if the shunt is failing. The shunt has a wireless sensor that communicates with a family’s smartphone-like device when held near their child’s head. If the sensor determines the pressure is too low, a magnet is used to manipulate the pressure. Another device within the valve allows the parent to potentially disocclude the shunt catheter if it’s plugged.

Dr. Michael Muhonen, pediatric neurosurgeon at CHOC

The technology received FDA approval last year, and it should be commercially available within one year.

“I have families who won’t go more than a few hours away from their pediatric neurosurgeon because of fear. If the shunt plugs up, what are they going to do?” Dr. Muhonen says. “Every neurosurgeon who treats hydrocephalus has a goal to make a simplified system for both the families and the doctor, and a system that saves future surgeries down the road. Having wireless technology so you can measure pressure with your smartphone would accomplish that. I’ll keep working on this until it happens.”

Smarter technology would also result in far fewer operations for patients.

“Some patients come in regularly with headaches, failures, broken and fractured shunts or calcium on the tubing; we follow them regularly and the chronically shunted patient has too many operations,” Dr. Muhonen says. “I am excited about the prospects that this new technology has to offer to many patients with hydrocephalus.”

The supportive culture at the CHOC Neuroscience Institute has aided Dr. Muhonen’s research and advancement of wireless technology in shunts.

“CHOC has been very supportive in morphing into a hospital that supports research and cure, rather than a purely clinical children’s hospital,” Dr. Muhonen says. “We’re also fortunate to have donors in the community who want to help. Together, we’re able to strengthen our focus on being a leading research and academic institution.”

Our Care and Commitment to Children Has Been Recognized

CHOC Hospital was named one of the nation’s best children’s hospitals by U.S. News & World Report in its 2020-21 Best Children’s Hospitals rankings and ranked in the neurology/neurosurgery specialty.

USNWR Neurology and Neurosurgery award

Learn how CHOC’s neuroscience expertise, coordinated care, innovative programs and specialized treatments preserve childhood for children in Orange County, Calif., and beyond.

CHOC Experts to Speak at International Hydrocephalus Conference

Two CHOC pediatric neurology experts will speak at an upcoming conference featuring the latest education on hydrocephalus.

Including internationally recognized medical professionals and researchers, HA Connect – National Conference on Hydrocephalus, held June 28-30 in Newport Beach, will address the medical, educational and social complexities of living with hydrocephalus through interactive discussions, workshops and hands-on exhibits.  Over 500 people are expected to attend the international conference.

Dr. Michael Muhonen

Dr. Michael Muhonen, board-certified neurosurgeon, division chief of neurosurgery and medical director, CHOC Neuroscience Institute, will present a lecture on the anatomy of the brain, with a focus on the cerebral ventricles, cerebral spinal fluid physiology and hydrocephalus.

On the second day of the conference, Dr. Muhonen and Dr. Anjalee Galion, board-certified pediatric neurologist and associate director of the CHOC Sleep Lab, will present on headaches and current management. Their lectures will be specifically focused on the etilogy and treatment of headaches in patients with hydrocephalus, pseudotumor cerebrii, and other brain pathology.

Dr. Anjalee Galion

All CHOC professionals and employees are invited to attend.  The CHOC Neuroscience Institute is offering assistance with the cost of registration. For details, email Rhonda Long, director, CHOC Neuroscience Institute at rlong@choc.org.

Learn more about HA Connect – 2018 National Conference on Hydrocephalus.

Dr. Michael Muhonen Discusses Dermoid Cysts

A dermoid cyst is a small bump that is present at birth, and is typically found near a child’s head, Dr. Michael Muhonen, director of neurosurgery and medical director of the CHOC Neuroscience Institute, tells “American Health Journal.”

These cysts continue to grow and enlarge with time, and treatment is always surgical, says Dr. Muhonen.

Learn more about diagnosis and treatment of dermoid cysts in “American Health Journal,” a television program that airs on PBS and other national network affiliates that reach more than 30 million households.

Each 30-minute episode features six segments with a diverse range of medical specialists discussing a full spectrum of health topics. For more information, visit www.discoverhealth.tv.

Michael Muhonen M.D., earned his medical degree and completed his residency at Oral Roberts University in Tulsa, Okla. His clinical interests include hydrocephalus, cerebral palsy, spina bifida, brain tumors, Moyamoya disease and vascular malformations of the brain.

Get more information about referring patients to CHOC, including a referral information directory, services directory and referral guidelines.