Advances in Children's Surgery - Summer 2019

A D VA N C E S I N

Children’s Surgery S U M M ER 2 019

IN THIS ISSUE MAGNETICALLY CONTROLLED GROWING RODS

For early-onset scoliosis, remotely expanded growing rods may offer a smoother road to a straighter spine

1 CHILD LIFE SURGICAL SERVICES

Creating a more comfortable, less fearful surgical experience

3 INTRAOPERATIVE MRI

Real-time imaging for greater surgical precision

4 THE REFRACTORY SLEEP APNEA CLINIC

When CPAP isn’t the answer: exploring surgical options for sleep apnea

6 SPINAL ANESTHESIA

An alternative to general anesthesia for very young children

7 First hospital in Michigan to receive Level 1 Children’s Surgery Center verification from the American College of Surgeons

8 In this issue of Advances in Children’s Surgery, we provide updates from five programs supporting surgical patients at C.S. Mott Children’s Hospital.

While the state-of-the-science technologies and services highlighted here are drawn from different corners of Mott, they have much in common:

M A GN ETICA L LY CON TROL L ED GROWIN G RODS

For early-onset scoliosis patients, remotely expanded growing rods may offer a smoother road to a straighter spine Early-onset scoliosis — a curvature of the spine diagnosed in infants and young children — is extremely rare, impacting fewer than two of every 10,000 children. Surgical procedures to correct the condition are even more rare, since many cases improve on their own as the child grows.

THE EVOLUTION OF GROWING RODS The established surgical modality to control early-onset scoliosis is the implantation of growing rods (typically two rods) in the spine. The growing rods are then expanded at regular intervals by a doctor to help the spine grow in a straighter position.

“When comparing treatment options, it’s important to first distinguish earlyonset scoliosis, diagnosed in infants and children under the age of ten, from adolescent idiopathic scoliosis, which occurs in children ten and older,” says pediatric orthopaedic surgeon, G. Ying Li, M.D. “Until age ten, the spine is still growing rapidly and the lungs are still developing. After age ten, there is less growth in the spine, but the lower extremities continue to grow.” So, fusing the spine, which is commonly recommended to treat adolescent idiopathic scoliosis, is not an option for early-onset scoliosis.

Traditional growing rods (TGRs) are manually expanded during successive surgical procedures, approximately every six months. Each procedure requires a portion of the implantation scar to be reopened to expand the rods. A relatively new alternative, magnetically controlled growing rods (MCGRs), have been FDA-approved for use in the U.S. since 2014. Like TGRs, MCGRs are surgically implanted in the spine. But expansion of the rods no longer requires a return trip to the OR. Instead, magnets embedded in the rods are manipulated using a remote controller outside the body.

• E ach is patient-focused – making surgery safer, more effective and more comfortable.

• Each illustrates our commitment to personalized medicine – no one approach is right for every child; it is important to seek care where the greatest variety of alternatives are available.

• E ach reflects our multidisciplinary approach to care – bringing a diversity of expertise to bear to help parents and referring physicians navigate options and make the right choice for the child.

• E ach are best-in-class offerings available at one of the nation’s leading children’s hospitals.

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MA GN E T I CALLY CO N T R O L L E D GR OWIN G R ODS

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The Mott team uses ultrasound, rather than x-rays, to monitor magnetic rod lengthening. An ultrasound technician scans each rod before and after the procedure to assess whether the targeted growth has been achieved. “Magnetic lengthening is a straightforward outpatient procedure performed in our clinic,” Li explains.

“There is no need to avoid eating or drinking prior to the appointment, no anesthesia, no mask, and no IV. It does take longer than a typical clinic visit, but our Child Life colleagues are there to distract the patient through the entire procedure.”

“With magnetically controlled rods, patients can usually resume all of their normal activities between expansions,” Li continues, “with no restrictions on activities like sports or dance.”

Compared with TGRs, patients with MCGRs may also fare better emotionally.

“There is some evidence that the repeated surgeries associated with TGRs can take a psychological and emotional toll on children,” Li says. “While it’s too soon for comparable data on MCGRs, the hope is that by decreasing the number of surgeries, young patients with MCGRs will experience less psychological distress.”

CHOOSING THE RIGHT OPTION FOR EACH PATIENT Mott surgeons have implanted MCGRs in about 30 patients with early-onset scoliosis. While they are a promising advance in the field, Li cautions that MCGRs are not a panacea. “It’s an

On the left: pre-op x-ray image. On the right: x-ray image after insertion of growing rods and one lengthening procedure. exciting option to consider, but magnetic rods are not right for every patient,” she says.

The size and shape of the magnetic rods may not match every child’s anatomy and have led to post-operative complications in some patients. Leading centers like Mott are working together to refine the indications for their use. “As with any new technology, it takes time to understand which patients are the best candidates for the magnetic rods,” she says.

The bottom line, says Li, is that there is no one-size-fits-all solution for earlyonset scoliosis. For younger patients,

serial casting is usually the best first option. For older children with less severe curvatures, bracing may be appropriate, and eventually spinal fusion if needed. When surgery is indicated, there are several factors to consider when selecting which growing rod technology to use.

The Pediatric Orthopaedic Surgery Clinic at C.S. Mott Children’s Hospital offers a comprehensive, multidisciplinary evaluation of patients with early-onset scoliosis, discussing all available options and helping physicians and parents make the best choice for the child. n

OF THE 100 PARTICIPANTS SURVEYED IN MARCH 2019 FROM OUR OPERATING ROOM: SOURCE: PACU PATIENT SATISFACTION SURVEY FOR PREPARING CHILDREN FOR SURGERY, MARCH 18, 2019–APRIL 1, 2019

100%

AGREED THEY FELT THEIR CHILD’S PAIN WAS ADEQUATELY CONTROLLED

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100%

AGREED THEY WERE SATISFIED WITH OVERALL CARE

97.79%

AGREED INFORMATION PROVIDED WAS CONSISTENT AMONG EACH PERSON THEY CAME ACROSS DURING PROCEDURE

87.5%

DID NOT EXPERIENCE ANY DELAYS DURING PROCEDURE

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CH ILD L I F E S URG I C A L S E R VIC E S

Creating a more comfortable, less fearful surgical experience The hospital environment is unfamiliar, scary and stressful for young patients and their families. First established in 1922 and certified in 1998, C.S. Mott Children’s Hospital’s Child and Family Life Department is the oldest program of its kind in the U.S. More than 30 certified child life specialists are embedded throughout our inpatient and outpatient units, helping children and their parents better understand and cope with the hospital experience.

The surgical experience can be particularly challenging for children with developmental or sensory challenges; Mott child life specialists provide individualized care to support the needs of these children and their parents.

“Prior to surgery, our focus is providing age-appropriate preparation and addressing common misconceptions,” says child life specialist Jessica Riederer, C.C.L.S. Families can review educational materials, including a video and storybook, together at home leading up

to the date of surgery. Families are also welcomed to attend regularly scheduled small group tours of the operating room.

“I am one of a team of three preoperative child life specialists,” Riederer explains. “On the day of surgery, we can meet with the patient and family along with everyone on their surgical team.” Photographs of the operating room and both real and play medical equipment are used to prepare children and teens for the sequence of events and anesthesia basics. This can reduce anxiety, allow for expression and enhance coping. “Each intervention is tailored to the child and the procedure,” she continues, “and might include practicing breathing through an anesthesia mask or learning about IV placement.”

Child life specialists are also available as needed to provide distraction or coping support during IV placement or induction, as well as after surgery to help with wakeup, stress management and non-pharmacological pain management.

Check out some of our pre-surgical resources for parents at www.mott children.org/preparingforsurgery. n

Our “Preparing for Surgery” YouTube video has been viewed more than 1 million times!

For referrals, transfers or physician consults, please call M-LINE at 800-962-3555.

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INTRAO P E R AT I V E M R I

Real-time imaging for greater surgical precision Whether sampling tissue for testing, resecting an entire tumor or reconstructing anorectal anatomy, the challenge is the same: locate the target and perform the procedure while sparing surrounding healthy tissue. While traditional pre-operative imaging modalities like MRI, CT and ultrasound are effective in most instances, they aren’t always able to provide the right information at the right time.

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The answer is the real-time guidance of intraoperative MRI (iMRI). The ability to generate images during a procedure means improved decision-making, greater accuracy, reduced risk to healthy tissue, and fewer incomplete resections requiring follow-up procedures.

Mott is one of only a few pediatric centers in the U.S., and the only pediatric hospital in Michigan with a dedicated iMRI surgical suite and the multidisciplinary expertise to utilize it effectively across a wide range of surgical applications. Mounted on overhead rails in an adjacent room, our iMRI scanner is ready to be moved into position while the patient remains in a stationary operative position.

Mott neurosurgery and pediatric surgery teams are among the highest volume users of the state-of-the-art surgical suite, using its advanced intraoperative imaging technology to meet some of the most difficult challenges in surgery.

BRAIN TUMORS MRI provides the best available preoperative information about the location and characteristics of a brain tumor.

“But the soft pliable nature of the brain makes static images insufficient,” says pediatric neurosurgeon Hugh Garton, M.D, M.H.Sc. “As soon as we begin removing a tumor, its shape begins to change, as does the surrounding tissue as it moves into the space previously occupied by the tumor.”

Guided by iMRI’s real-time images, Mott surgeons can continuously map the extent and success of a resection, identifying residual tumor cells and differentiating them from normal brain tissue. “It’s an especially powerful tool for resecting low-grade pediatric gliomas,” Garton adds, “which closely resemble healthy brain tissue.”

REFRACTORY EPILEPSY For patients whose epilepsy is not well managed with medications, surgery may be the best option. One of the most effective minimally invasive approaches is laser interstitial thermal therapy (LiTT), which reaches and destroys even deep epileptogenic lesions with a tiny laser, minimizing damage to surrounding tissue.

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“Here at Mott, we’re combining LiTT with iMRI to locate, measure and ablate epileptogenic tissue in one procedure,” explains Garton, whose practice also encompasses the surgical management of epilepsy. “By rotating the probe to generate precise scans every seven to eight seconds, we can see the exact size and shape of the area being ablated.”

After several years of successful use in adults, iMRI-guided LiTT is now being used in pediatric patients with epileptic foci in the temporal lobe or corpus callosum.

ANORECTAL MALFORMATIONS One of the rarest and most complex colorectal surgical challenges is the correction of anorectal malformations in infants. The standard surgical approach — locating and correcting a rectal fistula by slicing the sphincter through an incision in the gluteal crease — leaves the sphincter muscle permanently weakened. “A laparoscopic approach can be a better alternative,” explains Marcus Jarboe, M.D., a pediatric surgeon and interventional radiologist whose practice encompasses a range of gastroenterological conditions, “but only when the sphincter complex is relatively straight, which is not usually the case.”

Along with the pediatric colorectal team at Mott, Jarboe is building on that less invasive approach, using iMRI to guide a needle through the sphincter complex regardless of its shape. “We are the only center in the world using real-time MRI to correct anorectal malformations,” he says.

ATYPICAL BIOPSIES While ultrasound and traditional MRI technologies effectively guide most standard minimally invasive biopsies, the nature or location of some lesions require a different approach.

“A tumor deep in the liver is a good example,” says Jarboe. “Repeated images are necessary to first position the needle and then advance it to a remote lesion.” Contrast CT is of limited value, since contrast dye can only be administered once due to its impact on the kidneys. “Using iMRI,

we can generate consecutive images to both view the lesion and guide each movement of the needle, for a far more precise outcome.” Mott is the only pediatric hospital in the world that has published data from MRI-guided GI biopsies in children.

Find out more about Mott’s intraoperative MRI capabilities on our website: http://mottchildren.org/imri n

For referrals, transfers or physician consults, please call M-LINE at 800-962-3555.

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REFRACTORY SLEEP DISORDERS

When CPAP isn’t the answer: Exploring surgical options for sleep apnea Obstructive sleep apnea (OSA) is estimated to affect one to four percent of children. While most children experience mild symptoms and some outgrow the condition, a significant and growing number of young patients suffer with the persistent symptoms of moderate to severe OSA.

M.P.H., is one of the clinicians who performs the procedure. “This is an endoscopic examination of the airway under a light anesthesia that simulates sleep. It allows us to identify and grade obstruction from the nose to the trachea and formulate an individual surgical plan,” she explains.

For a number of reasons, not all of these children are good candidates for the predominant non-surgical treatment, continuous positive airway pressure (CPAP). Some are too young to tolerate CPAP; others have difficulty tolerating the mask or are not candidates for CPAP because of underlying medical conditions, such as aspiration. Poor adherence is especially common in teenagers, who often object to wearing the mask.

If the resulting recommendation calls for addressing soft tissues including the palate, tongue base/lingual tonsils, or supraglottis, these procedures can often be performed at the same time as the sleep endoscopy.

The Pediatric Refractory Sleep Apnea Clinic at C.S. Mott Children’s Hospital, which is one of the few multidisciplinary sleep programs for children in the nation, was formed to provide patients — many of whom have already had adenotonsillectomies that failed to resolve OSA — with a one-stop resource to explore additional treatment options.

THE CLINIC VISIT Patients see specialists in sleep medicine and otolaryngology (in one combined session), and specialists in oral/ maxillofacial surgery and orthodontics (together in a second session), all scheduled on the same day.

During the evaluation, a flexible laryngoscopy may be performed (quickly passing a very small scope through the nose to observe the airway for sources of obstruction), x-rays of the face and jaw are taken, and sleep study results are reviewed.

At the end of the clinic day, the multidisciplinary team meets to discuss each patient’s test results and formulate treatment options, giving parents a list of alternatives to consider. A clinic coordinator discusses the options with the family by phone, and a letter is sent to the referring physician.

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When a regressed jaw is the cause of upper airway narrowing, orthodontic appliances followed by surgery to expand the facial skeleton (maxilla- and/ or mandibular advancement) may be recommended. For these patients, followup appointments with orthodontics and oral maxillofacial surgery are scheduled.

A nasopharyngeal airway device is one of the emerging options for breathingrelated sleep problems, addressing upper airway collapse.

FOLLOWING THE INITIAL VISIT “To be seen in the clinic, patients must have a diagnosis of OSA, which requires a sleep study,” says Fauziya Hassan, M.B.B.S., M.S., a pediatric pulmonologist and a sleep medicine physician. “A repeat sleep study may be requested to confirm the diagnosis, preferably in a dedicated pediatric facility like ours.” Pediatric sleep studies may be done at the Med Inn location, the Brighton Center for Specialty Care, or, for older healthy children, at Domino’s Farms.

In many instances, the team will also recommend scheduling druginduced sleep endoscopy. Pediatric otolaryngologist Erin Kirkham, M.D.,

EMERGING OPTIONS Mott is home to one of the nation’s preeminent pediatric sleep research programs, opening the door to additional, experimental options for addressing breathing-related sleep problems.

In collaboration with U-M biomedical engineers, Kirkham and her otolaryngology colleague David Zopf, M.D., are developing and testing several new approaches. “We’re working on a nasopharyngeal airway device to address upper airway collapse, and new methods for quantifying airflow restriction to more precisely map upper airway collapse,” says Kirkham. “All of these tools have the potential to further refine our surgical approach.”

Find out more about Mott’s Pediatric Refractory Sleep Apnea Clinic on our website: https://www.mottchildren.org/ refractory-sleep-apnea-pediatric n

Pediatric Adenotonsillectomy for Snoring (PATS) Trial Mott is a site for this NIH-funded multi-center trial comparing early adenotonsillectomy with watch-and-wait for children who, despite testing that is negative or borderline for OSA, are experiencing mild sleep-disordered breathing. The trial has recently been expanded to include children with Down Syndrome who have sleep disordered breathing.

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S PINA L ANE S T H E SIA

An alternative to general anesthesia for very young children Spinal anesthesia, a needle-based procedure that provides sensory and movement block without loss of consciousness, is becoming a more common alternative to general anesthesia for infants.

That’s because general anesthesia is associated with some risks, such as need for placement of a breathing tube and potential changes in oxygen levels, blood pressure and heart rate.

Likewise, the Food and Drug Administration warned in 2017 that repeat or extended exposure to general anesthesia could harm developing brains. A consensus statement (SmartTots.org) recommended delaying surgery until a child is three years or older, unless it is urgent or needed to treat a significant condition.

Not only does a spinal-focused approach eliminate those concerns, it may also help shorten a surgery’s duration and patient recovery time. And the approach is simple.

“Essentially, we inject a little bit of numbing medicine via a tiny needle into the fluid that surrounds the spinal cord, which in turn numbs the baby from the chest down to the toes,” says Ashlee Holman, M.D., a pediatric anesthesiologist.

Typically reserved for adult patients, spinal anesthesia for children is offered at only a handful of specialized pediatric facilities across the U.S. — including Mott, where the option has been available since 2018.

Holman shared more facts: IT’S ONLY USED FOR CERTAIN PROCEDURES Spinal anesthesia lasts one to two hours and provides coverage from the chest down, so it’s only suitable for shorter procedures involving those regions, including certain urologic surgeries such as circumcisions, repairs of hypospadias and orchidopexies, as well as hernia

repairs and lower extremity orthopaedic procedures.

“Any procedure below the belly button that takes less than two hours is a candidate,” Holman says.

RECIPIENTS REMAIN AWAKE THROUGHOUT Patients become much calmer after the numbness kicks in — a “meditative-like state” known as sensory deafferentation.

“The babies usually fall asleep naturally right after the injection,” Holman says.

Those who don’t, however, are carefully monitored throughout. Doctors, nurses or child life specialists can help distract patients by singing lullabies, giving a pacifier dipped in sugar water, or offering an iPad for visual distraction.

RECOVERY AND DISCHARGE TIMES ARE QUICKER Once surgery is complete, babies are brought to the recovery room and are returned almost immediately to their parent.

They also can feed right away. General anesthesia, by comparison, may require waiting up to 30 to 60 minutes after the baby awakens.

“We’re finding that the total recovery time is shorter for these patients,” Holman says. “We’ve also seen quicker discharges from the recovery room as well as increased parent satisfaction.”

GENERAL ANESTHESIA RISKS ARE ABSENT Spinal anesthesia avoids the theoretical neurological risks associated with repeat or extended general anesthesia given to kids age three and younger, while still allowing the infant to have the needed surgery.

Another positive: Recipients tend to have less pain, so opioids are generally not needed in the operating and recovery rooms. n

For referrals, transfers or physician consults, please call M-LINE at 800-962-3555.

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First hospital in Michigan to receive Level 1 Children’s Surgery Center verification from the American College of Surgeons The American College of Surgeons’ Children’s Surgery Verification Quality Improvement Program was developed to improve the quality of children’s surgical care by ensuring that centers have the necessary resources to provide optimal surgical care.

The program is modeled after other nationally recognized ACS quality improvement programs that have measurably improved surgical quality and have prevented complications, reduced costs, and saved lives.

To become a verified center, Mott was required to meet criteria for staffing, training, and facility infrastructure and protocols for care. Mott also participates in a national data registry that produces semiannual reports on the quality of its processes and outcomes to help identify opportunities for improvement.

Centers seeking verification undergo an extensive site visit by an ACS team of surveyors that includes experienced children’s surgeons, anesthesiologists, and nurses. Surveyors review the center’s structure, process, and clinical outcomes data to ensure that children receive surgical care under a multidisciplinary program with quality improvement and safety processes, data collection, and appropriate resources.

University of Michigan C.S. Mott Children’s Hospital was the first hospital in Michigan to receive this designation.