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POEM procedure is safe and effective for children with esophageal achalasia

March 9, 2022

Drs. Petrosyan and Kane combined perform more POEM procedures for children than any other pediatric surgeons in the United States.

Peroral endoscopic myotomy (POEM) is a safe and effective procedure to treat pediatric achalasia according to a single-center outcomes study in the Journal of Pediatric Surgery.

Authors Timothy Kane, M.D., chief of General and Thoracic Surgery at Children’s National Hospital, and Mikael Petrosyan, M.D., MBA, associate chief of that division, together perform more of these procedures than any other pediatric surgeons in the United States.

Their experience with POEM shows that it is as safe and effective as the current standard of care for pediatric achalasia, which is a procedure called the laparoscopic Heller myotomy (LHM). Even better, previous research in adults and now in pediatric patients, has shown that those who undergo POEM as an alternative to LHM report less pain and often require shorter hospital stays after surgery.

Why it matters

POEM has been an option for adults with achalasia for many years, but not for children because it requires technical skill and expertise not readily available everywhere. More studies of young patients with successful outcomes following POEM procedures can help make the case for training more pediatric surgeons to learn this approach, and help this alternative method become an additional surgical option for children with achalasia.

The hold-up in the field

Achalasia is a rare condition in adults (1/100,000) and even less common in children, occurring in only 0.1 per 100,000 patients with an estimated prevalence of 10 per 100,000. The rarity of achalasia in children compared with adults makes collecting enough statistically significant evidence about how best to treat them difficult, more so than for other more common pediatric surgical conditions.

Children’s National Hospital leads the way

Children’s National Hospital is one of the only children’s hospitals in the country to offer the option of POEM for treatment of these conditions in children — and Drs. Kane and Petrosyan combined perform more of these procedures than any other pediatric surgeons in the United States.

The surgeons at Children’s National offer POEM as a primary intervention for children with esophageal achalasia and are also applying the same approach for pediatric gastroparesis as well.

Children’s National Hospital is one of only 12 children’s hospitals in the country, and the only hospital in the Washington, D.C., region, to be verified as a Level 1 Children’s Surgery Center by the American College of Surgeons (ACS) Children’s Surgery Verification Quality Improvement Program. This distinction recognizes surgery centers whose quality improvement programs have measurably improved pediatric surgical quality, prevented complications, reduced costs and saved lives.

Bottom line

Given their reported outcomes so far, the authors believe that in the long term this approach may replace the current pediatric standard of care, the LHM. More research is needed to make this case, however, including long-term follow-up studies of the patients who have undergone the procedure so far.

You can read the full study, “Per Oral Endoscopic Myotomy (POEM) for Pediatric Achalasia: Institutional Experience and Outcomes,” in the Journal of Pediatric Surgery.

How POEM may change the standard of care for pediatric achalasia

April 9, 2021

Today, Drs. Petrosyan and Kane have performed over 35 POEM procedures for children to resolve esophageal achalasia symptoms.

In 2016, pediatric surgeons Mikael Petrosyan, M.D., and Timothy Kane, M.D., published an article in the Journal of Pediatric Surgery reviewing their experience with the surgical treatment of achalasia and how peroral endoscopic myotomy (POEM) was a new technique being used at Children’s National Hospital to treat esophageal achalasia in children.

The procedure, first used in adults to treat esophageal achalasia, uses a different approach than the current minimally invasive standard of care for children, the Heller myotomy. At the time of the initial study, the team had performed only a few POEM procedures for children but was already starting to see the promise this procedure could offer if done correctly.

While esophageal achalasia affects only about one in every 1 million kids (versus one in 100,000 for adults), the condition, which occurs when the esophagus muscles fail to function properly and the lower sphincter of the esophagus doesn’t relax enough to allow food into the stomach, can have serious impacts on daily life. The tight sphincter may cause food backup, heartburn, chest pain, and many other painful symptoms. Unfortunately, medical interventions including balloon endoscopy or Botox injections, are only temporary fixes that don’t last longer than a month or so before further treatment is needed. For kids who have long lives ahead of them, a surgical solution is the best hope for permanent symptom relief.

At the time of the 2016 study, Dr. Kane said that, “Heller myotomy works very well for most kids — that’s why it’s the standard of care. Our study found that patients who underwent the POEM procedure experienced the same successful outcomes as Heller patients, and we already knew from adult data that POEM patients reported less pain following surgery — a win-win for children.”

Today, Drs. Petrosyan, the associate chief, and Kane, the chief, of General and Thoracic Surgery at Children’s National, have performed over 35 POEM procedures for children to resolve esophageal achalasia symptoms. Increasingly, they find themselves recommending the POEM for many reasons, including, of course, the faster recovery. Kids who have a POEM procedure also often go home in one to two days following surgery and report less pain — typically a sore throat from the endoscopy and the anesthesia, but very little pain at the surgical site.

The surgeons say that the while it takes a steep learning curve to perfect the technique of using POEM in children due to the size of the available instruments to perform the procedure coupled with the challenge of a child’s tiny esophagus, the benefits for patients are well worth it.

“There’s only a single incision in the esophagus, and no incision in the abdomen,” says Dr. Petrosyan. “Kids tolerate the surgery really well, report very little pain, and recover very quickly with minimal complications.”

Even better, he continues, unlike the Heller myotomy, POEM can be performed at any point in treatment, even if other therapies or surgical interventions (including a Heller myotomy) have been previously performed. It can also be repeated if needed — though so far, they haven’t needed to do any further revisions in the population at Children’s National.

Drs. Petrosyan and Kane have performed successful POEM procedures for children with esophageal achalasia between the ages of four and 12. Younger children, (under a year of age) with smaller anatomy, continue to be treated using the Heller procedure due to the limitations in size of the surgical instruments.

Children’s National Hospital is one of the only children’s hospitals in the country to offer the option of POEM for treatment of these conditions — and Drs. Kane and Petrosyan combined perform more of these procedures than any other pediatric surgeon in the United States.

A forthcoming peer-reviewed study will highlight the use of POEM for this population and weigh its success against the current standards of care for treating children with esophageal achalasia.

In the meantime, the surgeons at Children’s National continue to offer POEM as a primary intervention for children with esophageal achalasia and are also applying the same approach for pediatric gastroparesis as well.

Children’s National first-use of anterior vertebral body tethering system for idiopathic scoliosis

February 5, 2021

Boy with scoliosis during rehabilitation

On Monday, Feb. 1, 2021, Children’s National Hospital performed the first anterior vertebral body tethering procedure for a child in the Washington, D.C., area. The device is a recently approved option for treating children with idiopathic scoliosis, the most common type of scoliosis. It allows for gradual correction of a spinal deformity through the natural growth of the spine, leading to improvements in spinal alignment while maintaining spinal flexibility.

In August 2019, the U.S. Food and Drug Administration (FDA) approved the first spinal tether system for pediatric patients called The Tether – Vertebral Body Tethering System. This device is attached to the spine during a minimally invasive thorascopic procedure performed by a multidisciplinary medical team, which includes orthopaedic surgery, thoracic surgery and anesthesia.

“This promising technology may help maintain the flexibility of the spine as it grows straighter over time,” says Matthew Oetgen, M.D., chief of Orthopaedics at Children’s National. “Novel devices like The Tether offer additional treatment options for idiopathic scoliosis, which have the potential to improve pediatric surgical outcomes and quality of life for children and adolescents with significant spinal deformities.”

The procedure at Children’s National brought together some the region’s best pediatric orthopaedic and thoracic surgeons, including Dr. Oetgen, Shannon Kelly, M.D., associate chief of Orthopaedic Surgery, and Timothy Kane, M.D., chief of General and Thoracic Surgery.

“Children’s National can offer these types of procedures because the hospital is home to many talented pediatric surgeons across specialties, many of whom are experts in minimally invasive techniques for children,” says Dr. Kane. “We collaborate together, often, because we know that’s the best way to continually improve the care we provide to children and their families.”

Idiopathic scoliosis is the most common type of scoliosis and can occur in children between age 10 and 18 or until they are fully grown. Spinal fusion surgery is the most common treatment for children with the most severe spine curvatures and continues to be the “gold standard” for surgical treatment of this condition. It takes about 3 months for a child or adolescent to fully recover from the procedure. While spinal fusion produces excellent and reproducible outcomes, in certain patients with less severe deformities and significant skeletal growth remaining, vertebral body tethering may offer some benefits over spinal fusion. In clinical trials, the spinal tether was shown to shorten recovery time and increase range of motion. Early diagnosis is vital to dictate which treatment will be most beneficial.

Case study: Diagnosing a choledochal cyst in utero

March 18, 2019

The Feigel family worked with Timothy Kane, M.D., the division chief of general and thoracic surgery at Children’s National, to ensure an accurate diagnosis, coordinate a corrective procedure and support a strong recovery for Kinsley, who just celebrated a 5-month milestone.

On Sept. 30, 2018, Elizabeth Feigel gave birth to a healthy baby girl, Kinsley Feigel. Thirty-two days later, Elizabeth and her husband, Steven Feigel, delighted in another hospital moment: Kinsley, who developed a choledochal cyst in utero, was recovering from a surgical procedure to remove an abnormal bile duct cyst, which also required the removal of her gallbladder.

While the series of events, interspersed with multiple hospital visits, would likely create uneasiness in new parents, the Feigel family worked with Vahe Badalyan, M.D., a gastroenterologist at Children’s National Health System, and with Timothy Kane, M.D., the division chief of general and thoracic surgery at Children’s National, to ensure an accurate diagnosis, coordinate a corrective procedure and support a strong recovery for Kinsley, who just celebrated a 5-month milestone.

One of the keys to Kinsley’s success was close communication between her parents and providers.

Dr. Badalyan and Dr. Kane listened to Elizabeth and Steven’s concerns, explained complex medical terms in lay language, and provided background about Kinsley’s presenting symptoms, risk factors and procedures. Instead of second-guessing the diagnosis, Elizabeth and Steven put their trust into and remained in contact with the medical team, sharing updates about Kinsley at home. This parent-physician partnership helped ensure an accurate diagnosis and tailored treatment for Kinsley.

Here is her story.

An early diagnosis

During a 12-week prenatal ultrasound, Elizabeth discovered that Kinsley had an intra-abdominal cyst. Before Elizabeth came to Children’s National for an MRI, she met with several fetal medicine specialists and had a variety of tests, including an amniocentesis to rule out chromosomal abnormalities, such as Down syndrome.

The team at Children’s National didn’t want to prematurely confirm Kinsley’s choledochal cyst in utero, but additional ultrasounds and an MRI helped narrow the diagnosis to a few conditions.

After Kinsley was born, and despite looking like a healthy, full-term baby, she was transported to the neonatal intensive care unit (NICU) at Children’s National. Dr. Badalyan and Dr. Kane analyzed Kinsley’s postnatal sonogram and found the cyst was bigger than they previously thought. Over a five-day period, the medical team kept Kinsley under their close watch, running additional tests, including an additional sonogram. They then followed up with Kinsley on an outpatient basis to better understand and diagnose her cyst.

Outpatient care

Over the next few weeks, Kinsley, Elizabeth and Steven returned to Children’s National to coordinate multiple exams, ranging from an MRI to a HIDA scan. During this period, Elizabeth and Steven remained in contact with Dr. Badalyan. They heard about Kinsley’s lab results and sent updates about her symptoms, including her stool, which helped the medical team monitor her status.

Meanwhile, Dr. Badalyan and Dr. Kane worked closely with the lab to measure Kinsley’s bilirubin levels. Her presenting symptoms and risk factors, she had jaundice and is a female baby of Asian descent, are associated with both choledochal cysts and biliary atresia.

Over time and with the help of Elizabeth, Steven and the pediatric radiologists, Dr. Badalyan and Dr. Kane confirmed Kinsley had a type 1 choledochal cyst, the most common. Originally, the plan was to operate at three to six months, but Dr. Kane needed to expedite the procedure and operate on Kinsley at one month due to a rise in her bilirubin, a sign of progressive liver disease.

Higher bilirubin levels are common in newborns and remain elevated at about 5 mg/dL after the first few days of birth, but Kinsley’s levels peaked and remained elevated. Instead of her bile flowing into her intestine, her choledochal cyst reduced the flow of bile, which accumulated and started to pour back into her liver. The timing of the surgery was as important as the procedure.

The surgery

On Oct. 31, Halloween, Kinsley had laparoscopic surgery to remove the choledochal cyst. Approximately five to seven patients per year undergo choledochal cyst removal at Children’s National. Smaller infants typically undergo removal of a choledochal cyst using a large incision (or open procedure). Kinsley was the smallest baby at Children’s National to have this type of surgery performed by minimally invasive laparoscopic surgery, which required a few 3-mm incisions – the size of coriander seeds.

Some hospitals use the da Vinci robot, which starts at 8-mm incisions, the size of a small pearl, to conduct this procedure on infants, but this method cannot effectively be done in very small infants. Instead, Dr. Kane prefers to stitch sutures by hand. This technique keeps the incisions small and is technically demanding, but Dr. Kane doesn’t mind (he views this as an advanced technical skill). The goal for this surgery was to cut out the abnormal piece of Kinsley’s common bile duct, comprised of the cyst, remove this and then sew the bile duct to the small intestine (duodenum), creating a digestive pathway. The new digestive tube allows for bile to flow from her liver through the common hepatic duct, in place of the pathway where the cyst formed, and into her intestine.

Like other surgeries, Dr. Kane needed to adapt the procedure, especially with Kinsley’s size: Taking too much from the bile duct would create a tight space, and could create obstruction, blocking bile, while leaving too much room could create leakage and spilling of the bile, requiring a follow-up surgical procedure within a week or two of the original operation.

Dr. Kane had a few options in mind before he operated. He didn’t know which would be most suitable until the operation, but he remained open and prepared for all three. Adopting this mindset, instead of having one procedure in mind, has helped Dr. Kane with precise and tailored surgeries, which often result in the best procedure and a stronger recovery period for young patients.

After 4.5 hours, the surgery, a two-part procedure – removing the cyst and recreating a functional bile duct – was complete.

Kinsley moved into the recovery unit, where she rested and recovered under close medical supervision for five days. During the first few days, she didn’t have liquids or milk, but she did have two bedside nurses monitoring her status in addition to surgeons making regular rounds. Elizabeth and Steven were relieved: The diagnosis and surgery were over.

Managing risk factors

Before Kinsley left the hospital, Elizabeth and Steven scheduled a follow-up visit to ensure Kinsley was recovering well and avoided risk of infection, such as cholangitis, which can occur suddenly and become chronic.

Following Kinsley’s post-surgical bloodwork in early November, Dr. Badalyan noticed Kinsley’s white blood count was high, signaling infection, and he immediately brought the family back to the hospital. To help her body fight the infection, Kinsley received antibiotics and intravenous fluids. She stayed in the hospital for five days. Fortunately, cholangitis is easy to treat with antibiotics; the key is to detect it early.

Kinsley returned home in time for Thanksgiving. She came back to the hospital for biweekly visits. At this point, she was filling out, reaching a 2-month milestone and nearing a full recovery. She returned for follow-up visits in December and January – and has been healthy ever since. She will continue to make routine visits during her first year to ensure her white blood count remains in a healthy range.

Investing in youth resilience

Dr. Badalyan and Dr. Kane envision a healthy future for Kinsley. They don’t expect she’ll need additional operations. Her parents are also looking on the bright side: Since gallbladders aren’t essential for survival or long-term health outcomes, and since many people can easily live without them, Kinsley may be at an advantage. Elizabeth thinks Kinsley may be more cautious about lifestyle choices to support living without a gallbladder, which also support longevity.

Another perspective noted by Dr. Badalyan and Dr. Kane is Kinsley’s resilience factor. Having the surgery earlier brought unique challenges, but her age makes it easier for Kinsley to bounce back as her body rapidly develops. Her tissues were healthy, compared to adult patients undergoing surgery with chronic liver problems or heart disease, which puts her at an advantage for a faster healing process. Dr. Badalyan also mentions that while it’s good for her Kinsley and her family to continue to monitor risks for infections, she won’t have gallstones.

Elizabeth also started to notice something that Kinsley’s doctors likely wouldn’t pick up on: Her personality seems to be a result of her hospital experience and stay. Kinsley’s an easy baby. She eats well and sleeps well, which Elizabeth credits to being around clinicians and to learning the art of self-soothing, a skill she likely acquired while recovering from surgery.

This month, Kinsley has another adventure. She’ll travel with her parents to visit extended family in Seattle, Napa Valley, Calif. and West Virginia. She has several relatives and family friends, all of whom are looking forward to meeting her.

Minimally invasive surgery brings lasting relief to pediatric achalasia patients

October 27, 2016

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Achalasia affects only a small number of people around the world, estimated at 1.6 per 100,000, and children make up fewer than 5 percent of that total. In most cases, the causes are unknown, but it is attributed to a combination of heredity and autoimmune or nerve cell disorders. For adults, treatment might include oral medication to prevent narrowing, balloon dilation, or botulinum toxin injections to relax the muscle at the end of the esophagus. For a growing child, who faces not just months but a lifetime of injections and potential repeat procedures, these methods aren’t viable. Instead, surgical correction is the standard of care. In the past 10 years, the surgical option evolved from a traditional open procedure with weeks of recovery and pain to less-invasive approaches.

“The total number of children with achalasia is small,” says Timothy D. Kane, M.D., Division Chief of General and Thoracic Surgery at Children’s National Health System. “But Children’s National treats more of these cases than most other children’s hospitals around the world, and that gives us the ability to look at a larger population and see what works.”

Dr. Kane is senior author of a study recently published in the Journal of Pediatric Surgery that analyzed the outcomes from nearly a decade’s worth of these cases to gauge the effectiveness of two different minimally invasive surgical approaches for children with achalasia.

A look at the two surgical options

The most common surgical intervention is laparoscopic Heller myotomy, performed through small incisions in the belly. Additionally, Dr. Kane and the Children’s surgical team are one of only two teams in the country who perform a different procedure called peroral endoscopic myotomy (POEM) on children. The POEM procedure is completed entirely through the mouth using an endoscope, with no additional incision needed. The procedure is commonly used for adult achalasia cases, but is not widely available for children elsewhere as it requires specialized training and practice to perform.

“Heller myotomy works very well for most kids — that’s why it’s the standard of care,” Dr. Kane says. “Our study found that patients who underwent the POEM procedure experienced the same successful outcomes as Heller patients, and we already knew from adult data that POEM patients reported less pain following surgery — a win-win for children.”

The retrospective study included all children who had undergone surgical treatment for achalasia at Children’s from 2006 to 2015. Since achalasia cases are few and far between, with most children’s hospitals seeing maybe one to five cases over 10 years, collecting reliable data on outcomes is challenging. This study provides a large enough sample to allow doctors to use the findings as a guide to find the interventions that are the best fit for each patient.

“Now we’re very comfortable presenting families with two really good options and letting them choose the one that works best for them,” he concludes.

Imagine the feeling of food stuck in your throat. For children with esophageal achalasia, that feeling is a constant truth: The muscles in the esophagus fail to function properly and the lower valve, or sphincter, of the esophagus controlling the flow of food into the stomach doesn’t relax enough to allow in food — causing a backup, heartburn, chest pain, and many other painful symptoms. For children, surgery is the best hope for permanent relief.