The chest wall deformity program treats children with pectus excavatum (sunken chest), pectus carinatum (protruding chest) and several rare chest wall conditions, including slipping rib syndrome, congenital rib anomalies, Jeune syndrome and Poland syndrome. The most common chest wall deformity, pectus excavatum, can hinder cardiovascular function and stamina. It is also associated with psychosocial symptoms, such as social isolation.
The standard of care for pectus excavatum is minimally invasive surgical repair, which patients typically undergo as teenagers. The procedure involves placing a curved metal bar behind the sternum through incisions in the armpits. Over three years, the bar pushes the chest outward. Historically, perioperative care of these patients varied widely, but today, patients at Johns Hopkins All Children’s follow a standardized enhanced recovery pathway that offers a variety of benefits for safety, quality and comfort.
In 2013, the chest wall deformity team set out to address disparities in pain management, intensive care unit (ICU) admissions, chest X-rays, laboratory studies and length of stay among patients who underwent surgery for pectus excavatum. Four years later, the team published a description of the enhanced recovery pathway and its efficacy compared with the previous, nonstandardized approach to care in Pediatric Surgery International.
“Over time, we found no patients who needed a blood transfusion, so we stopped performing pre-transfusion testing for every patient because the chance of needing a transfusion was so low,” says Nicole Chandler, M.D., director of pediatric surgery research at Johns Hopkins All Children’s. “When we looked at patients who were admitted to the general surgical floor versus the ICU, we found no patients who needed to go to the ICU for something unplanned, so we eliminated routine ICU admissions. We also found zero cases of chest X-rays and blood work that prompted a change in plan of care, so we eliminated those daily tests.”
A key component of the enhanced recovery pathway is a standardized approach to managing pain.
“Many patients used to receive epidural catheters for postoperative pain control, which are effective, but they can take 40 minutes to place and are associated with longer length of stay in the hospital, so we abandoned them,” says Paul Danielson, M.D., chief of the division of pediatric surgery at Johns Hopkins All Children’s. “Patients used to receive a bladder catheter to help them urinate, but we’ve eliminated that. We now have a special combination of medications that the anesthesiologist administers preoperatively, intraoperatively and postoperatively to address patients’ pain and anxiety.”
That poly-pharmaceutical approach has allowed physicians to reduce the amount of morphine patients receive while maintaining a similar level of pain control. Patients now experience fewer narcotic-related side effects, such as urinary retention and constipation, and they are more aware and interactive following surgery.
“Previously, we kept patients in bed for a day following surgery, but now they sit up in a chair the same afternoon and walk the following day,” Danielson says. “Patients used to stay in the hospital for several days. Currently, on average, they go home on day two.”
Chandler and Danielson and the rest of the chest wall deformity team continue to search for ways to improve the process of care for patients with pectus excavatum. One way they are doing so is by testing a new form of pain relief that involves freezing the nerves on the chest wall. The resulting numbness may further reduce the need for postoperative pain medication, Danielson says.
“We’re hoping to develop a system that would allow patients to have all of their preoperative studies during one visit,” Chandler says. “Education is key for these patients and families, so we’re developing educational information to help them learn about pectus excavatum.”
An Alternative to Preoperative Imaging
Many insurance carriers require a CT or MRI scan to determine the severity of chest deformity before patients can undergo surgical repair for pectus excavatum. Those studies expose patients to radiation and provide scant clinical information, Chandler says. The chest wall deformity team is studying the comparative efficacy of an alternative approach.
“For years, we’ve used calipers to determine the depth of the sternum compared with the rest of the chest,” Chandler says. “That gives us a straightforward, low-tech, reproducible method we think is just as reflective of the severity of chest deformity as CT or MRI.”