What is congenital disphragmatic hernia?
Congenital diaphragmatic hernia (CDH) is a birth defect in which the diaphragm does not develop completely, allowing abdominal organs to migrate into the chest and potentially restrict lung development. CDH occurs in 2.7 to 3.5 in 10,000 births and has a survival rate of about 70% with newer treatment modalities. Children’s Healthcare of Atlanta is well equipped to treat these complex cases, with an in-house ECMO Center and surgeons at the forefront of treatment.
How does Children's approach treatment for patients with CDH?
Two of our pediatric surgeons, Matthew Clifton, MD, and Megan Durham, MD, recently published findings from a study on long-term quality of life (QoL) in CDH patients in the Journal of Pediatric Surgery, along with other co-authors.
In the introduction to the article,* “Parents reported long-term quality of life outcomes in children after congenital diaphragmatic hernia repair,” Drs. Clifton and Durham state their purpose clearly: “The goal of this study is to investigate long-term outcomes, including QoL, for patients with CDH. ... We aim to provide families with information as they consider their child’s life beyond immediate CDH treatment and repair.”
While common long-term morbidities of CDH are well known, few studies have assessed their impact on long-term quality of life as reported by the patients’ parents. Drs. Clifton and Durham hypothesized that babies treated with more invasive therapies like ECMO and open surgery would have lower long-term QoL scores.
Overall, the results of the study paint a positive picture for survivors of CDH: “Despite a high prevalence of chronic conditions and subsequent operations in CDH patients, long-term QoL outcomes seem reassuring.” Their hypothesis that treatment with ECMO and open surgery might lead to lower QoL scores was partially correct, in that an open surgical approach was indeed associated with a lower quality of life in their results.
The data from this and another recently published study** indicate what Dr. Clifton knows from experience that there are real advantages to doing CDH repairs thoracoscopically, despite a slightly higher recurrence rate of the CDH.
“It’s playing the long game,” Dr. Clifton says, “which is to say, if this is a child that’s going to live another 85 or 90 years, what are the implications [of an open or a minimally invasive approach] for their health over the course of their lifetime? And they’re substantial. We know that the risk of bowel obstruction from scar tissue is tremendously higher in the open operation, so in my mind, I’m willing to accept a slight increase in recurrence rate if it means I’m giving more of my patients a better long-term quality of life without bowel obstructions.”
At Children’s, CDH repairs are performed thoracoscopically, or in a minimally invasive way, whenever possible.
“We’re probably one of the only, if not the only group in the country, where every single one of us offers this operation thoracoscopically,” Dr. Clifton concludes. “What I think separates our practice from many others across the country is the fact that we all have this skill set, and regardless of which surgeon you end up with, you’re likely to have a minimally invasive operation if it’s the right thing for your child.”