Johns Hopkins Surgery
July 23, 2015

Hernia patients with connective tissue disorders, like Marfan syndrome or Loeys-Dietz syndrome, are in a delicate situation, because their tissues tend to be weak, and their bodies typically heal slowly. “This group doesn’t do as well with the same procedures we offer everyone for hernias,” says acute care surgeon David Efron. “Even though they make scar tissue that looks good, it doesn’t hold very well.”

Efron gained significant knowledge about connective tissue disorders when Johns Hopkins researcher Harry Dietz started referring his patients to Efron for surgery. Dietz is recognized as the world’s authority on Marfan syndrome; he and his colleagues described the related Loeys-Dietz syndrome for the first time in 2005.

Before this distinction, patients with Loeys-Dietz were often misdiagnosed with Marfan. While both disorders primarily affect the cardiovascular and skeletal systems, patients with Loeys-Dietz do not have ocular lens dislocation issues like patients with Marfan syndrome.

The most serious symptom associated with both disorders is the weakening and widening of the aorta, which can lead to an enlarged aortic root and requires surgery to replace. Both syndromes also typically cause heart murmurs and leaky mitral valves that need to be replaced.

After cardiac or other surgery in this population, hernias often occur because the surgical incisions do not heal as well. In fact, nearly all patients with Loeys-Dietz have recurrent hernias as well as easy bleeding, bruising and scarring problems.

At the Johns Hopkins Institute of Genetic Medicine’s Connective Tissue Disorder Clinic, these patients receive care and therapy from Dietz and others. When someone has a hernia in the abdomen, groin or diaphragm, Efron is the go-to surgeon.

Over the years, he has refined his approach for optimal repair in patients with connective tissue disorders. For example, whenever possible, he has patients stay on their medications for stabilizing connective tissue. In particular, patients with Loeys-Dietz remain on losartan throughout surgery or resume taking the drug as soon as possible after the procedure.

Because he finds patients with Loeys-Dietz often have an exaggerated immune response, Efron also ensures they have steroids or other medications to control inflammation before the operation. He prefers to make the repair in a setting where an emergency response can take place if needed, rather than in an outpatient setting.

Typically, Efron says, a standard single mesh approach does not suffice in these patients, because the tissue doesn’t hold. Instead, he uses a biological material from below the hernia and a plastic mesh on top to create a buttress from above and below. The materials are then secured through the abdominal wall.

“I’m cognizant of a potential weak spot in these patients more than in others,” he says. “It impacts my surgical decision-making and technique.”

 
To refer a patient: 443-997-1508