Published on in CHOP News
Pediatric anesthesiologists at Children’s Hospital of Philadelphia (CHOP) describe special considerations in providing anesthesia to patients undergoing an innovative treatment for lymphatic flow disorders. In these rare disorders, abnormal circulation of lymphatic fluid can cause debilitating, even fatal conditions, particularly in patients with congenital heart disease (CHD).
“These lymphatic procedures require general anesthesia, because the patient must be immobilized while clinicians place needles and catheters, treat pain, and frequently perform additional procedures,” said study leader David R. Jobes, MD, a pediatric anesthesiologist in the Cardiac Center at CHOP. “This report describes our experience in a developing clinical program for children with severe, complex disorders that were not previously responsive to treatment.”
In the journal Pediatric Anesthesia, the study team reviewed a one-year cohort of all 68 patients seen in the Jill and Mark Fishman Center for Lymphatic Disorders at CHOP in 2016 and 2017. The patients ranged from 4 days to 17 years old, with a median age of 4.4. Years. The majority of the patients had CHD; lymphatic flow disorders can result as a rare complication of altered circulation from the Fontan surgery for single-ventricle heart disease.
The Jill and Mark Fishman Center for Lymphatic Disorders is a joint program between CHOP and the Hospital of the University of Pennsylvania. The Center’s team pioneered a new imaging technique, dynamic contrast-enhanced magnetic resonance lymphangiography, which identifies the site of lymphatic leaks often not detectable by conventional imaging. Center staff members have also pioneered treatments to embolize, or block, abnormal lymphatic flow, using tools such as coils, iodized oils and covered stents, all delivered through catheters.
The authors note that lymphatic abnormalities present special challenges in maintaining circulatory and respiratory function during general anesthesia. One lymphatic problem called pleural effusion restricts normal lung expansion. Another condition, plastic bronchitis, involves putty-like casts that clog the branches of a patient’s airways, and must be removed through a bronchoscope — a tube inserted into the respiratory system.
All the children in the study who required intervention were admitted to an intensive care unit. The authors note that the severity and complexity of lymphatic disorders, technical requirements, specialized personnel needed, and the necessity of transporting anesthetized patients to other units, such as radiology and MRI, “will likely limit adoption of these techniques to tertiary children’s hospitals.”
David R. Jobes et al, “Lymphatic imaging and intervention in a pediatric population: Anesthetic considerations,” Pediatric Anesthesia, published online April 23, 2018.
Contact: The Children’s Hospital of Philadelphia,