When Elijah Smith of Wade, N.C., was born in December 2010 at Cape Fear Valley Hospital in Fayetteville, excess fluid had overrun his lymphatic vessels, engorging and distorting his neck and upper chest cavity. Diagnosed at birth, Elijah suffered from lymphatic malformations (LMs), and he represented the most common type of patient with this condition — a congenital case requiring an immediate, multidisciplinary course of treatment.
Thought to be caused by errors in fetal development of lymphatic channels, the LMs afflicting Elijah had begun in the womb and gone undetected in multiple ultrasounds. Marked by mass swelling distorting Elijah’s upper thoracic cavity, the malformed vessels could not efficiently transport tissue fluids back into the bloodstream via his lymph nodes. If left untreated, the accumulated lymphatic fluid would have increasingly obstructed his airways. Non-intervention would also have risked lifelong maxillofacial complications such as thickening of the tongue (macroglossia) and speech/eating challenges, as well as recurrent cellulitis, enlarged soft tissues and bones, and bleeds from the LMs themselves.
Elijah was referred to UNC Health Care shortly after birth in early 2011 for corrective treatment. Over a two-year span, his team included clinical faculty from the Departments of Pediatrics, Pediatric Surgery, Otolaryngology/Head and Neck Surgery and Radiology. UNC Professor of Pediatrics Dr. Timothy Weiner’s confirmation of Elijah’s diagnosis at birth was so brief that his case referral to UNC’s Department of Otolaryngology entailed escorting the Smith family directly over to Associate Professor and Pediatric Otolaryngology Chief Dr. Carlton Zdanski’s clinic.
As Zdanski examined the fluid-filled cysts throughout the boy’s throat, he found dilated cysts in the back of the throat and deep in the chest that could cause breathing problems. He knew immediately that UNC Health Care would be able to treat Elijah.
Dr. Zdanski explained to Elijah’s parents that the boy’s care would take multiple treatments over the next few years and eventual surgery to correct the skin and tissue below the cysts. Also, close surveillance was necessary in the event the cysts suddenly enlarged and compressed his throat or became infected.
Due to the invasive treatment Elijah’s case required for correcting the malformed lymph vessels, Zdanski referred him to UNC’s Vascular-Interventional Radiology (VIR) division for assistance and treatment. Since Elijah’s first corrective treatment in early 2011, Vascular-Interventional Radiology Professor Dr. Joe Stavas has worked closely with the Smith family over the course of 13 outpatient procedures, conducting the most recent one in September 2013. Drs. Zdanski and Stavas collaboratively mapped out a long-term treatment plan with Elijah’s parents that included MRI scans, drainage and ablating the cysts to cause shrinkage. These early steps would provide a basis for future surgery to remove extra skin and malformation tissue.
Elijah has progressed through his first two years of life with relative ease, yet the challenges related to his throat and chest distortions range from limited swallowing to the emotional toll on his family due to stares and even cruel comments in public from others. Fortunately, with each surgery, Elijah’s throat and airway obstructions have cleared, and the distortions in his lower neck have diminished. His only setback during the course of treatment occurred at five and 10 months, when he underwent inpatient stays for infected and inflamed lesions. Two hours after Dr. Stavas conducted his last procedure on Elijah in September, the toddler was back at home in Wade.
Elijah’s parents April and Tim are very involved in his care, and the entire UNCH team that cares for him have become very close to them. Tim often brings reading materials for the doctors and April sends pictures of Elijah “just being a toddler” around the house. “We’ve seen him grow and be happy boy since this all began,” remarked Dr. Stavas.
With each of Elijah’s surgeries, April Smith observed how her son’s throat and chest area restored itself to more natural form, giving her greater peace for how he will progress as a healthy, growing child.
“We were lucky from the beginning, because Elijah didn’t have the severe lesions I’ve seen from photos of other children with this condition. Don’t get me wrong, it took us a good bit of strength to get through 13 surgeries in two years of my child’s life, but we’re really blessed as a family because of it all. He’s such a happy, playful 3-year-old now!”