As a world center of excellence in radiology, the interventional radiology team at Nationwide Children’s Hospital has pioneered numerous minimally-invasive treatments for macrocystic, microcystic and solid lymphatic malformation in children and adults over the past two decades. Patients travel from around the world to Nationwide Children's Hospital for minimally-invasive treatment of lymphatic malformation.
While orbital lymphatic malformation is a unique challenge, our team provides the most comprehensive treatment solutions for outpatient treatment of both macrocystic and microcystic orbital lymphatic malformations. Patients have access to a full, multidisciplinary management team that includes oculoplastic and ophthalmologic surgeons, who have decades of treatment experience of orbital lymphatic malformations and vascular anomalies.
If you are interested in minimally-invasive treatment options, call our Interventional Coordinator at (614) 722-2375 to set up a consultation with an Interventional Radiologist. Our team works with other specialists in a multidisciplinary Vascular Anomalies and Hemangioma management team, and consultations with surgeons and dermatologists can also be coordinated.
Dr. Shiels dedicated his life to advancing the field of radiology in order to provide the best care to children in our community, across the country and around the world. As we remember and celebrate his work, we will remain ever-grateful for his passionate mentorship of our radiology team and staff – some of whom have worked side by side with Dr. Shiels for more than a decade. Everything Dr. Shiels learned over his career, he taught to and instilled in his staff including his revolutionary advancements in interventional radiology and ultrasound; treating lymphatic malformations; bone tumor ablation, aneurysmal bone cyst and salivary gland ablation. His passion inspires the entire team to continue delivering the best possible care to children everywhere and his legacy will live in the advancements we continue to make here at Nationwide Children’s.
Lymphatic malformations are now most often treated using interventional radiology minimally-invasive (percutaneous) techniques designed to ablate (or kill) the cysts within the body. In this situation, a chemical injected into the lymphatic malformation cyst kills the cyst. The body then cleans up the dead cyst tissue over time. This can be done without large scars or the need for hospitalization.
In the past, surgical removal has been considered standard treatment for these cavernous lesions, despite recurrences in 15 to 53 percent of reported clinical series. Percutaneous (interventional radiology) treatments for both macrocystic and microcystic lymphatic malformation now offer patients a predictable plan for both initial definitive treatment, as well as injection treatment of new cysts that fill up over time from the “solid” membranes that may be present. Microcystic injection therapy developed at Nationwide Children’s Hospital now offers patients a long-term option for percutaneous, minimally invasive treatment of new lymphatic malformation microcysts that are detected before they become problematic or develop into painful macrocysts.
Treatment options for lymphatic malformation include:
Macrocysts (large cysts)
Macrocysts are treated by placing a drainage tube in the cyst through a small skin opening, draining the cyst fluid, and placing two drugs (dual-drug technique) in the cyst to kill the cyst. The first drug is a medical detergent that “cleans” the cell covering, opening pores in the cell membrane. The second drug is ethanol (alcohol) that penetrates through the open pores, entering the cell to kill the cells within 15 minutes. The drainage tube is left in place for 3 days while the injured cyst “oozes” fluid that is carried out by the drain for two days, and on the third day, the injured area begins to heal with tissue “glue” that seals the treated area during the healing process.
Above: this illustration demonstrates the use of a pigtail catheter to drain a cyst and the injection of solution to ablate the macrocyst. It also demonstrates the dual syringe method for aspirating and injecting doxycycline foam into lymphatic malformation microcysts.
Microcysts (small cysts)
Microcysts are treated and ablated with precise injection of a drug known as doxycycline (an antibiotic that was found to kill lymphatic malformations as well as treat infections). Injection of doxycycline into the microcysts (each cyst individually injected) is performed with a very small needle, like the one used for a vaccine injection (example: tetanus shot). The doxycycline is injected as a drug foam, developed at Nationwide Children’s Hospital, the foam looks and acts like meringue, similar to the topping of a lemon meringue pie. The body cleans up the killed lymphatic malformation from within, and heals the treated area without needing surgery to remove the dead tissue.
Above: this animation video shows the treatment of microcystic lymphatic malformation in the eye
If a lymphatic malformation is left untreated, the large and small cysts can rapidly enlarge with bleeding and pain, or become infected. In areas such as the orbit, lymphatic malformation left untreated may result in severe pain, headaches and blindness.
Sclerotherapy is injection drug therapy of a lymphatic malformation that kills the cyst within the body, without surgical scars or hospitalization.
Macrocysts are treated by precisely placing a drainage tube (catheter) in the cyst(s), most often with ultrasound camera guidance, through a small skin opening. With the tube in proper position, the cyst fluid is drained, cells obtained for microscopic diagnosis, and a radiology contrast agent (X-ray dye) is injected to fully define the cysts. Treatment then begins by injecting two drugs (dual-drug technique) in the cyst(s) to kill them. The first drug is a medical detergent that "cleans" the cell covering, which opens pores in the cell membrane. The second drug is ethanol (alcohol) that penetrates through the open pores, entering the cell to kill it within 15 minutes. The drainage tube is left in place for 3 days while the injured cyst "oozes" fluid that is carried out by the drain for two days. On the third day, the injured area begins to heal with tissue "glue" that seals the treated area during the healing process.
Microcysts are treated and ablated with precise needle injection of a drug known as doxycycline (an antibiotic that was found to kill lymphatic malformations as well as treat infections). Injection of doxycycline into the microcysts (each cyst individually injected) is performed with a very small needle, like the one used for a vaccine injection (example: tetanus shot). With ultrasound guidance, the needle is precisely placed into each microcyst, the cyst fluid drained, and doxycycline is injected as a drug foam. Developed at Nationwide Children’s Hospital, the foam looks and acts like meringue, similar to the topping of a lemon meringue pie. The doxycycline foam allows the interventional radiologists to precisely treat tens, or even hundreds, of cysts knowing which cysts have been treated and which remain to be treated. The body cleans up the killed lymphatic malformation from within and heals the treated area without needing surgery to remove the dead tissue.
Since the treatment involves placing needles precisely into the lymphatic malformation, most patients are treated while asleep with anesthesia. Adults with small areas of lymphatic malformation may be successfully treated at Nationwide Children's Hospital without sedation or anesthesia.
Most treatments (macrocystic and/or microcystic) are performed within 1-2 hours, most often as outpatients without the need for hospitalization.
Treatment risks are rare but may include infection, mild pain and mild swelling. Bleeding is usually not a problem and injury to tissues or organs next to the lymphatic malformation is avoided by precise placement of drainage tubes and injection needles.
Patients being treated for macrocystic and microcystic lymphatic malformation are discharged to go home (or to a hotel or the Ronald McDonald House). Patients who have macrocystic treatment are able to leave after they're awake following the treatment. They will have their drainage tubes in place during the 3-day drainage period. Patients who have microcystic treatment are able to leave following their treatment session. Patients most often have little or no pain or "recovery time" following the procedure.
Patients being treated for macrocystic lymphatic malformation are not permitted to take baths or showers during the 3-day drainage period. Following drainage tube removal, patients are able to shower but should refrain from a submersion bath for 5 days after the drainage tube has been removed. Patients treated for microcystic lymphatic malformation are able to bathe and shower immediately following treatment.
Patients being treated for macrocystic lymphatic malformation can resume normal activities, avoiding activities that may cause the drainage tubes to be pulled or accidentally removed during the 3-day drainage period. Following drainage tube removal, patients are able to resume normal activities. Patients treated for microcystic lymphatic malformation are able to resume normal activity immediately following treatment.
Macrocystic and microcystic lymphatic malformation treatment is effective greater than 95 percent of the time, with the majority (85 percent) of macrocystic lymphatic malformation patients responding in a single treatment. Patients will be monitored annually for at least 5 years with ultrasound or MRI, watching for signs of new microcysts developing from solid lymphatic malformation tissue that is within the body. If and when new microcysts develop over time, minimally-invasive microcystic injection therapy can be planned before microcysts develop into painful macrocysts.
Macrocystic treatment is painless, with no cases of permanent nerve injury, muscle injury or other major complications. During the three days of catheter macrocyst drainage (after treatment), most patients (including orbit lymphatic malformation) are fully mobile and functional, without pain. Patients undergoing microcystic treatment may experience mild pain for two to four hours, responsive to oral narcotic analgesic therapy.
Following treatment, patients are able to resume normal life activities, including sports. Patients undergo annual follow-up ultrasound or MRI examination (minimum five years) to evaluate for new microcysts that may mature from remaining solid LM tissue. New microcysts, if any, that develop are readily treated with simple microcystic needle injection therapy as an outpatient. In the past 15 years, greater than 95 percent of patients respond with complete success of macrocystic and microcystic lymphatic malformation components.
Patients treated at Nationwide Children’s Hospital from states other than Ohio (or other countries) undergo clinical follow-up with their local referring physicians and send imaging studies for Nationwide Children’s radiologists to review, for telephone follow-up and future treatment decision-making.
Patients who have undergone treatment for lymphatic malformation at Nationwide Children’s Hospital, range in age from 1 month to 60 years. Locations of percutaneous treatment for lymphatic malformation include the orbit, ear, neck, head, arm, leg, hand, foot, axilla, mediastinum, chest wall, abdomen, scrotum and penis. Most procedures are performed as definitive first-line therapy, with occasional patients referred after surgical debulking or following surgical recurrence. Procedures are performed in the interventional radiology suite under deep, intravenous anesthesia or general anesthesia. Treatment is divided into two therapeutic regimens, one for macrocystic disease and one for microcystic disease. Most lymphatic malformation treatments are performed as outpatients, with extensive cases involving a one-night stay in the hospital for intravenous antibiotic therapy and fluid management.
In the treatment of large cysts, there is a 15% incidence of repeat hemorrhage in macrocysts following initial sclerotherapy. Following repeat hemorrhage, a second treatment has been effective for ablation in 100% of cases, without scarring or functional limitation. In our patients, no cases of neuropathy, unexpected skin necrosis or myoglobinuria have been encountered with sclerotherapy. Microcystic lymphatic malformations are successfully treated with 1-3 treatments in greater than 95% of patients.
Personally trained by Dr. Shiels, James Murakami, MD, MS, will be carrying on the care of Dr. Shiels' patients. A faculty member at Nationwide Children's for more than 15 years, Dr. Murakami has interventional radiology interests focusing on sclerotherapy of vascular malformations and benign cysts of the head and neck, orbits, and bones.