The Current Approach to Lymphatic Malformations
NYP/Morgan Stanley Children’s Hospital has one of the top programs in the nation for the diagnosis and treatment of pediatric vascular anomalies. The Vascular Anomalies Group evaluates patients with a variety of vascular tumors, malformations, and associated syndromes including the newly categorized PIK3CA-related overgrowth spectrum (PROS), formerly labeled overgrowth syndromes.
“Since 2001, we have managed children with various vascular anomalies and associated syndromes with a multi-disciplinary approach. Our group consists of specialists from pediatric dermatology, hematology, oncology, genetics, as well as pediatric surgery and surgical subspecialties such as ophthalmology, ENT, and orthopedics,” says Angela Kadenhe-Chiweshe, MD, who with June Wu, MD, in Plastics Surgery, heads up the surgical team.
The Vascular Anomalies Group is led by Dr. Maria Garzon of Pediatric Dermatology and Dr. Wu. A year ago a subgroup was established to provide directed, customized care to patients with lymphatic malformations.
“Dr. Wu and I developed this group to provide timely consultation and care to the increasing referrals for lymphatic malformations,” says Dr. Kadenhe-Chiweshe. “We take a multidisciplinary approach, collaborating with Dr. Garzon, and Philip Meyers, MD, a neuro-interventional radiologist, to provide current treatments to patients with lymphatic malformations in all parts of the body. This includes sclerotherapy, embolization, and/or surgery. We also offer the very latest medical options. In addition Carrie Shawber, PhD is studying the pathobiology of these lesions to identify new targets for treatment.”
Lymphatic malformations occur primarily in the cervicofacial region (formerly known as cystic hygroma), but can occur anywhere in the body. They are often sporadic and localized, but can be multifocal or part of a syndrome in some rare cases. These lesions can be diagnosed on prenatal ultrasound. The increasing use of fetal MRI has been instrumental in providing practitioners with detailed information about the lesions and its affect on the unborn child, particularly vital structures.
“Our colleagues in Maternal Fetal Medicine have a great deal of experience in the diagnosis and in some cases the prenatal treatment of these lesions,” says Dr. Kadenhe-Chiweshe. “When reviewed in our multidisciplinary Prenatal Conference, a fetal MRI can provide invaluable anatomical information allowing members of the delivery team – anesthesia, obstetrics, neonatology, ENT, surgery – to adequately prepare for the baby’s delivery.” This is crucial in situations where the lesion is encroaching and constricting the airway. The team is prepared for a delivery scenario called EXIT (ex utero intra partum).
While the newborn is still connected to placental circulation, the neonatologists establish a safe airway, then ligate the cord. If intubation is not possible, then the surgeons must perform a tracheostomy or place the patient on extracorporeal membrane oxygenation (ECMO) prior to ligating the cord. Once stabilized, a postnatal MRI is obtained that informs the treatment plan for the baby’s malformation.
“Infants and children with previously undiagnosed lymphatic malformations also are often referred to us from other hospitals, sometimes with the patient in critical condition from infection/sepsis or mass effect on vital structures,” says Dr. Kadenhe-Chiweshe. “We are fortunate to have an excellent transfer system that outside hospitals and practitioners can take advantage of through NYP-STAT to request transfer of a patient to our ICU.”
A recent transfer involved a 5 month-old girl with sepsis and a threatened airway from a large infected cervico-facial LM. The patient was transferred to our PICU where she was stabilized and her infection controlled. “We performed a combination of surgical debulking and sclerotherapy to which she responded well,” reports Dr. Kadenhe-Chiweshe. “She was eventually able to avoid a tracheostomy and come off the ventilator. She is now home and she’s thriving.”
Twenty years ago most hospitals performed extensive surgery on lymphatic malformations, resulting in large scars and significant morbidity. Current treatment often leads with sclerotherapy performed by interventional radiologists. When appropriate, surgery is the primary treatment. Some lesions require both interventions.
“When possible, we delay any diagnostic imaging or intervention that requires general anesthesia to achieve, until an infant is at least six months to a year old. All patients are monitored regularly to track the lesion’s affects on the patient and to identify an ideal time to intervene,” says Dr. Kadenhe-Chiweshe. "However, if the situation is life-threatening, we are prepared to move ahead with a high level of clinical experience in the current approach to lymphatic malformations with our specially trained medical and surgical team.”
More information about the vascular anomalies program can be found here
To refer a patient, please call our Vascular Anomalies Group at (212) 342-3646 or our Lymphatic Malformation Group at (212) 324-8586.
Our recent publications include:
- Initial Experience With Propranolol Treatment of Lymphatic Anomalies: A Case Series.
Wu JK, Hooper ED, Laifer-Narin SL, Simpson LL, Kandel J, Shawber CJ; Lymphatics Work Group at CUMC Pediatrics 2016 Sep;138(3)
- Vascular Anomalies Classification: Recommendations From the International Society for the Study of Vascular Anomalies.
Wassef M, Blei F, Adams D, Alomari A, Baselga E, Berenstein A, Burrows P, Frieden IJ, Garzon MC, Lopez-Gutierrez JC, Lord DJ, Mitchel S, Powell J, Prendiville J, Vikkula M; ISSVA Board and Scientific Committee.. Pediatrics 2015 Jul;136(1):e203-14.