Pediatric lymphatic malformations: evolving understanding and therapeutic options

Abstract

Multimodal treatment of lymphatic malformations continues to expand as new information about the biology and genetics of these lesions is discovered, along with knowledge gained from clinical practice. A patient-centered approach, ideally provided by a multidisciplinary medical and surgical team, should guide timing and modality of treatment. Current treatment options include observation, surgery, sclerotherapy, radiofrequency ablation, and laser therapy. New medical and surgical therapies are emerging, and include sildenafil, propranolol, sirolimus, and vascularized lymph node transfer. The primary focus of management is to support and optimize these patients' quality of life. Researchers continue to study lymphatic malformations with the goal of increasing therapeutic options and developing effective clinical pathways for these complicated lesions.

Successful management of extremely high‐output refractory congenital chylothorax with chemical pleurodesis using 4% povidone–iodine and propranolol: a case report

“…We were unable to confirm a diagnosis in our patient of lymphangiomas by CT and given her clinical improvement after pleurodesis and propranolol; lung biopsy was not indicated. However, her response to propranolol suggested the presence of mixed vascular malformations . The decision to begin a trial of propranolol was influenced by our patient's significant generalized edema that worsened even after pleurodesis.…”

Sirolimus as treatment for 19 patients with refractory cervicofacial lymphatic malformation

“…The airway can be narrowed by mass effect from an adjacent macrocyst, laryngeal or bronchial microcystic LM, or circumferential subglottic LM. In addition, LM in this location may cause social, swallowing, and speech or communication difficulties . Current management options include observation; sclerotherapy; radiofrequency ablation; laser therapy; and/or surgical interventions including debulking of symptomatic regions or total resection, if amenable.…”

“…In addition, LM in this location may cause social, swallowing, and speech or communication difficulties. 8 Current management options include observation; sclerotherapy; radiofrequency ablation; laser therapy; and/or surgical interventions including debulking of symptomatic regions or total resection, if amenable. Historically, the primary form of treatment was surgical resection.…”

Classification of Vascular Anomalies: An Update

“…These lesions are often superficial, underlying normal skin or subcutaneous tissue, though deeper spaces and structures (e.g., retroperitoneum, aerodigestive tract) are occasionally involved. 36 Cervical macrocystic LMs can be seen in association with chromosomal abnormalities, most notably in Turner's, Noonan's, and Down's syndromes. Likewise, microcystic LMs can occur in many tissues, but most commonly affect the skin or mucosal surfaces.…”