ABSTRACT
Conservative treatment, with rapamycin and/or sclerosis, of large lymphatic malformations in childhood is often ineffective and fails to eradicate the most important symptoms. Additionally, upper airway involvement can be a life-threatening condition in neonates. In these situations, the surgical treatment of lymphatic malformations should be considered. Massive malformations are the candidates for early surgery. Large debulking procedures are more effective than repeated sclerosis or long-term sirolimus treatment. Surgical excision can be safely performed in the first months of life in the head, neck, axilla, thoracic, or abdominal wall and limbs. The goals of a rational surgical management of lymphatic malformations early in life are to prevent prenatal mortality, preserving upper airway patency, avoiding tracheostomy and visceral functional impairment. Adjuvant sclerosis, pharmacological, and laser treatments in the context of an expert pediatric multidisciplinary team can provide the best expected outcome, minimizing the risk of sequelae.
