[Cervical cystic lymphangioma: still a challenge]

Cervical lymphangiomas can not only cause severe sequelae (aesthetic, phonatory or deglutory) but can also be life thereatening due to airway compresion or massive bleeding. This paper analyzes our surgical results, the value of prenatal diagnosis and the use of new techniques such as the EXIT procedure for airway control in sereve cases.

We retrospectively reviewed the medical record of patients with cervical lymphangiomas treated in our center between 1986 and 2009, according to our Clinical Documentation Database. Data referred to prenatal diagnosis, intrapartum airway management, surgical procedures and morbidity, sclerosing substance infiltration and long term sequelae was analyzed.

Thirteen cases were identified. 53.8% of the patients were diagnosed by prenatal ultrasound, and MRI was performed in 46.1%. The tongue was affected in 30.7%, parotid glands in 38.4% and airway in 38.4%. Four EXIT procedures were carried out (nasotracheal intubation) and one emergency tracheotomy was needed. Five patients required more than one surgical prodedures, including partial glossectomy, with severe intraoperatory complications in 23% (bleeding, pharyngeal damage). In eight patients primary or adyuvant sclerotherapy was used. Three children with giant masses died, two due to intracystic bleeding and one from sepsis. Among the survivors, 50% have no sequelae.

Cervical lymphangiomas are a very sereve condition, not only due to possible airway compresion or massive bleeding but also becose of the severe secualaes they may cause. Tongue or parotid gland infiltration are difficult to treat. In severe cases diagnosed prenatally a close follow up in selected centers, with multidisciplinary teams consisting of obstetricians and pediatric surgeons, trained in the EXIT procedure is warranted. Despite therapeutic efforts the prognosis of large masses is still poor.