Cystic hygroma and lymphangioma, currently defined as ‘lymphatic malformations', are developmental abnormalities of the lymphoid system with potential for rapid expansion. The management of these abnormalities depends on the site and extent of the lesion. A different disease is diffuse lymphangiomatosis. It is very rare at birth and its treatment remains controversial. A lymphatic malformation (cystic hygroma) of the neck of a newborn girl as the first sign of diffuse lymphangiomatosis and treatment of the latter condition with sirolimus are presented. A newborn girl with a presumed isolated lymphatic malformation of the neck and significant respiratory involvement was diagnosed via total-body nuclear magnetic resonance imaging to be affected by diffuse lymphangiomatosis; she was treated with sirolimus. The treatment with sirolimus was found to be very effective, with complete resolution of the disease, good tolerability, and no side effects. This report suggests the need to explore the possibility of diffuse lymphangiomatosis in neonates with apparently isolated lymphatic malformations. In the presence of clinical deterioration, sirolimus treatment may achieve excellent results with no adverse events.

Congenital pulmonary airway malformations (CPAMs) are a heterogeneous group of hamartomatous cystic and noncystic lung lesions that result from early airway maldevelopment. Usually they are distinguished according to Stocker's classification in type 0, 1, 2, 3 and 4.We present the case of a 2 weeks old baby who was admitted to hospital with RDS symptoms and left pleural effusion: X rays and CT were suggestive for a pulmonary cystic lesion with pleural complications. Because of the persistence of pleural empyema and the development of a pneumothorax the baby underwent surgery. The histological examination revealed a type 3 CPAM associated with pleural loculated empyema.According to this case, in newborns with RDS loculated pleural empyema may mimick pulmonary cystic lesions; a treatment-resistant pleural empyema or pyopneumothorax in a newborn can recognize a CPAM 3 as a probable underlying condition, even in the absence of lung suppurative changes; CPAM 3 involving only two lung segments can have an excellent prognosis after surgical excision.

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