Neonatal Generalized Lymphatic Anomaly with Skin Involvement
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Generalized lymphatic anomaly (GLA) is a rare congenital disorder of lymphatic develop- ment, presenting with multiple lymphatic malformations in different organs and tissues. Here, we present a case of a female neonate prenatally diagnosed with foetal hydrops and a mediastinal cystic lymphatic malformation that showed postnatal expansive and infiltrative growth into the major airways, compromising mechanical ventilation and further manage- ment of the neonate. Complications that arose during surgical treatment of mediastinal struc- tures led to the patient’s death. Lymphatic malformations were also noted in the skin at birth. Furthermore, a skin biopsy performed immediately after birth and the autopsy revealed an extremely rare diagnosis of combined macrocystic and microcystic forms of GLA with skin involvement.
Abstract
Generalized lymphatic anomaly (GLA) is a rare congenital disorder of lymphatic develop- ment, presenting with multiple lymphatic malformations in different organs and tissues. Here, we present a case of a female neonate prenatally diagnosed with foetal hydrops and a mediastinal cystic lymphatic malformation that showed postnatal expansive and infiltrative growth into the major airways, compromising mechanical ventilation and further manage- ment of the neonate. Complications that arose during surgical treatment of mediastinal struc- tures led to the patient’s death. Lymphatic malformations were also noted in the skin at birth. Furthermore, a skin biopsy performed immediately after birth and the autopsy revealed an extremely rare diagnosis of combined macrocystic and microcystic forms of GLA with skin involvement.
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