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Efficacy of sirolimus combined with sclerotherapy for giant cervical lymphatic macrocystic malformations: two newborn cases

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Lymphatic malformations (LMs) are classified as microcystic, macrocystic or mixed and can be superficial or deep (reaching organs) [1]. Macrocystic LMs may appear in the antenatal period, be apparent at… Click to show full abstract

Lymphatic malformations (LMs) are classified as microcystic, macrocystic or mixed and can be superficial or deep (reaching organs) [1]. Macrocystic LMs may appear in the antenatal period, be apparent at birth, or may develop after birth. Treatment, when required, is usually based on sclerotherapy and can be repeated [2]. Recently, several publications have reported the efficacy of sirolimus, a mammalian target of rapamycin (mTOR) inhibitor, for treating various types of vascular abnormalities, especially microcystic and mixed LMs [3-6]. Here, we report the remarkable reduction of two giant cervical macrocystic LMs in newborns that were detected in utero and had an expectedly poor prognosis. Treatment with sclerotherapy and sirolimus was effective. The first case was a retrocervical macrocystic LM diagnosed by ultrasonography in the foetus of a 29-year-old woman at 17 weeks of gestation (WG). Foetal MRI performed at 32 WG revealed a very large right cervical mass pushing down on the right shoulder and infiltrating the hypopharynx (figure 1A). Caesarean section (CS) delivery was planned for 38 WG. To reduce the mass, two

Keywords: sirolimus combined; sclerotherapy giant; combined sclerotherapy; giant cervical; efficacy sirolimus

Journal Title: European Journal of Dermatology
Year Published: 2019

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