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Breast reconstruction in a patient with Noonan syndrome.


ABSTRACT: Noonan syndrome (NS) is a relatively common genetic disorder with an autosomal dominant inheritance pattern affecting 1 in 1000-2500 births. Patients with this syndrome present with characteristic facial, musculoskeletal, cardiac and endocrine abnormalities. Lack of postpubertal breast development is a common manifestation of this syndrome and may result in severe hypomastia and a masculine appearance of the female chest. We report the first case of breast reconstruction in a 24-year-old woman with NS who lacked postpubertal breast development. Technical considerations for addressing the existing chest wall deformity, implant pocket selection as well as emphasis on the role of the plastic surgeon are presented.

SUBMITTER: Sharaf B 

PROVIDER: S-EPMC5695322 | biostudies-literature | 2017 Nov

REPOSITORIES: biostudies-literature

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Breast reconstruction in a patient with Noonan syndrome.

Sharaf Basel B   Sabbagh Mohamed Diya MD   Roh Si-Gyun SG  

BMJ case reports 20171101


Noonan syndrome (NS) is a relatively common genetic disorder with an autosomal dominant inheritance pattern affecting 1 in 1000-2500 births. Patients with this syndrome present with characteristic facial, musculoskeletal, cardiac and endocrine abnormalities. Lack of postpubertal breast development is a common manifestation of this syndrome and may result in severe hypomastia and a masculine appearance of the female chest. We report the first case of breast reconstruction in a 24-year-old woman w  ...[more]

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