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Multimed

versión On-line ISSN 1028-4818

Resumen

PEREZ DAJARUCH., María de los Ángeles; FERRER MONTOYA., Rafael  y  MONTERO AGUILERA., Alexis. Poland syndrome. Report of a case. Multimed [online]. 2020, vol.24, n.2, pp.434-442.  Epub 23-Abr-2020. ISSN 1028-4818.

Introduction:

Poland syndrome is a rare congenital disorder characterized by the unilateral absence or hypoplasia of the pectoralis major muscle associated with malformations of the upper limb and ipsilateral breast tissue. Clinical manifestations in newborns are only described in the literature, therefore, many aspects of their presentation in nurseries are ignored.

Presentation of the case:

we present the case of a newborn who manifests paradoxical respiration from the moment of birth. This type of clinical finding is loosely described in Poland Syndrome and even less in the neonatal period.

Discussion:

this syndrome can be related to diseases, such as paralysis of pairs VI and VII, which leads to a greater number of complex associations.9 It arises in association with dextrocardia, which according to some authors is secondary to the thoracic deformity caused by malformations ribs and their effect on cardiac formation in the embryonic period. No mental deficiencies or genetic transmission of the disease have been observed. An exceptional family association has been described. This could correspond to an autosomal dominant inheritance with reduced penetration.

Conclusions:

in this patient, Poland syndrome was characterized by hypoplasia of the left pectoralis major, associated with skeletal malformations of the hand on the same side.

Palabras clave : Poland syndrome; Congenital abnormalities.

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