Síndrome de Crouzon

Abstract

Crouzon syndrome is characterized by being a congenital disease in which premature closure of the cranial sutures is presented. Among the most frequent we have the sagittal, coronal and lambdoid sutures, which will produce an abnormal growth of the skull accompanied by malformations at the level facial. The treatment of this pathology will be aimed at the surgical treatment that will consist in the replacement of the cranial sutures and in this way allow the normal development of the brain and the skull, as well as releases the pressure at the facial level avoiding the deformations of it. The case presented below corresponds to a 2-month-old child who enters the General Provincial Hospital of Riobamba on October 11, 2017 for presenting as a reason for consultation cough and transfer of the SCS of the canton of Guamote with Crouzon syndrome diagnosed by genetic study. The patient presents the manifestations of this syndrome such as: head in tower with accipital flattening, skull with brachiocephalic synostosis, back of nose in parrot`s beak, ogival palate, and rest of normal physical examination according to age. In addition to physical examination, imaging exams such as computerized axial tomography of the skull, that transfontanel and genetic tests also confirm the diagnosis described above and allow early treatment which is purely surgical (cranial remodeling) which is carried out in a successful way and for which it is controlled to observe its evolution.