Abstract

BARRON MUNOZ, María de los Ángeles; HERNANDEZ REYES, Cecilia; SERNA VALDES, Raquel Emilia  and  TORRES FLORES, Jorge. Congenital hydranencephaly: report of an adolescent in the north of Mexico. AMC [online]. 2016, vol.20, n.5, pp.546-552. ISSN 1025-0255.

Background: hydranencephaly is the total or almost total absence of the cerebral hemispheres with persistent cerebrospinal fluid, which affects individuals around the world regardless of gender or ethnicity. There is no effective and curative treatment, and most patients die before reaching the third year of life, although some exceptions can come of age, always requiring multidisciplinary support. Objective: to present the case of an adolescent male with congenital hydranencephaly. Clinical case: a 11-year-old male from 11 who was admitted at the age of 5 referred to as holoprosencephaly. He was born by caesarean section at 38 weeks of gestation due to rupture of membranes with normal somatometry and Apgar 5, requiring advanced neonatal resuscitation maneuvers. At 30 days head circumference increased to 39 cm and was increasing, reaching 54 cm at 6 months of age. The patient has no control head and trunk, and he was not capable of bipedalism, or developed language or sound emission. A brain scan performed on admission revealed brain parenchyma islets and meninges, corresponding to hydranencephaly. The case is presented by the infrequency with which patients reach adolescence. Conclusions: hydranencephaly is a disease that is usually fatal, and cases that survive have severe and disabling neurological sequeles. Although some genetic syndromes associated with hydranencephaly are known, most cases are usually sporadic with no other manifestations. Though the hope that the case presented shows greater progress in their neurological development is not encouraging, physical, occupational and pulmonary therapy may allow better quality of life.

Keywords : HYDRANENCEPHALY; CEREBRAL PALSY; MUSCLE SPASTICITY; CHILD; CASE REPORTS.

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