Severe Hydranencephaly due to chorioamnionitis: case report

*Correspondence: [e-mail].

Abstract

Case Presentation: Newborn by cesarean at 37 weeks, with healthy and nonconsanguineous parents. Prenatal care with 8 consultations without intercurrences. Maternal serology non-reactive for Venereal Disease Research Laboratory (VDRL), Hepatitis B Surface Antigen (HBsAg) and Human Immunodeficiency Virus (HIV). At birth, he was a body length of 46cm, head circumference measured 33cm, a weight of 2455g, Apgar (Appearance, Pulse, Grimace, Activity and Respiration) 7/9, good muscle tone, presence of cleft lip, nasal hypoplasia, no crying, requiring room air resuscitation. During the placenta and amniotic fluid inspection, was noted an odor and color indicative of chorioamnionitis. Nine hours after childbirth, the baby had seizures with chewing and eye movements, in addition to upper and lower limb twitching. An attack dose of Phenobarbital - 20mg/kg was performed and Ampicillin 100mg/kg + Gentamicin 4mg/kg/day was started for 10 days for treatment of presumed neonatal sepsis. In addition, laboratory and imaging tests were requested (Hemoglobin: 15.6; Hematocrit: 44.1; Leukocyte Count: 19200; Platelet Count: 215000; Ca: 8.4; Mg: 1.58; K: 3.7; Na: 127; Capillary Blood Glucose: 120mg/dl; Chest X-ray: Cardiomegaly; Echocardiogram: Bulging of the interatrial septum towards the left ventricle, suggestive of interatrial septal aneurysm; Transfontanel USG: Hydranencephaly; Head CT: Hydranencephaly; USG abdomen: no changes). With these results, the patient was diagnosed with severe Hydranencephaly due to chorioamnionitis and a ventriculoperitoneal shunt was placed. At the 2nd month, he was discharged from the hospital on continuous use of Phenobarbital - 5mg/kg /day, orogastric tube and proposed palliative care.

Discussion: Hydranencephaly is a central nervous system disorder that affects 1 in 10,000 children worldwide, characterized by complete or partial absence of the cerebral cortex and basal ganglia, with thalamus and brainstem preservation. The disease pathophysiology is unknown, however, it is believed to be related to the presence of infectious processes, such as chorioamnionitis, which generated an intrauterine occlusion of the bilateral supraclinoidal internal carotid arteries. In this sense, the brain tissue supplied by the anterior circulation is reabsorbed.

Final Comments: This case reflects the importance of more detailed studies about Hydranencephaly, since it is a condition that is difficult to diagnose, with a high mortality rate and that requires specialized clinical and surgical treatments.

Publication History

Article published online:
12 May 2025