Prenatal Differential Diagnosis and Prospective Management of Hydranencephaly

Abstract

Objective: To report a rare case of hydranencephaly that was diagnosed during prenatal period. We also provided further review of differential diagnosis and management performed in Ende District General Hospital, based on appropriate literatures and guidelines available. Methods: Case Report. Case: A 27-year old primigravida women was diagnosed with term pregnancy (37 weeks of gestation) and intrauterine singleton live fetus with hydranencephaly via ultrasonography. In this patient, emergency caesarean section (CS) was performed to prevent complication of cephalo-pelvic disproportion (CPD), involving teamwork between obstetrics and perinatology. A male neonate was born weighing 3000 grams, head circumference of 32 cm, and APGAR score of 2/4/7/8 suggestive of asphyxia and respiratory distress. The newborn was immediately transferred to Neonatal Intensive Care Unit (NICU) for further resuscitative management and observation. The newborn remains in stable condition after resuscitative management was given. Post-natal transcranial sonography (TCS) of the newborn was performed by a pediatrician, and the diagnosis of hydranencephaly was confirmed. The newborn was later referred to facilities with neurosurgery department for further evaluation and intervention. Conclusion: Early prenatal recognition of hydranencephaly and exclusion of similar differential diagnosis, which includes: hydrocephalus, holoproscencephaly, porencephaly and schizencephaly, are fundamental in formulating proper multidisciplinary management with pediatric and neurosurgery department, which may consequently improve the newborn’s life expectancy.