A 20-year-old primigravida with no antenatal care presented to the Obstetric outpatient department at 37 wk completed gestational age. Antenatal ultrasound demonstrated isolated gross fetal ventriculomegaly with absent septum pellucidum. Adduction deformity was present in both thumbs. He had tumultuous post natal life with seizures from 2 h of life, recurrent apnea and death at 72 h of age. Postnatal computed tomography showed hydrocephalus due to aqueductal stenosis and absent septum pellucidum. The clinical and radiological features were consistent with the diagnosis of Bickers-Adams syndrome.
Keywords
Hydrocephalus - septum pellucidum - aqueductal stenosis - L1 cell adhesion molecule - Bickers-Adams syndrome
