Mallesh Kariyappa1, Shivashankar Diggikar2, Prakash Javarappa3, Soumya Katte Krishna4

Seven months old female infant, second born to non-consanguineous parentage presented with inability to recognize mother, lack of social smile, delayed attainment of head control. There was uneventful antenatal history. It was a full term vaginal delivery of baby weighing 2.5 kgs and cried immediately after birth. Vitals were stable. Head circumference was 33cms, length was 57cms, and weight was 5.5kgs. Receding forehead, prominent metopic sutures, closed anterior fontanels, low set ears, long philtrum and high arched palate. (Figure A) Neurological examination revealed global delay in milestones with spasticity of all four limbs with exaggerated reflexes and extensor plantar response. Other systems were normal clinically. Hemoglobin was 11.2gm/dl, total count was 11,600/mm3 . IgM and IgG antibodies for TORCH infection were negative. Karyotyping revealed 46, XX chromosomes. T1W and T2W Magnetic resonance imaging of Brain showed severe thinning of supra tentorial parenchyma with relative sparing of thalami and basal ganglia, falx is maintained. The lateral and third ventricles are dilated with disproportionate dilation of temporo occipital horns. Corpus callosum thinning is also noticed (Figure B, C, and D). There was evidence of tonsillar herniation and indentation of posterior craniocervical cord. All these features were suggestive of hydranencephaly with hydrocephalus. Counseling was done regarding prognosis of condition. Mother was trained about feeding, physiotherapy