G423(P) Uncommon presentation of septo-optic dysplasia

Introduction Neonatal metabolic acidosis is a common presentation of wide range of causes varying from sepsis to rare metabolic and endocrine problems. Metabolic acidosis with normal anion gap help as to narrow down the cause as GI loss, RTA or adrenal insufficiency. We present a rare case of neonatal metabolic acidosis turned out to be due to adrenal insufficiency and subsequently diagnosed as SOD. Septo-optic dysplasia (SOD) is a rare congenital malformation syndrome characterised by a triad of optic nerve hypoplasia, midbrain abnormality and pituitary dysfunction. Case Report 19 day-old girl baby presented with lethargy, poor feeding and poor weight gain. On examination, she was hypothermic, hypoglycaemic, hyperkalaemia with early shock and metabolic acidosis. She was treated as presumed sepsis with dextrose, fluid bolus and antibiotics. She had persistent metabolic acidosis with normal anion gap despite on full feeds. Her septic screening and initial metabolic screening were negative. She was noted to have adrenal insufficiency (cortisol Conclusion Clinical followed by hormonal evaluation and imaging can guide us to its appropriate diagnosis and treatment of SOD. Early recognition of SOD is paramount in order to reduce morbidity and to optimise management. Without adequate treatment, panhypopituitism can have devastating effects on growth and neurodevelopment of children.