Improvement in post-hemispherotomy cerebral salt-wasting syndrome following intubation: A case report

Cerebral salt-wasting syndrome (CSWS) is characterized by renal sodium loss following intracranial disorders, leading to hyponatremia, reduced extracellular volume, and dehydration. The mechanisms underlying CSWS remain unclear. To date, no reports have described the coexistence of arginine vasopressin deficiency (AVPD), also known as central diabetes insipidus (cDI), and CSWS following hemispherotomy. We report a case of cDI and CSWS occurring after hemispherotomy, in which CSWS improved following intubation. A 7-month-old girl with right hemimegalencephaly and cortical dysplasia underwent hemispherotomy. On postoperative day (POD) 1, AVPD was diagnosed and treated with intravenous AVP. By POD 3, she developed CSWS, characterized by increased urinary sodium excretion, decreased serum sodium levels, dehydration, polyuria, and negative fluid balance. Notably, CSWS improved markedly after intubation on POD 5. However, within one day of extubation, CSWS recurred on POD 8. Her condition gradually improved between POD 8 and POD 14. She is currently clinically stable, with her AVPD well-controlled. Conclusion: AVPD and CSWS can co-occur after hemispherotomy, even without hypothalamic-pituitary involvement. The improvement and recurrence of CSWS associated with intubation and extubation suggest that positive end-expiratory pressure may represent a novel therapeutic strategy for CSWS.