SUN-004 - Inpatient Stabilization and Management of Acute Exacerbation of Craniopharyngioma Associated Adipsic Diabetes Insipidus

Background: Diabetes insipidus (DI) is a known endocrine complication of craniopharyngioma before and after surgical resection and treatment. Standard management includes desmopressin and thirst-guided fluid intake to maintain normonatremia. (1) Adipsic diabetes insipidus (ADI) is a rare subtype characterized by loss of thirst response, placing patients at high risk for severe dysnatremias. (2) Published guidance on management of ADI exacerbations refractory to fixed desmopressin dosing is limited.

Clinical

Case: An 18-year-old woman with adamantinomatous craniopharyngioma status post resection, radiation therapy, ventriculoperitoneal shunt dependence, panhypopituitarism, ADI and type 2 diabetes mellitus, was admitted with fever, severe hypernatremia (Na 180 mEq/L), hyperosmolality (serum 394 mOsm/kg, urine 884 mOsm/kg), altered mental status, and acute kidney injury (sCr 1.9 mg/dL, eGFR 39). She was treated with stress-dose hydrocortisone, intravenous fluids, and antibiotics. Hypernatremia was slowly corrected to reduce the risk of cerebral edema. After initial stabilization, sodium hemostasis proved very challenging with frequent fluctuations in sodium levels despite strict fluid intake-output (I&O) monitoring. Her preadmission desmopressin regimen required multiple titrations and ultimately escalation of baseline dose with intermittent breakthrough dosing. The addition of fludrocortisone was more effective for sodium regulation and fluid balance than thiazide diuretic titration. Strict intake and output monitoring, daily weight checks and scheduled oral hydration reminders were required due to adipsia, baseline cognitive impairment, and hyperphagia. Maintenance hydrocortisone dose was also increased. After a 28-day hospitalization, she was discharged on revised medication regimens, a fixed daily fluid intake of 1.5–2 L and daily weight checks. She remains at home with nursing and caregiver support, frequent outpatient follow-up, and weekly laboratory monitoring.

Conclusion: This case highlights the complexity of inpatient stabilization of ADI, requiring cautious sodium correction, individualized desmopressin titration, fixed fluid prescriptions, and adjunctive therapies. ADI patients with hyperphagia also need weight checks as strict I&O monitoring alone can sometimes be unreliable in assessing fluid status. Careful titration of hormonal replacement and hydration was essential for achieving sodium control and safe discharge.

References

1. Flynn K et al. Front Endocrinol. 2024;15:1479764.
2. Dalan R et al. Front Endocrinol. 2019;10:630.

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