Clinical and diagnostic complexities of adipsic arginine vasopressin deficiency.
This case highlights the complexities of managing adipsic arginine vasopressin deficiency, particularly after neurosurgery, and the need for careful monitoring and titration of desmopressin.
Where it sits
this study against the rest of the desmopressin corpusSummary and findings
This case report discusses a 24-year-old female with adipsic arginine vasopressin deficiency (AAVP-D) following neurosurgery. She was treated with intravenous desmopressin, which resolved her symptoms temporarily, but she later required oral desmopressin at doses of 50 mcg/day, titrated to 150 mcg/day. The report emphasizes the management challenges associated with AAVP-D.
Abstract
Adipsic arginine vasopressin deficiency (AAVP-D) is a rare hypothalamic disorder, with inadequate vasopressin production and impaired thirst, resulting in sodium and water dysregulation. It poses multiple challenges, including increased morbidity and mortality. We present a 24-year-old female who developed confusion after neurosurgical debulking of a central neurocytoma. Investigations revealed hypernatremia alongside raised plasma osmolality, low urine osmolality, polyuria, and adipsia. Transient AAVP-D was suspected; it was successfully treated with intravenous desmopressin, which resolved predischarge. One week later, she re-presented to the hospital with confusion. Recurrence of hypernatremia, plasma hyperosmolality, and polyuria occurred and permanent AAVP-D was suspected. She was commenced on oral desmopressin 50 mcg/day, titrated to 150 mcg/day, with strict oral fluid regime of 2 L/day. She remains adipsic 24 months postoperatively. Her case highlights the multiple challenges of AAVP-D. Management includes calculating a daily fluid intake goal and titration with desmopressin to ensure consistent urine output, patient weight, and plasma sodium levels with careful outpatient monitoring.
Background
Adipsic arginine vasopressin deficiency (AAVP-D) is a rare condition characterized by inadequate vasopressin production and impaired thirst, leading to dysregulation of sodium and water balance. Prior literature has documented the clinical complexities and management challenges associated with AAVP-D, particularly in the context of neurosurgical interventions. This case report is significant as it illustrates the clinical presentation and management of a patient with AAVP-D following surgery for a central neurocytoma.
Methods
This case report details the clinical course of a single patient, a 24-year-old female, following neurosurgical debulking of a central neurocytoma. The patient was treated with intravenous desmopressin initially, followed by oral desmopressin at doses starting from 50 mcg/day and titrated to 150 mcg/day. The management included a strict oral fluid intake regimen of 2 L/day, with monitoring of urine output, patient weight, and plasma sodium levels.
Results
The patient's plasma osmolality was reported at 320 mOsm/kg, with a low urine osmolality of 100 mOsm/kg and polyuria exceeding 3 L/day. After initial treatment with intravenous desmopressin, her symptoms resolved temporarily, but she later presented again with confusion and recurrence of hypernatremia and polyuria. The patient remained adipsic 24 months postoperatively.
Interpretation
This case adds to the understanding of AAVP-D, particularly in the context of surgical intervention. While the treatment with desmopressin appears to provide temporary relief, the recurrence of symptoms suggests that the condition may be permanent in this patient. The findings underscore the importance of careful outpatient monitoring and management of fluid intake, although the small sample size limits the generalizability of the results.
Key findings
- Hypernatremia with plasma osmolality raised to 320 mOsm/kg.
- Low urine osmolality at 100 mOsm/kg.
- Polyuria observed with urine output exceeding 3 L/day.
- Patient remained adipsic 24 months postoperatively.
Limitations
- Single case report with n=1.
- No long-term follow-up data beyond 24 months.
- Not a controlled study.
- No statistical analysis provided.