Partial Central Diabetes Insipidus Following Endonasal Infrasellar Clival Chordoma Resection in Pediatric Patients: A Case Report and Review

Background: A 14-year-old male with recurrent clival chordoma, an infrasellar mass, underwent a repeat endonasal resection that was complicated by a CSF leak secondary to puncture of the dura and arachnoid near the posteroinferior pituitary gland, and postoperative DI that was initially thought to be transient however, recurred 7 days after cessation of DDAVP therapy. Our objective is to highlight a case of endonasal resection of clival chordoma which resulted in permanent, but partial, central DI in the pediatric setting. Clinical Presentation: In this patient, his CDI initially responded to desmopressin in the inpatient setting, which contributed to the premature conclusion that his CDI would remain transient. However, upon endocrinology follow-up, he continued to have severe thirst and increased urination and work up also included a serum osmolality of 294 MIU/KG, serum sodium of 142 mmol/L, urine specific gravity of 1.008, and urine osmolality of 339 MIU/KG, consistent with a partial central DI. Conclusion: CDI is a known complication of suprasellar masses as well as endonasal resection of these masses, however, this is poorly described in the literature surrounding pediatric patients being treated for infrasellar clival chordomas. This patient’s recurrence of partial CDI indicates a potential gap in care for patients who undergo endonasal infrasellar clival chordoma resection, as they may be underdiagnosed with permanent partial DI. We propose that these patients should be closely followed by an endocrinologist to screen for possible pituitary complications and that increased data should be collected in the pediatric setting regarding postoperative outcomes.