Pediatric prolactinoma-associated pituitary apoplexy presenting with acute visual loss: a case report, literature review, and management strategy

Abstract

Objectives Hemorrhagic pituitary apoplexy (PA) in children is exceedingly rare, with approximately 32 cases reported since 1980. Current guidelines recommend cabergoline as first-line therapy; however, acute complete visual loss mandates emergency surgical decompression. No guideline defines the optimal extent of resection, despite gross total resection carrying a 48-54 % risk of panhypopituitarism.Case presentation An 11-year-old prepubertal girl presented with a 2-day history of complete right-eye visual loss. Serum prolactin was 987 mu g/L, and MRI demonstrated a hemorrhagic sellar-suprasellar mass compressing the optic chiasm. Emergency endoscopic transsphenoidal surgery was performed with an intentionally limited strategy - hematoma evacuation, optic decompression, and partial debulking - deliberately avoiding aggressive capsular dissection. Histopathology confirmed prolactinoma. Vision was fully restored immediately after extubation, with near-complete visual field recovery on postoperative day 5 (VFI 98 %). Adrenocortical function was preserved. Central hypothyroidism developed, and levothyroxine was initiated. Cabergoline (0.25 mg/week) was started on postoperative day 5, achieving a 93.7 % prolactin reduction by day 20 (987 -> 61.9 mu g/L).Conclusions In pediatric prolactinoma-associated PA requiring emergency surgery, a decompression-first strategy prioritizing visual rescue and tissue diagnosis over gross total resection may reduce the risk of panhypopituitarism, with residual tumor subsequently managed with cabergoline. The rapid biochemical response documented here supports this approach, though multicenter prospective evaluation is required.