(1681) Germinomatous Germ Cell Tumor With Panhypopituitarism-Associated Shock Requiring ECMO

Introduction: Shock is one of the most common conditions managed in the pediatric ICU, but unusual forms may present diagnostic challenges. Hypopituitarism with pituitary crisis is a rare form of shock that manifested in our patient with a new suprasellar mass.

Description: A 9-year-old girl with history of growth failure presented in undifferentiated shock with profound hypotension, obtundation, hypothermia and electrolyte derangements most notable for hypernatremia to 180 mmol/L. She underwent a CT brain which demonstrated a cystic suprasellar mass with obstructive hydrocephalus. Initial suspicion was for hypovolemic shock due to uncontrolled diabetes insipidus. Management included large volume fluid resuscitation, multiple vasoactive medications and stress-dose hydrocortisone, with ongoing difficulty maintaining adequate organ perfusion and obtundation requiring intubation. Despite escalation of vasoactive agents, she required cannulation to VA-ECMO for shock refractory to maximal medical management. Given her history of growth failure and diabetes insipidus, pituitary markers were collected and reflected panhypopituitarism with central DI, adrenal insufficiency, hypothyroidism and growth hormone deficiency. An external ventricular drain was placed and demonstrated elevated opening pressure of approximately 30mmHg. Despite correction of coagulopathy, she developed a small intracranial track hemorrhage which precluded systemic anticoagulation on ECMO. Infusions of Triiodothyronine and Angiotensin II were added to shock-dose Vasopressin and Epinephrine with improvement in her hemodynamics enabling her successful decannulation from extracorporeal support. She underwent complete resection of her mass, identified by pathology as a germinomatous germ cell tumor, and is neurologically returned to baseline.

Discussion: The concurrence of hypovolemic shock was initially masked by the underlying pituitary crisis in this patient. Persistence of shock after large volume resuscitation for severe diabetes insipidus should prompt evaluation for further etiologies. Lack of secretory hormone function due to a suprasellar intracranial mass was the cause of refractory shock in this patient and initiation of hormone replacement therapy resulted in resolution of pituitary crisis.