Oxytocin Deficiency and Spontaneous onset of Labor and Lactation in Langerhans Cell Histiocytosis

ABSTRACT Objective: We report a case of spontaneous vaginal delivery and lactation in a female with central diabetes insipidus and suspected oxytocin deficiency secondary to childhood Langerhans cell histiocytosis (LCH) involving the pituitary stalk. Previous case reports describe spontaneous vaginal delivery in females with panhypopituitarism without ensuing lactation. Our case is the first to report successful spontaneous vaginal delivery and lactation in a female with posterior pituitary dysfunction secondary to LCH. Methods: A 27-year-old gravida 1, para 0 woman presented with a planned natural pregnancy. She developed diabetes insipidus at age 9. Magnetic resonance imaging demonstrated diffuse thickening of the midline infundibular pituitary stalk suggestive of an infiltrative process, most likely LCH. She was treated with 6 months of vinblastine, 6-mercaptopurine, and prednisone with normalization of pituitary stalk appearance. The diabetes insipidus persisted, requiring long-term desmopressin. She was presumed to have oxytocin deficiency given persistent diabetes insipidus and magnetic resonance imaging documentation of pituitary stalk involvement by LCH. Results: The patient had a spontaneous vaginal delivery at 39 weeks and 4 days. No exogenous oxytocin was required throughout parturition. Her desmopressin requirements remained unchanged throughout pregnancy and postpartum. She had colostrum at delivery and transitional milk without pharmacological intervention starting on postpartum day 4. Conclusion: Vasopressin and probable oxytocin deficiency from LCH involvement of the pituitary stalk in childhood may not prevent natural parturition or lactation. The case supports the growing literature that maternal pituitary oxytocin release may not be required for the onset and progression of labor and lactation in humans. Abbreviations: LCH = Langerhans cell histiocytosis;MRI = magnetic resonance imaging