Pregnancy complicated by multiple pituitary hormone deficiencies

Abstract

We report a case of pituitary dwarfism and diabetes insipidus due to pituitary stalk transection in a pregnant Japanese woman, 138 cm in height, born by breech delivery with no evidence of ante- or intrapartum asphyxia. The patient had no central nervous disturbance, was diagnosed with pituitary dwarfism during childhood and was treated at another hospital with growth hormone supplement from 5 to 14 years of age. This patient was referred to our department at 17 weeks' gestation due to a change of residence. At 30 weeks' gestation, she was hospitalized for assessment of hydronephrosis and polyuria (15-20 L/day). Analysis of a 24-h urine sample showed creatinine clearance of 157 mL/min and urine osmolality of 38 mOsm/L. The patient's urine output decreased after receiving a test dose of 0.75 g of 1-desamino-8-D-arginine vasopressin (DDAVP). Cranial magnetic resonance imaging showed transection of the pituitary stalk. Subsequently, the patient's urine output was well controlled by a maintenance dose of 0.275 mL/day intranasal DDAVP. A cesarean section was performed at 37 weeks, as the patient height was 138 cm, and a pelvic X-ray showed cephalopelvic disproportion. She delivered a female baby weighing 2302 g, and both 1- and 5-min Apgar scores were 9. The patient was followed up after 4 months and showed no visual deterioration or polyuria while on DDAVP therapy, while the neonate grew favorably.