Affiliations: Department of Reproductive Endocrinology and
Fertility, Palo Alto Medical Foundation, Fremont, CA, USA | Department of Reproductive Endocrinology and
Infertility, Stanford University Hospital, Stanford, CA, USA | Department of Pediatric Endocrinology, Children's
Hospitals and Clinics of Minnesota, Paul, MN, USA
Note: [] Corresponding author: Dr. Sunny H. Jun, Department of
Reproductive Endocrinology and Fertility, Palo Alto Medical Foundation,
Fremont, CA, USA. Tel.: +1 510 498 2876; Fax: +1 510 498 2133; E-mail:
juns@pamf.org
Abstract: In here, we describe a case of primary amenorrhea in the setting of
chronic hydrocephalus caused by a posterior fossa ependymoma. A 17-year-old
female with primary amenorrhea presented to University-affiliated teaching
hospital. Hormonal studies were all normal. Cranial magnetic resonance imaging
revealed chronic hydrocephalus with a 4 cm brain lesion that was determined to
be an ependymoma. After surgical resection, the patient had normal menstrual
cycles. Primary amenorrhea in association with hydrocephalus is usually due to
hypothalamic hypogonadism. We report a case of an adolescent female with normal
gonadotropin levels and chronic hydrocephalus who presented with primary
amenorrhea. Even in the presence of normal hormonal studies and withdrawal
bleeding after a progestational challenge, a head magnetic resonance imaging
should be performed in all females who do not have hypergonadotropic
amenorrhea.