A 49 y.o. woman comes in with lower abdominal pain; she recently was treated for a UTI.

She has a history of hyperestrogenemia and has been treated with progesterone and testosterone for seven years.

Our patient had bilateral chronic hydrosalpinx and fibroids. Her hydrosalpinx on the R was large enough to obstruct her R kidney. She had evidence of chronic infarcts in the R kidney  which probably came from prior infections.  

Hydrosalpinx is a fluid filled fallopian tube.  While the cilia of the inner lining of the Fallloian tube beat towards the uterus to deliver the egg, the fluid is discharged from the fimbrieated end into the peritoneal cavity. The major cause of distal tube obstruction is PID although rarely, it is caused by tuberculosis.  Adhesions from surgery , endometriosis and cancer can also obstruct the tube.  By the time a hydrosalpinx is detected the fluid may be sterile but if the patient presents with pain it is recommended that antibiotics be given. Hydrosalpinx i found in 10-30% of couples being treated for infertility.

obstruction of the fimbria on the end of the tube causes scarring and fluid build up

CAUSES OF HYPERESTROGENEMIA

 Her hyperestrogenemia may have come from her chronic liver disease .  She was diagnosed with fatty liver/fibrosis on the CT scan.

In liver disease, damage to the liver impairs its capacity to metabolize and inactivate estrogens resulting in increased estrogen in the circulation. While liver disease is a common cause of excess estrogen, there are other causes as well including excess estrogen taken as hormone replacement, ovarian tumors , and genetic conditions such as aromatase excess syndrome(AEXS).

Aromatase is the enzyme responsible for the biosynthesis of estrogen from androgen. If there is overexpression of the enzyme, high estrogen levels result.  The effects depend on the type of mutation in the CYP1941 gene. It can be duplicated or partially deleted resulting in chimeric genes. In males AEXS can result in precocious puberty, gynecomastia and short final stature due to early epiphyseal closure. In women precocious puberty, gynecomastia and short final height can also result.  Fertility is not always significantly impaired enough to prevent sexual reproduction.

It has been hypothesized that Pharaoh Akhenaten (husband of Queen Nefertiti) may have suffered from aromatase excess syndrome in the 18th dynasty of Egypt.  All of his relatives including men and young girls were described as having breasts and wide hips.  Akhenaten himself was described as having a “beautiful and feminine voice”.

Akhenaten

Urology was consulted because of the hydronephrosis in our patient.  Her UA did not show infection and they felt a stent was not needed because the condition was chronic.  She was discharged on antibiotics.

Braverman I, Redford D, Mackowiak P. Akhenaten and the strange physiques of Egypt’s 18th dynasty. Ann Intern Med 2009 150(8):556-60

Shozu M, Fukami M, Ogata T.  Understanding the pathological manifestations of aromatase excess syndrome. Lessons for clinical diagnosis. 2014. Expert Review of Endocrinology & Metabolism. 9(4)397-409.

Ozmen B, Diedrich K, Al-Hasani S. Hydrosalpinx and IVF. Assessment of treatments implemented prior to IVF. Reprod Biomed Online 2007 Feb 1492):235-41.