Amenorrhea resident survival guide

Osteoporosis Resident Survival Guide Microchapters
Overview
Classification
Causes
FIRE
Diagnosis
Treatment
Do's
Don'ts

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Eiman Ghaffarpasand, M.D. [2]

Overview

The first descriptions about disturbances in menstrual cycle are found in Papyrus Ebres [named after the Egyptologist Georg M. Ebers (1837-1898)], from New Kingdom period (1450-1550 B.C.E). Amenorrhea may be classified according to etiology into three subtypes, including primary amenorrhea, secondary amenorrhea, and functional amenorrhea. Primary amenorrhea is basically referred to a young girl that has not experienced menarche, at all. Secondary amenorrhea reflects a woman that has ordinary menstruation cycles, experiencing at least 3 months of menstruation cycle absence. Functional amenorrhea is a subtype of the amenorrhea caused by exaggerated different lifestyles. Distinguishing between primary and secondary amenorrhea is based on history, solely; because upon every cause of secondary amenorrhea can be a cause of primary amenorhea, indeed. Mainly the pathophysiology of amenorrhea is described in many categories, include hypothalamic, pituitary, thyroid, adrenal, ovarian, uterine, and vaginal pathogenesis. About 25 various genes, in 3 different group of Kallmann syndrome related genes, hypothalamus-pituitary-gonadal (HPG) axis related genes, and obesity related genes, play roles in amenorrhea. Common causes of amenorrhea include breastfeeding, pregnancy, menopause, and stress. Primary amenorrhea must be differentiated from other diseases that cause lack of menstrual cycle, such as Mullerian agenesis, 3-beta-hydroxysteroid dehydrogenase type 2 deficiency, androgen insensitivity syndrome, Kallmann syndrome, Turner syndrome, and 17-alpha-hydroxylase deficiency. In contrast, secondary amenorrhea must be differentiated from other diseases that cause menstrual cycle arrest, such as primary ovarian insufficiency, hypothyroidism, hyperprolactinemia, polycystic ovary syndrome, and Asherman's syndrome. The prevalence of amenorrhea is approximately 3,000 to 4,000 per 100,000 individuals worldwide. The most common risk factor in the development of primary amenorrhea is chromosomal disorder and the most common risk factor in the development of secondary amenorrhea is breastfeeding. If left untreated, all of patients with amenorrhea may progress to develop infertility and osteoporosis. Common complications of amenorrhea are based on the background disease that induced it. Prognosis is generally excellent and the mortality rate of patients with amenorrhea is approximately less than 1%, generally in brain lesions. The most first laboratory tests for evaluating amenorrhea are pregnancy test, thyroid stimulating hormone (TSH), follicle stimulating hormone (FSH), and prolactin (PRL). Second line laboratory tests include free and total testosterone, dehydroepiandrosterone sulfate (DHEAS), and also progesterone challenge test. There are no echocardiography findings associated with amenorrhea. However, an echocardiography may be helpful in the diagnosis of the diseases that can cause amenorrhea, such as Turner syndrome. Findings on an echocardiography suggestive of Turner syndrome include bicuspid aortic valve, elongation of transverse aortic arch, coarctation of aorta, and partial anomalous pulmonary venous return (PAPVR). There are no ultrasound findings associated with amenorrhea. However, an ultrasound may be helpful in the diagnosis of the diseases that can cause amenorrhea, such as polycystic ovary syndrome (PCOS), premature ovarian insufficiency, androgen insensitivity syndrome, 17-alpha hydroxylase deficiency, and also anatomic genital defects. Pharmacologic medical therapy is recommended among patients with hypothalamic causes, pituitary causes, ovarian insufficiency, and chronic anovulation. The general principle of the treatment in amenorrhea is sex hormones replacement therapy, mostly with suitable forms of estrogen and progesterone. The mainstay of treatment for amenorrhea is medical therapy. Surgery is usually reserved for patients with either hypothalamus or pituitary tumors, Turner syndrome, and genital anatomical defects (imperforate hymen or transverse vaginal septum). The surgical treatment for hypothalamus or pituitary tumors is tumor resection via endoscopic transsphenoidal surgery. Ovaries have to be excised in Turner syndrome to prevent malignant transformation. Small incision is the main surgery for imperforate hymen and septal excision is the main treatment for transverse vaginal septum.

Classification

Amenorrhea may be classified according to etiology into three subtypes:
- Primary amenorrhea
- Secondary amenorrhea
- Functional amenorrhea
Each of the subtypes of amenorrhea has their own classifications, as following: