An autopsy was performed on a 17-year-old girl after a fatal motor vehicle collision. Her ovaries appear small and underdeveloped on gross examination. Light microscopic examination shows ovaries primarily composed of connective tissue with no follicles. Which of the additional abnormalities is most likely to be found on postmortem examination?

Question

An autopsy was performed on a 17-year-old girl after a fatal motor vehicle collision.  Her ovaries appear small and underdeveloped on gross examination.  Light microscopic examination shows ovaries primarily composed of connective tissue with no follicles.  Which of the additional abnormalities is most likely to be found on postmortem examination?

Quizplus · Accepted Answer

11ec160b_375a_5215_9249_13fb03c44cd4_MD0001_00 The ovaries described in the vignette are the streak ovaries of Turner syndrome (TS). Patients with TS have normal ovarian development during fetal life. However, lack of the paternal X chromosome causes loss of ovarian follicles by age 2, as well as a karyotype of 45, X. At puberty, patients with TS usually have normal pubic hair but no breasts or menses due to ovarian failure. Premature ovarian failure is characterized by high LH and FSH levels, and TS is one of the most common causes. Patients with TS also have several extragonadal abnormalities. Short stature, shield chest (broad, with widely spaced nipples), and webbed neck (broad neck with low hairline) comprise the appearance of these patients. Lymphedema may be seen at any age, including neonates. Bicuspid aortic valve (20%-30%) is the most common cardiovascular anomaly; other common defects include coarctation of the aorta (3%-10%) and aortic root dilation with an increased risk of aortic dissection in adulthood. (Choice B) Pancreatic insufficiency occurs in patients with cystic fibrosis due to obstructed, inspissated pancreatic ducts. (Choices C and D) Mitral valve prolapse and lens displacement are seen in patients with Marfan syndrome. These patients are also at risk for aortic root dilation and aortic dissection. (Choice E) Vaginal adenosis is replacement of the vaginal squamous epithelium with glandular columnar epithelium. It occurs in the female children of women exposed to diethylstilbestrol (DES) during pregnancy. Vaginal adenosis is a precursor of clear cell adenocarcinoma of the vagina. Educational objective: Streak ovaries, amenorrhea, and infertility are the gynecologic complications of Turner syndrome (TS). In addition, patients with TS usually have short stature, webbed neck, shield chest, and low posterior hairline. Bicuspid aortic valve is the most common cardiac comorbidity. __________ References: Turner syndrome: diagnosis and management. (http://www.ncbi.nlm.nih.gov/pubmed/17708142) Aortic valve disease in Turner syndrome. (http://www.ncbi.nlm.nih.gov/pubmed/18466808)

An Autopsy Was Performed on a 17-Year-Old Girl After a Fatal