IN THE NAME OF GOD. 15 y. CC: Abdominal Pain+Primary Amenorrea PMH: (-) FH: Her sister had gross Hematuria during menstural cycle. PH/EX : NL Diagnosis: Sonography UT=NL. ET=2mm. 7×5 cm. mass. Treatment: Surgery. 3×4 cm. Ovaries:NL.
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CC: Abdominal Pain+Primary
FH: Her sister had gross Hematuria during menstural cycle.
Diagnosis: Sonography UT=NL
Fig.1. Unilateral obstruction of a horn (A) and the vagina (B). When there is failure of lateral fusion of the müllerian ducts with unilateral obstruction, absence of the ipsilateral kidney is the rule. Thus, an intravenous pyelogram is an essential diagnostic tool and may clarify the diagnosis in obscured circumstances.(Rock JA: Diagnosing and repairing uterine anomalies. Contemp Obstet
Fig. 2. Double uterus, complete or incomplete vaginal obstruction, and ipsilateral renal agenesis. A. Complete vaginal obstruction. B. Incomplete vaginal obstruction. C. Complete vaginal obstruction with a lateral communicating double uterus.(Rock JA, Jones HW Jr: The double uterus associated with an obstructed hemivagina and ipsilateral renal agenesis. Am J Obstet Gynecol 138:340, 1980)
Amenorrhea (absence of menses) is often classified as either primary (absence of menarche by age 16) orsecondary (absence of menses for more than three cycle intervals or six months in women who were previously menstruating).
Primary amenorrhea is defined as the absence of menses at age 16 in the presence of normal growth and secondary sexual characteristics. At age 13, if no menses have occurred and there is an absence of secondary sexual characteristics, such as breast developmentevaluation for primary amenorrhea should be begun.
The American Fertility Society
The remaining 5 % of cases are due to a combination of disorders including androgen insensitivity due to mutations in the androgen receptor, congenital adrenal hyperplasia, and polycystic ovary syndrome.
Pelvic or lower abdominal pain is a common presenting symptom in girls with primary amenorrhea and an obstructed reproductive tract.
An imperforate hymen is the simplest defect that results in primary amenorrhea. It may be associated with cyclic pelvic pain and a perirectal mass from sequestration of blood in the vagina (hematocolpos). Similar findings can be seen with defects in perineal development, which can result in absence of the distal third of the vagina and therefore absence of an outflow tract. Both of these conditions are diagnosed by physical examination. An imperforate hymen is easily corrected with surgery.
Fig. 5 Imperforate hymen. Notice the thin, transparent hymen stretched over the dark-colored accumulated menstrual blood.
One or more transverse vaginal septae can occur at any level between the hymenal ring and the cervix.
Vaginal agenesis is usually accompanied by cervical and uterine agenesis.
Primary amenorrhea is evaluated most efficiently by focusing on the presence or absence of breast development (a marker of estrogen action and therefore function of the ovary), the presence or absence of the uterus (as determined by ultrasound, or in more complex cases by magnetic resonance imaging) and the FSH level.
Step III: Basic laboratory testing
Treatment of primary amenorrhea is directed at correcting the underlying pathology.
Classification of Müllerian anomalies according to the AFS classification system
A. Vaginal (uterus may be normal or exhibit a variety of malformations)
B. Cervical C. Fundal D. Tubal
A1a. Communicating (endometrial cavity present)
A1b. Noncommunicating (endometrial cavity present)
A2. Horn without endometrial cavity
B. No rudimentary horn
Type III: Uterus didelphys Type IV: Uterus bicornuate:A. Complete (division down to internal os)B. PartialC. ArcuateType V: Septate uterus: A. Complete (septum to internal os)B. PartialType VI: Diethylstibestrol-related anomalies: A. T-shaped uterusB. T-shaped with dilated horns
Uterine didelphys: Uterine didelphys, or double uterus, occurs when the two müllerian ducts fail to fuse, thus producing duplication of the reproductive structures.
Associated renal anomalies : Renal anomalies are found in 20 to 30 percent of women with müllerian defects.
The signs and symptoms associated with müllerian anomalies vary greatly, depending upon the defect involved.
Longitudinal septa are typically associated with uterine anomalies, such as septate uterus and uterus didelphys.
Treatment:Treatment involves complete removal of the
septum. Excision is the traditional procedure with care to avoid
compromise to the bladder and rectum. The septal tissue should be
excised in total as retained fragments of septum may cause
dyspareunia. The septal tissue is resected and then the normal vaginal mucosas from each vagina are sutured together over the defect created by the resection. Surgery is not required in
asymptomatic women with a longitudinal vaginal septum, but will
facilitate vaginal delivery. Dr jerii 2007
An obstructed hemi-vagina is usually associated with ipsilateral renal agenesis.
Treatment: Women with an obstructed hemi-vagina require
surgical correction due to pain, increased risk of infection, and
retrograde menses. The obstructed vagina is entered, the fluid is
drained, and then the vaginal tissue between the two vaginas is
resected. Great care is taken to avoid the bladder, rectum and the
some surgeons prefer to drain the
obstructed vagina by the creation of a "window" with a second
operation performed at a later time after the inflammation and
distention have resolved.