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Special Feature
July 01, 2004

Image of the Month—Diagnosis

Author Affiliations
 

S. ROZYCKIGRACEMD

Arch Surg. 2004;139(7):798. doi:10.1001/archsurg.139.7.798
Answer: All of the Above

Figure 1. Groin mass containing the right ovary and fallopian tube.

Figure 2. Right ovary and fallopian tube dissected free. Notice the structures lying above the defect in the external oblique aponeurosis. The ruler is in centimeters.

Mayer-Rokitansky-Küster-Hauser syndrome, or complete müllerian agenesis, is a congenital disorder first described by Mayer in 1829.1It is thought to be secondary to an absence or incomplete canalization of the vaginal plate2and does not appear to represent a single genetic defect. Patients classically present with primary amenorrhea in adolescence and are found to have an absent or a hypoplastic vagina on results of physical examination. Subsequent work-up demonstrates an absent or rudimentary uterus, most commonly with normal ovarian function. In this case, diagnostic laparoscopy at the time of initial operation demonstrated absence of a uterus, as well as the left ovary and fallopian tube adherent to the internal ring. Results of a pelvic examination showed a blind-ended, shortened vagina and absence of the cervix and uterus. Patients have a normal 46,XX karyotype, with normal secondary sexual characteristics. Their only outward manifestation of a problem is lack of menses—prompting them to seek medical attention. Up to 70% of patients will also have a renal abnormality, with one third having a major defect that includes unilateral renal agenesis.3Our patient had normal results of a renal work-up that included ultrasonography and intravenous pyelography. Skeletal abnormalities, including spine, limb, and rib defects, are present in approximately 12% of cases.3Vaginal dilation is the mainstay of nonoperative management and involves the use of sequential dilation of the vaginal remnant over time. Surgical therapy involves vaginoplasty with neoconstruction using small bowel, free skin grafts, sigmoid colon, or rotational flaps.2

This case serves to reinforce the notion that a complete preoperative history and physical examination should contain a menstrual and sexual history in all women of appropriate age. In addition, a pelvic examination should be performed when indicated. When an ovary or a fallopian tube is found in a hernia sac, it is imperative to complete a full gynecological examination to ensure that no other abnormalities are present. Finally, because of the embryologic developmental association of the renal and gynecological systems, a screening examination such as ultrasonography or intravenous pyelography should be performed in all such cases.

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Due to the overwhelmingly positive response to the "Image of the Month," the Archives of Surgeryhas temporarily discontinued accepting submissions for this feature. It is anticipated that requests for submissions will resume in mid 2004. Thank you.

Correspondence: Scott R. Steele, MD (docsteele@hotmail.com).

References
1.
Mayer  R Uber Verdoppelungen des Uterus and ihre Arten, nebst Bemerkungen uber Hasenscharte und Wolfsrachren. J Chir Auger. 1829;13525- 564
2.
Hensle  TWChang  DT Vaginal reconstruction. Urol Clin North Am. 1999;2639- 47vii.
PubMedArticle
3.
Pletcher  JRSlap  GB Menstrual disorders: amenorrhea. Pediatr Clin North Am. 1999;46505- 518
PubMedArticle
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