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Indirect inguinal hernia with uterine tissue in a male: A case of persistent Mullerian duct syndrome and literature review.

ABSTRACT: A 32-year-old male presenting with a direct inguinal hernia was found to have uterine tissue extending through the inguinal canal, warranting a diagnosis of persistent Mullerian duct syndrome (PMDS). PMDS is an extremely rare form of internal male pseudo-hermaphroditism in which female internal sex organs, including the uterus, cervix and proximal vagina, persist in a 46XY male with normal external genitalia. The condition results from a congenital insensitivity to anti-Mullerian hormone, or lack of anti-Mullerian hormone, leading to persistence of the female internal sex organs in a male. Clinically, this condition is associated with cryptochoridism. Controversy persists regarding the appropriate treatment of PMDS, since resection of the remnant structures is associated with potential morbidity, but retention risks development of occasional malignancies. We review the literature and discuss various aspects of pathophysiology, diagnosis, and management of PMDS.

SUBMITTER: Ahdoot M

PROVIDER: S-EPMC3650826 | biostudies-other | 2013 Jan-Feb

REPOSITORIES: biostudies-other

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Indirect inguinal hernia with uterine tissue in a male: A case of persistent Mullerian duct syndrome and literature review.

Ahdoot Michael M Qadan Motaz M Santa-Maria Monica M Kennedy William A WA Ilano Aaron A

Canadian Urological Association journal = Journal de l'Association des urologues du Canada 20130101 1-2

A 32-year-old male presenting with a direct inguinal hernia was found to have uterine tissue extending through the inguinal canal, warranting a diagnosis of persistent Mullerian duct syndrome (PMDS). PMDS is an extremely rare form of internal male pseudo-hermaphroditism in which female internal sex organs, including the uterus, cervix and proximal vagina, persist in a 46XY male with normal external genitalia. The condition results from a congenital insensitivity to anti-Mullerian hormone, or lac ...[more]

PMID: 23671501

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Project description:ImportanceExfoliation syndrome (XFS) is a systemic connective tissue disease, and abnormal connective tissue metabolism is implicated in inguinal hernias (IH). Associating XFS with comorbid conditions may illuminate their underlying pathophysiology and affect clinical screening and treatment. Exfoliation syndrome involves altered systemic extracellular matrix (ECM) homeostasis involving elastin metabolism. Hernias occur owing to abnormal ECM synthesis, metabolism, or repair. Inguinal hernias involve weakening or rupture of the abdominal/groin wall.ObjectiveTo determine an association between patients with XFS and patients with IH in Utah, possibly differing between direct or indirect hernia.Design, setting, and participantsCross-sectional study in a large health care system of Utah hospitals and clinics. Conditional logistic regression odds ratios were used to estimate risk of XFS in patients with IH overall and by subtype (direct or indirect) compared with control individuals. Codes specific to direct and indirect IH with additional medical records review of 186 procedures were used to classify IH subtypes that were not prespecified. Bootstrap resampling with jackknife estimation used to calculate 95% confidence intervals. The model accounted for matching on sex and age and adjusted for body mass index and tobacco use. Population-based sample using medical records from 1996 to 2015 that identified 2594 patients 40 years or older on January 1, 1996, with surgical IH repair and 12 966 random control patients with no IH history matched 5:1 on sex and birth year. Data were analyzed between September 10, 2017, and October 23, 2017.Main outcomes and measuresExfoliation syndrome outcome defined by diagnosis codes for XFS or exfoliation glaucoma from 1996 to 2015.ResultsParticipants were primarily white (2532 of 2594 patients, [96.1%]; 12 454 of 12 966 control individuals [97.6%]) and non-Hispanic (2396 of 2594 patients [92.4%]); 250 participants were women (9.6%). Of study participants, 22 patients with IH and 43 control individuals were diagnosed as having XFS, respectively. Patients with IH had a 2.3-fold risk for an XFS diagnosis compared with control individuals (95% CI, 1.4-3.5; P = .03), and XFS risk with indirect IH appeared especially pronounced.Conclusions and relevanceInguinal hernia was associated with an increased risk of XFS in this Utah population. Further work is needed to understand the pathophysiology, genetics, and environmental factors contributing to both diseases.

Dataset Information

Indirect inguinal hernia with uterine tissue in a male: A case of persistent Mullerian duct syndrome and literature review.

Publications

Indirect inguinal hernia with uterine tissue in a male: A case of persistent Mullerian duct syndrome and literature review.

Similar Datasets

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