Guideline1 min read·Published Mar 2023Older

Mullerian Anomalies

Guideline · Mar 2023 · 1 min read

In brief

Educational content addressing Mullerian anomalies, congenital malformations of the female reproductive tract resulting from abnormal development of the Mullerian ducts. Relevant for gynecologists and pediatric specialists managing patients with uterine, cervical, or vaginal structural abnormalities.

Müllerian anomalies include a spectrum from complete agenesis to duplicated structures (didelphys, bicornuate, septate uterus).
Unicornuate uterus may present with or without atrophic/functional uterine remnants requiring different management approaches.
Vaginal anomalies include longitudinal/transverse septa and obstructed hemivagina, which may cause menstrual obstruction.
Cervical duplication often accompanies uterine duplication and may be associated with vaginal septation.
Classification of Müllerian anomalies guides surgical planning and reproductive counseling for affected patients.

Written by the GCMD Library team from the guideline.

Müllerian Anomaly Classification Overview

This guideline presents a systematic classification of congenital müllerian duct anomalies affecting the fallopian tubes, uterus, cervix, and vagina. The classification system organizes anomalies by anatomic location and structural variation to guide clinical diagnosis and management. Understanding these developmental variations is essential for pediatric gynecologic and surgical care.

Fallopian Tube and Uterine Anomalies

Uterine anomalies range from complete müllerian agenesis (absence of uterus) to variations in uterine structure including didelphys (complete duplication), bicornuate (partial fusion), septate (internal division), and unicornuate (single-sided development). Each variant may present with or without functional or atrophic uterine remnants that can cause obstructive symptoms. Accurate classification impacts fertility counseling and surgical planning.

Cervical Variations

Cervical anatomy may present as a single typical cervix without septum or as duplicated cervix structures. Cervical duplication often accompanies uterine duplication anomalies and requires coordinated evaluation with uterine and vaginal anatomy. Recognition of cervical variants is critical for gynecologic examination and procedural planning.

Vaginal Anomalies and Obstructive Lesions

Vaginal anomalies include longitudinal septa (with or without obstructed hemivagina), transverse septa, and distal vaginal agenesis or atresia. Obstructive vaginal anomalies can cause hematocolpos, pain, and infection requiring urgent surgical intervention. Longitudinal septum with obstructed hemivagina is particularly associated with ipsilateral renal anomalies and requires comprehensive imaging evaluation.

Full text

MÜLLERIAN Anomalies Fallopian Tube Uterus Cervix V agina Fallopian tubes/Uterus Single uterus Müllerian agenesis Müllerian agenesis with atrophic and/ or functional uterine remnant Uterus didelphys Bicornuate uterus Complete septate uterus Unicornuate uterus Unicornuate uterus with atrophic uterine remnant Unicornuate uterus with functional uterine remnant For questions please contact the Colorectal Clinical T eam at: 202-476-2656 (COLO) | ColorectalNurse@childrensnational.org Typical

V agina Cervix MÜLLERIAN Anomalies Fallopian Tube Uterus Cervix V agina For questions please contact the Colorectal Clinical T eam at: 202-476-2656 (COLO) | ColorectalNurse@childrensnational.org Single cervix No septum Duplicated cervix Longitudinal septum Transverse septum Distal vaginal agenesis (atresia) Longitudinal septum with obstructed hemivagina Typical

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