Differential diagnosis and management tactics in patients with a rare developmental Müllerian duct anomaly ACUM and a rudimentary uterine horn at early reproductive age

Khashchenko E.P., Allakhverdieva E.Z., Arakelyan A.S., Uvarova E.V., Chuprynin V.D., Kulabukhova E.A., Luzhina I.A., Uchevatkina P.V., Mamedova F.Sh., Asaturova A.V.

1) Academician V.I. Kulakov National Medical Research Center of Obstetrics, Gynecology, and Perinatology, Ministry of Health of the Russian Federation, Moscow, Russian Federation; 2) M.V. Lomonosov Moscow State University, Moscow, Russian Federation; 3) I.M. Sechenov First Moscow State Medical University (Sechenov University) Ministry of Health of the Russian Federation, Moscow, Russian Federation; 4) Russian Medical Academy of Continuing Professional Education, Ministry of Health of the Russian Federation, Moscow, Russian Federation
Background. There has recently been a 10-fold increase in the detection rate of genital malformations in girls. Accessory and Cavitated Uterine Mass (ACUM) is one of the rare forms of developmental Müllerian duct anomaly, is more common in adolescents and is an isolated accessory uterine cavity in a normal uterus. The paper summarizes the currently available data on the clinical features and differential diagnosis of ACUM and a double uterus with a rudimentary uterine horn, as well as nodular adenomyosis. Due to the typical clinical presentation and the rarity of ACUM, the patients are often diagnosed with genital malformation and a complete uterine duplication in the presence of a functioning uterine rudiment or nodular adenomyosis. Some authors believe that ACUM and juvenile cystic adenomyoma are the same structure that results from gubernacular dysfunction. The differential instrumental diagnosis of ACUM is of great importance. The paper presents the main criteria for visualization of ACUM, uterine rudiment, and nodular adenomyosis according to pelvic ultrasound and MRI. An example of differential diagnosis and management is analyzed in a series of clinical cases in adolescent patients with developmental Müllerian duct anomaly (ACUM) and a rudimentary uterine horn.
Conclusion. The described clinical cases emphasize the importance of imaging methods in the differential diagnosis of ACUM and uterine rudiment, as well as the need for timely surgical intervention for mass removal and metroplasty.

Keywords

accessory and cavitated uterine mass (ACUM)
rudimentary uterine horn
cystic adenomyosis
adolescent girls
Müllerian duct anomalies
morphological pattern
laparoscopy

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Received 25.12.2020

Accepted 12.05.2021

About the Authors

Elena P. Khashchenko, PhD, Senior Researcher of the 2nd Gynecological (children and adolescent) Department, Academician V.I. Kulakov National Medical Research Сenter for Obstetrics, Gynecology and Perinatology, Ministry of Health of the Russian Federation. E-mail: khashchenko_elena@mail.ru. ORCID: 0000-0002-3195-307X.
4, Ac. Oparina str., Moscow, 117997, Russia.
Elvina Z. Allakhverdieva, student of Faculty of Fundamental Medicine, M.V. Lomonosov Moscow State University. E-mail: elya.elya2014@mail.ru.
ORCID: 0000-0002-7750-4675. 1, Leninskye Gory, Moscow, 119991, Russia.
Alek S. Arakelyan, PhD, Senior Researcher of the Gynecological Department, V.I. Kulakov National Medical Research Center of Obstetrics, Gynecology and Perinatology, Ministry of Health of the Russian Federation. E-mail: а_arakelyan@oparina4.ru. ORCID: 0000-0003-3655-7938.
4, Ac. Oparina str., Moscow, 117997, Russia.
Elena V. Uvarova, Dr. Med. Sci., Professor, Corresponding Members of the RAS, Head of 2nd Gynecological (children and adolescent) Department, Academician V.I. Kulakov National Medical Research Сenter for Obstetrics, Gynecology and Perinatology, Ministry of Health of the Russian Federation; Professor of the Department of Obstetrics, Gynecology, Perinatology and Reproduction of the Institute of Professional Education, I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia (Sechenov University). E-mail: elena-uvarova@yandex.ru. ORCID: 0000-0002-3105-5640. 4, Ac. Oparina str., Moscow, 117997, Russia.
Vladimir D. Chuprynin, PhD, Head of General Surgery department, Academician V.I. Kulakov National Medical Research Сenter for Obstetrics, Gynecology and Perinatology, Ministry of Health of the Russian Federation. E-mail: v_chuprynin@oparina4.ru. 4, Ac. Oparina str., Moscow, 117997, Russia.
Elena A. Kulabukhova, PhD, radiologist at the Department of Radiation Diagnostics, Academician V.I. Kulakov National Medical Research Сenter for Obstetrics, Gynecology and Perinatology, Ministry of Health of the Russian Federation. E-mail: e_kulabukhova@oparina4.ru. 4, Ac. Oparina str., Moscow, 117997, Russia.
Irina A. Luzhina, radiologist at the Department of Radiation Diagnostics, Academician V.I. Kulakov National Medical Research Сenter for Obstetrics, Gynecology and Perinatology, Ministry of Health of the Russian Federation. E-mail: i_luzhina@oparina4.ru. 4, Ac. Oparina str., Moscow, 117997, Russia.
Polina V. Uchevatkina, Doctor of Department of Radiation Diagnostics, Academician V.I. Kulakov National Medical Research Сenter for Obstetrics, Gynecology and Perinatology, Ministry of Health of the Russian Federation. E-mail: p_ uchevatkina@oparina4.ru. 4, Ac. Oparina str., Moscow, 117997, Russia.
Fatima Sh. Mamedova, PhD, Doctor of Department of Ultrasound Diagnostics in Neonatology and Pediatrics, Academician V.I. Kulakov National Medical Research Сenter for Obstetrics, Gynecology and Perinatology, Ministry of Health of the Russian Federation; Assistant, Department of Radiation Diagnostics of Childhood, Russian Medical Academy of Continuing Professional Education, Ministry of Health of the Russian Federation. E-mail: mamedova_f@mail.ru. 4, Ac. Oparina str., Moscow, 117997, Russia.
Aleksandra V. Asaturova, PhD, Head of 1th Pathology Department, Academician V.I. Kulakov National Medical Research Сenter for Obstetrics, Gynecology and Perinatology, Ministry of Health of the Russian Federation. E-mail: a_asaturova@oparina4.ru. ORCID: 0000-0001-8739-5209. 4, Ac. Oparina str., Moscow, 117997, Russia.

For citation: Khashchenko E.P., Allakhverdieva E.Z., Arakelyan A.S., Uvarova E.V., Chuprynin V.D., Kulabukhova E.A., Luzhina I.A., Uchevatkina P.V., Mamedova F.Sh., Asaturova A.V. Differential diagnosis and management tactics in patients with a rare developmental Müllerian duct anomaly ACUM and a rudimentary uterine horn at early reproductive age.
Akusherstvo i Ginekologiya/Obstetrics and Gynecology. 2021; 6: 156-167 (in Russian)
https://dx.doi.org/10.18565/aig.2021.6.156-167

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