Reconstruction of anatomofunctional in a patient with short urethra, genital dysgenesis, and total incontinence with Turner syndrome in mosaic

Hugo Rivera-Astorga, Department of Urology, Hospital General de México, Dr Eduardo Liceaga, Secretary of Health, Mexico City, Mexico
Jorge A. Jaspersen-Gastelum, Department of Urology, Hospital General de México, Dr Eduardo Liceaga, Secretary of Health, Mexico City, Mexico
Yahir Cortes-Patiño, Department of Urology, Hospital General de México, Dr Eduardo Liceaga, Secretary of Health, Mexico City, Mexico
José F. Virgen-Gutiérrez, Department of Urology, Hospital General de México, Dr Eduardo Liceaga, Secretary of Health, Mexico City, Mexico
Yuki F. Nagano-Palacios, Department of Urology, Hospital General de México, Dr Eduardo Liceaga, Secretary of Health, Mexico City, Mexico
Fabiola A. Ayala-Meléndez, Department of Urology, Hospital General de México, Dr Eduardo Liceaga, Secretary of Health, Mexico City, Mexico
Alexa P. Aquino-Reyes, Department of Urology, Hospital General de México, Dr Eduardo Liceaga, Secretary of Health, Mexico City, Mexico
Marcelo Robles-Rodríguez, Department of Urology, Hospital General de México, Dr Eduardo Liceaga, Secretary of Health, Mexico City, Mexico

The case of a 34-year-old female patient with Turner syndrome (TS) mosaicism (45X, 46XX) and severe urogenital malformations is presented. These malformations include clitoral agenesis, hypoplasia of the labia minora, and a short, patulous urethra that resulted in total urinary incontinence. After a multidisciplinary evaluation and a series of advanced surgical interventions, a significant improvement in urinary function and the patient’s quality of life was achieved. This case highlights the clinical and surgical complexity associated with TS mosaicism, a condition that can present atypical and severe manifestations in the urogenital tract. Congenital short patulous urethra in women is a rare condition that delays diagnosis and treatment. Clinical identification, such as total incontinence, is a key sign for precise diagnosis and detailed surgical planning, essential for the effective management of patients with congenital anomalies. Advanced surgical techniques, such as subtrigonal sling placement and mobilization of vaginal wall flaps, have proven to be effective in the treatment of stress urinary incontinence, significantly improving quality of life.

Keywords: Turner syndrome mosaicism. Urogenital malformations. Urinary incontinence. Genitourinary reconstruction. Subtrigonal sling. Congenital anomalies of the urinary tract.