Series of clinical cases demonstrates functional state of lower urinary tract in girls with disorders of sex development (DSD) and hypospadias after the first stage of feminization. The study included 27 girls and women with DSD with hypospadias. Most of them have congenital adrenal hyperplasia (24), fewer girls have partial gonadal dysgenesis (1) and idiopathic virilization (2). Patients were examined before second stage surgical feminization in 115 years after the first stage. Concomitant pathology of the urogenital tract was detected in 19 (70%) patients. Urinary tract infection (UTI) was verified in 13 (48%), bladder dysfunction (BD) was diagnosed in 7 (26%), trapped menstrual secretions presented as hematometra, hematocolpos, and urine accumulation and stagnation in the vagina in anamnesis or as a result of preoperative studies were diagnosed in 9 (33%). Combination of the listed complications were observed in five patients (14%). Results of second stage of feminization confirmed connection of hypospadias with listed complications. This were detected in 11 (69%) patients after introitoplasty without separation of urinary and genital tracts (UGT). Introitoplasty with separation of UGT and elimination of hypospadias was complicated only four patients (36%), herewith the UTI and BD were eliminated. Hypospadias in girls with DSD is risk of development such complication as urinary tract infection, trapped menstrual secretions and bladder dysfunction. This circumstance requires change in surgical feminization tactics in girls with DSD, taking into account the anatomical components of genitalia malformations.
Introduction. Currently, the application of ultrasonic energy for tissue dissection is a routine practice in surgery. Its effectiveness and safety has been proven in proctology, pediatric coloproctology, surgeries in the endocrine organs and in the abdominal organs.Material and methods. Given the features of the endocrine system, the anatomy of virilized genitalia and positive experience in applying ultrasonic energy for surgical interventions, the authors present a comparative trial on the effectiveness of ultrasonic knife (USK) and traditional electrocoagulation in vaginoplasty in patients with disorders of sex development.Results. The obtained findings demonstrate that USK in comparison to traditional electrocoagulation significantly reduces vaginoplasty duration by 30%: median and quartiles of this index with USK were 57.5 [50; 65] min, while in the electrocoagulation group – 82.5 [75; 90] min. Difference is statistically significant (U-test 83.5, p <0.001) too. Difference between medians and its 95% confidence interval was 25 [20; 35] min.Conclusion. USK has better effects due to less tissue bleeding and, as a consequence, better visualization of the surgical field. An additional advantage is a unique ability to gently mobilize and preserve anatomical structures of the pelvis.
The clinical case of a rare variant of disorder of sex development (DSD) is described. This disorder was diagnosed with an emergency operation for the traumatic rupture of the gonad. A patient (14 years old) with a male phenotype and lack of muller duct derivatives had a female SRY negative karyotype (46,XX) and an ovotesticular gonad structure as a result of duplication in the regulatory zone of the SOX9 gene. Ovotesticular disorders of sex development with karyotype 46,XX, as a rule, are accompanied by an ambiguous genitalia and derivatives of the muller structures. Early diagnosis of the described variant of DSD was difficult due to the development of male type genitalia. Timely identification of DSD including the presented option of DSD, is possible during routine examinations of the urologist with mandatory ultrasound examination of the scrotum and pelvis.
In his clinical lecture on visceral syphilis (Kaz. Med. J., 1925, No. 4) prof. R. Luria says: The possibility of syphilis affecting internal organs was considered universally recognized in the 17th century. However, - such is the power of routine and medical tradition, - the idea of the possibility of visceral syphilis is still not clearly understood, and not only by patients who turn almost exclusively to venereologists about their former infection, but also by the wide masses of doctors. "
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