The Use of Urogenital Mobilization Extended by Two Flaps From the Redundant Distal Part of the Urethra in the Repair of the Severe Form of Distal Vaginal Agenesis.

OBJECTIVE: To describe and present the results of the use of urogenital mobilization extended by two flaps from the redundant distal part of the urethra in the correction of a severe form of distal vaginal agenesis. METHODS: During the past 4years (from December 2018 to March 2023), we have performed 2 vaginoplasties using the described procedure. The age of the patients at surgery was 13years. Two girls presented with primary amenorrhea associated with cyclic pelvic pain evolving over several months. Clinical examination revealed a blind vagina and an anterior pelvic mass. Ultrasound and magnetic resonance imaging showed a severe form of distal vaginal agenesis. One patient had a 47,XX+mark karyotype and right vesicoureteral reflux. Two patients underwent urogenital mobilization, guided by a tube introduced into the upper part of the vagina through an abdominal approach, and the use of two flaps from the redundant distal part of the urethra to extend the neovagina to the perineum. RESULTS: The esthetic results were very satisfactory with the disappearance of pelvic pain, and the restoration of a regular flow of menstrual blood without pain. Urinary continence was preserved in two patients. CONCLUSION: This is the first report of the use of urogenital mobilization extended two flaps from the redundant distal part of the urethra in the correction of a severe form of distal vaginal agenesis. This procedure yielded a very satisfactory esthetic and functional results.

Enlarged prostatic utricle and vagina masculinus in boys with disorders of sex development.

PURPOSE: To determine the prevalence, radiological characteristics, and clinical symptomatology of enlarged prostatic utricles and vagina masculinus in boys with disorders of sex development. METHODS: Over 10 years (from February 2012 to March 2022), 102 boys with severe hypospadias underwent voiding cystourethrography. All patients presented with disorders of sex development and Y material in the karyotype (46,XY karyotype, 46,XY/45,X mosaic, etc.). The age of the patients at the first examination ranged from 4 days to 27 months (mean age 2.7 months). RESULTS: Voiding cystourethrography revealed the presence of a cyst posterior to the urethra in 66 patients (64.7% of cases). There were 15 cases of "vagina masculinus" and 51 of enlarged prostatic utricles. These dilations were classified according to the Ikoma classification and cyst size. In more than 2/3 of cases, the cysts were small (less than 20 mm), and in less than 8% of cases, these cysts were large. In addition, retrograde opacification revealed the presence of vesicoureteral reflux in 20% of boys with a male vagina. The most severe hypospadias with a scrotal or perineal meatus are most at risk of developing an enlarged prostatic utricle, and 80% of patients with Ikoma Grade III had a scrotal or perineal meatus. CONCLUSION: This study shows that the prevalence of enlarged prostatic utricles and vagina masculinus is high in patients with severe hypospadias. Therefore, their search should be systematic, and for clinical and therapeutic interest, the enlarged prostatic utricles should be classified according to cyst size.

Ectopic lingual thyroid.

Thyroid ectopy is characterized by the presence of thyroid tissue outside its normal position resulting from a defect of the thyroid diverticulum migration from the base of the tongue until its final pre-tracheal position. One case is presented in a 12-year-old girl patient who consults for a failure to thrive estimated at less than three standard deviations (SD). Bone age was estimated at 8 years late compared to chronological age. The hormonal assessment showed hypothyroidism with negative thyroid antibodies. Cervical ultrasound was revealed thyroid parenchyma pre-dominantly left in place while sweeping the area under chin showed a nodular formation of the base of the tongue. Thyroid scan with technetium 99 m showed a selective uptake of radiotracer in sublingual position. Cervical computed tomography revealed a posterior median sublingual mass spontaneously hyperdense and enhancing sharply after injection of contrast. Treatment with thyroxine allowed obtaining euthyroidism. This case asks us to be careful before aetiological diagnosis of hypothyroidism in children, because although this is rare, the presence of a thyroid parenchyma up to the cervical ultrasound does not eliminate the presence of ectopic tissue.