We report 2 cases of persistent urogenital sinus (UGS) with duplication of the bladder and vagina in different planes. Cystoscopy and vaginoscopy were used to diagnose persistent UGS with bladder and vaginal duplication in 2 infants with recurrent urinary tract infection. Surgical repair was done by way of a midline abdominal and perineal approach using tubularized UGS to reconstruct the urethra. Persistent UGS represents a major anomaly; these patients had the added complexity of duplication of the bladder and vagina. In these complex patients, the true anatomy may only become apparent during the surgical reconstruction, necessitating a flexible, individual approach.
Download full-text PDF |
Source |
---|---|
http://dx.doi.org/10.1016/s0090-4295(02)02545-1 | DOI Listing |
J Clin Endocrinol Metab
January 2025
Department of Pediatrics, University of Colorado Anschutz Medical Campus, Aurora, CO 80045, USA.
The most common form of congenital adrenal hyperplasia (CAH) is secondary to 21-hydroxylase deficiency (21OHD). This review will summarize the clinical manifestations, recommended treatments, monitoring, clinical challenges and management strategy, and treatment challenges in special situations for infants and children with classic CAH due to 21OHD. Specifically, we review newborn screening and the initial diagnosis, glucocorticoid and mineralocorticoid treatment, and recommended monitoring, including anthropometric and laboratory measures.
View Article and Find Full Text PDFDev Biol
January 2025
Institute for Stem Cell Science and Regenerative Medicine (iBRIC-inStem), GKVK-Post, Bellary Road, Bengaluru, Karnataka, 560065, India. Electronic address:
J Pediatr Adolesc Gynecol
January 2025
Division of Pediatric and Adolescent Gynecology, Department of Obstetrics, Gynecology and Reproductive Sciences, Yale University School of Medicine.
Objective: Subocclusive variants of the hymen are a group of hymenal anomalies that result due to a failure of canalization of the urogenital sinus. One of these variations is the microperforate hymen, for which accurate incidence is unknown. Microperforate hymen is a non-obstructing variation that allows for menstrual egress and often patients present due to inability to use a tampon.
View Article and Find Full Text PDFJCEM Case Rep
January 2025
Department of Pediatrics, Division of Pediatric Endocrinology, Yale School of Medicine, New Haven, CT 06510, USA.
46,XY sex reversal 11 (SRXY11) is a rare and recently identified form of 46,XY difference in sexual development (DSD), caused by variants in the DEAH-Box Helicase 37 gene (). is crucial for ribosome biogenesis, but its specific role in gonadal development remains unclear. The genital phenotype varies widely, ranging from typical female to typical male.
View Article and Find Full Text PDFJ Clin Res Pediatr Endocrinol
January 2025
Marmara University Faculty of Medicine, Department of Pediatric Endocrinology, İstanbul, Turkey
Signs of virilization, such as clitoromegaly, labio-scrotal fusion, and urogenital sinus may be observed in females with 21-hydroxylase deficiency (21-OHD) and other rare virilizing forms of congenital adrenal hyperplasia (CAH). This makes sex determination difficult, and multiple reconstructive surgeries in the postnatal period may be required. As 21-OHD is an autosomal recessive disease, the chance of any child being affected is one in four and so only one in eight will be an affected female.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!