Abokifa, A., El Badawy, R., Anwar, H., El Sagheer, Y. (2019). Disorders of Sexual Development in Proximal Hypospadias in Children. The Egyptian Journal of Hospital Medicine, 77(6), 5771-5775. doi: 10.21608/ejhm.2019.63574
Amin M. Abokifa; Refaat I. El Badawy; Hesham W. Anwar; Yahia E. El Sagheer. "Disorders of Sexual Development in Proximal Hypospadias in Children". The Egyptian Journal of Hospital Medicine, 77, 6, 2019, 5771-5775. doi: 10.21608/ejhm.2019.63574
Abokifa, A., El Badawy, R., Anwar, H., El Sagheer, Y. (2019). 'Disorders of Sexual Development in Proximal Hypospadias in Children', The Egyptian Journal of Hospital Medicine, 77(6), pp. 5771-5775. doi: 10.21608/ejhm.2019.63574
Abokifa, A., El Badawy, R., Anwar, H., El Sagheer, Y. Disorders of Sexual Development in Proximal Hypospadias in Children. The Egyptian Journal of Hospital Medicine, 2019; 77(6): 5771-5775. doi: 10.21608/ejhm.2019.63574
Disorders of Sexual Development in Proximal Hypospadias in Children
Department of General Surgery, Faculty of Medicine, Al-Azhar University
Abstract
Background: Hypospadias is seen in around one in 300 live births, making it the most prevalent congenital penile abnormality. Objective: to evaluate of the incidence and diagnosis of disorders of sexual development in proximal hypospadias. Patients and Methods: This prospective study was conducted on 44 children attending the Pediatric Surgery Department, Al-Azhar University Hospitals during the period from Dec 2018 to Nov 2019. Detailed history taking; including complete physical examination, examination with special focus on external genitalia and gonads; routine and specific laboratory investigation as genetic karyotyping, serum testosterone (T), dihydrotestosterone, dehydroandrostenedione,17 OH progesterone, HCG stimulation test, serum levels of luteinizing hormone (LH) and follicle-stimulating hormone (FSH), Adrenal hormones “serum cortisol & plasma adrenocorticotropic hormone (ACTH) “to exclude congenital adrenal hyperplasia. Ultrasound abdomen and pelvis was routinely done to assess the internal genital organs " uterus, fallopian tubes " and gonads " testis, ovaries ". Results: there are (5) patients diagnosed as DSD: Three cases (6.8 %) have Karyotyping (46, XX), diagnosed as CAH. One case (2.2%) has Karyotyping (46XY (70%)/45X (30) sex chromosome DSD (45, X/46, XY PGD) or “mixed gonadal dysgenesis”. One case (2.2%) has Karyotyping (46, xy), diagnosed as complete Androgen insensitivity syndrome. Conclusion: Patients presenting with proximal hypospadias and one or more of the co-existing anomalies of micro penis, undescended/impalpable testes, and penoscrotal transposition/bifid scrotum should warrant DSD evaluation. Presence of bilaterally descended testes in scrotum does not preclude the possibility of DSD.