Anatomical measurements of the urogenital sinus in virilized female children due to congenital adrenal hyperplasia

Marei, Mahmoud Marei; Fares, Ahmed E.; Abdelsattar, Ayman H.; Abdullateef, Khaled S.; Seif, Hadeel; Hassan, Mona; Elkotby, Montasser; Eltagy, Gamal; Elbarbary, Mohamed M.

doi:10.1016/j.jpurol.2016.02.008

Cited by 25 publications

(22 citation statements)

References 17 publications

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“…Because of difficulties in performing usual skin preparation for clitoridectomy, antibiotics were used postoperatively. Instead of resembling the normal anatomical position of either a male (distal end of the pars longa glands) or female (approximately 3 cm cranial to the clitoris), the urethral opening was located along the caudoventral surface of the clitoris at the caudal end of the hypospadias, which is abnormally located urinary orifice in the male due to incomplete masculinization of urogenital sinus (Marei et al, 2016). Initially, the embryo has the undifferentiated ducts and structures, from which both internal and external genitalia develop (Marei et al, 2016;Araujo et al, 2018).…”

Section: Discussionmentioning

confidence: 99%

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2021

PVJ

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The disorder of the X chromosome causes defects in sex differentiation in the female phenotype. This work reports a six-month-old, Cocker Spaniel dog with intersex disorder diagnosed as pseudo-hermaphroditism. The dog was brought to the clinic with a swollen reddish penis-like protrusion, which was partially covered by the sheath. Clinical examination revealed the presence of raised nipple-like structures, resembling the nipples and extending from chest to groin region. However, the testes could not be detected. PCR analysis revealed the absence of the Y chromosome. A corrective surgery, including clitoridectomy, and removal of uterus and testicleslike structures, was performed. Histological examination of the removed organs showed underdeveloped testes and well-recognized endometrium. This report suggests that surgical correction may be used to overcome any possible complications in a six-month-old dog with pseudo-hermaphroditism.

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Section: Discussionmentioning

confidence: 99%

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2021

PVJ

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“…Twelve guidelines and general care recommendations were identified [7,8,13,[20][21][22][23][24][25][26][27][28]. Moreover, articles with a special focus on the somatic, biochemical, and genetic assessment, as well as on imaging and psychosocial care in children with a suspected DSD diagnosis, were selected [29][30][31][32][33][34][35][36][37][38][39][40][41][42].…”

Section: Resultsmentioning

confidence: 99%

Diagnosis of DSD in Children—Development of New Tools for a Structured Diagnostic and Information Management Program within the Empower-DSD Study

Wechsung

Marshall²,

Jürgensen³

et al. 2022

JCM

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Background: Current recommendations define a structured diagnostic process, transparent information, and psychosocial support by a specialized, multi-professional team as central in the care for children and adolescents with genital variations and a suspected difference of sex development (DSD). The active involvement of the child and their parents in shared decision-making should result in an individualized care plan. So far, this process has not been standardized. Methods: Within the Empower-DSD study, a team of professionals and representatives of patient advocacy groups developed a new diagnostic and information management program based on current recommendations and existing patient information. Results: The information management defines and standardizes generic care elements for the first weeks after a suspected DSD diagnosis. Three different tools were developed: a guideline for the specialized multiprofessional team, a personal health record and information kit for the child with DSD and their family, and a booklet for medical staff not specialized in DSD. Conclusions: The new information management offers guidance for patients and professionals during the first weeks after a DSD diagnosis is suspected. The developed tools’ evaluation will provide further insight into the diagnostic and information-sharing process as well as into all of the involved stakeholders’ needs.

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“…The length of the upper urethra depends on the vagina entering above or below external urethral sphincter and is an important factor in the categorization suggested by Rink and colleagues. 13 This should be the main determinant of the complexity of surgical intervention and outcomes of the patients, 11 because the upper urethra is a richly innervated structure surrounded completely by circumferential innervations; therefore, authors are advocating against the disruption of the high periurethral area. The mobilization of the pubourethral ligament during TUM could negatively impact continence postoperatively.…”

Section: Discussionmentioning

confidence: 99%

Laparoscopic Resurrection of an Old Technique: A New Approach for Total Urogenital Separation and Rectal Pull-Through in Patients with Long Common Channel Cloacal Malformation

Taher

Fares

Wishahy

2022

Journal of Endourology

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Background and Aims: Before the significance of urethral length was highlighted in patients with cloacal malformation, total urogenital mobilization using a posterior sagittal approach was recommended for common channel (CC) length <3 cm, those >3 cm it was followed by urogenital separation. However, many urologists are advocating that the urethral length rather than length of the CC should influence the choice of operation. It is also recommended that total urogenital mobilization should be avoided in patients with short urethral length as intraoperative decision to shift to urogenital separation will risk devascularization of the urethra, advocating total urogenital separation (TUS) from the start; the later technique was deemed difficult using open approach. We describe our experience with laparoscopic TUS and rectal pull-through in patients with cloacal malformation. Methods: Six patients were operated for a period of 3 years from December 2017 to July 2021; they underwent laparoscopic TUS and rectal pull-through. Preoperative investigations included cystoscopy, genitogram, and MRI pelvis and abdominal ultrasound. IRB approval has been obtained from research ethical committee at Cairo University. Results: Six female patients born with single perineal opening had colostomy at birth. Age during the second operation ranged from 1 to 4 years. Length of the CC ranged between 2 and 5 cm. Proximal urethral length ranged between 0.5 and 1.5 cm and vaginal depth >3 cm. Average operative time was 4.25 hours. Postoperative period was 1–5 days and uneventful. On the long-term follow-up. No patient developed urethrovaginal fistula and one patient developed vaginal stenosis. All patients had no urinary problems, dry over 4-hour interval, voiding spontaneously, and had normal kidney functions. Conclusions: Laparoscopic urogenital separation, as well as vaginal and rectal pull-through for cloacal malformation, is feasible in cloacal malformation providing anatomical repair.

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Anatomical measurements of the urogenital sinus in virilized female children due to congenital adrenal hyperplasia

Cited by 25 publications

References 17 publications

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Diagnosis of DSD in Children—Development of New Tools for a Structured Diagnostic and Information Management Program within the Empower-DSD Study

Laparoscopic Resurrection of an Old Technique: A New Approach for Total Urogenital Separation and Rectal Pull-Through in Patients with Long Common Channel Cloacal Malformation

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