Urinary Continence Following Repair of Intermediate and High Urogenital Sinus (UGS) in CAH. Experience with 55 Cases

Abstract

Aim: To evaluate postoperative urinary continence in patients with congenital adrenal hyperplasia (CAH) with intermediate (IT) and high urogenital sinus (UGS) who underwent a UGS mobilization maneuver.Methods: We called IT to those that although needing an aggressive dissection to get to the vagina, still have enough urethra proximal to the vaginal confluence. Very low variants are excluded from this analysis. Dissection always started in the posterior wall of the UGS with an aggressive separation from the anterior rectal wall. If the wide portion of the vagina was reached dissection stopped and the UGS opened ventrally widening to the introitus. Nineteen patients were treated using this maneuver (Group 1). When more dissection was required the anterior wall of the UGS was dissected and carefully freed from the low retropubic space. Then the UGS was opened either ventrally or dorsally. Thirty three patients required this approach (Group 2). Combined procedures were used in three patients with high UGS (Group 3).Results: Mean age at the time of the repair and length of the UGS were 12.2 years (4 months–18 years) and 3.75 cm (3–8 cm) for G1; 8 years (5 months–17 years) and 6.34 cm (4–12 cm) in G2 and 8.3 years (2–14 years) and 11.5 cm (11–12 cm) in G3. All patients had been regularly followed. Mean age at last follow up was 14.3, 17, and 9.9 years for Groups 1, 2, and 3, respectively. All patients continue to void normally and are continent. All patients have two separate visible orifices in the vulva. Only three are sexually active.Conclusion: Urogenital sinus mobilization for vaginoplasty in girls with CAH does not compromise voiding function or urinary continence.

Surgical Treatment after Failed Primary Correction of Urogenital Sinus in Female Patients with Virilizing Congenital Adrenal Hyperplasia: Are Good Results Possible?

“…This technique avoids dissection of the proximal urethra, preserving the perineal innervations, and the urethral sphincter. There are no reports of incontinence with this technique (19–21). It was employed in three of the first reoperations and one of the second reoperations, totaling four patients with high vaginal insertion, and all of them achieved separate urethral and vaginal orifices in the perineum.…”

Cinsiyet Gelişim Bozukluklarının Tarih İçindeki Evrimi ve Tedavi Yöntemlerinin Gelişimine Kısa Bir Bakış

“…Bailez ve arkadaşları, 55 intermediate ve yüksek vajina yerleşimli hastada uyguladıkları Passerini flep ve ASTRA (Anterior Sagittal Transrectal Approach) kombinasyonlu TUM sonrası tüm hastaların üriner kontinansının tam olduğunu ve perinede iki açıklığın elde edildiğini bildirmişlerdir (71). Aynı ekip son on yılda bu girişimlerde yüksek vajina mobilizasyonu ve UGS'den ayırma işlemini, anorektal malformasyonlarda rektovezikal fistüllerin ayrılmasına benzer bir yaklaşımla vajina ve UGS kanlanmasına zarar vermeden laparoskopi ile yaptıklarını, perineden küçük bir insizyonla çalışarak laparoskopi yardımlı abdomino-vajinal pull-through işlemini tamamladıklarını bildirmekte ve teknik detaylarını anlatmaktadırlar 38 .…”

Disorders of sex differentiation: state of the problem 15 years after the Chicago consensus

“…Также необходимо отметить, что отдаленные результаты при использовании новых техник, прежде всего, мобилизации урогенитального синуса, все еще не опубликованы, но сообщения о непосредственных и среднесрочных результатах внушают оптимизм [11,[47][48][49][50].…”