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Surgical management of urogenital sinus abnormalities.

This report describes 12 cases of urogenital sinus malformations and their management in female infants, 10 with imperforate anus. Excluded were cases of urogenital sinus malformation secondary to the adrenogenital syndrome or primary sexual ambiguity. Immediate definition of the anatomy by appropritae endoscopy and radiography is essential to plan the correct operative approach. This can be a relatively simple flap vaginoplasty from below, together with perineal anoplasty in some cases. Others may require an extensive combined abdominal-perineal approach with vaginal pullthrough, ureteral reimplantation, creation of a urethra from the urogenital sinus, resection of vaginal septum, and creation of perineal skin flaps to join to the pulled through vagina which may be too short to reach the perineum.

Adolescent

Urogenital sinus abnormalities in the female patient.

Three patients with a urogenital sinus and 1 with a cloaca presented with complex diagnostic and therapeutic anomalies. A classification of these and other vaginal anomalies is based on the theory that they all represent an arrest in normal embryological development. Six categories are described, including vaginal agenesis, urogenital sinus, vaginal atresia, vaginal septa, vaginal stenosis and cloaca.

Cloaca

[The development of urogenital organs after lateral mesoblast heterotopic graft in the toad (Bufo bufo) neurula. II. Simultaneous inversion of anteroposterior and dorsoventral axis of the lateral plate].

Simultaneous inversion of the anteroposterior and dorsoventral axes of the lateral mesoblast in the early toad neurula leads to the total agenesis of the urogenital organs. The experimental results show that the lateral mesoblast is unable to undergo self-differentiation, and that three factors at least are required for the formation of the urogenital blastemata. The mesoblast must be competent: only the posterior (dorsal, but also ventral) part of the lateral mesoderm is endowed with this competence. It must receive two stimulating influences, from the cordomesoblast on the one hand, and from the dorsocaudal endoblast on the other hand.

Animals

[Ectopic ureter ending in the seminal vesicle in adults. Apropos of a case and review of the literature].

The authors report a 38 years old patient in whom an intravenous pyelogram was performed for moderate arterial hypertension. This showed a large filling defect in the right lower part of the bladder without renal function on that side. Endoscopic separation of the jet coming from the right half of the trigone showed a liquid containing numerous spermatozoa. A cystogram opacified by reflux a cavity in the genital system via this, the right ureter. With the diagnosis of ectopic ureter ending in the genital system, a nephro-ureterectomy demonstrated the attachment of the ureter into a pouch which communicated with the bladder and the seminal vesicle. A view of the normal embryology insists on the fact that contrary to the classic view, the pronephros disappears entirely and that the metanephros is at the origin of the Woffian canal which opens on the posterior wall of the urogenital sinus at the 28th day when the ureter springs from a ureteral bud. Since 1960 this would be the 34th case in the literature, seminal ectopia (24%) coming after ectopia in the prostatic urethra (54%). Epididymitis is a frequent presenting symptom (44%). The workup shows: absence of renal function (100%), raising of the ipsilateral trigone by a cystic swelling (76%), absence of the meatus on that side on endoscopy, presence of an ipsilateral mass above the prostate on rectal examination (30%). The diagnosis is confirmed by vasography where the ureter is opacified by the seminal vesicle or by systography after endoscopic incision of the mass. Excision of the seminal vesicle was done in conjunction with total nephroureterectomy in 17% of cases.

Adult

[Herlyn-Werner and Wunderlich syndromes (author's transl)].

Reported in this paper are four cases of rare, combined, congenital, urogenital abnormalities, that is combination of inhibitory malformation (duplication) of uterus, paracervically localised cystic resistance, and renal aplasia on the same side. With all apparent similarity of the four cases, there were two different syndromes, the Herlyn-Werner syndrome in two cases and the Wunderlich syndrome in the other two. Both syndromes are presented in diagrams and described.

Abnormalities, Multiple

Induction of urogenital neoplasia and abnormalities from prenatal exposure to diethylstilbestrol.

The occurrence of vaginal clear cell adenocarcinoma in young women following exposure in utero to diethylstilbestrol (DES) is now well documented. In addition to this carcinogenic potential. DES has been shown to be teratogenic. In females, the DES-related malformations include vaginal adenosis, transverse ridges of the vagina or cervix and uterine abnormalities. Although no neoplasms have been observed in DES-exposed males, malformations of the epididymis, testes and phallus are relatively common and may result in infertility. The carcinogenic mechanism of DES may be either a direct induction of malignant potential in vaginal cells or a teratogenic effect causing ectopic Müllerian epithelium which could be exposed later to mutagenic agents in the vagina. The absence of malignancy in DES-exposed males may favor the latter hypothesis since male Müllerian remnants are internal structures and thus would not be exposed to surface carcinogens.

Adenocarcinoma

Surgical correction of caudal duplication.

The authors report the rare case of a dipygus monster. The infant had an omphalocele, a secondary pelvis with two limbs, an accessory pelvic kidney, and two bladders. At operation, the secondary pelvis and extremities were removed, and the omphalocele was repaired. The postoperative course was complicated by wound infection, urinary infections, proteinuria, cardiomegaly, and hypertension of renovascular origin. The infant died after right nephrectomy, 5 mo after the first operation.

Abnormalities, Severe Teratoid

[The triad syndrome].

The triad syndrome includes manifestations from urologic pathology to orthopedics, passing through the gastrointestinal to the respiratory pathology. Treatment of these syndromes calls for an intensive management, carried out by a multidisciplinarian pediatric team, to whom the surgical technique is as important as the genetic counseling; this is the only way to offer these children, who have been precociously damaged, a happy infancy. On the other hand, it is the purpose of this work to make an early discovery of congenital malformations, detecting their minimal expressions, which is the only manner to reach an early diagnosis so as to indicate an adequate therapy at the opportune moment. All this added to the fact that in 1973-1975 we gathered a few cases of triple syndrome, plus our limited bibliography (Rahman) resulted in the necessity to bring up-to-date the topic. At present, 5 years later, the satisfactory course in these patients has allowed us to indicate guidelines in the diagnosis and treatment.

Abnormalities, Multiple