Crossed testicular ectopia with bilateral duplication of the vasa deferentia: an unusual finding in cryptorchism.

A very unusual association of crossed testicular ectopia, with bilaterally duplicated vasa deferentia, was encountered in an otherwise normal 10-year-old boy who presented with bilateral cryptorchism. The left testis was palpable in the groin; the right was nonpalpable. Because of ductal fusion, both testes were brought down on the same side to avoid damage during separation, with placement of the testes in either hemiscrotum through a transseptal incision.

Alleged resection of the vas deferens: medicolegal implications.

New York state law requires that significant complications be reported by telephone within 24 hours, followed by a full written report within five days with the names of the alleged involved physicians. This case illustrates the potential medicolegal consequences following the mandated immediate nonsubstantiated report of an alleged bilateral vas deferens injury during a bilateral inguinal herniorrhaphy in a 7-month-old male. Review of the surgical pathologic specimen showed that both hernial sacs did indeed contain segments of vas deferens structures, which based on the findings at reexploration, were consistent with duplication or embryonic remnants of the vas deferens. The vas deferens was found to be normal and uninjured bilaterally. The legal ramifications remain unclear. Since there is no clear cut method to retract the allegation, the physician's profile may be permanently damaged.

Early gender assignment in true hermaphroditism.

True hermaphroditism is a rare form of intersex anomaly where both male and female gonads are present. True hermaphrodites diagnosed in the past have been preferentially reared as males. However, based on the appearance of their external genitalia and the ability to reconstruct a functional genitourinary system, most of these children should be raised as girls. Between 1968 and 1987, we managed eight children with true hermaphroditism. The karyotypes were as follows: five 46XX, one 46XY, one 47XXY, and one 46XX/mosaic. Gonadal distribution was bilateral (two ovotestes) in two, lateral (testis and ovary) in two, and unilateral (ovotestis and ovary) in four. Seven patients were diagnosed shortly after birth. Six were raised as girls, two as boys. Based on a review of the relationship of external-internal genitalia, gonads, and karyotype of our own patients and those published by others, we believe that the appearance of the external genitalia alone allows a prompt and definitive gender assignment in infancy. This led us to develop a classification of external genitalia based on the following characteristics. Class I represents normal female genitalia, class II an enlarged clitoris, class III partially fused labioscrotal folds, class IV fused labioscrotal folds, class V a hypoplastic scrotum and penoscrotal hypospadias, and class VI represents normal male genitalia. We had two patients that were class II, two class III, three that were class IV, and one to IV should be raised as females; in class V, male gender assignment is preferable. This classification, based on the appearance of the external genitalia, allows a simple screening and prompt gender assignment in infants identified as true hermaphrodites.

Results of levatorplasty for anal incontinence.

Since the introduction of the levatorplasty, 44 patients were operated upon for anal incontinence on our service. In 31 patients, a levatorplasty was performed. The selection of the levatorplasty as operative procedure was based on the availability of a functional levator as demonstrated by physical examination, anorectal manometry, and defecography. The 31 patients ranged from infancy to 76 years of age and 30 of them were followed up to 7 years with an average of 3 years. In 18 patients with fecal incontinence following operative procedures for anorectal malformations, satisfactory continence was restored in 8, improvement was found in 6, and 4 patients remain incontinent. In 13 patients with incontinence following operative procedures or trauma, the postoperative results were satisfactory in 9 patients, 2 improved, and 2 failed. The review showed that the levatorplasty was most successful in patients in whom the revised but normally innervated levator replaces a destroyed external sphincter in the presence of an intact internal sphincter. Only one half of all patients with major incontinence following an operative procedure for anorectal malformations had an adequate levator that allowed successful restoration of function. The review showed that the levatorplasty can improve or restore anal continence in a variety of conditions in properly selected patients.

Utero vaginal malformations: a trap for the unsuspecting surgeon.

Eighteen girls with major uterovaginal malformations were admitted to the Pediatric Surgical Service over a 17-year period. The diagnosis was not suspected or delayed in more than one half of the patients. The encountered anomalies were divided into four groups: I, isolated uterovaginal malformations (UVM) (4); II, UVM with anorectal anomalies (8); III, UVM with cloacal (urogenital sinus) abnormalities (5); and IV, caudal twinning (1). Imperforate hymen, vaginal web, low vaginal obstruction, or disorders of gonadal or chromosomal development were excluded. Patients presented with an abdominal mass or distension (5), abdominal pain (4), "sciatic"-like pain (1), purulent vaginal discharge with perineal pain (1), amenorrhea (2), and a pelvic and prerectal mass (1). The introitus was reported as normal in 11 patients with vaginal atresia or agenesis by the primary physician. Diagnostic studies, in addition to clinical and endoscopic examination, included routine radiological workup, genitourinary contrast studies, pelvic and perineal sonography, computerized tomography (CT) scanning, and more recently, magnetic resonance imaging. In complicated UVM, especially vaginal duplications with unilateral atresia, the CT scan was the most helpful diagnostic tool. Laparotomy was necessary, not only for therapeutic, but diagnostic reasons; even so, complex anomalies, such as vaginal duplication with unilateral atresia and a septate uterus, could not be suspected. Treatment was directed toward the restoration of a functional uterovaginal tract and the frequently associated anorectal anomalies. A review indicated that contrary to our expectations, the major UVM occurred in children with a low imperforate anus rather than the high variety.

The Koyanagi-Nonomura 1-stage bucket repair of severe hypospadias with and without penoscrotal transposition.

PURPOSE: We report our experience with 1-stage Koyanagi-Nonomura hypospadias repair, which may be performed even when penoscrotal transposition is present. Repair involves bilateral parameatal skin flaps based on the meatus and urethral plate. MATERIALS AND METHODS: A total of 14 boys 10 to 20 months old underwent 1-stage Koyanagi-Nonomura repair for severe hypospadias. The meatus was proximal to the penoscrotal junction in all patients, and in 8 penoscrotal transposition was corrected during the same operation. RESULTS: In 7 of the 14 boys 1 or more fistulas developed that were subsequently repaired. In all cases the fistula was on the proximal shaft and/or penoscrotal junction. In no case was there stenosis or breakdown of the distal neourethra. CONCLUSIONS: The Koyanagi-Nonomura 1-stage repair provides excellent cosmetic results in severe hypospadias while preserving the available urethral plate tissue. It is particularly suitable when there is associated penoscrotal transposition. Most if not all 1-stage repairs are inappropriate in patients with severe hypospadias and penoscrotal transposition. In other repairs vascular supply to Byar's flaps and the neourethra may be compromised by mobilization of the anteriorly transposed scrotal tissue. We expect that the fistula rate will decrease with experience but now it is acceptable, considering the severity of hypospadias.

MR and CT demonstration of levator sling in congenital anorectal anomalies.

Demonstration of the levator ani muscle is important in evaluating children with anorectal anomalies. Using magnetic resonance and CT we have imaged the levator sling in three children prior to their definitive surgery for imperforate anus. The levator mechanism was seen as a low signal band extending from the coccyx to the pubis.