Ureteral triplication: double extravesical ureteral ectopia

A 21-month-old girl presented with ureteral triplication, representing an exceptional variant of Smith's type 1. One ureter was intravesical and the other 2 were extravesical (bladder neck and vaginal).     

Crossed ureteral ectopia with solitary kidney

A case of crossed ectopia with a solitary kidney in a 73-year-old man is presented. The fact that the kidney is normally situated and vascularized, and the presence of a second blind homolateral ureter leads us to conclude that this is a case of crossed ureteral ectopia in a solitary kidney. The embryogenesis of the malformation is discussed.     

A case of ureteral triplication associated with ureteropelvic junction obstruction

Ureteral triplication is one of the rarest malformations of the upper urinary tract. The association of ureteral triplication and obstruction is even rarer. We report a case of ureteral triplication associated with hydronephrosis due to ureteropelvic junction (UPJ) obstruction at the middle pole ureter. To our knowledge, such a malformation has not been described previously.     

Giant hydronephrosis of a duplex system associated with ureteral ectopia

Giant hydronephrosis is an infrequent urological entity. To our knowledge, only 1 case has been reported previously that was associated with a duplicated collecting system and ureteral ectopia.     

Postpartum onset of urinary incontinence associated with ureteral ectopia.

The onset of urinary incontinence in adult women in association with extrasphincteric ureteral ectopia is rare. Herein we report 2 cases of this unusual occurrence. The incontinence in both patients began after significant obstetric trauma. Ureteral ectopia should be included in the differential diagnosis of incontinence beginning in the immediate postpartum period.     

Single system ureteral ectopia in boys associated with bladder outlet obstruction

PURPOSE: Ureteral ectopia is frequently associated with dysplasia of the associated renal segment in girls with ureteral duplication. However, single system ureteral ectopia is an uncommon anomaly more frequently noted in boys. We report on 6 boys with single system ureteral ectopia into the prostatic urethra above the verumontanum, who presented with radiological and clinical findings of bladder outlet obstruction. MATERIALS AND METHODS: Antenatal ultrasound in 3 boys demonstrated renal abnormalities and postnatal studies suggested the diagnosis of posterior urethral valve obstruction. Older boys presented with symptoms suggestive of bladder outlet obstruction. RESULTS: An ectopic ureter inserting into the prostatic urethra was noted in all 6 boys. The distal ureter was dilated elevating the bladder neck causing outlet obstruction. Surgical management consisted of nephrectomy and transurethral endoscopic incision of the distal ureter or nephroureterectomy with reconstruction of the prostatic urethra. In 2 younger boys voiding dysfunction with inability to empty developed. CONCLUSIONS: Single system ectopic ureters in boys may present with symptomatic and radiological findings resembling posterior urethral valves. Surgical treatment should include nephroureterectomy with reconstruction of the hypoplastic prostate. Some patients may have later voiding dysfunction.     

Renal dysplasia associated with ureteral ectopia and ipsilateral seminal vesicle cyst

The simultaneous presence of renal aplasia/dysplasia, ureteral ectopia and seminal vesicle cyst represents an exceptionally rare malformation. The literature is reviewed here and a new case of such malformation, concerning a 45-year-old man affected by recurrent epididymitis, is reported.     

Immediate operation for ectopia cordis.

Forty-one infants with thoracoabdominal ectopia cordis have been reported to date; 9 of them survived. Among the patients with an omphalocele as the abdominal wall defect, however, only 2 survived. Death in this group of patients occurred almost exclusively as a consequence of either attempted coverage of the heart or secondary to the associated intracardiac lesion. We report here the case of a patient recently treated in whom coverage with a temporary Silastic prosthesis protected the exposed heart from infection and rupture of the sac, thus permitting full cardiac investigation before operation.     

Laparoscopic ureteroureterostomy in children with a duplex collecting system plus obstructed ureteral ectopia

One of the complex upper urinary tract anomalies is a duplicated collecting system. In cases with a functioning upper moiety, ureteroureterostomy (UU) is the preferred operation to redirect the urine to the normal collecting system. Although open UU is a well-described operation, experience with laparoscopic repair pediatric patients is scarce. We describe the successful application of laparoscopic UU in 2 children and suggest that laparoscopic UU for the duplicated collecting system is a promising minimally invasive procedure.     

Experience with Repair of Complete Atrioventricular Canal

Twenty-nine patients at our institution have undergone repair of complete atrioventricular canal since 1969. There were 3 operative and 5 late deaths, 4 of which were of infectious etiology. Age at operation ranged from 2 months to 12 years (mean, 50 months). Weight ranged from 3.6 kg to 30 kg (mean, 12 kg). Before repair, catheterization studies revealed pulmonary hypertension in all patients with unobstructed pulmonary arteries. Pulmonary to systemic flow ratio ranged from 1.5 to 10.6 (mean, 3.5). Pulmonary vascular resistance (PVR) ranged from 0.7 to 21.7 (mean, 5.3) Wood units/m². At repair, 14 patients had Rastelli type A anatomy, 14 had type C, and 1 patient had a variant with crossing chordae and double-outlet right ventricle (DORV).Postoperative catheterization has been done in 16 patients 2 months to 8 years (mean, 30 months) after repair. One patient had residual ventricular shunting and later underwent successful repair. A 2-year-old patient had severe mitral regurgitation and died following mitral valve replacement. One patient required a permanent pacemaker. The 21 surviving patients have been followed from 7 months to 10 years 7 months and have excellent hemodynamic status. Long-term studies are needed to assess the ultimate effect on patients with high PVR.     

Nonduplication of upper urinary tract associated with horseshoe kidney and bilateral ureteral ectopia

A three-month-old white baby girl was found to have marked hydroureteronephrosis associated with ectopic, nonduplicated ureters and horseshoe kidney. Her clinical and radiographic course are discussed.     

Tetralogy of Fallot Associated with Complete Atrioventricular Canal

A 6-year-old girl had the clinical appearance of tetralogy of Fallot but was found at cardiac catheterization to have tetralogy of Fallot associated with complete atrioventricular canal. She underwent repair, and at recatheterization two years later, the hemodynamic result was excellent. Proper angiography is necessary to diagnose this combination, and correct preoperative diagnosis is necessary to plan a rational operative approach.