Yang-Jann Liao

DIAGNOSING THE COMBINATION OF RENAL DYSGENESIS, GARTNER'S DUCT CYST AND IPSILATERAL MULLERIAN DUCT OBSTRUCTION

PURPOSE We describe the differential points in the diagnosis of the combination of renal dysgenesis, Gartners duct cyst and ipsilateral müllerian duct obstruction. Various imaging studies and urological procedures were performed. We report our experience in detecting these anomalies in 10 girls and review the literature. MATERIALS AND METHODS Ten girls, 7 to 13 years old, with this combination of anomalies were identified in the last 10 years. Imaging studies as well as urological procedures were selectively performed, especially at puberty following menarche. Patients received long-term followup with ultrasound. RESULTS Cystic dilation of Gartners duct protruded into the bladder and presented as a ureterocele in 5 patients and posterior to the bladder in 5. Surgical removal of a partial portion of a Gartners duct cyst was performed in 5 patients for alleviation of urinary symptoms. Unilateral müllerian duct obstruction was demonstrated in all 10 patients. Excision of the vaginal septum was performed in 6 patients for relief of genital obstruction. CONCLUSIONS When cystic dilatation of the pelvis, especially a ureterocele-like cyst without ureteral dilatation, is found in girls with ipsilateral renal dysgenesis, the possibility of a Gartners duct cyst should be considered. For early detection and treatment of unilateral obstruction of duplicated müllerian ducts pelvic sonography should be performed at puberty, especially just after menarche, in girls with renal dysgenesis and ipsilateral Gartners duct cyst.

Early detection of unilateral occlusion of duplicated müllerian ducts: the use of serial pelvic sonography for girls with renal agenesis.

For early detection of unilateral occlusion of duplicated müllerian ducts, serial pelvic sonography was performed in 215 girls with known renal agenesis, especially following menarche, during the last 6 years. To date, 16 girls with unilateral occlusion of duplicated müllerian ducts were detected. We stress the value of this modality, which can lead to a prompt diagnosis, and allow for early and appropriate surgical intervention.

Seminal Vesicle Cyst Associated with Ipsilateral Renal Malformation and Hemivertebra: Report of 2 Cases

AbstractTwo cases of a seminal vesicle cyst, ipsilateral renal malformation and a hemivertebra are reported.

BILATERAL CONGENITAL CYSTS OF THE SEMINAL VESICLE WITH BILATERAL DUPLEX KIDNEYS

Congenital cyst of the seminal vesicle is a rare anomaly, which is associated with ectopic insertion of an ipsilateral ureter into the seminal vesicle or other ectopic site.3 Since the embryonic event that causes this anomaly occurs early in gestation, urinary obstruction with distant ectopic ureteral insertion may lead to dysgenesis of the involved kidney. This anomaly usually develops in a single collecting system. It is rarely associated with an ipsilateral duplex ectopic ureter, and only a few such cases have been reported.3 To our knowledge bilateral congenital cysts of the seminal vesicle in a patient with bilateral duplex kidneys have not been described previously. In a single collecting system congenital cysts of the seminal vesicle are often associated with ipsilateral renal dysgenesis. Therefore, bilateral congenital cysts of the seminal vesicle are usually fatal because of severe functional impairment of both kidneys. On the other hand, since normal or nearly normal function of the lower pole moieties develops in bilateral duplex kidneys, bilateral congenital cysts of the seminal vesicle, as in our case, are possible. In our patient mild straining to void and progression of hydronephrosis of both lower moieties may be caused by distension of the seminal vesicle cysts and associated dilated ureters. However, no thickened wall of the bladder was noted on sonography or MRI. Treatment of the seminal vesicle cyst usually is restricted to symptomatic cases. Heminephrectomy with transabdominal excision of the cyst is indicated in patients with no function of the upper renal segment, as in our case. Trans

DUPLEX KIDNEY, GARTNER'S DUCT CYST AND IPSILATERAL MULLERIAN DUCT OBSTRUCTION

Unilateral mullerian duct obstruction associated with ipsilateral Gartner’s duct cyst and renal dysgenesis is extremely rare. To date only 10 cases have been documented and all have occurred in a single collecting system.’ A duplex collecting system with an ectopic ureter inserted into a Gartner’s duct cyst is also rare and, to our knowledge, has not been reported in combination with ipsilateral mullerian duct obstruction. CASE REPORT

Spontaneous rupture of the uterovaginal septum in 2 girls with unilateral hematocolpos and ipsilateral vaginal ectopic ureter

Unilateral hematocolpos associated with ipsilateral single vaginal ectopic ureter is unusual with fewer than 30 cases reported. 1,2 Spontaneous rupture of the common uterovaginal septum in unilateral hematocolpos has been described only rarely.3 We report on 2 girls with unilateral hematocolpos and an ipsilateral vaginal ectopic ureter in whom the common uterovaginal septum ruptured spontaneously.

Splenic Cysts in Children

Splenic cysts were found, incidentally, in eight children during the past nine years (1987-1995) in Taipei Municipal Womens and Childrens Hospital. Five of the children were boys and three were girls. The age at diagnosis ranged from 8 to 15 years. Evidence of possible splenic cyst development was found initially by ultrasonography; six patients received further evaluation with computerized tomography (CT); one patient received radionuclide scanning. The cysts ranged from 2 cm to 14 cm in diameter. Four of the patients received surgical treatment (three partial splenectomy and one total splenectomy) because of huge splenic cysts (diameter > 10 cm). Subsequent pathological examination revealed that all cysts had epithelial cell lining in the cyst wall, meaning they were all congenital in origin. The remaining four cases were followed up at the Out-patient Clinic here. All cases had a benign clinical course.

Serial Ultrasonic Changes of the Bladder Diverticulum During Urination: Report of One Case

A seven-year-old boy with a retrovesical diverticulum and ipsilateral renal dysplasia is reported. He had had a history of frequent urination and dysuria for four years. According to sonography, the volume of bladder diverticulum obviously changed during urination. The diverticulum presented as a temporary large reservoir of the bladder. In this case, serial ultrasonic changes assisted understanding of the role of the diverticulum during urination.

A giant retroperitoneal ganglioneuroma with intraspinal involvement : Report of one case

A 9-year-old girl was discovered to have had a huge retroperitoneal mass causing moderate hydronephrosis and anterior displacement of the right kidney and which was found on a renal ultrasonographic examination. A series of examinations including (1)IVP, (2) abdominal computed tomography, (3) MRI and(4) incisional biopsy revealed a giant dumb-bell shaped retroperitoneal ganglioneuroma with intraspinal involvement. Secondary scoliosis was also noticed. We successfully resected the huge ganglioneuroma of the right retroperitoneum in a two stage procedure: her postoperative course was uneventful.

Sonographic presentation of unilateral hematometra: report of two cases.

An 11 year old girl was referred to our department because of nonvisualization of the right kidney detected incidentally on sonography; her menarche had occurred in the same month. Physical examination was normal. Detailed abdominal sonography and magnetic resonance imaging revealed absence of the right kidney, with compensatory hypertrophy of the left kidney. Double uteri with right hematometra (Fig. lA, B) were found. The right