Toshiaki Gotoh

Clinical Experience of Incidentally Discovered Adrenal Tumor with Particular Reference to Cortical Function

We reviewed 14 male and 9 female patients with adrenal tumor discovered incidentally by imaging studies in an attempt to assess adrenal function, mainly cortical function. Pathological diagnosis consisted of cortical adenoma in 12 patients, cortical nodular hyperplasia in 2, medullary hyperplasia in 1, cavernous hemangioma in 1, metastatic tumor in 4 and cyst in 2. Another adrenal cyst was diagnosed by percutaneous puncture. In all patients peripheral levels of plasma cortisol and aldosterone were normal. Plasma catecholamine levels were also normal except for 1 patient with medullary hyperplasia who had equivocal elevation. Among the patients with cortical adenoma and hyperplasia, however, 5 of 10 (50%) had excessive excretion of 24-hour urinary 17-hydroxycorticosteroids, 4 of 13 (31%) had a loss of plasma cortisol circadian rhythm and 7 of 14 (50%) had insufficient suppression on the dexamethasone test. Furthermore all patients had an increased ipsilateral uptake that was not suppressed after dexamethasone on 131iodine-adosterol scintigraphy, while a diminished contralateral uptake was noted in 5 of 15 (33%). Postoperatively, hypertension decreased to normal in 2 of 3 patients and impaired glucose tolerance was improved in 1 of 2. Two patients with cortical adenoma who exhibited a loss of plasma cortisol circadian rhythm experienced postoperative adrenal insufficiency. These data indicate that a considerable number of incidentally discovered adrenal tumors are not nonfunctioning particularly in reference to glucocorticoid secretion, and adrenalectomy seems to be beneficial in some of those patients with hypertension or impaired glucose tolerance.

Single Ectopic Ureter

We report on 17 female and 4 male patients with single ectopic ureters, 9 of whom also had vaginal ectopic ureters. The clinicopathological features and surgical management are presented, with particular emphasis on the associated renal dysplasia and the complete excision of the ectopic ureteral stump. Although single ureteral ectopia with drainage to the vagina is rare in the English literature the condition is common in Japan.

Clinicopathological and Embryological Considerations of Single Ectopic Ureters Opening Into Gartner’s Duct Cyst: A Unique Subtype of Single Vaginal Ectopia

We report 6 cases of single ectopic ureters opening into a Gartners duct cyst. All but 1 patient were children. The presenting symptoms were urinary incontinence, urinary tract infection, a vaginal mass or weight loss. All but 1 patient had a mass protruding into the vagina. In 5 cases nephroureterectomy was performed successfully. The cyst wall was left untouched to allow for its spontaneous collapse. Although 2 cysts were found to communicate with the vagina, 4 presented as a closed space. All resected kidneys were more dysplastic than those with vaginal ectopia without cyst formation. The etiology and pathogenesis of the anomaly are unknown but some speculations are made.

Muscle Dysplasia in Megaureters

We have previously reported the relevance of muscle dysplasia to the nonreflux megaureter. On electron microscopy, muscle cells which are scattered in large amounts of connective tissue without any bundle formation are found to be deficient in myosin filaments, which, with actin filaments, are believed to be an essential contractile unit of smooth muscle. Investigations of these dysplastic features of the ureter were extended to various other congenital disorders of the ureter experienced in our institution from 1963 to 1981. Muscle dysplasia was found in 8 of 34 cases of nonreflux megaureter, in 1 of 22 cases of reflux megaureter, in 4 of 23 cases of ectopic ureter of single system, in 4 of 9 cases of ectopic ureter of duplex system, 0 of 4 cases of the ureter of ureterocele of single system and in 1 of 13 cases of the ureter of ureterocele of duplex system. When muscle dysplasia was extensive, involving the whole length of the dilated ureter, incidence of associated renal dysmorphism was high in that 12 of 17 ureteral units as such demonstrated either severe renal dysplasia (9) or hypoplasia (3). Similar muscle dysplasia was also found in most of the dome of ureterocele (in 5 of 6 and 12 of 13 ureteroceles of single and duplex systems respectively). Muscle dysplasia is discussed as to its genesis, relevance to various congenital ureteral disorders and clinical implications.

Adrenal Hemorrhage in the Newborn with Evidence of Bleeding in Utero

A case is reported of adrenal hemorrhage in a newborn with evidence of bleeding while in utero. The patient had a large cystic mass on ultrasound at 36 weeks of gestation. Abdominal computerized tomography after birth also revealed the cystic mass above the left kidney, which became smaller with peripheral calcification when he was 5 months old. Thus, adrenal hemorrhage was presumed. The cystic mass had disappeared on followup ultrasound when the patient was 8 months old. This case suggests that adrenal hemorrhage can occur even before delivery.

One-stage repair of perineal hypospadias and scrotal transposition.

Our experience with one-stage repair of severe perineal hypospadias and scrotal transposition is described. In essence the urethra is formed by our (extended parameatal) wing flap-flipping method, while the scrotum is normally repositioned with skin closure. Comparison is made with other repairs, while the safety of the extended parameatal pedicle flap is stressed.

Paraureteral Diverticula: Clinical Experience with 17 Cases with Associated Renal Dysmorphism

We report our experience with 17 cases of paraureteral diverticula seen in the last 12 years. These 17 cases represent a particular group of patients with ureteral reflux in that they often had ureteral obstruction too. Early operative intervention is recommended. There is a trend towards renal dysmorphism and dysplasia as the position of the ureteral orifice laterlizes in the diverticulum. A paraureteral diverticulum was considered to be the developmental extravesical expansion of the terminal ureteral end by virtue of the caudally positioned ureteral bud in the wolffian duct.

Diagnosis and management of sacral agenesis

Herein, the diagnosis and management of 14 cases with sacral agenesis experienced in the last 15 years were reviewed. Age at first presentation tended to be older than that of meningomyelocele patients. All the children displayed partial sacral agenesis or hemisacrum. Occult spinal dysraphism was found in 8. Frequent association of genitourinary and anorectal anomalies required important alterations in the management. Vesicoureteral reflux was found in 8 patients but most of them were treated conservatively. Thirteen children underwent urodynamic evaluation on more than 1 occasion and 12 displayed neurogenic bladder dysfunction (upper motor type 6, lower motor type 5, upper and lower type 1). However, the diagnosis of bladder dysfunction was not final. Clean intermittent catheterization was most frequently assigned but it was difficult in some cases with urethral abnormalities. Overall, 5 children required surgical intervention for their urinary tract, 4 of whom needed multiple procedures. As a result, half the children achieved continence and 1 child, who awaited reconstructive surgery, had slightly elevated serum creatinine. In summary, early diagnosis, complete neurourological evaluation and adequate management with careful follow-up monitoring risk factors to the upper urinary tract are mandatory.

Two Cases of Ectopic Ureter Opening into the Ejaculatory Duct: Double Vas Deferens Revisited

We report 2 rare cases of single ectopic ureter simulating double vas deferens. Clinical, radiographic and macroscopic signs at operation indicated a single ectopic ureter opening into the seminal tract. However, histological examination of the surgical specimen revealed that the lumen of the supposed ureter was lined in part by columnar epithelium, although chiefly by transitional epithelium. The wall of the ureter contained 3 well defined muscular coats in case 1 but only 1 layer was distinguishable in case 2. Histological composition of the structure removed from the cranial site resembled that of the epididymis. A review of the literature revealed a few comparable cases. Analysis of these cases suggests that double vas deferens and a single ectopic ureteral opening into the seminal tract are such that their differentiation may only have semantic significance. We speculate that these 2 diseases are included as 1 entity, only to be distinguished by the difference of ureterization of the ureteral bud.

Morphological Study of the Ureterocele: a Possible Clue to its Embryogenesis as Evidenced by a Locally Arrested Myogenesis

The wall of a ureterocele and the proximal ureter involved were investigated with light and electron microscopy. Emphasis in our study was on the muscular structure. In most cases, that is in patients with single or duplex, or simple or ectopic ureteroceles, muscle bundles were hardly developed in the dome of the ureterocele but were well developed in the proximal ureter, with and without dilatation. In the dome of the ureterocele muscle cells were much smaller than those in the proximal ureter. Thick and thin myofilaments were demonstrated in muscle cells in the proximal ureter but thick myofilaments were absent in the dome of the ureterocele. These findings may suggest that a ureterocele is a segmental embryonal arrest of the most distal portion of the ureter.