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A one-month-old girl was refereed to our department because of urinary tract infection. Ultrasonography showed grade III left hydronephrosis and cystic mass at the dome of the urinary bladder. Voiding cystourethrography revealed grade 4 vesicoureteral reflux associated with Hutch's diverticulum. In addition, magnetic resonance imaging revealed multiple cysts at the dome of the urinary bladder. The vesicoureteral reflux was repaired and the urachal cyst removed.

Diseases associated with persistent urachus are relatively rare. During the past 9-year period, there have been 14 patients with urachal disease consisting of 10 with urachal abscess and 4 with urachal cancer. The 10 patients with urachal abscess consisted of 7 males and 3 females aged 19-77 years (mean, 46 years). The 4 patients with urachal cancer consisted of 2 males and 2 females aged 48-81 years (mean, 57 years). As symptoms, lower abdominal pain was frequently observed in the patients with persistent urachus with abscess and gross hematuria in those with urachal cancer. Echo and magnetic resonance imaging (MRI) were useful for visualizing the lesion. Computed tomogtaphic (CT) scanning could not visualize the lesion in 2 patients. Nine patients underwent MRI, which visualized the lesion in all of them. As urachal abscess, an umbilical fistula was observed in 3 patients, urachal cyst in 4, and urachal diverticulum in 1. The preoperative diagnosis was urachal cancer in 6 patients, and pathological examination showed 4 patients with adenocarcinoma, 1 with inflammatory granuloma, and 1 with pseudosarcoma. Urachal abscess was treated by resection of the abscess in 6 patients, transurethral resection in 1, and resection of the umbilicus and urachus and total cystectomy in the other. Of the patients with urachal cancer, 1 underwent total cystectomy and the other 3 underwent total urachal resection and partial cystectomy. In 2 patients with persistent urachus with abscess, the differentiation between abscess and malignant tumor was difficult.

We encountered 6 patients with pyourachus (male, 1; female, 5) who ranged in age from 17 to 58 years (mean, 41 years) during the 7 years from 1993 to 1999. Three of them had a history of gynecological surgery, and 2 had a history of appendectomy. Improvement was observed in one patient with conservative therapy alone, but the other 5 patients underwent surgery. Preoperatively, 2 patients underwent drainage, one of them through the umbilicus, and the other through a position on the midline percutaneously. Pathological examination in the 5 patients revealed no evidence of cancer. With reference to postoperative complications, adhesive ileus was recognized in 1 case one year postoperatively but no other complications were noted in the other 5 cases. No evidence of recurrence has been seen in any of these patients to date.

Disorders of urachal remnants are common. While urachal cysts are usually asymptomatic, infection may mimic a variety of acute abdomen. Here we report a very rare case of urachal cyst that protruded in the urinary bladder cavity and among 99 accumulated cases, only 4 cases have been reported similar to this case characterized by intravesical development from 1990 to 1999. An uninfected urachal cyst was found in a 79-year-old male who had died of bile duct carcinoma. The cyst showed ovoid protrusion into urinary bladder cavity from the dome (3.5 x 2.0 x 2.0 cm in size). Histopathologically, the cyst wall was thin and consisted of fibrous connective tissue with muscular tissue and peripheral nerve, and lined by cuboidal epithelium but no inflammatory cells could be seen. Urachal cysts occur in both sexes are affected with equal frequency, and frequently occur in a younger population. In clinical symptoms the umbilical manifestations are predominant in patients younger than 30 years old, while the bladder manifestations are predominant in those older than 30.

A 42-year-old man complained of lower abdominal pain. Computed tomographic scan and magnetic resonance imaging revealed an infected urachal cyst. A drainage catheter, which had multiple holes over a 10 cm length from the catheter tip, was placed in the urachal cyst. The catheter was inserted from the subumbilicus region and the catheter tip was intended to be situated at the caudal end of the urachal cyst. However, the catheter tip accidentally perforated the bladder and urine flowed out of the bladder through the catheter. Because the urine diluted and washed out the pus in the urachal cyst, the infected urachal cyst was successfully drained. Percutaneous drainage and antibiotics allowed resolution of the inflammatory process. On the twenty-third day after catheter placement, excision of the urachal cyst and partial cystectomy were performed with relative ease and without any complications.

We report a case of transitional cell carcinoma of urachus in a 72-year-old man. At follow up cystoscopy for past history of bladder cancer, we found a papillary tumor in the right orifice that came out to the bladder cavity intermittently. Although there was no cancerous lesion on the surface of the bladder mucosa, a submucosal eminence at the dome of bladder was observed. Sagittal magnetic resonance imaging (MRI) revealed an extravesical tumor (2 cm) at the position of urachus. Under the diagnosis of right ureteral cancer and urachal cancer, we performed right distal ureterectomy, ureteral reimplantation and total resection of urachus. Pathological examination revealed transitional cell carcinoma in the urachus and right ureter. The urachal cavity was isolated completely from the bladder cavity. Tumor infiltrated to the muscularis of the bladder dome from the urachal cavity, but there was no cancerous lesion on the surface of the bladder mucosa. Therefore, our diagnosis was urachal transitional cell carcinoma and right ureteral carcinoma.

A 61-year-old man and a 59-year-old woman were referred to our hospital because of lower abdominal pain and discomfort, pollakisuria and a lower abdominal mass. In both patients, radiological studies and cystoscopy caused us to suspect a urachal abscess. We performed operations transperitoneally. In the male patient, fish bones were detected between the mass and ileum. A partial cystectomy was performed on the female patient, and the histological diagnosis was Sparganosis mansoni. In both cases, it was very difficult to make a correct diagnosis before the operations, but surgical treatment was successfully performed.

A 68-year-old man visited our hospital with complaints of abdominal pain and fever. Physical examination disclosed findings consistent with acute abdomen. Computed tomographic (CT) scan revealed a 5 cm cystic mass contiguous with the dome of the bladder and fluid collection in the peritoneal cavity. Cystogram demonstrated deformity of the bladder and no communication between the mass and the bladder. A diagnosis of generalized peritonitis either due to the infected urachal cyst or ruptured bladder was made, and emergency exploratory laparotomy was carried out. Based upon findings consistent with an infected urachal cyst associated with its intraperitoneal perforation, resection of the entire urachal remnant including the dome of the bladder was performed. Pathologic examination showed an acutely inflammed urachal cyst and chronic inflammation of the bladder wall.

A 33-year-old man who had been treated for chronic prostatitis was diagnosed to have urachal cysts by transabdominal ultrasonography. Cystoscopy revealed protuberance at the dome of the bladder. Computerized tomography scan and magnetic resonance imaging showed the mass to be mostly cystic but partly solid. Resection of the urachal cysts and partial cystectomy were performed. Histopathologically, most cysts had a normal cylindrical epithelium with retention of mucinous substance. However, several small cysts contained epithelial cells resembling tubulo-villous adenoma and showing mitotic figures. This case was concluded as urachal carcinoma detected in its very early stage.

Here is presented a surprisingly rare case in a 40-year-old male who had patent vitelline duct by nature. However, his congenital disease appeared deceptively to be an acquired umbilical urachal sinus on the diagnostic evaluations including fistulography before surgery. The diagnosis was definitely confirmed after the successful surgical procedure. The principal reason why these diseases were indistinguishable was reviewed. The incidence of each disease and incidence of association with umbilical fistula in each disease were discussed. With regard to these incidences, we compared urachal anomalies with vitelline duct anomalies through reference of several literatures. This is the most unique event we have ever clinically experienced.

A 52-year-old woman complained of persistent discharge from an umbilicus which she had for 18 years. Abdominal X-ray showed a stone-like shadow measuring 5 x 3 cm at the 5th lumbar spine. Cystoscopic examination revealed a hole at the dome of the bladder and a ureteral catheter was inserted approximately 3 cm through the hole. Computerized tomography scan and magnetic resonance imaging demonstrated a cyst communicating to the umbilicus and bladder. The preoperative diagnosis was a patient urachus associated with stone. Urachal cyst containing a stone was removed and partial cystectomy was performed. The stone was a mixture of magnesium ammonium phosphate and calcium phosphate. Pathological examination showed no evidence of malignancy. More than 210 cases of urachal cyst have been reported in Japan. About 10% of the cases were associated with stones.

A case of vitello intestinal cyst was reported. A 16-month-old girl was referred to our clinic with a complaint of a cystic mass in the region of the navel. With a diagnosis of urachal cyst, resection of the cyst was performed. Histopathologically, the cyst wall consisted of fibrous and fat tissue, and a small polypoid tumor which was found on the inner surface of the cyst was covered by intestinal epithelia. Pancreatic and gastric mucosal elements were observed in the submucosal layer. The histopathological diagnosis was vitello intestinal cyst. Serum amylase level elevated preoperatively normalized after removal of the cyst. We collected 11 cases of vitello intestinal cyst reported in Japan including the present case. Ectopic pancreatic tissue is considered a characteristic of vitello intestinal cyst and that serum or fluid amylase level may be useful for differential diagnosis of the disease.

Case 1: A lower abdominal large painful mass was recognized by palpation, CT scan and ultrasonography in a 64-year-old house wife. Urine cytology was negative. The mass at the dome of bladder was covered with normal epithelium cystoscopically. Aspiration cytology of the lower abdominal mass demonstrated no malignancy and total excision of urachal remnant with a portion of bladder wall was carried out. Histologically, the mass was an urachal cyst with granulomatous change infected with C group beta-streptococcus. Case 2: A 46 year-old male engineer complained of asymptomatic hematuria. Cystoscopic examination revealed a small bleeding lesion at the dome of bladder. Urine cytology was negative. CT scan and ultrasonography revealed a tiny cystic mass lesion with irregular density. Biopsy or aspiration cytology appeared difficult because of the size and localization of the mass. En bloc segmental resection of urachal remnant was carried out. Since intraoperative rapid histological examination of the specimen confirmed no malignancies, dissection of pelvic lymph node was not performed. Urachal cysts presented above were suspicious of malignant degeneration from findings of imaging examination. Either preoperative or intraoperative histological examination in such cases appears to be indispensable to avoid unnecessary extensive operation as well as to perform radical operation required for malignant lesions.

An 8-year-old boy with infected urachal cyst presenting with macroscopic hematuria, pain on urination and high fever attack is reported. He had recurrent episodes of cystitis. At admission, a quail-egg sized solid tumor was palpated in the midline of his lower abdomen. The urine was infected. Ultrasonographic examination revealed an oval tumor 2 x 2.7 cm in diameter at the dome of the bladder with a thin funicular structure running toward the umbilicus. Cystoscopy revealed a round tumor covered with hyperemic epithelia. Transurethral biopsy revealed no malignant lesions of the epithelia. Partial cystectomy with excision of the funicular urachal ligament was performed. The pathological findings were consistent with persistent urachus with chronic inflammation. We reviewed 210 cases of infected urachal cysts reported in the Japanese literature.

A case of an infectious urachal cyst that presented as a lower abdominal mass in a 44-year-old man is reported. There was no urinary tract infection. Cystoscopy revealed the dome retracted. All other investigations were normal without microscopic hematuria. Laparotomy revealed the mass attached to the small intestine because of infection. The mass was excised after partial resection of small intestine and partial cystectomy.

A 24-year-old female with infected urachal cyst is reported. She was admitted to the hospital with complaints of a lower abdominal mass, abdominal pain and cystitis symptom. Brownish pus discharges from her umbilicus were recognized by manual compression of the lower abdominal mass. Cystoscopy revealed a small orifice at the dome of the bladder, and pus discharges from this orifice. A pooling of contrast medium (8 X 2.5 cm) under the umbilicus was detected by a fistelography from the umbilicus, and a low density mass was detected under the abdominal wall between the umbilicus and the dome of bladder on a CT scan. So she was diagnosed as an infected urachal cyst and operated on. The urachal cyst which was adhered to the peritoneum had penetrated both the umbilicus and bladder at the time of operation. Complete removal of the urachal cyst with partial cystectomy was done. We also reviewed the Japanese case reports of urachal cyst.

A case of urachal cyst prolapsed from external orifice in a 15-year-old female is reported. She was admitted to the hospital with complaints of macrohematuria and palpation of a thumb's head size tumor prolapsed from external urethral orifice. An urachal cyst was suspected from the cystogram, CT and the cystoscopy. We extirpated the tumor with the bladder wall. Pathological examination demonstrated a urachal cyst.