Management of Urachal Remnants in Early Childhood

Urachal anomalies are more common than previously thought, with more cases discovered incidentally, because of the increased use of cross-sectional imaging. Although an abnormal persistence of an embryologic communication between the bladder and the umbilicus is often recognized and managed in childhood, it may persist into adulthood, with a greater risk of morbidity. Congenital urachal anomalies that are detected early can benefit from an optimized management including surgical approach with a complete resection of the urachal remnant in cases when spontaneous resolution or medical management has failed. At imaging, the different types of urachal anomalies have a distinct appearance. A patent urachus is recognized as an elongated patent connection between the bladder and the umbilicus. An umbilical-urachal sinus is depicted as a blind focal dilatation at the umbilical end, whereas a vesicourachal diverticulum is a focal outpouching at the vesical end. Urachal cysts are visualized as midline fluid-filled sacs most frequently located near the bladder dome. Complications of urachal anomalies have nonspecific clinical findings and can mimic other abdominal and pelvic processes. Potential complications, such as infection and tumors, should be recognized early to ensure optimal management. Understanding of the embryonic development of the urachus is necessary for the radiologist to diagnose the wide variety of urachal disease. ©RSNA, 2016.

Imaging of the urachus: Anomalies, complications, and mimics

Urachal cancers are uncommon malignancies with a location that often permits considerable local extension before they are discovered. The most common histological type is adenocarcinoma, which may produce mucus that is a valuable aid in diagnosis. The presence of stippled calcification in a midline abdominal wall mass is almost pathognomonic for urachal carcinoma. More commonly, however, the symptoms are less specific, such as hematuria and an abdominal mass. Many lesions are visible endoscopically and, thus, the diagnosis can be made preoperatively from a biopsy. Most treatment failures occur because the tumor is not controlled locally by the initial operation and, therefore, we recommend en bloc cystectomy with umbilectomy and pelvic lymphadenectomy unless the tumor is known to be a sarcoma or early stage (I) carcinoma. If these patients are undertreated and there is a local recurrence then the patient usually is not salvageable. Because of the difficulty in identifying the origin of a bladder adenocarcinoma, any tumor on the dome or anterior wall should be approached initially as if it were a urachal tumor.

Malignant urachal lesions.

The impact of preoperative ultrasound on correct diagnosis of urachal remnants in children

Laparoscopic management of symptomatic urachal remnants in adulthood.

The objective of this study is to define optimal diagnosis and treatment strategies for patients with urachal anomalies in the pediatric age group. The medical records of 21 children who had undergone surgery for urachal anomalies at Severance Hospital, Yonsei University College of Medicine from January 1990 to April 2005 were reviewed. The subjects included 14 males and 7 females (M:F 2:1). The four types of urachal anomalies confirmed were a urachal cyst in 10 patients (47.6%), a patent urachus in 6 (28.6%), a urachal sinus in 4 (19.0%) and a urachal diverticulum in 1 (4.8%) patient. The most common presenting complaint was umbilical discharge (n = 10, 40.0%), followed by abdominal mass (n = 9, 36.0%). Urachal anomalies were diagnosed by ultrasonography in 18 patients, and 7 of them were additionally examined by computed tomography. The remaining patients were diagnosed solely by surgical exploration. Excision was performed in all patients and was supplemented by partial cystectomy in three. Umbilical discharge was the most common clinical manifestation in our patients, suggesting that ultrasonography should be performed in patients with umbilical discharge to differentiate urachal anomalies. We found the most common anomaly to be the urachal cyst, and all patients were successfully treated by surgical excision.

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Youn Joung Choi

Papers

Urachal anomalies in children: A single center experience