Current management of the urachal anomalies (UA). Lessons learned from the clinical practice.

PURPOSE: It has been suggested that symptomatic UA requires surgical excision. However, the management of asymptomatic urachus is still controversial. We aimed to evaluate the clinical presentation, the efficacy of current modalities used, and postoperative pathology in patients with UA. MATERIALS AND METHODS: We have performed a retrospective review of all patients diagnosed with UA and treated surgically or conservatively over 18 years. Demographic data, clinical presentation, imaging modalities, pathology, treatment, and postoperative complications were analyzed. RESULTS: Twenty-five symptomatic patients (18 males and seven females) with a median age of 13 years (1 month to 37 years) were identified. 15 (60%) were diagnosed with a urachal cyst, 4 (16%) with sinus, 3 (12%) with urachal diverticulum, and the remaining 3 (12%) with patent urachus. Of those, 20 (80%) underwent surgical repair, and the remaining five (20%) patients were managed conservatively. 4 (20%) underwent laparotomy, 7 (35%) laparoscopic incision, and the remaining 9 (45%) laparoscopic robotic-assisted surgery. Nine patients required bladder cuff excision. The median operative time was 75 min (42-140 min). One patient developed Clavien-Dindo grade IIIA complication resulting in infected hematoma, which resolved after drainage. Another patient with a complication of grade IIIB needed reoperation as a result of recurrent events of an abscess. 13 (65%) demonstrated epithelium lining of the urachus on postoperative pathology. CONCLUSIONS: Our data show that most of the patients with UA presented with epithelial lining, which might lead to the later malignant transformation. It might cause a shift from the conservative management of asymptomatic patients to surgical intervention. Robotic-assisted surgery appears beneficial in these patients, especially when the bladder cuff excision is required.

Are urachal remnants really rare in children? An observational study.

Urachal remnants are thought to have a low prevalence. However, recent studies indicate a higher rate. The aim of this study was to evaluate the prevalence of urachal remnants in children referred for imaging by abdominal/urinary/suprapubic ultrasonography in one calendar year. Files of children who underwent abdominal/urinary/suprapubic ultrasonography in one calendar year were retrospectively reviewed. Data regarding children ≤17 years that underwent ultrasonography of the ventral abdominal wall while being assessed for various reasons were collected. Anomalies detected, age, gender, reason for ultrasonography request, and diagnosis of the urachal remnant were noted. All ultrasonography assessments were performed by a single pediatric radiologist in a single institution. There were 4836 patients in the study (1919 boys). Median age of the whole cohort was 7 years. A total of 10 patients were found to have urachal remnants, which included eight urachal cysts (three boys) and two urachal diverticula (one boy).Conclusion: The prevalence of urachal remnants in our cohort was nearly 2 in every 1000 children. More specifically, the prevalence of urachal cysts was one in 600. What is Known: • Urachal remnants were believed to be rare. • Recent studies indicated a higher incidence than previous reports. What is New: • This study shows that urachal remnant may be observed in one every 500 children that undergo ultrasonography of the abdomen for various reasons. • Parental counselling and management of urachal remnants should be planned accordingly.

A rare case of eosinophilic cystitis involving the inside and outside of the urinary bladder associated with an infected urachal cyst.

BACKGROUND: Eosinophilic cystitis is a rare inflammatory disease of the bladder characterized by eosinophilic infiltration of the bladder wall. Most Eosinophilic cystitis cases present with mucosal lesions of the urinary bladder. We present a very rare case of large mass-forming eosinophilic cystitis, involving the inside and outside of the bladder associated with an infected urachal cyst. CASE PRESENTATION: A 59-year-old man presented with gross hematuria, fever, dysuria, and suprapubic pain. Computed tomography showed a heterogeneously enhancing mass that measured 7.6 cm × 4 cm located on the anterosuperior portion of the bladder with an internal fluid collection. Cystoscopy revealed a raspberry-like mass lesion on the bladder dome. Transurethral resection of the bladder was initially performed. The mass lesion protruding from inside the bladder was removed, and pus-like fluid was drained. The pathologic diagnosis was eosinophilic cystitis. Follow-up computed tomography showed a remnant mass outside the bladder and urachal cyst. To eliminate the remnant lesion, robot-assisted partial cystectomy was performed. The patient showed no evidence of recurrent disease on follow-up cystoscopy and computed tomography for up to 2 years. CONCLUSIONS: Clinicians should consider the possibility of eosinophilic cystitis in patients who present with hematuria, fever, and suprapubic pain and have both intravesical and extravesical masses.

Bladder prolapse through a patent urachus presenting as an umbilical mass in the newborn: characteristic prenatal sonographic findings and the diagnostic benefit of postnatal cystography.

Bladder prolapse through a patent urachus is rare. We present a newborn with an unusual exophytic, erythematous umbilical mass. Voiding cystography readily demonstrated continuity of the bladder dome with the umbilical mass, confirming bladder prolapse through a patent urachus. The diagnosis of bladder prolapse was rapidly made in a second newborn with similar imaging and clinical findings and confirmed by cystography. We discuss the embryology of this condition including the association with a vesico-allantoic cyst in utero. Pre- and postnatal images are presented. The use of cystography in diagnosis is emphasized.

Identification of an Infected Urachal Cyst Using Point-of-Care Ultrasound.

ABSTRACT: This case describes a 6-year-old girl who presented to the pediatric emergency department with 3 days of fever and suprapubic pain in the setting of 1 month of worsening, dull abdominal pain. On presentation, she had a tender, erythematous, and fluctuant mass on her lower abdomen. Point-of-care ultrasound was used to identify an abnormal fluid collection anterior to her bladder, suspicious for an infected urachal cyst. In this case, point-of-care ultrasound helped identify this uncommon finding in a timely fashion, which expedited definitive care and prevented unnecessary exposure to ionizing radiation.

An Unusual Case of Meckel Diverticulitis Misdiagnosed as an Infected Urachal Cyst.

Introduction: Meckel's diverticulum (MD), a remnant of the omphaloenteric duct, is among the most frequent intestinal malformations. Another embryonic vestige is the urachus, which obliterates, becoming the median umbilical ligament; the failure of this process can lead to a urachal cyst formation. We present a case of Meckel diverticulitis misdiagnosed as an infected urachal cyst. Presentation of case: A 16-year-old girl presented with hypogastric pain, fever and vomiting. She had undergone an appendectomy 6 years prior and no digestive malformation had been documented. In the last 2 years, she had 3 events of urinary tract infections with Escherichia coli, and anabdominal ultrasound discovered a 28/21 mm hypoechogenic preperitoneal round tumor, anterosuperior to the bladder. We established the diagnosis of an infected urachal cyst, confirmed later by magnetic resonance imaging. Intraoperative, we found MD with necrotic diverticulitis attached to the bladder dome. Discussions: Meckel's diverticulum and urachal cyst (UC) are embryonic remnants. Both conditions are usually asymptomatic, being incidentally discovered during imaging or surgery performed for other abdominal pathology. Imaging diagnosis is accurate for UC, but for MD they are low sensitivity and specificity. For UC treatment, there is a tendency to follow an algorithm related to age and symptoms, but there is no general consensus on whether to perform a routine resection of incidentally discovered MD. Conclusion: Preoperatory diagnosis of MD represents a challenge. We want to emphasize the necessity of a thorough inspection of the small bowel during all abdominal surgical interventions and MD surgical excision regardless of its macroscopic appearance. These two actions seem to be the best prophylaxis measures for MD complications and consequently to avoid emergency surgery, in which case more extensive surgical procedures on an unstable patient may be needed.

Prenatally diagnosed patent urachus with umbilical cord cyst and early surgical intervention.

OBJECTIVE: Description of rare diagnosis of patent urachus. DESIGN: Case report. SETTING: Department of Obstetrics and Gynecology, 2nd Faculty of Medicine and Faculty Hospital Motol Prague. CASE REPORT: Patent urachus is a rare diagnosis, which in this case was detected prenatally by ultrasound. Involution of the urachus is not fully completed upon birth, therefore in cases of small persisting communication between the urinary bladder and the umbilicus conservative approach and waiting for spontaneous closure is usually chosen. In our case surgery treatment has chosen as a prevention of urinary infection because of patent urachus manifested as a wide communication. CONCLUSION: This congenital defect usually manifests itself early after birth as a visible structural anomaly of the umbilicus and/or as urine leakage in the umbilicus opening area. It is important to keep in mind that urachus irregularities may be accompanied by other urinary system defects. Every child presenting with such an anomaly should therefore be thoroughly examined. If the procedure is performed by an experienced surgical team postoperative complications are uncommon and overall long-term prognosis for patients is excellent.

Management of symptomatic urachal cysts in children.

AIM: We report the results of the surgical treatment of symptomatic urachal cysts. MATERIALS AND METHODS: The medical records of patients who underwent urachal cyst excision between 2012 and 2017 were reviewed retrospectively at our hospital. The age, sex, presenting complaint, method of diagnosis, average cyst diameter, surgical procedure, and postoperative complications of each patient were recorded. RESULTS: Twenty-seven patients who had urachal cyst were included in this study; 5 out of 27 patients were treated conservatively and the rest of patients were treated surgically, made up of 16 males (72%) and 6 females (28%). The average age of the patients was 7 years (range: 1-17). The most common reason for referral was abdominal pain in 12 patients (54%), discharge in 6 patients (28%), fever in 2 patients (9%), and an abdominal mass in 2 patients (9%). An ultrasound scan was performed in all patients as an initial imaging study. The average cyst diameter was 1.5 cm (range: 1-6 cm). Laparotomy was performed in 16 patients, with 6 patients undergoing laparoscopic excision. Postoperative wound infection developed in two patients. CONCLUSIONS: Patients with urachal cysts may be managed conservatively initially. However, patients who do not show any clinical and radiological signs of regression, or those who have large cysts, should undergo surgical excision through laparotomy or a laparoscopic approach.

Sonographic detection of an infected maternal urachal cyst during pregnancy.

Infection of a maternal urachal cyst during pregnancy is rare; Sonography is an important diagnostic tool that can help minimize maternal and fetal complications. We describe the case of a 35-year-old multiparous woman presenting in the third trimester with 2 weeks of fever, abdominal pain, and urinary symptoms. Imaging showed a 5-cm complex anterior midline mass, found intraoperatively to be eroding into the uterus. Sonographic imaging aided in the diagnosis and management of the urachal cyst, and antepartum sonographic measurements of the lower uterine segment helped to counsel regarding a trial of labor. Following treatment, the patient stabilized and had an uncomplicated vaginal delivery.

[Laparoscopic Resection of Urachal Cysts]. / Die laparoskopische Resektion einer Urachuszyste.

Objective This video is a step-by-step description of the laparoscopic technique for the resection of urachal cysts. Indication The urachus is an embryonic extraperitoneal structure between the urinary bladder and the umbilicus. Failure of the allantois to obliterate in utero leads to urachal anomalies such as fistulas or cysts. Because of the risk of infection and malignant degeneration later in life, urachal fistulas or cysts are usually removed by surgical excision. Method The procedure is performed in supine position with 3 mm trocars in the epigastric angle, the left upper abdomen and the left middle abdomen. During the operation, the trocar in the epigastric angle will be upsized to 5 mm in order to introduce a 5 mm polymer clip applier. We use a 5 mm 30° optic and a 3 mm monopolar hook for dissection. Conclusion This laparoscopic approach to removing urachal remnants allows for the involved structures to be clearly identified and accurately dissected. A complete resection of urachal remnants is facilitated by direct visualization of the entire anomaly down to the bladder dome.

Imaging of the Urachus: Anomalies, Complications, and Mimics.

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.

Ultrasound evaluation of urachal abscess in a young infant.

The urachus is a tubular structure extending from the dome of the bladder to the umbilicus. Normally, this allantoic and cloacal remnant obliterates into a fibrous band by late fetal development. Urachal abnormalities include a patent urachus, urachal cyst, umbilical urachal sinus, and a vesicourachal diverticulum. These abnormalities are most often asymptomatic and only come to clinical attention when associated with infection. A 2-month-old male infant presented to our tertiary pediatric hospital with several days of intermittent fevers and a focal region of swelling and erythema over the umbilicus. On physical examination, a tender mass was palpated in the umbilical region. Ultrasound revealed a long tubular structure coursing from the dome of the bladder anteriorly and superiorly to the subumbilical region. This tubular structure was fluid filled with internal echoes suggestive of infection with abscess formation. The urachal abscess was drained surgically, at which time a patent urachus was also excised. This case demonstrates the importance of having a thorough understanding of urachal anomalies when evaluating infants presenting with umbilical pathology.

Comprehensive imaging review of abnormalities of the umbilical cord.

A complete fetal ultrasonographic (US) study includes assessment of the umbilical cord for possible abnormalities. Knowledge of the normal appearance of the umbilical cord is necessary for the radiologist to correctly diagnose pathologic conditions. Umbilical cord abnormalities can be related to cord coiling, length, and thickness; the placental insertion site; in utero distortion; vascular abnormalities; and primary tumors or masses. These conditions may be associated with other fetal anomalies and aneuploidies, and their discovery should prompt a thorough fetal US examination. Further workup and planning for a safe fetal delivery may include fetal echocardiography and karyotype analysis. Doppler US is a critical tool for assessment and diagnosis of vascular cord abnormalities. US also can be used for follow-up serial imaging evaluation of conditions that could result in fetal demise. Recent studies suggest that three- or four-dimensional Doppler US of the fetal umbilical cord and abdominal vasculature allows more accurate diagnosis of vascular abnormalities. Doppler US also is invaluable in assessment of fetal growth restriction since hemodynamic changes in the placenta or fetus would appear as a spectral pattern of increased resistance to forward flow in the fetal umbilical artery. Early detection of umbilical cord abnormalities and close follow-up can reduce the risk of morbidity and mortality and assist in decision making.

Umbilical cord cysts in the first trimester: are they associated with pregnancy complications?

OBJECTIVES: To determine whether umbilical cord cysts found by transvaginal sonography in the first trimester of pregnancy are associated with poor pregnancy outcomes. METHODS: We conducted a matched cohort study between July 2006 and July 2008. Patients with umbilical cord cysts found on transvaginal sonography in the first trimester were matched to patients with normal umbilical cords. After the completion of these pregnancies, medical histories and pregnancy outcomes were reviewed from the hospital's electronic record. Sonograms were reviewed to obtain descriptive information about the umbilical cord cysts. Outcomes between the cohorts were compared. RESULTS: Forty-five patients with umbilical cord cysts were identified and compared to 85 patients with normal umbilical cords. The mean gestational age of the cysts ± SD at diagnosis was 8 weeks 3 days ± 3.5 days. The mean cyst diameter was 3 ± 2.1 mm. All cysts resolved on follow-up sonography, which was performed between 9 weeks 4 days and 20 weeks 5 days. Patients with umbilical cord cysts were found to have a lower body mass index than those with normal umbilical cords. There was no significant difference in abnormal sonographic findings between cohorts. Five sonographic fetal abnormalities were found in the umbilical cord cyst cohort (11.1%) and 8 in the normal umbilical cord cohort (9.4%). There were 2 intrauterine fetal demises in the umbilical cord cyst cohort and 1 in the normal umbilical cord cohort. There was no difference between the cohorts when comparing gestational age at delivery and birth weight. CONCLUSIONS: There does not appear to be an association between poor pregnancy outcomes and umbilical cord cysts during the first trimester.

Imaging features of intravesical urachal cysts in children.

BACKGROUND: Urachal cysts, part of the spectrum of congenital urachal anomalies, are typically extrinsic to the urinary bladder. OBJECTIVE: The purpose of this study is to present the salient imaging features of a pediatric series of unusual intravesical urachal cysts that protrude into the bladder lumen. MATERIALS AND METHODS: Five children with intravesical urachal cysts depicted on imaging studies were retrospectively identified during a 6-year period at a children's hospital. The clinical charts and findings on ultrasound (US) and voiding cystourethrogram (VCUG) were reviewed. RESULTS: In all five children, US revealed a thin-walled ovoid cystic structure containing anechoic fluid or echogenic debris and residing along the midline of the anterosuperior aspect of the urinary bladder protruding into the bladder lumen. Histological examination of the partial cystectomy specimen from one child revealed a cystic urachal remnant with intestinal mucosal lining and reactive lymphoid hyperplasia. The cysts in the four other children were managed conservatively without operative intervention. CONCLUSION: The purpose of this report is to expand the spectrum of urachal remnant anomalies to include these newly recognized intravesical urachal cysts, which are characterized on US by the presence of a thin-walled cyst along the midline anterosuperior aspect of the urinary bladder.

Intravesical urachal cyst masquerading as a bladder malignancy: a case report.

BACKGROUND: Urinary bladder masses in children are extremely rare. Certain benign conditions (e.g., ureterocele) can mimic malignant bladder masses. In this report, we present a unique case of a urachal cyst masquerading as a bladder malignancy. Unlike the typical location of urachal cysts along the course of the urachal tract, the cyst in this case was unexpectedly situated within the urinary bladder, leading to diagnostic difficulties. CASE PRESENTATION: A 2-year-old Bahraini boy presented with hematuria and dysuria for 2 weeks. There was no history of fever, abdominal pain, or vomiting. Physical examination yielded normal findings. Urinalysis showed numerous red blood cells and revealed positive results for nitrites and leukocyte esterase. Abdominal ultrasound showed a well-defined soft tissue lesion with internal vascularity located at the apex of the urinary bladder. Subsequently, magnetic resonance imaging demonstrated a thick-walled cystic structure arising from the anterosuperior wall of the bladder and protruding into its lumen. The patient underwent complete excision of the bladder lesion for the presumed diagnosis of rhabdomyosarcoma. Histopathological examination showed a fluid-filled space lined by stratified squamous epithelium with areas of intestinal metaplasia, revealing an unexpected diagnosis of a urachal cyst. The patient was discharged with complete resolution of symptoms. CONCLUSIONS: Intravesical urachal cysts are a rare type of congenital urachal anomaly that may simulate a bladder malignancy, particularly if associated with infection. This case emphasizes the importance of considering urachal cysts in the differential diagnosis of bladder masses, especially in children, and specifically when the lesion is midline in the anterosuperior wall of the bladder.