Journal of Pediatric Urology (2007) 3, 500e504

EDUCATIONAL ARTICLE

A diagnostic algorithm for urachal anomalies

Jenny H. Yiee a,*, Nilda Garcia b, Linda A. Baker c, Robert Barber b, Warren T. Snodgrass c, Duncan T. Wilcox c
Department of Urology, University of California Los Angeles, 650 Charles Young Drive, Los Angeles, CA 90095-1738, USA b Department of Surgery, University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX 75390-CMCH, USA c Department of Urology, University of Texas Southwestern Medical Center, Childrens Medical Center, 6300 Harry Hines Boulevard #1401, Dallas, TX 75235, USA Received 5 March 2007; accepted 30 July 2007 Available online 10 October 2007
a

KEYWORDS
Urachus; Abnormalities; Radiography; Ultrasonography; Surgery

Abstract Objective: Urachal anomalies are rare. Variable presentations can be a diagnostic challenge. We combine our series with a review of literature to delineate the most common presentations, highest yield diagnostic studies and a diagnostic algorithm. Methods: We reviewed records of 22 boys and 15 girls with the diagnosis of a urachal anomaly from 2000 through 2005. This revealed 19 cysts, seven patent urachuses, ve sinuses, four patients with unspecied urachal anomalies, and two patients with no urachal anomaly by surgical exploration. Ultrasound was the most common diagnostic study, followed by computed tomography (CT), voiding cystourethrogram and sinogram. Thirty-ve of 37 patients underwent surgery. Results: Clinical presentation included periumbilical leakage in 54%, pain in 30%, periumbilical mass in 22% and irritative voiding symptoms in 14%. In many patients diagnosis was made on clinical examination alone. When an ultrasound was used it was diagnostic for 82% of cysts, 100% of sinuses and 100% of patent urachuses. A voiding cystourethrogram was diagnostic for 100% of patent urachuses, but less successful in the other anomalies. CT scans correctly diagnosed 71% of cysts. Overall complication rate was 9%, all wound infections. Conclusion: Reviewing the results of this and four other large series showed that the most common anomaly is the urachal cyst followed by urachal sinus and patent urachus. Periumbilical drainage is the most common presentation. Physical exam alone can be diagnostic. When this is not possible ultrasound is our recommended initial study, followed by a CT scan if unsuccessful. 2007 Published by Elsevier Ltd on behalf of Journal of Pediatric Urology Company.

* Corresponding author: David Geffen School of Medicine at UCLA, Department of Urology, 650 Charles Young Drive, Box 951738, Los Angeles, CA 90095-1738, USA. Tel.: 1 011 310 254 7766; fax: 1 011 310 206 5343. E-mail address: jennyyiee@yahoo.com (J.H. Yiee). 1477-5131/$30 2007 Published by Elsevier Ltd on behalf of Journal of Pediatric Urology Company. doi:10.1016/j.jpurol.2007.07.010

Presentation and diagnosis of urachal anomalies

501 ultrasound indicated that there was no urachal anomaly; this was conrmed at operation performed for continued clinical suspicion, giving a specicity of 100%. A sinogram was diagnostic for a urachal anomaly in the one patient in which it was performed. This patient presented with serous leakage. Seven patients underwent a VCUG; in none was there bladder outow obstruction. Six of the VCUGs were performed for patients with umbilical drainage while one was performed for symptoms of a urinary infection and suspected urachal cyst. Operative conrmation showed a sensitivity of 33% for this modality. VCUG was able to correctly detect all cases of patent urachus, giving a sensitivity of 100% for this subtype. CT scan was performed in eight patients, was diagnostic in six of these, and did not identify the urachal anomaly in two. Eleven patients did not undergo preoperative imaging. Two of these cases were discovered incidentally while undergoing surgery for an unrelated disease. Of the remaining nine patients, ve presented with umbilical drainage and four with a periumbilical mass. All of these cases without preoperative imaging were conrmed to be urachal anomalies intraoperatively. A urachal cyst was found in three, urachal sinus in three, and patent urachus in three. Thirty-ve patients underwent surgical repair or exploration. This resulted in the diagnosis of a urachal cyst in 19 (54.3%) patients, a patent urachus in seven (18.9%), a urachal sinus in ve (13.5%), urachal anomaly not otherwise specied in two (5.7%), and no surgically apparent urachal anomaly in two (5.7%) (Table 2). Two patients did not undergo surgical exploration due to resolution of symptoms in one and loss to follow up in the other. Two patients underwent exploration, but were found to have no apparent urachal anomaly to explain preoperative symptoms. One patient had presenting symptoms of a cystic periumbilical mass while the other presented with abdominal pain and possible serous umbilical drainage. Preoperative imaging consisting of MRI in one and ultrasound in the other were negative for any urachal anomalies. The median presenting age for a urachal cyst was 3.4 years. Median presenting age for a urachal sinus was 0.3 years. Median presenting age for a patent urachus was 0.3 years. Of the 35 patients who underwent surgical excision, three (8.6%) had complications, all due to wound infection.

Introduction
During gestation, the urachus represents the connection between the dome of the bladder and the allantoic duct. Normally the lumen obliterates into a brous tract during embryologic development. A urachal anomaly occurs when obliteration is incomplete thereby leaving a persistent lumen. This can occur along the whole tract, resulting in a patent urachus, or it can occur along part of the tract creating a urachal sinus, cyst or diverticulum [1]. Screening studies have shown that some urachal anomalies are asymptomatic; however, the majority present clinically with leakage, signs of infection or a palpable mass [2]. We reviewed the Childrens Medical Center of Dallas experience and present a concise review of the diagnosis, presentation, and role of radiographic imaging in urachal anomalies.

Materials and methods

We performed a retrospective review of cases from the Departments of Urology and Surgery at the Childrens Medical Center of Dallas from August 2000 through March 2006 with the diagnosis of urachal anomaly (ICD 753.7). This identied 37 patients: 22 boys and 15 girls aged 1 week to 20 years (mean 4.0 years, median 1.0 years). Thirty-ve of the 37 patients underwent surgical exploration and repair as conrmation of their diagnosis. Diagnostic imaging included ultrasound, VCUG, CT, MRI, sinogram and cystoscopy. These ndings comprise the practices of 14 adult and pediatric urologists and general surgeons. Additionally, we reviewed the literature for the last 10 years for case series with greater than 20 patients in which presentation, diagnostic and surgical data were given. We compared these ndings with our own case series.

Results
Thirty-seven patients were identied. The most common presentations in these patients can be divided into two groups. Group 1 presented with uid leakage from the umbilicus, 14 with clear and six with cloudy. Group 2 presented with periumbilical mass (8), non-specic abdominal pain (4) and urinary symptoms (3). Additional clinical symptoms are shown in Table 1. In two patients the urachal anomaly was discovered incidentally during operations for other procedures (Table 1). Our most common diagnostic imaging modality was ultrasonography in 21 patients. Twelve of these patients presented with umbilical leakage. Operative conrmation of diagnosis showed a sensitivity of 82%. In one patient the
Table 1 Distribution of presenting clinical symptoms Total Little et al. [9] Cilento et al. [8] Mesrobian et al. [7] Present series Total 56 45 21 37 159 Drainage 24 19 12 20 75 (43) (42) (57) (54) (47) Pain 8 10 4 11 33

Discussion
The incidence of a persistent urachal remnant varies within the literature. While one autopsy study reported 32% of adult patients with tubular urachal structures [3], others have reported less than two cases per 100,000 hospital

Cyst/mass 8 15 0 8 31 (14) (33) (0) (22) (19)

Erythema 24 0 4 2 30 (43) (0) (19) (5) (19)

UTI/dysuria 0 1 1 5 7 (0) (2) (4) (14) (4)

(14) (22) (19) (30) (21)

Values are number of patients with percentage of total in brackets.

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Table 2 Distribution of types of urachal anomalies Total No. of patients Cilento et al. [8] Yiee Mesrobian et al. [7] Total 45 31 21 97 Urachal cyst No. (%) 16 19 9 44 (36) (61) (43) (45) Urachal sinus No. (%) 22 5 9 36 (49) (16) (43) (37) Patent urachus No. (%) 7 7 2 16 (15) (23) (10) (16)

J.H. Yiee et al.

Urachal diverticulum No. (%) 0 0 1 1 (0) (0) (4) (1)

and surgical admissions [4e6]. More recently, Ueno et al. reported a screening ultrasound series of 3400 children and found a 1.6% rate of detected urachal anomalies; 71% of those detected were symptomatic [2]. Combining data from three large series in the last 10 years shows the most common type of urachal anomaly to be cysts (45%), followed by sinuses (37%), then patent urachuses (16%) (Table 2). Urachal diverticuli are rare and were not observed in two of the three series. Our series agrees with the literature in nding periumbilical drainage to be the most common presentation [7e9]. Other presentations, including abdominal pain, periumbilical mass, periumbilical erythema and urinary symptoms, are summarized in Table 1. The majority of patients with voiding symptoms presented with concurrent abdominal pain or umbilical leakage, which helped in distinguishing the presentation of a urachal anomaly from a simple urinary infection. The differential diagnosis for this constellation of presenting symptoms includes anomalies of the vitelline ducts, appendicitis, omphalitis, and granulation tissue from an umbilical stump. In the differential diagnosis of abdominal pain, appendicitis is relatively common, while anomalies of the vitelline ducts are less common with a 2% overall incidence. The most common vitelline duct anomaly is a Meckels diverticulum [10]. At our institution, the incidence of Meckels diverticulum exceeds that of all urachal anomalies combined by 50%. A patent omphalomesenteric duct is less common with an incidence of 0.3% [11]. In our series, the average age of presentation was highest for a cyst and lowest for a patent urachus. All of our patent urachus diagnoses were in infants under the age of 1 year. The only anomalies to present over the age of 5 years were cysts. Diagnosis can be made by clinical signs and symptoms alone, but commonly radiographic imaging is used to aid in diagnosis. We found an ultrasound to be diagnostic in 82% of urachal cysts, 100% of patent urachuses and 100% of sinuses. In all patients with non-diagnostic ultrasounds and surgically conrmed urachal anomalies, preoperative CT scan and MRI were diagnostic in the face of nondiagnostic ultrasounds. All of our patients with diagnoses made on imaging were conrmed by surgical exploration. Both of our surgical explorations that revealed no apparent urachal anomalies followed preoperative diagnostic studies that were negative for any urachal anomalies. While Cilento et al. reported that ultrasound correctly diagnosed 100% of urachal cysts, they found a lower diagnostic rate for urachal sinus (50%) and patent urachus (33%) [8]. They recommended sinogram for all patients with drainage given their 100% detection rate in diagnosing sinuses with this technique. In contrast, McCollum et al.

reported successful diagnosis with 91% of their performed ultrasounds [12]. They noted that none of their diagnostic VCUGs and sinograms added new information. Little et al. concur with using ultrasound as diagnostic modality of choice, and reported that little new information was gained from additional VCUGs and CT scans [9]. Often diagnosis can be made by physical exam alone, especially when there is persistent leakage as seen in 20 of our patients. All of our cases diagnosed by physical exam alone were conrmed surgically to be urachal anomalies. The practice at our institution has evolved such that we currently do not obtain radiographic imaging when the diagnosis is not in doubt. This can be especially evident with presenting symptoms such as periumbilical drainage and mass. Other general symptoms such as abdominal pain and urinary infection still warrant imaging. When imaging is needed, given the high detection rate of ultrasound for all presentations of urachal anomalies, we recommend this as the initial radiographic study (Fig. 1). If ultrasound is nondiagnostic and periumbilical drainage is present, a sinogram can be performed. Alternatively, a sinogram rather than an ultrasound can be performed as the initial diagnostic study in all patients presenting with drainage. This would differentiate between a urachal sinus, a patent urachus and a patent omphalomesenteric duct. If ultrasound is non-diagnostic and there is no drainage, CT scan should be the next diagnostic study. This would differentiate between a urachal cyst and other intra-abdominal differential diagnoses such as appendicitis and Meckels diverticulitis.

Suspected Urachal Anomaly

Ultrasound

Diagnostic

Non-Diagnostic

Drainage

No Drainage

Sinogram

CT Scan

Surgical Excision

Figure 1

Diagnostic algorithm.

Presentation and diagnosis of urachal anomalies VCUG has been recommended to rule out obstruction as well as aid in diagnosis. The exact cause and effect relationship of urinary obstruction and patent urachus is unclear; however, there has historically been reported a 14% rate of association between patent urachuses and urinary obstruction [3]. Review of the literature and of our series questions this quoted rate. In the ve largest symptomatic series in the last 10 years, totaling 167 patients, no cases of urinary obstruction were found. The most common method of repair remains excision of the tract via an infraumbilical incision. A laparoscopic approach has been established in adults and more recently tried in children [13e15]. McCollum et al. advocated a twostep repair in the case of infected urachal anomalies, in which incision and drainage of abscess is later followed by denitive excision of the anomaly [12]. They found a 40% complication rate in patients who underwent a one-step repair versus no complications in those who underwent such a two-step repair. All of our patients underwent a one-step open repair regardless of preoperative infection, although some underwent a delayed repair following antibiotic administration. In our 36 operative patients, we experienced three (9%) cases of wound infection. This compares with 5e7% in the literature. Two of these patients presented preoperatively with evidence of infection, while the third patient was a premature newborn admitted for failure to thrive whose skin dehiscence likely reected nutritional status rather than an infective complication. The treatment of incidental urachal anomalies remains controversial. Ueno et al. [2] followed 36 patients with incidental urachal anomalies found by ultrasound. None developed further symptoms, suggesting that excision to prevent future symptoms, i.e. infections, is not warranted. The most compelling justication for excision remains the potential for malignant transformation. Though rare, urachal adenocarcinomas have a poor prognosis. A recent population-based study by Pinthus et al. [16] reported a 10-year disease specic survival rate of 49.2%. Upadhyay and Kukkady performed histologic examinations of excised symptomatic and incidental urachal remnants in children ranging from 4 days to 16 years old [17]. They found a 50% incidence of abnormal mucosal linings in their incidentally found urachal remnants. Whether these abnormal linings represent metaplastic change with malignant potential is unknown. Consequently, we do not excise asymptomatic urachal remnants. Exceptions include a remnant found intraoperatively where the risks of surgery have already been undertaken or a remnant of large size.

503 A VCUG has traditionally been used to exclude urinary obstruction; however, this is not considered necessary as obstruction is exceedingly rare. 1. Which are the three most common types of urachal anomalies in descending order? a. Patent urachus, urachal cyst, urachal sinus b. Urachal cyst, urachal sinus, patent urachus c. Urachal sinus, urachal diverticulum, urachal cyst d. Urachal cyst, patent urachus, urachal diverticulum 2. A 4-week-old newborn presents with clear umbilical drainage; which of the following is the next step in management? a. CT scan b. Re-examination in 1 month c. Surgical exploration d. VCUG 3. What is the most common complication in urachal anomaly excision and repair? a. Persistent drainage/tract b. Urinary tract infection c. Wound infection d. Malignant degeneration 4. Which symptom listed below is the most common presentation of urachal anomalies? a. Abdominal mass/cyst b. Abdominal pain c. Umbilical drainage (serous or purulent) d. Umbilical erythema 5. What is the embryological function of the urachus? a. Artery b. Vein c. Fecal conduit d. Urinary conduit 6. In adults, malignant transformation of urachal anomalies leads to which type of cancer? a. Transitional cell carcinoma b. Adenocarcinoma c. Squamous cell carcinoma d. Myosarcoma This article can be used to gain CME credits. To gain these credits, please visit the website at www.eu-acme.org and click on the button Journal of Pediatric Urology where these multiple choice questions may be answered and the answers scored accordingly.

References Conclusion
Urachal anomalies remain a rare nding. The most common type of anomaly is a cyst or sinus followed by a patent urachus. A urachal diverticulum is the least common nding. Presenting symptoms include periumbilical drainage, pain and palpable mass. When clinical presentation is clear, such as with periumbilical drainage, we proceed directly to surgery. When diagnostic conrmation is needed, we recommend an ultrasound as the initial diagnostic study. This can be followed by a sinogram if drainage is present or CT scan if drainage is not present.
[1] Moore K, Persaud T. Before we are born: essentials of embryology and birth defects. Philadelphia: WB Saunders; 1998. [2] Ueno T, Hashimoto H, Yokoyama H, Ito M, Kouda K, Kanamaru H. Urachal anomalies: ultrasonagraphy and management. J Pediatr Surg 2003;38:1203e7. [3] Schubert E, Pavkovic B, Bethke-Bedurftig A. Tubular urachal remnants in adult bladder. J Urol 1981;13:40e9. [4] Nix J, Menville M, Wendt D. Congenital patent urachus. J Urol 1958;79:264e73. [5] Blichert-toft M, Nielsen O. Congenital patent urachs and acquired variants. Acta Chir Scand 1971;137:807e14. [6] Rubin A. A handbook of congenital malformations. Philadelphia: Saunders; 1967.

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[7] Mesrobian H, Zacharias A, Balcom A, Cohen R. Ten years of experience with isolated urachal anomalies in children. J Urol 1997;158:136e1318. [8] Cilento B, Bauer S, Retik A, Peters C, Atala A. Urachal anomalies: dening the best diagnostic modality. Urology 1998;52:120e2. [9] Little D, Sohail R, Peter SS, Calkins C, Murphy J, Gatti J. Urachal anomalies in children: the vanishing signicance of the preoperative voiding cystourethrogram. J Pediatr Surg 2005;40:1874e6. [10] Yahchouchy E, Marano A, Etienne J, Fingerhut A. Meckels Diverticulum. J Am Coll Surg 2001;192:658e62. [11] Nursal T, Yildirim S, Tarim A, Noyan T. Laparoscopic resection of patent omphalomesenteric duct in an adult. Surg Endosc 2002;16:1638.

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