Urachal diseases are rare and may develop from a congenital anomaly in which a persistent or partial reopening of the fetal communication between the bladder and the umbilicus persists. The most frequently reported urachal anomalies in adults are infected urachal cyst and urachal carcinoma. The diagnosis of this entity is not always easy because of the rarity of these diseases and the atypical symptoms at presentation. Imaging techniques, such as ultrasonography and computed tomography have a significant role in recognizing the presence of urachus-derived lesions.
Case presentation 1: A 25-year-old Arab-Berber man presented with a 10-day history of progressive lower abdominal pain accompanied by fever, vomiting, and low urinary tract symptoms to our emergency department. Laboratory data revealed leucocytosis. The diagnosis of an acute peritonitis was made initially. Abdominal ultrasonography revealed a hypoechoic tract from the umbilicus to the abdominal wall, and the diagnosis was rectified (infected urachal remnants). The patient was initially treated with intravenous antibiotics in combination with a percutaneous drainage. Afterwards an extraperitoneal excision of the urachal remnant including a cuff of bladder was performed. The histological analysis did not reveal a tumor of the urachal remnant. Follow-up examinations a few months later showed no abnormality.
Case presentation 2: A 35-year-old Arab-Berber man, without prior medical history with one week of abdominal pain, nausea and vomiting, associated with fever but without lower urinary tract symptoms visited our emergency department. Laboratory data revealed leucocytosis. Abdominal ultrasonography was not conclusive. Computed tomography of the abdomen was the key to the investigation and the diagnosis of an abscess of urachal remnants was made. The patient underwent the same choice of medical-surgical treatment as previously described for case one, with a good follow-up result.
Case presentation 3: A 22-year-old Arab-Berber man, with no relevant past medical history, presented to our emergency department because of suspected acute surgical abdomen. Physical examination revealed umbilical discharge with erythema and a tender umbilical mass. Abdominal ultrasonography and computed tomography scan confirmed the diagnosis of infected urachal sinus. Initial management was intravenous antibiotics associated with a percutaneous drainage with a good post-operative result, but a few days later, he was readmitted with the same complaint and the decision was made for surgical treatment consisting of excision of the infected urachal sinus. The clinical course was uneventful. Histological examination did not reveal any signs of malignancy.
We describe our clinical observations and an analysis of the existing literature to present the various clinical, radiological, pathological and therapeutic aspects of an abscess of urachal remnants. To the best of our knowledge, this manuscript is an original case report because this atypical presentation is rarely reported in the literature and only a few cases have been described.
Urachus; Bladder; Neoplasms; Urachal cyst; Urachal remnant; Urachal sinus; Abcess
Abdominal tuberculosis (TB) is an uncommon affliction in adolescence. It is usually associated with pulmonary tuberculosis. The disease is caused by lymphohaematogenous spread after primary infection in the lung or ingestion of infected sputum and has a typically protean and nonspecific presentation. The occurrence of TB in an urachal remnant is probably from the contiguous spread of an abdominal focus or mesenteric lymph node. Urachal TB is a rare entity, with only two reported cases in the literature. We report here a case of clinically silent pulmonary and abdominal TB that manifested in the infection of an urachal sinus and highlight the role of laparoscopy in its diagnosis and treatment.
A 14-year-old boy presented to our institution with peri-umbilical swelling and purulent discharge from his umbilicus for 2 weeks duration. There were no radiological, microbiological or clinical evidences of TB in the initial presentation, though he had close social contact with someone who had TB. A computed tomography scan of the abdomen confirmed the diagnosis of an urachal abscess. An incision and drainage procedure was performed followed by a course of antibiotics. A scheduled laparoscopic approach later showed that the peritoneum and serosal surface of the small and large intestines were studded with nodules of variable sizes, in addition to the urachal sinus. The histology of the resected tissues (urachal sinus and nodules) was consistent of TB infection. He recovered fully after completing 6 months of anti-tuberculous therapy.
This report highlights a rare case of TB urachal abscess in an adolescent boy, the difficulties in the diagnosis of abdominal tuberculosis, the need to consider TB as a cause of urachal infection in endemic areas and the use of laparoscopy in both diagnosis and treatment.
Tuberculosis; Urachal sinus; Urachal abscess; Infection; Laparoscopy
Urachal cysts are rare congenital anomalies that often prompt referral to the paediatric general surgeon because of their associated complications such as infection, abdominal pain and the young age at presentation. In this report we describe a rare case of fever of unknown origin caused by an urachal cyst which was successfully treated with incision and drainage only. Since the first description of urachal anomalies by Cabriolus in 1550, few cases have been reported and, until now, only one case of infected urachal cyst presenting as fever of unknown origin has been described in the literature. Moreover, the spontaneous resolution of an urachal cyst without excision is extremely rare.
We report our experience in the management and treatment of an infected urachal cyst that occurred in a 12-year-old Caucasian girl who presented to our Department of Paediatric Surgery with a 30-day history of evening fever. The urachal cyst was treated only with incision and drainage through a minimally invasive laparoscopic approach.
The incision and drainage of an infected urachal cyst is a simple and safe procedure. It assures a complete recovery and avoids potential surgical complications related to the total excision of the urachal cyst. This report may provide important clues regarding the management of this rare anomaly and we emphasise the importance for paediatricians, who should consider the possibility that a fever of unknown origin can be caused by an urachal cyst, and for surgeons and urologists, because it suggests that conservative treatment of this rare anomaly should be considered when possible.
Incision and drainage; Laparoscopic approach; Regression of urachal cyst; Treatment of infected urachal anomalies; Urachal cyst
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.
Urachal anomaly; children; ultrasonography; surgical excision
Urachal cyst is one of a spectrum of urachal abnormalities most commonly found in children. They are very rarely seen in adults because the urachus is normally obliterated in early infancy.
We describe a case of a 32 year old male Caucasian who presented with a tender, midline, infraumbilical mass and purulent umbilical discharge. Diagnosis of an infected urachal cyst was confirmed on magnetic resonance scan. He was treated initially with broad spectrum antibiotics in order to allow sepsis to resolve prior to surgical excision of the cyst and fibrous tract. Cystoscopy was performed intraoperatively to exclude sinus communication with the bladder. Histology of the excised specimen showed chronic inflammation with no evidence of malignancy. Postoperative recovery was uneventful.
Urachal abnormalities are rare in adults. Clinical presentation is non-specific; therefore, a high index of suspicion is required in order to make the diagnosis. When diagnosed, surgical excision is advised because of the risk of malignant transformation.
The urachus is a fibrous cord that arises from the anterior bladder wall and extends cranially to the umbilicus. Traditionally, infection has been treated using a two-stage procedure that includes an initial incision and drainage which is then followed by elective excision. More recently, it has been suggested that a single-stage excision with improved antibiotics is a safe option. Thus, we intended to compare the effects of the two-stage procedure and the single-stage excision. We performed a retrospective review on nine patients treated between May 1990 and September 2005. The methods used in diagnosis were ultrasonography, computed tomography (CT), magnetic resonance imaging (MRI), and cystoscopy. The study group was comprised of three males and six females with a mean age of 28.2 years (with a range from three to 71 years). Symptoms consisted of abdominal pain, abdominal mass, fever, and dysuria. The primary incision and drainage followed by a urachal remnant excision with a bladder cuff excision (two-stage procedure) was performed in four patients. The mean postoperative hospitalization lasted 5.8 days (with a range of three to seven days), and there were no reported complications. A primary excision of the infected urachal cyst and bladder cuff (single-stage excision) was performed in the other five patients. These patients had a mean postoperative hospitalization time of 9.2 days (with a range of four to 15 days), and complications included an enterocutaneous fistula, which required additional operative treatment. The best method of treating an infected urachal cyst remains a matter of debate. However, based on our results, the two-stage procedure is associated with a shorter hospital stay and no complications. Thus, when infection is extensive and severe, we suggest that the two-stage procedure offers a more effective treatment option.
Urachal cyst; infection
An infected urachal cyst is one of a spectrum of presentations of urachal pathology, all of which are rare in adulthood.
PRESENTATION OF CASE
We report the case of a 45-year-old obese Russian lady who presented with a 2-week history of suprapubic pain radiating to the right iliac fossa. Although previously fit and well, she had a history of 17 miscarriages. Both USS and CT suggested a complicated inflammatory mass in the lower abdomen. Ultimately the diagnosis was made by laparotomy, which revealed an abscess of an urachal cyst. The infected cyst and bladder dome were excised. The patient made a good recovery with an uneventful follow up.
Urachal cysts are the commonest type of urachal anomaly. Infection is the usual mode of presentation amongst adult cases otherwise the condition usually remains asymptomatic. An infected urachal cyst is an important diagnosis to make as complications include sepsis, fistula formation, and rupture leading to peritonitis. Treatment is by complete excision, however, techniques have been debated.
This is a rare but important diagnosis however we recommend that in patients with atypical histories, it should be included in the differential diagnosis.
Urachus; Urachal cyst; Infected urachal cyst
A 59-year-old woman presents with decreased appetite and abdominal pain. Her symptoms lead to lethargy and weakness. Abdominal pain is a common presentation in the primary care and emergency room setting. She was initially diagnosed with an abscess and treated with antibiotics and drainage. Upon further evaluation and cystoscopy she was discovered to have a urachal cyst. Urachal cysts are extremely rare and even more uncommon in adults, as it is usually diagnosed in children. It is an important diagnosis not to miss in the differential of adult abdominal pain as surgical intervention is often necessary for treatment. This case highlights urachal cyst as a rare and serious differential of adult abdominal pain.
Urachal affections are rare. Their variable ways of presentation may represent a diagnostic challenge. Urachal sinuses are a rare type of these abnormalities. They are usually incidental findings and remain asymptomatic unless a complication (most commonly the infection) occurs. Infection of the urachal sinus would clinically present as purulent umbilical discharge, abdominal pain, and periumbilical mass. We report herein a case of infected urachal sinus in male adult. The diagnosis was suspected clinically and confirmed with ultrasonography and computed tomography scan. A preoperative cysto-fibroscopy showed normal aspect of the bladder and excluded sinus communication. An initial broad spectrum antibiotic therapy followed by complete excision of the sinus and fibrous tract without cuff of bladder has been therefore performed. The postoperative course was uneventful. No recurrence was observed after 18 months of followup. Histological examination did not reveal any sign of malignancy.
The urachus is the remnant of the cloaca, which in adults attaches the bladder dome to the umbilicus. After birth it obliterates and presents as the midline umbilical ligament. Patent urachal anomalies are usually detected in childhood. In adults they occur very rarely and the presentation and diagnosis may be occasionally challenging. We present and discuss the case of an infected urachal cyst found in a 30–year–old adult.
urachus; abdominal pain; antibiotics; laparotomy
The urachus is a duct connecting the allantois with the fetal bladder, forming the median umbilical ligament; it usually obliterates during fetal life. Carcinomas arising from urachal remnants are rare but associated with a poor prognosis. We present one case of non-invasive urachal papillary urothelial carcinoma, and through a systematic literature search, we identified 12 additional cases of urachal urothelial carcinoma reported in English literature in the past 20 years. The cases were compared according to the Sheldon Staging System and the Mayo Staging System presented by Ashley et al in 2006, and both Staging Systems tend to predict clinical outcome. The urachal carcinoma is an important differential diagnosis in patients presenting with haematuria or an infraumbilical mass, because the symptoms may be sparse and diagnosis at an early stage is essential for successful treatment.
Urachus anomalies are remnants of the urachus, an embryonic connection between the fetal bladder and umbilicus. These anomalies usually remain asymptomatic but can cause symptoms in case of infection. An extremely rare complication of symptomatic urachal remnants is fistulizing to adjacant viscera. We describe the first case of diverticulitis associated sigmoid-urachal-cutaneous fistula.
An 88-year-old male visited the outpatient clinic with intermittent bleeding and gassy discharge from his umbilicus. Examination showed a normal looking umbilicus with a small fissure at the bottom in which a 10 cm probe could be inserted. Laparotomy revealed a fistula extending from the umbilicus to a pus filled cyst superior to the bladder. A second fistula between the cyst and a mid-sigmoid diverticulum was identified. The involved sigmoid segment was resected. Pathological examination demonstrated no malignancy.
Urachal remnants are extremely rare in adults. They typically present with abdominal pain or palpable masses. Additional imaging can aid the diagnostic process.
Due to the risk of recurrence and malignant transformation complete surgical excision of urachal anomalies is the treatment of choice. This can be done in a 1-step or 2-step procedure.
Cyst; Diverticulitis; Fistula; Sigmoid; Urachus; Laparatomic surgery
Abnormalities of the urachus, the vestigial remnant of the allantois, result when the embryonic lumen fails to completely obliterate during fetal development. In adults, urachal abnormalities are most commonly masses, with urachal adenocarcinoma representing the most frequent etiology. Due to the low incidence of urachal masses, guidelines for diagnostic workup and treatment are based off of a limited body of evidence comprised primarily of case reports and retrospective series. We present the case of a fifty-two-year-old woman with a urachal mass. Full radiologic workup consisting of computed tomography, cystoscopy and ultrasonography is included, and the risk factors, treatment and prognosis are discussed.
Urachal mass; Fistula; Diabetes; Cystectomy; Urachal abscess; Urachal adenocarcinoma
Adenocarcinomas of the urinary bladder are rare (1-5% of bladder tumours) and of notoriously poor prognosis. About one third of such tumours arise in urachal remnants related to the bladder. This is believed to be the first report of in situ change in the urachal remnant. The patient presented with mucusuria and computed tomography showed a typical urachal cyst. After excision the cyst was found to contain mucinous adenomatous epithelium but without invasion of the basal lamina. Pathological stage is the best prognostic indicator in urachal tumours. Prompt investigation and management of mucusuria may allow the diagnosis of urachal tumours in this preinvasive stage.
Urachal carcinomas occur mostly in the bladder dome, comprising 22 to 35% of vesical adenocarcinomas, and are generally treated by partial cystectomy with en bloc resection of the median umbilical ligament and umbilicus. Detailed pathologic studies with clinical outcome correlation are few.
We reviewed histologic material and clinical data from 24 cases selected from a database of 67 dome-based tumors diagnosed and treated at our institution from 1984 to 2005. Follow-up information was available for all 24 patients.
The mean age at diagnosis was 52 years (range 26-68). 15 patients were male and 9 were female. Location was the dome in 23, and dome and anterior wall in 1. Thirteen cases were pure adenocarcinoma, NOS, 9 were enteric type adenocarcinoma and 2 were adenocarcinoma with focal components of lymphoepithelioma-like carcinoma and urothelial carcinoma with cytoplasmic clearing. Signet ring cell features were focally seen in 2 cases. Cystitis cystica and cystitis glandularis were seen in 4 and 2 cases, respectively. In all instances but one, cystitis cystica/ glandularis was focal and predominantly in the bladder overlying the urachal neoplasm. Urachal remnants were identified in 15 cases: the urachal epithelium was benign urothelial-type in 6 cases and showed adenomatous changes in 9. The overlying bladder urothelium was colonized by adenocarcinoma in 3 cases. In all three, urachal remnants were identified and showed transition from benign to adenomatous epithelium. On immunohistochemistry, these tumors were positive for CK20 and variably positive for CK7 and 34BE12. The majority showed a cytoplasmic membranous staining pattern for beta-catenin, although in 1 case, focal nuclear immunoreactivity was identified.. The Sheldon pathologic stage was pT1 in 0, pT2 in 2, pT3a in 8, pT3b in 11, pT3c in 1, pT4a in 1 and pT4b in 1 patient. One patient had a positive soft tissue margin. The mean follow-up period was 40 months (range 0.3-157.6). Seven of 24 (29%) cases recurred locally. The incidence of local recurrence was higher in patients who underwent a partial cystectomy alone (37.5%) versus those who had a more radical surgery (27%). Distant metastases occurred in 9 (37.5%) patients, 4 of which had no prior local recurrence. Seven patients (29%) died of the disease. All cases with locally recurrent and metastatic disease belonged to stage pT3 or higher.
Pathologic stage is an important prognostic factor in urachal carcinoma. Surface urothelial involvement by carcinoma and presence of cystitis cystica/ glandularis do not necessarily exclude the diagnosis of urachal carcinoma. Immunostains do not unequivocally discriminate a urachal from a colorectal carcinoma, but diffuse positivity for 34BE12 would support, and diffuse nuclear immunoreactivity for beta catenin would militate against, a diagnosis of urachal carcinoma.. Local recurrence may be due to seeding within the distal urothelial tract, particularly in tumors with a configuration that is polypoid and which open into the bladder cavity. The type of surgery performed may have an effect on local recurrence despite negative margins of resection.
Urachal remnant disease is uncommon in adults and can present with symptoms ranging from drainage near the umbilicus to a severe abdominal infection. Most cases are referred for treatment once diagnosed either clinically or radiographically with ultrasound or computerized tomography. We present a unique case of an infected urachal cyst visualized on a series of CT scans in an adult patient with abdominal pain over a period of years.
An 18-year-old female patient with Crohn's disease had abdominal pain secondary to a retained pill camera. After several weeks of medical management, the camera spontaneously passed. However, the patient also had an intra-abdominal abscess that worsened, despite medical therapy. Surgical therapy was recommended and a 5cm infected urachal cyst with entero-urachal and vesico-urachal fistulas was discovered. An en-bloc resection of the entire area was performed to include the urachal cyst, the adherent portion of the dome of the bladder, and 15cm of associated ileum. The bladder was repaired, a suprapubic catheter was placed, and an ileo-ileal anastamosis was performed. Microscopic findings were consistent with active Crohn's disease and fistula formation. The entero-uracho-vesical fistula site was likely the site of the retained pill camera. The patient did well postoperatively and was discharged on postoperative day six without complications.
Crohn's Disease; Capsule Endoscopy; Pill Camera; Retained Pill Camera; Entero-Urachal Fistula; Entero-Uracho-Vesical Fistula
The urachus is a vestigial structure between the dome of the bladder and the umbilicus. Tumors may develop from the remnants, most of which are well-differentiated, mucinous adenocarcinomas. Urachal adenocarcinoma is an exceedingly rare type of tumor.
We present a case of a 51-year-old female presenting to our institution with complaints of abdominal pain for 36 hours. The patient was taken to the operating room for an acute appendicitis. Laparoscopy was performed, and gross purulence and appendiceal perforation were noted as well as a mass on the anterior abdominal wall. Based on the location of the mass, we converted to an open midline laparotomy to treat both the perforated appendicitis and to remove the mass.
Pathology confirmed the diagnosis of perforated appendicitis and a mucinous-producing urachal adeno-carcinoma.
Data support both open and laparoscopic approaches for appendicitis. This case, although rare, highlights the importance of laparoscopy in a complete and thorough examination of the abdominal cavity. A standard right lower quadrant incision for an open technique would likely have resulted in omission of this lesion, and the patient would have presented at a more typical late stage of her cancer development with significantly more morbidity.
Urachal adenocarcinoma; Ruptured appendicitis; Laparoscopy
Stone-containing urachal cysts are extremely rare in adults. Here, we report the case of a 58-year-old man with a urachal cyst who had lower abdominal pain and urinary frequency. Abdominal ultrasonography and computed tomography showed hyperdense stones in the urachus. He was treated with a laparoscopic excision using a transperitoneal approach. The pathological diagnosis was an inflammed urachal cyst. This rare case illustrates an inflammed urachal cyst containing stones treated with laparoscopy.
Urachal cyst; stone; laparoscopy
The urachus is a vestigial structure located between the dome of the bladder and the umbilicus, which results from the involution of the allantoic duct and the cloaca. Persistence of an embryonic urachal remnant can cause various problems during childhood and young adulthood. Urachal leiomyoma is a rare entity with very few cases being reported in literature. It can be misdiagnosed and confused with a wide spectrum of intra-abdominal or pelvic disorders. We hereby report a case of angiomatous leiomyoma originating from the urachal remnant in a 45-year-old lady, masquerading as extraluminal gastrointestinal stromal tumor. Understanding the embryological basis of these urachal disorders and their imaging features coupled with histopathological examination is crucial for the correct diagnosis and management. Pathological diagnosis is required to optimize the surgical approach and preclude unnecessary radical surgery.
Urachus; leiomyoma; angiomatous
Urachal adencarcinoma is rare and its metastasis to the ovary is extremely rare. I report here on a case of urachal adenocarcinoma that metastasized to bilateral ovaries in a 72-year-old female. She presented with vaginal spotting. Abdominal CT revealed a huge multiloculated cystic mass in the recto-uterine pouch and a solid mass with dot calcification in the anterior pelvic cavity. The resected ovaries were equal-sized at about 10cm at the greatest diameter. The sectioned surfaces were predominantly multicystic with a solid nodule. Microscopically, both ovaries were mainly composed of cystic glands lined by mucin-containing epithelium with atypical nuclei. The solid nodule consisted of irregularly infiltrating glands and single tumor cells. Two years later, the patient was admitted with hema-turia. The kidney CT revealed a solid mass with calcification in the bladder dome, which suggested urachal carcinoma. The partial cystectomy specimen revealed an ill-defined ulcerative tumor. Histologically, the tumor corresponded to mucinous adenocarcinoma and centered at the bladder wall with predominant invasion of the muscularis. The immunohistochemical profiles of the ovarian and urachal tumors were exactly the same. The tumor cells were diffusely positive for CK20, CDX-2, MUC2 and MUC5AC, focally positive for 34(3E12 and negative for CK7.
Urachus; ovarian neoplasm; metastasis; mucinous; adenocarcinoma
Urachal carcinoma is an uncommon neoplasm associated with poor prognosis.
A 45-year-old man was admitted with complaints of abdominal pain and pollakisuria. A soft mass was palpable under his navel. TC-scan revealed a 11 × 6 cm tumor, which was composed of a cystic lesion arising from the urachus and a solid mass component at the urinary bladder dome. The tumor was removed surgically. Histological examination detected poor-differentiated adenocarcinoma, which had invaded the urinary bladder. The patient has been followed up without recurrence for 6 months.
The urachus is the embryological remnant of urogenital sinus and allantois. Involution usually happens before birth and urachus is present as a median umbilical ligament. The pathogenesis of urachal tumours is not fully understood. Surgery is the treatment of choice and role of adjuvant treatment is not clearly understood.
Urachal cyst is an unusual clinical condition, which is usually asymptomatic. In some adult cases, it may lead to complications. The cyst is between umbilicus and urinary bladder. It is diagnosed via ultrasonography and computed tomography. However, in some cases, the diagnosis is made by means of surgical exploration and histopathological evaluation. In this paper, we report a case of a 17-year-old female presenting with painful abdominal mass. At the first evaluation, the case was diagnosed as a mesenteric cyst because the mass located in the mesentery, and final histopathological report revealed the urachal cyst.
Urachal cysts present very uncommonly in adults. We describe a 25-year-old man who attended with a discharging umbilical swelling and pain. Ultrasonography and CT scan demonstrated an infected urachal cyst which, following cystoscopy, was excised without complication.
The urachus is an embryologic remnant which degenerates after the birth. Defective obliteration of the urachus leads to urachal abnormalities. An infected urachal cyst is one of the urachal abnormalities and this pathology is rare in adult women. We report a case of 33-year-old woman with pelvic pain and dysuria who was diagnosed with infected urachal cyst. Infected urachal cyst is a rare pathology in adult women and this pathology should be considered in the differential diagnosis of acute abdomen.