Management of urachal anomalies in pediatric patients: A comparison of treatment strategies between pediatric urology and general surgery.

INTRODUCTION: Persistence of embryonic urachal structures due to a failure of the urachus to involute into the median umbilical ligament is known as a urachal anomaly (UA). UAs may remain asymptomatic or lead to abdominal pain and recurrent infections. Management of UAs in pediatric patients has historically lacked a clear consensus between conservative and surgical management. While both urologists and general surgeons manage this pathology, a comparison of management style and outcomes between these specialties has not been published to our knowledge. OBJECTIVE: To (1) evaluate trends in management of UAs among pediatric urologists and general surgeons across three tertiary care children's hospitals and (2) identify factors that place patients at higher risk for requiring surgery. STUDY DESIGN: All patients diagnosed with a UA from 2016 to 2020 at our multi-site institution were identified by ICD-10 code Q64.4 "malformation of the urachus" and retrospectively reviewed. Patient demographics, treatment specialty, remnant subtype, and management strategy were recorded. Data was dichotomized between both urology and general surgery as well as between surgical and nonsurgical intervention to identify and compare management strategies. RESULTS: Overall, 143 patients diagnosed with UAs were identified. Of these patients, 74 were treated by urology and 69 were treated by general surgery. Patients who were treated by urology were significantly more likely to receive conservative treatment (66.2% treated conservatively vs. 33.8% treated surgically), while patients treated by general surgery were significantly more likely to undergo surgery (84.1% treated surgically vs. 15.9% treated conservatively, p < .0001). Though, urology was more likely to treat patients who presented incidentally (p < .01), and general surgery was more likely to treat patients who presented with an infected remnant (p < .01). Patients of male sex were more likely overall to receive surgery compared to female patients (p < .01). DISCUSSION: Management of UAs by urologists was more conservative than general surgeons. However, both specialties treat distinctly different patient presentations, with urology managing more incidental remnants and general surgery operating on more emergent, infected urachi. Limitations of the study included its retrospective nature and the insufficient reporting of urachal remnant subtypes and presence of infection among patients. CONCLUSIONS: Management strategies of UAs differ among urology and general surgery, but surgical and conservative treatments are necessary to appropriately treat their distinct patient populations. This study provides valuable insight into current practices of UA management and may help to inform future treatment.

Surgical treatment of urachal remnants in an adult population-a single-centre experience.

BACKGROUND: Urachal remnants are a rare congenital defect resulting from failure of obliteration of a fibrous tube that connects the umbilicus to the bladder dome during embryological development. Oftentimes a urachal remnant will go undiagnosed, but occasionally a patient may present with a variety of symptoms, ultimately leading to the identification of the remnant. Given its rarity, there is very limited literature available on the management of symptomatic urachal remnants, especially in adults. Surgical resection has been the first-line management of urachal remnants for years, especially given the risk of the development of urachal adenocarcinoma secondary to recurrent infection, persistent irritation, and urinary stasis associated with some urachal remnants. AIM: We present our experience in the management of symptomatic urachal remnants in adults at our institute and perform a brief literature review of the same. METHODS: A retrospective review of all cases who underwent surgical management of symptomatic urachal remnants between December 2015 and January 2022 was performed. Seven cases of urachal remnant excision in total were identified over the time period. Patient characteristics and perioperative parameters were analysed. Post-operative complications were measured in accordance with the Clavien-Dindo grading system. RESULT: In total, 7 cases of urachal remnants were treated at our institute over the study period. Four patients were treated with a TURBT and 3 patients were treated with a laparoscopic partial cystectomy. There were no intraoperative complications and one post-operative complication requiring readmission for intravenous antibiotics. There was one mortality but this was not as a direct result of the operative procedure. Mean length of stay was 1.71 days. Two of patients had histologically confirmed urachal adenocarcinoma and the remaining five patients had benign histology. Each patient was seen in the outpatients department 6 weeks post-operatively for clinical review and review of histology. No further follow-up was required for the patients with benign histology given resolution of symptoms and follow-up for the malignant histology was arranged appropriately following MDM. CONCLUSION: There is a paucity of data available on the management of urachal remnants in the adult population; however, an endoscopic or laparoscopic approach is a safe and effective method of excising symptomatic urachal remnants.

Giant umbilical cord in a normal preterm infant: a case report and review of the literature.

BACKGROUND: Giant umbilical cord, defined as a cord diameter of more than 5 cm, is an extremely rare malformation. There are few case reports of giant umbilical cord often associated with patent urachus duct or cystic malformation. These cases are usually managed by surgical excision and repair of patent urachus or cyst resection. CASE PRESENTATION: We report the case of a 1-day-old Iranian boy with giant umbilical cord detected postnatally. The pregnancy course was uneventful, except for preterm premature rupture of the membrane and preterm delivery. There was no relevant family history. The patient was delivered by vaginal delivery with a good Apgar score. On clinical examination, the umbilical cord was very thick (about 6 cm in diameter), and huge fluctuating Wharton's jelly was observed. Other organs were normal. During the hospital stay, the patient did not develop any complications except borderline hyperbilirubinemia, which improved with conventional phototherapy. Since the umbilical cord had no discharge and was dried, the newborn was discharged with advice for cord drying care. CONCLUSION: The newborn was well, and the dried umbilical stump was detached after 32 days, leaving a granulomatous structure without discharge. The patient was followed up for 4.5 months and had no problems except delayed separation of the umbilical cord.

Prenatal Detection of Vesico-Allantoic Cyst: Ultrasound and Autopsy Findings.

Introduction: The vesico-allantoic cyst is a communication between the fetal bladder and the allantois through a patent urachus.Case Report: We describe a 17-week of gestational age (WGA) fetus with a 40 x 30 mm vesico-allantoic cyst. At 19 WGA, ultrasound (US) detected bilateral dilatation of renal pelvis (5-6 mm), hydroureters, and hypospadias. Amniotic fluid, umbilical cord flow, and fetal biometry were regular. Due to uncertain prognosis, the parents opted for legal termination of pregnancy. Autopsy confirmed the prenatal findings, also revealing intestinal malrotation and Meckel's diverticulum.Discussion/Conclusion: Probably an initial urinary tract obstruction occurred, not yet affecting the amniotic fluid volume, but evident as pyelectasis. This case highlights the possibility that genito-urinary and intestinal anomalies may be found in association with the vesico-allantoic cyst.

Urachal remnants: from embryology to clinical practice.

The urachus is a tubular structure that is apparent on the third week and connects the ventral cloaca to the yolk sac, as a progression from the allantois. Following the normal regression procedure, the urachus remains as the median umbilical ligament. Urachal remnants are present in 1.03% of paediatric patients while in 92.5% of cases represent incidental findings. Urachal anomalies are classified in four types as patent urachus (50-52%), urachal sinus (15%), urachal cyst (30%) and urachal diverticulum (3-5%). Ultrasound scan is the most commonly performed diagnostic imaging study. In case of symptomatic urachal remnants, surgical excision is indicated. Asymptomatic urachal remnants that are diagnosed at the neonatal period or early infancy should be watched up to 6 months of age, as they are likely to resolve. In persistent or symptomatic urachal remnants there is a risk of inflammation or even malignancy development, therefore we believe that there is indication for preventive surgical excision that may be performed either open or laparoscopically or by robot-assisted laparoscopy.

Prenatally Ruptured Patent Urachus: A Case Report and Review of Literature.

Patent urachus is a type of urachal anomaly in which the urachus does not tail off but remains connected to the bladder in the umbilicus. The prevalence of patent urachus is very low. Herein, we report a case of patent urachus ruptured and exposed to amniotic fluid in utero. In this case, the size decreased after the second trimester, which was thought to be due to rupture in utero. After delivery, patent urachus was confirmed by inserting a foley catheter, which runs through a ruptured cyst on umbilical cord insertion. The day after delivery, the neonate underwent surgical excision of the urachal cyst and closing umbilicus. The mechanism of patent urachus rupture is unknown. As the fetus matures, it is thought that the higher intravesical pressure may affect the rupture of the cyst. Patent urachus could be ruptured in the uterus spontaneously, and surgical correction is needed. Therefore, prenatal differential diagnosis is important.

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.

Successful treatment of a patent urachus concurrent with pyocele in a newborn: A case report.

RATIONALE: A patent urachus is a rare congenital anomaly that atypically presents as an umbilical cord cyst or large umbilical cord. Here we describe a case of a giant umbilical cord cyst in a newborn diagnosed as a patent urachus. PATIENT CONCERNS: A male infant with a birth weight of 3260 g was transferred because of an antenatally diagnosed giant umbilical cord cyst accompanied by yellowish discharge and granulation in the umbilical cord after birth. DIAGNOSES: Patent urachus. INTERVENSIONS: The patent urachus was treated by excision of the urachal remnant followed by partial cystectomy. OUTCOMES: Postoperative orchitis with pyocele occurred and was treated with a course of antimicrobial therapy; and no other complications developed. LESSONS: Newborns with a giant umbilical cord or umbilical cord cysts should be examined for possible accompanying urachal anomalies, even if antenatal ultrasound shows no other suspicious findings, to prevent delayed diagnosis and subsequent complications.

Persistent omphalomesenteric duct and urachus presenting as an umbilical hernia.

Vitellointestinal duct anomalies, although one of the most frequent malformations to be found (2%-3% in population), they are most unlikely to cause symptoms. A persistent Vitellointestinal duct can induce abdominal pain, bowel obstruction, intestinal haemorrhage and umbilical sinus, fistula or hernia which commonly occurs in children. Patent vitellointestinal duct or persistent omphalomesenteric duct is a very unusual congenital anomaly which occurs in 2% of the population related to the embryonic yolk stalk. Similarly, urachal anomalies remain a rare finding, with the most common being a cyst or sinus followed by patent urachus and rarely a urachal diverticulum. Presenting symptoms include periumbilical discharge, pain and a palpable mass.Here, we report a case of an adult patient with patent vitellointestinal duct and urachus identified intraoperatively on diagnostic laparoscopy when being operated for umbilical hernia repair.

Quistes de uraco en adultos. Presentación de 3 casos y revisión de la literatura / Three cases of urachal cyst in adults

El uraco es un grueso cordón fibroso que aparece a partir del quinto mes de gestación como consecuencia de la obliteración de la alantoides. Los quistes de origen uracal se producen como consecuencia de un defecto en la obliteración de dicho conducto, anomalía que se detecta principalmente en niños y raramente en adultos. Presentamos 3 casos de quistes uracales en adultos, uno de ellos detectado durante el estudio de dolor abdominal y los otros 2 hallados de forma incidental durante el estudio de otras patologías. En ningún caso se sospechó clínicamente la posibilidad de quistes uracales. Histológicamente, estas lesiones se encuentran revestidas por epitelio de tipo urotelial con expresión de CK7, CK20, CK5/6, P63 y GATA3. El diagnóstico de certeza recae en el estudio histopatológico donde la morfología, la inmunohistoquímica y un adecuado correlato clínico-patológico, permiten diferenciarlo de otras lesiones quísticas mucho más frecuentes en el adulto.(AU)

The urachus is a thick fibrous cord that appears in the fifth month of pregnancy as a result of the allantois obliteration. Urachal cysts occur as a result of a defect in the obliteration of the duct, anomaly detected mainly in children and very rarely in adults. We present three cases of urachal cysts in adults, one of them detected during the study of abdominal pain and the other two, found incidentally during the study of other pathologies. In any case the possibility of urachal cysts was clinically suspected. Histologically, these lesions are lined by epithelium of urothelial type with expression of CK7, CK20, CK5/6, P63 and GATA3. The diagnosis of urachal cysts certainty lies in the histopathological study where the morphology, immunohistochemistry and a proper clinical-pathological correlation, allow to differentiate it from other more frequent abdominal cystic lesions in adults.(AU)

Case of Infected Urachal Cyst in an Adult Presenting as an Incarcerated Umbilical Hernia.

The urachus is a remnant of the allantois. Failure to obliterate can result in one of four anomalies, urachal cyst being most common. Urachal cysts are relatively rare, especially in adults. This paper presents a patient with an umbilical hernia and a ruptured urachal cyst. A 39-year-old male presented with concern for umbilical hernia, but he also noted drainage. Computed tomography scan showed a urachal cyst and umbilical hernia. The urachal cyst was excised and umbilical hernia closed primarily. The incidence of an urachal cyst is unknown, but persists in roughly 2% of adults. Diagnosis is with ultrasound or CT scan. Management is excision due to risk of urachal carcinoma, which is present in over 50% of specimens. Review of literature did not reveal any other cases of a patient with both an urachal cyst and an umbilical hernia, thus making this case a unique presentation for this condition.

Urachal Pathology: Review of Cases.

INTRODUCTION: The urachus is an embryologic remnant which is formed from the obliteration of the allantois. Urachal abnormalities are caused when defective obliteration of the urachus happens. They are an infrequent condition. Incidence is estimated to be between 5,000 and 8,000 live births. Its diagnosis and management remain a challenge due to the lack of an specific clinical picture and the controversy about the management. OBJECTIVE: The objective of this study is to assess the clinical presentation, diagnosis, therapeutic management, and outcomes of urachal anomalies in our health area. MATERIALS AND METHODS: We performed a retrospective review of all cases of urachal anomalies recorded Tenerife (southern health area), La Gomera, and El Hierro Islands during a 5 year period. RESULTS: Twenty-three cases of urachal pathology were included. The mean age of presentation was 32 years old. 73.9% were male. 65% were diagnosed in adults. In 30.3% of the cases, it was a casual finding. Symptoms included fever, umbilical exudate, hematuria, abdominal pain, and umbilical granuloma. The main diagnostic tests were ultrasound and computed tomography scan. Treatments were selected conservative management (43.5%), selective resection, partial cystectomy, and radical cystectomy. All patients had a good evolution. However, 2 cases where benign tumors were suspected, clinically, had a final histology of cancer in the specimen. CONCLUSIONS: Due to the lack of a specific clinical picture and undefined findings in image tools, diagnosis is difficult and it may be inaccurate. Despite more data are needed, our results suggest that the systematic excision of urachal lesions could result in safer outcomes since cases where a benign lesion is clinically suspected might result in malignant tumors.

Cystic dilatation of umbilical cord associated with patent urachus.

An urachus is a remnant of allantois in embryo development constituting a connection between the dome of the bladder and umbilicus. It develops on day 16 after conception and closes during the pregnancy to form the median umbilical ligament. Patent urachus results from a failure in closing its lumen in 10-12 gestational weeks. This anomaly occurs in 1-2 babies in 100,000 births. We present the case of patent urachus. In 20 gestational weeks, a dilatation of the umbilical cord with an anechoic mass with a transverse dimension of 19 x 12 mm starting from the fetal insertion and length of 30 cm was seen on ultrasound without any other fetal and placental disorders. Histology showed cystic edema. Prenatal diagnosis of patent urachus can be difficult because this pathology may be mistaken with other, more dangerous causes of cord cysts; thus, the occurrence of cord cysts should be closely monitored.

Congenital Bladder Prolapse Through a Patent Urachus: Two Institutions' Experience.

We describe our experience in 2 institutions handling bladder prolapse through a patent urachus (PU), together with a brief review of published literature. Case 1: A term neonate with congenital prolapsed bladder via PU. Ultrasound at 21 weeks gestation revealed a male fetus with a large midline pelvic cyst communicating with the bladder which disappeared on subsequent 27 weeks ultrasound. Case 2: A term female neonate with congenital prolapsed bladder via PU with no prenatal diagnosis. In both cases the bladder closure was undertaken during the newborns' first days of life.

Transumbilical laparoendoscopic single-site surgery versus conventional laparoscopic surgery for patients with symptomatic urachal remnants: an experience with 57 patients.

PURPOSE: To evaluate the surgical feasibility and postoperative cosmesis of a novel transumbilical laparoendoscopic single-site (LESS) surgical approach involving Y-shaped incisions and three-flap umbilicoplasty in urachal remnant patients. METHODS: Patients with symptomatic urachal remnants who underwent either conventional laparoscopic surgery (CL group, n = 21) or LESS surgery with Y-shaped incisions and three-flap umbilicoplasty (LESS group, n = 36) between May 2010 and September 2019 were retrospectively assessed. Perioperative factors and postoperative esthetic outcomes were compared between the groups using univariate and multivariate analyses. Esthetic outcomes were assessed using the body image questionnaire consisting of the body image scale (BIS) and the cosmetic scale (CS); a higher score indicated a better outcome. RESULTS: The median operative time was greater and the insufflation time was shorter in the LESS group than in the CL group. The estimated blood loss and postoperative hospital stay and surgical site infection rate did not differ significantly between the groups. While the BIS score also did not differ significantly between the groups, the CS score was greater in the LESS group than in the CL group. Multivariate analysis revealed that the surgery type (LESS surgery) was an independent predictor of greater postoperative esthetic satisfaction. CONCLUSION: Transumbilical LESS surgery with Y-shaped incisions and subsequent three-flap umbilicoplasty is feasible in patients with symptomatic urachal remnants. Regarding postoperative cosmesis, the higher CS score suggests that this technique is superior to CL surgery. Furthermore, the selection of this procedure was an independent predictor of good postoperative esthetic outcomes.

Evaluation and management of urachal remnants in children.

BACKGROUND: This study aimed to evaluate the diagnosis and treatment of pediatric patients with urachal remnants. METHODS: Medical records of patients diagnosed with urachal remnants between 2015-2019 were evaluated retrospectively. Age, gender, admission complaints, diagnosis, size of cysts, the complications that developed, and treatment modalities were recorded. RESULTS: The study population consisted of 15 cases including seven girls. Most of the cases were asymptomatic. The urachal remnants were detected by ultrasonography. The most common symptom in symptomatic patients was abdominal pain and umbilical discharge. Three patients required surgery and most of them were conservatively followed up. Complication, infection, and bladder rupture were observed in one case. CONCLUSIONS: Follow-up of patients with urachal remnants can be performed conservatively. The type of complaint and the presence of clinical and radiological regression (if any) are important for deciding the treatment during the follow-up. However, conservative follow-up is more prominent today, as shown in this study.

Operative management of urachal remnants: An NSQIP based study of postoperative complications.

PURPOSE: The identification of urachal remnants is occurring more in infancy. Despite evidence that nonoperative management is effective, operative management remains common and has a high complication rate. We sought to determine if the complication rate after urachal resection is associated with age. METHODS: Patients undergoing urachal remnant resection were identified from ACS NSQIP Pediatric from 2013 to 2017. Exclusion criteria included emergent operations, contaminated wounds, and any additional procedures. Patients were compared based on complication rates, need for reoperation or readmission, and length of stay. RESULTS: A complication occurred in 16 of 476 patients (3.3%), 6 (1.3%) had reoperation, and 11 (2.3%) were readmitted. The median age for patients requiring reoperation was lower (0.1 years) than those not (1.3 years; p = 0.004). The median age of those readmitted was lower (0.4 years) than those not (1.4 years, p = 0.03), and a weak trend of longer length of stay in younger patients was identified (ρ = -0.16, p < 0.001). CONCLUSIONS: Operative management of younger patients resulted in greater risk of reoperation, readmission, and longer length of stay. Given that nonoperative management is effective, it may be of benefit to delay resection of urachal remnants to after 1 year of age. STUDY TYPE: Treatment study. LEVEL OF EVIDENCE: Level III.