Congenital anomalies of the urinary tract are considered to be rather common in the general population, with an incidence of 1 in 500 individuals [1]. A congenital megaureter, an important cause of obstructive uropathy in the neonatal and pediatric population [3], is defined as abnormal dilation of the ureter, together with dilation of the renal pelvis in most cases [1]. Several types have been described in the literature - obstructed or nonobstructed and refluxing or nonrefluxing [1] [2] [3]. The clinical presentation of a congenital ureter is often asymptomatic and the majority of patients are incidentally diagnosed on prenatal ultrasonography [3]. When the congenital megaureter is not visualized during this period, recognition might be delayed until adulthood due to the absence of complaints [3] [4]. Symptomatic cases, on the other hand, may manifest as more frequent urinary tract infections (UTIs), recurrent urolithiasis, nonspecific abdominal pain, and fever [3] [4]. Hematuria (both microscopic, and in the presence of calculi, macroscopic) can also be a part of the clinical presentation [3]. Renal failure is rarely seen due to congenital megaureter, but renal dysplasia and hydronephrosis are reported as more severe complications [3] [5].

• Surgical Procedure

  About My Practice I evaluate and treat most urologic conditions, and have a special interest in minimally invasive techniques such as the use of the daVinci Robot in surgical procedures. I see patients of all ages, and treat both men and women. [ucosc.com]

  If there is a blockage of the urinary tract, however, a megaureter may require surgical intervention. The surgical procedure involves removing the section of the ureter that is abnormal, reducing it, and reconnecting the ureter. [childrensnational.org]

  Percutaneous nephrostomy followed by balloon pyeloplasty or by endoscopic endopyelotomy has been reported as a primary procedure and as a secondary procedure in patients in whom surgical therapy has failed. [radiologykey.com]

  Transplantation is achievable in most cases of CALUT, but there are questions regarding the indication for surgical procedures, as well as the frequency of complications, especially urinary tract infections (UTI) and their prophylaxis 4. [scielo.mec.pt]

  Transplantation is achievable in most cases of CALUT, but there are questions regarding the indication for surgical procedures, as well as the frequency of complications, especially urinary tract infections (UTI) and their prophylaxis4. [scielo.pt]

• Epilepsy

  /8p%20duplications%20FTNW.pdf[OTHER]_20880309[PMID]_12225557[ PMID] ...... epilepsy-paroxysmal exercise-induced dystonia-writer's cramp syndrome ... AÑO 2017 - extrofia ; congénito de la vejiga urinaria con profundo impacto en la morbilidad. [xuqyjtln.tk]

• Short Stature

  stature-advanced bone age-early-onset osteoarthritis syndrome Skeletal overgrowth-craniofacial dysmorphism-hyperelastic skin-white matter lesions syndrome Slender bone dysplasia Smith-Lemli-Opitz syndrome Spondylodysplastic dysplasia Spondyloepiphyseal [se-atlas.de]

• Abdominal Mass

  Congenital giant megaureter should be considered as one of differential diagnosis in children presenting as abdominal mass. [omicsonline.org]

  Congenital UPJ obstruction is the most common cause of an abdominal mass in a neonate. The disorder is being discovered increasingly in the antenatal period because of the almost routine use of obstetric ultrasound. [radiologykey.com]

  Most patients present with nonspecific symptoms, including dull abdominal pain, of less than 12 months' duration but patients can present with the complications of the fibrosis. [patient.info]

• Abdominal Pain

  Symptomatic cases, on the other hand, may manifest as more frequent urinary tract infections (UTIs), recurrent urolithiasis, nonspecific abdominal pain, and fever. [symptoma.com]

  Most patients present with nonspecific symptoms, including dull abdominal pain, of less than 12 months' duration but patients can present with the complications of the fibrosis. [patient.info]

• Hypertension

  […] due to gain-of-function mutations in the mineralocorticoid receptor Hypertrophic cardiomyopathy and renal tubular disease due to mitochondrial DNA mutation Hyperuricemia-pulmonary hypertension-renal failure-alkalosis syndrome Hypocomplementemic urticarial [se-atlas.de]

  Apply new evidence-based management options to treat genetic and constitutional factors leading to a high-risk pregnancy (such as diabetes, obesity, hypertension, and cardiac disease) through new and expanded coverage of these increasingly common presentations [books.google.com]

  Reflux-induced renal injury may range from clinically silent focal scars to generalized scarring and renal atrophy (reflux nephropathy), which may lead to morbidity during pregnancy, renin-mediated hypertension, renal insufficiency, and even end-stage [emedicine.medscape.com]

  Pheochromocytoma: laparoscopic right adrenalectomy in a child In the context of major headaches in a 9-year-old patient whose brother had been operated on for pheochromocytoma, a right adrenal pheochromocytoma with severe arterial hypertension was found [websurg.com]

• Heart Block

  ; Feb 6, 2012 ... ... of right pulmonary artery familial, Anonychia congenita, Anonychia ectrodactyly, ..... lipodystrophy type 2, Congenital giant megaureter, Congenital heart block, ..... and writer's cramp, Epileptic encephalopathy Lennox-Gastaut [xuqyjtln.tk]

• Osteoporosis

  […] oxidation and ketogenesis Disorder of purine or pyrimidine metabolism Disorder of urea cycle metabolism and ammonia detoxification Distal renal tubular acidosis Distal renal tubular acidosis with anemia Dominant hypophosphatemia with nephrolithiasis or osteoporosis [se-atlas.de]

• Encephalopathy

  ; Feb 6, 2012 ... ... of right pulmonary artery familial, Anonychia congenita, Anonychia ectrodactyly, ..... lipodystrophy type 2, Congenital giant megaureter, Congenital heart block, ..... and writer's cramp, Epileptic encephalopathy Lennox-Gastaut [xuqyjtln.tk]

• Dystonia

  /8p%20duplications%20FTNW.pdf[OTHER]_20880309[PMID]_12225557[ PMID] ...... epilepsy-paroxysmal exercise-induced dystonia-writer's cramp syndrome ... AÑO 2017 - extrofia ; congénito de la vejiga urinaria con profundo impacto en la morbilidad. [xuqyjtln.tk]

• Cryptorchidism

  Bilateral Trifid ureters associated with fused kidney, ureterovesical stenosis, left cryptorchidism and angioma of the bladder. J Urol 1978;120:249-50. [jiaps.com]

  It consists of a group of findings including: decreased or absent abdominal muscles, cryptorchidism (failure of the testicles to descend into the scrotum; they remain in the abdomen), and dilatation of the ureters and bladder, often with associated renal [cybernephrology.ualberta.ca]

• Ureteral Disorder

  Ureteral Disorders Your kidneys make urine by filtering wastes and extra water from your blood. The urine travels from the kidneys to the bladder in two thin tubes called ureters. The ureters are about 8 to 10 inches long. [icdlist.com]

Imaging studies are the cornerstone for recognizing a congenital megaureter, but the role of the physician in raising clinical suspicion through a detailed physical examination and history taking is essential in order to make a presumptive diagnosis [5]. Signs and symptoms (if present) should be assessed during the interview with the patient (although the young age of the patient often necessitates collection of information from the parent) and during the physical exam, after which several procedures can be employed. Grey-scale ultrasonography (US), being noninvasive and readily used both in the prenatal and postnatal setting, is widely described as the optimal method for detecting a congenital megaureter [3] [5]. In addition, several other methods are recommended [3] [4] [5] [6]:

• Voiding cystourethrography (VCUG) - Often used after the initial diagnosis is made by US, VCUG is an important tool for excluding ureteral reflux and possibly obstruction [2] [3].
• Intravenous urography (IVU) and radioisotope renography - The insufficient quality of images obtained due to the immature anatomical landmarks of newborns and infants makes this procedure of limited benefit in the first few weeks of life, which is why radioisotope renography is sometimes preferred for early assessment [2] [3].

In addition, magnetic resonance urography (MRU), intravenous pyelography (IVP) and X-ray urography, but also computed tomography (CT) have all been described as very good methods to confirm a congenital megaureter, dilation of the renal pelvis and urolithiasis [3] [5] [6].

Ureterocele Treatment of the ureterocele is based upon relief of obstruction. [emedicine.medscape.com]

Treatment and prognosis Usually asymptomatic and requiring no treatment. If complications occur or the degree of obstruction is marked then, reimplantation following resection of the aganglionic segment may be performed. [radiopaedia.org]

Conclusion Laparoscopic pyeloplasty, intracorporeal excisional tailoring, and non-refluxing reimplantation are safe and effective for the treatment of obstructive congenital megaureter. [moh-it.pure.elsevier.com]

Prepare for clinical challenges and save time in addressing them thanks to expert advice on treatment options from international contributors. [books.google.com]

Treatment and prognosis Usually asymptomatic and requiring no treatment. If complications occur or the degree of obstruction is marked then, reimplantation following resection of the aganglionic segment may be performed. [radiopaedia.org]

Surgical intervention virtually is universal and factors bearing upon recoverability and prognosis have been analyzed. [ncbi.nlm.nih.gov]

The prognosis depends on the associated malformations [3]. Case Report A newborn boy 28 days old admitted for urinary infection associated diarrhea (digestive table). [scitechnol.com]

Vesicoureteric reflux disease (VUR): although actually a cause of primary congenital megaureter it is usually considered separately as prognosis and treatment, depending on degree of reflux, is different Causes of secondary megaureter include (Figs. 7.3 [abdominalkey.com]

Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]

The exact etiology of this aperistatic segment is not known. Several etiological theories have been proposed including: Excessive collagen deposition. [abdominalkey.com]

Therapy usually entails tunnelled reimplantation to prevent reflux. megaureter A large ureter of any etiology, divided by some urologists into reflux megaureter, obstructed ureter and non-reflux or idiopathic megaureter meg·a·lo·u·re·ter, megaureter [medical-dictionary.thefreedictionary.com]

Thirty years later, Swenson postulated a neurologic etiology for both megacolon and megaureter and treated such patients with urinary diversion, ureteral substitution, and ileal augmentation to provide peristalsis. [emedicine.medscape.com]

Megaureters are treated differently based on their etiology. [urology.ucsf.edu]

Pan African Medical Journal. 2020;37(296). 10.11604/pamj.2020.37.296.26867 Articles from The Pan African Medical Journal are provided here courtesy of African Field Epidemiology Network [ncbi.nlm.nih.gov]

Epidemiology Age Urinary tract obstruction occurs most commonly in the young and the old: In older men, this is a relatively common condition due to prostatic enlargement: Incidence of lower urinary tract symptoms (LUTs)/BPH averages 15 per 1,000 man-years [patient.info]

[…] ureter to ongoing obstruction. [5] Animal models of congenital megaureter are lacking, but Mortell et al have developed a rat model of prenatal doxorubicin (Adriamycin) exposure that may help elucidate the etiology of this developmental defect. [6] Epidemiology [emedicine.medscape.com]

Epidemiology of chronic kidney disease in children. Pediatr Nephrol 2012;27(3):363–373. [ Links ] 2. Toka HR, Toka O, Hariri A, Nguyen HT. Congenital anomalies of kidney and urinary tract. Semin Nephrol 2010;30(4):374–386. [ Links ] 3. [scielo.mec.pt]

Brenner/Rector remains the go-to resource for practicing and training nephrologists and internists who wish to master basic science, pathophysiology, and clinical best practices. [books.google.com]

Primary refluxing obstructed megureter: This occurs in the presence of an incompetent VUJ that allows reflux through an adynamic distal segment. 7.3 Etiology and Pathophysiology Primary obstructive megaureter: It is a common cause of obstructive uropathy [abdominalkey.com]

The pathophysiology of primary megaloureter. J Urol. 1973 May. 109(5):805-11. [QxMD MEDLINE Link]. Mortell A, Fourcade L, Solari V, Puri P. Bilateral megaureters in the Adriamycin rat model. Pediatr Surg Int. 2005 Mar. 21(3):212-6. [emedicine.medscape.com]

Several theories have been speculated regarding the pathophysiology of segmental megaureter. [omicsonline.org]

The pathophysiology of congenital obstructive uropathy is poorly understood. There are very few experimental models of prenatal obstruction of the urinary tract, except in the fetal lamb or inbred rats. [pubfacts.com]

Prevention How can megaureter be prevented? There is no known way to prevent megaureter. More information on what causes the condition is needed. Researchers are still trying to determine if it’s genetic. [my.clevelandclinic.org]

Successful nonoperative management of VUR requires preventing renal damage from pyelonephritis and has involved the use of continuous antibiotic prophylaxis and treating bowel or bladder dysfunction. [emedicine.medscape.com]

Prevention & Risk Assessment Prevention & Risk Assessment What causes a megaureter? A megaureter that is not associated with other problems occurs during fetal development. [childrensnational.org]

This prevents normal peristalsis. A megaureter may also occur if there is a blockage stopping urine from entering the bladder. This causes a backflow of urine toward the kidney, widening the ureter. [cedars-sinai.org]

Therefore, research efforts in this field have recently focused on improving our ability to diagnose clinically relevant obstructive uropathy and examining the developmental causes of megaureter, and how this disorder may be prevented. [ncbi.nlm.nih.gov]

1. Vlad M, Ionescu N, Ispas AT, Ungureanu E, Stoica C. Morphological study of congenital megaureter. Morphological study of congenital megaureter. Rom J Morphol Embryol. 2007;48(4):381-390.
2. Berrocal T, López-Pereira P, Arjonilla A, Gutiérrez J. Anomalies of the distal ureter, bladder, and urethra in children: embryologic, radiologic, and pathologic features. Radiographics. 2002;22(5):1139-1164.
3. Shokeir AA, Nijman RJ. Primary megaureter: current trends in diagnosis and treatment. BJU Int. 2000;86(7):861-868.
4. Rosenblatt GS, Takesita K, Fuchs GJ. Urolithiasis in adults with congenital megaureter. Can Urol Assoc J. 2009;3(6):E77-E80.
5. Yu M, Ma G, Ge Z, Lu R, Deng Y, Guo Y. Unilateral congenital giant megaureter with renal dysplasia compressing contralateral ureter and causing bilateral hydronephrosis: a case report and literature review. BMC Urol. 2016;16:7.
6. Li TR, Du XK, Huo TL. Magnetic resonance urography and X-ray urography findings of congenital megaureter. Chin Med Sci J. 2011;26(2):103-108.