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Radiographics : a Review Publication of... 2021The upper urinary tract is the most common human system affected by congenital anomalies. Congenital anomalies of the kidneys and ureters comprise a wide spectrum of... (Review)
Review
The upper urinary tract is the most common human system affected by congenital anomalies. Congenital anomalies of the kidneys and ureters comprise a wide spectrum of disorders ranging from simple variants with no clinical significance to complex anomalies that may lead to severe complications and end-stage renal disease. They may be classified as anomalies of renal form, which are subclassified as structural anomalies (eg, persistent fetal lobulation, hypertrophied column of Bertin, and dromedary hump) and fusion anomalies (eg, horseshoe kidney and pancake kidney); anomalies of renal position (eg, renal malrotation, simple renal ectopia, and crossed renal ectopia) and renal number (eg, renal agenesis and supernumerary kidney); and abnormalities in development of the urinary collecting system (eg, pyelocaliceal diverticulum, megacalycosis, ureteropelvic junction obstruction, duplex collecting system, megaureter, ectopic ureter, and ureterocele). US is usually the first imaging modality used because of its low cost, wide availability, and absence of ionizing radiation. Intravenous urography and voiding cystourethrography are also useful, mainly for characterization of the collecting system and vesicoureteral reflux. However, intravenous urography has been replaced by CT urography and MR urography. These imaging methods not only allow direct visualization of the collecting system but also demonstrate the function of the kidneys, the vascular anatomy, adjacent structures, and complications. Comprehension of congenital anomalies of the upper urinary tract is crucial for an accurate diagnosis and correct management. The authors discuss the spectrum of these anomalies, with emphasis on embryologic development, imaging findings, clinical manifestations, and complications. RSNA, 2021.
Topics: Humans; Kidney; Ureter; Urinary Tract; Urogenital Abnormalities; Urography
PubMed: 33513074
DOI: 10.1148/rg.2021200078 -
Clinical Case Reports Feb 2021Recurrent UTI's represent a challenge for physicians and anatomic abnormalities should be investigated thoroughly in this setting. Not all urinary tract dilatations mean...
Recurrent UTI's represent a challenge for physicians and anatomic abnormalities should be investigated thoroughly in this setting. Not all urinary tract dilatations mean obstruction. Several entities can mimic hydronephrosis without actual interruption of urinary flow. Uniform calyceal dilatation without dilatation of pelvis and ureter suggests megacalycosis.
PubMed: 33598298
DOI: 10.1002/ccr3.3687 -
IJU Case Reports Jul 2023Megacalycosis is a rare disorder related to congenital underdevelopment of the renal papilla or structural defect of the renal calyces. Megacalycosis has a wide spectrum...
INTRODUCTION
Megacalycosis is a rare disorder related to congenital underdevelopment of the renal papilla or structural defect of the renal calyces. Megacalycosis has a wide spectrum of clinical presentations ranging from simple variants without any significance on renal function to severe complications. Any prevention strategy is recommended yet since megacalycosis is mostly asymptomatic and usually discovered either accidentally or as result of its complications.
CASE PRESENTATION
We observed megacalycosis progression in a young female with a single kidney toward progressive calyx dilatation for years, which ended in acute pyelonephritis. Conservative management, urinary drainage, and large-spectrum antibiotics were unsuccessful and nephrectomy was required.
CONCLUSION
This rare case and literature review add evidence to identify prognostic factors to select patients with a high risk of complications (single kidney, bilateral disease, female gender, associated genetic syndromes, vesicoureteral reflux, and contralateral renal disorder). One o more factors should activate close monitoring and prophylactic therapy if needed.
PubMed: 37405038
DOI: 10.1002/iju5.12597 -
Research and Reports in Urology 2015To describe the radiological findings and the clinical importance of megacalycosis.
OBJECTIVE
To describe the radiological findings and the clinical importance of megacalycosis.
MATERIALS AND METHODS
On the basis of a case report and literature review, diagnostic criteria and clinical significance of megacalycosis are presented.
RESULT
Megacalycosis is mostly asymptomatic and is usually discovered either accidentally or as a result of its complications, such as stone formation, flank pain, hematuria, infection, and fever. The renal pelvis, infundibulum, and ureter are not dilated. Calyces have a semilunar configuration rather than the conventional triangular or conical form. The tip of each pyramid is flat, and the calyces possess neither fornix nor papillae impressions. The number of calyces is increased compared to the healthy condition, typically from 20-25. The renal parenchyma has a normal width but with a slight narrowing of the renal medulla. The kidney exhibits normal function, in particular with respect to its ability to concentrate the urine.
CONCLUSION
Megacalycosis is a rare, usually unilateral dilatation of the kidney calyces in the presence of a normal, undilated renal pelvis and ureter. Its pathological significance lies in the occurrence of complications.
PubMed: 26528455
DOI: 10.2147/RRU.S81519 -
Hong Kong Medical Journal = Xianggang... Dec 2020
Topics: Diagnosis, Differential; Female; Humans; Kidney Calices; Kidney Diseases; Medical Illustration; Radiography; Young Adult
PubMed: 33350970
DOI: 10.12809/hkmj208463 -
Current Opinion in Urology Jul 2023Nowadays, due to the increase of imaging diagnosis, we identify easily renal anomalies, and we can choose between a wide range of armamentarium to treat symptomatic... (Review)
Review
Retrograde intrarenal surgery for stones associated with renal anomalies: caliceal diverticulum, horseshoe kidney, medullary sponge kidney, megacalycosis, pelvic kidney, uretero-pelvic junction obstruction.
PURPOSE OF REVIEW
Nowadays, due to the increase of imaging diagnosis, we identify easily renal anomalies, and we can choose between a wide range of armamentarium to treat symptomatic stones in those challenging cases. However, there is a lack of evidence and consensus on its use. The aim of this narrative review is to collect all the available data about safety and efficacity of retrograde intrarenal surgery (RIRS) in the treatment of kidney stones associated to a renal anomaly.
RECENT FINDINGS
Renal anomalies are uncommon findings and even more if it has to be associated with renal stones. After a literature review of the past 2 years, there are a small number of studies that compare the outcomes in patients who have been treated with minimally invasive modalities and they are mainly focus on RIRS.
SUMMARY
It is of extreme importance to know the advances on the stone treatment in anomalous kidneys. With the development of new laser technologies, RIRS is becoming a more interesting technique with high success rate and safety. Further studies are needed to make an accurate statement about the adequate surgical technique for each renal anomaly and also, clinical trials using new laser technologies.
Topics: Humans; Fused Kidney; Medullary Sponge Kidney; Treatment Outcome; Kidney Calculi; Kidney; Diverticulum; Retrospective Studies; Nephrostomy, Percutaneous
PubMed: 37014757
DOI: 10.1097/MOU.0000000000001093 -
Polski Merkuriusz Lekarski : Organ... Apr 2018Renal agenesis occurs in pediatric population with the incidence 1:500- 2000 children. It is more often diagnosed in boys and on the left side of the body. Renal...
UNLABELLED
Renal agenesis occurs in pediatric population with the incidence 1:500- 2000 children. It is more often diagnosed in boys and on the left side of the body. Renal agenesis may be isolated or it may be a part of complex malformation syndrome. Megacalycosis is a very rare anomaly of urinary tract associated with abnormal structure of the kidney pyramids.
AIM
The aim of the study was to present for the first time in the medical literature the case of a girl with unilateral renal agenesis and megacalycosis.
CASE REPORT
A girl, born at term in good general medical condition, and with normal birth weight was admitted to the hospital because of urinary tract infection caused by E.coli. Antenatal abdominal ultrasounds were normal. In a diagnostic, repeated ultrasound studies, unilateral, left renal agenesis and the righ-sided megacalycosis were found. The right kidney had dilated collecting system, with normal size of renal pelvis and enlarged calyces up to 26 mm. The kidney function was normal. Voiding cystourethrography excluded vesicoureteral reflux. Dynamic scintigraphy 99mTc-EC showed the lack of function of the left kidney, postinflammatory changes and dilation of collecting system without signs of obstruction. During two-years follow up we didn't observe clinical relapse of urinary tract infection. Blood pressure and kidney function were normal.
CONCLUSIONS
Complex congenital anomalies of the kidney and the urinary tract (CAKUT) can be diagnosed at any age. Normal antenatal abdominal ultrasound does not exclude CAKUT. Every patient with congenital abnormalities of the kidney and the urinary tract requires long-term follow up, because of increased risk of chronic kidney disease.
Topics: Escherichia coli Infections; Female; Humans; Infant; Kidney Calices; Radionuclide Imaging; Solitary Kidney; Ultrasonography; Urinary Tract Infections
PubMed: 29775450
DOI: No ID Found -
Urology Case Reports Sep 2022What happens when kidney stone clearance is not feasible? We report the case of a 46-year-old male who presented for review with bilateral congenital non-obstructive...
What happens when kidney stone clearance is not feasible? We report the case of a 46-year-old male who presented for review with bilateral congenital non-obstructive calyceal dilatation (megacalycosis) and high volume bilateral renal calculi in the setting of stage four chronic kidney disease. Since complete stone clearance was deemed futile, thus a consensus was made between Urology and Nephrology, and treatment goals were focused on addressing symptoms, preserving renal function and preventing urinary tract infections until renal transplantation is needed. This case highlights that for some patients with severe complex kidney stone disease, an alternative management plan is needed.
PubMed: 35795248
DOI: 10.1016/j.eucr.2022.102146 -
Case Reports in Genetics 2017Schinzel-Giedion syndrome (SGS) is a rare autosomal dominant disorder that results in facial dysmorphism, multiple congenital anomalies, and an increased risk of...
Schinzel-Giedion syndrome (SGS) is a rare autosomal dominant disorder that results in facial dysmorphism, multiple congenital anomalies, and an increased risk of malignancy. Recently, using exome sequencing, de novo heterozygous mutations in the gene have been identified in patients with SGS. Most affected individuals do not survive after childhood because of the severity of this disorder. Here, we report mutation confirmed by molecular analysis in a case of SGS with congenital megacalycosis.
PubMed: 29333303
DOI: 10.1155/2017/3740524 -
The Pan African Medical Journal 2015
Topics: Dilatation, Pathologic; Escherichia coli Infections; Female; Humans; Kidney Calices; Kidney Diseases; Urethral Stricture; Urinary Tract Infections; Young Adult
PubMed: 26977241
DOI: 10.11604/pamj.2015.22.334.6868