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Archivos Espanoles de Urologia Sep 1997Megacalycosis or Puigvert's disease is a congenital anomaly of renal development characterized by caliceal dilatation, an increased number of calyces, associated with...
OBJECTIVE
Megacalycosis or Puigvert's disease is a congenital anomaly of renal development characterized by caliceal dilatation, an increased number of calyces, associated with hypoplasia of the pyramids of Malpighi, and a normal renal pelvis. Renal function is always normal and there is no evidence of obstruction to urinary flow. The foregoing are important in distinguishing megacalycosis from congenital hydronephrosis. We have studied two pediatric patients with this renal anomaly by means of non invasive techniques, in order to demonstrate they had no urinary flow obstruction despite the caliceal dilatation.
METHODS
Two cases of megacalycosis that had been diagnosed at our hospital from 1991 to 1995 are described and the literature is reviewed. Diagnosis was basically by intravenous urography. A diuretic renogram with technetium 99m diethylenetriaminepentaacetic acid (Tc 99m DTPA) showed no urinary flow obstruction in the anomalous kidney. This test was repeated several times, together with renal function studies like the technetium 99m dimercaptosuccinic acid scan (DMSA Scan).
RESULTS
Both patients are asymptomatic and have a normally functioning kidney with no scars demonstrable on the DMSA scan. Renal urinary flow studies have remained within the normal ranges (elimination of more than 40% of the radionuclide 20 min after the administration of furosemide). US control evaluations have shown adequate renal growth and persistent caliceal dilatation in both cases.
CONCLUSIONS
This congenital malformation must be considered when investigating renal dilatation, since megacalycosis does not require surgical treatment. Intravenous urography is useful in the diagnosis of this condition and Tc 99m DPTA is the best test for subsequent control evaluations.
Topics: Child; Dilatation, Pathologic; Female; Humans; Kidney Calices; Male; Radionuclide Imaging; Radiopharmaceuticals; Technetium Tc 99m Pentetate
PubMed: 9412381
DOI: No ID Found -
Infection 1988This is report on a boy with megacalycosis in whom infectious urolithiasis after eradication of Proteus mirabilis was maintained by Corynebacterium group D2.
This is report on a boy with megacalycosis in whom infectious urolithiasis after eradication of Proteus mirabilis was maintained by Corynebacterium group D2.
Topics: Child; Corynebacterium Infections; Humans; Kidney Calices; Kidney Diseases; Kidney Pelvis; Male; Proteus Infections; Proteus mirabilis; Urinary Calculi
PubMed: 3053458
DOI: 10.1007/BF01650763 -
Anales de Pediatria (Barcelona, Spain :... May 2004Dilation of the genitourinary tract is not always synonymous with obstruction. It is well known that malformations with apparent hydronephrosis and normal urinary flow...
Dilation of the genitourinary tract is not always synonymous with obstruction. It is well known that malformations with apparent hydronephrosis and normal urinary flow can exist. In addition, two or more distinct malformations of the genitourinary tract can coexist in the same patient. We present a case of coexistence of a unilateral multicystic dysplastic kidney and contralateral megacalycosis associated with ipsilateral distal segmental megaureter. This association is unusual in the literature. Glomerular renal function was normal, with only a slight defect of renal concentration capacity.
Topics: Child, Preschool; Glomerular Filtration Rate; Humans; Kidney; Male; Multicystic Dysplastic Kidney
PubMed: 15105004
DOI: 10.1016/s1695-4033(04)78308-3 -
Pediatric Radiology 1987Four cases of congenital megacalycosis associated with ipsilateral segmental megaureter in children are presented. This association has not been emphasized, although...
Four cases of congenital megacalycosis associated with ipsilateral segmental megaureter in children are presented. This association has not been emphasized, although review of the English and French literature revealed eight such cases. In all cases, the concurrent entities are unilateral, with a left-sided predominance and a male prevalence. The diagnosis of megacalycosis is presumed in four of the cases by normal function and prompt emptying of the calyceal systems on diuretic renography and/or urography. The presence of normal caliber renal pelvis interposed between the dilated collecting system and the distal dilated ureter without evidence of vesicoureteral reflux implies the coexistence of ipsilateral idiopathic megaureter. The patients usually present because of urinary tract infection and/or calculus formation, but respond well to conservative therapy.
Topics: Adolescent; Child; Child, Preschool; Female; Humans; Kidney Calices; Kidney Pelvis; Male; Radiography; Sex Factors; Ureter
PubMed: 3822580
DOI: 10.1007/BF02386591 -
Revista Paulista de Medicina 1975
Topics: Adolescent; Glomerulonephritis; Humans; Kidney Calices; Kidney Pelvis; Male; Radiography
PubMed: 1052375
DOI: No ID Found -
Urology Oct 1987Multiple benign vascular polyps of the ureter are rare, and the associated congenital megacalycosis and nephrolithiasis are very uncommon. Herein we report a case with...
Multiple benign vascular polyps of the ureter are rare, and the associated congenital megacalycosis and nephrolithiasis are very uncommon. Herein we report a case with vascular polyps of the right ureteropelvic junction dissociated with extensive hydronephrosis which was considered to be megacalycosis. Clinical features and treatment modalities are reviewed.
Topics: Adolescent; Humans; Kidney Calculi; Kidney Calices; Kidney Pelvis; Male; Neoplasms, Multiple Primary; Polyps; Radiography; Ureteral Neoplasms
PubMed: 3660528
DOI: 10.1016/0090-4295(87)90306-2 -
American Journal of Medical Genetics May 2002Schinzel-Giedion syndrome is a rare multiple congenital malformation syndrome defined by an evocative midfacial retraction, kidney and urinary malformations and multiple... (Review)
Review
Schinzel-Giedion syndrome is a rare multiple congenital malformation syndrome defined by an evocative midfacial retraction, kidney and urinary malformations and multiple skeletal abnormalities associated to a recently described neurodegenerative process. Two children with SGS are reported with identical clinical findings: megacalycosis, progressive neurodegeneration with infantile spasms and hypsarrhymtic activity. Ocular investigations revealed alacrimia and corneal hypoesthesia. Computed tomography of the temporal bone showed a tuning-fork malformation of the stapes for both children. These features may contribute to further delineation of SGS as additional clinical criteria.
Topics: Abnormalities, Multiple; Child, Preschool; Craniofacial Abnormalities; Electroencephalography; Electroretinography; Evoked Potentials, Auditory; Eye Diseases; Heart Defects, Congenital; Humans; Hydronephrosis; Infant; Intellectual Disability; Karyotyping; Male; Stapes; Syndrome
PubMed: 11977181
DOI: 10.1002/ajmg.10348 -
Schweizerische Medizinische... Jun 1988Poland's syndrome is characterized by congenital aplasia of the sternocostal head of the pectoralis major muscle associated with ipsilateral hand deformities (most often...
Poland's syndrome is characterized by congenital aplasia of the sternocostal head of the pectoralis major muscle associated with ipsilateral hand deformities (most often synbrachydactylia). 20% of patients have rib cage abnormalities, some with herniation of the lung. In some cases other associated ipsilateral anomalies (hypoplasia or absence of the nipple and/or the breast, defects of the vertebrae, dextrocardia, renal aplasia of hypoplasia, undescended testes, Möbius syndrome) have been described. We describe a patient with right-sided Poland's syndrome, the first published case associated with megacalycosis of the ipsilateral kidney. It is also the first publication of a familial case with this renal anomaly, since a brother has megacalycosis of the right kidney without Poland's syndrome. The majority of cases of Poland's syndrome are sporadic. The incidence has been estimated at between 1:30,000 and 1:80,000 of live births. The great uncle of our patient had a short forearm and only rudimentary fingers. The etiology of the syndrome is unknown. Toxic effects early in fetal life or effects on primary asymmetrical blood vessels, e.g. the subclavian artery, followed by uneven growth, are discussed as possible causes of Poland's syndrome.
Topics: Abnormalities, Multiple; Adult; Breast; Female; Humans; Hypertension, Renal; Kidney Medulla; Male; Pedigree; Poland Syndrome; Syndactyly; Urography
PubMed: 2839896
DOI: No ID Found -
Archivos Espanoles de Urologia 1987
Comparative Study
Topics: Adult; Child; Dilatation, Pathologic; Female; Humans; Kidney Calices; Kidney Diseases; Kidney Pelvis; Male; Radiography; Radionuclide Imaging
PubMed: 3579381
DOI: No ID Found -
Journal of Indian Association of... Jan 2014Segmental dilatation of ureter is a giant, focal segmental ureteral dilatation producing an elongated and distorted ureter. Two children presented with this condition,...
Segmental dilatation of ureter is a giant, focal segmental ureteral dilatation producing an elongated and distorted ureter. Two children presented with this condition, one had ipsilateral megacalycosis and contralateral vesicoureteric reflux. The other had duplication of the kidney. The non-functioning lower moiety showed structure of xanthogranulomatous pyelonephritis.
PubMed: 24604984
DOI: 10.4103/0971-9261.125965