Multicystic Dysplastic Kidney Disease: An In-Utero Diagnosis (original) (raw)
Related papers
Pediatrics & Neonatology, 2011
Multicystic dysplastic kidney (MCDK) is a congenital maldevelopment in which the renal cortex is characteristically replaced by numerous cysts of multiple sizes. MCDK presenting as a single predominant large cyst in morphology is less common. We report on the prenatal imaging findings and perinatal management of a fetus with MCDK unusually presenting as a single predominant large cyst, erroneously interpreted as a severe fetal hydronephrosis. Details of the perinatal history, radiological evaluation, morphological characteristic, and clinical aspect of this case are presented. We also discuss a few studies addressing the sensitivity of magnetic resonance urography for the prenatal diagnosis of MCDK.
Prenatal Diagnosis, 1999
To better delineate the natural history of multicystic displastic kidney disease (MCDKD) and provide insights into the pathogenesis of this condition, we report our experience in 102 prenatally detected cases. MCDKD is most commonly an incidental finding on prenatal ultrasound examination. The abnormality may be unilateral (76 per cent) or bilateral (24 per cent). In unilateral cases, abnormality of the contralateral kidney is common (33 per cent). Associated non-renal abnormalities occur frequently with both unilateral (26 per cent) and bilateral (67 per cent) MCDKD, and increase the risk for an abnormal chromosome study. Males are more likely to be affected than females with a ratio of 2.4:1, but females are twice as likely to have bilateral MCDKD and associated non-renal abnormalities, and four times more likely to have an abnormal chromosome study. We suggest that the option of chromosomal analysis should be discussed with all patients diagnosed with MCDKD in their fetus, if there is bilateral renal involvement or if an associated non-renal abnormality is present. Unilateral MCDKD without associated renal or non-renal abnormalities was not associated with an abnormal chromosome study, and resulted in favourable outcomes. While unilateral MCDKD, lack of associated anomalies, normal chromosome study and adequate amniotic fluid are all reassuring findings, a complete neonatal urologic work-up should be performed in all newborns. We believe the evaluation should include voiding cystourethrography to rule out vesicoureteral reflux. Our findings allow more precise counselling of patients regarding prognosis, and subsequent management of the fetus found to have MCDKD.
Bilateral multicystic dysplastic kidneys: Case report
2016
Multicystic dysplastic kidney (MCDK) is a congenital, non-heritable maldevelopment in which the renal cortex is replaced by numerous cysts of varying sizes. Typically, MCDK is a unilateral disorder in 76% of cases and bilateral in 24%. The latter is incompatible with life. We present the case of a male child with bilateral MCDK who was followed up with ultrasound scan (USS) from 22nd week of intrauterine life till death on the 19th day after delivery. Serial antenatal USS revealed oligohydramnios and both kidneys showed multiple cysts which appeared to be communicating. Postnatal USS however, revealed multiple sub-cortical thin walled cysts of varying sizes which apparently appeared to be communicating. A diagnosis of bilateral MCDK was made and was confirmed at autopsy.
Multicystic dysplastic kidney (MCDK) is one of the most common forms of congenital cystic diseases of the kidney that usually occurs sporadically and may rarely be associated with concomitant anomalies of the heart, spine, brain, and gastrointestinal system. The disorder is known to be commonly associated with a normally situated functional contralateral kidney and without significant symptoms. We report a case of an infant who presented with acute urinary tract infection on a background of an unusual association of normally positioned but nonfunctional MCDK and functional ectopic (pelvic) kidney on the contralateral side. We suggest the inclusion of diagnostic ultrasound as a routine investigation for pregnant women during antenatal care at all levels of health care to aid prenatal detection of renal abnormalities and a multidisciplinary approach to early management. Keywords: Infant, multicystic dysplastic kidney, renal ectopia, urinary tract infection
An infant girl with bilateral multicystic dysplastic kidney: a case report
Journal of Nephropathology
Multicystic dysplastic kidney (MCDK) is a congenital kidney abnormality with an overall incidence rate of 1/4300 live births that is more prevalent in males than females. Here, we describe a rare case of bilateral MCDK complicated by hypertension, severe vesicoureteral reflux (VUR), and urinary tract infection (UTI), who stabilized during hospitalization with prospective kidney transplantation due to poor prognosis. Since bilateral MCDK is a rare disease with numerous complications and a poor prognosis, patient symptom management and treatment may be more challenging.
Pediatric Nephrology, 1994
A multicystic dysplastic kidney (MCDK) is a developmental anomaly in which the renal parenchyma is virtually replaced by non-functioning, tense, usually non-communicating cysts. Historically, before the era of ultrasonography (US), MCDK was recognized as a common cause of abdominal mass in the newborn and frequently was removed surgically because of: (1) difficulty in precisely determining the diagnosis of a cystic retroperitoneal mass and/or (2) concern about potential complications, such as malignancy, hypertension, or infection.
Multicystic dysplastic kidney: Ten-year evaluation
IP innovative publication pvt. ltd, 2019
ntroduction: Multicystic dysplastic kidney (MCDK) is the most common form of developmental abnormality seen in infants and children. It is almost always unilateral and slightly more frequent in boys and on the left side. The other associated extra-renal abnormalities include esophageal atresia, tracheoesophageal atresia, ventricular septal defect and patent ductus arteriosus. The routine widespread use of antenatal ultrasonography has led to early detection of this abnormality. Materials and Methods: We retrospectively assessed fourteen cases of Multicystic dysplastic kidney between January 2004 and December 2014. All the cases underwent total nephrectomy and the specimens were sent to the department of Pathology with their respective clinical details and pathological findings were analyzed. Results: A total of fourteen (14) patients were diagnosed with unilateral MCDK over a period of 10 years with no sex predilection. Majority were on left side and antenatally diagnosed. Some patients had associated hypertension, vesicoureteral reflux and ureteropelvic junction obstruction. Microscopy revealed renal parenchyma replaced by cysts of variable sizes lined by flattened cuboidal epithelium. Intervening fibrotic stroma with mesenchymal components, primitive glomeruli, nerve bundles and dysplastic ducts. Conclusion: MCDK is mostly a unilateral sporadic anomaly usually found on the left side, however can be familial. It may be associated with hypertension, vesicoureteral reflux and ureteropelvic junction obstruction. Most of the patients can be diagnosed antenatally and confirmed on histopathology.
Fetal Magnetic Resonance Imaging of Bilateral Cystic Renal Dysplasia
Taiwanese Journal of Obstetrics & Gynecology, 2008
Renal dysplasia, defined as abnormal parenchymal development from anomalous differentiation of metanephric tissue, implies irreversible renal damage. The functional capacity of an affected kidney depends upon the extent and severity of the dysplasia. Conglomerates of disorganized epithelial structures surrounded by abundant fibrous tissue characterize a dysplastic kidney. Cortical cysts are often, but not necessarily, present .
Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology, 2006
To identify important factors in the differential diagnosis of renal cysts associated with hyperechogenic kidneys. This was a retrospective multicenter study. We identified 93 fetuses presenting between 1990 and 2002 with hyperechogenic kidneys and which had a diagnosis of nephropathy confirmed later. We analyzed retrospectively the prenatal ultrasound findings of those fetuses which were found sonographically to have renal cysts. Of the 93 fetuses presenting with hyperechogenic kidneys and with a later diagnosis of nephropathy, there were 28 with autosomal dominant polycystic kidney disease (ADPKD), 31 with autosomal recessive polycystic kidney disease (ARPKD), 11 with Bardet-Biedl syndrome, nine with Meckel-Gruber syndrome, six with Ivemark II syndrome, one with Jarcho-Levin syndrome, one with Beemer syndrome and one with Meckel-like syndrome. One third of the fetuses (30/93) had renal cysts. Cystic characteristics (size, location, number) were not very useful for diagnosis; more ...