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PubMed1986

The dilemma of the multicystic dysplastic kidney.

Multicystic dysplastic kidney is the most frequent cause of an abdominal mass in the neonate, but controversy continues as to the optimal management of these lesions, since little is known about their natural history. Experience with two complicated cases and a review of reports of retained multicystic dysplastic kidneys suggest that such lesions pose a significant risk to their hosts. Malignancy, reversible hypertension, pain, and mass effect have been associated with retained lesions. Infection is another potential hazard that is frequently cited but poorly documented in the literature. In light of the currently low morbidity and mortality associated with operation and anesthesia in the neonatal period, resection appears to be the treatment of choice for the neonate with a multicystic dysplastic kidney.

Bacterial Infections↗
PubMed1990

Percutaneous decompression: treatment for respiratory distress secondary to multicystic dysplastic kidney.

Multicystic dysplastic kidney is a common renal anomaly in the newborn. Long-term problems, such as pain, infection, hypertension and neoplasm, although infrequent, have been reported. Acute, life-threatening complications resulting from the size of the affected kidney are rare and emergency nephrectomy has been the only reported effective therapy. We present a case of ultrasound-guided percutaneous cyst decompression used as definitive treatment of respiratory failure associated with multicystic dysplastic kidney.

Diagnosis, Differential↗
PubMed2002

Serial followup of the contralateral renal size in children with multicystic dysplastic kidney.

PURPOSE: Multicystic dysplastic kidneys have negligible renal function and the contralateral kidney (solitary kidney) frequently exhibits abnormalities that may affect growth. We previously showed that nomograms related to renal size constructed from digitalized ultrasonographic measurements of renal parenchymal area are a sensitive measure of renal growth and correlate with functional mass. We assess the age-dependent characteristics of compensatory renal growth in infants and children with multicystic dysplastic kidneys by construction of a growth curve for the contralateral kidney, assess these characteristics in comparison to normal renal growth of right and left kidneys, analyze the extent of compensatory renal growth and evaluate abnormal growth in solitary kidneys in infants and children. MATERIALS AND METHODS: From 1988 to 2000 we reviewed 152 serial sonograms from 48 patients with a diagnosis of multicystic dysplastic kidneys. We also reviewed 209 renal sonograms in patients whose studies, done for other purposes, showed normal bilateral kidneys. Using computer planimetry, parenchymal area and pelvicaliceal area were determined after digitalization of ultrasound images. Parenchymal area was calculated by parenchymal area minus pelvicaliceal area and expressed as a mean of 3 measurements. A parenchymal area growth curve was generated for the contralateral kidney in the multicystic dysplastic kidney group from birth to 216 months, and for right and left normal kidneys from birth to 338 months. Data were plotted as mean parenchymal area +/- 2 SD on a nomogram generated by linear regression. Differences in parenchymal area between normal right and left kidneys, between normal kidneys and the contralateral to multicystic dysplastic kidney were analyzed by unpaired Student t test. RESULTS: Of the 48 patients with multicystic dysplastic kidneys 36 had contralateral normal kidneys and 12 (25%) had a contralateral abnormality. Of the 12 cases 4 and an additional 5 without an identified abnormality (9 of 48) or 18.7% had solitary kidneys 2 SD below the normal growth curve for total parenchymal area, indicating a smaller than expected increase in compensatory renal growth. Conversely, 8 of 12 including 1 with grade V reflux into a solitary kidney exhibited normative compensatory renal growth. Left normal kidneys demonstrated a small but statistically significantly larger parenchymal area throughout growth. Solitary kidneys did not demonstrate growth differences associated with side. Solitary kidneys showed accelerated growth from 0 to 22 months while normal kidneys showed accelerated growth from 0 to 15 months. CONCLUSIONS: Nomograms constructed from ultrasonographic measurements of renal parenchymal area may be useful for assessing abnormal renal growth in solitary kidneys. Patients with solitary kidneys identified by conventional ultrasonographic measurement as normal may not exhibit expected growth. Clinical decision making may be improved by identification of solitary kidneys at risk for poor growth.

Adolescent↗
PubMed2001

Ambulatory blood pressure monitoring in children with unilateral multicystic dysplastic kidney.

UNLABELLED: Multicystic dysplastic kidney (MCDK) is one of the most common congenital renal anomalies. Arterial hypertension is a potential complication of MCDK. Blood pressure (BP) has so far been measured only casually and the frequency of hypertension has been estimated to be between 0%-8%. Ambulatory blood pressure monitoring (ABPM) provides more precise information on BP than the casual BP measurement. The aim of this study was to investigate the BP profile in children with MCDK using ABPM. A group of 25 children (16 girls), with a mean age of 7.8 years (range 3.8-17.7 years) were investigated. ABPM was performed using the oscillometric SpaceLabs 90207 device. Hypertension was defined as mean systolic and/or diastolic BP during the day and/or in the night exceeding 95th percentile for ABPM. Five (20%) children showed hypertension, two of them had combined daytime and night-time hypertension and three had isolated nocturnal hypertension, although daytime BP was between the 90th-95th percentile in two of them. Children with ultrasonographical and/or laboratory signs of contralateral kidney abnormalities showed a higher incidence of hypertension than those without abnormalities (two of four versus 3 of 21). The mean night-time systolic and diastolic BP of children with MCDK was significantly higher than in healthy children (+ 0.50 and + 0.54 SDS, respectively, P = 0.012 and 0.03, respectively). Three of the hypertensive children were already nephrectomised. All five hypertensive children showed ultrasonographical and/or laboratory signs of contralateral kidney abnormalities. Hypertensive children had significantly higher microalbuminuria than normotensive children (6.9 +/- 3.2 mg/mmol creatinine versus 1.8 +/- 0.7, P = 0.03). The nocturnal BP fall (dip) was attenuated in five children, only one of whom was hypertensive. CONCLUSION: Arterial hypertension in children with multicystic dysplastic kidney is seen more often if based on ambulatory blood pressure monitoring than on casual blood pressure recordings. The main risk factor for developing hypertension is contralateral kidney damage. Ambulatory blood pressure monitoring should be performed in children with multicystic dysplastic kidney, especially in those with contralateral kidney abnormalities.

Adolescent↗
PubMed1985

Neonatal hypertension from a unilateral multicystic dysplastic kidney.

Multicystic renal dysplasia is an extremely uncommon cause of hypertension in children and the few reported cases have not been of newborns. We report on a neonate in whom severe hypertension associated with elevated peripheral plasma renin resulted from a unilateral multicystic dysplastic kidney. Hypertension and plasma renin activity normalized after unilateral nephrectomy. No evidence of perfusion or excretory renal function in the dysplastic kidney was present on a radionuclide renal scan. This child demonstrates a relationship between hypertension and unilateral multicystic renal dysplasia.

Blood Pressure↗
PubMed1987

Prenatal diagnosis and obstetrical management of multicystic dysplastic kidney disease.

Multicystic dysplastic kidney disease (MDKD) is one of the most common congenital renal anomalies. We report 16 consecutive cases of MDKD recognized in the antenatal period by sonography. Diagnosis is usually easy as MDKD has in the vast majority of cases a striking ultrasound appearance including enlargement of the kidney and multiple renal cysts. However, differentiation from obstructive uropathy may be difficult, and we made a total of five erroneous diagnoses. Unilateral MDKD has almost invariably a good prognosis. However, severe life-threatening associated anomalies were found in six cases. Therefore, a detailed survey of fetal anatomy and determination of karyotype are strongly recommended.

Diagnostic Errors↗
PubMed1999

Autosomal dominant inheritance of multicystic dysplastic kidney.

Unilateral multicystic dysplastic kidney (MCDK) in a normal infant is believed to be a sporadic disorder, with an incidence of about 1 in 4,300 live births. Isolated unilateral MCDK occurring in a family without other genitourinary abnormalities has not been described. We report a family in which isolated unilateral MCDK occurred in a woman and her two children. The mother presented with a palpable abdominal mass during infancy, which on excision was found to be a MCDK. Both the children were found to have MCDK on prenatal ultrasonography, which was later confirmed on postnatal evaluation. The MCDK in the children continues to involute on follow-up urinary tract ultrasonography. The inheritance of MCDK appears to be autosomal dominant in this family.

Child, Preschool↗
PubMed1995

Abnormal contralateral kidney in unilateral multicystic dysplastic kidney disease.

We performed a retrospective study of infants with unilateral multicystic dysplastic kidney (MCDK) in order to investigate the associated urological abnormalities. We examined the records of seven patients, in five of whom a diagnosis had been made prenatally using ultrasonography. Our investigation focused on the associated urological abnormalities, particularly on the contralateral side, and the results of voiding cystourethrography (VCUG). Four of the seven patients (57%) had urological abnormalities other than MCDK: three exhibited vesicoureteral reflux (VUR) of the contralateral side including one patient who also had an ipsilateral ectopic ureter, and the fourth patient had a ureterocele of the ipsilateral side. The results indicate that contralateral VUR, was the most common abnormality associated with MCDK. Two infants had high-grade VUR and underwent anti-reflux surgery soon after the diagnosis. Both have remained free of recurrent urinary tract infection.

Female↗
PubMed1993

The multicystic dysplastic kidney in children.

Multicystic dysplastic kidney (MCDK) is the most common renal cystic disease in infancy and the second most common neonatal abdominal mass. It represents an extreme form of renal dysplasia. The key features in MCDK include variability in size, loss of kidney shape, absence of function and obstruction of ureter. Current management is still controversial. The prognosis is largely depended upon the problems of associated anomalies and the condition of the contralateral kidney. From September '82 to September '91, twelve patients with multicystic kidney have been treated at Taichung Veterans General Hospital. Ten of them received elective nephrectomy. The postoperative condition was uneventful. However, there was a high frequency of associated anomalies (41.6%) and pathologic contralateral kidney (50%). Two patients who refused operation were put on close observation and follow-up with ultrasound. During the surgical patients' follow-up period, one suffered from repeated urinary tract infection and the other died of multiple congenital anomalies.

Female↗
PubMed1992

Contralateral renal abnormalities in multicystic-dysplastic kidney disease.

Multicystic-dysplastic kidney (MCDK) disease has been associated with contralateral genitourinary tract abnormalities; however, neither the nature nor the prevalence of the contralateral renal lesion has been delineated. We retrospectively studied patients with MCDK disease to determine the prevalence of contralateral renal abnormalities and of other urologic anomalies. Forty-nine patients, ranging in age from neonates to 6 years, were examined by renal ultrasonography, excretory urography or nuclear medicine scanning, and voiding cystourethrography; 51% of the patients had an associated urologic abnormality. The abnormality was contralateral to the MCDK in 19 patients (39%), ipsilateral in 3 patients (6%), and involved the bladder wall in 3 patients (6%). The most common contralateral genitourinary tract abnormality was vesicoureteral reflux (18%) followed by ureteropelvic junction obstruction (12%). We conclude that urologic anomalies are common in patients with MCDK disease; contralateral vesicoureteral reflux is the most common abnormality identified.

Child↗
PubMed1992

Hypertension as a complication of multicystic dysplastic kidney.

The detection of multicystic dysplastic kidney in utero using prenatal ultrasound is becoming a more frequent occurrence. An accurate diagnosis of multicystic dysplastic kidney usually can be made radiographically, and therefore the main indication for surgery in the asymptomatic patient may be the potential risk of complications developing later in life. We report hypertension as a complication of multicystic dysplastic kidney and review the literature.

Child, Preschool↗
PubMed1997

[Conservative treatment of dysplastic multicystic kidney].

BACKGROUND: Recent developments regarding the conservative management of multicystic dysplastic kidney disease (MDKD) have shown a reduction in the well-established practice of elective surgical treatment. It has been seen that in the majority of cases the evolution of MDKD is either partial or complete atrophy. The incidence of complications due to MDKD is very low. The aim was to study the ultrasound changes of MDKD in a group of patients from 1990, since this date the elective surgical procedure had been eliminated. MATERIALS AND METHODS: This group consisted of 16 patients. The diagnosis in each patient was confirmed by ultrasound and isotopic examinations. Other investigations were performed where there was a suspect of associated contralateral renal abnormalities or when a urinary tract infection occurred. RESULTS: Ultrasound follow-up revealed: no enlargement whatsoever in the kidney size in any patient; in 10 patients a reduction in the size of the MDK; in 3 patients a complete atrophy of MDK; in 3 patients there was no change at all. Five patients were found to have contralateral renal abnormalities. Compensatory hypertrophy of the contralateral kidney was present in all patients. No complications occurred. A nephrectomy was performed only in one patient aged 6 due to the express wishes of his parents. CONCLUSIONS: The results agree with recently published literature that routine surgical removal of the MDK is unnecessary.

Child, Preschool↗
PubMed2006

Segmental multicystic dysplastic kidney in children.

Pediatric segmental multicystic dysplastic kidney is a rare subtype of multicystic dysplastic kidney disease, with only 18 case reports published. All children previously reported with this condition presented during infancy. This is the first case of segmental multicystic dysplastic kidney in an adolescent girl. The case is discussed, along with a review of published reports.

Adolescent↗
PubMed1996

[Therapeutic attitude in patients with multicystic dysplastic kidneys].

We presented seven multicystic dysplastic kidney cases treated in our Hospital during the last 15 years. Four children were diagnosed prenatally and three during the first term of life. Five were girls and two boys. We found three contralateral upper urinary tract anomalies. We did five nephrectomies. In two cases because compressive abdominal mass during the neonatal period, in two cases during the first year of life because symptomatic urinary tract infections and one case at the age of 13 months with persistent multicystic dysplastic kidney followed by ultrasound. Two cases were not operated. In one patient with partial regression of the multicystic kidney the family refused operation. We have lost follow-up in the last patient.

Cysts↗
PubMed2004

What is the fate of the refluxing contralateral kidney in children with multicystic dysplastic kidney?

PURPOSE: We clarify the natural history of contralateral vesicoureteral reflux (VUR) in children with multicystic dysplastic kidney (MCDK), including its impact on long-term renal growth. MATERIALS AND METHODS: We performed a retrospective cohort study of 75 patients with MCDK. The primary outcomes were resolution of contralateral VUR and long-term renal growth. The Kaplan-Meier method was used to compare time to spontaneous resolution based on VUR grade. Mixed regression models were used to compare longitudinal changes in sonographic renal length. RESULTS: Contralateral VUR was present in 19 of the 75 cases (26.4%) and was low grade (I-II) in 9. After a median followup of 4.4 years spontaneous resolution was more common in patients with grades I and II (89%) versus grades III and IV VUR (50%) (p = 0.14). Median time to resolution did not differ by grade (20.1 vs 31.3 months, p = 0.3). One child underwent ureteral reimplantation and no patients had hypertension or renal insufficiency. Mixed regression analysis demonstrated no significant difference in the annual increase in renal length based on VUR status (p >0.20). CONCLUSIONS: In the largest series to date of patients with MCDK and contralateral VUR we found that spontaneous resolution in a relatively short period can be anticipated for most children. Furthermore, VUR is not a significant threat to the growth of the solitary renal unit in the first few years of life.

Cohort Studies↗
PubMed2002

[Multicystic dysplastic kidney: natural history of the affected and the contralateral kidney compared to the normal and solitary kidney].

INTRODUCTION: Complications of multicystic dysplastic kidney are rare, but hypertension and malignant transformation represent real danger. PATIENTS/METHODS: In a 10-year period, 94 infants (60 boys, 34 girls) with multicystic dysplastic kidney were diagnosed. The data obtained from these patients were compared to 70 children with solitary kidney of which 36 were newborns. In 80 cases (85%) the diagnosis of multicystic dysplastic kidney was already suspected by prenatal sonography. RESULTS: Abnormalities of the contralateral kidney were found in 15 of the 94 patients (16%). Complete involution of the multicystic dysplastic kidney was observed in 19, and a decrease in size in 42%. In 37 children (39%) the dysplastic kidney has been removed at the age of about 1 year because of no involution. The length of the contralateral kidney, compared to the normal standard was already significantly larger at birth. In newborn babies with unilateral renal agenesia the solitary kidney was also significantly longer. CONCLUSIONS: Compensatory hypertrophy of single functioning kidneys occurs in utero both in patients with multicystic dysplastic kidney and in those with unilateral renal agenesia. Based on the results of these and previous studies, early nephrectomy cannot be recommended in the newborn period. Surgery remains an option for patients who have no involution at the time of about 1 year of age.

Female↗
PubMed2002

A family study and the natural history of prenatally detected unilateral multicystic dysplastic kidney.

PURPOSE: We document the inheritance pattern of multicystic dysplastic kidney in 3 affected families and screen first-degree relatives of a cohort of children with prenatally detected multicystic dysplastic kidney for renal anomalies. The study also afforded an opportunity to document the natural history of prenatally detected multicystic dysplastic kidney. MATERIALS AND METHODS: We identified 3 families during clinical treatment of children with prenatally detected multicystic dysplastic kidneys. Other members of these families were evaluated with renal ultrasonography. For the family screening study index cases were identified from a fetal uropathy database. A total of 94 first-degree relatives (52 parents, 35 full siblings and 7 half siblings) of 29 children with prenatally detected multicystic dysplastic kidneys were studied with urinary tract ultrasonography, blood pressure measurement, urinalysis and plasma biochemistry. RESULTS: Two families had affected sibling pairs, 1 of which also had a half sibling with vesicoureteral reflux. The third family included 3 individuals with multicystic dysplastic kidney and 1 with renal agenesis thought to have resulted from involution of multicystic dysplastic kidney. This family is consistent with autosomal dominant inheritance with variable expressivity and reduced penetrance. In the screening study ultrasonography did not demonstrate significant renal anomalies in any of the 94 first-degree relatives of the multicystic dysplastic kidney index cases. Followup assessment of prenatally detected multicystic dysplastic kidneys in index cases demonstrated total involution in 52% at a median age of 6.5 years with no multicystic dysplastic kidney related morbidity. CONCLUSIONS: Multicystic dysplastic kidney can be familial but is most commonly a sporadic anomaly. Formal screening of relatives is not recommended. Followup data on a cohort of children with prenatally detected multicystic dysplastic kidney add further support to conservative management.

Female↗