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CASE SERIES
Year : 2023 | Volume
: 50
| Issue : 1 | Page : 121-124
Multicystic dysplastic disease of kidney in pediatric age group: A series of five cases
Sibnath Gayen1, Arabinda Kar Chowdhury2, Picaso Chowdhury3, Pankaj Kumar Halder4, Damini Dutta4, Partha Chakraborty4
1 Department of Pediatric Medicine, R G Kar Medical College, Kolkata, West Bengal, India
2 Department of General Surgery, R G Kar Medical College, Kolkata, West Bengal, India
3 Department of Pediatrics, Tarakeswar Rural Hospital, Hooghly, West Bengal, India
4 Department of Pediatric Surgery, R G Kar Medical College, Kolkata, West Bengal, India
Correspondence Address:
Pankaj Kumar Halder
Sarada Palli, Baruipur, Kolkata - 700 144, West Bengal
India
Source of Support: None, Conflict of Interest: None
CheckDOI: 10.4103/jss.jss_137_22
Multicystic dysplastic kidney (MCDK) is a congenital somatic maldevelopment in which the renal cortex is replaced by numerous cysts of varying sizes with insinuating dysplastic parenchyma in between. In 76% of cases, it is unilateral occurring on the left kidney. The condition could be diagnosed by ultrasound during pregnancy or after delivery. According to various studies, it follows a benign course and can be managed conservatively. Here, we present a series of five cases of unilateral MCDK that required surgical management due to either parent's psychosocial constraints or incidental discovery during surgery.
Keywords: Dysplastic, kidney, nephrectomy, nephrectomy, observation, unilateral
The multicystic dysplastic kidney (MCDK) is characterized by numerous cysts of various sizes, dysplastic parenchyma in the interstices that resembles a cluster of grapes, and the absence of the pelvicalyceal system.[1] It usually manifests as a unilateral disorder, more typically in men, and more often on the left kidney. The bilateral MCDK is incompatible with life.[2] The condition is effectively diagnosed by antenatal ultrasonography (USG), and the mean age at the time of diagnosis is 28th gestational weeks. In addition, 3D images can be used to evaluate the severity and extent of MCDK, which greatly helps the medical team in the prenatal counseling of mother.[3] MCDK has a benign course with a few sequelae. Most often it regresses and disappears eventually.
Case ReportsCase 1
A 30-year-old pregnant woman was referred to us for antenatal counseling, as her transabdominal fetal USG at 28 weeks of gestation revealed right-sided hydronephrosis. She underwent magnetic resonance imaging to confirm the diagnosis which revealed that the fetal kidney (right) has been replaced by multiple thin-walled, noncommunicating cysts of various diameters, with nondilated pelvicalyceal systems. The left kidney, however, was normal. Postnatal USG suggested MCDK and isotope renogram revealed a nonfunctional right kidney. We were planning to follow-up the baby with serial USG. However, according to the baby's mother, the baby began experiencing flank pain, dysuria, and hematuria. Therefore, at the age of 11 months, we performed a right nephrectomy. The biopsy revealed multiple cysts, the largest of which measured 2 cm × 1.7 cm. The cyst wall was lined by cuboidal or flattened epithelium and had a thickness of 0.1 cm. A few primitive glomeruli and tubules could also be seen in addition to dysplastic mesenchymal components in the interstitium.
Case 2
A 4-month-old boy with a lump on the left side of the abdomen and occasional pain was brought to our outdoor. On local inspection, a ballotable mass was found in the left lumbar region. Contrast-enhanced computed tomography (CECT) of the abdomen suggested a multicystic lesion in the left kidney. The isotope renogram (99mTc Diethylenetriamine pentaacetate (DTPA)) revealed a very low function in the kidney. The patient was symptomatic, and the parents declined to have a long-term follow-up due to their psychosocial obligations. At the age of 7 months, we, therefore, performed a left nephrectomy. During surgery, we discovered a distorted left kidney with a dilated renal pelvis [Figure 1]. The biopsy findings suggested MCDK.
Figure 1: Intraoperative pictures of antenatally diagnosed multicystic dysplastic kidney in a 7-month-old male childCase 3
A 28-year-old pregnant woman was sent to our department after an antenatal USG (36th gestational week) of the fetus revealed an enlarged left kidney that was studded with multiple anechoic cysts. We advised the parents that a cystic kidney disease might exist and requested them to follow up with our department following the delivery. Clinicoradiological evaluation was carried out on the infant at the age of 2 months. On CT urography, the left kidney was found to have multiple parenchymal cysts that did not appear to communicate with the pelvicalyceal system. The ethylene dicysteine renogram (EC scan) revealed nonfunctional left kidney. We diagnosed the patient as having MCDK and recommended close monitoring, which included periodic USG. However, because the parents were concerned about subsequent issues, we had to perform the surgery (nephrectomy) at the age of 6 months. During surgery, cystic abnormalities in the left kidney were found [Figure 2]. The biopsy revealed multiple cystic spaces with columnar-to-cuboidal epithelium, as well as foci of renal tubule and abortive glomeruli.
Figure 2: Intraoperative pictures of dysplastic kidney (antenatally detected) in a 6-month-old male childCase 4
A 5-month-old female infant was sent to us for assessment of recurrent urinary tract infection (UTI) and hematuria. A lump on the left side of the abdomen (renal mass) was discovered during a physical examination. The CECT revealed a hydronephrotic left kidney with severely impaired excretion. A micturating cystourethrogram (MCU) identified vesicoureteral reflux (VUR) on the opposite kidney. We intended to perform pyeloplasty through the dorsal lumbotomy approach; however, we discovered MCDK during surgery and performed a left nephrectomy [Figure 3]. The biopsy of the excised specimen revealed MCDK.
Figure 3: Intraoperative picture shows evidence of multiple cystic changes in the left kidney with blind-ending ureter in a 6-month-old female childCase 5
A 2-month-old female baby presented with abdominal distension for a 1-month duration. We found a soft bimanually palpable renal mass and suspected to have hydronephrosis. The USG suggested that the left hydronephrosis was likely caused by ureteropelvic junction obstruction. Her blood pressure was found to be somewhat elevated as well, but no medication was required. On a renal scan (Tc-99m DTPA), there was evidence of gross hydronephrosis of the left kidney as well as decreased renal parenchymal function, with a split function of 4.3% in the left kidney. We opted to have a nephrostomy ± pyeloplasty done as soon as feasible to prevent further damage to the left kidney. However, during surgery, we discovered macroscopic evidence of MCDK, and a nephroureterectomy was carried out. The histology suggested MCDK.
DiscussionMCDK is one of the most common congenital renal abnormalities in children; its prevalence ranges from 1 in 1000 to 4300 live births, and it is also among the most often found aberrations on prenatal USG.[4] It was once believed that the MCDK originated from the aberrant induction of metanephric mesenchyme. Teixeria et al. showed that mutations in particular genes, such as EYA1, SIX 1, and PAX 2, may be responsible. In few cases, genetic abnormality has also been identified.[5],[6]
MCDK is typically unilateral. Bilateral diseases or unilateral MCDK with contralateral renal agenesis is incompatible with life. According to a meta-analysis, unilateral MDCK is more common in male patients and on the left side.[7] Sometimes a mass effect might cause respiratory discomfort in a fetus, requiring repeated percutaneous cyst aspiration, and this can lead to fetal death.[2]
All of the cases in our series had unilateral disease, and 4 (80%, n = 5) of them had right-sided pathology. Bilateral cases most likely died unexpectedly shortly after birth and went unreported. In our series, there were two prenatal diagnoses (40%, n = 5). Importantly, the diagnosis of MCDK was made based on intraoperative findings and tissue biopsy in 2 (40%, n = 5) cases that were initially misinterpreted as hydronephrosis [Table 1].
Table 1: Clinical spectrum of multicystic dysplastic kidney in children (n=5)The initial step should be the evaluation of recurrent UTI due to a coexisting VUR; however, routine MCU in healthy children with unilateral MCDK is not recommended due to the low incidence of VUR. To select patients who require MCU, the USG must be repeated to detect any associated congenital urinary tract defects.[8] Blachman-Braun et al. said that MCU should be avoided in children with MCKD unless signs and symptoms of recurrent UTI or a decline in renal function arise.[9] While assessing such a patient, a Dimercaptosuccinic acid (DMSA) scan should be performed to look for renal scarring due to VUR.[10]
MCDK is incorrigible; however, research has demonstrated that it has a benign course with few sequelae. Several studies revealed that roughly 41% of cases may still have renal remnants after a 10-year follow-up. According to Tiryaki et al., the initial size and side (right > left) of the lesion play a part in the management strategy. A large left-sided kidney had a lower likelihood of involution than a small right-sided kidney (67%).[11] In about one-fourth of cases, spontaneous involution occurs within 14 months of life. Ylinen et al. reported no significant involution after the age of 18 months; therefore, surgery should be carried out at 2 years of age.[12] According to Bloom et al., 40%–65% of pediatric urologists advocated nephrectomy.[13]
Nephrectomy is also indicated in presence of arterial hypertension, which typically develops within the first year of life (23.1%), recurrent UTI (15.9%), flank pain (2.8%), ureteroceles, renal calculi, hematuria, and persistent large cystic kidneys.[14] All of our cases underwent nephrectomy due to either symptomatic MCDK or incidentally discovered MCDK during the pyeloplasty. Schreuder et al. reported that even if the MCDK is removed, a protocol of regular follow-up is still required to assess the growth and function of the contralateral kidney after surgery because one-third of the patients may have associated urinary tract malformations in the contralateral kidney, such as VUR (11%–30%), Pelviureteric junction obstruction (PUJO), or vesicoureteral junction obstruction.[7] The psychosocial limitations in developing nations and the expense of repeated USG may make a longer follow-up challenging. The parents avoided the hassles of routine follow-up because they were worried about the unknown problems with the MCDK. A recent study indicates that MCDK has a very low risk of developing cancer. However, the presence of Cowden disease carries the potential of kidney cancer.[6]
ConclusionIn view of our findings, we suggest selecting an isotope renogram to assess renal function, and a USG to look for concurrent renal anomalies in the contralateral kidney is important. The knowledge of the progression of the MCDK and its consequences is also important for the effective counseling of parents. When long-term frequent check-ups are not feasible, nephrectomy is the only option that should be taken into consideration. Moreover, after surgery, a routine follow-up must be advised.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
References
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