|Year : 2016 | Volume
| Issue : 2 | Page : 122-124
Multicystic dysplastic kidney with associated contralateral renal ectopia in an infant: A rare association
Abdullahi Musa1, Lawal Waisu Umar2, Mairo A Bugaje2, Akuse M Rosamund2
1 Department of Paediatrics, Ahmadu Bello University Teaching Hospital, PMB 06, Shika, Nigeria
2 Department of Paediatrics, Ahmadu Bello University, Zaria, Nigeria
|Date of Submission||22-Oct-2015|
|Date of Acceptance||30-May-2016|
|Date of Web Publication||21-Jun-2016|
Dr. Abdullahi Musa
Department of Paediatrics, Ahmadu Bello University Teaching Hospital, PMB 06, Shika, Zaria
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
|How to cite this article:|
Musa A, Umar LW, Bugaje MA, Rosamund AM. Multicystic dysplastic kidney with associated contralateral renal ectopia in an infant: A rare association. Sub-Saharan Afr J Med 2016;3:122-4
|How to cite this URL:|
Musa A, Umar LW, Bugaje MA, Rosamund AM. Multicystic dysplastic kidney with associated contralateral renal ectopia in an infant: A rare association. Sub-Saharan Afr J Med [serial online] 2016 [cited 2020 May 31];3:122-4. Available from: http://www.ssajm.org/text.asp?2016/3/2/122/184381
| Introduction|| |
Multicystic dysplastic kidney (MCDK) is a structural renal anomaly characterized by the presence of multiple, varying size of noncommunicating cysts separated by dysplastic renal parenchyma, with the absence of normal pelvicalyceal system.  It is one of the most common causes of abdominal masses in newborns and cystic malformation of the kidney in infancy.  The prevalence of unilateral MCDK is between 1 in 4300 and 1 in 2400 live births.  It occurs more commonly in males with the left kidney more frequently affected.  Bilateral MCDK is rare and usually not compatible with life.  The wide variation in prevalence has been attributed to the fact that the congenital anomaly has been known to regress spontaneously.  Hiraoka et al. reported that many patients diagnosed in later childhood or adulthood with unilateral renal agenesis could actually have been cases of regressed MCDK, rather than a total agenesis of the affected kidney.  Unilateral MCDK is much more common than the bilateral anomaly. Bilateral MCDK is associated with nonfunctional fetal and neonatal kidneys and pulmonary hypoplasia, a combination that is incompatible with extrauterine life. ,
Although the anomaly is usually associated with contralateral genitourinary tract abnormalities such as vesicoureteral reflux and obstruction of the ureteropelvic junction, ectopic kidney is a rare combination. ,
We report a case of MCDK disease with contralateral renal ectopia in an 8-month-old child with urinary tract infection (UTI) who presented at the Ahmadu Bello University Teaching Hospital (ABUTH), Zaria, Nigeria.
| Case Report|| |
A.U. was an 8-month-old boy who presented for the first time to the Emergency Paediatric Unit of ABUTH, Zaria, with fever of 6 weeks duration, progressive abdominal distension for 3 weeks, crying on micturition and pyuria of 1 week. His urinary stream had been normal since birth. His mother had a home delivery at term with no complications after a supervised pregnancy that was free of significant medical and obstetrics events. He had not been observed to fall sick since delivery, this being his first presentation with medical complaints to hospital. Although he was small in size from birth (birth weight unknown), his developmental milestones were satisfactory as compared to his older siblings, none of whom had similar problems. Examination revealed an acutely ill, febrile (38.9°C), with moderate pallor and moderate dehydration, but with otherwise no obvious dysmorphic features. He weighed 8 kg (94%) and had a length of 66 cm (97%). His abdomen was generally distended with more fullness around the right iliac fossa. The left kidney was palpable 4 cm below the left costal margin while the right kidney was neither palpable per abdomen nor balloted at the renal angle. There was a superficial firm mass in the right iliac fossa measuring 6 cm × 5 cm, which was smooth and nontender. There were no palpable liver or splenic enlargements. His blood pressure was normal (78/56 mmHg) and other systemic examination findings were essentially normal. A clinical diagnosis of UTI with underlying renal anomaly was made. His urine was cloudy and microscopy showed numerous pus cells with Gram-negative rods while culture grew Klebsiella spp. Abdominal ultrasound revealed large multiple cysts in the position of the left kidney [Figure 1] while contrast-enhanced computed tomography scan [Figure 2] revealed an ectopic right kidney located in the pelvic region that showed contrast excretion with a normally situated left MCDK that had no excretion of contrast. His serum biochemistry, hematological, and other laboratory results were essentially within normal limits.
|Figure 1: Patient's abdominal ultrasound showing multiple cysts in the region of the left kidney|
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|Figure 2: Contrast-enhanced computed tomography scan of the abdomen showing the right pelvic kidney with contrast excretion and lack of excretion from the left kidney|
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The UTI was treated with intravenous ceftriaxone at a dose of 75 mg/kg/24 h given for 10 days and therapy completed with oral cefixime for an additional 4 days. He also had intravenous and oral fluids to correct and maintain hydration. The parents were duly informed of the nature of the disorder and were counseled about the need for protecting and preventing the child from undue rigorous activity, rough handling, or aggressive play. The importance of cautious restraint in handling and training the child to refrain from strenuous contact sports to reduce chances of blunt injury, and protect the functional kidney, as well as regular follow-up for clinical monitoring was also discussed at discharge. The UTI responded well to treatment before he was discharged, but he was brought to the clinic for follow-up care only once after discharge and he subsequently defaulted from follow-up despite adequate counseling. Effort to contact parent was hindered by lack of phone number on the case note.
| Discussion|| |
In this report, unilateral MCDK was detected in a male child and on the left side, a finding that is consistent with previous reports. , Although prenatal diagnostic imaging is possible, the mother of this patient did not have prenatal ultrasound. This is consistent with what prevails in most developing countries where ultrasound is not routinely done as part of antenatal care. , Consequently, this anomaly is often incidentally discovered during physical examination or from abdominal ultrasound for other reasons  as demonstrated by this case. Diagnosis may therefore not be made because the anomaly usually remains asymptomatic only to be discovered much later in childhood or even in adult life.  The presentation in this patient was of unilateral MCDK disease in the second half of infancy that was yet to manifest features to suggest renal impairment or hypertension. Previous case series of MCDK showed association with a contralateral normally situated kidney, with urinary tract abnormalities. The commonest abnormalities were vesicoureteral reflux and pelviureteric junction obstruction which occurred in about one-third of patients. ,,, In contrast to these reports, however, this patient had a functional kidney situated in the pelvis on the contralateral side, which is an uncommon occurrence that is rarely observed. In an 11-year review of cases of MCDK, Rios et al. reported only a single case of unilateral left-sided MCDK with the right pelvic kidney. 
Although extrarenal anomalies including congenital heart disease, esophageal atresia, neural tube defect, and genital and skeletal anomalies have been reported in patients with MCDK,  no such abnormalities were found in this patient. While some studies have reported increased incidence of hypertension, infection and malignant transformation (Wilm's tumor, adenocarcinoma, and embryonal tumor), ,, this patient only presented with infection with no evidence of other complications. This could have been the first of recurrent episodes of UTI arising from the onset of vesicoureteral reflux or obstructive sequelae; conditions that have been reported to progressively develop over time in such patients. ,, This could not be ascertained however as the child was lost to follow-up.
The disorder occurs sporadically although chromosome abnormalities have been detected in about 3% of MCDK cases, especially those with accompanying extra-renal anomalies. There was no family history suggestive of similar illness, and although chromosomal studies were considered as part of follow-up evaluation, they could not be carried out due to the child's loss to follow-up. Comprehensive prenatal evaluation including fetal ultrasonography as a routine is the best approach to early diagnosis of MCDK, which affords the opportunity to plan its prompt multidisciplinary management with a pediatric urologist after delivery. ,
| Conclusion|| |
We reported an unusual case of unilateral MCDK associated with a functional contralateral pelvic kidney demonstrated by imaging, a rare variant different from the much more common form of the anomaly known to occur with a normally situated, contralateral kidney. In such patients, there is a need for multidisciplinary follow-up care including monitoring for the development of hypertension and urologic surgery for possible malignant transformation of the nonfunctional dysplastic kidney.
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Conflicts of Interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]