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Late Presenting Unilateral Multicystic Dysplastic Kidney with
Ipsilateral Pelviureteric Junction Obstruction and Hypertension in

an Adolescent: An Incidental Finding
Lere P. Oluwadare1, Sampson C. Aliozor2, Pauline K. Akowundu3, Adebowale T. Odunafolabi4,5,

Joshua O. Awofeso6, Olatunbosun O. Olawanle7, Oluwaseye F. Oyeniran8, Odutola I. Odetunde9,10,11

1Division of Early Childhood Development, Neurology & Infectious Diseases, Department of Paediatrics,
Redeemer’s Health Village, Redemption City, Ogun State, Nigeria

2Division of Paediatric Surgery, Department of Surgery, Redeemer’s Health Village, Redemption City,
Ogun State, Nigeria

3 Division of Neonatology, General Paediatrics & Adolescent Medicine, Department of Paediatrics,
Redeemer’s Health Village, Redemption City, Ogun State, Nigeria

4Division of General & Laparoscopic Surgery, Department of Surgery, Redeemer’s Health Village,
Redemption City, Ogun State, Nigeria

5Department of Surgery, Faculty of Clinical Sciences, College of Medicine, Afe Babalola University,
Ado-Ekiti, Nigeria

6 Department of Radiodiagnosis & Interventional Radiology, Redeemer’s Health Village, Redemption
City, Ogun State, Nigeria

7Department of Anaesthesia & Critical Care, Redeemer’s Health Village, Redemption City, Ogun State,
Nigeria

8Division of Fetomaternal Medicine & Gynae-oncology, Department of Obstetrics & Gynaecology,
Redeemer’s Health Village, Redemption City, Ogun State, Nigeria

9Department of Paediatrics, Faculty of Clinical Sciences, College of Medicine, University of Nigeria,
Nsukka, Nigeria

10Paediatric Nephrology Unit, Department of Paediatrics, University of Nigeria Teaching Hospital,
Enugu, Nigeria

11Division of Nephrology & Paediatric Critical Care, Department of Paediatrics, Redeemer’s Health
Village, Redemption City, Ogun State, Nigeria

DOI: https://doi.org/10.51584/IJRIAS.2025.100900088
Received: 21 Sep 2025; Accepted: 28 Sep 2025; Published: 23 October 2025

ABSTRACT

15 year old female adolescent who presented on account of recurring abdominal pain of 4 years. Nil preceding
history of trauma or fall. Not a known sickle cell disease patient. Nil previous abdominal surgery. Blood
pressure at presentation was elevated for age and gender. Investigations revealed a non functioning dysplastic
left kidney with hydronephrosis. She subsequently had left nephrectomy. Blood pressure thereafter normalised.
She remains clinically stable after 12 months of follow up.

Key words: Abdominal pain, Hypertension, Multicystic dysplastic kidney, Congenital anomalies of the kidney
and urinary tract, Pelviureteric junction obstruction

Aim: To highlight the importance of detailed clinical clerkship, thorough physical examination, high index of
suspicion and exhaustive relevant investigations (laboratory and radiological) for all cases presenting to any
healthcare facility.

INTERNATIONAL JOURNAL OF RESEARCH AND INNOVATION IN APPLIED SCIENCE (IJRIAS)
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Presentation

15 year old senior secondary student who resides with her parents in Southwest Nigeria. Presented on account
of recurring abdominal pain of 4 years’ duration. Onset of pain was insidious. Nil history of trauma or fall
prior to onset of symptom. Nil history of frequent passage of loose stool or vomiting. Nil associated fever. No
history of reduced urination. Nil urinary frequency, urgency or urge incontinence. Nil history of bedwetting.
Pain is generalised and is not referred to other parts of the body. Aggravated by exertion. Relieved by the use
of analgesic and rest. Pain occurs about once in three months, intermittent within a 24-48 hour period and then
resolves. It is not associated with her menstrual cycle.

Nil previous history of hospital admission. Nil prior blood transfusion or surgery. Not a known HbSS patient
(Hb Genotype AA). Nil known drug allergies. Attained menarche at the age of 11 years. Menstrual flow lasts
4days in a 28-day cycle. Nil history of coitarche or contraceptive use. No dysmenorrhea, intermenstrual
bleeding or mucopurulent vaginal discharge. Product of term, uneventful gestation. Details of the prenatal
ultrasound findings could not be recalled by the mother. Neonatal period was not adversely eventful. She has
no known food or drug allergies. She is in the upper 10th percentile of her year in school. The 3rd child in a
monogamous family of three children. Nil family history of renal disorders. There was no history of parental
consanguinity.

Examination revealed a healthy-looking female adolescent who was not in any obvious respiratory or painful
distress. She was neither pale nor icteric. Breasts, axillary hair and pubic hair were all at Tanner Stage 5 of
development.

Anthropometric Findings:

Weight: 72.5kg

Height: 1.54m

Body surface area: 1.76m2

Abdomen was full and moved with respiration. There were no areas of tenderness. Intra-abdominal organs
were not palpably enlarged. Bowel sounds were normal. Digital rectal examination did not reveal any
abnormality. Cardiovascular system examination revealed a blood pressure of 134/85mmHg (which was
elevated for her age and gender). Other cardiovascular findings were normal. The other systemic examination
findings were normal.

The initial working diagnoses were:

 ?Functional Abdominal Pain in a female adolescent
 Secondary Hypertension ?cause in an adolescent

Table 1: Initial investigation findings

FBC E,U,Cr Abdominal USS CT Abdomen Others
HCT: 34% K+: 3.5mmol/L A huge multiloculated

cystic mass (measuring
232x133mm) with
homogenous internal
echoes was visualised
in the left renal bed. No
normal kidney was
visualized, on the left.

Bilateral obstructive
uropathy and non
functioning left kidney 2º
to ?PUJ Obstruction.

Renal scintigraphy
(MAG3) showed a
non-functioning left
kidney with no
obstruction on the
right.


WBC: 6,490/µL Na+: 127mmol/L Urinalysis: Normal
findings

Neutrophil: 61% Cl-: 117mmol/L
Lympocytes: 35% HCO3

-: 20mmol/L RVS: Non reactive to
HIV I & II

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Eosinophils: 1% Urea: 9mg/dL
Basophils: 2% Creatinine: 0.7mg/dL HBsAg: -ve
Monocytes: 2%

Platelets:
263,000/µL

AntiHCV: -ve

MAG3: Mercaptoacetyltriglycine

Treatment

Parents and the patient were informed about the investigation results. They were also counselled on the need
for left nephrectomy for long term optimal blood pressure control in order to prevent complications associated
with persistently elevated blood pressure. She was commenced on low dose antihypertensive (oral Amlodipine
2.5mg daily) which she had for a week and was scheduled for surgery. Blood pressure prior surgery was
129/78mmHg.

Intra operative findings were:

 Grossly normal right kidney; no compensatory hypertrophy
 Markedly dilated multi lobulated left kidney of about 30cm by 20cm extending towards the midline

from the left lumbar region
 Dilated renal pelvis with constriction at the left pelviureteric junction
 Normal bowel, spleen and liver

Course

Immediate post-operative period was essentially not adversely eventful. Blood pressure was initially elevated
(range 145/80 – 150/85mmHg) but it subsequently normalised over the succeeding 24hour period (120/70 –
126/72mmHg). She had adequate analgesia for pain relief. Post-operative haematocrit was 33%. Graded oral
sips were commenced on post-operative day (POD) 3 and she was tolerating full enteral feeds by POD4. She
was allowed home on the sixth post-operative day (POD6). Anti-hypertensive was discontinued after the first
week of follow up. Blood pressure readings remained optimal. The follow up periods were as follows: one
week, six weeks, three months, six months and twelve months post surgery.

Table 2: Anthropometry, Blood Pressure, Serial Serum Electrolytes, Urea and Creatinine in the immediate
post-operative period and during follow up

Parameter 24hours 1 week 6 Weeks 3 Months 6 Months 12 Months
Weight (kg) - 72.5 77.0 73.5 80.5 78.0
Height (m) - 1.54 1.55 1.55 1.55 1.55
Body surface area (m2) - 1.76 1.82 1.78 1.86 1.83
Blood pressure (mmHg) 122/70 120/75 118/70 120/71 116/70 120/72
K+ (mmol/L) 4.2 3.4 3.9 4.3 4.0 4.0
Na+ (mmol/L) 116 140 126 137 137 133
Cl- (mmol/L) 122 114 115 115 107 111
HCO3

- (mmol/L) 21 29 19 22 22 19
Urea (mg/dL) 5 7 7 9 11 6
Creatinine (mg/dL) 0.6 0.5 0.6 0.8 0.7 0.7

Summary of histopathologic finding of the specimen (Left kidney)

 A nephrectomy specimen with overlying perinephric fat
 Weighed 500g
 Dimensions: 12cm x 7cm x 6cm
 Cut section shows replacement of normal renal tissue by multiple large cystic cavities lined by

urothelium in some areas and flattened epithelial cells in other areas
 There was extensive fibrosis of the interstitium

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 Overall features were in keeping with multicystic renal dysplasia

The other first degree relatives were evaluated for hypertension and were also screened for the possibility of
kidney dysplasia with ultrasound sonography. The outcomes were not suggestive of kidney disease.

DISCUSSION

Multicystic dysplastic kidney (MCDK) is one of the common renal cystic diseases that are identified as

congenital anomalies of the kidney and urinary tract (CAKUT).1 Other examples of CAKUT include the
following: Polycystic kidney disease (PKD), Duplex kidneys, Horse shoe kidneys, Ectopic kidneys and
Ectopic ureters.2,3 Urinary malformations in patients with MCDK consist of vesicoureteral reflux (VUR),
ureteropelvic junction obstruction, and kidney stones.2 They are the commonest form of congenital anomalies
in the paediatric age group.4 The genetic causes for most cases remain unknown.5,6

Although the pathogenesis of MCDK remains unclear, failure of the ureteric bud to integrate and branch
appropriately into the metanephros during development in early childhood is one of the postulates.2,3,7 The
incidence of MCDK varies widely, and it has been reported to be more frequently unilateral, with a higher
prevalence in the left kidney,4,8 although some other studies have a higher prevalence on the right.9,10 Some
studies put the estimate at in 1 in every 1000 to 4300 live births.7,11

Majority of cases are detected either during routine antenatal ultrasonography studies or shortly after birth.3,4
In extremely rare cases, they were not detected until adolescence or in early adulthood.10,11 The child reported
in this case study was one of the late presenters. Reasons for the late presentation could be due to: ease of
access to over-the-counter medications in our environment (for pain relief),12,13 apparent clinical stability of
the patient (symptom not severe enough to necessitate hospital admission) and the level of clinical expertise at
the health facilities she had previously presented to before arrival at our facility.

Pain (abdominal and flank), painful micturition and poor urinary stream have been identified as some of the
presenting symptoms in children with CAKUT with chronic abdominal pain either being silent or vague.14
MCDK, however, is generally not symptomatic.15 CAKUT have been reported to be the leading cause of
chronic renal failure.2,4 The frequency of hypertension in children with unilateral MCDK (UMCDK) was
reported as 5.4 per 1000 in a systematic review and those with an abnormality in the opposite kidney had a
higher risk of hypertension.16 Two mechanisms have been postulated for the pathogenesis of hypertension in
UMCDK. Primarily, hyperreninemia due to the dysplastic kidney and secondarily, hyperfiltration in the
contralateral kidney.1,17 Older studies state that following the removal of a unilateral poorly or nonfunctioning
kidney (including MCDK), resolution of hypertension occurs,17 however, more recent studies favour a
conservative approach.9,10 The patient in this study was an exception because of her late presentation, hence,
surgical removal of the non-functioning left kidney was opted for because she had grown well past the age
when spontaneous involution of the dysplastic kidney was expected to have occurred.8,10,18 Removal of the
dysplastic kidney reduces excessive renin production which contributes to hypertension in UMCDK.1,17 She
has been followed up for twelve months since she had left nephrectomy, and there has been no record of
hypertension during any of the outpatient visits.

Malformations of the reproductive system on the ipsilateral (same) side and various other malformation
syndromes are often associated with CAKUT.3,4 Examples include seminal vesicle cyst (in males), Müllerian
anomalies and Gartner’s cyst (in females).19,20 The radiological investigations done on this patient did not
suggest any reproductive organ abnormality and the adolescent is being kept on active surveillance by the
gynaecological team of the study centre. No other malformation of any organ or system was detected in patient
of this case study. Studies have reported that about 10-50% of children with CAKUT have affected family
members (first degree relatives) with a positive history of renal anomalies or disorders of the urinary tract (e.g
Renal agenesis, Nephrolithiasis, Pelvic Kidney, MCKD), ,8,21 however, the relatives of the child in this case
study were not affected. Because the initial screening done on the relatives was negative for CAKUT, genetic
evaluation is not being considered for them. However for the child, it is being actively considered to predict
the impact of the disease on her reproductive capacity.

INTERNATIONAL JOURNAL OF RESEARCH AND INNOVATION IN APPLIED SCIENCE (IJRIAS)
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This study is of note because of the following reasons. First, most cases of MCDK are diagnosed prenatally
and involution of the affected kidney (either complete or partial) is expected to have occurred latest by the fifth
year of life.8,10,18 Second, the patient presented with recurring abdominal pain of four years (with onset in her
teenage years) without a definite aetiology of the pain being arrived at until presentation at our facility. Vital
signs at presentation in our facility showed elevated blood pressure for age and gender. This necessitated
further investigations and eventual nephrectomy. If the diagnosis of the multicystic dysplastic kidney had been
missed, hypertension would have persisted which places the patient at a significantly higher risk of chronic
renal failure compared to the general population.22 This underscores the importance of detailed clinical
clerkship, thorough physical examination, exhaustive relevant investigations (laboratory and radiological) for
all cases presenting to any healthcare facility in order to arrive at definite diagnosis and offer the most
appropriate modality of care.

CONCLUSION

Unilateral multicystic dysplastic kidney, although usually diagnosed very early, occasionally presents late and
systemic hypertension in an older child or adolescent may be a clue to this diagnosis.

Strengths of the Study

Apart from the radiologic and nuclide studies, we were able to get a histological confirmation which is the
hallmark of the diagnosis of renal dysplasia.

LIMITATION

The patient has only been followed up for a 12-month period. A longer duration of follow up would have been
more appropriate in determining the long term outcome of the patient vis-à-vis kidney function and
reproductive capacity.

ACKNOWLEDGEMENTS

The authors wish to thank all the medical personnel, the nursing team and the ancillary members of staff that
provided optimal care to this patient while on admission.

Funding

This work did not receive any financial support from any funding agency.

Disclosures

The authors have no conflicts of interest to declare.

Consent

Written informed consent from the parents (and assent from the patient) was obtained.

Ethical Clearance

The medical ethics committee of the study centre granted a full waiver for a retrospective examination of the
patient’s records for the purpose of this case study.

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