|Year : 2015 | Volume
| Issue : 1 | Page : 69-71
Giant hydronephrosis mimicking as gross ascites in a 6-year-old boy
Chandra Madhur Sharma1, Deepti Sharma2, Ashok Upadhyaya1, Rahul Ranjan3
1 Department of Paediatrics, Rama Medical College, Kanpur, Uttar Pradesh, India
2 Department of Obstetrics and Gynecology, GSVM Medical College, Kanpur, Uttar Pradesh, India
3 Department of Radiodiagnosis, Rama Medical College, Hospital and Research Centre, Kanpur, Uttar Pradesh, India
|Date of Web Publication||8-Jan-2015|
Chandra Madhur Sharma
8/218, Flat No. H, Indralok Apartment, Arya Nagar, Kanpur, Uttar Pradesh - 208 002
Source of Support: None, Conflict of Interest: None
Giant hydronephrotic kidney is a rare form of obstructive uropathy in children and adolescents. The congenital ureteropelvic junction obstruction is the most frequent cause. The CT scan is very important for the diagnosis. We report a 6-year-old boy with progressively increasing abdominal distension since birth. There were no associated urinary or gastrointestinal symptoms. He had bilateral hydronephosis with giant hydronephrosis over right side. The cystic mass occupied the right retroperitoneal space and crossed the midline.
Keywords: Giant hydronephrosis, kidney, ureteropelvic junction obstruction
|How to cite this article:|
Sharma CM, Sharma D, Upadhyaya A, Ranjan R. Giant hydronephrosis mimicking as gross ascites in a 6-year-old boy. Med J DY Patil Univ 2015;8:69-71
| Introduction|| |
The giant hydronephrosis has been defined by Stirling in 1939, as the presence of more than 1000 mL or 1.6% of body weight of fluid in the collecting system. [1,2] It has also been defined as the kidney that occupies a hemiabdomen, which meets or crosses the midline and which is at least 5 vertebrae in length.  The ureteropelvic junction (UPJ) obstruction is defined as an impairment of urine flow from the renal pelvis into the proximal ureter, leading to progressive dilatation of the collecting system and potential renal injury.  It is more common in male newborns (M:F - 2:1) is bilateral in 10% to 40% of the cases and may be associated with other anomalies like vesico-ureteral reflux, renal dysplasia or agenesis.  UPJ obstruction can be intrinsic, due to excessive connective tissue and decrease smooth muscle content in the ureteral wall, or extrinsic, probably because of an anomalous renal vasculature, leading to an aberrant, accessory or early branching lower pole vessel. However, it is still difficult to define if the presence of an aberrant vessel is the real cause of obstruction or just a co-variable of an intrinsic obstruction. With the increasing use of ultrasonography in the prenatal evaluation, nowadays cases of giant hydronephrosis due to UPJ obstruction are uncommonly diagnosed. 
We report a case of a 6-year-old boy with bilateral hydronephrosis and right-sided giant hydronephrosis due to chronic UPJ obstruction despite absence of symptoms or relevant urologic history.
| Case Report|| |
A 6-year-old boy presented to us with complaint of progressively increasing abdominal distension since birth. He had no history of associated urinary or gastrointestinal symptoms. On detailed history the patient was the only child, born to a non-consanguineous marriage with no perinatal or postnatal complications. The patient was delivered by vaginal delivery and there was no significant antenatal history suggestive of oligohydramnios. His motor and social developmental milestones were normal for his age. There was no similar history in any of the relatives of both the parents. On examination child was of average built, weighing 18.2 kg and having a height of 110.5 cm. On physical examination, a large painless, soft, cystic mass was palpable which mimic as ascites. It occupied almost the whole abdomen but predominantly the right hemi-abdomen [Figure 1]. His blood pressure was 92/58 mmHg and routine hematology tests were normal. Surprisingly his blood urea was 38 mg% and serum creatinine was 0.9 mg%. Urinalysis showed 20-25 pus cells/HPF. Abdominal ultrasonography revealed a large cystic mass. Computed tomography revealed bilateral hydronephrosis with severe right hydronephrosis and thinning of renal parenchyma [Figure 2] and [Figure 3] due to probable bilateral congenital UPJ obstruction, with compression of adjacent viscera and lateralization of the great vessels. The peritoneum was compressed and moved to the left but there was no intestinal obstruction. Volume of the right-sided hydronephrotic kidney was 1820 mL. On the basis of the clinical examination and CT scan findings, the diagnosis of a bilateral hydronephrosis with right-sided giant hydronephrotic kidney was made.
|Figure 1: A large painless, soft, cystic mass, occupied almost the whole abdomen but predominantly the right hemi-abdomen, mimicking as gross ascites|
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|Figure 2: Abdominal CT scan showing giant right hydronephrosis with a pressure effect over the bowels and thinning of renal parenchyma|
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|Figure 3: Coronal reconstruction CT scan showing bilateral hydronephrosis with giant right hydronephrosis|
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He was sent to our Pediatric Surgery and Urology Department for further management. In the urology department percutaneous nephrostomy was performed on the right side and the initial drainage was over 1.8 L of clear urine. As right kidney was not salvageable, right nephrectomy was done after 1 month of nephrostomy and pyeloplasty has been planned for the left-sided UPJ obstruction.
| Discussion|| |
Giant hydronephrosis is a rare form of obstructive uropathy, especially in children and adolescents. Comparatively large sizes of kidneys are encountered in newborns and infants where the kidney is larger than other parenchymatous organs. The distended kidney can be palpable through the poorly developed abdominal wall. In older children symptoms of recurrent urinary infections, intermittent flank pains with vomiting and nausea, nephrolithiasis, hematuria, and blood hypertension dominate. In our case, the course was asymptomatic revealing a slow and chronic disease process.
Nowadays most cases of UPJ obstruction are suspected during pregnancy due to the recent widespread use of ultrasonography in the antenatal period, especially after the 28th week when it is most accurate to study the urogenital tract.  In our case patient's mother never had antenatal ultrasonography during her antenatal period. After labor, in case of suspicion of UPJ obstruction is highly recommended close surveillance of the newborn by clinical examination, US and renography with MAG3. Detection of impairment or decrease in split renal function, increase in the antero-posterior diameter, recurrent renal infection and flank pain are the most common indications for surgical treatment. [4,5] Despite most patients being diagnosed in the early years, there are still cases of late diagnosis, including in adulthood, usually associated with an asymptomatic evolution.  The delay in detection of UPJ obstruction can lead to progressive and severe hydronephrosis and loss of function of the affected kidney at the time of diagnosis.  Our case had progressive abdominal distension since birth but his parents never seek medical advice because he had no other associated problems.
Huge distension of the collecting system poses the potential risk of major complications. Rupture of a giant hydronephrotic kidney after weak blunt trauma, like compression by a seat belt, can easily be expected. Ipsilateral ileofemoral vein thromboses due to compression have been reported. 
Conservative treatment or salvage procedures had been reported in solitary or bilateral kidney disease. If unit is salvageable then based upon the anatomical configuration, the type of reconstructive procedure should be selected. Ureteral tapering with reimplantation, dismembered pyeloplasty with nephropexy, primary calycoureterostomy, primary Boari flap calycovesicostomy provide effective dependent drainage in relevant cases. In very poorly functioning unit with gross infection, nephrectomy is the procedure of choice. Laparoscopic nephrectomy for giant hydronephrosis has been reported in few articles.  In Hemal's group of patients who underwent laparoscopic nephrectomy, the greatest volume of fluid aspirated was 5 L in adult men, while the others had from 1.2 to 2.6 L. 
Our case had giant hydronephrosis of the right side. After proper counseling of parents we referred them to pediatric surgery and urology department for further management of their child. In the urology department percutaneous nephrostomy was performed on the right side. As right kidney was not salvageable, right nephrectomy was done after 1 month of nephrostomy and pyeloplasty has been planned for the left-sided UPJ obstruction.
| Conclusion|| |
Giant hydronephrosis is an uncommon entity that may mimic progressive and benign abdominal cystic tumors. Despite most cases being diagnosed with the use of prenatal ultrasonography, giant hydronephrosis may still be seen in children and adult population. CT scan is the gold-standard diagnostic modality for diagnosing giant hydronephrosis.
Giant hydronephrotic kidney can cross the midline and pose as a diagnostic dilemma, therefore in an obscure case of ascites, the possibility of hydronephrosis has to be considered before paracentesis is attempted.
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[Figure 1], [Figure 2], [Figure 3]