Giant Hydronephrosis in aChild
Tarek M. ElKaramany
Department of Urology, Benha Faculty of Medicine, Benha, Egypt
ABSTRACT
Giant hydronephrosis is rare in children. A 7-year- old male child with giant hydronephrosis due to congenital ureteropelvic junction (UPJ) obstruction is reported. Radiological features and management are briefly discussed. The literature is reviewed
KEWORDS: Giant hydronephrosis; UPJ obstruction; Intrinsic.
INTRODUCTION
Giant hydronephrosis is defined as a kidney containing more than 1L of fluids in its collecting system [1]. The radiological diagnostic criteria are a hydronephrotic renal pelvis that meets or crosses the midline, occupies a hemi-abdomen and extends for a length offive vertebrae or more [2]. The commonest cause is UPJ obstruction which occurs in 80% of causes [2]. Giant hydronephrosis is a slowly progressive disease and a huge abdominal mass or distended abdomen may be the only sign. The differential diagnosis includes ascites, intraperitoneal, and retroperitoneal cysts, pancreatic pseudocyst, and ovarian cyst or tumors [3]. The majority of giant hydronephrotic kidneys are non-functioning and symptomatic, making nephrectomy the procedure of choice [4]. With higher standards of medical care, giant hydronephrosis is now a rare urological entity.
The author feels that this case reported here is worth documenting, since the degree of pelvic dilatation was most unusual especially in a child of 7 years.
CASE REPORT
A 7-year-old boy presentedwith a threemonths history of progressive painless abdominaldistention. Physical examination revealed a huge swelling of the abdomen (figure 1). The swelling was not tender, smooth, tense, dull topercussion, and trans-
illumination test was positive. The rest of physical examination was unremarkable. Urinalysis demonstrated one to two RBCs and two to three pus cells per high power field. Serum creatinine andblood chemistry tests were normal.An ultrasonogram of the abdomen revealed a huge multilocular cystic abdominal mass occupying most of the abdominal cavity, the origin of which was not determined. A multi-slice contrast enhanced CT scan of the abdomen showed a massively enlarged non- enhanced left hydronephrotickidney filling the entire abdomen, displacing the spleen superiorly, pancreatic tail anteriorly, and bowel loops to the periphery (Figure2a;b). On the basis of CT images and DTPA renography, the diagnosis of a non-functioning giant hydronephrosis was ascertained. Therefore, total left nephrectomy was performed. Transperitoneal approach through midline incision extended from xiphoid to 3 cm. below the umbilicus provided excellent exposure (Figure3). The hydronephrotic kidney was completely mobilized and slowly decompressed by using a catheter, and approximately 6.4 L of urine was drained. Cardiorespiratory status was stable throughout the operation. The UPJ was explored and confirmed to be stenosed, no aberrant vessels were found(Figure 4). The patient had uneventual recovery and discharged on Day 4. The histopathology was reported as a hydronephrosis due to intrinsic UPJ obstruction with resultant chronic tubulointerstitial nephritis and permanent renal atrophy.
DISCUSSION
The differential diagnosis of large cystic retroperitoneal mass arising from the kidney includes most commonly UPJ obstruction, but it can also occur as a consequence of giant renal cysts, vesicoureteral reflux, megaureter, and posterior urethral valves [2,5].
Children with UPJ obstruction may present with intermittent abdominal pain, hematuria or urinary tract infection [6]. Fewer delayed cases are presented, as this one, with progressive abdominal distension [7,8].
The most important aspect of management is early diagnosis with accurate preoperative delineation of anatomy and function. Therefore CT and DTPA renography was helpful in ruling out other cystic masses of abdomen and kidney and to confirm the final diagnosis.
The majority of giant hydronephrosis are non- functional and symptomatic, making the nephrectomy the procedure of choice [4]. However, there have been reports of functional giant hydronehrotic kidneys treated with reconstructive procedures [2,9].
CONCLUSION
Giant hydronephrosis in children is rare. Preoperative evaluation with CT scan is useful in confirming the diagnosis and delineation of the extent and relations to surrounding structures. Midline transperitoneal approach provides safe and efficient approach to the hydronephrotic sac.
REFERENCES
1. Whitmore RB, 3 rd, Schellhammer PF. Giant hydronephrosis of a duplex system associated with ureteral ectopia. J Urol. 1989 ; 141: 1186 – 88
2. Crooks KK, Hendren WH, Pfister RC. Giant hydronephrosis in children. J Ped Surg. 1979; 14: 844- 50.
3. Wootton – Gorges SL , Thomas KB , Harned RK , Wu SR , Stein – Wexler R,Strain JD . Giant cystic abdominal masses in children. Ped Radiol. 2005; 35: 1277 – 88.
4. Hemal AK, Wadhwa SN, Kumar M, and Gupta NP. Transperitoneal and retroperitoneal laparoscopic nephrectomy for giant hydronephrosis . J Urol. 1999; 162: 35 – 39.
5. Chiang PH , Chen MT , Chou YH , Chiang CP , Huang CH , and Chien CH . Giant hydronehrosis: report of 4 cases with review of literature. J Formos Med Assoc. 1990; 89: 811 – 17.
6. Rooks VJ, and Lebwitz RL. Extrinsic uretropelvic junction obstruction from a crossing vessel: demography and imaging. Ped Radiol. 2001; 31: 120 – 4.
7. Dahniya MH, RathiAK, Ahmed R. Giant pelviureteric hydronephrosis in a child. Ped Radiol. 1988; 18: 501 – 2.
8. Yilmaz E, Guney S. Giant hydronephrosis due to ureteropelvic junction obstruction in a child: CT and MR appearencs. J Clin Imag. 2002; 26: 125 – 28.
9. Hemal AK, Aron M, Wadhawa SN. Nephroplication and nephropexy as an adjunct to primary surgery in the management of giant hydronephrosis. Br J Urol. 1989; 81: 673 – 77.
Figure1. Appearance of the patient before operation
Figure2a. A CT scan showing a giant nonenhancing multilocular mass corresponding to the left kidney, note; the left colon is displaced anteriorly just behind the anterior abdominal wall.
Figure2b. CT sagittal view showing an enormous dilatation of the pelvicayceal system occupying the entire abdomen.
Figure3.Excellentexposure through a transabdominal midline approach.
Figure4. Full mobilization of the kidney, evident UPJ obstruction.
Corresponding Author:
Tarek El-Karamany ,MD.
Benha Faculty of Medicine, Benha, Egypt .Tel:0020123940648
E-mail:
Published in" Arab Journal of Urology", Volume 8- No. 4- December 2010