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Clinicopathological spectrum of ureteropelvic obstruction at a tertiary care center Niloufer hospital, Hyderabad, Andhra Pradesh.

INTRODUCTION: Ureteropelvic junction obstruction (UPJO) is the most common cause of obstructive nephropathy; accounting for about 1:1,500 new-borns. The widespread use of obstetric ultrasonography has resulted in early and more frequent observations of congenital hydronephrosis, improving its post-natal management. UPJO may be considered, in most cases, as a functional obstructive condition due to maturative abnormalities, during the gestation, in the smooth muscle and/or the innervation of the pyelo-ureteral transitional segment, defective peristaltic waves and resulting in aperistaltic segment and difficulty to eject urine from the renal pelvis into the ureter. Smooth muscle discontinuity or disproportionate presence of longitudinal smooth muscle fibers and excessive collagen deposition are sometimes associated with an inappropriate innervation. This results in a defective propagation of the electrical activity produced by a pacemaker located in the pyelocaliceal region. The present study aims at correlating the ureteropelvic junction abnormality with clinico-radiological and histopathological findings.

METHODS: A retrospective and prospective study of 7 years from May 2006-April 2013 with review of clinical presentation, Ultrasound findings, renal isotope scan findings and microscopy of UPJO segments of 52 patients under 14 yrs, who underwent reconstruction at our hospital, a tertiary care pediatric center were studied. Extrinsic causes of UPJO were ruled out by ultrasonography and operative findings were noted .Only patients with UPJO due to intrinsic causes confirmed on histopathology were studied. Special stains--PAS and Masson Trichrom were done to highlight the histological alterations.

RESULTS: Our study reviewed 52 cases, of which most cases belonged to the age group of 0-2 years with 34(65.38%) cases and only 1(1.92%) case after 10 years of age. (Table 1).Sex incidence showed male preponderance- 43(82.69%) male children and 9(17.30%) female children. (Table 1). Left sided UPJOs were more common-34(65.38%), right sided PUJOs were 16(30.76%) and there were 2 cases with bilateral PUJO.

All antenatally diagnosed cases had ipsilateral severe renal dysfunction and contralateral hydronephrosis. Older children presented with severe urosepsis, oliguria, hematuria and failure to thrive with ipsilateral severe renal dysfunction and contralateral moderate renal dysfunction. (Table 2).

The most common clinical presentation was pain, fever, distension of abdomen and mass per abdomen in 34 cases. (5) cases were diagnosed antenatally. (14) cases presented with only pain. (4) cases presented with severe urinary complications like urosepsis, oliguria, hematuria and failure to thrive. (Table 2)

At the time of presentation the renal isotope scan showed ipsilateral moderate dysfunction in most of the cases. (Table 4) Intraoperatively kidneys were enlarged with narrowed UPJO in all 52/52 cases. Extrinsic causes were ruled out.

Anderson--Hynes pyeloplasty was done in 49/52 cases and 3/52 underwent nephrectomy. 49 UPJO segments and 3 nephrectomy specimens were processed. Histopathology of 36 cases showed transitional epithelial lining, 16 cases showed metaplastic changes (Fig 1, 2). All cases showed hypertrophied muscle bundles, increased fibrocollagenous tissue and chronic inflammatory infiltrate (Fig 3, 4, 5, 6). Nephrectomy specimens showed-thinned out renal parenchyma, marked glomerulosclerosis, interstitial nephritis, fibrosis, dilated tubules and dense chronic inflammatory infiltrate--end stage kidney (7, 8, 9). Special stains Massons Trichrome demonstrated extensive fibrocollagenous tissue in all UPJO segments. PAS and Massons Trichrome showed glomerulosclerosis and interstitial fibrosis in the nephrectomy specimens studied.

DISCUSSION: UPJO is a congenital condition occurring at all ages in children with an autosomal inheritance (1, 2). Congenital UPJO is mostly due to intrinsic causes, in most cases, due to inadequate canalization of this area or as a functional obstruction (3, 4). Other extrinsic causes for UPJO are renal /ureteral stones, aberrant lower pole renal vessels, bands, kinks, tumors (3). UPJO is the most common cause of hydronephrosis in children (5, 6).

Antenatal sonography has increased the chances of UPJO detection in 1500 fetuses showed UPJO during antenatal screening (7). There was an increased incidence in male children and more so on the left side (7). Our study also showed similar findings with 82.69% males with 65.38% cases being left sided (8). The most common clinical presentation was a symptom triad of pain, fever and abdominal mass (4) which was a common presentation in our study also--46.15%. Other presentations include severe urinary tract complications, and failure to thrive. In our study all antenatally diagnosed children had unresolving hydronephrosis with severe renal dysfunction in ipsilateral and hydronephrosis with normal renal function in contralateral kidney. We had two cases above 9 years with bilateral renal dysfunction. This correlates well with the study by Gonzalez R, et al. (4)

There have been many studies encompassing the entity of UPJO obstruction that include clinical findings, radiographic imaging, pathological examination of ureteropelvic junction obstruction per se and renal biopsies during pyeloplasty procedures (9). Symptomatic children usually require operative intervention. (6) In these patients, pyeloplasty is performed and consists of resecting the atretic or stenotic segment and reattaching the normal ureter to the renal pelvis, there by relieving the obstruction. Renal biopsies in patients in whom pyeloplasty is done show mostly relatively well maintained parenchyma, with overt changes in glomeruli (10). More subtle alterations have been described that relate to shifts in proximal-to-distal tubular ratios. Extreme thinning of the renal parenchyma can occur with only limited tubulointerstitial injury (9). In our study microscopy of UPJO in 52/52 cases showed fibrosis and excessive collagen fibres around the hypertrophied muscle bundles.

Histological alterations in kidney with UPJO were marked glomerulosclerosis, dilated tubules and dense chronic inflammatory infiltrate--end stage kidney similar to features noticed in a study by Elder J S Stansbrey et al (11). In UPJO segments extensive fibrosis, hypertrophied muscle bundles and chronic inflammatory infiltrate, metaplastic changes were noted. S k ozel (12) in his study of 22 UPJO cases (1 month-10 years) showed similar histological features and demonstrated high expression of fibronectin, laminin, type 4 collagen and BCL-2 thus showing their relationship to the pathogenesis of UPJO. Defective kidney morphogenesis, during branching and tubulogenesis of ureteric bud, may also be responsible for this congenital pathology (8).

Open pyeloplasty is still considered the gold standard for UPJ obstruction in children (removal of stenotic segment and re-anastomosing the ureter to renal pelvis). Other novel surgical options includes endopyelotomy, endo pyeloplasty, laparoscopic pyeloplasty, robotic-assisted laparoscopic pyeloplasty. Studies by Gonzalez R, Schimke CM recommend early surgical treatment to prevent obstructive damage to the immature infant kidney and because better recovery of function is possible when surgery is done in the first year of life. Similar findings were noticed in our study also. 4/52 cases which presented later in life showed features like failure to thrive, urinary complications and deterioration of renal function in both the kidneys and finally lead to nephrectomy (13). In our study we found that when patients presented late in life there was marked deterioration of renal function and finally lead to nephrectomy. Histology also showed features of end stage kidney.

CONCLUSION: UPJO in pediatric age group is most common cause of hydronephrosis, with an incidence of 1 in 1500 cases. There is a marked male preponderance with most cases being left sided. Histological study shows that it is mainly due to intrinsic abnormalities--fibrosis, excessive collagen fibres around the hypertrophied muscle bundles. Severity depends on extent of obstruction, so early correction by pyeloplasty prevents deterioration of kidney function leading to nephrectomy. Study of nephrectomy specimens reveals features of end stage kidney. Our study also suggests early intervention and management is mandatory in cases of UPJO.

REFERENCES:

(1.) Brown T, Mandell J, Lebowitz RL. Neonatal hydronephrosis in the era of sonography. AJR Am J Roentgenol. May 1987; 148(5):959-63.

(2.) A.H. Colodny et al. Ureteropelvic junction obstruction in children, Urol Clin North Am 1980; (7): 273-290.

(3.) Peter Kench. A Morphometric Study of the Pelvi-Ureteric Junction and Review of the Pathogenesis of Upper Ureteric, Pathology, 1982, Vol. 14, No. 3, 309-312.

(4.) Gonzalez, R, Schimke, CM. Ureteropelvic junction obstruction in infants and children. Pediatr Clin North Am 2001; 48:1505.

(5.) D. Innes Williams, CM Karlaftis, Hydronephrosis due to pelvi-ureteric obstruction in the new born. British Journal of Urology April 1966 Volume 38, Issue 2, 138-144.

(6.) Josephson, S. Antenatally detected pelvi-ureteric junction obstruction: concerns about conservative management. BJU Int 2000; 85:973.

(7.) M.K. Hanna, R.D. Jeffs, J.M. Sturgess et al. Ureteral structure and ultrastructure. Part II. Congenital ureteropelvic junction obstruction and primary obstructive megaureter. J Urol 1976; 116: 725-730.

(8.) Zhang, PL, Peters, CA, Rosen, S. Ureteropelvic junction obstruction: morphological and clinical studies. Pediatr. Nephrol. 2000. 14:820-826.

(9.) S Rosen, CA Peters, The Kidney in Congenital Ureteropelvic Junction Obstruction: A Spectrum From Normal to Nephrectomy. The Journal of Urology April 2008, 179, 125-126

(10.) Chertin B, Pollack A, Koulikov D, et al. Does renal function remain stable after puberty in children with prenatal hydronephrosis and improved renal function after pyeloplasty? J Urol 2009; 182:1845.

(11.) Elder JS, Stansbrey R, Dahms BB, Selzman AA Renal histological changes secondary to ureteropelvic junction obstruction The journal of urology 1995 Aug; 154(2):719-722.

(12.) S-K. Ozela, H. Emirb, S. Dervisogl. The roles of extracellular matrix proteins, apoptosis and c-kit positive cells in the pathogenesis of ureteropelvic junction obstruction Journal of Pediatric Urology, April 2010, 6, 125-129.

(13.) Gonzalez R, Schimke CM The prenatal diagnosis of hydronephrosis, when and why to operate? Arch Esp Urol. 1998 Jul-Aug; 51(6):575-579.

Manimekhala [1], M. Lavanya [2], M. Ramani [3], K. Ramesh Reddy [4], Srinivas Reddy [5], Sara [6]

AUTHORS:

[1.] Manimekhala

[2.] M. Lavanya

[3.] M. Ramani

[4.] K. Ramesh Reddy

[5.] Srinivas Reddy

[6.] Sara

PARTICULARS OF CONTRIBUTORS:

[1.] Associate Professor, Department of Pathology, Niloufer Hospital, Hyderabad.

[2.] Assistant Professor, Department of Pathology, Niloufer Hospital, Hyderabad.

[3.] Professor, Department of Pathology, Niloufer Hospital, Hyderabad.

[4.] Professor and H.O.D, Department of Paediatric Surgery, Niloufer Hospital, Hyderabad.

[5.] Assistant Professor, Department of Paediatric Surgery, Niloufer Hospital, Hyderabad.

[6.] Post Graduate, Department of Pathology, Niloufer Hospital, Hyderabad.

NAME ADRRESS EMAIL ID OF THE CORRESPONDING AUTHOR:

Dr. Manimekhala, Associate Professor of Pathology, Flat No.--204, A Block, Vishnu Residency, Gandhinagar, Hyderabad. Email--manimekhalarao@gmail.com

Date of Submission: 22/08/2013.

Date of Peer Review: 31/08/2013.

Date of Acceptance: 02/09/2013.

Date of Publishing: 13/09/2013

Table 1. Age and sex incidence of PUJO

           0-2 yr       2-5 yr     5-10 yr    10-14yrs

Male     28(53.84%)   12(23.07%)   2(3.84%)   1(1.92%)
Female   6(11.53%)     2(3.84%)    1(1.92%)      0

Table 2: Clinico-radiological findings of 52 cases of PUJO

No. of cases,        Clinical         USG abdomen
age-group          presentation

34 (0-2yrs)        Pain, fever,          Gross
                dis-tension, mass    hydronephrosis
                     abdomen

14 (2-5yrs)         Only pain           Moderate
                                     hydronephrosis

4 (9-12yrs)       Severe urinary         Gross
                  complications-     hydronephrosis,
                    urosepsis,        MCUG-normal
                    oliguria.
                hematuria, failure
                    to thrive

5 (3-9 months)     Antenatally        Unresolving
                 diagnosed--upjo     hydronephrosis,
                                         1 case
                                      multicystic
                                      kidney with
                                          upjo
                                     hydronephrosis

No. of cases,   Renal isotope scan   Contralateral
age-group                                kidney

34 (0-2yrs)       partial Upjo--         Normal
                  Moderate renal
                   dysfunction

14 (2-5yrs)      Upjo with normal        Normal
                  renal function

4 (9-12yrs)     Significant upjo--   hydronephrosis
                   severe renal      with Moderate
                   dysfunction           renal
                                      dysfunction

5 (3-9 months)   Upjo with severe    hydronephrosis
                renal dysfunction     with normal
                                     renal function

Table 3: Clinical presentation of PUJ obstruction

Clinical presentation            Number (%)

Fever                            24 (46.15%)
Distension and mass abdomen      20 (38.46%)
Abdominal pain                   24 (46.15%)
Failure to thrive                04 (7.69%)
Hematuria, oliguria, urosepsis   04 (7.69%)
convulsions                      04 (7.69%)

Table 4: Renal isotope scan findings in 52 cases in UPJO

No. of cases         Kidney with UPJO         Contralateral Kidney

32              Moderate renal dysfunction           Normal
10               Severe renal dysfunction        Hydronephrosis
6                 Normal renal function              Normal
4              Non-functioning kidney-UPJO    Moderate dysfunction
                   with multiple cysts
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Title Annotation:ORIGINAL ARTICLE
Author:Manimekhala; Lavanya, M.; Ramani, M.; Reddy, K. Ramesh; Reddy, Srinivas; Sara
Publication:Journal of Evolution of Medical and Dental Sciences
Article Type:Report
Geographic Code:9INDI
Date:Sep 16, 2013
Words:1930
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