Wednesday, February 9, 2022

Iris Publishers-Open access Journal of Urology & Nephrology | Congenital Pelvi-ureteric Junction Obstruction Associated with Crossing Lower Pole Vessels

 


authored by Abubakar Sadiq Muhammad1,2*

Abstract

Background: Congenital pelvi-ureteric junction obstruction [PUJO] occurs when there is impairment of flow of urine from the renal pelvis to the proximal ureter due to inherited abnormalities of pelvi-ureteric junction [PUJ]. The presentation is usually at the 4th or 5th decade of life. It may rarely be associated with crossing lower pole vessel [CLPV]. Treatment is by Anderson- Hynes [dismembered] pyeloplasty which remove the abnormal PUJ and transposes the PUJ anterior to the offending vessels. I report a case of 45-year-old man with congenital PUJO associated with crossing lower pole vessel vessels who had dismembered Anderson -Hynes pyeloplasty.

Case report: This is a 45-year-old man who presented with 4 years history of recurrent colicky left flank pain which was relieved by passing large volume of urine. The general examination was non remarkable. The left kidney was ballotable. The abdominopelvic computerized urogram revealed severe left hydronephrosis and hyperdense structure in the region of the left kidney. He had exploration via left Gibson incision with finding of multiple crossing lower pole vessels and aperistaltic segment at PUJ. He had excision of the abnormal PUJ, transposition of the crossing vessels posteriorly and dismembered Anderson-Hynes pyeloplasty. He had uneventful recovery and was discharged home 5 days postoperatively.

Conclusion: Congenital PUJO associated with lower pole vessel is rare and may present in the 5th decade of life. Anderson- Hynes dismembered pyeloplasty is the procedure of choice and associated with good outcome.

Keywords: Congenital; Pelvi-ureteric junction obstruction; Crossing vessels; Dismembered pyeloplasty; Anderson hynes; Hydronephrosis

Abbreviations: PUJO: Pelvi-ureteric junction obstruction; PUJ: Pelvi-ureteric junction; CLPV: Crossing lower pole vessel

Introduction

Congenital pelvi-ureteric junction obstruction [PUJO] occurs when flow of urine is impeded from renal pelvis to the proximal ureter due to inherited abnormalities at pelvi-ureteric junction [PUJ] [1]. It may also be acquired from stone disease, inflammatory/ infective conditions and urothelial carcinoma of the renal pelvis [2]. The inherited causes may be intrinsic or extrinsic [2]. The intrinsic causes include aperistaltic segment due abnormal musculature, valves and folds. The extrinsic causes include crossing lower pole vessel, adhesions and bands [2]. The crossing lower pole vessel is the cause of obstruction in 11-20% of cases [3]. This is referred to currently to as vascular bar [4]. It may also co-exist with other causes. Even though a congenital problem, the presentation may occur at the 4th or 5th decade of life. The routine diagnostic investigations of intravenous or computerized tomographic scan urogram can only diagnose the PUJO with accuracy. The lucency of the crossing vessels or short segment sign [5] may suggest the presence of a crossing vessels in 20-60% of the cases [5]. The crossing vessel can be accurately detected on Helical CT angiogram or Magnetic Resonance Angiogram [MRA] [6,7]. Doppler ultrasound can also assist in making the diagnosis when angiography is not feasible [8]. The gold standard treatment is by Anderson- Hynes dismembered pyeloplasty which remove the abnormal PUJ and transposes the PUJ anterior to the offending vessels. This can be done laparoscopically, robotically or by open method [1, 9-11]. Alternatively, cranial relocation of the crossing polar vessel can be done by open, laparoscopic or robotic technique. This was first described by Hellstrom [12] and later modified by Chapman [13,14]. In the original procedure the polar vessels were anchored to the anterior pelvic wall using vascular adventitial sutures while in the modified technique the vessels were wrapped in a more superior position in the anterior pelvic wall without need of vascular adventitial sutures. No case of PUJO due to crossing lower pole vessel reported in our environment. I report a case of 45-yearold man with congenital PUJO associated with crossing lower pole vessels who had dismembered pyeloplasty.

Case Report

This is a 45-year-old man who presented with 4 years recurrent colicky left flank pain which is relieved by passing large volume of urine. The general examination was not remarkable. The left kidney was ballotable. The abdominopelvic ultrasound and computerized urogram revealed severe left hydronephrosis and a renal stone. Full blood count, urine microscopy, urea and creatinine were normal. Clotting profile, chest x ray and electrocardiogram were normal. Plain abdominal x ray revealed radiopacity in the in the region of the right kidney (Figure 1). The diagnosis of left hydronephrosis left renal pelvic stone done. He had exploration via left Gibson incision with findings of Moderate Hydronephrosis (Figure 2), Grade II multiple crossing lower pole vessels (Figure 3) at the level of an aperistaltic PUJ which did not drain the kidney even after the transposition of the vessels posteriorly. There was no stone found. He had excision of the aperistaltic PUJ with adjoining proximal ureter and redundant pelvis. Anderson-Hynes dismembered pyeloplasty was done with the neo PUJ anterior (Figure 4) to the crossing lower pole vessels. Double J stent was inserted and a retroperitoneal drain. He was placed on the intravenous Ceftriaxone 1 g daily for 48hours, pentazocine 60 mg for 24 hours and intravenous fluid Normal saline 1L hourly for 48 hours. He was started on oral feed 2 days post operatively and the retroperitoneal drain was removed 3 days post operatively. He had uneventful recovery and was discharged home 5 days postoperatively. Stitches were removed 10 days post operatively. He was asked to come for double J stent removal 2 weeks post operatively, but he absconded with the stent in situ (Figures 1-4).

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