Blind-ending complete ureteric duplication with reflux and ectopia
We report a rare case of complete blind-ending refluxing ectopic ureteric duplication.
Authors: Dr. Abdelmoniem Hassan Koko FRCS(Ed), Dr. Khaled Ezzeddin MD,PhD, Dr.Hussien Al Ghahtani FACHARTS, Dr. Khaled Al Gammal MD
Armed Forces Hospital, King Abduaziz Air Base ,Dhahran
P.O.BOX 4192, Al Khobar, 31952
Corresponding Author: Dr. Abdelmoniem Hassan Koko FRCS(Ed), Armed Forces Hospital, King Abduaziz Air Base, Dhahran P.O.BOX 4192, Al Khobar, 31952 Saudi Arabia. E-mail: [email protected]
Blind-ending ureteric duplication is a rare developmental anomaly of the ureter. Embryologically, it was postulated that the affected ureteric bud is abortive and fails to make contact with the mesonephros. Histologically, it contains all the ureteric layers; it tends to have bulbous dilatation (1). Sometimes they are short without reflux and are minimally symptomatic. However, patients with long blind-ending duplications can present with severe infection and reflux, requiring extensive surgery (2). Here we report a rare case of complete blind-ending refluxing ectopic ureteric duplication.
A 20 year old female was admitted to the hospital with right sided abdominal pain, lower urinary tract symptoms and fever. She gave a history of recurrent febrile urinary tract infections since childhood, with multiple hospital admissions. On examination she was febrile, and there was tenderness and guarding on the right side of the abdomen. Urine culture grew E coli and renal profile was normal. Ultrasound showed mild dilatation of the right pelvicalyceal system, with a cystic area above and medial to the upper pole of the right kidney, and a cystic area on the right side of the pelvis, representing the dilated right ureter (Fig 1).
Fig 1. Ultrasound showed mild clayceal dilatation and cystic mass at the medial aspect of the upper pole of the right kidney.
Intravenous pyelography showed right sided calyectasia; the renal pelvis was not dilated and there was lateral displacement of the right kidney. The left kidney and urinary bladder were normal (Fig 2).
Fig 2. IVU showing lateral displacement of the right kidney, calyceal dilatation, renal pelvis and ureter not dilated.
After controlling the infection, she was scheduled for micturating cystourethrogram (MCUG). She developed severe intolerable pain when the Foley’s catheter was inserted. The radiologist found the catheter in the right ureter so it was removed immediately and the procedure was aborted. Indirect radioisotope MCUG demonstrated right sided vesicoureteric reflux (Fig 3).
Fig 3. Indirect micturating cystourethrogram with radioactive tracer in the dilated distal right ureter indicating vesicoureteric reflux.
She was scheduled for cystography and cystoscopy under anaesthesia. The Foley’s catheter was found again in the right ureter, which was dilated, tortuous and blind-ending (Fig 4).
Fig 4A. Preferential passage of the urethral catheter into the right ureter.
Fig 4B. Retrograde study showing blind ending tortuous dilated ureter as well as the collecting system on the right side.
Fig 4C, D. CT scan with contrast material through ureteric catheter showing the blind ending duplication, and it’s relation to the right kidney.
Urethroscopy showed the opening of the ectopic right ureter just below the bladder neck (Fig 5).
Fig 5. Urethroscopy with guide wire in the ectopic ureteric orifice in the urethra.
Surgical exploration and excision of the blind ending right ureter was done (Figs 6A, B, C).
Fig 6. Duplication of the ureters on the right side.
Fig 6B. Upper pole right kidney after complete excision of the blind ending ureter.
Fig 6C. Surgical specimen.
The post operative recovery and subsequent follow up for four years was uneventful.
Blind ending ureteric duplication is a rare anomaly of the ureter. Most of the cases reported were involving one limb of a bifid system; it is more common in females than in males, and present twice as often on the right side. The majority of the identified cases are diagnosed in the third or fourth decade of life. Embryologically, it was postulated that the affected ureteric bud is abortive and fails to make contact with the mesonephros. Histologically, it contains all the ureteral layers; it shares a common sheath and blood supply with the normal ureter (1). Our patient had recurrent severe febrile urinary tract infections, which led to frequent hospitalizations.
The only reported similar case found in the English literature described ureteric ectopia with preferential passage of the Foley’s catheter into an ectopic blind- ending ureter in the prostatic urethra, in a 67 year old man (3).
Surgical dissection of the lower segment of the blind ending ureter may compromise the vascularisation of the remaining ureter on that side. This can be prevented by conserving the common ureteric sheath or re-implanting the normal ureter. Since the ensheathment is less dense at the proximal end, the dissection should start there and care is taken not to denude or injure the normal ureter (1, 4, and 5). Careful and systematic steps must be adhered to in diagnosis and management of children presenting with febrile urinary tract infections. If the correct diagnosis was made initially, our patient could have been saved from unnecessary morbidity.
Blind-ending complete ureteral duplication is a rare developmental anomaly of the ureter. Careful and systematic steps must be followed in diagnosis and management of children presenting with febrile urinary tract infections.
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Date added to bjui.org: 27/11/2011