Ureter Quadruplication with Huge Ureteral Cyst

Ureteral quadruplication is an extremely rare congenital anomaly. It was first reported in 1975. The patients may present with different features and complications in respect to this congenital anomaly. They may also present with other congenital anomalies. Diagnosis sometimes is difficult and may need to be investigated with Magnetic Resonance Urogram, CT scan apart from routine investigations like ultrasonography and intravenous urogram. Surgical treatment should be based on the operative and investigational finding. We present the twelfth reported case but the first from Nepal. It was associated with a large ureteral cyst with diagnostic dilemma. Surgical exploration was done with a provisional diagnosis of large retroperitoneal cyst.


Introduction
U reter quadruplica on is the rarest congenital anomaly among ureter malforma ons 1,2,3,4 . First reported case was by Sodedahl DW et al in 1976 2 . Since then 11 such cases have been reported worldwide. Our paediatric case is the fi rst to be reported from Nepal and twel h in the world. This malforma on may be associated with vesico-ureteral refl ux (VUR) 5 , dystrophic kidney 6 , or blind ending proximal branches 7 . These pa ents may also present with contra lateral malforma on like contra lateral mul cys c dyspas c kidney 8 , triplet ureter, agenesis of the kidney or even bilateral ureteral quadruplica on 2 . Recently in 2014 quintoplica on of ureter has been reported by Jurkiewicz et al which is fi rst of its kind 9 . The present case is of a girl of 5 years old who presented with distended painful abdomen. A er relevant inves ga ons we were unable to come to a defi nite diagnosis for which surgical explora on was carried out.

The Case
A fi ve year old girl child presented with abdominal distension and episodic pain in her right side of the abdomen. The intensity of pain had increased for past 20 days. She had no history of urinary or bowel problems in the past. On physical examina on, the pa ent had distended painful abdomen on the right side. She had no fever, jaundice, or pallor. Haematological and biochemical studies were all within normal limits. Her urine showed plenty pus cells with 75-80 rbc /hpf and E. coli on urine culture. Ultrasonography reported as thick walled retroperitoneal cyst with right mild hydronephrosis and bilateral malrotated kidneys. On Intravenous urogram (IVU), it showed right mild hydronephrosis with bilateral malrotated kidneys ( Figure 1).With no obvious conclusion CT-scan was done and revealed thick walled retroperitoneal cyst abu ng the right kidney with internal contrast in delayed image and duplex right kidney with obstructed hydronephro c lower moiety ? Urinoma ( Figure 2). She was admi ed and intravenous an bio c was started as per her culture and sensi vity of urine culture.
A er counselling about the diffi culty in diagnosis, pa ent's parents agreed on surgical explora on and also for possible nephrectomy. On explora on, we found a large retroperitoneal cyst which on further dissec on kidney was found a ached to it with four branches (Figure 3). Not recognizing what they really were, we cut two branches thinking it as adhesion. On due course of dissec on, we no ced a long tube distally connected to the cyst. We were unable to understand the anomaly, we requested our one of the resident to web-search immediately. We later concluded this must be a case of quadruplica on of ureter with large ureteral cyst and a single ureter ( Figure 4). Kidney looked grossly normal as IVU and CT-scan suggested, func oning kidney though DTPA scan was not done. The iatrogenic injury of two upper moiety were anastomosed by Wallace fashion and then to the cyst. The large ureteral cyst underwent reduc on pyeloplasty with the single ureter distally as there was a par al stenosis of about 1 cm at the junc on of cyst and ureter. DJ stent of 5 French size was placed to safeguard the anastomosis. The girl was hospitalized for 7 days with removal of drain on the 6 th day post-opera vely. On follow up a er 6 weeks she was free of any post-opera ve complica on and DJ stent was removed. Her parents have been advised to a end the follow up clinic on 3 rd and 6 th month. She will undergo IVU and possibly DTPA scan on the 6 th month.

Discussion
During the early fourth week of gesta on ureteric bud arises as a diver culum from the Wolffi an duct. Once in contact with metanephric mesenchyme it undergoes a dichotomous branching morphogenesis 10 . It has been postulated that AT2 (angiotensin II receptor)

Quadruplication Quadruplication
Ureteral cyst Ureteral cyst kidney kidney plays a vital role in direc ng the site of ureteric bud outgrowth. The defect in this process leads to defec ve ming and loca on of outgrowth of ureteric bud leading to congenital ureteral anomalies 10 .
Quadruplica on of the ureter is a very rare type of congenital anomaly. The fi rst reported case was by Soderdahl in 1976 and only 11 such cases has been reported worldwide ll date 2 . Our case is unique as it presented with huge distended abdomen due huge dilata on of ureteral cyst caused by stenosis of "ureterocysto-pelvic junc on" with normal four congenital ureter. Triplica on of ureter as described in literature has four varie es according to Smith classifi ca on but no such variety is described for the quadruplica on of ureter. Quadruplica on is associated with many other anomalies like three blind-ending braches, mul cys c dysplas c kidney, bilaterality and massive vesicoureteral refl ux in the contra-lateral side.
Diagnosis is usually made during surgical explora on as in our case. Preopera ve evalua on with IVU, retrograde pyelography may show congenital abnormali es. CT-scan and VCCUG may be needed for further evalua on to look for other ipsi /contralateral anomalies and VUR. DMSA or DTPA scan will indicate the func onal status. Magne c Resonance urography (MRU), if available, may be more diagnos c and may provide high quality images of the urinary tract without using ionizing radia on. MRU can provide both excretory func onal and morphological informa on by using diure c and contrast media.
There are no general consensus on the surgical procedure .It may diff er due to varied presenta on of the anomaly. Our case underwent Wallace anastomosis for the iatrogenic injury of the upper two moiety and reduc on pyeloplasty for "ureterocystopelvic stenosis". The kidney looked healthy though DTPA scan was not done preopera vely. Massive VUR with impaired renal func on can undergo nephreureterectomy 5 . Akaba et al 1 preserved the kidney by termino-terminal pyelorenal anastomosis. To preserve the affl icted kidney is jus fi able and defendable as in our case but the pa ent needs to be in regular follow up. Our pa ent is s ll under rou ne regular medical follow up. At sixth month the pa ent will undergo test like IVU and DTPA to look for postopera ve complica ons like re-stenosis and func onal condi on.

Conclusion
Quadruplica on of ureter like our case is an extremely rare ureter congenital anomaly. Very few cases are reported worldwide and there is no consensus regarding its treatment op ons