The incidence of UPJO in the pediatric age group is well defined, affecting around 60 % of all newborns with hydronephrosis. The exact incidence of primary congenital UPJO in adults is unknown, although estimated as “not uncommon”[20].

In contrast to prenatal screening, adult UPJO may be discovered in several ways: (a) evaluation following symptoms such as chronic back pain, acute renal colic, especially after fluid overload, hematuria, UTI and pyelonephritis; and (b) incidentally during diagnostic imaging of the abdomen or spinal area for other problems such as abdominal or back pain.

Diagnosis of adult UPJO does not differ from that of the pediatric age group, using nuclear medicine static and dynamic renal scans, CT urography or MR urography, and intravenous pyelography. Unfortunately, there is no consensus regarding which is the optimal imaging modality to determine obstruction. Moreover, there are no uniform standards for how to perform the above-mentioned diagnostic tests, and various protocols exist.

Management decisions in the adult are different. In children, the main goal is to eliminate the obstruction in order to restore maximal renal function and to allow maximal growth of the kidney. In adults, the ability to restore renal function or to allow neo-compensatory renal growth following surgery is limited, as chronic obstruction may have caused irreversible changes in the renal parenchyma and vasculature, and the maximal growth potential has already been achieved. Factors that should guide the physician regarding the management of adult UPJO are: side effects such as recurrent infections and stone formation, symptoms, function, age and co-morbidities.

Symptomatic patients with UPJO should be treated when the UPJO endanger the patient: recurrent pyelonephritis or hypertension can be life threatening, especially in elderly patients with other co-morbidities. Non-life-threatening symptoms such as chronic pain that interfere with a patient’s quality of life may also be an indication for definitive treatment.

Definitive treatment includes reconstructive surgery to eliminate the obstruction or nephrectomy. In order to choose the most suitable treatment for the patient, several factors should be taken into consideration: the function of the obstructed kidney, function of the other kidney, patient’s age and co-morbidities.

A non-functioning symptomatic kidney in the presence of a normal functioning contralateral kidney should be removed. In patients with a single kidney or those with overall poor renal function, reconstructive surgery should be offered.

Nephrectomy in adults is a well-established operation. The decision between open and laparoscopic/robotic routes should be made by the patient and the urologist based on individual circumstances. Both can achieve the surgical goal successfully, with the advantage of a shorter recovery period with less analgesic consumption in patients who undergo laparoscopic nephrectomy [46]. Surgeon should bear in mind that post infection nephrectomy could be more complex due to tissue reaction and adhesions [25].

Reconstructive surgery in adults can be performed by a wider range of surgical techniques than in children. The open surgical approach – open pyelopasty – is still considered the gold standard in adults, with a 91 % success rate overall and 100 % success rate for grade 1–3/4 hydronephrosis [19]. Laparoscopic and laparoscopic robotic pyeloplasty already show similar success rates to open surgery. Bauer et al. compared 42 laparoscopic pyeloplasties to 35 patients who underwent open surgery, and the overall success rates were comparable (98 % and 94 %, respectively) [5]. Robot-assisted laparoscopic pyelopasty also showed comparable success rates with minimal complications and short recovery time [31]. In contrast to the pediatric age group, adult patient may choose to undergo endoscopic correction of the UPJO. Endoscopic endopyelotomy is a minimally invasive procedure that may be performed as ambulatory or day-care surgery, with short recovery time and rapid return to normal activity. The ability to perform the procedure under regional anesthesia, endoscopic endopyelotomy is more suitable to patients with co-morbidities. It was originally described by Wickham, using an endoscopic urethrotome via an antegrade approach, performed a full-thickness incision of the upper ureter and UPJ. Among the three initially treated patients, two achieved improved drainage [51]. Danuser et al. assessed the results of 212 consecutive antegrade endopyelotomies over a period of 8 years and showed overall 85 % success [13]. With the introduction of smaller endoscopes with better visual imaging and the use of laser energy antegrade and later on retrograde, endopyelotomies became even easier to perform. Today, endopyelotomy for UPJO in adults is considered as first-line treatment in various urological centers. Recent reviews with longer-term follow up show that success rates are worse than previously reported. Dimarco et al. assessed the long-term results of antegrade endopyelotomy (182 patients) and open pyeloplasty (175 patients) [14]. The estimated 3-, 5-, and 10-year recurrence-free survival rates for the endopyelotomy group were only 63 %, 55 % and 41 %, respectively, compared to 85 %, 80 % and 75 % for the pyeloplasty group (p < 0.001). Additional crucial information arising from this study is the fact that failures continue to appear after 5 and 10 years, and patients should be followed accordingly. Rassweiler et al. showed similar results comparing the success rates of laser endopyelotomy (113 patients) and laparoscopic pyelopasty (143 patients). The laparoscopic procedure showed a superior overall success rate of 94.4 % versus 72.6 % [37].

Cryptorchidism is noted in 1–4 % of full term newborns and up to 45 % in preterm babies. The exact incidence of UDT in adulthood is not defined in the literature.

Acute pain due to torsion, or pain following blunt trauma, or pain related to abdominal content herniation may lead to the discovery of UDT [33].

As in the pediatric age group, physical examination is the key tool for the diagnosis of UDT. In contrast to children, older man may have different body habitus, hence physical examination may be less informative mandating inguinal sonography. There are no guidelines what is the proper diagnostic algorithm for non-palpable testis in adults.

In the pediatric age group, orchidopexy at an early age is guided by the need to preserve functions, and to prevent complications (torsion, susceptibility to blunt trauma and hernia). Orchidopexy may facilitate earlier detection of cancer. Regarding adult UDT, unfortunately, spermatogenesis decays with time and after the age of 2 the rate of germ-cell aplasia irreversibly accelerates. Fertility potential is better if the UDT is unilateral and if surgery was performed at earliest age possible [45]. Rogers et al. analyzed the histology of 52 resected UDTs. Patients’ mean age at surgery was 26 years, and among the 52 specimens only one (1.9 %) testis showed normal spermatogenesis. The remaining testicles showed: Sertoli cells only, in 30 (58 %) patients, maturation arrest in 15 (28.5 %), and testicular agenesis in six (11.5 %). The authors concluded that the majority of UDTs cannot contribute to fertility [38]. Grasso et al. found similar results by performing testicular biopsies in 22 patients who had post-pubertal orchidopexy for cryptorchidism. More than 83 % of the biopsies showed azoospermia and severe oligospermia [21]. Although Leydig cells are less vulnerable to damage, endocrine function is also impaired in the adult UDT [22].

From fertility point of view, adult orchidopexy will not improve spermatogenesis. Concerning malignancy, it has already been shown that orchidopexy does not prevent or change the rate of testicular cancer [34]. Moreover, high percentage of UDT related cancer are at a high stage at diagnosis [39]. Orchidopexy may only improve the ability to palpate the testicle. For these reasons a unilateral orchiectomy in a presence of normal contralateral testicle is a valid option. If orchidopexy is chosen, lifelong follow up should be established.

Due to its abnormal location, UDT may cause discomfort and undergo torsion. Zilberman et al. showed that the rate of salvaging UDT torsion is lower than for normally positioned testes. Among 11 patients with torsion of UDT only two testicles (18 %) remained viable; five had massive necrosis during surgery and had to be resected and four vanished post orchidopexy [48].

The majority of adult UDTs have very low fertility potential, impairment of endocrine function and increased risk of testicular cancer. In patients with a normal contralateral testicle, UDT orchiectomy should be offered. In patients with a single testis or bilateral UDT, preservative management may be considered mandating awareness of the possible complications and close follow up with monthly self-examination and periodical physical examination [9].

In the pediatric age group ureterocele may be related to a wide variety of complex anomalies, such as duplex kidney, ectopic ureter, bladder outlet obstruction, incontinence and reflux. In adults most of the ureteroceles are related to a single system. They are intravesically located and the degree of obstruction is less severe, however exception exist.

The exact incidence is not reported in the literature. Most of the cases are reported in women (4:1 female to male ratio), however most cases of stone formation in ureteroceles were reported in males. Stone formation in ureterocele was reported up to 40 % [40].

Presentation in an adult could be either by flank/back pain and recurrent UTI, or asymptomatic hydronephrosis detected incidentally. Stasis at the ureteral meatus due to the partial obstruction by the ureterocele, may induce stone formation inside the ureterocele, causing renal colic and/or UTI. A single case report in the English medical literature described a patient who deteriorated to renal failure due to bilateral ureterocele [47]. Rarely, prolapsed ureterocele may cause acute urinary retention in adults [49].

Ultrasound will demonstrate a cystic mass within the bladder. Doppler mode can demonstrate a urine jet at the meatus of the ureterocele with volume changes during urine expulsion from the meatus. Evaluation of renal parenchyma, degree of hydronephrosis and jet sign combined with the advantage of no radiation energy exposure, ultrasound may be a sufficient tool to diagnose and follow up adult patients with ureterocele [28]. In cases where the anatomy is unclear, CT or MRI urography may give an accurate delineation of the anatomy, renal function, excretion delay and the degree of hydronephrosis. Renal function could also be assessed using nuclear medicine studies.

Management should be based on symptoms and renal function. Symptomatic patients should be treated. In the case of no or poorly functioning kidney with normal contralateral kidney and normal GFR, nephrectomy should be offered. In case of duplex kidney, only the non-functioning pole should be removed, and the functioning pole should be preserved. Open, laparoscopic and robotic laparoscopic procedure can be safely performed according to patient’s condition and surgeon capabilities [32].

For symptomatic patients with functioning kidney, endoscopic incision puncture or resection of the ureterocele and stone fragmentation when present will lead to symptom elimination with minimal morbidity. The main complication of these procedures is secondary vesico-ureteral reflux (VUR). Although it seems logical that puncture may less lead to VUR, prospective comparison has not been performed. The significance of post incision reflux has not completely clarified in adults.

Chtourou et al. described 20 patients (mean age 48 years) with ureterocele diagnosed due to chronic back pain; 16 had a single system and four duplex. All had an endoscopic incision, and in the presence of stones, fragmentation. Elimination of pain was successfully achieved in all patients. In a single patient the procedure was complicated with sepsis and one patient developed transient vesico ureteral reflux [10]. Vasu et al. described a rare case of bilateral ureterocele causing progressive renal failure that reversed following bilateral incision. Although these are single case reports, such complications should be taken in management considerations [47].