Neonatal adrenal masses: do we have reliable criteria for differential diagnosis and expectant management?

AIM: Differential diagnosis of neonatal adrenal masses (NAM) is often based on empirical criteria. Expectant management relies on spontaneous regression, described either for NB as for adrenal hemorrhage (AH). Histology was available for biopsied cases only. Aim of the study was to correlate clinical, laboratory and imaging data of a series of NAM, to final diagnosis. METHODS: Records of 23 NAM, diagnosed before or after birth, were reviewed, collecting data about: obstetrical history, clinical features, imaging, laboratory data, surgical findings, outcome. RESULTS: M/F ratio was 1.6/1. Size of the lesion ranged from 17 to 50 mm.. Doppler sonography (PD) showed no flow in 14/23. Urinary catecholamines (UCM) were elevated in 5/23. Reduction in a mean time of 3 months occurred in 14/23. MIBG and CT scans resulted positive in 8 and 9 cases among 16 NAM, unvaried or increased at one month. Three cases developed as IV S NB; diagnosis was confirmed by CT guided biopsy; regression occurred within 20 months. Surgery was decided for 6 unchanged/increasing NAM after 3-6 months; they were all NB. Predictive value for NB was high for MIBG and CT scan and was lower for high UCM level and positive PD findings. CONCLUSION: Clear criteria to differentiate AH from NB are still missing. Despite spontaneous regression is thought to indirectly confirm a non neoplastic lesion, benign evolution has also been documented for NB. As histology is available only in operated or biopsied cases, we still lack a reliable set of signs for early differentiation and to reduce repeated, invasive investigations.

Patients with a history of infection and voiding dysfunction are at risk for recurrence after successful endoscopic treatment of vesico ureteral reflux and deserve long-term follow up.

AIM OF THE STUDY: Subureteral endoscopic injection of a bulking agent is an attractive alternative to open surgery or antibiotic prophylaxis for vesico ureteral reflux (VUR). Little information is available about long-term risk of recurrence after an initially successful treatment. Aim of this paper was to review short- and long-term success rate of endoscopic treatment in a single Center series after risk stratification of individual patients. MATERIALS AND METHODS: The records of 126 patients who underwent Deflux injection for primary VUR were examined. Indications to treatment were an unvaried high grade VUR (IV-V) at 1 year from diagnosis and/or and recurrent urinary tract infection (UTI) on antibiotic prophylaxis even in the presence of mild grade VUR (III grade). Gender, age and mode of diagnosis, infections (UTI), voiding dysfunctions, VUR grade and side, renal function, number of treatments were correlated to outcome. Long-term evaluation was planned at a minimum of 1 year from the last negative post-injection cystogram (MCUG). A new MCUG and DMSA scan were also offered to those complaining new UTI episodes. Late recurrences were correlated to history and grade of reflux. Data were analyzed with Graph Pad Instat software; the Chi-square test was used for univariate comparisons, the Fisher's exact test for categorical variables.and multiple regression tests for factors influencing outcome. RESULTS: M/F ratio was 62 to 64; median age at diagnosis was 28 months. VUR affected 198 renal units. Preinjection VUR grade was I in 1, II in 27, III in 107, IV in 59, and V in 4 units. Reduced DMSA uptake was evidenced in 51 units and scarring in 24. Median age at treatment was 34.5 months, for persistent high grade VUR (IV-V) in 55 patients and recurrent IVU in 92. Two hundred sixty seven injections were performed on 198 ureters. Complete resolution was documented by MCUG at 3-5 months in 68%, low grading < II in 20%, persistence or unsignificant reduction in 11%. Preoperative recurrent UTI, higher grade VUR, and bilaterality were correlated to a poorer surgical outcome. Among 80 successfully treated cases, 12 complained of persistent UTI. Recurrence of VUR was demonstrated in 31% of them. Deteriorated uptake or additional scarring in 25% was independent from VUR recurrence. Preoperative recurrent UTI and voiding dysfunction correlated significantly to late outcome. CONCLUSIONS: Preoperative recurrent IVU, together with high-grade reflux, seem to correlate to lower success rate of Deflux injection for primary VUR. Even after successful endoscopic treatment, long-term surveillance may be needed among these cases, mainly if voiding dysfunction is also recorded. Late recurring VUR must be actively excluded in case of new IVU episodes.

An all-endo Approach to Complete Ureteral Duplications Complicated by Ureterocele and/or Vesicoureteral Reflux: Feasibility, Limitations, and Results.

Purpose. Totally endoscopic management (all-endo) of patients with a duplicated renal system (DS) associated with severe vesicoureteral reflux (VUR) or obstructive ureterocele (UC) is an attractive alternative to traditional open procedures. The authors discuss feasibility and results of an all-endo approach on a consecutive series of patients. Methods. From 1999 to 2009, all patients with a complete DS associated with UC and/or VUR were proposed for primary all-endo approach. UC puncture was performed using a 3 Fr Bugbee electrode. Deflux (dextranomer/hyaluronic acid copolymer) injection was administered for VUR. The need for secondary surgery was evaluated on followup. Results. Of the 62 patients recruited, 46 were treated using a primary all-endo approach and 16 patients received no treatment. Of the 46 treated patients with 56 affected renal units, 32 (97%) UCs collapsed following puncture and 29 (63%) VURs were resolved or downgraded. Secondary VUR occurred in 13 (39%) renal units. Secondary surgery was performed on 23 (41%) renal units. Conclusion. The all-endo approach for VUR in DS is an effective therapeutic option. UC collapse was achieved by puncture in most of the patients; secondary VUR was the main complication in a small group of extravesical UC.

Endoscopic subureteral injection for vesicoureteral reflux and the risk of overtreatment.

AIM: Risk of vesicoureteral reflux (VUR) overtreatment was anticipated following introduction of endoscopic treatment (ET). New tool reduces hospitalization and patients discom-fort, with good results and parental preferences may prevail on questions about benefits of treatment. The authors analyzed two series of patients to evaluate impact of ET on management. METHODS: Two hundred sixty-four patients were selected; group A (90 cases) were observed and treated before adoption of ET. Group B included 174 patients who benefited of ET (Deflux). Treatment started from grade III (infections or renal damage). Lower refluxes were treated only in association to an higher grade in the other kidney. 81 ureters were reinplanted in Group A (92% success rate); 67 ureters were reinplanted in group B (98% sr) and 115 had a ET (89% sr). Rate of treatment, time of follow up and age at operation were compared. RESULTS: No differences were found in order to reflux grade distribution, treatment rate and time spent waiting for spontaneous before reinplant or ET. ET was a first choice procedure, for grade III, in group B. Reinplant continued to have a role for grade IV cases, which responded to ET in 85%, and for grade V. Spontaneous resolution was observed respectively in 29% and 30% (grade III) and 13% and 7% (grade IV). CONCLUSIONS: Despite ET is accessible and effective, there are no evidences that extensive indications are of any benefit. Enthusiasm for new tools must be submitted to the need for long term, prospective studies to support our indications.

The risk of associated urological abnormalities in children with pre and postnatal occasional diagnosis of solitary, small or ectopic kidney: is a complete urological screening always necessary?

OBJECTIVE: Voiding cystourethrogram (VCUG) and radionuclide scan is recommended for patients with solitary (secondary to aplasia or multicystic dysplasia), hypoplasic or single ectopic kidney, to detect associated anomalies (vesicoureteric reflux, obstructive uropathies). With the increase of occasional diagnosis, mainly by fetal ultrasound (US), the possibility of an unjustified extension of diagnostic work up must be prevented. Aim of this paper was to estimate the incidence of associated anomalies in asymptomatic cases without associated US signs of hydronephrosis. MATERIALS AND METHODS: Among 158 Patients examined there were 81 solitary kidneys (26 multicystic dysplasia), 27 small kidneys, 50 single ectopic kidneys); prenatal diagnosis was recorded in 86%. Incidence of associated anomalies was compared with figures resulting when symptomatic cases or with hydronephrosis were excluded. RESULTS: Vesicoureteral reflux or obstruction were found in 17% of solitary kidneys, 70% of hypoplasic kidneys and 2% of single ectopic kidneys. Among those (120 cases) without infection or hydronephrosis, incidence decreased, respectively to 5, 60 and 0%. CONCLUSIONS: Associated anomalies are reported to affect up to 48% of solitary kidneys and about 30% of single ectopic; 80% of severe reflux are usually associated to small kidneys. In our series of solitary and ectopic kidneys incidence of abnormalities was significantly less and fell to negligible values when occasionally detected, undilated cases were considered. On this basis, indiscriminate urological screening simply based on the occasional pre or postnatal detection of undilated solitary or ectopic kidney appears to be unjustified. Small kidneys deserve special attention and VCUG is always indicated.

Diagnostic workup of urinary tract infections within the first 24 months of life, in the era of prenatal diagnosis. The contribution of different imaging techniques to clinical management.

AIM: The aim of this study was to evaluate the role of different techniques in the diagnostic workup of children with negative prenatal ultrasonogram, referred for urinary tract infection (UTI) within the first 24 months. METHODS: One-hundred and forty-seven patients, 71 males/76 females were studied. All patients were submitted to renal ultrasonogram (RUS) and to micturating cystourethrogram (MCU) independently from the results of RUS. In a small group (48 children) DMSA scan was performed independently from the results of RUS and MCU. Sensitivity and predictive value of RUS for vesicoureteric reflux (VUR) were estimated. Multiple regression analysis was performed on a selected number of signs to evaluate their predictive value. The group investigated by DMSA scan was analysed to evaluate how the presence of VUR on MCU anticipated renal damage. RESULTS: Nineteen (21%) patients with normal RUS, had VUR. Predictive value of RUS (0.21) was influenced by the grade of the VUR but more than 30% of high grade refluxing renal units appeared normal at initial ultrasonography. DMSA scan was abnormal in 27% of 48 patients; its result was independent from the presence of VUR of whatever grade. CONCLUSIONS: The increasing number of renal abnormalities detected before birth reduces the possibility of late abnormal RUS findings. It makes RUS screening for abnormalities, after a first episode of UTI, scarcely useful. VUR may be easily missed when RUS resulted normal and MCU is omitted. Negative MCU cannot exclude renal damage in presence of UTI. Renal defects at DMSA scan may be unrelated to a demonstrable VUR and could have a different pathogenesis.

Functional outcome after pyeloplasty in children: impact of the cause of obstruction and of the mode of presentation.

OBJECTIVE: To compare functional outcome after pyeloplasty in two groups of patients affected by hydronephrosis, which had different modalities of diagnosis. The first, asymptomatic, detected by prenatal ultrasonography and the second referred later because of clinical symptoms. PATIENTS AND METHODS: 84 patients (54 prenatally detected and 30 symptomatic) among 339 observed with hydronephrosis, operated in a single centre for ureteropelvic junction obstruction, have been retrospectively studied. Seventeen cases with prenatal diagnosis had an early treatment and thirty-seven were operated on after an initial observation. Symptomatic cases were all operated on at diagnosis. Ultrasonographic and renographic assessment were made at diagnosis, before and after surgery. RESULTS: Renal function of prenatally detected cases was not influenced by early or delayed surgery. Insignificant functional loss was recorded among some cases operated after conservative management. Improvement in differential renal function (p<0.05) was recorded in symptomatic patients after pyeloplasty. A high percentage of obstructions secondary to lower polar crossing vessel were found among symptomatic cases (12 out of 30). Intrinsic obstructions were predominant among cases with in utero diagnosis (48 out of 54). Whenever the cause of obstruction is considered, postoperative functional improvement was limited to crossing vessels cases. CONCLUSIONS: Renal function among antenatally diagnosed obstructions is scarcely influenced by surgery; potential risk of further renal damage cannot be excluded when expectant management is decided. Later detected, symptomatic cases show a better functional response to surgery. This seems corresponding to distinct clinical entities based on different causes of hydronephrosis. A reversible renal damage seems to be associated to extrinsic obstructions from polar vessel, which are predominant among symptomatic, later detected cases. A congenital, irreversible loss of function accompanies intrinsic obstructions, typical of prenatally diagnosed cases.

[Ureterocele associated to duplex system: an individualized approach to endoscopic incision]. / Ureterocele e duplicità pieloureterale completa: criteri per un approccio individualizzato e ruolo dell'incisione endoscopica.

BACKGROUND: Management of Ureterocele (UC) associated to duplex kidney depends from the type of UC (ectopic or intravesical), from the presence of symptoms and from associated vesicoureteral reflux (VUR) in the lower pole of the duplex system. Individualized approach to UC must consider endoscopic puncture as a possible option to reduce hydronephrosis and preserve function. METHODS: The records of 68 patients with duplex system have been reviewed. UC was detected in 27 renal units: it was intravesical in 11 and ectopic in 16. VUR was present in the lower pole in 16 cases. Patients were observed between 1991 and 2001 and mean follow up lasted 20 months (range 2-70). Endoscopic incision was performed in 21 patients. Upper pole nephrectomy, intravesical reconstruction or simple observation were decided for six patients. RESULTS: Endoscopic incision was effective in the treatment of intravesical UC and in some cases, without VUR in the lower pole, it was the unique procedure. Among ectopic UC, a secondary VUR developed following endoscopic incision in 43% of cases and required further treatment. Renogram did not show any significant functional improvement in the affected renal pole, secondary to endoscopic treatment. An open antireflux procedure was necessary in cases with associated VUR. CONCLUSIONS: Total or partial nephrectomy were performed among the first cases of the present series and in most of them it required a double step operation (abdominal and intravesical). In few cases of uncomplicated UC with a non functioning upper pole, a conservative approach was followed by spontaneous reduction of dilatation.