Impact of low- or moderate-risk gonadotoxic chemotherapy prior to testicular tissue freezing on spermatogonia quantity in human (pre)pubertal testicular tissue.

STUDY QUESTION: What is the impact of low- or moderate-risk gonadotoxic chemotherapy received prior to testicular tissue freezing (TTF), and of the cancer itself, on spermatogonia quantity in testicular tissue from (pre)pubertal boys? SUMMARY ANSWER: Vincristine, when associated with alkylating agents, has an additional adverse effect on spermatogonia quantity, while carboplatin has no individual contribution to spermatogonia quantity, in testicular tissue of (pre)pubertal boys, when compared to patients who have received non-alkylating chemotherapy. WHAT IS KNOWN ALREADY: The improved survival rates after cancer treatment necessitate the inclusion of fertility preservation procedures as part of the comprehensive care for patients, taking into consideration their age. Sperm cryopreservation is an established procedure in post-pubertal males while the TTF proposed for (pre)pubertal boys remains experimental. Several studies exploring testicular tissue of (pre)pubertal boys after TTF have examined the tubular fertility index (TFI, percentage of seminiferous tubule cross-sections containing spermatogonia) and the number of spermatogonia per seminiferous tubule cross-section (S/T). All studies have demonstrated that TFI and S/T always decrease after the introduction of chemotherapeutic agents, especially those which carry high gonadotoxic risks such as alkylating agents. STUDY DESIGN, SIZE, DURATION: Testicular tissue samples from 79 (pre)pubertal boys diagnosed with cancer (from 6 months to 16 years of age) were cryopreserved between May 2009 and June 2014. Their medical diagnoses and previous chemotherapy exposures were recorded. We examined histological sections of (pre)pubertal testicular tissue to elucidate whether the chemotherapy or the primary diagnosis affects mainly TFI and S/T. PARTICIPANTS/MATERIALS, SETTING, METHODS: (Pre)pubertal boys with cancer diagnosis who had been offered TTF prior to conditioning treatment for hematopoietic stem cell transplantation were included in the study. All the patients had previously received chemotherapy with low- or moderate-risk for future fertility. We have selected patients for whom the information on the chemotherapy received was complete. The quantity of spermatogonia and quality of testicular tissue were assessed by both morphological and immunohistochemical analyses. MAIN RESULTS AND THE ROLE OF CHANCE: A significant reduction in the number of spermatogonia was observed in boys treated with alkylating agents. The mean S/T values in boys exposed to alkylating agents were significantly lower compared to boys exposed to non-alkylating agents (P = 0.018). In contrast, no difference was observed for patients treated with carboplatin as the sole administered alkylating agent compared to the group of patients exposed to non-alkylating agents. We observed an increase of S/T with age in the group of patients who did not receive any alkylating agent and a decrease of S/T with age when patients received alkylating agents included in the cyclophosphamide equivalent dose (CED) formula (r = 0.6166, P = 0.0434; r = -0.3759, P = 0.0036, respectively). The TFI and S/T decreased further in the group of patients who received vincristine in combination with alkylating agents (decrease of 22.4%, P = 0.0049 and P < 0.0001, respectively), but in this group the CED was also increased significantly (P < 0.0001). Multivariate analysis, after CED adjustment, showed the persistence of a decrease in TFI correlated with vincristine administration (P = 0.02). LIMITATIONS, REASONS FOR CAUTION: This is a descriptive study of testicular tissues obtained from (pre)pubertal boys who were at risk of infertility. The study population is quite heterogeneous, with a small number of patients in each sub-group. Our results are based on comparisons between patients receiving alkylating agents compared to patients receiving non-alkylating agents rather than chemotherapy-naive patients. The French national guidelines for fertility preservation in cancer patients recommend TTF before highly gonadotoxic treatment. Therefore, all the patients had received low- or moderate-risk gonadotoxic chemotherapy before TTF. Access to testicular tissue samples from chemotherapy-naive patients with comparable histological types of cancer was not possible. The functionality of spermatogonia and somatic cells could not be tested by transplantation or in vitro maturation due to limited sample sizes. WIDER IMPLICATIONS OF THE FINDINGS: This study summarizes the spermatogonial quantity of (pre)pubertal boys prior to TTF. We confirmed a negative correlation between the cumulative exposure to alkylating agents and spermatogonial quantity. In addition, the synergistic use of vincristine in combination with alkylating agents showed a cumulative deleterious effect on the TFI. For patients for whom fertility preservation is indicated, TTF should be proposed for chemotherapy with a predicted CED above 4000 mg/m2. However, the data obtained from vincristine and carboplatin use should be confirmed in a subsequent study including more patients. STUDY FUNDING/COMPETING INTEREST(S): This study had financial support from a French national research grant PHRC No. 2008/071/HP obtained by the French Institute of Cancer and the French Healthcare Organization. The sponsors played no role in the study. The authors declare no conflicts of interest. TRIAL REGISTRATION NUMBER: N/A.

Outcome of long gap esophageal atresia at 6 years: A prospective case control cohort study.

BACKGROUND DATA: EA is the most frequent congenital esophageal malformation. Long gap EA remains a therapeutic challenge for pediatric surgeons. A case case-control prospective study from a multi-institutional national French data base was performed to assess the outcome, at age of 1 and 6 years, of long gap esophageal atresia (EA) compared with non-long gap EA/tracheo-esophageal fistula (TEF). The secondary aim was to assess whether initial treatment (delayed primary anastomosis of native esophagus vs. esophageal replacement) influenced mortality and morbidity at ages 1 and 6 years. METHODS: A multicentric population-based prospective study was performed and included all patients who underwent EA surgery in France from January 1, 2008 to December 31, 2010. A comparative study was performed with non-long gap EA/TEF patients. Morbidity at birth, 1 year, and 6 years was assessed. RESULTS: Thirty-one patients with long gap EA were compared with 62 non-long gap EA/TEF patients. At age 1 year, the long gap EA group had longer parenteral nutrition support and longer hospital stay and were significantly more likely to have complications both early post-operatively and before age 1 year compared with the non-long gap EA/TEF group. At 6 years, digestive complications were more frequent in long gap compared to non-long gap EA/TEF patients. Tracheomalacia was the only respiratory complication that differed between the groups. Spine deformation was less frequent in the long gap group. There were no differences between conservative and replacement groups at ages 1 and 6 years except feeding difficulties that were more common in the native esophagus group. CONCLUSIONS: Long gap strongly influenced digestive morbidity at age 6 years.

Assessment of the architecture and integrity of frozen-thawed testicular tissue from (pre)pubertal boys with cancer.

BACKGROUND: Testicular tissue freezing is proposed for fertility preservation to (pre)pubertal boys with cancer before highly gonadotoxic treatment. Studies accurately comparing human (pre)pubertal testicular tissue quality before freezing and after thawing are exceptional. No study has reported this approach in a systematic manner and routine care. OBJECTIVES: To assess the impact of a control slow freezing protocol on testicular tissue architecture and integrity of (pre)pubertal boys after thawing. MATERIALS AND METHODS: (Pre)pubertal boys (n = 87) with cancer from 8 Reproductive Biology Laboratories of the French CECOS network benefited from testicular tissue freezing before hematopoietic stem cell transplantation. Seminiferous tubule cryodamage was determined histologically by scoring morphological alterations and by quantifying intratubular spermatogonia and the expression of DNA replication and repair marker in frozen-thawed testicular fragments. RESULTS: A significant increase in nuclear and epithelial score alterations was observed after thawing (p < 0.0001). The global lesional score remained lower than 1.5 and comparable to fresh testicular tissue. The number of intratubular spermatogonia and the expression of DNA replication and repair marker in spermatogonia and Sertoli cells did not vary significantly after thawing. These data showed the good preservation of the seminiferous tubule integrity and architecture after thawing, as previously reported in our studies performed in prepubertal mice and rats. DISCUSSION: The current study reports, for the first time, the development of a semi-quantitative analysis of cryodamage in human (pre)pubertal testicular tissue, using a rapid and useful tool that can be proposed in routine care to develop an internal and external quality control for testicular tissue freezing. This tool can also be used when changing one or several parameters of the freezing-thawing procedure. CONCLUSION: Control slow freezing protocol without seeding maintains the seminiferous tubule architecture and integrity, the concentration of spermatogonia and the expression of DNA replication and repair marker in spermatogonia and Sertoli cells after thawing.

Fetal megacystis-microcolon: Genetic mutational spectrum and identification of PDCL3 as a novel candidate gene.

Megacystis-microcolon-intestinal-hypoperistalsis syndrome (MMIHS) is a severe congenital visceral myopathy characterized by an abdominal distension due to a large non-obstructed urinary bladder, a microcolon and intestinal hypo- or aperistalsis. Most of the patients described to date carry a sporadic heterozygous variant in ACTG2. More recently, recessive forms have been reported and mutations in MYH11, LMOD1, MYLK and MYL9 have been described at the molecular level. In the present report, we describe five patients carrying a recurrent heterozygous variant in ACTG2. Exome sequencing performed in four families allowed us to identify the genetic cause in three. In two families, we identified variants in MMIHS causal genes, respectively a nonsense homozygous variant in MYH11 and a previously described homozygous deletion in MYL9. Finally, we identified compound heterozygous variants in a novel candidate gene, PDCL3, c.[143_144del];[380G>A], p.[(Tyr48Ter)];[(Cys127Tyr)]. After cDNA analysis, a complete absence of PDLC3 expression was observed in affected individuals, indicating that both mutated transcripts were unstable and prone to mediated mRNA decay. PDCL3 encodes a protein involved in the folding of actin, a key step in thin filament formation. Presumably, loss-of-function of this protein affects the contractility of smooth muscle tissues, making PDCL3 an excellent candidate gene for autosomal recessive forms of MMIHS.

Diffusion tensor imaging in acute pyelonephritis in children.

BACKGROUND: Diffusion-weighted imaging plays a key role in the imaging of acute pyelonephritis by MRI. However the use of respiratory triggering is challenging and time-consuming in children. Diffusion tensor imaging without respiratory triggering might provide satisfying images of the moving kidneys. OBJECTIVE: To compare mean diffusivity diffusion tensor images obtained with free breathing with diffusion-weighted images obtained with respiratory triggering. MATERIALS AND METHODS: Thirty-one children with suspected acute pyelonephritis underwent axial diffusion tensor imaging acquisition with free breathing and axial and coronal diffusion-weighted imaging acquisitions with respiratory triggering. We compared image quality and detection of nephritis between the two sequences. RESULTS: Diffusion tensor imaging demonstrated agreement with diffusion-weighted imaging in all cases, with no difference in the detection of nephritis areas. The image quality was significantly better with diffusion tensor imaging (P<0.01). CONCLUSION: Diffusion tensor imaging could replace diffusion-weighted imaging for diagnosis of acute pyelonephritis.

Intradetrusor Injections of Botulinum Toxin Type A in Children With Spina Bifida: A Multicenter Study.

OBJECTIVE: To assess the effectiveness of intradetrusor injections of botulinum toxin type A (IDBTX-A) in children with spina bifida. METHODS: All patients aged less than 16 years old who underwent IDBTX-A between 2002 and 2016 at 6 institutions were included in a retrospective study. Our primary endpoint was the success rate of IDBTX-A defined as both clinical improvement (no incontinence episodes between clean intermittent catheterizations [CICs], absence of urgency, and less than 8 CICs per day) and urodynamic improvement (resolution of detrusor overactivity and normal bladder compliance for age) lasting ≥12 weeks. Predictive factors of success were assessed through univariate analysis. RESULTS: Fifty-three patients with a mean age of 8.5 years were included. All patients were under CIC and 88.7% had received anticholinergics with either poor efficacy or bothersome adverse events. The global success rate of the first injection (clinical and urodynamic) was 30%. Patients with closed spinal dysraphism had a significantly better success rate than patients with myelomeningocele (P = .002). The clinical success rate was 66% and was significantly associated with maximum urethral closure pressure (34 cm H2O vs 54.4 cm H2O, P = .02). The urodynamic success rate was 34%. Maximum cystometric capacity (P <.0001) and compliance (P = .01) significantly improved after the first IDBTX-A and maximum detrusor pressure tended to decrease (P = .09) except in the subgroup of patients with poor compliance. After a mean follow-up of 3.7 years, 23 patients (43.4%) required augmentation cystoplasty. Excluding 6 patients who were lost to follow-up, 38.3% of patients were still undergoing botulinum toxin injections at last follow-up. CONCLUSION: In this series, despite the fact that IDBTX-A enabled clinical improvement in 66% patients, urodynamic outcomes were poor resulting in a low global success rate (30%).

Supranormal differential renal function in unilateral hydronephrotic kidney: insights from functional MR urography.

PURPOSE: To retrospectively assess supranormal differential renal function (DRF) in unilateral hydronephrotic kidney by functional MR urography (fMRU). MATERIALS AND METHODS: The ethics committees granted exempt status for this study and also waived the need for informed consent. A retrospective analysis was performed of all patients undergoing fMRU from January 2008 to November 2011. DRF was measured by both the area under the curve method and Rutland-Patlak plot. Glomerular DRF per unit of volume and kidney volumes were analyzed. Kidney volumes were compared with nomograms. RESULTS: Of 170 children, 3 patients (2, 12, and 14 years) with a left ureteropelvic junction obstruction were included. Glomerular DRF per unit of volume was slightly higher on the dilated side but remained within normal range (<55%). Dilated kidney volumes were higher on the dilated side (≥55%). The volumes of nondilated kidneys were within normal range. CONCLUSION: The data support the fact that the dilated kidney was not hyperfunctioning and that the nondilated kidney was not hypofunctioning. Based on these three patients, it could be assumed that supranormal DRF on the dilated side might be explained by a slight kidney volume asymmetry, in association with higher DRF per unit of volume, remaining within normal physiological range. J. Magn. Reson. Imaging 2014;40:577-582. © 2013 Wiley Periodicals, Inc.

MRI and suspected acute pyelonephritis in children: comparison of diffusion-weighted imaging with gadolinium-enhanced T1-weighted imaging.

OBJECTIVES: To evaluate the performance of diffusion-weighted imaging (DWI) against the reference standard of gadolinium-enhanced T1-weighted imaging (Gd-T1-WI) in children. METHODS: Thirty-nine consecutive patients (mean age 5.7 years) with suspected acute pyelonephritis underwent magnetic resonance imaging (MRI) including DWI and (the reference standard) Gd-T1-WI. Each study was read in double-blinded fashion by two radiologists. Each kidney was graded as normal or abnormal. Sensitivity and specificity of DWI were computed. Agreement between sequences and interobserver reproducibility were calculated (Cohen κ statistic and the McNemar tests). RESULTS: Thirty-two kidneys (41 %) had hypo-enhancing areas on Gd-T1-W images. The sensitivity and specificity of DWI were 100 % (32/32) and 93.5 % (43/46). DWI demonstrated excellent agreement (κ = 0.92,) with Gd-T1-W, with no significant difference (P = 0.25) in detection of abnormal lesions. Interobserver reproducibility was excellent with DWI (κ = 0.79). CONCLUSION: DWI enabled similar detection of abnormal areas to Gd-T1-WI and may provide an injection-free means of evaluation of acute pyelonephritis. KEY POINTS: • Diffusion weighted magnetic resonance imaging (DWI) can confirm acute pyelonepritis. • DWI provided comparable results to gadolinium enhanced T1-W MRI in acute pyelonepritis. • Contrast medium injection could be avoided for diagnosing acute pyelonephritis by MRI. • MRI with T2-WI and DWI provide a fast and comprehensive diagnostic tool.

Idiopathic varicocele in adolescents: risks of the inguinal approach.

INTRODUCTION: The aim of this study was to evaluate the medium-term results and complications of open inguinal varicocelectomy, including vein ligation, intraoperative venography, and antegrade sclerotherapy. MATERIALS AND METHODS: Sixty-four children were treated between 2000 and 2009 for idiopathic varicocele. Fifty children were examined 6 months after surgery. In 2010, 22 patients were recalled for testicular ultrasound scans (US) to evaluate the medium-term results of the technique. RESULTS: The mean age of the children was 12.8 years at first consultation. Of the 50 cases, 35 children were asymptomatic, 13 experienced pain, 3 suffered from discomfort, and 1 had testicular asymmetry. Thirteen children had delayed left testicular growth compared with the right testis. The mean age at surgery was 13.3 years, and follow-up duration was 8.3 months ± 13.9. Thirty-eight patients achieved good results postsurgery; there was varicocele recurrence in 3, testicular hypotrophy in 7, and complete testicular atrophy in 2 patients. CONCLUSION: Naked eye inguinal surgical ligation does not appear to be safe enough to treat young adolescents, with the theoretical risk of a decrease in fertility in the future. In teams which are untrained in microsurgical or laparoscopic varicocelectomy, we suggest referring adolescent patients to a radiologist for embolization.

Uterus didelphys, hemihydrocolpos and empty ipsilateral lumbar fossa in a newborn girl: involuted dysplastic kidney rather than renal agenesis.

We report a case of Herlyn-Werner-Wunderlich syndrome diagnosed in the neonatal period. US revealed the classic association of a uterus didelphys with blind hemivagina and no ipsilateral kidney. The diagnosis was established by postnatal US and confirmed by MRI. Differential diagnoses are discussed. A trans-hymeneal resection of the vaginal septum was performed at 1 month of age. Intra operative endoscopy revealed no left hemitrigone but showed an atretic orifice in the ipsilateral blind hemivagina, probably corresponding to the insertion of an ectopic ureter. Follow-up was unremarkable.

MR urography in children. Part 1: how we do the F0 technique.

MR urography (MRU) has been widely accepted as a substitute to intravenous urography for investigating children with a dilated urinary tract after preliminary assessment by US and voiding cystourethrography. Hydronephrosis is by far the main indication for MRU because upper tract dilatation is a frequent condition in infants and children. Recent advances in technology have allowed MR to go beyond morphology and to assess renal function parameters such as split renal function and drainage. In this article we report our routine practice of the F0 MRU technique. The main advantages of our protocol are no requirement for general anaesthesia, no bladder catheterization, use of low-dose gadolinium-based contrast agent (0.05-0.1 mmol/kg) and total acquisition time of 30 min or less.

Pediatric urinary incontinence: evaluation of bladder wall wraparound sling procedure.

PURPOSE: In 1996 a surgical procedure for sphincter deficiency was reported in patients with neurogenic urinary incontinence. To our knowledge we report the first series that assesses this procedure since it was initially described. MATERIALS AND METHODS: Seven female and 7 male patients with spinal dysraphism and neurogenic intrinsic sphincter deficiency underwent surgery between 1997 and 2005. All patients had an associated poorly compliant or small capacity bladder. Patient age ranged from 8 to 22 years (mean 14) and all patients presented with persistent urinary incontinence despite intermittent catheterization and pharmacotherapy. Evaluation was based on clinical examination, as well as preoperative and postoperative urodynamic assessments. The surgical procedure involved wrapping a pedicle strip of anterior bladder wall around the bladder neck and fixing it on the pubic symphysis. Bladder augmentation cystoplasty was also routinely performed during the same procedure. RESULTS: Of 14 patients 13 were completely dry and 1 had stress incontinence. Followup was 2 to 8 years (mean 5). Postoperative urodynamic evaluation showed a maximum urethral pressure increase of 40%. We also observed an increase in bladder capacity of 105% and improved bladder compliance (7 ml/cm H(2)O preoperatively vs 35 ml/cm H(2)O postoperatively). CONCLUSIONS: The bladder wall wraparound sling procedure provides excellent results for continence in association with bladder augmentation.

Outcome of surgical management of concealed penis.

OBJECTIVE: To review the etiological factors, clinical presentations and outcome of our surgical technique to correct the anomaly of concealed penis. PATIENTS AND METHODS: Thirty consecutive patients were treated in 2000-2004. Mean age at operation was 6.7 years (range 13 months-15.4 years). They underwent penoplasty alone (22 patients) or penoplasty with liposuction of prominent prepubic fat pad (eight patients). The medical records of all the patients were retrospectively reviewed, and 23 patients were interviewed, examined clinically and questioned about their initial complaint and the results of our technique. Mean follow up was 28 months (range 6 months-5 years). RESULTS: The patients most commonly presented with cosmetic (60.0%), voiding (56.6%) and psychosocial (50.5%) concerns. Three underlying anatomic defects contributing to concealment were observed: fibrotic dartos fascia with poor skin attachment at the base of the penis (93%), prominent prepubic fat pad (40%) and postoperative phimosis leading to trapped penis (26%). Early postoperative complications occurred in 4 patients (13.3%) and all were treated conservatively and successfully. Fourteen patients (46.6%) underwent secondary minor day-case operations, the majority for excision of redundant subcoronal preputial collar. Clinical examination of 23 patients showed very good results in terms of general appearance and accessibility. The older boys were generally more satisfied with the results of surgery than the parents of younger children, whose main source of dissatisfaction was the final circumcised appearance of the penis. CONCLUSION: The surgical approach used to correct concealed penis alleviates the initial complaint and provides good cosmetic and functional results with greater satisfaction in older patients. Our technique had a low early complication rate but a significant number of patients needed a secondary procedure to improve the final cosmetic results.

Quantitative analysis of 99mTc-DMSA during acute pyelonephritis for prediction of long-term renal scarring.

UNLABELLED: This study was performed to evaluate a quantitative method based on (99m)Tc-DMSA renal planar scintigraphy performed during acute pyelonephritis (APN) to detect kidneys at risk of scarring. METHODS: A total of 43 children (5.8 +/- 3.6 y old [mean +/- SD]) were examined by (99m)Tc-DMSA scintigraphy during (DMSA 1) and 8 +/- 2 mo after (DMSA 2) APN. Two levels of interpretation were performed independently: first, a semiquantitative analysis to classify the kidneys by considering the evolution between DMSA 1 and DMSA 2 (i.e., to determine which kidneys had developed scarring), and second, an automatic quantitative analysis of DMSA 1 to define and to evaluate a predictive index for kidney evolution from DMSA 1 to DMAS 2. The method consisted of determining an automatic threshold for the kidney and then calculating ratios of the count density in a given isocount n% (region of interest containing all the pixels with a value > or = n% of the value of the pixel with the maximal activity value) to the count density in a 20% isocount (C(n%)) and the number of pixels in a given isocount to the number of pixels in a 20% isocount (S(n%)). RESULTS: All kidneys normal at DMSA 1 remained normal at DMSA 2. For the automatic index, the C(70%) ratio was considered the best index for the prediction of scarring. When this C(70%) ratio was used, a cutoff value of 0.45 was able to predict scarring with a sensitivity of 0.83, a specificity of 0.78, a positive predictive value of 0.85, and a negative predictive value of 0.77. CONCLUSION: A cutoff value of 0.45 for the C(70%) ratio calculated for (99m)Tc-DMSA scintigraphy performed during APN may be useful for detecting kidneys at risk of scarring.

[Uroflowmetric functional evaluation of modified Duplay procedure in hypospadias surgery]. / Evaluation fonctionnelle par débitmétrie de l'urétroplastie de Duplay modifiée dans la chirurgie de l'hypospade.

We perform a modified Duplay procedure in our department without incision of the urethral plate for correction of distal hypospadias. The objective of this retrospective study was to analyse the long-term functional and aesthetic results of this technique. Strictures are a frequent complication of hypospadias surgery, but their frequency is often underestimated as they can remain asymptomatic. We decided to systematically detect urethral strictures by performing voiding uroflowmetry in all children over the age of 4 years, i.e. toilet trained, operated by Duplay procedure in our department. The results were compared to the standard results in paediatric populations published in the literature. The maximum flow rate was below the 5th percentile in 20% of cases and the average flow rate was abnormal in 30% of cases. Voiding uroflowmetry is a simple, noninvasive method to detect asymptomatic urethral strictures. Uroflowmetry is essential to evaluate the functional results of surgical procedures used for the treatment of hypospadias.

Ureteropelvic junction obstruction in children: two variants of the same congenital anomaly?

OBJECTIVE: To compare the characteristics of prenatally and postnatally diagnosed ureteropelvic junction obstruction (UPJO) in children. PATIENTS AND METHODS: We reviewed the records of 74 children who underwent pyeloplasty or nephrectomy for UPJO between 1995 and 2000. The patients were divided into 2 groups: prenatally and postnatally diagnosed UPJO. In each group, we compared age at surgery, gender, affected side, anteroposterior diameter (APD) of the renal pelvis, surgical findings, and renal function as determined by creatinine clearance. RESULTS: Of the 74 children, 44 (59.4 percent) had a prenatal diagnosis of UPJO and 30 (40.6 percent) had a postnatal diagnosis despite the fact that all had had a fetal ultrasonography. Median age at the time of surgery was 6.3 years (4 months to 16 years) for children with postnatal UPJO and 3.6 months (1 month to 4 years) for the prenatal group. Forty-three percent of the children in the postnatal group and 25 percent in the prenatal group were females. Clinical manifestations in children with postnatal UPJO included abdominal pain in 13 (43 percent) patients, pyelonephritis in 7 (23 percent), urinary tract infection in 5 (16.6 percent), and occasional findings upon ultrasound in 5 (16.6 percent). Excretory urography suggested obstruction in most children. The surgical findings included ureteral kinks due to adhesions in 93.3 percent of postnatally diagnosed UPJO cases and in 27.3 percent of prenatal cases (p < 0.01). A reduction in mean creatinine clearance of hydronephrotic kidneys was observed for both groups when compared to reference values for the respective ages, but this difference was not statistically significant. CONCLUSIONS: Postnatally diagnosed UPJO may be considered, at least in part, an entity different from prenatally detected obstruction due to its peculiar characteristics, i.e., postnatal UPJO more frequently affects females, manifests later in life with urinary infection or abdominal pain, and is frequently associated with ureteral kinking.

Ureteropelvic junction obstruction in children: two variants of the same congenital anomaly?

OBJECTIVE: To compare the characteristics of prenatally and postnatally diagnosed ureteropelvic junction obstruction (UPJO) in children. PATIENTS AND METHODS: We reviewed the records of 74 children who underwent pyeloplasty or nephrectomy for UPJO between 1995 and 2000. The patients were divided into 2 groups: prenatally and postnatally diagnosed UPJO. In each group, we compared age at surgery, gender, affected side, anteroposterior diameter (APD) of the renal pelvis, surgical findings, and renal function as determined by creatinine clearance. RESULTS: Of the 74 children, 44 (59.4%) had a prenatal diagnosis of UPJO and 30 (40.6%) had a postnatal diagnosis despite the fact that all had had a fetal ultrasonography. Median age at the time of surgery was 6.3 years (4 months to 16 years) for children with postnatal UPJO and 3.6 months (1 month to 4 years) for the prenatal group. Forty-three percent of the children in the postnatal group and 25% in the prenatal group were females. Clinical manifestations in children with postnatal UPJO included abdominal pain in 13 (43%) patients, pyelonephritis in 7 (23%), urinary tract infection in 5 (16.6%), and occasional findings upon ultrasound in 5 (16.6%). Excretory urography suggested obstruction in most children. The surgical findings included ureteral kinks due to adhesions in 93.3% of postnatally diagnosed UPJO cases and in 27.3% of prenatal cases (p < 0.01). A reduction in mean creatinine clearance of hydronephrotic kidneys was observed for both groups when compared to reference values for the respective ages, but this difference was not statistically significant. CONCLUSIONS: Postnatally diagnosed UPJO may be considered, at least in part, an entity different from prenatally detected obstruction due to its peculiar characteristics, i.e., postnatal UPJO more frequently affects females, manifests later in life with urinary infection or abdominal pain, and is frequently associated with ureteral kinking.

Color and power Doppler sonography versus DMSA scintigraphy in acute pyelonephritis and in prediction of renal scarring.

UNLABELLED: Capabilities of color and power Doppler sonography (DS) were prospectively evaluated for diagnosis of acute pyelonephritis and for prediction of scarring by comparison with 99mTc-dimercaptosuccinic acid scintigraphy (DMSA). METHODS: Fifty-seven children (mean age, 5 +/- 3 y) with acute pyelonephritis were investigated by biologic testing, DS (DS 1), and DMSA (DMSA 1). Patients who were <6 mo old or had high-grade reflux or obstruction were excluded. Forty-five children had a clinical follow-up examination, biologic testing, DS (DS 2), and DMSA (DMSA 2) at a mean of 7 +/- 2 mo after acute infection. Sonography (gray-scale and DS) was performed by 1 experienced radiologist who was unaware of patient data. DMSA studies were interpreted by 2 physicians who were unaware of patient data. RESULTS: Temperature, neutrophil count, and C-reactive protein value were significantly higher in patients with abnormal DMSA 1 findings than in those with abnormal DS 1 findings (P < 0.05). When compared with DMSA 1, DS 1 had a sensitivity and specificity of 80% and 81%, respectively. At follow-up, all clinical and biologic data had normalized. Scarring after infection occurred in 51% of children. When compared with DMSA 2, DS 1 had positive and negative predictive values of 57% and 75%, respectively, and DMSA 1 had respective values of 62% and 100%. Reflux was not considered a good predictor of scarring. CONCLUSION: DS and DMSA results were concordant in 81% of kidneys with acute pyelonephritis. The predictive value of DS for renal scarring was not considered sufficiently high for DS to be used in routine practice.

[Survey on satisfaction of patients with Malone continent cecostomy]. / Enquête sur la satisfaction des patients porteurs d'une caecostomie continente de Malone.

OBJECTIVE: A questionnaire to assess their level of satisfaction was sent to 28 patients who underwent a continent cecostomy for antegrade enema (Malone procedure). MATERIAL AND METHOD: All the patients presented with neurogenic bladder and a severe constipation due to spina bifida in 21 cases, anorectal malformation in 4, cloacal malformation in 1, sacral agenesis in 1 and post-myelitis paraplegia in 1. The mean age at surgery was 15 years. The mean follow-up is 3 years and 8 months. Surgery on the bladder was done in 18 patients of which 10 at the same stage. Urinary continence between bladder catheterizations occurred in 5 patients with isolated cecostomy. RESULTS: 24 patients answered the questionnaire. Among the remaining 4, 1 has a too short follow-up, 1 refused the enemas and his cecostomy definitely closed and 2, when seen at clinics, have an excellent result. Enemas are done twice a week, they last on average one hour with an average of 3 litres of liquid, usually tap water. All the 24 patients consider to have benefited from the operation mainly regarding their personal, family and social wellbeing. All have acquired anal continence, only 3 have some soiling between enemas. The main reported drawbacks concern pain at passing of stools (14 cases) well improved by addition of Colopeg in the enema, fatigue (21 cases) or headache (5 cases) after enemas. These troubles, probably due to a temporary hyponatremia, are well improved by addition of table salt in the water. CONCLUSION: This enquiry established the high degree of satisfaction given by the Malone procedure in the treatment of severe constipation frequently associated with a congenital neurogenic bladder. Despite some drawbacks this solution must be considered in such situation.