Isolated Fallopian Tube Torsion in Children With Hydrosalpinx: Is Conservative Management an Option?

BACKGROUND: Isolated fallopian tube torsion (IFTT) is very rare gynecological emergency in pediatric population. Our objective is to assess treatment options and discuss outcome of a cohort of IFTT with a focus on the association between IFTT and hydrosalpinx (HSX). METHODS: A retrospective review was conducted. Pediatric patients with IFTT operated in the same center were included. RESULTS: Seventeen girls (aged: 11-16 years) were managed for acute abdominal pain between 2008 and 2018, with intraoperative diagnosis of IFTT. All patients underwent laparoscopic exploration, with laparoscopically fallopian tube detorsion in all patients. Based on the association of IFTT with HSX after fallopian tube detorsion, patients were divided into 2 groups: group 1 (IFTT without HSX; 12 girls) and group 2 (IFTT with HSX; 5 girls). During the same surgery, complementary surgical procedures were done. In group 1: salpingectomies (4), partial salpingectomies (2) and cystectomies (6) were done. In group 2: salpingectomy (1), salpingotomy (1), and cyst ablation (1). The treatment was called conservative when the tube was preserved.Follow-up was uneventful in group 1. In group 2, for all patients with initial fallopian tube preservation, further surgical procedures were necessary (1-4 surgeries/patient), and, finally, another 3 patients required salpingectomy. CONCLUSIONS: Conservative treatment with tube preservation of IFTT without HSX appeared to be beneficial compared to those with HSX, with no recurrence of torsion or symptoms during the follow-up. However, the same conservative treatment was not sufficiently effective for IFTT with HSX and required further procedures due to recurrence of torsion. LEVEL OF EVIDENCE: IV.

Ultrafast cadmium-zinc-telluride-based renal single-photon emission computed tomography: clinical validation.

BACKGROUND: One of the main limitations of 99mtechnetium-dimercaptosuccinic acid (DMSA) scan is the long acquisition time. OBJECTIVE: To evaluate the feasibility of short DMSA scan acquisition times using a cadmium-zinc-telluride-based single-photon emission computed tomography (SPECT) system in children. MATERIALS AND METHODS: The data of 27 children (median age: 4 years; 16 girls) who underwent DMSA SPECT were retrospectively analyzed. Both planar and SPECT DMSA were performed. SPECT images were analyzed using coronal-simulated planar two-dimensional images. A reduction in SPECT acquisition time was simulated to provide 4 series (SPECT-15 min, SPECT-10 min, SPECT-5 min and SPECT-2.5 min). A direct comparison of the planar and SPECT series was performed, including semi-quantification reproducibility, image quality (mean quality score on a scale of 0 to 2) and inter- and intra-observer reproducibility of the scintigraphic patterns. RESULTS: The overall image quality score (± standard deviation) was 1.3 (± 0.6) for the planar data set, 1.6 (± 0.5) for the SPECT-15 min data set, 1.4 (± 0.5) for the SPECT-10 min data set, 1.0 (± 0.5) for the SPECT-5 min data set and 0.6 (± 0.6) for the SPECT-2.5 min data set. Median Kappa coefficients for inter-observer agreement between planar and SPECT images were greater than 0.83 for all series and all readers except one reader for the SPECT-2.5 min series (median Kappa coefficient = 0.77). CONCLUSION: Shortening SPECT acquisitions to 5 min is feasible with minimal impact on images in terms of quality and reproducibility.

Hypospadias preputial flap onlay technique - Step by step.

PURPOSE: To present one way of performing onlay preputial flap for hypospadias. PATIENTS AND METHODS: This procedure was performed following the methodology used in one hypospadias expert center to correct hypospadias in boys who are not elective for Koff procedure and in whom Koyanagi procedure is not needed. Operative details were described, and post-operative management were given as example. RESULTS: Long-term results of this technique showed a 10% complication rate (dehiscence, strictures or urethral fistulas) 2 years after surgery. CONCLUSIONS: This video is a step by step description of the onlay preputial flap technique giving the general methodology and also the details resulting from years of practice in one hypospadias expert center.

Early detection of ureteropelvic junction obstruction in neonates with prenatal diagnosis of renal pelvis dilatation using 1H NMR urinary metabolomics.

Renal pelvis dilatation (RPD) is diagnosed in utero on prenatal ultrasonography (US) and can resolve spontaneously. However, isolated RPD can also reflect ureteropelvic junction obstruction (UPJO), which requires surgical treatment to prevent progressive renal deterioration. The diagnosis of UPJO can only be confirmed after birth with repeat US and renal isotope studies. 1H Nuclear Magnetic Resonance spectroscopy (NMR) was performed on urine of newborns with prenatally diagnosed unilateral RPD and healthy controls to identify specific urinary biomarkers for UPJO. The original combination of EigenMS normalization and sparse partial-least-squares discriminant analysis improved selectivity and sensitivity. In total, 140 urine samples from newborns were processed and 100 metabolites were identified. Correlation network identified discriminant metabolites in lower concentrations in UPJO patients. Two main metabolic pathways appeared to be impaired in patients with UPJO i.e. amino acid and betaine metabolism. In this prospective study, metabolic profiling of urine samples by NMR clearly distinguishes patients who required surgery for UPJO from patients with transient dilatations and controls. This study will pave the way for the use of metabolomics for the diagnosis of prenatal hydronephrosis in clinical routine.

Rituximab as induction therapy in pediatric kidney transplantation: A single-center experience in four patients.

BACKGROUND: The anti-CD20 rituximab is often used in the treatment of children with steroid-resistant nephrotic syndrome or EBV-induced post-transplant lymphoproliferative disorder. This single-center series reports the use of rituximab as induction therapy in pediatric kidney transplantation. METHODS: Four children who received rituximab as induction therapy for kidney transplantation since 2016 were retrospectively analyzed. Clinical and laboratory data were extracted from medical records. RESULTS: The patients (2 boys and 2 girls) were aged from 6.1 to 11.9 years and were treated with rituximab on the day of the transplantation procedure; all the transplants came from deceased donors. In all patients, rituximab was used because of positive EBV viral loads before kidney transplantation. Viral loads remained undetectable for the first 6 months after the transplantation procedure and remained below the 4.5 log threshold thereafter. After a median follow-up of 2.3 years, none of the patients displayed rejection or de novo donor-specific antibodies; the glomerular filtration rate remained above 70 ml/min/1.73 m2 . No post-transplant lymphoproliferative disorder was observed. CONCLUSION: The results suggest that rituximab can be used as induction therapy to prevent EBV replication and its complications in case of positive viral load prior to kidney transplantation.

Composition of urinary stones in children: clinical and metabolic determinants in a French tertiary care center.

As the epidemiology of urolithiasis is constantly evolving, analyzing the composition of stones is crucial to better understand the determinants of lithogenesis. The aim of this study was to describe the composition of stones of pediatric patients in a tertiary center. Clinical and metabolic data from all pediatric patients with at least one stone that was analyzed by Fourier transformed infrared spectroscopy (FTIR) in the Hospices Civils de Lyon between 2013 and 2017 were retrospectively collected. A total of 111 patients (sex ratio 1.4:1) were included; their median ([IQR]) age was 7.5 (3.1-10.5) years. The main component of stones was calcium oxalate (weddellite for 34 (31%) stones, whewellite 23 (21%)), calcium phosphate (carbapatite 32 (29%), brushite 6 (5%), amorphous calcium phosphate 3 (3%)), struvite 5 (5%), cystine 4 (4%), uric acid 2 (2%), and ammonium acid urate 2 (2%). A total of 20 (18%) stones were pure and 24 (22%) were infectious. Carbapatite stones were the most frequent in patients < 2 years and calcium oxalate stones in patients > 2 years old. Metabolic abnormalities (most frequently hypercalciuria) were found in 50% of tested patients and in 54% of patients with infectious stones. Congenital anomalies of the kidney and/or urinary tract (CAKUT) or neurogenic bladder were present in 9/24 (38%) patients with infectious stones and 12/16 (76%) patients with bladder stones.Conclusion: This study confirms that calcium oxalate stones are the most frequent among pediatric patients, which could reflect the nutritional habits of predisposed patients. In contrast, infectious stones are less frequent and occur mostly in association with anatomic or metabolic favoring factors.

Tumoral and pseusotumoral processes of the vagina in the pediatric population: A 26-YEAR retrospective study.

BACKGROUND: Vaginal lesions are rare and of various types in children. Clinical presentation is generally undifferenciated. Histological examination is fundamental to ascertain the nature of the lesion. Regarding tumoral lesions, histological subtypes encountered are radically different from those seen in adults, dominated by stromal benign lesions. OBJECTIVE: The aim of this retrospective study was to describe characteristics and pathological aspects of pediatric vaginal lesions, diagnosed in a single pediatric experienced center. STUDY DESIGN: A database analysis was performed on all vaginal samples of patients under 18 years old received in a pediatric-specialized pathology laboratory of an academic hospital, over a 26-year period. RESULTS: Among 36 vaginal tissue samples reported, a total of 15 tumoral or pseudotumoral processes was recorded. Primitive malignant tumors included embryonal rhabdomyosarcoma (n = 3) and germ-cell tumors, yolk-sac type (n = 2). Benign tumoral or pseudotumoral processes included inflammatory stromal polyps (n = 8), epidermic cyst (n = 1), and benign Müllerian papilloma (n = 1). DISCUSSION: Over 15 primitive vaginal tumors, 1/3 was malignant with embryonal rhabdomyosarcoma being the most common. The remaining 2/3 specimens were benign, with stromal inflammatory lesions being the most commonly observed. Fibro-epithelial polyp is a debated entity, which covers a wide histological spectrum, with varying inflammation and stromal cellularity, raising sometimes the question of the differential diagnosis with rhabdomyosarcoma. Stromal cells morphology along with their immunohistochemical profile suggest their reactive myofibroblastic nature. Pseudotumoral inflammatory lesions display very similar histological findings with these entities. A common pathogenesis beginning with an inflammatory process, potentially accelerated by chronic traumatic factors, could be discussed. CONCLUSION: We confirmed the rarity and the diversity of vaginal lesions in children. Vaginoscopy and biopsy sample should be systematic, given the non-specific presentation of tumoral processes. Myogenin immunostain must be systematic in case of vaginal polypoid mass, in order to rule out malignancy.

Low level myelomeningoceles: do they need prenatal surgery?

BACKGROUND: Postnatal closure of a myelomeningocele remains the standard of care in many countries. The prenatal closure has given hope for decreasing the damage to the neural placode and has challenged classic management. However, this technique presents potential sources of complications. Patients with MMC with an anatomical level of L4 and below have a better functional prognosis than higher level malformations. Are they still candidates for prenatal surgery? OBJECTIVE: To evaluate outcome of MMC with an anatomical level of L4 and below and discuss, with support of the literature, the indications to perform prenatal closure in this particular group of patients. MATERIALS AND METHODS: Twenty-nine children were included in this observational study. The level of the vertebral malformation was sacral in 12 cases (41.4%) or lumbar (level ≤ L4) in 17 cases (58.6%). All the patients was operated postnatally for closure of the MMC with microsurgical technique as soon as possible after clinical evaluation (range 0-97 days). RESULTS: Only 11 out of 29 patients (37.9%) needed of a CSF diversion. A Chiari II malformation was present before MMC closure in 17 patients (58.6%) and only in 5 (17%) after. Twenty-six patients (89.7%) were able to walk. Seven (23%) and 16 (55%) of our patients have a normal bladder and bowel control, respectively. All school-aged children attend school. CONCLUSIONS: The functional outcome for low-level MMC is good when managed with modern microneurosurgical techniques with a low risk for the patient and the mother. Therefore, we do not suggest prenatal surgery for subgroup of infant with MM.

Prognostic Value of Ultrasound Grading Systems in Prenatally Diagnosed Unilateral Urinary Tract Dilatation.

PURPOSE: We compared the prognostic value of anteroposterior intrasinus diameter of the renal pelvis, urinary tract dilatation and the Society for Fetal Urology grading system in children with prenatally diagnosed unilateral urinary tract dilatation. MATERIALS AND METHODS: All newborns with prenatally diagnosed unilateral urinary tract dilatation, normal bladder and anteroposterior intrasinus diameter 10 mm or greater on the first postnatal ultrasonography were prospectively enrolled from January 2011 to February 2015. Indications for surgery were recurrent febrile urinary tract infections and/or decrease of relative renal function more than 10% on serial isotope studies and/or increasing anteroposterior intrasinus diameter greater than 20% on serial ultrasounds. Sensitivity, specificity and ROC curves were calculated to evaluate the accuracy of anteroposterior intrasinus diameter, urinary tract dilatation and Society for Fetal Urology grading system in determining which children would need surgery within 24 months. RESULTS: A total of 57 males and 13 females were included. Of the patients 33 required surgery at a median age of 5 months (IQR 3.8 to 6.4). Urinary tract dilatation remained stable in 14 cases and decreased in 23 with a median followup of 42 months (IQR 25 to 67). Anteroposterior intrasinus diameter, urinary tract dilatation and Society for Fetal Urology scores were all correlated with the need for surgery. Anteroposterior intrasinus diameter with a threshold of 20 mm had the best prognostic value, with a sensitivity of 81.8% and a specificity of 91.7%. CONCLUSIONS: Our study confirms that the prognostic value was comparable between anteroposterior intrasinus diameter of the renal pelvis, urinary tract dilatation and Society for Fetal Urology grading system in newborns with prenatally diagnosed unilateral urinary tract dilatation. Anteroposterior intrasinus diameter and abnormal parenchymal thickness are the most important ultrasound criteria to identify children at risk for requiring surgery.

Concealed epispadias associated with a buried penis.

OBJECTIVE: The aim was to describe the clinical presentation and the surgical management of penile epispadias associated with a buried penis in five children. PATIENTS AND METHODS: This is a 5-year retrospective review of patients presenting with a buried penis, a congenital defect of the penile skin shaft associated with an unretractable foreskin for whom a penile epispadias was found at the time of surgery. All had undergone surgery combining a Cantwell-Ransley procedure and refashioning of the penile skin following the authors' technique. RESULTS: Three children had a glanular epispadias and two had a midshaft epispadias. Four had a satisfactory outcome, and one required a complementary urethroplasty for glanular dehiscence. CONCLUSION: Buried penis and epispadias are usually isolated congenital anomalies, although they can be associated. It is therefore recommended to warn parents about the possibility of underlying penile anomaly in children with buried penises and unretractable foreskin. Careful palpation of the dorsum of the glans through the foreskin looking for a dorsal cleft could indicate an associated epispadiac urethra. Surgical correction of both anomalies can be done at the same time. Parents of boys with buried penises should be warned that underlying penile anomaly may exist.

The consequences of pediatric renal transplantation on bone metabolism and growth.

PURPOSE OF REVIEW: During childhood, growth retardation, decreased final height and renal osteodystrophy are common complications of chronic kidney disease (CKD). These problems remain present in patients undergoing renal transplantation, even though steroid-sparing strategies are more widely used. In this context, achieving normal height and growth in children after transplantation is a crucial issue for both quality of life and self-esteem. The aim of this review is to provide an overview of pathophysiology of CKD-mineral bone disorder (MBD) in children undergoing renal transplantation and to propose keypoints for its daily management. RECENT FINDINGS: In adults, calcimimetics are effective for posttransplant hyperparathyroidism, but data are missing in the pediatric population. Fibroblast growth factor 23 levels are associated with increased risk of rejection, but the underlying mechanisms remain unclear. A recent meta-analysis also demonstrated the effectiveness of rhGH therapy in short transplanted children. SUMMARY: In 2013, the daily clinical management of CKD-MBD in transplanted children should still focus on simple objectives: to optimize renal function, to develop and promote steroid-sparing strategies, to provide optimal nutritional support to maximize final height and avoid bone deformations, to equilibrate calcium/phosphate metabolism so as to provide acceptable bone quality and cardiovascular status, to correct all metabolic and clinical abnormalities that can worsen both bone and growth (mainly metabolic acidosis, anemia and malnutrition), promote good lifestyle habits (adequate calcium intake, regular physical activity, no sodas consumption, no tobacco exposure) and eventually to correct native vitamin D deficiency (target of 25-vitamin D >75 nmol/l).

Long-term critical issues in pediatric renal transplant recipients: a single-center experience.

Data on long-term outcomes after pediatric renal transplantation (Tx) are still limited. We report on a 20-year single-center experience. Medical charts of all consecutive pediatric Tx performed between 1987 and 2007 were reviewed. Data of patients who had been transferred to adult units were extracted from the French databases of renal replacement therapies. Outcomes were assessed using Kaplan-Meier and Cox models. Two hundred forty Tx were performed in 219 children (24.1% pre-emptive and 17.5% living related donor Tx). Median age at Tx was 11.1 years and median follow-up was 10.4 years. Patient survival was 94%, 92%, and 91% at 5, 10, and 15 years post-Tx, respectively. Overall, transplant survival was 92%, 82%, 72%, and 59% at 1, 5, 10, and 15 years post-Tx, respectively. The expected death-censored graft half-life was 20 years. Sixteen patients developed malignancies during follow-up. Median height at 18 years of age was 166 cm in boys and 152 cm in girls with 68% of patients being in the normal range. The proportion of socially disadvantaged young people was higher than in general population. Excellent long-term outcomes can be achieved in pediatric renal Tx, but specific problems such as malignancies, growth, and social outcome remain challenging.

[Kidney transplantation in childhood: from milimeter to centimeters]. / Transplantation rénale chez l'enfant : du millimètre aux centimètres.

Graft survival is worse in recipient aged less than 5 years due to the greater risk of vascular thrombosis. Thrombosis may be prevented by the choice of the donor, method of surgery, perioperative hemodynamic optimisation and preventive anti-coagulation. Normal growth is a major objective of the management of transplanted children. The mean final height increased during the 20 last years to be between -1.63 and -0.92 SDS depending on age and period of the transplantation.

Does androgen stimulation prior to hypospadias surgery increase the rate of healing complications? - A preliminary report.

OBJECTIVE: Androgens have a positive effect on penile growth in children, but they may also have a repressive effect on the healing process. The aim of this prospective study was to compare the outcomes of onlay urethroplasty with and without preoperative androgen stimulation in patients with severe hypospadias. PATIENTS AND METHOD: Of 300 severe hypospadias cases treated at a single institution, 126 operated on by the same surgeon had complete follow-up data, and 30 of these received preoperative androgen treatment (human chorionic gonadotrophin and/or systemic testosterone) 1-24 months before surgery. RESULTS: Thirty-five patients presented with a complication (27.7%) of whom 26 (20.6%) had a fistula or dehiscence. Among patients on androgen stimulation there was a 30% healing complication rate (9/30) whereas for those without this was 17.7% (17/96). When androgenic treatment was given > 3 months prior to surgery the healing complication rate was 21.7% (5/23), and when < 3 months prior to surgery the rate reached 57% (4/7). Mean follow up was 41 months (10-97). CONCLUSION: Although the numbers were too small in this series to reach statistical significance, the tissular interactions of androgens in the healing process reported by dermatologists should alert the hypospadiologists and lead to a further prospective study to define the optimal protocol for stimulation of the penis in specific cases without affecting outcome.

Outcome of severe hypospadias repair using three different techniques.

OBJECTIVE: To compare the outcomes of three different urethroplasty techniques (onlay, buccal mucosa, Koyanagi type I) used in the reconstruction of severe hypospadias. PATIENTS AND METHODS: Over 10 years (1997-2007), 300 severe hypospadias cases were treated with a mean follow up of 2 years (1-105 months); 203 were operated by the same surgeon of whom 184 completed follow up. Three main techniques were used according to the quality of the urethral plate: onlay urethroplasty (133), buccal graft urethroplasty (25) and Koyanagi type I (26). The mean age at surgery was 36 months (8-298); 76 required preoperative androgen stimulation (onlay 37, buccal 11, Koyanagi 26); 18 required a corporoplasty to straighten the penis (onlay 13, buccal 3, Koyanagi 2). RESULTS: Thirty-eight onlay (28.5%); 14 buccal (56%); 16 Koyanagi (61.5%) urethroplasties had a complication. The fistula rate was 15% for the onlay group; 32% for the buccal mucosa group; 19.2% for the Koyanagi cases. The dehiscence rate was, respectively, 11.3%, 20% and 42.3%. The stricture rate was, respectively, 1.5%, 20% and 34.6%. Urethrocele was found in seven Koyanagi patients. Final functional and cosmetic results were satisfactory in 126/133 (94.7%) onlay, 20/25 (80%) buccal and 14/26 Koyanagi (53.8%) urethroplasties. Primary cases had better results (89%) than redo cases (75.9%). Patients submitted to preoperative androgen therapy developed more complications (onlay: 40.5% vs 23.9%; buccal: 70% vs 43.7%). CONCLUSION: Two striking results are the low number of severe hypospadias cases requiring an additional corporoplasty, and the increased complication rate found in androgen-stimulated patients. The excellent results of the onlay procedure could be related to the use of dorsal preputial tissue, which in hypospadias is characterized by a well-balanced protein platform compared to the ventral tissues.

Vesicoureteral reflux: why we can't agree on its management! An evidence based approach.

Vesicoureteral reflux remains one of the most controversial subjects in paediatric urology. The flooding of publications on reflux makes the understanding of this anomaly and its treatments quite opaque. Evidence Based Medicine might be a helpful tool to clarify the various approaches of reflux reflected in 6.715 publications found on Medline with the key-words "vesicoureteral reflux" and "vesicoureteric reflux". These articles were critically reviewed and graded according to EBM scorings, with regard to their methodological designs. It appears clearly after this review of literature concerning VUR that most of our beliefs are based on low evidence publications and that EBM has not sufficient arguments to establish recommendations for diagnostic and treatment of VUR. It appears yet that antenatal dilatation of the urinary tract and symptomatic UTIs justify looking for VUR. Surgery should be discussed in recurrent UTIs or deterioration of renal function. There is no consensus in the case of persistent asymptomatic VUR, indication and duration of antibioprophylaxis, and choice of radical treatment.

Management of severe hypospadias.

Many classifications of hypospadias have been published, mainly based on the position of the ectopic meatus, which is an insufficient criterion to define the severity of this malformation. What really marks the proximal landmark of this malformation is the level of division of the corpus spongiosum, which is always proximal to the ectopic meatus. In this article, we will focus on the most severe forms of hypospadias which include those with a proximal division of corpus spongiosum (below the midshaft), important chordee and a poor development of the ventral radius, reflecting a marked hypovirilization of the genital tubercle, and cripple hypospadias resulting from several previous failed surgical procedures. The principle of hypospadias surgery will be reviewed together with the outcome of the current surgical techniques. Furthermore, common complications will be outlined. There is no minor or major hypospadias and all forms require a solid experience of the surgeon, as minor looking hypospadias may turn out to be far more complex to repair than they appear once the ventral radius of the penis has been dissected.