Publications by authors named "Pierre Mouriquand"

73 Publications

Preoperative Topical Estrogen Treatment vs Placebo in 244 Children With Midshaft and Posterior Hypospadias.

J Clin Endocrinol Metab 2020 07;105(7)

Centre de Référence Maladies Rares Développement Génital: du Fœtus à l'Adulte, Hospices Civils de Lyon, Bron, France.

Purpose: Urethral fistula and dehiscence are common after hypospadias surgery. Preoperative androgens have been considered to reduce these complications although this consideration is not evidence-based. Dermatologists have reported the benefits of topical estrogens on skin healing. We investigated whether the preoperative use of topical promestriene could reduce healing complications in hypospadias surgery. Our primary objective was to demonstrate a reduction of healing complications with promestriene vs placebo. Impact on reoperations and other complications, clinical tolerance, bone growth, and biological systemic effects of the treatment were also considered.

Methods: We conducted a prospective, randomized, placebo-controlled, double-blind, parallel group trial between 2011 and 2015 in 4 French centers. One-stage transverse preputial island flap urethroplasty (onlay urethroplasty) was selected for severe hypospadias. Promestriene or placebo was applied on the penis for 2 months prior to surgery. The primary outcome was the presence of postoperative urethral fistula or dehiscence in the first year postsurgery. For safety reasons, hormonal and anatomical screenings were performed.

Results: Out of 241 patients who received surgery, 122 patients were randomized to receive placebo, and 119 patients received promestriene. The primary outcome was unavailable for 11 patients. Healing complications were assessed at 16.4% (19/116) in the placebo vs 14.9% (17/114) in the promestriene arm, and the odds ratio adjusted on center was 0.93 (95% confidence interval 0.45-1.94), P = 0.86.

Conclusions And Relevance: Although we observed an overall lower risk of complications compared to previous publications, postsurgery complications were not different between promestriene and placebo, because of a lack of power of the study or the inefficacy of promestriene.
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http://dx.doi.org/10.1210/clinem/dgaa231DOI Listing
July 2020

[Atypical genital development and tumor risk].

Bull Cancer 2019 May 23;106(5):461-467. Epub 2019 Mar 23.

Groupement hospitalier Est, hospices Civils de Lyon, institut Multisite de pathologie, 69500 Bron, France.

Atypical genital development (AGD), also called disorders of sex development are a set of miscellaneous pathologies who have in common a morphological and/or functional abnormality of the internal and/or external genital organs. The Chicago classification identifies 3 major groups based on karyotype, hormone balance and genetic studies. Some AGD predispose to the occurrence of tumors, mainly malignant germ cell tumors. The tumor risk depends on many factors: the type of AGD, the position of the gonad, the age of the patient, the phenotype, the function of the gonad and the presence of germ cells in the gonad. AGD with the highest tumor risk are those with gonadal dysgenesis, implying an incomplete differentiation of the bipotential gonad (dysplasia). Monitoring of patients with AGD and indication of prophylactic gonadectomies should be individualized according to tumor risk.
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http://dx.doi.org/10.1016/j.bulcan.2019.01.018DOI Listing
May 2019

Surgery of Anomalies of Gonadal and Genital Development in the "Post-Truth Era".

Urol Clin North Am 2018 Nov 7;45(4):659-669. Epub 2018 Sep 7.

Department of Paediatric Urology, Université Claude-Bernard, Hospices Civils de Lyon, Lyon, France; Service d'Urologie Pédiatrique, Hôpital Mère-Enfant, 59, Boulevard Pinel, Bron 69500, France. Electronic address:

This article aims to examine the current issues of debate concerning the management of atypical gonadal and genital development (AGD). Understanding this complex subject begins with defining the distinct AGD conditions, the aims and nature of surgical treatments, and the perceptions of affected individuals and their families. The evolving societal and medical contexts in this field are confronting facts and opinions, leading to a significant change in attitudes and approaches.
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http://dx.doi.org/10.1016/j.ucl.2018.06.012DOI Listing
November 2018

Male Sexuality, Fertility, and Urinary Continence in Bladder Exstrophy-Epispadias Complex.

J Sex Med 2018 Mar;15(3):314-323

Service d'urologie, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Université Claude-Bernard-Lyon 1-France.

Background: The bladder exstrophy-epispadias complex is a rare congenital malformation associated with severe dysfunction of the genital and urinary tracts and requiring a staged surgical reconstruction.

Aim: The primary aims of this study were to report the sexuality, infertility, and urinary incontinence outcomes in a cohort of men born with bladder exstrophy-epispadias complex. The secondary aim was to highlight some predictive factors of infertility in this population.

Methods: We conducted a descriptive, cross-sectional study of men diagnosed with classic presentations of bladder exstrophy or epispadias.

Outcomes: Patients were asked to complete 4 validated questionnaires: the International Index of Erectile Function (IIEF)-5, the Erection Hardness Score (EHS), the Self-Esteem and Relationship, and the International Consultation Incontinence modular Questionnaire-Short Form. Fertility potential was assessed with semen analysis and a non-validated questionnaire.

Results: 38 Patients 18-64 years old (M [mean] = 32.2) completed the questionnaires. The average IIEF-5 score was 18.1/25 (ranging from 3-25; SE = 7.62), with results indicating that 55% of the sample had normal erectile function. Results also showed higher scores for patients with normal spermatozoa concentration (M = 22.75, SE = 1.89, P = .08) than for those with oligospermia (M = 17.30, SE = 8.53, P = .08). Results on the IIEF-5 also indicated higher scores for patients who conceived children without assisted reproductive technologies (ART) (M = 22.83, SE = 2.317, P = .02) than for patients without children (M = 15.76, SE = 8.342, P = .02). The average EHS was 3.43/4 (ranging from 1-4, SE = 0.9). EHS was higher for patients who had reconstruction than for patients who had cystectomy (M = 3.88, SE = 1.07 and 2.78, SE = 1.09, P = .02). The average total Self-Esteem and Relationship score was 67.04/100 (ranging from 10.71-96.43, SE = 22.11). The average total International Consultation Incontinence modular Questionnaire-Short Form score was 4.97/21 (ranging from 0-18, SE = 5.44), higher score indicating more urinary incontinence. Among the patients surveyed, 31.6% were parents at the time of study and 50% of them benefited from ART. With regards to the 14 semen analyses performed, only 7.1% produced normal results and 44.7% indicated that ejaculation was weak and dribbling.

Clinical Translation: Erectile function appears to be decreased and psychological aspects of sexuality indicate low self-esteem about sexual relationship. Although ethical problems could not allow prospective spermograms, our cohort is large enough to provide significant data.

Conclusions: Early sperm storage for future ART, sexual medicine management, and complementary genital reconstruction in adulthood constitute potential treatment options for this population. Reynaud N, Courtois F, Mouriquand P, et al. Male Sexuality, Fertility, and Urinary Continence in Bladder Exstrophy-Epispadias Complex. J Sex Med 2017;15:314-323.
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http://dx.doi.org/10.1016/j.jsxm.2018.01.004DOI Listing
March 2018

Standardization of pediatric uroradiological terms: A multidisciplinary European glossary.

J Pediatr Urol 2017 Dec 13;13(6):641-650. Epub 2017 Jul 13.

Pediatric Radiology, University Hospital LKH Graz, Graz, Austria.

To promote the standardization of nephro-uroradiological terms used in children, the European Society of Pediatric Radiology uroradiology taskforce wrote a detailed glossary. This work has been subsequently submitted to European experts in pediatric urology and nephrology for discussion and acceptance to improve the quality of radiological reports and communication among different clinicians involved in pediatric urology and nephrology.
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http://dx.doi.org/10.1016/j.jpurol.2017.05.026DOI Listing
December 2017

Standardization of pediatric uroradiological terms: a multidisciplinary European glossary.

Pediatr Radiol 2018 02 15;48(2):291-303. Epub 2017 Nov 15.

Pediatric Radiology, University Hospital LKH Graz, Graz, Austria.

To promote the standardization of nephro-uroradiological terms used in children, the European Society of Paediatric Radiology uroradiology taskforce wrote a detailed glossary. This work has been subsequently submitted to European experts in pediatric urology and nephrology for discussion and acceptance to improve the quality of radiological reports and communication between different clinicians involved in pediatric urology and nephrology.
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http://dx.doi.org/10.1007/s00247-017-4006-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5790858PMC
February 2018

Evolution of Newborns' Urinary Metabolomic Profiles According to Age and Growth.

J Proteome Res 2017 10 25;16(10):3732-3740. Epub 2017 Aug 25.

Service de chirurgie pédiatrique, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, Université Claude Bernard Lyon 1 , F-69677 Bron, France.

Improving the management of neonatal diseases and prevention of chronic diseases in adulthood requires a better comprehension of the complex maturational processes associated with newborns' development. Urine-based metabolomic studies play a promising role in the fields of pediatrics and neonatology, relying on simple and noninvasive collection procedures while integrating a variety of factors such as genotype, nutritional state, lifestyle, and diseases. Here, we investigate the influence of age, weight, height, and gender on the urine metabolome during the first 4 months of life. Untargeted analysis of urine was carried out by H-Nuclear Magnetic Resonance (NMR) spectroscopy for 90 newborns under 4 months of age, and free of metabolic, nephrologic, or urologic diseases. Supervised multivariate statistical analysis of the metabolic profiles revealed metabolites significantly associated with age, weight, and height, respectively. The tremendous growth occurring during the neonatal period is associated with specific modifications of newborns' metabolism. Conversely, gender appears to have no impact on the urine metabolome during early infancy. These results allow a deeper understanding of newborns' metabolic maturation and underline potential confounding factors in newborns' metabolomics studies. We emphasize the need to systematically and precisely report children age, height, and weight that impact urine metabolic profiles of infants.
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http://dx.doi.org/10.1021/acs.jproteome.7b00421DOI Listing
October 2017

Commentary to "Attitudes towards 'disorders of sex development' nomenclature among affected".

J Pediatr Urol 2017 12 19;13(6):609. Epub 2017 Jun 19.

Hôpital Mère-Enfant - Hospices Civils de Lyon Université Claude-Bernard Bron, France. Electronic address:

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http://dx.doi.org/10.1016/j.jpurol.2017.03.041DOI Listing
December 2017

Commentary to "Prospective assessment of cosmesis before and after genital surgery".

J Pediatr Urol 2017 02 23;13(1):29. Epub 2016 Nov 23.

Hôpital Mère-Enfant, Hospices Civils de Lyon, Université Claude-Bernard, Service d'Urologie Pédiatrique, 59 boulevard Pinel, Bron Cedex 69500, France. Electronic address:

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http://dx.doi.org/10.1016/j.jpurol.2016.09.018DOI Listing
February 2017

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

J Urol 2017 04 1;197(4):1144-1149. Epub 2016 Dec 1.

Pediatric Surgery Department, Femme Mère Enfant Hospital-University Hospital of Lyon and University Claude Bernard Lyon 1, Lyon, France.

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.
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http://dx.doi.org/10.1016/j.juro.2016.11.103DOI Listing
April 2017

A novel morphological approach to gonads in disorders of sex development.

Mod Pathol 2016 11 29;29(11):1399-1414. Epub 2016 Jul 29.

Service d'Anatomie Pathologique, Centre de Biologie et de Pathologie Est, Bron, France.

Disorders of sex development are defined as congenital conditions with discordance between the phenotype, the genotype, the karyotype, and the hormonal profile. The disorders of sex development consensus classification established in 2005 are mainly based on chromosomal and biological data. However, histological anomalies are not considered. The aims of this study were to define the specific pathological features of gonads in various groups of disorders of sex development in order to clarify the nosology of histological findings and to evaluate the tumor risk in case of a conservative approach. One hundred and seventy-five samples from 86 patients with disorders of sex development were analyzed following a strict histological reading protocol. The term 'gonadal dysgenesis' for the histological analysis was found confusing and therefore excluded. The concept of 'dysplasia' was subsequently introduced in order to describe the architectural disorganization of the gonad (various degrees of irregular seminiferous tubules, thin albuginea, fibrous interstitium). Five histological types were identified: normal gonad, hypoplastic testis, dysplastic testis, streak gonad, and ovotestis. The analysis showed an association between undifferentiated gonadal tissue, a potential precursor of gonadoblastoma, and dysplasia. Dysplasia and undifferentiated gonadal tissue were only encountered in cases of genetic or chromosomal abnormality ('dysgenesis' groups in the disorders of sex development consensus classification). 'Dysgenetic testes', related to an embryonic malformation of the gonad, have variable histological presentations, from normal to streak. Conversely, gonads associated with hormonal deficiencies always display a normal architecture. A loss of expression of AMH and α-inhibin was identified in dysplastic areas. Foci of abnormal expression of the CD117 and OCT4 immature germ cells markers in dysplasia and undifferentiated gonadal tissue were associated with an increased risk of neoplasia. This morphological analysis aims at clarifying the histological classification and gives an indication of tumor risk of gonads in disorders of sex development.
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http://dx.doi.org/10.1038/modpathol.2016.123DOI Listing
November 2016

Response to 'Re. Surgery in disorders of sex development (DSD) with a gender issue: If (why), when, and how?'

J Pediatr Urol 2016 Dec 26;12(6):441. Epub 2016 Jul 26.

Hôpital Mère-Enfant, Hospices Civils de Lyon, Université Claude-Bernard, Service d'Urologie Pédiatrique, Bron, France. Electronic address:

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http://dx.doi.org/10.1016/j.jpurol.2016.05.043DOI Listing
December 2016

Concealed epispadias associated with a buried penis.

J Pediatr Urol 2016 Dec 18;12(6):347-351. Epub 2016 Sep 18.

Department of Paediatric Urology, Hôpital Mère-Enfant, Université Claude-Bernard, Bron, France. Electronic address:

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.
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http://dx.doi.org/10.1016/j.jpurol.2016.07.016DOI Listing
December 2016

Response to letter to the Editor re 'Surgery in Disorders of Sex Development (DSD) with a gender issue: If (why), when and how?'

J Pediatr Urol 2016 Dec 24;12(6):444. Epub 2016 Aug 24.

Hasbro Children's Hospital, Department of Urology, 30 Devon Court, Suite 174, East Greenwich, RI 02818, USA.

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http://dx.doi.org/10.1016/j.jpurol.2016.07.009DOI Listing
December 2016

Response to "Re. Surgery in Disorders of Sex Development (DSD) with a gender issue: If (why), when and how?"

J Pediatr Urol 2016 Dec 21;12(6):438. Epub 2016 Jul 21.

Hasbro Children's Hospital, Urology, 30 Devon Court, Suite 174, East Greenwich, RI 02818, United States.

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http://dx.doi.org/10.1016/j.jpurol.2016.05.040DOI Listing
December 2016

Spontaneous bladder rupture in non-augmented bladder exstrophy.

J Pediatr Urol 2016 Dec 16;12(6):400.e1-400.e5. Epub 2016 Jul 16.

Department of Paediatric Urology, Royal Children's Hospital, Flemington Road, Parkville, VIC, Australia.

Objective: Bladder perforation is not commonly described in bladder exstrophy patients without bladder augmentation. The goal of this study was to identify the risk factors of spontaneous perforation in non-augmented exstrophy bladders.

Methods: The study was a retrospective multi-institutional review of bladder perforation in seven male and two female patients with classic bladder exstrophy-epispadias (E-E).

Results: Correction of E-E was performed using Kelly repair in two and staged repair in seven (Table). Bladder neck repair was performed in eight patients at a mean age of 6 years. Three patients had additional urethral surgery. Before rupture, six patients were voiding only per urethra. Two patients were voiding urethrally but were also performing occasional CIC via a Mitrofanoff. One patient was performing CIC 3 hourly per urethra. Six were dry during the day. Six of the patients had lower urinary tract symptoms: five had frequency and four were straining to void. Two had suffered episodes of urinary retention. Pre-rupture ultrasound showed that the upper urinary tract was dilated in four patients. Micturating cystourethrogram was performed in six showing vesico-ureteral reflux in five. Two had urethral stenosis. Nuclear medicine was done in three patients with two abnormal differential function. Urodynamics was performed in two patients with low capacity (100 mL) and hypocompliant (<10) bladders. Both had high leak point pressures: 60 cmHO at 100 mL. The mean age at rupture was 11 years, with a range of 5-20 years. Patients presented with abdominal pain, associated with signs of intestinal obstruction in seven and fever in two. Eight patients underwent laparotomy and one prolonged drainage via SPC. Simple closure was performed in seven and bladder neck closure in one, because of extension of the rupture inferiorly. All patients recovered well. Following rupture, five underwent augmentation and Mitrofanoff. One of these suffered a recurrent rupture. Two other patients refused augmentation and Mitrofanoff and one of these has since had a subsequent rupture.

Conclusions: The limitations of this series include the small number of patients and its retrospective nature, without knowledge of the incidence. Bladder rupture is a risk even in non-augmented bladder exstrophy. It is potentially life-threatening and most often requires laparotomy. Rupture occurs because of poor bladder emptying and/or high pressure. Urodynamics may identify those at risk. CIC with or without augmentation should not be delayed once poor bladder emptying and/or high pressure are identified.
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http://dx.doi.org/10.1016/j.jpurol.2016.04.054DOI Listing
December 2016

Commentary to "Biometry of the hypospadic penis after hormone therapy (testosterone and estrogen): A randomized double-blind controlled trial".

J Pediatr Urol 2016 08 16;12(4):201. Epub 2016 Jul 16.

Hôpital Mère-Enfant - Hospices Civils de Lyon-Université Claude-Bernard, Bron Cedex, France. Electronic address:

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http://dx.doi.org/10.1016/j.jpurol.2016.04.055DOI Listing
August 2016

What is the outcome of paediatric gastrocystoplasty when the patients reach adulthood?

BJU Int 2016 Dec 24;118(6):980-986. Epub 2016 Jul 24.

Aix-Marseille Université, Marseille, France.

Objective: To document the long-term outcomes of paediatric augmentation gastrocystoplasty (AGC) in terms of preservation of renal function and maintenance of dryness, and to analyse the rate of complications.

Patients And Methods: The medical records of children who had undergone AGC between 1992 and 2000 (minimum time interval of 15 years) were reviewed retrospectively. The following data were collected: age at surgery, the cause of bladder dysfunction, functioning of the AGC, any complications, and the long-term outcome of the patients. All of the patients were re-contacted by telephone.

Results: A total of 11 AGCs were carried out between 1992 and 2000, at a median (range) age of 11 (6.5-14) years. The diagnosis of patients undergoing AGC included myelomeningocele (four), bladder exstrophy (four), posterior urethral valves (one), irradiated bladder (one), and Prune Belly syndrome (one). The median [interquartile range (IQR)] follow-up was 17 (15-19.5) years. Renal function was preserved or improved in seven of the 11 patients and nine patients were dry after AGC. Seven of the 11 patients reported symptoms linked to haematuria-dysuria syndrome, which was resistant to treatment in one case and requiring excision of the gastric patch. Three of the 11 patients developed a tumour on the gastric graft after a median (range) delay of 20 (11-22) years after the initial procedure. All had gastric adenocarcinoma of which two were metastatic at the time of diagnosis requiring pelvectomy with pelvic lymph node dissection and adjuvant chemotherapy. Seven of the 11 patients underwent excision of the gastric patch after a median (IQR) time of 11 (8.5-20.5) years.

Conclusions: Our long-term data confirmed that most patients undergoing AGC had preservation of their renal function and were continent. However, long-term, AGC was associated with a significant risk of malignant transformation and a high rate of surgical re-intervention involving removal of the gastric patch. These results question the use of this technique for bladder augmentation, irrespective of the indication. We highlight the importance of strict endoscopic follow-up of all patients already having undergone an AGC and the need to inform and educated patients about tumour-related symptoms.
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http://dx.doi.org/10.1111/bju.13558DOI Listing
December 2016

Surgery in disorders of sex development (DSD) with a gender issue: If (why), when, and how?

J Pediatr Urol 2016 Jun 9;12(3):139-49. Epub 2016 Apr 9.

Penn State Hershey Pediatric Endocrinology, PA, USA.

Ten years after the consensus meeting on disorders of sex development (DSD), genital surgery continues to raise questions and criticisms concerning its indications, its technical aspects, timing and evaluation. This standpoint details each distinct situation and its possible management in 5 main groups of DSD patients with atypical genitalia: the 46,XX DSD group (congenital adrenal hyperplasia); the heterogeneous 46,XY DSD group (gonadal dysgenesis, disorders of steroidogenesis, target tissues impairments …); gonosomic mosaicisms (45,X/46,XY patients); ovo-testicular DSD; and "non-hormonal/non chromosomal" DSD. Questions are summarized for each DSD group with the support of literature and the feed-back of several world experts. Given the complexity and heterogeneity of presentation there is no consensus regarding the indications, the timing, the procedure nor the evaluation of outcome of DSD surgery. There are, however, some issues on which most experts would agree: 1) The need for identifying centres of expertise with a multidisciplinary approach; 2) A conservative management of the gonads in complete androgen insensitivity syndrome at least until puberty although some studies expressed concerns about the heightened tumour risk in this group; 3) To avoid vaginal dilatation in children after surgical reconstruction; 4) To keep asymptomatic mullerian remnants during childhood; 5) To remove confirmed streak gonads when Y material is present; 6) It is likely that 46,XY cloacal exstrophy, aphallia and severe micropenis would do best raised as male although this is based on limited outcome data. There is general acknowledgement among experts that timing, the choice of the individual and irreversibility of surgical procedures are sources of concerns. There is, however, little evidence provided regarding the impact of non-treated DSD during childhood for the individual development, the parents, society and the risk of stigmatization. The low level of evidence should lead to design collaborative prospective studies involving all parties and using consensual protocols of evaluation.
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http://dx.doi.org/10.1016/j.jpurol.2016.04.001DOI Listing
June 2016

Global Disorders of Sex Development Update since 2006: Perceptions, Approach and Care.

Horm Res Paediatr 2016 28;85(3):158-80. Epub 2016 Jan 28.

Department of Pediatrics, Penn State College of Medicine, Hershey, Pa., USA.

The goal of this update regarding the diagnosis and care of persons with disorders of sex development (DSDs) is to address changes in the clinical approach since the 2005 Consensus Conference, since knowledge and viewpoints change. An effort was made to include representatives from a broad perspective including support and advocacy groups. The goal of patient care is focused upon the best possible quality of life (QoL). The field of DSD is continuously developing. An update on the clinical evaluation of infants and older individuals with ambiguous genitalia including perceptions regarding male or female assignment is discussed. Topics include biochemical and genetic assessment, the risk of germ cell tumor development, approaches to psychosocial and psychosexual well-being and an update on support groups. Open and on-going communication with patients and parents must involve full disclosure, with the recognition that, while DSD conditions are life-long, enhancement of the best possible outcome improves QoL. The evolution of diagnosis and care continues, while it is still impossible to predict gender development in an individual case with certainty. Such decisions and decisions regarding surgery during infancy that alters external genital anatomy or removes germ cells continue to carry risk.
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http://dx.doi.org/10.1159/000442975DOI Listing
December 2016

Laparoscopic-assisted vaginal pull-through: A new approach for congenital adrenal hyperplasia patients with high urogenital sinus.

Afr J Paediatr Surg 2015 Jul-Sep;12(3):177-80

Department of Paediatric Surgery, University Claude Bernard Lyon 1, Hôpital Femme Mère Enfant, 69677 Bron Cedex, France.

Background: To open vaginal cavity to the pelvic floor is part of surgical treatment for urogenital sinus (UGS) in girls with congenital adrenal hyperplasia (CAH). For high UGS, this operative procedure can be challenging and may jeopardise urinary continence. Combined perineal and laparoscopic approaches could be useful to minimise perineal dissection and to facilitate the vaginal lowering.

Patients And Methods: We report the procedure of a laparoscopic-assisted vaginal pull-through for supra-sphincteric UGS in a 5-year-old girl with CAH. Laparoscopic dissection of the vagina from the posterior wall of the bladder and urethra, division of the confluence and vaginal pull-through to the perineum are described.

Discussion: The technique is derived from laparoscopic-assisted treatment for high ano-rectal malformations. Compared with current procedures for treatment for high UGS, laparoscopic-assisted approach allows mobilising vagina with minimal dissection of perineum and complete preservation of urethra. Another major advantage is to provide a direct vision for dissection of the space between rectum and urethra prior to vaginal pull-through.

Conclusion: Laparoscopic-assisted vaginal pull-through appears to be an interesting approach for high UGS in CAH patients, reducing dissection and risk of urinary incontinence. This new approach needs to be strengthened by other cases.
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http://dx.doi.org/10.4103/0189-6725.170191DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4955429PMC
December 2016

Late prenatal dexamethasone and phenotype variations in 46,XX CAH: concerns about current protocols and benefits for surgical procedures.

J Pediatr Urol 2014 Oct 15;10(5):941-7. Epub 2014 Mar 15.

Service d'Urologie Pédiatrique, Hôpital Mère-Enfant, Centre Hospitalo-Universitaire de Lyon, GHE, 59, boulevard Pinel, 69677 Bron Cedex, France. Electronic address:

Objective: To describe the action of prenatal dexamethasone (PreDex) on the anatomy of female congenital adrenal hyperplasia (CAH) genitalia when started at later stages of gestation.

Materials And Methods: Our group follows a large cohort of French CAH patients who underwent PreDex therapy, of whom 258 were recently reported. Four 46,XX patients with a delayed PreDex treatment presented with a virilized genitalia and required surgical reconstruction. This is a retrospective report on genital phenotyping at the time of surgery of these four patients who began PreDex therapy at 8, 12, 20, and 28 weeks of gestation.

Results: Although this series is limited in number, the anatomical description of the length of the genital tubercle, the height of the urethra-vaginal confluence, and the degree of fusion of the genital folds seems to be dependent upon the starting date of PreDex. Most PreDex treatments prescribed up to now have covered the full duration of gestation.

Conclusions: Our findings suggest that PreDex therapy could be limited to the period of the partitioning window. It is hoped that further prospective multicentric clinical studies will obtain ethical approval in order to elucidate the place and protocols of PreDex therapy in the management of CAH.
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http://dx.doi.org/10.1016/j.jpurol.2014.02.003DOI Listing
October 2014

Portal vein thrombosis after laparoscopic splenectomy during childhood.

J Thromb Thrombolysis 2014 ;38(2):218-22

Pediatric Surgery Department, Femme Mère Enfant Hospital - Hospices Civils de Lyon and Claude Bernard University Lyon 1, 59 Boulevard PINEL, 69500, Bron, France,

Portal vein thrombosis (PVT) is a rare but potentially life-threatening complication of laparoscopic splenectomy (LS) and can lead to bowel ischemia or portal hypertension. In childhood, this complication is reported in 5-10 % of the cases whereas it can be up to 50 % in adult population. Our aim was to evaluate PVT incidence after LS and associated risks factors. A retrospective chart review identified 37 children who underwent elective LS from 2005 to 2013. The main indications were spherocytosis or sickle cell disease. Median age and weight were respectively 7.4 years and 25.1 kg. Thromboembolic prophylaxis was not routinely given. Duration of surgery was 129 min and hospital length of stay 4 days. Doppler ultrasound scan (USS) was performed post-operatively in 26 cases. Post-operative course was uneventful in all but one patient. She was a 17 year-old girl previously operated for an ovarian tumor with hyperandrogenism. Histopathology revealed a splenic lymphoma. At day 4, a systematic USS showed a PVT extending in the portal branches. Therapeutic low molecular weight heparin was used and then transitioned to fluindione for 3 months. Follow-up USS performed at 1 and 4 months demonstrated complete resolution of the PVT. PVT after pediatric LS is a rare event in our series. Clinician should be cautious in oncologic cases and if very large spleen or if thrombocythemia >650.10(9)/L is present. If detected early, PVT can be treated efficiently. We therefore recommend a systematic USS during the first postoperative week.
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http://dx.doi.org/10.1007/s11239-013-1037-2DOI Listing
February 2015

Mini-invasive surgery for adrenocortical carcinoma in children: is it safe?

Pediatr Hematol Oncol 2013 May 27;30(4):288-90. Epub 2013 Feb 27.

Laparoscopy in adrenocortical carcinoma is controversial due to high risk of local recurrence in case of per-operative spillage. We wanted to report the case of a successful laparoscopic transperitoneal adrenalectomy performed in a 3-year-old girl presenting a 5.5-cm adrenocortical carcinoma.
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http://dx.doi.org/10.3109/08880018.2013.771389DOI Listing
May 2013

Adult care of children from pediatric urology: part 2.

J Urol 2012 Sep 19;188(3):717-23. Epub 2012 Jul 19.

Urology Centre and Department of Gynecology, University College Hospitals, London, United Kingdom.

Purpose: We describe the outcomes of undescended testes and sex development disorders in adolescence and young adulthood. We reviewed the requirements for the long-term care of children born with these and other major congenital anomalies of the genitourinary system.

Materials And Methods: The current English language literature was retrieved with a PubMed® search for articles on these subjects. Only articles covering outcomes at ages past puberty were included in analysis. The material was supplemented from the database of the clinic for adults with sex development disorders at University College London Hospitals.

Results: An undescended testis has impaired spermatogenesis. In men in whom a unilateral undescended testis was corrected before puberty the incidence of paternity is normal at around 90% of those who attempt it. The equivalent rate for those with bilateral undescended testes is about 65%. If surgery for bilateral undescended testes is delayed until after puberty, fertility is unlikely. The risk of testicular neoplasms is overestimated and the relative risk is between 2.5 and 8. Children born with a sex development disorder receive multidisciplinary treatment throughout childhood and require the same care as adults. Males who are under virilized likely have a micropenis (greater than 2 SD below the mean stretched length) but they may have normal sexual function. Fertility depends on the underlying condition. Virilized females, who most commonly have congenital adrenal hyperplasia, currently present to adult clinics with an inadequate vagina after infantile surgery. Reconstruction is required to allow intercourse.

Conclusions: The care of adults born with abnormalities of the genitalia is complex. Early management may define upbringing in childhood but requirements for sexuality and fertility in adult life are different. Multidisciplinary care is essential and a case can be made to establish a subspecialty of urology to coordinate it.
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http://dx.doi.org/10.1016/j.juro.2012.05.001DOI Listing
September 2012

Editorial.

J Pediatr Urol 2011 Oct;7(5):503

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http://dx.doi.org/10.1016/j.jpurol.2011.07.007DOI Listing
October 2011

Editorial comment.

Arab J Urol 2011 Sep 18;9(3):206-7. Epub 2011 Nov 18.

Department of Paediatric Urology, Hôpital Mère-Enfants - GHE, Claude-Bernard University - Lyon I, 69500 Bron, France.

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http://dx.doi.org/10.1016/j.aju.2011.08.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4150569PMC
September 2011

Long-term outcome of hypospadias surgery: current dilemmas.

Curr Opin Urol 2011 Nov;21(6):465-9

Department of Paediatric Urology, Claude-Bernard University - Lyon 1, Lyon, France.

Purpose Of Review: To list the main questions and dilemmas raised by hypospadias management and its long-term evaluation.

Recent Findings: Criteria of evaluation of hypospadias have changed in terms of anatomical description and biological screening. A better understanding of the causative and epidemiological aspects of the development anomalies of the genital tubercle has led to a more adjusted biological and surgical approach. The place of preoperative biological screening, preoperative hormonal stimulation and the choice of urethroplasty are the three main fields of discussion between hypospadiologists. Evaluation of outcome is the critical point as there are no current consensual protocols, and long-term results are often lacking as well as psychological evaluation.

Summary: Efforts are made to coordinate the management and evaluation of disorders of sex development (DSD) patients and more specifically hypospadias patients. National, European (EuroDSD) and international (consensus conferences) aim at finding common tracks to improve the quality of treatment and follow-up.
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http://dx.doi.org/10.1097/MOU.0b013e32834a6da3DOI Listing
November 2011

Hypospadias dilemmas: a round table.

J Pediatr Urol 2011 Apr 14;7(2):145-57. Epub 2011 Jan 14.

Department of Pediatric Urology, University of Texas, Southwestern Medical Center and Children's Medical Center, Dallas, TX 75390, USA.

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http://dx.doi.org/10.1016/j.jpurol.2010.11.009DOI Listing
April 2011