Publications by authors named "Alaa El-Ghoneimi"

87 Publications

New method for early evaluation of clitoris innervation using clitoro-perineal reflex after feminizing genitoplasty in early childhood: a pilot-study.

Sci Rep 2021 Mar 29;11(1):7087. Epub 2021 Mar 29.

Department of Pediatric Surgery and Urology, University Children Hospital Robert-Debré, APHP, University of Paris, 48, Bd Sérurier, 75935, Paris Cedex 19, France.

A major complication of feminizing genitoplasty in children is the loss of clitoral sensation with serious impact at adult life. We suggest a new method to evaluate the surgical results during childhood based on the bulbocavernosus or clitoro-perineal reflex (CPR). The afferent pathway of CPR implies the intact sensory receptors on the clitoral glans. Girls with congenital adrenal hyperplasia who were followed-up medically without surgery or who underwent feminizing genitoplasty with or without clitoroplasty were included (2002-2018). All clitoroplasties were standardized reduction clitoroplasty with preservation of neurovascular bundles associated with vaginoplasty and vestibuloplasty. Standardized examinations were prospectively performed including the CPR starting at one year postoperatively. The reflex was triggered by gentle touch of the glans by a cotton swab. Contraction of the perineal muscles was considered positive. Thirty-two children were operated at a median age of 8.6 months (5.8-12.1). Median follow-up (FU) was 3.9 years (1.3-6.4). Twenty-four patients had clitoroplasties: 17 were tested for CPR at one-year FU, and all had a positive test. Eight girls had genitoplasty without clitoral surgery, two of them were tested and were positive. Ten patients were managed without surgery, two of them were tested for the CPR and were positive. The reflex was always triggered easily and repeated at least twice during the FU. The clitoro-perineal reflex is a simple, non-invasive and reproducible test in early childhood and may serve as an early evaluation tool of clitoral innervation after feminizing genitoplasty. These results need to be confirmed at long term and completed at adult life.
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http://dx.doi.org/10.1038/s41598-021-86434-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8007626PMC
March 2021

Impact of the COVID-19 pandemic on oncological and functional robotic-assisted surgical procedures.

J Robot Surg 2021 Jan 28. Epub 2021 Jan 28.

Urology, Predictive Onco-Urology, GRC 5, APHP, Pitié-Salpêtrière, Sorbonne University, 75013, Paris, France.

The COVID-19 pandemic led to a decrease in surgical activity to avoid nosocomial contamination. Robotic-assisted surgery safety is uncertain, since viral dissemination could be facilitated by gas environment. We assessed the impact and safety of the COVID-19 pandemic on robotic-assisted surgery. Data were collected prospectively during lockdown (March 16th-April 30th 2020) in 10 academic centres with robotic surgical activity and was compared to a reference period of similar length. After surgery, patients with suspected COVID-19 were tested by RT-PCR. During the COVID-19 lockdown we evidenced a 60% decrease in activity and a 49% decrease in oncological procedures. However, the overall proportion of oncological surgeries was significantly higher during the pandemic (p < 0.001). Thirteen (7.2%) patients had suspected COVID-19 contamination, but only three (1.6%) were confirmed by RT-PCR. The COVID-19 pandemic resulted in a significant decrease in robotic-assisted surgery. Robotic approach was safe with a low rate of postoperative COVID-19 contamination.
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http://dx.doi.org/10.1007/s11701-021-01201-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7843004PMC
January 2021

Mast Cell Chymase and Kidney Disease.

Int J Mol Sci 2020 Dec 30;22(1). Epub 2020 Dec 30.

Centre de Recherche sur l'inflammation, CNRS ERL8252, Faculté de Médecine site Bichat, Université de Paris, Inserm UMR1149, 16 rue Henri Huchard, F-75018 Paris, France.

A sizable part (~2%) of the human genome encodes for proteases. They are involved in many physiological processes, such as development, reproduction and inflammation, but also play a role in pathology. Mast cells (MC) contain a variety of MC specific proteases, the expression of which may differ between various MC subtypes. Amongst these proteases, chymase represents up to 25% of the total proteins in the MC and is released from cytoplasmic granules upon activation. Once secreted, it cleaves the targets in the local tissue environment, but may also act in lymph nodes infiltrated by MC, or systemically, when reaching the circulation during an inflammatory response. MC have been recognized as important components in the development of kidney disease. Based on this observation, MC chymase has gained interest following the discovery that it contributes to the angiotensin-converting enzyme's independent generation of angiotensin II, an important inflammatory mediator in the development of kidney disease. Hence, progress regarding its role has been made based on studies using inhibitors but also on mice deficient in MC protease 4 (mMCP-4), the functional murine counterpart of human chymase. In this review, we discuss the role and actions of chymase in kidney disease. While initially believed to contribute to pathogenesis, the accumulated data favor a more subtle view, indicating that chymase may also have beneficial actions.
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http://dx.doi.org/10.3390/ijms22010302DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7795820PMC
December 2020

SRY-negative 46,XX testicular/ovotesticular DSD: Long-term outcomes and early blockade of gonadotropic axis.

Clin Endocrinol (Oxf) 2021 Apr 26;94(4):667-676. Epub 2020 Dec 26.

Pediatric Endocrinology Department, Centre de Référence des Maladies Endocriniennes Rares de la Croissance, Robert Debré Hospital, Assistance publique-Hôpitaux de Paris, Paris, France.

Objective: SRY-negative 46,XX testicular and ovotesticular disorders/differences of sex development (T/OTDSD) represent a very rare and unique DSD condition where testicular tissue develops in the absence of a Y chromosome. To date, very few studies have described the phenotype, clinical and surgical management and long-term outcomes of these patients. Particularly, early blockade of the gonadotropic axis in patients raised in the female gender to minimize postnatal androgenization has never been reported.

Design: Retrospective description of sixteen 46,XX T/OTDSD patients.

Results: Sixteen 46,XX SRY-negative T/OTDSD were included. Most (12/16) were diagnosed in the neonatal period. Sex of rearing was male for six patients and female for ten, while the clinical presentation varied, with an external masculinization score from 1 to 10. Five patients raised as girl were successfully treated with GnRH analog to avoid virilization during minipuberty. Ovotestes/testes were found bilaterally for 54% of the patients and unilaterally for the others (with a contralateral ovary). Gonadal surgery preserved appropriate tissue in the majority of cases. Spontaneous puberty occurred in two girls and one boy, while two boys required hormonal induction of puberty. One of the girls conceived spontaneously and had an uneventful pregnancy. DNA analyses (SNP-array, next-generation sequencing and whole-exome sequencing) were performed. A heterozygous frameshit mutation in the NR2F2 gene was identified in one patient.

Conclusions: This study presents a population of patients with 46,XX SRY-negative T/OTDSD. Early blockade of gonadotropic axis appears efficient to reduce and avoid further androgenization in patients raised as girls.
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http://dx.doi.org/10.1111/cen.14389DOI Listing
April 2021

Redo Laparoscopic Pyeloplasty in Infants and Children: Feasible and Effective.

Front Pediatr 2020 10;8:546741. Epub 2020 Nov 10.

Department of Pediatric Urology, Robert-Debré University Hospital, Assistance Publique - Hôpitaux de Paris (APHP), Paris, France.

To determine the feasibility and effectiveness of redo laparoscopic pyeloplasty among patients with failed previous pyeloplasty, specifically examining rates of success and complications. We retrospectively reviewed the charts of all patients, who underwent redo laparoscopic pyeloplasty from 2006 to 2017. This included patients who underwent primary pyeloplasty at our institution and those referred for failures. Analysis included demographics, operative time, complications, length of hospital stay, complications, and success. Success was defined as improvement of symptoms and hydronephrosis and/or improvement in drainage demonstrated by diuretic renogram, especially in those with persistent hydronephrosis. Descriptive statistics are presented. We identified 22 patients who underwent redo laparoscopic pyeloplasty. All had Anderson-Hynes technique except two cases in which ureterocalicostomy was performed. Median (IQR) follow-up was 29 (2-120) months, median time between primary pyeloplasty and redo laparoscopic pyeloplasty was 12 (7-49) months. The median operative time was 200 (50-250) min, and median length of hospital stay was 3 (2-10) days. The procedure was feasible in all cases without conversion. During follow-up, all but two patients demonstrated an improvement in the symptoms and the degree of hydronephrosis. Ninety-one percent of patients experienced success and no major complications were noted. Redo laparoscopic pyeloplasty is feasible and effective with a high success rate and low complication rate.
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http://dx.doi.org/10.3389/fped.2020.546741DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7683417PMC
November 2020

The role of a pediatric tertiary care center in avoiding collateral damage for children with acute appendicitis during the COVID-19 outbreak.

Pediatr Surg Int 2020 Dec 18;36(12):1397-1405. Epub 2020 Oct 18.

Department of General Pediatric Surgery and Urology, Robert-Debré University Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), 48 boulevard Sérurier, 75019, Paris, France.

Purpose: To evaluate the impact of the COVID-19 pandemic-related lockdown on management and outcomes of children with acute appendicitis.

Methods: A retrospective cohort study was conducted, including children treated for acute appendicitis (January 20th-May 11th, 2020). The data regarding the severity of appendicitis and outcome were collected and compared for two time periods, before and after the nationwide lockdown (March 17th, 2020).

Results: The number of cases of acute appendicitis increased by 77% during the lockdown (n = 39 vs. n = 69, p = 0.03). During the lockdown, children treated for appendicitis were older (11.1 vs. 8.9 years, p = 0.003), and were more likely to live more than 5 km away from our institution (77% vs. 52%, p = 0.017). Less children had previously consulted a general practitioner (15% vs. 33%, p = 0.028), whereas more children were transferred from other hospitals (52% vs. 31%, p = 0.043). There was no difference in terms of length of hospital stay, rate of postoperative intra-abdominal abscess, ER visits, and readmissions between both periods. Three children (4%) were diagnosed with COVID-19 and appendicitis.

Conclusions: Despite an increase in the number of children with appendicitis managed at our hospital during the COVID-19-related lockdown, management, and outcome remained similar. Although our pediatric center was strongly affected by this pandemic, maintaining our prior practice strategies for acute appendicitis avoided the occurrence of collateral damage for those children.
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http://dx.doi.org/10.1007/s00383-020-04759-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7568762PMC
December 2020

Commentary Re: Staged repair of proximal hypospadias: Reporting outcome of staged tubularized autograft repair (STAG).

Authors:
Alaa El Ghoneimi

J Pediatr Surg 2020 12 31;55(12):2717. Epub 2020 Jul 31.

Pediatric Urologist, Professor of Pediatric Surgery, University of Paris, Coordinator of National Reference Center for Rare Urinary Tract Malformations (MARVU), Head of Department of Pediatric Surgery and Urology, University Hospital Robert Debré, APHP, 48 Bd Serurier, Paris 75019, France. Electronic address:

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http://dx.doi.org/10.1016/j.jpedsurg.2020.07.025DOI Listing
December 2020

Realization of Open Software Chain for 3D Modeling and Printing of Organs in Simulation Centers: Example of Renal Pelvis Reconstruction.

J Surg Educ 2021 Jan-Feb;78(1):232-244. Epub 2020 Jul 20.

Simulation Centre of University of Paris, iLumens Paris Diderot, Paris, France.

Objective: Three-dimensional (3D) printing has many uses in healthcare such as in surgical training. It is becoming an interesting tool finding new pedagogical purposes in medical simulation. In this study, using a process consisting of 3D modeling, a simulator dedicated to pyeloplasty was designed, manufactured, and evaluated by experts.

Design: With the aid of open-source software and computer-aided design software, 3D models of a renal parenchyma, a renal pelvis and a ureter were created. This renal apparatus was processed and crafted with additive manufacturing using soft polymer materials. Polyvinyl alcohol material was used to print the components in order to make them dissectible and to evaluate their use in surgical teaching.

Setting And Participants: Seven expert surgeons evaluated the model by performing a pyeloplasty sequence established in a previous work. An evaluation grid with 8 items related to surgical movement was rated on a 5-point Likert scale to assess how similar working with the model was to actual surgery.

Results: Three items were rated with a score greater than or equal to 4 (Needle penetration, Thread-sliding, and Cutting Strength). Suture strength was rated with a score above 3.5 for both renal pelvis and ureter, whereas elasticity was rated below 3. Handling and mobility properties were rated above 3 for the renal pelvis and below 3 for the ureter. The cost of the unit was $0.30 per renal unit. The primary difference identified was a difference in elongation between polyvinyl alcohol material and real biological tissue.

Conclusions: It is feasible to generate and print a low cost upper urinary tract model from patient data imagery using environmentally friendly products that can be used effectively in surgical training. The simulator has been able to reproduce sensations related to surgical movements for a low cost. Hereafter, research into the pedagogical benefits provided to students, and through them, patients, should be performed. 3D printing models can offer new opportunities for healthcare simulation specific to different surgical fields.
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http://dx.doi.org/10.1016/j.jsurg.2020.06.035DOI Listing
July 2020

Double-face preputial island flap revisited: is it a reliable one-stage repair for severe hypospadias?

World J Urol 2020 Jun 29. Epub 2020 Jun 29.

Department of Pediatric Surgery and Urology, Hôpital Robert Debré, Centre de Référence Maladies Endocriniennes de la Croissance et du Développement (CRMERC), APHP, University of Paris, Paris, France.

Purpose: We have studied outcome of double-face preputial island flap (DFPIF) technique in severe types of hypospadias: penoscrotal, scrotal and perineal.

Methods: We have used DFPIF in 75 boys at a median age of 1.1 years (1.0-1.5). The meatus was penoscrotal, scrotal or perineal after de-gloving the penis. The inner face of the foreskin was used for urethroplasty and the outer face for ventral skin covering. Modifications were added: proximal anastomosis was protected by a spongioplasty; in case of urethral plate transection, we anastomosed on onlay proximal and distal segments of the flap (onlay-tube-onlay) and the tubularized part was sutured to corpus cavernosa. FU was scheduled at one month then every 3 months for a year then annually. At each consultation, the surgeon filled out a detailed cosmetic and functional sheet including flowmeter.

Results: Thirty-four patients had onlay preputial flap repair with urethral plate preservation. Forty-one had the onlay-tube-onlay technique. All children had a curvature, 19 had a significant residual curvature after dissection, corrected by dorsal plication (n = 9) and ventral lengthening (n = 10). Median FU was 4.2 years (2.7-6.5). 36 children (48%) had complications and needed redo surgery: 12 fistulas, 11 diverticula, 7 meatal stenosis, 3 strictures and 2 residual curvatures. All children but three voided within the normal limits for their age.

Conclusion: DFPIF remains a good option for a one-stage repair of severe hypospadias. After a median of 1.8 procedures, the final success rate was 96%. The healthy well-vascularized ventral skin allows safe redo surgery when needed.
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http://dx.doi.org/10.1007/s00345-020-03324-7DOI Listing
June 2020

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

Mast cell chymase protects against acute ischemic kidney injury by limiting neutrophil hyperactivation and recruitment.

Kidney Int 2020 03 10;97(3):516-527. Epub 2019 Oct 10.

Center of Research on Inflammation, Inserm UMRS-1149, Paris, France; Center of Research on Inflammation, CNRS ERL 8252, Paris, France; Center of Research on Inflammation, Université Paris Diderot, Sorbonne Paris Cite, Laboratoire d'excellence INFLAMEX, Paris, France. Electronic address:

Here we investigated the role of murine mast cell protease 4 (MCPT4), the functional counterpart of human mast cell chymase, in an experimental model of renal ischemia reperfusion injury, a major cause of acute kidney injury. MCPT4-deficient mice had worsened kidney function compared to wildtype mice. MCPT4 absence exacerbated pathologic neutrophil infiltration in the kidney and increased kidney myeloperoxidase expression, cell death and necrosis. In kidneys with ischemia reperfusion injury, when compared to wildtype mice, MCPT4-deficient mice showed increased surface expression of adhesion molecules necessary for leukocyte extravasation including neutrophil CD162 and endothelial cell CD54. In vitro, human chymase mediated the cleavage of neutrophil expressed CD162 and also CD54, P- and E-Selectin expressed on human glomerular endothelial cells. MCPT4 also dampened systemic neutrophil activation after renal ischemia reperfusion injury as neutrophils expressed more CD11b integrin and produced more reactive oxygen species in MCPT4-deficient mice. Accordingly, after renal injury, neutrophil migration to an inflammatory site distal from the kidney was increased in MCPT4-deficient versus wildtype mice. Thus, contrary to the described overall aggravating role of mast cells, one granule-released mediator, the MCPT4 chymase, exhibits a potent anti-inflammatory function in renal ischemia reperfusion injury by controlling neutrophil extravasation and activation thereby limiting associated damage.
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http://dx.doi.org/10.1016/j.kint.2019.08.037DOI Listing
March 2020

Association of Maternal First Trimester Serum Levels of Free Beta Human Chorionic Gonadotropin and Hypospadias: A Population Based Study.

J Urol 2020 05 19;203(5):1017-1023. Epub 2019 Dec 19.

Sorbonne Universite, INSERM, Maladies génétiques d'expression pédiatrique, Hôpital d'Enfants Armand Trousseau, APHP, Paris, France.

Purpose: Human chorionic gonadotropin stimulates fetal testosterone production and contributes to normal development of male genitalia. Using population based data we hypothesized that differences in maternal free beta human chorionic gonadotropin may be associated with hypospadias.

Materials And Methods: Data were obtained from the Paris Registry of Congenital Malformations (REMAPAR) (2011 to 2016). The initial study population included 3,172 pregnant women who gave birth to a singleton live born male infant with a congenital malformation. After exclusion of cases with unknown beta human chorionic gonadotropin and those with chromosomal or genetic abnormalities, the study population included 194 boys with isolated hypospadias and 1,075 controls. For cases with operative notes (125) we obtained data on type (proximal/distal) of hypospadias. Using quantile regression we compared median values of multiple of median beta human chorionic gonadotropin measured for first trimester Down syndrome screening (10th to 13th gestational weeks) for overall as well as by type of hypospadias vs controls. We also considered possible effects of placental dysfunction (maternal age, intrauterine growth retardation and preterm births) as potential confounding factors.

Results: Overall the median beta human chorionic gonadotropin multiple of median was comparable for women who had an infant with hypospadias vs controls (0.99 vs 0.95, p=0.3). However, proximal hypospadias was associated with a statistically significant higher median multiple of median than distal hypospadias or unspecified (1.49 vs 0.92 vs 1.05, p=0.02). The estimates were comparable after adjustment for placental dysfunction.

Conclusions: Our findings support the hypothesis that an alteration in maternal beta human chorionic gonadotropin levels is associated with hypospadias. However, this association appears to be limited to proximal hypospadias.
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http://dx.doi.org/10.1097/JU.0000000000000708DOI Listing
May 2020

Isolated congenital inter-costal pulmonary hernia: a case report.

J Med Case Rep 2019 Jul 18;13(1):232. Epub 2019 Jul 18.

Robert DEBRE Hospital, Paris, France.

Background: Intercostal lung herniation is a rare condition that may be congenital (20%) or acquired (80%). The isolated congenital form is exceptional, with one case reported in the literature.

Case Presentation: We report a case of a 10-year-old French boy of Algeria origin, born with intermittent swelling of his right hemithorax. The swelling and pain gradually increased with age. A clinical examination revealed a localized swelling of his right hemithorax at the level of the midclavicular line and the fifth intercostal space. The swelling increased in size during respiratory movements and enlarged with Valsalva maneuvers. The intercostal lung hernia was treated by thoracoscopy.

Conclusions: This is the second case of isolated congenital intercostal pulmonary hernia reported in the French and English literature. It is the first to be treated by thoracoscopy. Based on this case we performed a review of the diagnosis and therapeutic aspect of pulmonary hernias.
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http://dx.doi.org/10.1186/s13256-019-2142-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6637642PMC
July 2019

Laparoscopic retroperitoneal approach for retrocaval ureter in children.

World J Urol 2020 Aug 11;38(8):2055-2062. Epub 2019 Jun 11.

Department of Pediatric Surgery and Urology, Robert-Debré University Children's Hospital, Assistance-Publique Hôpitaux de Paris, University Paris Diderot, Sorbonne Paris Cité, 48, Boulevard Sérurier, 75935, Paris, France.

Purpose: Retrocaval ureter (RCU) is a rare congenital anomaly and published data on pediatric laparoscopic management are poor. The aim of this study was to report our experience of retroperitoneal laparoscopic approach for management of RCU in children.

Methods: A retrospective review of data from patients treated for RCU between 2002 and 2018 in our institution was performed. All patients were positioned in a flank position and underwent a three-port (5-mm optical trocar and two 3-mm trocars) laparoscopic retroperitoneal ureteroureterostomy. Anastomosis was made by 6/0 absorbable sutures. A JJ stent was always inserted.

Results: Five patients with a median age of 94 months (5-152) were operated on and followed up for a median time of 103 months (46-201). Median operating time was 200 min (160-270). No conversion and no transfusion occurred. Median hospital stay was 2 days (1-4). Ureteral stent was removed after 52 days (47-82). Complications included pyelonephretis (N = 1). In all cases, hydronephrosis decreased postoperatively.

Conclusions: Retroperitoneal laparoscopic approach for RCU is safe and effective in children. Our video demonstrates different patients with specific surgical details to show how to manage these children. The global vision of the upper tract by laparoscopy leads to optimal management of these children even if the anomaly was not detected preoperatively.
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http://dx.doi.org/10.1007/s00345-019-02849-wDOI Listing
August 2020

Reflective Practice About Retroperitoneal Laparoscopy in Comparison to Open Surgery for Ureteropelvic Junction Obstruction Repair in Children Less Than 1 Year of Age.

Front Pediatr 2019 24;7:194. Epub 2019 May 24.

Robert-Debré University Hospital, AP-HP; Université Paris Diderot, Sorbonne Paris Cité, Pediatric Surgery and Urology, National Reference Center of Rare Urinary Tract Malformations (MARVU), Paris, France.

The interest in laparoscopy in the treatment of ureteropelvic junction obstruction (UPJO) in children under 12 months of age remains controversial. The aim of this study is to evaluate feasibility and benefits of retroperitoneal laparoscopy (RL) compared to open surgery in this age group. Between January 2012 and May 2017, we performed 222 pyeloplasties: 144 by laparoscopy and 78 by open surgery. From 2012, the choice of operative technique was decided according to the laparoscopic experience of the surgeon; two surgeons operated laparoscopically on all children <12 months of age, while others operated using posterior lumbotomy (PL). The RL is standardized and performed by 3 trocars (5, 3, 3). Pre, per and postoperative parameters were analyzed retrospectively. Statistical tests: Pearson, Fisher, Student and Mann-Whitney. During this 5-year period, 24 RL and 53 PL were included with a median follow-up of 27 months (5-63). In the LR group, postoperative drainage was performed by JJ (13 cases) and external stent (11 cases). No conversion has been listed in this group. In each group there was one failure that needed redo pyeloplasty. Duration of hospitalization and intravenous acetaminophen use were significantly lower in the RL group (2.8 vs. 2.3 days, = 0.02, respectively) while operating time was significantly longer (163 vs. 85.8 min, = 0.001). The postoperative complication rate was statistically identical in each group (urinary tract infection, wall hematoma, hematuria…). RL is feasible in children under 1 year of age in the hands of well-experienced surgeons with longer operative time but without added morbidity. Subject to the retrospective nature of our study, the RL seems to offer a benefit regarding duration of hospitalization and analgesics consumption.
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http://dx.doi.org/10.3389/fped.2019.00194DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6543804PMC
May 2019

Urine biochemistry to predict long-term outcomes in fetuses with posterior urethral valves.

Prenat Diagn 2018 11 27;38(12):964-970. Epub 2018 Sep 27.

Biochemistry-Hormonology, Robert Debré Hospital, AP-HP, Paris, France.

Objective: Because the literature on the predictive value of fetal urinalysis is controversial in fetuses with lower urinary tract obstruction, we determined the best model of fetal urine biochemical markers correlated with long-term postnatal renal function based on glomerular filtration rate (GFR).

Method: This retrospective study concerned 89 fetuses with lower urinary tract obstruction and their renal function after 10 years of age. We correlated fetal urine biochemical markers (total protein, β2-microglobulin, sodium, chloride, glucose, calcium, and phosphorus) with GFR at 10 to 30 years of age in 89 patients with posterior urethral valves. We defined five stages of chronic kidney disease (CKD).

Results: Of the 89 patients, 18 (20%) are 20 years old or over. Postnatal renal function was good in 67.4% (GFR > 60 mL/min/1.73 m ) and poor in 17% (GFR < 30 mL/min/1.73 m ). All fetal urine markers differed between CKD stage 1 + 2 and CKD stage 4 + 5 (P < 0.001). β2-microblobulin showed an 87% sensitivity for a 72% specificity. A combination of β2-microglobulin and chloride gave the best results (93% sensitivity and 71% specificity) versus amniotic fluid volume (80% sensitivity and 73% specificity).

Conclusion: Fetal urine biochemistry predicts long-term (10-30 years) postnatal renal function.
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http://dx.doi.org/10.1002/pd.5359DOI Listing
November 2018

How the ESPU grades clinical abstracts.

J Pediatr Urol 2018 10 21;14(5):451-452. Epub 2018 Jul 21.

Department of Pediatric Urology, Astrid Lindgren Children's Hospital, Karolinska University Hospital, Stockholm, Sweden; Dept. of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden. Electronic address:

The ability to consistently review abstracts in an unbiased and objective fashion is a skill that most academics hope to master. However, robust standardized rating systems are sparse, with most scientific boards leaving the task of rating abstracts poorly defined and at the whim of the reviewer. In an effort to bring consistency to this process, in 2013, the ESPU board adopted an abstract rating system that has been previously used in the field of plastic surgery and orthopedics. (van der Steen et al., 2004; Poolman et al., 2007). The aim of this manuscript is to outline this practice.
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http://dx.doi.org/10.1016/j.jpurol.2018.07.009DOI Listing
October 2018

Editorial Comment.

Authors:
Alaa El-Ghoneimi

J Urol 2018 10 28;200(4):894. Epub 2018 Jun 28.

Division of Pediatric Surgery, University of Paris-Diderot Faculty of Medicine, Paris, France.

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http://dx.doi.org/10.1016/j.juro.2018.04.089DOI Listing
October 2018

Renal parenchyma impairment characterization in partial unilateral ureteral obstruction in mice with intravoxel incoherent motion-MRI.

NMR Biomed 2018 02 27;31(2). Epub 2017 Nov 27.

INSERM UMR 1149, Paris, France.

Ureteropelvic junction obstruction constitutes a major cause of progressive pediatric renal disease. The biological mechanisms underlying the renal response to obstruction can be investigated using a clinically relevant mouse model of partial unilateral ureteral obstruction (pUUO). Renal function and kidney morphology data can be evaluated using renal ultrasound, scintigraphy and uro-magnetic resonance imaging (uro-MRI), but these methods are poorly linked to histological change and not all are quantitative. Here, we propose to investigate pUUO for the first time using an intravoxel incoherent motion diffusion sequence. The aim of this study was to quantitatively characterize impairment of the kidney parenchyma in the pUUO model. This quantitative MRI method was able to assess the perfusion and microstructure of the kidney without requiring the injection of a contrast agent. The results suggest that a perfusion fraction (f) reduction is associated with a decrease in the volume of the renal parenchyma, which could be related to decreased renal vascularization. The latter may occur before impairment by fibrosis and the findings are in accordance with the literature using the UUO mice model and, more specifically, on pUUO. Further investigation is required before this technique can be made available for the diagnosis and management of children with antenatal hydronephrosis and to select the optimal timing of surgery if required.
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http://dx.doi.org/10.1002/nbm.3858DOI Listing
February 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

Imported Schistosomiasis in Children: Clinical, Diagnostic Aspects And Outcome in 5 Tertiary Hospitals in France.

Pediatr Infect Dis J 2017 Dec;36(12):e349-e351

From the *Department of Pediatrics, Paris 13 University, Jean Verdier Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Bondy, France; †Department of Microbiology, University Paris-Diderot, University Hospital Robert-Debré, ‡Department of Pediatric Emergency, University Paris-Diderot, Sorbonne Paris Cité, Hospital Robert Debré, §Department of Pediatric Surgery and Urology, University Paris-Diderot, University Hospital Robert Debré, and ¶Department of Parasitology-Mycology, University Pierre et Marie Curie, Universitary Hospitals Pitié-Salpétrière/Charles Foix, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France; ‖Department of General Pediatrics, Hospital Delafontaine, Saint-Denis, France; **Department of Surgery, University Paris Descartes, Necker-Enfants Malades Hospital, ††Department of General Pediatrics and Pediatric Infectious Diseases, University Paris Descartes, Necker-Enfants Malades Hospital, ‡‡Department of Parasitology-Mycology, University Paris Descartes, Cochin Hospital, and §§Department of Nephrology and Kidney Transplantation, University of Pierre et Marie Curie, Trousseau University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; ¶¶Department of Infectious and tropical diseases, Paris 13 University, Avicenne Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Bobigny, France; ‖‖Department of Parasitoloy, Paris 13 University, Jean Verdier Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Bondy, France; ***UMR 190, Unité des virus émergents, Université Aix-Marseille, Marseille, France; and †††Department of General Pediatrics, University Paris-Diderot, University Hospital Robert Debré, INSERM 1123 Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France.

The objective of this retrospective study is to describe imported schistosomiasis in children in the Paris region between 2010 and 2015. Forty children with a diagnosis of schistosomiasis were included. Thirty-seven (93%) had a chronic urinary form with hematuria. The lost-to-follow up rate for the second consultation was 25%. The diagnosis and management of imported schistosomiasis must be improved-notably by raising awareness among clinicians and providing families with more information.
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http://dx.doi.org/10.1097/INF.0000000000001679DOI Listing
December 2017

Mast Cells and MCPT4 Chymase Promote Renal Impairment after Partial Ureteral Obstruction.

Front Immunol 2017 4;8:450. Epub 2017 May 4.

INSERM UMRS 1149, Paris, France.

Obstructive nephropathy constitutes a major cause of pediatric renal progressive disease. The mechanisms leading to disease progression are still poorly understood. Kidney fibrotic lesions are reproduced using a model of partial unilateral ureteral obstruction (pUUO) in newborn mice. Based on data showing significant mast cell (MC) infiltration in patients, we investigated the role of MC and murine MCPT4, a MC-released chymase, in pUUO using MC- (W), MCPT4-deficient (), and wild-type (WT) mice. Measurement of kidney length and volume by magnetic resonance imaging (MRI) as well as postmortem kidney weight revealed hypotrophy of operated right kidneys (RKs) and compensatory hypertrophy of left kidneys. Differences between kidneys were major for WT, minimal for W, and intermediate for mice. Fibrosis development was focal and increased only in WT-obstructed kidneys. No differences were noticed for local inflammatory responses, but serum CCL2 was significantly higher in WT versus and W mice. Alpha-smooth muscle actin (αSMA) expression, a marker of epithelial-mesenchymal transition (EMT), was high in WT, minimal for W, and intermediate for RK. Supernatants of activated MC induced αSMA in co-culture experiments with proximal tubular epithelial cells. Our results support a role of MC in EMT and parenchyma lesions after pUUO involving, at least partly, MCPT4 chymase. They confirm the importance of morphologic impairment evaluation by MRI in pUUO.
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http://dx.doi.org/10.3389/fimmu.2017.00450DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5415561PMC
May 2017

[Urothelial tumors in children].

Bull Cancer 2017 Feb 26;104(2):195-201. Epub 2016 Dec 26.

Institut Curie, service d'oncopédiatrie, 26, rue d'Ulm, 75005 Paris, France.

Urothelial tumors are very rare in children (to date, only about 150 cases have been reported worlwide). Only 20% occur before the age of ten. The aim of this study is to specify the clinicopathologic features of urothelial tumor in young patients, which require a slightly different approach to treatment. On the basis of the WHO/ISUP (World Health Organisation/International Society of Urological Pathology) consensus classification report, these lesions are usually low-grade lesions, non invasive, and rarely recurrent. The sex ratio is three boys to one girl. These tumors are located preferentially in the low urinary tract, especially in the bladder. The main symptom is the macroscopic hematuria, which requires ultrasound examination in all cases. Cystoscopy is indicated in case of lesion of the bladder wall, or in case of persistent or recurrent hematuria, to obtain definitive diagnosis and biopsies. The tumors are mainly located on the posterior or lateral bladder wall above the trigone or near the ureteral orifices. Treatment is based on the transurethral resection of the lesion. The subsequent monitoring is sparsely codified, due to the exceptional occurrence of these tumors in the paediatric age group. These patients are likely to have better outcome than older patients, but it is due to the predominance of noninvasive papillary urothelial tumors. Tumor recurrences are not uncommon. In case of invasive, high-grade urothelial carcinomas, metastases or even lethal outcome may occur in rare cases.
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http://dx.doi.org/10.1016/j.bulcan.2016.11.018DOI Listing
February 2017

Commentary to "Hypospadias: Are we as good as we think when we correct proximal hypospadias?".

Authors:
Alaa El-Ghoneimi

J Pediatr Urol 2016 08 16;12(4):197. Epub 2016 Jul 16.

Department of Pediatric Surgery and Urology, University of Paris-Diderot, University Hospital Robert Debre, APHP, Paris, France. Electronic address:

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http://dx.doi.org/10.1016/j.jpurol.2016.05.039DOI Listing
August 2016

Posterior urethral valves and vesicoureteral reflux: can prenatal ultrasonography distinguish between these two conditions in male fetuses?

Prenat Diagn 2016 Sep 29;36(9):831-7. Epub 2016 Jul 29.

Department of Obstetrics and Gynecology, Hôpital Robert Debré AP-HP, Paris, France.

Objective: The objective of the study was to evaluate prenatal sonographic signs that distinguish male fetuses with posterior urethral valves (PUV) from those with vesicoureteral reflux (VUR).

Methods: Prenatal data were retrospectively retrieved from all consecutive women delivering between 2003 and 2012 of a male newborn with a postnatal diagnosis of PUV or VUR. Prenatal parameters included fetal bladder characteristics, identification of a dilated posterior urethra, and change in shape and size in the fetal renal pelvis or ureter.

Results: One hundred thirty-six women gave birth to a male newborn with a postnatal diagnosis of PUV (n = 49) or VUR (n = 87). The presence of posterior urethral dilatation [21 (42.9%) fetuses versus 0 (0%), p = 0.000], a thickened fetal bladder wall [37 (75.5%) vs 4 (4.6%), p = 0.000], and anhydramnios [14 (28.6%) vs 0, p = 0.000] were strongly associated with the postnatal diagnosis of PUV. Change in shape and size in the fetal renal pelvis or ureter was observed in 15 (17.2%) of 87 children with VUR versus 1 (2.0%) out of 49 with PUV (p = 0.010).

Conclusions: Prenatal ultrasound may differentiate with reasonable accuracy male fetuses with a postnatal diagnosis of PUV from those with VUR. © 2016 John Wiley & Sons, Ltd.
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http://dx.doi.org/10.1002/pd.4868DOI Listing
September 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

Re: Laparoscopic Mitrofanoff procedure in children: Critical analysis of difficulties and benefits. Blanc T, Muller C, Pons M, Pashootan P, Paye-Jaouen A, El Ghoneimi A. J Pediatr Urol 2015;11, 28.e1-8.

Authors:
Alaa El Ghoneimi

J Pediatr Urol 2015 Oct 23;11(5):303-4. Epub 2015 Jul 23.

Faculty of Medicine Diderot, University Paris-Diderot, University Hospital Robert Debré, APHP, Paris, France. Electronic address:

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http://dx.doi.org/10.1016/j.jpurol.2015.05.028DOI Listing
October 2015

Laparoscopic treatment of ureteropelvic junction obstruction in five pediatric cases of pelvic kidneys.

J Pediatr Urol 2015 Dec 17;11(6):353.e1-5. Epub 2015 Jul 17.

Department of Pediatric Surgery and Urology, Hôpital Robert Debré, APHP, Paris, France. Electronic address:

Objective: Ectopic kidney is a rare urologic condition and may be revealed by ureteropelvic junction obstruction (UPJO) in children, with pelvic kidney being the most common location. Our experience using a minimally invasive approach to treat UPJO by dismembered pyeloplasty led us to report five cases of UPJO with pelvic kidney operated on by transperitoneal laparoscopy, focusing on medium-term follow-up results and surgical technique.

Patients And Methods: From 1999 to 2010, we reviewed all cases of UPJO managed in our center, a total of 391 patients. Among those, 125 patients were operated on via a minimal invasive approach, 104 by retroperitoneoscopy, and 21 by transperitoneal laparoscopy. Five patients presented ectopic pelvic kidney and were retrospectively reviewed. US-scan and uro-MRI were done preoperatively in all cases for anatomic and functional evaluation. Peri-operative data such as operative time, transfusion, and complications were reported. At last clinic, sonographic and functional evaluation was noted. Focusing on surgical technique, we reported the tips and tricks to successfully and easily perform the dismembered pyeloplasty: optimization of the trocars' location according to the kidney location and use of a transparietal stay stitch to stabilize the suture line of the pyeloplasty.

Results: All five patients underwent dismembered pyeloplasty, at a mean age of 8 years and a mean weight of 23.4 kg. Mean operative time was 213 min (min-max: 180-245). One case of giant hydronephrosis and megacalicosis required conversion at the beginning of the learning curve. None of the patients required blood transfusion. Mean hospital stay was 2.4 days (range: 1-4). All the patients had an uneventful postoperative recovery and good postoperative functional results, with either stable or improved pelvic dilatation and renal function at a mean follow-up of 3.3 years (range: 2-5.6).

Discussion: This small series of five ectopic kidneys reflects the surgical challenge for the pediatric surgeon to perform reconstructive surgery on an abnormal anatomy. However, increasing experience in robotics in urologic pediatric surgery may lead to shortening of operative time and facilitate both dissection and suturing.

Conclusion: Dismembered pyeloplasty by transperitoneal laparoscopy is a feasible although technically demanding, safe and effective approach in the management of ureteropelvic junction obstruction in pelvic kidney in children.
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http://dx.doi.org/10.1016/j.jpurol.2015.04.042DOI Listing
December 2015

Laparoscopic management of congenital duodenal atresia or stenosis: A single-center early experience.

J Pediatr Surg 2015 Nov 28;50(11):1833-6. Epub 2015 May 28.

Pediatric Surgery Department, Robert Debré University Hospital, APHP, Paris, France; Paris VII University, Paris, France. Electronic address:

Background: The background is to review our experience with laparoscopic repair of congenital duodenal atresia or stenosis (CDAS) and compare postoperative outcome with a group control of laparotomy repair.

Methods: Retrospective chart review of all cases of CDAS undergoing laparoscopic surgery at our institution between July 2013 and May 2014 and comparison with a group control of open operation performed between 2007 and 2010. Data were compared using Fisher's exact test for qualitative values and Mann-Whitney test for quantitative values. P values less than 0,05 were considered statistically significant.

Results: Ten consecutive cases were identified in laparoscopic group (7 duodenoduodenostomy and 3 duodenojejunostomy) and 19 cases in laparotomy group (16 duodenoduodenostomy and 3 web excision). Median birth weight was lower in laparoscopic group (2125 grams Vs 2777 grams p=0,04). In laparoscopic group, there was no conversion and no intraoperative complication. Median duration of surgery was 90minutes (80-150). In both groups, the surgical morbidity rate was 10%. Median time to initiation of oral feeding was significatively shorter in laparotomy group (8days Vs 4 p=0,009). Median time to full oral feeding and length of stay were shorter in laparotomy but not statistically different. (36days Vs 16,5 p=0,14 and 45,5days Vs 25,5 p=0,09 respectively) After a median follow up of 149,5days (24-355) in laparoscopic group, 8 children had a full oral intake. Five children had a weight below the 10th percentile.

Conclusion: The laparoscopic approach for CDAS is safe and reproducible with outcomes similar to open repair even in the beginning of a learning curve for pediatric surgeons with appropriate laparoscopic skills. In this small series, laparoscopy did not appear to decrease time to full oral intake or length of stay. Larger studies are suggested to provide more conclusive results.
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http://dx.doi.org/10.1016/j.jpedsurg.2015.05.007DOI Listing
November 2015