Publications by authors named "Naima Smeulders"

21 Publications

  • Page 1 of 1

Local treatment of rhabdomyosarcoma of the female genital tract: Expert consensus from the Children's Oncology Group, the European Soft-Tissue Sarcoma Group, and the Cooperative Weichteilsarkom Studiengruppe.

Pediatr Blood Cancer 2020 Aug 6:e28601. Epub 2020 Aug 6.

Department of Pediatric Surgery, University Hospital Giessen-Marburg, Marburg, Germany.

The International Soft-Tissue Sarcoma Consortium (INSTRuCT) was founded as an international collaboration between different pediatric soft-tissue sarcoma cooperative groups (Children's Oncology Group, European Pediatric Soft-Tissue Sarcoma Group, and Cooperative Weichteilsarkom Studiengruppe). Besides other tasks, a major goal of INSTRuCT is to develop consensus expert opinions for best clinical treatment. This consensus paper for patients with rhabdomyosarcoma of the female genital tract (FGU-RMS) provides treatment recommendations for local treatment, long-term follow-up, and fertility preservation. Therefore, a review of the current literature was combined with recommendations of the treatment protocols of the appropriate clinical trials. Additionally, opinions of international FGU-RMS experts were incorporated into recommendations. Results were that the prognosis of FGU-RMS is favorable with an excellent response to chemotherapy. Initial complete surgical resection is not indicated, but diagnosis should be established properly. In patients with tumors localized at the vagina or cervix demonstrating incomplete response after induction chemotherapy, local radiotherapy (brachytherapy) should be carried out. In patients with persistent tumors at the corpus uteri, hysterectomy should be performed. Fertility preservation should be considered in all patients. In conclusion, for the first time, an international consensus for the treatment of FGU-RMS patients could be achieved, which will help to harmonize the treatment of these patients in different study groups.
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http://dx.doi.org/10.1002/pbc.28601DOI Listing
August 2020

Seamless post-operative removal of JJ stents in the paediatric population: Securing the JJ stent to the tip of the urethral balloon catheter avoids a further operation.

J Pediatr Urol 2020 Aug 26;16(4):500-501. Epub 2020 Jun 26.

Department of Paediatric Urology, Great Ormond Street Hospital for Children NHS Foundation Trust, Great Ormond Street, London WC1N 3JH, UK. Electronic address:

In children, removal of a post-procedural JJ stent requires a further cystoscopy under general anaesthetic. We describe securing the distal end of the ureteric stent to the tip of a balloon catheter at the end of the primary procedure. Once post-operative oedema has subsided, the stent is removed seamlessly in tandem with the balloon catheter. This can be done on the ward by nursing staff without the need for general anaesthesia or sedation. Our experience in the first 10 successive patients (aged 1.6-16.3 years) demonstrates this technique to be safe, easy to learn and well tolerated.
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http://dx.doi.org/10.1016/j.jpurol.2020.06.028DOI Listing
August 2020

Paratesticular rhabdomyosarcoma-Impact of locoregional approach on patient outcome: A report from the European paediatric Soft tissue sarcoma Study Group (EpSSG).

Pediatr Blood Cancer 2020 09 23;67(9):e28479. Epub 2020 Jun 23.

Department of Paediatric Surgery, Bicêtre Hospital, Hôpitaux Universitaires Paris-Sud, Le Kremlin-Bicetre, France.

Background: Paratesticular rhabdomyosarcoma (PT RMS) is rare compared to benign scrotal pathology. Inappropriate first surgery (InFS) required supplementary treatment to maintain excellent outcomes. Initial staging of regional lymph nodes is important. The aim of this study was to determine to what extent the quality of locoregional approach impacted on patient morbidity and survival.

Design/methods: Analysis was performed on all nonmetastatic PT RMS patients enrolled in the European paediatric Soft tissue sarcoma Study Group (EpSSG) RMS 2005 protocol. Aspects assessed were adherence to surgical guidelines and impact of protocol violations, relapse analysis, and survival outcomes.

Results: Analysis was performed on 237 patients, with median follow up of 67.1 months. Median age was 9.0 years. InFS occurred in 75 of 237 (32%) patients. InFS required intensified chemotherapy (10) and local therapy. After InFS, 61 required primary reexcision and five delayed surgery. Of 26 recurrences, the risk of relapse was higher in patients ≥10 years (21/26) and was mainly locoregional in 16 of 26 recurrences (± metastatic). Sixteen of 26 died with 14 of 16 patients ≥10 years. Nodal relapse neither occurred when N1 nodes were identified at diagnosis, nor after surgical staging. Five-year overall survival (OS) at age <10 years versus ≥10 years was 98.1 and 86.7%, respectively (P = .0013). Event-free survival (EFS) at age <10 years versus ≥10 years was 95.8 and 79.6%, respectively (P = .0004). OS and EFS did not highlight a significant difference in patients undergoing appropriate versus InFS (P = .8479, P = .2780, respectively).

Conclusions: InFS required intensified therapy to maintain excellent OS and EFS, so better anticipation of malignancy is required. Surgical staging of the retroperitoneal lymph nodes should be performed in patients ≥10 years old.
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http://dx.doi.org/10.1002/pbc.28479DOI Listing
September 2020

Testicular-Epididymal Dissociation: Vas and Vessels May "Lead up the Garden Path".

European J Pediatr Surg Rep 2019 Jan 13;7(1):e96-e99. Epub 2019 Dec 13.

Department of Paediatric Urology, Great Ormond Street Hospital For Children NHS Foundation Trust, London, United Kingdom of Great Britain and Northern Ireland.

The vas deferens and spermatic vessels entering the inguinal canal through the internal inguinal ring is thought to exclude an intra-abdominal testis. We present a case of high bilateral intra-abdominal testes on a 46,XY boy despite the vas deferens and good-sized vessels passing through the deep rings. Data were collected from clinical records, radiology (ultrasound, magnetic resonance imaging [MRI]), and endocrine blood tests. This case underlines the importance of following the pathway of embryological descent of the testis cranially as well as caudally during diagnostic laparoscopy, to avoid missing this rare anatomical variant.
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http://dx.doi.org/10.1055/s-0039-1688485DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6923713PMC
January 2019

Embryonal precursors of Wilms tumor.

Science 2019 12;366(6470):1247-1251

Wellcome Sanger Institute, Hinxton CB10 1SA, UK.

Adult cancers often arise from premalignant clonal expansions. Whether the same is true of childhood tumors has been unclear. To investigate whether Wilms tumor (nephroblastoma; a childhood kidney cancer) develops from a premalignant background, we examined the phylogenetic relationship between tumors and corresponding normal tissues. In 14 of 23 cases studied (61%), we found premalignant clonal expansions in morphologically normal kidney tissues that preceded tumor development. These clonal expansions were defined by somatic mutations shared between tumor and normal tissues but absent from blood cells. We also found hypermethylation of the locus, a known driver of Wilms tumor development, in 58% of the expansions. Phylogenetic analyses of bilateral tumors indicated that clonal expansions can evolve before the divergence of left and right kidney primordia. These findings reveal embryonal precursors from which unilateral and multifocal cancers develop.
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http://dx.doi.org/10.1126/science.aax1323DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6914378PMC
December 2019

Acoustic shadowing in pediatric kidney stone ultrasound: a retrospective study with non-enhanced computed tomography as reference standard.

Pediatr Radiol 2019 05 14;49(6):777-783. Epub 2019 Mar 14.

Department of Paediatric Radiology, Great Ormond Street Hospital for Children, Great Ormond Street, London, WC1N 3JH, UK.

Background: The usefulness of acoustic shadowing as a feature of pediatric kidney stone ultrasound (US) may be underestimated.

Objective: The hypothesis was that the majority of stones in children have acoustic shadowing and that its specificity is high (>90%) in pediatric kidney stones.

Materials And Methods: Our retrospective observational study included children who had undergone abdominal non-enhanced computed tomography (CT) for kidney stones in a pediatric renal stone referral centre between 2015 and 2016. US examinations prior to CT were retrospectively assessed for US features such as acoustic shadowing, twinkle artifact and stone size. These features were compared to CT as reference standard.

Results: Thirty-one patients (median age: 13 years, range: 1-17 years) with 77 suspected kidney stones were included. The median stone size was 5 mm (interquartile range [IQR]: 5 mm). For acoustic shadowing, sensitivity was 70% (95% confidence interval [CI] 56-80%) and specificity was 100% (95% CI 56-100%). All kidney stones with a diameter ≥9 mm demonstrated shadowing. Sensitivity for twinkle artifact was 88% (95% CI 72-96%), but specificity for twinkle artifact could not be calculated due to the lack of true negatives. All false-positive stones on US demonstrated twinkle artifact, but none showed shadowing.

Conclusion: Acoustic shadowing was demonstrated in the majority of pediatric kidney stones. Specificity was high, but this was not significant. Twinkle artifact is a sensitive US tool for detecting (pediatric) kidney calculi, but with a risk of false-positive findings.
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http://dx.doi.org/10.1007/s00247-019-04372-xDOI Listing
May 2019

Epidemiology of paediatric renal stone disease: a 22-year single centre experience in the UK.

BMC Nephrol 2017 Apr 18;18(1):136. Epub 2017 Apr 18.

Great Ormond Street Hospital for Children NHS Foundation Trust, Great Ormond Street, London, WC1N 3JH, UK.

Background: Whilst still rare, the incidence of paediatric stone disease is increasing in developed countries and it is important to evaluate the aetiology. We set up a dedicated renal stone service for children combining medical and surgical expertise in 1993 and now have a large case series of children to investigate the epidemiology.

Methods: A retrospective hospital note review of children presenting with kidney stones during the last 22 years (1993-2015) was conducted. All patients had a comprehensive infective and metabolic screen and were classified as metabolic, infective or idiopathic stone disease.

Results: Five hundred eleven patients (322 male) were reviewed. The median age of presentation was 4.4y for males (1 m-16.6y) and 7.3y (1-18.5y) for females with a median height and weight on the 25th centile for male and on 10th and 25th for female, respectively. One hundred seventy five (34%) had an underlying metabolic abnormality, 112 (22%) had infective stones and 224 (44%) were classified as idiopathic. Of the 175 patients with a metabolic abnormality: 91 (52%) had hypercalciuria (76 persistent and 15 transient), 37 (21%) hyperoxaluria, 38 (22%) cystinuria, 3 (2%) abnormalities in the purine metabolism and the remainder other metabolic abnormalities. Bilateral stones occurred in 27% of the metabolic group compared to 16% in the non-metabolic group (OR 0.2, p < 0.05). Urinary tract infection was a common complication (27%) in the metabolic group.

Conclusions: In this paper, we present the largest cohort of paediatric stone disease reported from a developed country giving details on both, clinical and laboratory data. We show that in the majority of the patients there is an identifiable underlying metabolic and/or infective aetiology emphasizing the importance of a full work up to provide adequate treatment and prevent recurrence. Moreover, we show that stone disease in children, in contrast to the adult population, does not seem to be associated with obesity, as children have a weight below average at presentation.
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http://dx.doi.org/10.1186/s12882-017-0505-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5395926PMC
April 2017

Nuclear Medicine in Pediatric Nephro-Urology: An Overview.

Semin Nucl Med 2017 05 6;47(3):204-228. Epub 2017 Mar 6.

Department of Radiology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK. Electronic address:

In the context of ante-natally diagnosed hydronephrosis, the vast majority of children with a dilated renal pelvis do not need any surgical treatment, as the dilatation resolves spontaneously with time. Slow drainage demonstrated at Tc-99m-mercaptoacetyltriglycine (MAG3) renography does not necessarily mean obstruction. Obstruction is defined as resistance to urinary outflow with urinary stasis at the level of the pelvic-ureteric junction (PUJ) which, if left untreated, will damage the kidney. Unfortunately this definition is retrospective and not clinically helpful. Therefore, the identification of the kidney at risk of losing function in an asymptomatic patient is a major research goal. In the context of renovascular hypertension a DMSA scan can be useful before and after revascularisation procedures (angioplasty or surgery) to assess for gain in kidney function. Renal calculi are increasingly frequent in children. Whilst the vast majority of patients with renal stones do not need functional imaging, DMSA scans with SPECT and a low dose limited CT can be very helpful in the case of complex renal calculi. Congenital renal anomalies such as duplex kidneys, horseshoe kidneys, crossed-fused kidneys and multi-cystic dysplastic kidneys greatly benefit from functional imaging to identify regional parenchymal function, thus directing further management. Positron emission tomography (PET) is being actively tested in genito-urinary malignancies. Encouraging initial reports suggest that F-18-fluorodeoxyglucose (FDG) PET is more sensitive than CT in the assessment of lymph nodal metastases in patients with genito-urinary sarcomas; an increased sensitivity in comparison to isotope bone scans for skeletal metastatic disease has also been reported. Further evaluation is necessary, especially with the promising advent of PET/MRI scanners. Nuclear Medicine in paediatric nephro-urology has stood the test of time and is opening up to new exciting developments.
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http://dx.doi.org/10.1053/j.semnuclmed.2016.12.002DOI Listing
May 2017

Isolated bilateral simplex ureteric ectopia: Bladder capacity as an indicator of continence outcome.

J Pediatr Urol 2017 Oct 6;13(5):493.e1-493.e9. Epub 2017 Mar 6.

Department of Paediatric Urology, Great Ormond Street Hospital NHS Foundation Trust, London, UK; Departments of Paediatric and Adolescent Urology, University College London Hospitals NHS Foundation Trust, London, UK. Electronic address:

Introduction: Isolated bilateral simplex ectopic ureters (BSEUs) are rare but pose a therapeutic challenge: ureteric reimplantation alone does not accomplish continence in all. Identifying the patients needing additional procedures for continence early could prevent multiple operations.

Objective: Potential preoperative indicators for postoperative continence are explored in eight BSEU girls without cloacal, anorectal, or spinal anomalies.

Study Design: With institutional approval, all patients with BSEU between 1985 and 2012 were retrospectively reviewed. Cystoscopy determined the site of ureteric ectopia (6 of 16 at the bladder neck [BN], 5 of 16 below the BN, and 5 of 16 in the distal urethra). Bladders were assessed by a combination of ultrasound, urodynamics, micturating cystourethrogram, cystoscopic, and intraoperative observations. Expected bladder capacity for age (EBCA) was calculated by 30 ml + (30 ml × age in years) or 38 ml + (2.5 ml × age in months) for children greater or less than 2 years, respectively. Continence outcomes were appraised at a minimum of 4 years. The small number of patients precludes credible statistical analysis and therefore raw data are presented.

Results: Patients underwent cross-trigonal ureteric reimplantation at 1-5.5 years, in five without BN surgery and in three with a Young-Dees-Leadbetter BN tightening. Of those without BN surgery at reimplantation, four achieved satisfactory continence for their age, but one has had multiple procedures culminating in BN closure, ileocystoplasty, and Mitrofanoff. Among the BN-tightening group, one was in nappies at 4 years, one had residual stress incontinence after two further BN injections, and one proceeded to artificial urinary sphincter after two BN injections. Five patients had significant renal impairment.

Discussion: Patients with satisfactory continence after reimplantation alone and those needing further procedures tended to differ in their preoperative observations of bladder capacity and apparent BN competence. This study suggests preoperative observations of an empty bladder on serial ultrasound and/or a wide-open BN with small or even moderate bladder capacity at cystoscopy to indicate the need for BN surgery. In contrast, children with bladder filling to at least 30% of expected bladder capacity for age on preoperative ultrasound or apposition of the BN at cystoscopy may achieve satisfactory continence after ureteric reimplantation alone. Bladder capacity as an indicator of BN competence can also be correlated to continence outcomes in previously published series. Polyuria associated with renal impairment can exacerbate the challenge for continence.

Conclusion: Preoperative bladder capacity appears to be an indicator of inherent BN function and a thorough assessment of the urinary tract by cystoscopy, ultrasound, micturating cystourethrogram, and functional imaging may guide the surgeon on the need for BN surgery at the time of ureteric reimplantation. Where continence remains elusive, patients should be counselled that a further BN injection is occasionally of value although more significant BN procedures are required for most.
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http://dx.doi.org/10.1016/j.jpurol.2016.12.032DOI Listing
October 2017

Cardiac Dysrhythmias in Children Undergoing Extracorporeal Shock Wave Lithotripsy Under General Anesthesia or Propofol Sedation: A Prospective, Observational Cohort Study.

Urology 2015 Nov 22;86(5):1008-12. Epub 2015 Jul 22.

Department of Anaesthesia, Great Ormond Street Hospital, London, United Kingdom.

Objective: To assess the frequency and severity of cardiac dysrhythmias and identify any intraoperative or postoperative complications in children undergoing extracorporeal shock wave lithotripsy (ESWL).

Methods: All children coming to our institution for ESWL from June 2014 to January 2015 were prospectively enrolled in an observational cohort study. Intraoperative cardiac dysrhythmias and perioperative and postoperative complications were recorded.

Results: In total, 21 children aged 1-18 years were enrolled receiving a total of 26 treatments. Intravenous sedation was used in 19 cases and general anesthesia with an inhalational agent in 7 cases. Cardiac dysrhythmias occurred in 58% of children. No hemodynamic instability was noted. No therapies were terminated because of dysrhythmias, and there were no postoperative cardiac dysrhythmias.

Conclusion: ESWL remains a safe therapy for children with urinary stone disease. Although we experienced more dysrhythmias than currently published literature, there were no long-term adverse outcomes and children were able to go home the same day.
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http://dx.doi.org/10.1016/j.urology.2015.07.017DOI Listing
November 2015

What is the risk of urinary tract infection in children with antenatally presenting dilating vesico-ureteric reflux?

J Pediatr Urol 2015 Apr 10;11(2):93.e1-6. Epub 2015 Mar 10.

Great Ormond Street Hospital for Children, Great Ormond Street, London, UK. Electronic address:

Introduction: The incidence of recurrent urinary tract infection (UTI) in children with primary vesico-ureteric reflux (VUR) presenting symptomatically is well documented. The risk of UTI in asymptomatic primary VUR diagnosed on investigation of antenatal hydronephrosis (ANH) is less clear. Paradoxically, several previous studies have suggested a lower risk (1-25%). We ascertain the incidence of UTI amongst antenatally-presenting primary VUR and explore risk factors.

Study Design: All patients <16 years managed for primary VUR between 1997 and 2013 were retrospectively reviewed. Patients were identified by searching 'VUR, vesicoureteric reflux' and 'vesico' in the clinical database. Sex, follow up, antibiotic prophylaxis, age at UTI, grade of VUR, radioisotope imaging findings (CRN-congenital reflux nephropathy, NRD-new renal defects), evidence of bladder dysfunction, surgical intervention and resolution were recorded. UTI diagnosis was based on positive urine culture with symptoms including fever. SPSS statistical package and Pearson's Chi-squared test were used to explore significance.

Results: Of 308 patients with primary VUR aged <16 years treated, 242 were diagnosed following presentation with UTI. The remaining 66 (21%) were initially asymptomatic, and VUR was diagnosed on investigation of ANH. All were given prophylaxis from birth. Six months to 16years (median 6years) follow-up was available for 54 (42 males, 12 females). All but two patients had grade III-V VUR (96%), bilaterally in 41 (76%). CRN was evident in 30 (56%; all male) and bladder dysfunction in 12 (22%; 10 males). Twenty-eight patients (52%) developed a UTI. The risk of UTI was 58% in girls, 33% in boys without CRN and 57% in boys with CRN (p = 0.17). Bladder dysfunction was a significant risk factor for UTI (p = 0.03). All 8 (15%; 7 males) with NRD had had a UTI. A single UTI appeared responsible for the majority of NRD (6/8; 75%). UTI occurred in 6/27 (22%) boys after circumcision compared to 17/25 (68%) prior/without circumcision (p < 0.05).

Conclusions: The incidence of UTI in VUR detected after presentation with ANH was 52%. CRN and bladder dysfunction were risk factors for developing a UTI. Circumcision appears to significantly reduce the risk of infection. Antenatal presentation of primary VUR does not carry a reduced risk of UTI. A single UTI, in half before the age of six months, seemed responsible for the majority of NRD. In boys, the highest risk of UTI is in the first few months of infancy, despite antibiotic prophylaxis, and other interventions, particularly circumcision, should therefore be considered as early as possible.
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http://dx.doi.org/10.1016/j.jpurol.2015.01.009DOI Listing
April 2015

Testicular outcome following laparoscopic second stage Fowler-Stephens orchidopexy.

J Pediatr Urol 2014 Feb 31;10(1):186-92. Epub 2013 Aug 31.

Department of Paediatric Urology, Great Ormond Street Hospital NHS Foundation Trust, Great Ormond Street, London, UK; Department of Paediatric Urology, University College London Hospitals NHS Foundation Trust, Euston Road, London, UK. Electronic address:

Objective: To assess outcome after laparoscopic second-stage Fowler-Stephens orchidopexy (L2(nd)FSO).

Patients And Methods: Retrospective review of 94 children (aged 0.75-16 years, median 2.75 years), who underwent L2(nd)FSO for 113 intra-abdominal testes between January 2000 and May 2009: 75 unilateral, 19 bilateral (11 synchronous; 8 metachronous). Follow-up (range 3 months-10.9 years, median 2.1 years) was available for 88 children (102 testes: 71 unilateral, 31 bilateral).

Results: Testicular atrophy occurred in 9 out of 102 (8.8%), including 8 out of 71 (11.3%) unilateral and 1 out of 31 (3.2%) bilateral intra-abdominal testes (multivariate analysis: p = 0.59). Testicular ascent ensued in 9 out of 102 (8.8%), comprising four (5.6%) unilateral and five (16.1%) bilateral testicles (multivariate analysis: p = 0.11). Of the 18 bilateral testes brought to the scrotum synchronously none atrophied and four (22.2%) ascended, compared to one (7.7%) atrophy and one (7.7%) ascent among the 13 testes brought to the scrotum on separate occasions (Fisher exact test: p = 0.42 and p = 0.37, respectively). Mobilization of the testis through the conjoint tendon tended towards less ascent (multivariate analysis p = 0.08) but similar atrophy (p = 0.56) compared to mobilization through the deep-ring/inguinal canal. Logistical regression analysis identified no other patient or surgical factors influencing outcome.

Conclusions: This is the largest series of L2(nd)FSO to date. A successful outcome is recorded in 85 out of 102 (83.3%) testicles. Atrophy occurred in 8.8% and ascent in 8.8%.
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http://dx.doi.org/10.1016/j.jpurol.2013.08.005DOI Listing
February 2014

Primary obstructive megaureter: cutting balloon endo-ureterotomy.

J Pediatr Urol 2013 Oct 5;9(5):692.e1-2. Epub 2013 Jun 5.

Department of Paediatric Urology, Great Ormond Street Hospital NHS Foundation Trust, London, UK.

Objective: In this video we will demonstrate endo-ureterotomy using a cutting balloon for vesico-ureteric junction (VUJ) dilatation and stenting of the primary obstructive megaureter.

Method: For the technique, a 0.014 inch guide-wire is endoscopically inserted through the VUJ and allowed to curl in the megaureter. A 3 mm atherotome-bladed cutting balloon is inflated with iohexol contrast solution. Under fluoroscopy or cystoscopically, the stenotic VUJ segment is observed to open and post-dilated with a 4 mm simple balloon before JJ stent placement for six weeks.

Results: This video demonstrates the equipment and technique of VUJ endo-ureterotomy using a cutting balloon and stenting of the primary obstructive megaureter.

Conclusion: Where intervention for the primary obstructive megaureter is indicated, we propose VUJ endo-ureterotomy as the first line treatment.
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http://dx.doi.org/10.1016/j.jpurol.2013.04.010DOI Listing
October 2013

Voluminous "cobra-head" stone in a duplex system ureterocele: combined cysto-ureteroscopic and percutaneous cystolithotomy approach.

J Laparoendosc Adv Surg Tech A 2013 May;23(5):484-5

Department of Paediatric Urology, Great Ormond Street Hospital NHS Foundation Trust, London, UK.

We report a case of a 7-year-old boy with a voluminous 8.5-cm "cobra-head" calculus in a duplex system ureterocele causing flank pain, hematuria, and infection. Combined cysto-ureteroscopy and percutaneous cystolithotomy enables dependent ureterocele incision along its inferolateral border and efficient stone clearance in a single minimally invasive procedure. Protracted and repeated urethral instrumentation is avoided.
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http://dx.doi.org/10.1089/lap.2012.0495DOI Listing
May 2013

Surgical technique: utriculus masculinum excision by a laparoscopic-cystoscopic approach.

J Pediatr Urol 2013 Jun 10;9(3):388.e1-388.e2. Epub 2013 Feb 10.

Department of Paediatric Urology, Great Ormond Street Hospital NHS Foundation Trust, Great Ormond Street, London WC1N 3JH, UK.

Objective: A utriculus masculinum is encountered in 12% of hypospadias. However, patients rarely present with symptoms, such as post-void incontinence or infection. We describe a combined laparoscopic-cystoscopic approach to overcome the challenge posed by the location of the utriculus deep within the pelvis and to accurately identify the confluence of the utriculus with the urethra.

Methods: A 12-year-old boy with Williams syndrome and previous hypospadias surgery presented with new-onset progressive day-time incontinence. Investigations (cystoscopy, urodynamics, MCUG) demonstrated a normal caliber urethra and the incontinence to result from a large utriculus masculinum. This video demonstrates the surgical technique: simultaneous utriculoscopy enabled the dome of the utriculus to be identified, laparoscopic dissection to progress under guidance of transillumination, and the urethral-utricular convergence to be clarified.

Results: Histology demonstrated an inflamed utriculus with squamous metaplasia and cystitis glandularis. Symptoms resolved post-operatively and at 4 months follow-up, the patient remains dry by day.

Conclusions: A utriculus masculinum rarely requires excision. A combined laparoscopic-cystoscopic approach facilitates the delineation of the utriculus and allows its confluence to the urethra to be accurately determined.
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http://dx.doi.org/10.1016/j.jpurol.2012.12.014DOI Listing
June 2013

Incidence of Deflux® calcification masquerading as distal ureteric calculi on ultrasound.

J Pediatr Urol 2013 Dec 24;9(6 Pt A):820-4. Epub 2012 Nov 24.

Department of Paediatric Urology, Great Ormond Street Hospital NHS Foundation Trust, Great Ormond Street, London WC1N 3JH, UK. Electronic address:

Objective: Dextranomer-hyaluronic acid (Deflux(®)), the most widely used compound in the endoscopic treatment of vesico-ureteric reflux (VUR) today, is believed to provoke only minimal inflammation. Reports of calcification of Deflux(®) are increasing. We ascertain the incidence of Deflux(®) calcification appearing as distal ureteric calculi on ultrasound.

Methods: Three cases (2 external patients) of ureteroscopy for calcified submucosal Deflux(®) prompted a retrospective review of the notes and imaging of all children treated with Deflux(®) for VUR between December 2000 and January 2011 at Great Ormond Street Hospital.

Results: 232 children (M:F = 5:3) received Deflux(®) for VUR at median age 2 years (range 2 months-12 years). Follow-up annual ultrasound, performed in all, identified calcification in 2. The interval between Deflux(®) injection and presentation of its calcification was 4 years. 104 of the 232 children had been followed up for 4-10 years. Considering the observed lag-period, after 4 years the incidence of calcification of Deflux(®) on ultrasound was 2% (2/104).

Conclusions: Patients should be warned that calcification of Deflux(®) can occur. Misinterpretation as ureteric stones is common and may lead to unnecessary ureteroscopy. In this series, the incidence of calcification of Deflux(®) on ultrasound after 4 years was 2%.
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http://dx.doi.org/10.1016/j.jpurol.2012.10.025DOI Listing
December 2013

Persistent non-visualisation of the fetal stomach: diagnostic and prognostic implications.

Arch Dis Child Fetal Neonatal Ed 2010 Nov 10;95(6):F439-42. Epub 2010 Jun 10.

Fetal Medicine Unit, Elizabeth Garrett Anderson Wing, University College Hospital, London, UK.

Design: All fetuses diagnosed with 'absent stomach' at anomaly screening over an 8-year period were identified using the University College Hospital fetal medicine database. These were cross-referenced with records from the paediatric surgical unit at Great Ormond Street Hospital and pathology department at University College Hospital to ascertain postnatal or postmortem diagnosis and outcome in each case.

Results: Of the 84 cases identified, eight were found to have normal stomachs on subsequent antenatal scans, while 76 had persistent non-visualisation of the stomach. Underlying diagnoses included 24 gastro-intestinal tract and/or respiratory anomalies, 22 aneuploidies, six neuromuscular syndromes, three central nervous system anomalies, seven renal anomalies and five genetic syndromes. Seven cases had no identifiable postnatal abnormalities, 26 pregnancies were terminated and nine fetuses died in utero. Of the 33 live births, eight died in the neonatal period and three died in infancy. Only 28 survived into childhood. Two patients were lost to follow up.

Conclusions: Persistent non-visualisation of the fetal stomach in the antenatal period was associated with a wide range of underlying diagnoses. In many cases, prognosis was poor. Only 37% of pregnancies resulted in liveborn infants surviving more than 6 months. The incidence of an abnormal karyotype was 29%. Diagnosis and outcome was normal in only 9.2% of cases. We propose an algorithm for the management of persistent non-visualisation of the fetal stomach on antenatal ultrasound.
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http://dx.doi.org/10.1136/adc.2009.179341DOI Listing
November 2010

The predictive value of a repeat micturating cystourethrogram for remnant leaflets after primary endoscopic ablation of posterior urethral valves.

J Pediatr Urol 2011 Apr;7(2):203-8

Department of Paediatric Urology, Great Ormond Street Hospital NHS Trust, London WC1N 1EH, UK.

Objective: We routinely perform a cystourethroscopy 3 months after initial ablation of posterior urethral valves. The aim of this study was to determine the predictive value of the urethral appearance on preoperative micturating cystourethrogram (MCUG) for further valve resection at check cystoscopy.

Patients And Methods: We retrospectively reviewed 31 consecutive boys (aged 4-18 months) who underwent check cystoscopy and repeat MCUG between 2006 and 2008.

Results: Repeat MCUG suggested remnant valves in 10, but no residual leaflets were identified cystoscopically in 4. In 20 boys, the valves appeared completely ablated on MCUG but valve leaflets received further resection in 10. One study was undiagnostic. Residual valves were resected in 83% (5/6) where valves and urethral dilatation were noted on MCUG. Where MCUG suggested either valves or persistent dilatation alone, further resection occurred in 40% (4/10). Remnant leaflets were also present in half of those (7/14) in whom the repeat MCUG had shown complete ablation and resolved/reduced posterior urethral dilatation.

Conclusions: The positive predictive value of valve leaflets and/or posterior urethral dilatation on repeat MCUG for subsequent resection of valve remnants was 56%; the negative predictive value was 50%. We found repeat MCUG alone imprecise in excluding residual valve tissue and recommend check cystoscopy in all.
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http://dx.doi.org/10.1016/j.jpurol.2010.04.011DOI Listing
April 2011

Retroperitoneoscopic para-aortic lymph node sampling in bladder rhabdomyosarcoma.

J Pediatr Urol 2010 Apr 13;6(2):185-7. Epub 2009 Aug 13.

Departments of Urology and Oncology, Great Ormond Street Hospital NHS Trust, Great Ormond Street, London WC1N 3JH, United Kingdom.

Determining lymph node involvement is an important step in the pre-treatment evaluation of non-metastatic rhabdomyosarcoma. We describe retroperitoneoscopy for para-aortic lymph node biopsy in a 4-year-old boy with embryonal rhabdomyosarcoma of the bladder with pelvic and para-aortic lymph node enlargement on magnetic resonance imaging. This technique affords access to the para-aortic region with minimal dissection, permitting quick recovery and early commencement of chemotherapy.
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http://dx.doi.org/10.1016/j.jpurol.2009.07.008DOI Listing
April 2010

Adenocarcinoma at the ureterosigmoidostomy site in a 16-year-old demonstrates the importance of screening in children.

J Pediatr Urol 2008 Jun 26;4(3):234-5. Epub 2007 Nov 26.

Department of Urology, Great Ormond Street Hospital for Children NHS Trust, Great Ormond Street, London WC1N 3JH, UK.

We report the death of a 17-year-old male exstrophy patient from adenocarcinoma arising at the ureterosigmoidostomy. This was detected at reconstructive surgery at the age of 16 years, 13 years after ureterosigmoidostomy. This case highlights the importance of including children in endoscopic surveillance. We recommend annual sigmoid-colonoscopy commencing 10 years after ureterosigmoidostomy irrespective of age.
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http://dx.doi.org/10.1016/j.jpurol.2007.10.004DOI Listing
June 2008

BCG vaccine-associated suppurative lymphadenitis.

Vaccine 2005 Apr;23(20):2676-9

Centre for Child Health, Barts and the London School of Medicine and Dentistry, Queen Mary, University of London, 38 New Road, London E1 2AX, UK.

Clusters of BCG vaccine-associated suppurative lymphadenitis (BCG-ASLD) have been reported in many countries. We have observed an increase in BCG-ASLD following the change from the percutaneous Evans vaccine to the intradermal BCG Statens Serum Institute (SSI) vaccine.
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http://dx.doi.org/10.1016/j.vaccine.2004.07.052DOI Listing
April 2005