5th Joint Meeting of ESPU-SPU - Virtual

S4: CASE REPORT (parallel session, room 2)

Moderators: Marco Castagnetti (Italy)

ESPU-SPU Meeting on Thursday 23, September 2021, 15:00 - 15:42


15:00 - 15:02
S4-1 (CRP)

A UNIQUE CASE OF PENILE PROSTHESIS IMPLANTATION IN A PATIENT WITH CONGENITAL MEGALOURETHRA.

Alfredo BERRETTINI 1, Michele GNECH 1, Dario Guido MINOLI 1, Erika Adalgisa DE MARCO 1, Vincenzo MIRONE 2, Luigi DI LUISE 2, Marco CAPECE 2, Alessandro PALMIERI 2, David RALPH 3, Massimo DI GRAZIA 4 and Gianantonio MANZONI 1
1) Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Paediatric Urology Unit, Milan, ITALY - 2) Azienda Ospedaliera Universitaria "Federico II", Urology Unit, Naples, ITALY - 3) University College Hospital, Urology Unit, Milan, ITALY - 4) Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Urology Unit, Milan, ITALY

PURPOSE

A 20-year-old young man born with congenital megalourethra came back to our department complaining of refractory lifelong erectile dysfunction. Past medical history included urinary retention, recurrence UTI, bilateral VUR and renal impairment. He underwent to a definitive 2 kidney’s transplant, a partial urethrectomy for seborrheic emission and a definitive perineal urethrostomy .

MATERIAL AND METHODS

Past medical history included urinary retention, recurrence UTI, bilateral VUR and renal impairment. He underwent to a definitive 2 kidney’s transplant, a partial urethrectomy for seborrheic emission and a definitive perineal urethrostomy .

RESULTS

MRI of the penis showed scafoid megalourethra and absence of the distal part of both corpora cavernosa. The patient underwent implantation of an inflatable penile prosthesis (Titan® OTR, Coloplast S.p.A) device after intravenous administration of Gentamicin and Cefazoline. Surgery was carried out through a longitudinal peno-scrotal incision in order to guarantee adequate exposure of the corpora from the crura to the distal tips. After the corporotomies, dilation of both corpora was performed. As expected, distal dilation was possible up to three quarters of the shaft.

CONCLUSIONS

The patient is successfully using the prosthesis with satisfactory erections after two years of follow-up. To our knowledge this is the first successful report of inflatable penile prosthesis in a patient born with a scafoid megalourehra.


15:02 - 15:04
S4-2 (CRP)

GENITAL OEDEMA AND CROHN'S DISEASE IN CHILDREN- THE MISSING LINK!

Neetu KUMAR 1, Fevronia KIPARISSI 2 and Abraham CHERIAN 1
1) Great Ormond Street Hospital, Paediatric Urology, London, UNITED KINGDOM - 2) Great Ormond Street Hospital, Paediatric Gastroenterology, London, UNITED KINGDOM

PURPOSE

Genital manifestations of Crohn's disease are rare. We present a case series of children presenting to the Paediatric Urology out-patients with genital oedema as the only sign of granulomatous disease without any gastrointestinal symptoms.

MATERIAL AND METHODS

We retrospectively reviewed consecutive children presenting with chronic genital oedema between the years 2013-2019. The chronicity of the oedema was defined as lasting at least 6 months. These children had been followed up by a variety of clinicians including General Practitioners, A&E clinicians, Paediatricians, Dermatologists, adult Urologists and Gynaecologists. A wide array of investigations was reviewed including ultrasound soft tissue, MRI pelvis, upper and lower GI endoscopy with biopsies, genital skin biopsies, as well as specific haematological and faecal test such as calprotectin.

RESULTS

Five children [1 girl, 4 boys; mean age of symptom onset 11 years (range 8-14 years)] with chronic genital oedema were included. None of the children had any gastrointestinal symptoms. Penile and/or scrotal oedema was noted in boys and clitoral hood oedema in the girl. Genital skin biopsy demonstrated the presence of specific non-caseating granulomata. All other investigations including gastrointestinal biopsies were normal. Treatment with biologic immunomodulators was offered in 4 patients with resolution of symptoms.

CONCLUSIONS

In a child with no preceding gastrointestinal symptoms, genital oedema could be the first and only presenting feature of Crohn's disease. Repeat skin biopsies may be required for diagnosis. This case series alerts urologists to the existence of this entity, and stresses the need to have a targeted investigative pathway for early confirmation and treatment.


15:04 - 15:06
S4-3 (CRP)

THE DOUBLE BARREL SHOTGUN TECHNIQUE FOR CONSERVATIVE MANAGEMENT OF COMPLETE DIPHALLIA.

Martin KAEFER 1, Fernando GONZALEZ 2 and Javier BOLANOS 2
1) Riley Hospital for Children, Urology, Indianapolis, USA - 2) Roosevelt Hospital, Pediatric Surgery, Guatemala City, GUATEMALA

PURPOSE

Diphallia is an exceedingly rare form of structural DSD that occurs in less than one in 5 million births. Variable degrees of penile and urethral development can be seen with complete duplication of the penis and urethra being the least common.  In this era of concern regarding surgery for children with DSD, one option is to postpone surgical decisions until the child reaches the age of assent/consent.  However, in cases where surgery is undertaken, it should be the surgeon’s primary goal to preserve as much genital tissue as possible.  The Double Barrel Shotgun Technique is the most conservative surgical procedure that can be used to achieve a functional and cosmetically acceptable phallus.  

MATERIAL AND METHODS

An incision is made from the medial aspect of the coronal margin of one phallus to that of the other. Both penises are degloved, Buck’s fascia is reflected and the medial aspects of the two phalluses are brought into apposition with a series of inverted 4-0 polypropylene suturess spaced at 3 mm intervals. A small segment of the medial facing half of each glans is removed and the two glans components are combined.  The two functional urethras are left separate.  Skin is reconstructed to provide optimal coverage.   

RESULTS

Two patients underwent this procedure.  The procedure was performed in under two hours.  In both cases the penis was documented to be straight postoperatively.  In one case the child is old enough to confirm the presence of a completely straight penis with full erections.  Both children void simultaneously through both urethras. 

CONCLUSIONS

The Double Barrel Shotgun Technique for preservation of both penises in patients with complete diphallia is simple in concept and provides an excellent cosmetic and functional result.  By avoiding corporotomies, minimal risk is incurred to the erectile mechanism of either penis.  By leaving the urethras separate there is no risk of urethral injury or stricture.  


15:06 - 15:08
S4-4 (CRP)

PENILE TUMOR IN AN ADOLESCENT BOY - A VERY RARE CASE

Marcel DRLÍK, Josef SEDLÁČEK, Zuzana VAĹOVÁ and Radim KOČVARA
General Teaching Hospital and Charles University 1st Faculty of Medicine, Urology, Prague, CZECH REPUBLIC

PURPOSE

In contrast to the adults, penile tumors are extremely rare in children and adolescents. We present a case of penile myointimoma. Very few cases have been reported in the literature so far.

MATERIAL AND METHODS

A 15- year old boy presented for a 6 months history of a slowly growing, hard, whitish nodule within the glans, around 1 cm in diameter, visible under the unchanged skin, without palpable groin lymphonodes. He referred no history of trauma.

RESULTS

According to the EAU guidelines for penile cancer in adult men we performed the Doppler Ultrasonography (DUS) and Magnetic Resonance Imaging (MRI). DUS revealed hypoechogenic, hypoperfused non well-defined area inside the glans spongiosum. MRI did not confirm any other pathologic mass inside corpora cavernosa. A mass excision with peroperative biopsy was decided. Biopsy and subsequent detailed histopathological study revealed histological changes diagnostic of myointimoma (benign tumor characterized by nodular structures derived from the intima of the cavernous vessels, formed of elongated cells with no atypia or mitoses). Due to the benign nature of the lesion, staging for distant metastases was not necessary and we simply performed an outpatient follow-up. Ten months after the excision, there is no local recurrence, no urethral stricture and the cosmetic appearance is good.

CONCLUSIONS

Both clinician and pathologist must be aware of this rare benign pathology. Organ sparing surgery is a method of choice. Local excision is safe and effective treatment modality.


15:08 - 15:10
S4-5 (CRP)

Y-TYPE DUPLICATION OF URETHRA AND MEGALOURETHRA: WHEN THEY APPEAR TOGETHER

Sara LOBO 1, May BISHARAT 2, Jan TRACHTA 3 and Imran MUSHTAQ 4
1) Great Ormond Street Hospital for Children NHS Foundation Trust, Urology, London, UNITED KINGDOM - 2) Great Ormond Street Hospital for Children NHS Foundation Trust, Paediatric Urology, London, UNITED KINGDOM - 3) Motol University Hospital, Charles University in Prague, Paediatric Surgery and Paediatric Urology, Prague, CZECH REPUBLIC - 4) Hospital for Children NHS Foundation Trust, London WC1N 3JH., Paediatric Urology, London, UNITED KINGDOM

PURPOSE

Y-type urethral duplication is a rare congenital anomaly, commonly associated to other very well established anomalies, including anorectal malformation and hemivertebrae. The concomitant presence of a megalourethra has only been described in a single case in the literature.

MATERIAL AND METHODS

We describe 3 cases of children with this association along with the complex and distinct management required.

RESULTS

Two of the children had a diagnosis of Y-type duplication in neonatal period and the third at 3 years of age. Other congenital malformations were present in 2, including cardiac anomalies, anorectal malformation and distal tracheo-esophageal fistula. On clinical examination, the diagnosis of megalourethra was obvious in 2 patients and the third had the diagnosis by MCUG. All patients had a hypoplastic corpora cavernosa and a similar pattern of voiding through a ventral urethra with small volume leaks from an orthotopic urethra. Since the urethral anatomy was slightly different in each other, the management was performed according to the findings. Two children are undergoing a 2-staged urethroplasty, one performs CIC per perineal fistula.

CONCLUSIONS

Y-type urethral duplication in association with megalourethra is a rare finding. Y-duplication needs to be considered in children presenting with megalourethra. We highlight the importance of early diagnosis to tailor appropriate treatment towards the patient with view to minimising unnecessary procedures, surgical morbididities and improving outcomes.


15:10 - 15:15
Discussion
 

15:15 - 15:17
S4-6 (CRP)

MANAGEMENT OF UROLOGICAL COMPLICATIONS IN 2 BROTHERS WITH JUNCTIONAL EPIDERMOLYSIS BULLOSA

Yaqoub JAFAR 1, Delphine DEMEDE 1, Bruno RANCHIN 2, Pierre-Yves MURE 1 and Pierre De MOURIQUAND 1
1) HFME, Pediatric urology, Bron Cedex, FRANCE - 2) Hfme, Pediatric Nephrology, Bron Cedex, FRANCE

PURPOSE

Epidermolysis bullosa (EB) is a rare and potentially lethal inherited cutaneous-mucosal disorder. Renal and urinary tract complications are found in 15-30% of EB, and are more frequent in junctional sub-type. These complications can lead to end-stage renal disease and death. We report surgical management of 2 brothers with junctional EB and severe urinary tract complications.

MATERIAL AND METHODS

The older brother (A) presented an antenatal diagnosis of hydramnios. He had a chronic bladder dysfunction, with recurrent UTIs, dysuria, painful micturitions since the age of 2. Symptoms gradually increased with hydronephrosis leading to chronic renal disease and hypertension at age 8. Alpha-blocker, oxybutinin and suprapubic catheter provided a partial improvement of renal function.
Younger brother (B) had chronic bladder dysfunction at age 4 followed by severe hypertension, dilated cardiomyopathy and renal failure. He was first treated with an undwelling suprapubic catheter leading to partial renal function and cardiac recovery. Both had major urethral damage making catheterization impossible.

RESULTS

Persistent upper tract dilatation and recurrent UTI led to enterocystoplasty with a Mitrofanoff conduit in both children. With a 24 month post-operative follow-up, no complications were recorded, a stable renal function in A, improved in B, with a better growth in both, less UTIs, improved quality of life after surgery, better school performance.

CONCLUSIONS

Enterocystopasty with Mitrofanoff procedure appeared useful for the management of severe urological complications in junctional epidermolysis bullosa to improve renal function, growth and quality of life.


15:17 - 15:19
S4-7 (CRP)

RENAL TUMORS DIAGNOSED PRENATALLY

Gabriela GROCHOWSKA 1, Piotr GASTOL 1, Agnieszka BROŻYNA 2, Bożenna DEMBOWSKA-BAGIŃSKA 2 and Malgorzata BAKA-OSTROWSKA 1
1) CHILDREN'S MEMORIAL HEALTH INSTITUTE IN WARSAW, PAEDIATRIC UROLOGY, Warsaw, POLAND - 2) CHILDREN'S MEMORIAL HEALTH INSTITUTE IN WARSAW, PAEDIATRIC ONCOLOGY, Warsaw, POLAND

PURPOSE

To evaluate the outcome of children with prenatally diagnosed renal tumors.

MATERIAL AND METHODS

We analyzed medical database of 17 newborns with primary renal tumors resected since 2005. There were 4 (23.5%) children in this group (2 boys and 2 girls) with prenatally diagnosed renal mass. Prenatal findings, clinical records, radiological, surgical and pathological reports were reviewed.

RESULTS

Renal mass in all patients was detected on ultrasound examination performed in the third
trimester. Tumors were described as hypoechoic heterogeneous solid renal mass. Two of them
were confirmed by fetal MRI. Complications during the perinatal period were identified in 1
pregnancy (threatened preterm birth). Median gestational age of the 4 neonates was 35,5 weeks
(range 34 to 38), including 1 newborn who was pre-term. All children were born via cesarean
section. Hypertension after birth was detected in 2/4 patients. Three children underwent unilateral
nephrectomy in the first week of life without intraoperative complications. In one newborn because
of nonspecific sonographic image, abdominal CT and urinary VMA level were performed. Finally,
prenatal adrenal hemorrhage was suspected. After 3 weeks of observation, because of
persistence of tumor, patient received pre-chemotherapy (VCR) and underwent nephrectomy at 5
months of age. Wilms tumor (stage 1) was diagnosed.
Histopathological examination showed congenital mesoblastic nephroma (CMN) in 3/4 patients
(2 cellular variant and 1 mixed subtype) and Wilms tumor in 1 patient. All children are alive from 11 to 84 months from diagnosis (median 30.7 mo).

CONCLUSIONS

1. Congenital mesoblastic nephrons is the most common fetal renal neoplasm in our material
2. After prenatal diagnosis of renal mass fetal MRI or at birth is recommended


15:19 - 15:21
S4-8 (CRP)

CYTOPENIA IN GENITAL MALFORMATIONS: WATCH OUT FOR MIRAGE SYNDROME

Arthur LAURIOT DIT PREVOST 1, Dyuti SHARMA 1, Benedicte BRUNO 2, Maryse CARTIGNY-MACIEJEWSKI 3, Sylvie MANOUVRIER 4 and Rémi BESSON 1
1) Univ. Lille, CHU Lille, Clinique de Chirurgie et Orthopédie de l'Enfant, Centre de référence du développement génital (DEV-GEN), Lille, FRANCE - 2) Univ. Lille, CHU Lille, Service d'Hématologie pédiatrique, Lille, FRANCE - 3) Univ. Lille, CHU Lille, Service d'endocrinologie pédiatrique, Centre de référence du développement génital (DEV-GEN), Lille, FRANCE - 4) Univ. Lille, CHU Lille, Service de génétique clinique, Centre de référence du développement génital (DEV-GEN), Lille, FRANCE

PURPOSE

MIRAGE syndrome (Myelodysplasia, Infection, Restriction of growth, Adrenal hypoplasia, Genital phenotypes and Enteropathy) is associated with SAMD9 mutation and loss of chromosome 7. In the initial description on 11 patients, 6 out of 7 46XY patients exhibited genital underdevelopment, and all had thrombocytopenia and/or anemia (Narumi et al. Nature Genetics 2016;48(7):792–7).

CASE REPORT

We present the case of a boy, referred at 17 months for a perineal hypospadias with severe chordee. Karyotype and testosterone level at birth were normal. Apart from the genital phenotype, endocrine assessment showed a mild testicular dysgenesis, a normal rate of ACTH, and weight was 9.7kg (-1 standard deviation).

Urethroplasty was performed at the age of 21 months, preoperative hematological assessment showed moderate leukocytopenia (WBC 4940/mm3, Platelets 129000/mm3, Hemoglobin 12.5g/dl), and post-operative control showed anemia (8g/dl, stable over 5 days), leukocytopenia (2350/mm3, decreasing), and improvement of the platelets (140000/mm3).

Five days after hospital discharge, he was admitted for sepsis — with no clinically identifiable cause — associated with pancytopenia. Medullar karyotype showed a loss of chromosome 7, but pancytopenia resolved spontaneously. Because of the poor prognosis of myelofibrosis, bone marrow transplant was planned. But since pre-transplantation medullar karyotype did not found monosomy 7, transplantation was cancelled. After the publication of the MIRAGE syndrome, CGH-array detected a mutation in SAMD9.

CONCLUSIONS

Pediatric urologist care young patients with genital anomaly, and usually request pre- operative hematological assessment. We should be aware of this recently described —and thus possibly under diagnosed— entity in our patients with genital anomaly.


15:21 - 15:23
S4-9 (CRP)

LASER FULGURATION OF MULTIPLE VENOUS MALFORMATIONS OF BLADDER IN AN ADOLESCENT WITH KLIPPEL - TRENAUNAY SYNDROME

Virender SEKHON 1, Praful MISHRA 2 and Manav SURYAVANSHI 2
1) Medanta- The Medicity, Urology, Renal transplant and Robotic Surgery, Gurugram, INDIA - 2) Medanta - The Medicity, Urology, Renal transplant and Robotic Surgery, Gurugram, INDIA

PURPOSE

Congenital venous malformations (VM) of the urinary tract are a very rare disorder, representing less than 0.6% of all bladder lesions. We describe endoscopic fulguration of multiple bladder lesions in an adolescent with Klippel - Trenuanay (KT) syndrome.

MATERIAL AND METHODS

A 12 year old boy presented with multiple episodes of painless gross hematuria. He was a known case of KT syndrome and had undergone multiple surgeries for rectal hemangiomas before. Medical treatment for bladder VMs had failed. Contrast enhanced computed tomography of the abdomen showed a partially delineated, heterogeneously enhancing bladder dome lesion of size 2 X 2 cms, along with mild thickening of surrounding walls.

Cystoscopic examination showed multiple bluish sessile VMs of variable sizes, ranging from 0.5 to 1.5cms in the supra-trigonal region of bladder. An endoscopic fulguration with Holmium laser [1 Joule, 10 Hz] was performed for majority of the larger lesions.

RESULTS

The operative duration was 45 mins and the blood loss was 10 ml. The hematuria episodes did not recur after surgery and the patient remains asymptomatic on a one year follow-up, with a good bladder capacity.

CONCLUSIONS

Multiplicity of lesions, along with location, size and severity of symptoms should be accounted for while deciding the management strategies for VMs of bladder. Laser fulguration of multiple VMs of bladder is a safe and successful treatment modality.


15:23 - 15:25
S4-10 (CRP)

A RARE CASE WITH BOTH ANTERIOR URETHRAL VALVE AND POSTERIOR URETHRAL VALVE

Aykut AKINCI 1, Murat Can KARABURUN 2, Can Utku BAKLACI 2, Perviz HAJIYEV 3, Tarkan SOYGUR 1 and Berk BURGU 1
1) Ankara University School of Medicine, Pediatric Urology, Ankara, TURKEY - 2) Ankara University School of Medicine, Urology, Ankara, TURKEY - 3) HB Guven Clinic, Pediatric Urology, Baku, AZERBAIJAN

PURPOSE

Posterior urethral valve (PUV)s are the most common cause of lower urinary tract obstruction in pediatric patients.Anterior urethral valve(AUV)is a rare but well-known cause of lower urinary tract obstruction congenital-anomaly.The concomitant both an AUV&PUV is an extremely rare congenital-abnormality. We aimed to demonstrate endoscopic diagnosis and treatment in a patient with both anterior urethral and posterior urethral valve.

MATERIAL AND METHODS

A 26-year-old(gravida 2) mother presented for ultrasonographic examination at 20-weeks gestation and was found bilateral gradeI renal dilatation, bilateral dilated ureters, increased bladder size (30*25 mm) and mild oligohydramnios on antenatal 20.week ultrasonography. The patient was delivered via cesarean-section at 37.weeks.On examination of the newborn, there was no anomaly.The creatinine was 2.49 mg/dl at 3. day.Postnatal ultrasonography was performed and revealed bilateral dilated ureters, bilateral Grade III pelvicaliectasis,diffuse increased bladder thickness and dilated posterior urethra.The urethral catheter was placed. The creatinine was regressed(2.49 mg/dl to 1.53 mg/dl).A voiding cystogram was performed and revealed concomitant both an AUV&PUV at postnatal 1. week.Cystourethroscopy and circumcision were performed and concomitant both AUV&PUV were found and incised at the age of 2weeks.

RESULTS

The creatinine was regressed (1.53 mg/dl to 0.99 mg/dl). No postoperative-complication was observed. The urethral catheter removed at postoperative 3.day. The infant was discharged 4 days later with normal spontaneous-bladder-voiding.

CONCLUSIONS

Congenital concomitant both AUV&PUV an uncommon but important cause of lower urinary tract obstruction.Early-diagnosis and management of this rare condition are very important to prevent further damage, infection and end-stage renal failure.


15:25 - 15:30
Discussion
 

15:30 - 15:32
S4-11 (CRP)

BLADDER AGENESIS, BILATERAL ECTOPIC URETERS DRAINING INTO THE VAGINA, COLIC ATRESIA AND HIRSCHSPRUNG DISEASE IN A FEMALE INFANT.

Francesco LACONI 1, Charline BISCHOFF 1, Nadia BOUDAOUD 1, Marta SPODENKIEWICZ 2 and Marie-Laurence POLI-MEROL 1
1) CHU Reims, Pediatric Surgery and Urology, Reims, FRANCE - 2) CHU Reims, Genetics, Reims, FRANCE

PURPOSE

Complete agenesis of the bladder is an extremely rare congenital anomaly that and has been reported in few patients. We report the case of 38 weeks of pregnancy female newborn who presented bladder agenesis with bilateral ectopic ureters draining into the vagina, sigmoid-descending-transverse colic atresia and Hirschsprung disease.

MATERIAL AND METHODS

The patient was transferred on day 1 of life from an outside hospital for delayed passage of meconium and several episodes of bilious vomiting. She had no routine prenatal US controls, parents were no consanguineous and we found a 46 XXFRA(10)(Q25.3) karyotype. We conducted a laparotomy in emergency and we found a sigmoid-descending-transverse colic atresia with a huge dilated right colon. An ascending colostomy was realized, and additionally, colon-rectal biopsies (routinely performed) showed absence of ganglion cells on histological analysis. On the other hand, abdominal US showed bilateral hydroureteronephrosis with no signs of bladder and on the urography we found bilateral ectopic ureters with vaginal implantation. No bladder cavity was visualized, that confirm the US suspect of bladder agenesis.

RESULTS

We performed a transanal pull-through (Soave) at 6 month of life and, at 3 years of life, an ileocystoplasty with bilateral ureteral reimplantation (Lich-Gregoir) and continent ileal catheterizable cystostomy.

CONCLUSIONS

At the last control, patient aged 18 years, we found no hydroureteronephrosis, there was no leakage from the cystostomy and the patient conducted clean intermittent self-catheterization.  The renal function was stable.


15:32 - 15:34
S4-12 (CRP)

URETEROSCOPIC LASER INFUNDIBULOTOMY FOR SYMPTOMATIC CALYCEAL DIVERTICULUM

David COYLE 1, Salvatore CASCIO 2 and Rustom MANECKSHA 3
1) Children's Health Ireland at Crumlin, Department of Paediatric Surgery, Dublin, IRELAND - 2) Children's Health Ireland at Temple Street, Department of Paediatric Surgery, Dublin, IRELAND - 3) Tallaght University Hospital, Department of Urology, Dublin, IRELAND

PURPOSE

Calyceal diverticulum is characterized by outpouching of a renal calyx lined by non-secretory transitional epithelium. While many are asymptomatic, some present with intermittent pain, infection or stone formation within the diverticulum due to a stenotic infundibulum. We aimed to describe the operative management of this rare problem.  

PATIENTS AND METHODS

The medical, radiological and operative records for a patient referred to a tertiary paediatric hospital with a symptomatic calyceal diverticulum confirmed on intravenous urography and computed tomography. Patient demography, method of identification of the infundibulum, infundibulotomy technique, post-operative drainage/stenting and clinical outcome were all recorded.

RESULTS

One male (aged 14 years) with symptomatic calyceal diverticulum, presenting with flank pain, was identified. The calyceal diverticulum was 24mm x 23mm in maximum dimension.  Day-case flexible ureterorenoscopy and laser infundibulotomy was performed. The infundibulum was identified using the Blue Spritz technique (a fluoroscopic-endoscopic technique utilizing contrast and methylene blue). Infundibulotomy was performed using Holmium laser (270μm fibre, 500mJ, 5Hz respectively, short pulse duration). A double-J stent was placed. The patient is currently pain-free at 6 months.

CONCLUSIONS

Flexible ureterorenoscopy offers a minimally invasive means of treating symptomatic calyceal diverticula. Laser infundibulotomy has been a safe and efficaceous means of promoting effective drainage from calyceal diverticula, can be performed as a day case procedure, and can avoid the morbidity of percutaneous access or laparoscopic procedures.


15:34 - 15:36
S4-13 (CRP)

LAPAROSCOPIC APPROACH OF VAGINA IN CHILDREN: WHO MAY BE CONCERNED?

Aurora MARIANI 1, Daniela GORDUZA 1, Jacques BIRRAUX 2, Yaqoub JAFAR 1, Marc BARRAS 1, Faustin TAMBO 3 and Pierre Yves MURE 1
1) Femme-Mere Hospital, Paediatric Urology, Bron, FRANCE - 2) Hôpitaux Universitaires Genève, Pediatric Surgery, Genève, SWITZERLAND - 3) Hôpital Gynéco Obstétrique et Pédiatrique, Pediatric Surgery, Yaoundé, CAMEROON

PURPOSE

Laparoscopic approach of vagina (LAV) is poorly developed in children. Our first experience with laparoscopic assisted vaginal pull-through (LAVPT) in case of high vagino-urethral confluence for congenital adrenal hyperplasia (hCAH) showed that this approach appeared safe, and it minimized perineal dissection.
We expanded our experience to other malformations: long gap partial vaginal atresia (pVA), long common channel cloacas (LCC) and high anorectal malformation (hARM).

MATERIAL AND METHODS

Charts of girls operated after 2012 using LAV for hCAH, pVA, LCC and hARM were retrospectively reviewed.
For hCAH, vagina was dissected from posterior wall of bladder, divided from posterior urethra and lowered to perineum. For pVA, vagina pouch was freed from surrounding tissues and pull through with perineal anastomosis. For LCC, rectum, vagina and urinary tract were separated. For hARM recto-vaginal fistula was divided, and rectum separated from vagina.

RESULTS

Seven patients were concerned: 2 hCAH (3.5&5 years), 2 pVA (13&14 years), 2 LCC (5&6 months) and 1 hARM (13 months). For hCAH, confluence of vagina into urethra was less than 15 mm below bladder neck. For pVA, distance between lower extremity of vagina and perineum was 4.5 and 5 cm. For LCC, common channel length was 2.5 and 4 cm. For hARM fistula was 3 cm from perineum. No intraoperative complication was observed but separation of vagina from urinary tract was not possible to achieve in LCC.

CONCLUSIONS

LAV in children appears to be an interesting approach. For hCAH, pVA and hARM it allows minimal dissection of pelvis and perineum. Preliminary experience for LCC shows some limits


15:36 - 15:38
S4-14 (CRP)

A NEW MINI-INVASIVE SURGICAL PROCEDURE FOR TREATING URINARY INCONTINENCE IN MALE DUE TO NEUROGENIC BLADDER OUTLET DEFICIENCY.

Giovanni MOSIELLO 1 and Alessandro GIAMMÒ 2
1) Bambino Gesù Pediatric Hospital, Urology, Rome, ITALY - 2) AOU TURIN , Spinal Unit, Neuro-Urology, Turin, ITALY

PURPOSE

Urinary incontinence(UI) due to neurogenic bladder outlet deficiency is quite common to observe in boys and young male adults with spina bifida(SB). Different solutions have been proposed: bulking agents injections, sling, bladder neck surgical procedure, artiphicial urinary sphincter(AUS). We report our preliminary experience with an adjustable hydraulic system in the treatment of UI.

MATERIAL AND METHODS

In two different Neuro-Urology Departments, a  same pre-post operative protocol selection and management has been defined in order to treat boys presenting severe neurogenic UI, > 5 pads/day, not responding to standard treatment  and failure of previous surgical treatment . All have been selected using diaries, QoL questionnaires, renal ultrasound , videourodynamic. Patients  with neurogenic detrusor overactivity((NDO) have been excluded. Patients were operated after a written consent was signed with approval of institutional  Committee.

RESULTS

5 patients , 17.6 years mean age ( 16,5-18,7) have been operated using a a new self anchoring adjustable transobturator hydraulic system (ATOMS)  susbstituting urinary sphincter function. Patients were placed in lithotomy position, under endoscopic control, using a perineal access the system was implanted over bulbospongious muscle, compressing indirectly urethra below, increasing urethral resistance. The large cushion was fixed using mesh passing the obturator foramen , anchoring the device on the inferior pubic ramus. Mean operative time 49 minutes (37-61), no postoperative complications have been reported. All patients reported improvement in QoL. 4 are completely dry, 1 reported 80% improvement UI. Not increased Urinary tract infections or post operative hydroneprhosis have been reported. Hydraulic adjustment of reservoir have been done in all without anaesthesia ( mean 2 adjustment).

CONCLUSIONS

Our preliminary experience with this device showed a significant improvement in UI and QoL scores. ATOMS seems to be effective in well selected boys with SB, as alternative to other treatment, overcoming sling and AUS concerns.


15:38 - 15:42
Discussion