30th ESPU Congress - Lyon, France - 2019

S2: CASE REPORTS

Moderators: Rita Gobet (Switzerland), Alexander Springer (Austria)

ESPU Meeting on Wednesday 24, April 2019, 14:15 - 15:30


14:15 - 14:17
S2-1 (CP)

BOTRYOID WILMS TUMOR WITH EXTENSION TO THE URETER: CASE REPORT AND LITERATURE REVIEW

Ana Maria GOMEZ 1, Victor Hugo FIGUEROA 2, Laura ARENAS 1, Alba J RAMOS 1 and Maria Jose DIAZ 1
1) UNIVERSIDAD AUTONOMA DE BUCARAMANGA / CLINICA FOSCAL, UROLOGY, Floridablanca, COLOMBIA - 2) UNIVERSIDAD AUTONOMA DE BUCARAMANGA / CLINICA FOSCAL, Floridablanca, COLOMBIA

PURPOSE

Wilms tumor (WT) is one of the most frequent pediatric solid tumors. Rarely, it arises from the renal pelvis growing in an exophytic fashion similar to botryoid sarcomas, hence the name Botryoid Wilms tumor (BWT).

MATERIAL AND METHODS

We describe the case of a 15 month-old girl who was referred to the emergency department with history of febrile urinary tract infection, gross hematuria with clots and urinary retention. After her Foley was removed she prolapsed a mass through her urethral meatus. A right-side hydronephrosis without an associated renal mass was seen on the renal ultrasound (US). Cystoscopy showed a necrotic-appearing mass emanating from the right ureteral orifice. A CT scan confirmed the presence of right hydroureteronephrosis with a small hypo-dense mass in the renal pelvis extending into the right ureter.

RESULTS

We performed a right radical nephroureterectomy with retroperitoneal lymph-node sampling. Severe hydroureteronephrosis with multiple polypoid masses in the collecting system extending down the ureter was found. Pathology report revealed a nephroblastoma with favorable histology originating at the pelvis underneath the urothelium. The typical triphasic features of WT were present. No anaplasia was seen. Lymph nodes, ureter, renal artery and vein were free from tumor. Adjuvant chemotherapy was given following the NWTS-4 protocol.

CONCLUSIONS

BWT is an atypical form of WT, and it can present without a large parenchymal mass on US or CT scan. BWT should be suspected in patients with macroscopic hematuria and hydroureteronephrosis with passage of clots or tissue fragments per urethra.


14:17 - 14:19
S2-2 (CP)

NEPHRON-SPARING SURGERY IN CHILDREN WITH WILMS TUMOR USING A SINGLE LAPAROSCOPIC ACCESS

Nail AKRAMOV 1, Aydar ZAKIROV 1 and Rashit BAYBIKOV 2
1) Kazan State Medical University, Pediatric urology, Kazan, RUSSIAN FEDERATION - 2) Children's Republican Clinical Hospital of the Ministry of Health of the Republic of Tatarstan, Pediatric urology, Kazan, RUSSIAN FEDERATION

PURPOSE

The question of preserving treatment in children with Wilms tumor is being discussed in the international literature. Unfortunately, the problem of nephron sparing surgery is not limited by the improvement of surgical techniques. Only the joint efforts of various experts can cure the patients with such disease.

MATERIAL AND METHODS

Two children with Wilms tumor were treated using preserving surgery, the first case - the bilateral process and the second case - the volume of tumor less than 30% of the kidney. Pre- and postoperative therapy was carried out in accordance with the Protocol SIOP 2001 / GPOH. In both cases, as a surgical technique we were used partial nephrectomy by a single laparoscopic access. That is why the access to the abdominal cavity was performed through a 2.5 cm length incision of umbilicus through single-port. The 5 mm laparoscope30 °, rotating curved instruments and ultrasonic scissors were used. The rubber band was applied on the renal vessels with the exposition of no more than 40 minutes. The tumor was dissected entirely after the renal resection at the level of the healthy parenchyma (5 mm from the tumor), and removed using a laparoscopic evacuation bag. The renal wound was closed with hemostatic mesh and was sprinkled with hemostatic powder. Top of the wound was applied with kidney fat. Intraoperative blood loss in both cases were less 25 ml.

RESULTS

Six months later the surgery both children were tested on the computerized tomography, which showed no signs of metastasis or recurrence of the tumor. Parents of the patients were satisfied by the cosmetic results of the surgical treatment.

CONCLUSIONS

So, the partial nephrectomy in children with Wilms tumor using of a single laparoscopic approach may be the choice with good functional and cosmetic results.


14:19 - 14:21
S2-3 (CP)

RHABDOMYOSARCOMA OF THE BLADDER AND PROSTATE IN THE FIRST MONTHS OF LIFE.

Solon CASTILLO MALDONADO 1, B NAVA 1, A BUENO 1, P LOPEZ PEREIRA 1, B LOPEZ-IBOR 2, S RIVAS 1, V AMESTY 1, R LOBATO 1 and M.J. MARTINEZ-URRUTIA 1
1) University Hospital La Paz, Pediatric Urology, Madrid, SPAIN - 2) Monteprincipe Hospital, Pediatric Oncology, Madrid, SPAIN

PURPOSE

Bladder/prostate (BP) location is the most common primary genitourinary site of rhabdomyosarcoma (RMS) and accounts for 5% of all RMS. Survival has improved significantly with the advent of the multidisciplinary approaches.
The current treatment of the non-metastasic BP RMS includes chemotherapy and radiotherapy for local control. What options are there when this strategy is not enough?.

MATERIAL AND METHODS

A 6-month-old boy consulted for UTI, bilateral ureterohydronephrosis and impared renal function; MRI shows BP tumor and no distant metastases (Pet-scan). Endoscopic biopsy revealed bothryoid rhabdomyosarcoma. Induction chemotherapy was started (Ifosfamide, vincristine and actinomycin x 4) and radiotherapy from the 16th week after beginning the chemotherapy. Great reduction of the mass was obtained but residual tumor occluded the urethra with a transurethral biopsy showing residual embryonal rhabdomyosarcoma.

Surgical intervention was initially proposed given the persistence of residual mass at the end of the treatment, but a multidisciplinary evaluation of the situation was carried out and it was decided to prolong the treatment with chemotherapy (5 cycles) obtaining extraordinary results. The patient presented proximal tubulopathy secondary to chemotherapy and during follow-up required a vesicostomy to stabilize bilateral hydronephrosis and renal function.

RESULTS

The patient is in complete remission confirmed by biopsy 2 years after the end of treatment.
In terms of renal function he presents a GFR in the limits of normality and has achieved urinary and fecal continence.

CONCLUSIONS

Non-metastasic BP RMS can be treated without mutilating surgery thus avoiding the significant morbidity from radical extirpation. A multidisciplinary approach should always followed.


14:21 - 14:23
S2-4 (CP)

TRANS URETHRAL RESECTION "EN-BLOC" OF TRANSITIONAL BLADDER CELL CARCINOMA WITH HOLMIUM:YAG LASER IN TWO PEDIATRIC PATIENTS.

Michele INNOCENZI 1, Mele ERMELINDA 1, Simona GEROCARNI NAPPO 2, Laura DEL PRETE 1 and Nicola CAPOZZA 1
1) Bambino Gesù Children's hospital, Dept of Urology, Rome, ITALY - 2) Bambino Gesù Children's hospital, Urology, Rome, ITALY

PURPOSE

Urothelial bladder carcinoma frequently occurs in adults over 60 years of age and affects only 0,1%-0,4% of patients before the first two decades of life. We want present two cases of transitional cell carcinoma in two young patients treated with Holmium:YAG laser

MATERIAL AND METHODS

A 14-year-old female presented with macroscopic haematuria in the previous 1 month, not associated with other symptoms. Physical examination and laboratory exams were all normal. Bladder ultra-sound revealed an intravesical endophytic lesion in the trigone zone of 10mmx6mmx10mm.
A 12-year-old female presented with acute macroscopic haematuria. No other symptoms were associated. Physical examination and laboratory exams were all normal. Bladder ultrasound revealed an irregular 15mm intravesical endophytic lesion in the posterior-superior area.
All 2 patients are treated with the same approach. For resection, a Holmium:YAG fiber laser of 272 micron in diameter is used through the 12ch cystoscopy working channel with an energy of 0.8-1 J/pulse and a frequency of 8-10 Hz.

RESULTS

There was not haematuria after the procedure, and the trans-urethral catheter remained for 12 hours. The histological diagnosis was Papillary Urothelial Neoplasm of Low Malignant Potential (PUNLMP) according to WHO/ISUP 2004 classification.
We performed a cystoscopy at 3 and 9 months, and bladder ultrasonography at 6 and 12 mounths, resulted normal. The follow-up for each patients, consists of a cystoscopy once a year, according with EAU guidline.

CONCLUSIONS

According to us Holmium:YAG laser is a good alternative to treat superficial transitional cell carcinoma.
Although there are few cases, with this technique, we have achieved good results in the absence of post-operative complications.


14:23 - 14:25
S2-5 (CP)

MALIGNANT PEROPHERAL NERVE SHEATH TUMOR OF THE DORSAL PENIS ; A CASE REPORT AND REVIEW OF THE LITERATURE

Ruankha BILOMMI
Mitra keluarga hospital, Pediatric surgery, Jakarta Utara, INDONESIA

ABSTRACT

Malignant peripheral nerve sheath tumors are a rare type of cancer that occurs in the lining of the nerves that extend from the spinal cord into the body. Malignant peripheral nerve sheath tumors can occur anywhere in the body, but most often occur in the deep tissue of the arms, legs and trunk. They tend to cause pain and weakness in the affected area and may also cause a growing lump or mass. Malignant peripheral nerve sheath tumors (MPNSTs) is an aggressive neoplasm in which local recurrence is common and complete excision of the mass should be the goal of surgery. This is the first report of this case from Indonesia. We report a case of MPNST involving the penis in a 13 month-old boy. That had complain of growing lump on dorsal penis and suprapubic area for two weeks. The lesion was excised and a formal circumcision performed. Histology of the lesion revealed a spindle cell tumour. Immunohistochemistry showed the tumour cells to be strongly positive for a diagnosis of high grade malignant peripheral nerve sheath tumour was made. Malignant schwannoma is rare in children and is previously unreported in the penis in the paediatric age group without evidence of neurofibromatosis. The surgery is followed by post-operative radiotherapy and adjuvant chemotherapy.


14:25 - 14:27
S2-6 (CP)

MEGALOURETHRA-LIKE MALFORMATION AFFECTING DUPLICATED URETHRA IN A GIRL. A CASE REPORT.

Arthur LAURIOT DIT PREVOST, Dyuti SHARMA, René-Hilaire PRISO, Estelle AUBRY and Remi BESSON
Jeanne de Flandre Hospital, Lille University Hospital, Paediatric Surgery, Lille, FRANCE

PURPOSE

Both megalourethra and urethral duplication are rare urogenital malformation. Although urethral duplication has already been reported in girl (approximatively 40 cases), megalo-urethra is a specific malformation of male genitalia. There are few report of (single urethra) megalourethra-like malformation (mega-urethra) in girl.

CASE REPORT

A 31 years old pregnant woman (G4P2, one miscarriage), was diagnosed on 21st week of gestation, with a 45*25mm anterior perineal cystic mass affecting her female fetus, without reno-vesical dilatation. Biochemical analysis indicated urine and amniocentesis confirmed a 46 XX karyotype. Prenatal MRI showed no associated malformation. Ultrasound follow-up was maintained. At birth the child presented a median perineal cystic mass, above orthotopic meatus, repelling labia majora. Blood test showed no hormonal anomaly. Micturating cystourethrogram catheterizing both meatus confirmed urethral duplication, communicating with orthotopic urethra. We decided a two stages treatment. During neonatal period (day 6), we incised the ventral part of the mega-urethra to improve drainage. At 6 month, we planned a surgery for resection of duplicated urethra, reconstruction of external genitalia using skin flap from the dilated mega-urethra, and clitoroplasty with corpus cavernosum preservation.

RESULTS

Postoperative follow up at 3 months was simple, with no voiding difficulties, nor urinary tract infection. Ultrasound showed no reno-vesical dilatation either.

CONCLUSIONS

Presence of a dilated duplicated urethra in girl is a very rare condition. Primary objective should be to optimize urine drainage. Timing for reconstructive surgery should be discussed for each case. In these urogenital malformation, prenatal diagnosis is essential to estimate renal prognosis, and detect associated malformation.


14:27 - 14:39
Discussion
 

14:39 - 14:41
S2-7 (CP)

TRANSURETHRAL UNROOFING OF SEMINAL CYSTS IN ZINNER SYNDROME- A SAFE AND EFFECTIVE ALTERNATIVE MINIMAL INVASIVE PROCEDURE

Aybike HOFMANN 1, Christian ROTH 2, Vauth FRANZISKA 3 and Wolfgang H ROESCH 3
1) University Medical Center of Regensburg, Pediatric Urology, Regensburg, GERMANY - 2) University Hospital of Leipzig, Paediatric Radiology, Leipzig, GERMANY - 3) University Medical Center of Regensburg, Paediatric Urology, Regensburg, GERMANY

PURPOSE

Zinner syndrome is rare anomaly of the mesonephric (Wolffian) duct comprising a triad of unilateral renal agenesis, ipsilateral seminal vesicle obstruction and ipsilateral ejaculatory duct obstruction. This syndrome was first described by A. Zinner in 1914 since then about 200 cases are reported. In symptomatic cases a surgical intervention is demanded.

MATERIAL AND METHODS

2 boys in the age 15 and 18 years presented with scrotal pain and alguria. They were sent with suspected urinary tract infection and bladder stone. Ultrasound revealed in both patients a left sided renal agenesis and an ipsilateral cystic enlargement of the seminal vesicle. MRI confirmed cystic-tubular retrovesical structures. Both patients went to the OR for transurethral incision (unroofing) of a cystic mass in the prostatic portion of the urethra.

RESULTS

After transurethral incision both boy showed immediate improvement. In the nearly 2 year follow-up with ultrasound and MRI neither new seminal vesical cysts nor inflammatory changes of the ejaculatory ducts were seen. The clinical presentation was unremarkable and there were no more urinary tract infections.

CONCLUSIONS

Particularly in non-giant seminal vesical cysts transurethral incision (unroofing) is  a reliable and successful option. Due to the high infertility-rate related with retrovesical operations, primarily transurethral incision should be tried.


14:41 - 14:43
S2-8 (CP)

CHALLENGING DECISION MAKING IN A 46XY DSD PATIENT

Corina ZAMFIR SNYKERS 1, Cecile BRACHET 2, Veronique DELVENNE 3 and Karim KHELIF 1
1) "Queen Fabiola" Universitary Children's Hospital, Pediatric Surgery, Bruxelles, BELGIUM - 2) "Queen Fabiola" Universitary Children's Hospital, ENDOCRINOLOGY, Bruxelles, BELGIUM - 3) "Queen Fabiola" Universitary Children's Hospital, PEDIATRIC PSYCHIATRY, Bruxelles, BELGIUM

PURPOSE

To discuss and highlight the challenging decision making progress encountered in a patient with a particular form of DSD 46 XY.

MATERIAL AND METHODS

A full term baby born to non-consanguineous parents was addressed for ambiguous genitalia (well developed penis with posterior hypospadias, scrotal bifidity with non-palpable testes and a vaginal opening at the perineum). Postnatal diagnosis was 46XY DSD gonadal dysgenesis and male gender assigned after multidisciplinary assessment and parental choice. At one year of age a laparoscopy and genitoscopy confirmed the internal anatomy: normal uterus with fallopian tubes and vaginal opening at the perineum. Two streak gonads were removed and a right testis biopsied. The second part of the surgery consisted in a modified Koyanagi procedure with preservation of the perineal vaginal opening. Histology described the streaks as undifferentiated gonadal material and the right gonad biopsy as dysgenetic testicular tissue. During regular out-patient clinic visits, the patient’s psychological development was monitored. Parental concerns consisted in mainly two questions: when should the remaining gonad be removed as to the malignancy risk and to remove or not the Mullerian structures (irreversible surgery). The former concern was addressed ten months later by laparoscopic removal and cryopreservation of the remaining testis. Despite conflicting specialists’ opinions, at 2 years of age, considering the clear masculine behavior of their child the parents opted for definitive resection surgery in early infancy in order to avoid later psychological problems. Uneventful total laparoscopically utero-vaginectomy were performed.

CONCLUSIONS

This case illustrates the difficult parental decision making process in a complex moving social and legal context where specialist opinions invariably differ.


14:43 - 14:45
S2-9 (CP)

VAGINOPLASTY WITH OXIDIZED CELLULOSE IN MAYER-ROKITANSKY-KUSTER-HAUSE SYNDROME

Mila LEITE 1, Marcia EMILIA FRANCISCO SHIDA 2, Claudia CRISTINA TAKANO 3 and Camila GIRARDI FACHIN 4
1) FEDERAL UNIVERSITY OF SÃO PAULO, PEDIATRIC SURGERY, São Paulo, BRAZIL - 2) FEDERAL UNIVERSITY OF SÃO PAULO, São Paulo, BRAZIL - 3) FEDERAL UNIVERSITY OF SÃO PAULO, Gynecology, São Paulo, BRAZIL - 4) Federal University of Paraná, PEDIATRIC SURGERY, Curitiba, BRAZIL

PURPOSE

Mayer–Rokitansky–Kuster–Hause Syndrome(MRKHS) is the major cause of vaginal agenesis. They present 46XX karyotype, appropriate external genitalia and primary amenorrhea, hyploplasic or absent uterus and the ovaries are normal. Surgical reconstruction of the vagina may be performed with different surgical techniques. A case of MRKHS that underwent Vaginoplasty with oxidized cellulose will be reported.

MATERIAL AND METHODS

46, XX, 17 years old patient, with MRKHS. The patient was kept in a gynecological position. After bladder catheterization, a transverse incision was made in the vaginal introitus, followed by blunt digital dissection between rectal  and retrouretral space to a depth of 10-15 cm. The vaginal silicone mold wrapped in oxidized cellulose was placed into the dissected cavity and it was kept there for five days.The vaginal mold was used daily during the night At the end of the second month of follow-up, the vaginal canal had a length of 8-9 cm  and there was no pain

RESULTS

The vaginoplasties with intestinal segments are certainly the most widespread surgical techniques among pediatric urologic surgeons. The necessity of intra-abdominal access is a major disadvantage of these procedures. Gynecologists use mechanical dilatation of the introitus as the first non-surgical attempt. Among the surgical interventions, the Abbe-McIndoe vaginoplasty is the preferred technique and consists of perineal incisions and, classically, the use of skin grafts. The use of oxidized cellulose in modified Abbe-McIndoe procedure has been reported with good cosmetic and functional results. After 6-month follow-up, this neovaginas present histological characteristics like a normal vagina. The best results were obtained in patients with vaginal agenesis and no functional uterus. In MRKHS, the uterus is absent in most patients, thus making vaginoplasty with oxidized cellulose an excellent surgical option.

CONCLUSIONS

The vaginoplasty with oxidized cellulose may be considered as a surgical alternative in the MRKHS.


14:45 - 14:47
S2-10 (CP)

OLBERT'S BALLOON AS A POSSIBILITY OF TREATING URETERAL STRICTURE AFTER COMPLICATED URS-L IN CHILDREN - PRELIMINARY REPORT

Adam Maksymilian HALINSKI and Andrzej HALINSKI
University Hospital in Zielona Góra, Department od Paediatric Surgery and Urology, Zielona Gora, POLAND

PURPOSE

Olbert's balloon dilatation is a surgery technique used for the treatment of the ureteral stricture. It is very often used in adult patients, however, due to the advancing miniaturization of the equipment as well as its precision, this technique has also become possible in the treatment process in children.

MATERIAL AND METHODS

We would like to present 3 cases of Olbert's balloon dilatation carried out in children with ureteral stricture after complicated URS-L, aged 12 to 17 years. The average age was 14.3 years and the children were treated in our department from January 2016 to February 2017. All ureteral stricture was noticed after complicated URS-L procedure. All of those children were diagnosed for at least one year because of the abdominal pain!, before there were referred to our Clinical Department. All of the children had hydronephrosis of the 3rd grade. The lack of function of the kidney in DMSA was observed with average on about 20%. During the URS-L procedure in all children the ingrown to the ureteral wall stone was noticed. Stone free rate was 100% and the DJ stent was inserted. After removing DJ stent hydronephrosis was observed in US. Retrograde pyelogram revealed ureteral stricture in the place were stone was ingrown. DJ stent was placed and Olbert's Baloon dilatation under fluoroscopy was performed.

RESULTS

We achieved an efficiency of 100%. In DMSA function of the kidney after 3 months increased to the average of 36%. The level of creatinine shaped at averege 0.6. In US two children had no hydronephrosis and one child had 1st grade on hydronephrosis. The longest follow up is now 1.5 year still with the same good results.

CONCLUSIONS

Olbert's balloon dilatation is an effective, safe and minimally invasive tool both for the ureteral stricture, but only in the hands of endourological experienced paediatric urologist. Still more and more prospective, randomized trials are needed.


14:47 - 14:49
S2-11 (CP)

BLADDER EXSTROPHY ACCOMPANIED WITH RECTAL DUPLICATION, BIFID PHALLUS AND EPISPADIAS COMPLEX, AS AN EXTREMELY RARE CASE

Özkan CESUR 1, Levent DUMAN 2 and Mustafa Çağrı SAVAŞ 2
1) Suleyman Demırel Unıversity Research Hospital, Pediatric Surgery, Isparta, TURKEY - 2) Süleyman Demirel University Medicine Faculty, Pediatric Surgery, Isparta, TURKEY

PURPOSE

The bladder exstrophy is a midline closure defects which must be treatment with primary reconstruction in infancy period. The aim with this case is to discuss the evaluation and management of bladder exstrophy abnormality with rectal duplication.

MATERIAL AND METHODS

On hospital records of patient was evaluationed included clinical and radiological findings, examination under anesthesia and assessment of the surgical findings, operative procedures and the results of management.

RESULTS

The present patient was 4 month-old-age male. He referred with exstrophy vesicae and perineal mass. On physical examination, exstrophy vesicae, bifid phallus and rectal duplication placed on anus anteriorly were observed. Bladder was be able to primary closure, but couldn't performed iliac ostomy due to late presentation. Prior to closure, pseudopolyps were excised on the bladder mucosa. Urethral plate was created as epispadias. So urinary drainage was provided. Low abdomen anatomy was be able to restore anatomically. Symphysis pubis opening was greater than 5 cm. Duplication structure was excised. On histopathological examination, rectal duplication cyst abnormal structure was reported as intestine tissue. Bilateral gonadal structures were placed on intrascrotal. Perineal anatomy was restored by excising the mucosa lined lesion. Penile reconstruction was planned in one year old. On postoperative following vesico-ureteral reflux was not observed.

CONCLUSIONS

The association of classical bladder exstrophy with ruptured rectal duplication cyst has never previously been described in the literature. The urinary bladder must be closed at the very
early period. It was thought that this case report will contribute to the rare congenital anomalies of the literature.


14:49 - 14:03
Discussion
 

14:59 - 15:01
S2-12 (CP)

PRENATALLY DETECTED PATENT URACHUS WITH BLADDER PROLAPSE: FETAL COURSE AND POSTNATAL OUTCOME

Fumi MATSUMOTO, Satoko MATSUYAMA, Futoshi MATSUI and Koji YAZAWA
Osaka Women's and Children's Hospital, Urology, Osaka, JAPAN

PURPOSE

Patent urachus is a rare anomaly that may be identified as an abnormality of the umbilicus in the newborn.In some cases, patent urachus is detected prenatally.Although correct diagnosis is not always easy, this kind of anomaly displays some specific findings in utero.The purpose of this study was to assess the antenatal course and outcome of fetuses with bladder prolapse through a patent urachus.

MATERIAL AND METHODS

A review of medical charts of 4 patients(3 boys and one girl)with prenatally detected patent urachus and bladder prolapse between 1996 and 2017 in our institute was conducted.All underwent serial ultrasound evaluation at the diagnosis and during follow-up.

RESULTS

Gestational age at diagnosis ranged from 14 to 19 weeks. At presentation,a cystic mass was located in the base of the umbilical cord, communicating with the fetal bladder in all cases. The cystic mass suddenly disappeared as aresult of spontaneous rapture at 26-34 weeks of gestational age.All patients were delivered at term without complication. All neonates had urinary leakage through the inferior portion of the umbilicus associated with bladder prolapse and omphalocele. Surgical repair was carried out within 48 hours in all cases. Postoperative voiding cystourethrography showed an adequate bladder capacity and no bladder outlet obstruction in all cases, and vesicoureteral reflux, which resolved subsequently,in 2 patients. 

CONCLUSIONS

Bladder prolapse through a patent urachus can be predicted by prenatal ultrasound. Prognosis of this condition is good.


15:01 - 15:03
S2-13 (CP)

SYMPTOMATIC PARAPELVIC CYSTS IN CHILDREN: A RARE ENTITY. REPORT OF 4 CASES

Jean-Baptiste MARRET 1, Thomas BLANC 2, Vincenzo BAGNARA 3 and Henri LOTTMANN 2
1) Caen University Hospital, Pediatric Surgery, Caen, FRANCE - 2) Necker Enfants-Malades Hospital, Pediatric surgery, Paris, FRANCE - 3) "G.B Morgagni" Policlinic, Pediatric Surgery, Catania, ITALY

PURPOSE

Parapelvic cysts (PC) lie directly adjacent to the renal pelvis and sinus. Most of them are asymptomatic. We report characteristics of symptomatic PC that mimic an ureteropelvic junction (UPJ) obstruction. The objective was to highlight specific features to avoid a misdiagnosed UPJ obstruction.

MATERIAL AND METHODS

We reviewed retrospectively the files of four symptomatic PC.

RESULTS

All children (18 months to 7 years) presented acute abdominal pain crises and vomiting with a large intra-sinusal liquid mass (42 to 85 mm) on ultrasound evoking a UPJ obstruction.
On dynamic renography (n = 3) there was no dilatation of the renal pelvis and in 2 cases differential function of the affected kidney was severely impaired (21% and 28%).
The diagnosis was suspected preoperatively for 3 patients with CT scan (n=1) and RMI (n=2) and made peroperatively in 1.
Injection of methylene blue (n=4) or retrograde pyelography (n=3) failed to identify communication between the renal pelvis and the cyst. Subtotal resection of the cyst was performed in all cases. There was no renal pelvis in two patients.
With a follow up of 17 to 65 months, no recurrence was observed. Renal function returned to normal in one patient and stayed impaired in the other.

CONCLUSIONS

Diagnosis of PC should be considered with symptoms of UPJ obstruction associated with a medio renal liquid mass on ultrasound and no dilatated pelvis on dynamic renography.
In such cases, the evaluation of the upper urinary tract with MRI or CT scan and retrograde pyelography is mandatory prior to surgery.


15:03 - 15:05
S2-14 (CP)

PAEDIATRIC XANTHOGRANULOMATOUS CYSTITIS (XC) AS A PELVIC MASS: CASE REPORT

Sadaf ABA UMER KODWAVWALA 1, Bashir AHMED 2, Sajid SULTAN 2, Sana SHEIKH 3, Mohammad MUBARAK 4, Philip G. RANSLEY 2 and Adeeb-Ul-Hassan RIZVI 2
1) Sindh Institute of Urology & Transplantation, Philip G. Ransley Department of Paediatric Urology, Karachi, PAKISTAN - 2) SINDH INSTITUTE OF UROLOGY AND TRANSPLANTATION, Philp G. Ransley Department of Paediatric Urology, Karachi, PAKISTAN - 3) SINDH INSTITUTE OF UROLOGY AND TRANSPLANTATION, DEPARTMENT OF RADIOLOGY, Karachi, PAKISTAN - 4) SINDH INSTITUTE OF UROLOGY AND TRANSPLANTATION, DEPARTMENT OF PATHOLOGY, Karachi, PAKISTAN

PURPOSE

Xanthogranulomatous cystitis (XC) is a rare condition. Few cases have been reported in the literature so far. Here we present the case of XC in a girl with spina bifida.

MATERIAL AND METHODS

Twelve years old girl, operated for MMC in infancy, presented with three months history of fever and lower abdominal pain. She was urinary and faecal incontinent.

RESULTS

The girl was febrile and anaemic. A firm tender mass was palpable in lower abdomen. She had low haemoglobin, high leukocyte counts and a high serum creatinine. Ultrasound showed 8 x7 cms. vascularized heterogenous mass on anteriosuperior aspect of thick wall urinary bladder with bilateral hydroureteronephrosis. Her fever, leukocytes count and lower abdominal tenderness responded to I/V antibiotics and per urethral bladder drainage but mass of same size persisted. Her CT scan revealed 8x6x6 cms. heterogeneously enhancing mass involving  anteriosuperior aspect of urinary bladder and invading the anterior abdominal wall. Biopsy showed sheet of foamy histiocytes and fragments of necroinflammatory tissue. Mass gradually decreased in size and disappeared completely in a month time.

CONCLUSIONS

This case highlights the importance of recognizing an unusual lesion that can present in individuals of any age. Histopathology can differentiate XC from other pathologies. To our knowledge this is the second reported case of paediatric xanthogranulomatous cystitis.


15:05 - 15:07
S2-15 (CP)

GIANT BILATERAL HYDRONEPHROSIS - A CASE REPORT

Vivienne SOMMER 1, Ulrike SUBOTIC 1, Stefan HOLLAND-CUNZ 1 and Martina FRECH 2
1) University Children's Hospital Basel, Pediatric Surgery, Basel, SWITZERLAND - 2) University Children's Hosptial Basel, Paediatric Urology/Surgery, Basel, SWITZERLAND

PURPOSE

Prenatal hydronephrosis is common and may vary in severity. Although mostly unproblematic, it may be a sign for urinary tract obstruction of different severity.

MATERIAL AND METHODS

We present a boy with known massive prenatal bilateral hydronephrosis. Prenatal ultrasound showed the whole abdominal cave of the fetus filled with urine. Renal parenchyma could not be seen. Due to prenatal counseling of the mother she was recommended to terminate pregnancy. The boy was born at 34 weeks of gestation.

RESULTS

After delivery the boy showed a severely distended abdomen. Insertion of nasogastric tube was not possible and he hat to be intubated due to respiratory insufficiency. Immediate percutaneous nephrostomy was performed bilaterally. After a few hours he could be stabilized and extubated. Ultrasound on the following day showed two renal units with normal renal parenchyma. The initially slight elevated serum creatinine level normalized within one week. An antegrade pyelography via the nephrostomy tubes was performed, where bilateral pyeloureteral junction obstruction could be verified.

CONCLUSIONS

Severe bilateral hydronephrosis is not compulsorily associated with poor outcome of renal function. Prenatal counseling should be done carefully with discussion of the different possibilities and without definitive prediction of outcome. We would like to present this case with a complicated course but an excellent prognosis despite these severe prenatal findings.


15:07 - 15:09
S2-16 (CP)

EXTRARENAL CALYCES MIMICKING RETROPERITONEAL CYSTIC MASS WITH CONCOMITANT URETEROPELVIC JUNCTION OBSTRUCTION: RENAL PELVIS RECONSTRUCTION USING CALYCES UNIFICATION

Irfan WAHYUDI 1, Prahara YURI 2, Fakhri RAHMAN 1, Gerhard SITUMORANG 1 and Arry RODJANI 1
1) Cipto Mangunkusumo National Referral Hospital - Faculty of Medicine, Universitas Indonesia, Urology, Jakarta Pusat, INDONESIA - 2) Sardjito General Hospital - Faculty of Medicine, Universitas Gajah Mada, Yogyakarta, Indonesia, Surgery, Division of Urology, Yogyakarta, INDONESIA

BACKGROUND

Extrarenal calyces (ERC) has still become one of the highly rare anomalies, especially in concomitant with ureteropelvic junction obstruction (UPJO). Its treatment is dominated by the use of dismembered pyeloplasty. We present a case in which its treatment was done by renal pelvis reconstruction using calyces unification.

PATIENT

An eleven-year-old boy was referred to us due to a bilateral hydronephrosis and left multicystic kidney disease by ultrasonography examination. MRI showed left severe hydronephrosis with concomitant left cystic mass and left UPJO. Renal function was in preserved based on TC-99m DTPA diuretic renal scan. Intraoperatively, we found ERC with severe dilated renal pelvis. Renal pelvis was excised, major calyces which protrude from the kidney were anastomosed to each other using side-to-side anastomosis to become renal pelvis-like structure and further anastomosed to ureter. DJ stent, nephrostomy and 12 Fr drain were placed on the patient. Pathological examination revealed atrophic transitional epithelial cells. 

RESULTS

Patient underwent removal of 12 Fr drain, nephrostomy tube and DJ stent in 3 days, 2 weeks and 3 months after surgery, respectively. Patients had no complain and ultrasonography examination revealed no hydronephrosis after DJ stent removal. Therefore, the surgery is considered successful.

CONCLUSIONS

Renal pelvis reconstruction using calyces unification could become one of the treatment choices in a patient with ERC coinciding with UPJO, especially in severe dilated renal pelvis or difficult to maintain portion of renal pelvis cases.


15:09 - 15:11
S2-17 (CP)

OCHOA SYNDROME: BEHIND PECULIAR SMILE. CASES REPORTS

Anna BUJONS 1, Erika LLORENS DE KNECHT 2, Yesica QUIROZ 2, Guilherme LANG MOTTA 2, Helena VILA DEL REY 2 and Joan PALOU 2
1) Fundació Puigvert Barcelona, Paediatric Urology, Barcelona, SPAIN - 2) Fundació Puigvert, Pediatric Urology Unit, Barcelona, SPAIN

PURPOSE

The Ochoa Syndrome (OS) is characterized by urinary bladder voiding dysfunction (BVD) (vesico-sphincter dyssynergia, VSD) associated to inversion of the facial expression when laughing and crying and often bowel dysfunction. The BVD increasesthe risk of end-stage renal disease, because of vesicoureteric reflux (VUR), hydroureteronephrosis and urosepsis. The aim of this report is to describe three cases of  OS with different start symptoms and its long term follow up.

MATERIAL AND METHODS

Three patients are described with these initial symptoms, diagnosis process and treatments received before and after the final diagnosis of OS, also the long term follow up of two of them who are adults actually.

RESULTS

Case 1: 41 year old male started with enuresis at 7 years old and had bladder neck surgery because VSD. 6 years later had an augmentation cystoplasty for renal exclusion and has 29 years of follow up with preserved renal function. Case 2: 27 year old male with incontinence since 7 year of age who started clean intermittent self-catheterization. However he presented febrile ITU by VUR, which was treated with endoscopic treatment without success and finally  he needed an augmentation cystoplasty. Case 3: 4 year old female with antenatal bilateral hydronephrosis who at birth was treated with vesicostomy. Three years later she come to our institution with a left renal exclusion and recurrent febrile ITU for laparoscopic nephroureterectomy.

CONCLUSIONS

The OS is a rare pathology with serious repercussions in the upper urinary tract and for that reason it must be recognized to start the urological management early and improve the kidney function in the adulthood.


15:11 - 15:30
Discussion