A unique defect - persistent posterior cloaca: An example of staged genito-urinary and digestive tract reconstruction with an alternative vaginal creation using the urinary bladder wall.

We present a case of a girl with an extremely rare, posterior type of persistent cloaca, which was associated with other abnormalities, including an undeveloped vulva and vagina, agenesis of the right kidney, secondary obstructive megaureter, unicornate uterus, persisted tailgut, sacral bone hypoplasia, and pubic symphysis hypertrophy. An operative approach was as follows: (i) colostomy and ureterocutaneostomy; (ii) creation of an ileal conduit with antirefluxing uretero-ileal anastomosis, and then creation of a continent catheterizable ileal reservoir; (iii) anastomosis of sigmoid colon to rectal stump; and (iv) vaginal and external genital reconstruction. Because of abnormal anatomical conditions where the uterus was situated adjacent to the open, incompetent bladder neck, we decided to create a vagina using the bladder wall instead of the bowel segment.

Reconstructive option after radical mutilating surgery in children with genitourinary rhabdomyosarcoma: When sparing the bladder is not an option.

OBJECTIVES: To present versatile surgical reconstructive techniques and their outcomes in pediatric patients with genitourinary rhabdomyosarcoma. METHODS: We retrospectively analyzed the oncological and urological outcomes of seven patients treated between 1992 and 2014 according to the Cooperative Weichteilsarkom Studiengruppe protocols. Intergroup Rhabdomyosarcoma Study staging: local, six patients; and IV, one patient. HISTOLOGY: embryonal, five patients; unclassified, one patient; triton tumor one patient. Surgical treatment included: cystectomy, uterectomy and partial vaginectomy, one patient; radical cystectomy, two patients; cystectomy, one patient; cystectomy with partial prostatectomy, one patient; partial cystectomy, one patient; and partial prostatectomy, one patient. RESULTS: All patients were alive in complete remission at last follow up. In four cases, ileal conduit with ureteral reimplantation with serous-lined extramural tunnel (Abol-Enein technique) was carried out, which was followed by conversion into ileal continent bladder with continent appendiceal stoma for clean intermittent catheterization in three patients. In one boy, partial cystectomy and continent reconstruction was carried out during a single surgical procedure. One child with incontinent urinary diversion is still awaiting a continence solution. One child after partial prostatectomy is continent without any voiding disturbances. CONCLUSIONS: The timing and extent of radical surgery for treatment of genitourinary rhabdomyosarcoma depend on the local anatomical conditions, and the response to previous chemo- and radiotherapy. Cystectomy followed by various reconstructive techniques still remains an important option in the local treatment.

[Program of early nephro-urologic management of children with congenital hydronephrosis]. / Zalozenia programu wczesnej opieki nefro-urologicznej nad dziecmi z wrodzonym wodonerczem.

Congenital anomalies of kidney and urinary tract (CAKUT) are the main cause of end stage renal disease in childhood. Early prenatal detection with planned postnatal diagnosis and therapy are the mainstay of management of neonates with CAKUT which is aimed at the conservation of renal tissue. The above assumptions led to the establishment of the Pommeranian Program for Management of Children with CAKUT. The strategy of the program is to coordinate prenatal diagnosis performed by obstetricians, postnatal care by neonatologists and early management by pediatric nephrologists and urologists. It will involve approximately 200 neonates annually. The basic concept of the program includes the following: 1. Delivery of a child with congenital hydronephrosis detected prenatal should take place in a center with specialist neonatal care. 2. Child with a congenital hydronephrosis should remain under specialist nephro-urologic care immediately after delivery. 3. Child with a congenital hydronephrosis should be qualified to scheduled urologic surgery after results of diagnostic tests and according to general status. 4. Model of integrated care on a child with congenital hydronephrosis should consist in close cooperation between obstetricians, neonatologists, pediatric nephrologists, and urologists.

[Preliminary results of a program for the early management of children with congenital hydronephrosis]. / Wstepne wyniki programu wczesnej opieki nefro-urologicznej nad dziecmi z wrodzonym wodonerczem.

UNLABELLED: Antenatal hydronephrosis is one of the most frequently diagnosed congenital abnormalities in the fetus. The aim of the study is to present the preliminary results of a newly launched Pommeranian Program for Early Management of CAKUT in Children. MATERIAL AND METHODS: 105 neonates and infants with hydronephrosis were assessed between Jan and Dec 2007 (27 girls and 78 boys). All patients had postnatal ultrasound performed. Static and dynamic scyntigraphy and cystography were performed according to set indications. Therapeutic decisions were made following a team meeting between pediatric nephrologists and urologists. RESULTS: 56.2% of children with hydronephrosis had documented prenatal findings: 61%--pyelectasis, 15.3% renal cysts, 5.1% unspecified renal pathology, in 18.6% no renal pathology. 82.6% of newborns had term births but perinatal complications were observed in 30.5%. Initial postnatal diagnosis was performed by neonatal wards in 22.8% newborns and by the children's pediatrician in 31.4% of infants. Following referral to nephro-urology centre cystography was performed in 22.9% of infants and scintigraphy in 36.2%. 21.9% of infants were qualified for surgical intervention at an average age of 24 + 13 weeks. CONCLUSIONS: 1. Only half of the children with congenital hydronephrosis are to nephro-urology care following prenatal diagnosis. 2. A large number of perinatal complications are observed in newborns with prenatally diagnosed hydronephrosis. 3. The management of newborn hydronephrosis requires better cooperation between obstetricians, neonatologists, pediatric nephrologists and urologists.

[Pre-emptive renal transplantation in a boy with obstructive uropathy following reconstruction procedures]. / Przeszczep nerki u pacjenta z zastawka cewki tylnej (ZCT) po zabiegach plastyki pecherza moczowego.

Patients with bladder dysfunction comprise over 30% of pediatric patients on renal replacement therapy. We report on a successful cadaveric pre-emptive renal transplantation performed in a boy born with posterior urethral valve. Following bilateral ureterocutaneostomies, left nephrectomy and valve resection, at 6 years of age a continent ileocolonocystoplasty was performed. The boy started intermittent daytime catheterization, passing urine both via urethra and fistula. At the age of 18 he received a renal transplant. Continuing the previous regime, at 1.5 years follow up his graft is well functioning (GFR >75 ml/min/1.73 m2) with sporadic episodes of urinary tract infection.

Dye-assisted lymph vessels sparing laparoscopic varicocelectomy.

OBJECTIVE: Hydrocele, the main complication of laparoscopic varicocelectomy, is thought to result from a disruption of gonadal lymphatics. The aim of this study was to evaluate the effectiveness of patent blue V dyeing to identify and preserve lymphatic vessels and to assess whether the lymphatic sparing technique avoids postoperative hydrocele in adolescent boys undergoing a laparoscopic procedure. MATERIALS AND METHODS: Fifty-two (52) boys affected by varicocele Grade III (range, 12-16 years) underwent a left-sided laparoscopic varicocelectomy. Twenty-six (26) boys were randomly assigned to a lymphatic nonsparing (LNS) group, and the others to a lymphatic sparing (LS) group. Before surgery in the LS group, 2 mL of patent blue V was injected under the tunica dartos on the left side. RESULTS: All varicocelectomies were performed laparoscopically. Lymphatic vessels were identified in 23 (88.5%) boys of the LS group. In the remaining three (11.5%), the lymphatics could not be identified clearly. No adverse local or generalized reactions were noted. At a mean follow-up of 14 months, no recurrent varicocele or testicular volume reduction were detected. Hydrocele developed in 4 LNS patients and 1 was operated on. No patient from the LS group developed a hydrocele. CONCLUSIONS: Staining gonadal lymph vessels with patent blue V is an effective and simple method of visualization of the lymphatic drainage from the testis. Blue-stained lymph vessels could be readily distinguished and preserved during a laparoscopic varicocelectomy, which results in a decrease of hydrocele development. To validate an efficacy of vital staining of lymphatic vessels in avoiding hydrocele formation, a larger series and longer follow-up are necessary.

A review on surgical techniques and organ sparing procedures in bladder/prostate rhabdomyosarcoma.

The treatment outcomes in children with bladder/prostate rhabdomyosarcoma (B/P RMS) have considerably improved in the past few decades. Current protocols incorporate chemotherapy, radiotherapy, and surgery (radical or organ sparing). Using this multimodal and multidisciplinary approach, the cure rates for nonmetastatic RMS have gradually increased from 25% in the 1970s, and 70% in the 1990s, to over 80% in the 2000s. Surgery plays a role not only in the diagnosis and management of all stages of B/P RMS, but also in the urinary reconstruction and long-term follow-up. Furthermore, the tendency to avoid mutilating surgery and to preserve functional genitourinary tract has been noticeable. Authors describe the current status and future surgical treatment approaches and possibilities for patients with B/P RMS and how reconstructive urologic surgery in B/P RMS continues to evolve.

Laparoscopic versus open upper pole heminephroureterectomy for the treatment of duplex kidneys in children.

AIM: To evaluate the safety and feasibility of laparoscopic upper pole heminephroureterectomy (HNU) in pediatric patients with duplex kidneys in comparison with open surgery. PATIENTS AND METHODS: From 2007 to 2011, 27 patients underwent HNU. A dimercaptosuccinic acid (DMSA) isotope scan revealed hypofunctioning of ipsilateral moieties in all cases. The patients were divided randomly into laparoscopic HNU (LHNU) and open surgery HNU (OHNU) groups. LHNU was performed by the transperitoneal approach in 10 girls and 5 boys with a mean age of 33 months (range, 9-108 months). All procedures were performed using three or four ports. Open surgery was performed in 10 girls and 2 boys with a mean age of 29 months (range, 7-174 months) by the retroperitoneal approach in all cases. Follow-up included ultrasound and DMSA. RESULTS: Mean operative time in the LHNU and OHNU groups was 148 minutes (range, 100-220 minutes; 95% confidence interval [CI] 129-167 minutes) and 124 minutes (range, 100-150 minutes; 95% CI 115-133 minutes), respectively. In the LHNU and OHNU groups, mean analgesic requirement was 2.8 days (range, 2-4 days; 95% CI 2.4-3.2 days) and 3.7 days (range, 3-5 days; 95% CI 3.3-4.1 days), and mean hospital stay was 4.0 days (range, 2-8 days; 95% CI 3.2-4.8 days) and 5.1 days (range, 3-8 days; 95% CI 4.3-5.9 days), respectively. No intraoperative or major postoperative complications occurred. No conversion to open surgery was necessary in the LHNU group. Statistical analysis showed no statistical significant difference (P>.05) in the operating time between groups, whereas differences in the mean hospital stay (P=.048) and analgesic requirements (P=.005) were significant. The function of the remnant pole was preserved in all patients. Follow-up ultrasound showed asymptomatic cystic structures in 1 patient. CONCLUSIONS: The laparoscopic upper pole HNU is a safe and feasible procedure, even in infants. It is associated with minimal morbidity, and the operative time is acceptable and not significantly longer in comparison with the open approach. LHNU reduces analgesic requirements and hospital stay. In our opinion it should be the preferred option for HNU in children.

Rupture of the normal renal pelvis after blunt abdominal trauma.

Blunt abdominal trauma with isolated rupture of the normal renal pelvis is an extremely rare clinical entity. The correct preoperative diagnosis may be difficult and challenging because of the insidious onset, lack of blood loss, and frequently, lack of urinary symptoms. Intraoperative inspection of the collecting system is the most accurate method to fully define an extent of injury. The object of the case report is to present the case of a 15-year-old male with isolated pelvic rupture of a normal kidney because of strong punch in the abdomen during the handball match.

[Neurofibromatosis type 1 in children. Experiences of the Gdansk Paediatric Oncohaematology Centre. Preliminary results]. / Nerwiakowlókniakowatosc typu 1 u dzieci. Obserwacje osrodka gdanskiego. Doniesienia wstepne.

INTRODUCTION: Neurofibromatosis type 1 (NF1) is a frequent genetic disorder of autosomal-dominant pattern. The incidence is about 1 per 3000 live births. Patients with NF1 are predisposed to malignancies including soft tissue sarcomas and leukaemias. The aim of the study was assessment of the most frequent symptoms on the basis of long term observation of children with NF1 and presentation of implemented diagnostic and therapeutic procedures. MATERIAL AND METHODS: In our department there are 149 children (71 boys and 78 girls) aged from 7 months to 18 yrs with diagnosed or suspected NF1. Each child is carefully followed up every 6 months on outpatient basis. Paediatric, neurological and opthalmological examinations are performed during the first visit and in cases of any new symptoms. Number of Lisch nodules, vision field, audiogram, dermatological evaluation of skin abnormalities as well as orthopaedic examination are also investigated. In any case of NF1 without neurological symptoms, MRI of the brain and spine is carried out every 2 years. Moreover, each child is consulted in the Genetic Clinic. RESULTS: Cafe-au-lait spots were observed in all 149 children, freckling of the armpits in 40, peripheral neurofibromas in 30, Lisch nodules in 2 patients. Secondary symptoms and complications such as mental retardation (9 cases) and epilepsy (10 cases), cognitive disorders and learning disabilities (21), abnormalities in MRI examination (53), benign or malignant CNS tumours (9), scoliosis (99) were diagnosed. In 5 patients malignant neoplasms occurred (3.4%) including: RMS--2 cases, Triton tumour--1 case, MPNST--1 case. Two children died of disease progression, one of treatment complications (sepsis) and two children are alive. CONCLUSIONS: 1. Patients with NF1 need regular specialist medical care. 2. Continuous education of the families with this disease is necessary. 3. Diagnostic and therapeutic procedures recommended for patients with NF1 need to be implemented at different levels of health care.

Abdominal inflammatory masses mimicking neoplasia in children-experience of two centers.

Despite progress in modern imaging, some inflammatory masses are difficult to distinguish clinically from neoplastic processes. In such cases the pathology report has a great distinctive value, but even then the final diagnosis may be difficult to reach. Eight patients with abdominal tumors of inflammatory origin were treated in two institutions, the Department of Pediatric Surgery of the Medical University of Gdansk, Poland, and Helios Center of Pediatric Surgery in Berlin, Germany, during the last 10 years. Four tumors were located in the pelvis, two in the liver, and two in the colonic mesentery. Five of them were inflammatory pseudotumors (two subclassified as inflammatory fibrosarcoma), one had nonspecific inflammatory changes, one was diagnosed as idiopathic retroperitoneal fibrosis, and one was diagnosed as bacillary angiomatosis. All patients underwent surgical tumor biopsy, excisional in four and incisional in four. All but two children underwent macroscopically complete tumor excision (four primarily, two secondarily). In one case the tumor resolved with antibiotherapy. Surgery in retroperitoneal masses was often extensive and associated with significant complications because of invasive tumor growth. In conclusion, intraabdominal inflammatory lesions may closely mimic neoplasia in children. Clinical doubts result in repeated biopsies, and for this reason excisional biopsy should be preferred. In some cases, when excisional biopsy is not feasible due to invasive growth of the tumor, delayed complete mass excision should follow, despite occasional significant morbidity. The etiology and exact nature of inflammatory pseudotumors are still obscure, and it is unknown whether they represent inflammatory lesions or true neoplasia.

[Relapses in children with nephroblastoma--a single centre experience]. / Wznowy w nerczaku plodowym u dzieci. Badania wlasne.

UNLABELLED: The aim of the study was to assess the incidence of relapse and outcome of treatment in children with nephroblastoma relapse. MATERIAL AND METHODS: In the years 1989-2002 at our centre 67 children were treated for nephroblastoma. Patients were divided into two subgroups. Group I comprised 25 children treated in accordance with the SIOP-9 programme, whilst group II consisted of 42 patients following the SIOP 93-01 protocol. RESULTS: In group I, five children had relapse diagnosed: local in 2, pulmonary in 2 and cerebral in one case. Two of the cases were diagnosed during the treatment, and the other 3 after 1 to 19 months from the completion of treatment. In group II relapse was diagnosed in 4 children: local in 2 and pulmonary in 2 cases. One case was diagnosed while on treatment, and the other 3 were diagnosed 1,2 and 4 months after completion of treatment. Among the children who followed the SIOP-9 programme three patients died, while among those following SIOP 93-01 two patients died. CONCLUSIONS: The prognosis in patients who relapsed remains uncertain. The histological type of tumour and the moment of relapse occurrence are significant prognostic factors.