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.

Insights into Kidney Dysplasia in Duplex Kidneys: From Radiologic Diagnosis to Histopathologic Understanding.

Duplex kidney is a urinary tract anomaly commonly associated with a wide range of primary and secondary parenchymal structural abnormalities. We present a unique comparison of US and MRI findings with histopathology following partial resection of duplex kidneys due to nephropathy. We examined a group of 21 children with duplex kidneys who were qualified for heminephrectomy (24 kidney units (KU)). All patients underwent US and MRI prior to the surgery. The imaging results were compared with histopathologic findings. In 21/24 KU, dysplastic changes were found on histopathology, including all with obstructive nephropathy and 7/10 specimens with refluxing uropathy. The loss of corticomedullary differentiation on US and increased signal on T2-weighted images (T2WI) on MRI were the imaging findings that best correlated with fibrosis. In children with megaureter, there were no statistical differences in histopathological findings between primary megaureter, megaureter with ureterocele, and megaureter with ectopia (p > 0.05). The extent of dysplasia of the affected pole correlated negatively with residual function in MRI. Kidney dysplasia and inflammation in the kidney with obstructive nephropathy are the most important histopathologic findings of this study. US is a valuable screening tool, and MRI enables morphologic and functional assessments of the nephropathy in duplex kidneys.

Laparoscopic nephron-sparing surgery and laparoscopic nephrectomy for Wilms' tumor.

Introduction: The place of laparoscopy in the resection of Wilms' tumors has remained debatable, but evidence that neoadjuvant chemotherapy causes tumor shrinkage has made laparoscopic nephrectomy (LN) a feasible option. Laparoscopic nephron-sparing surgery (NSS) is technically very demanding and seems to be feasible and effective only when performed by a very experienced surgeon. Aim: We report 7 cases of laparoscopic nephrectomy and 1 case of laparoscopic heminephrectomy for Wilms' tumor. Material and methods: Forty-two consecutive children with primary renal tumor underwent nephrectomy between 2013 and 2020; 11 had an LN. Among them there were 8 children with Wilms' tumor, with age between 13 months and 7 years. All patients received neoadjuvant chemotherapy according to the current SIOP protocol. The mean tumor volume before and after chemotherapy was 174.4 ml (range: 14.7-501) and 32.8 ml (range: 4.3-68) respectively. Results: Seven laparoscopic nephrectomies and one heminephrectomy were performed. No patient had an intraoperative tumor rupture. All children had an attempt of lymph node sampling (their number per pathology assessment was in the range 0-5). There were no intraoperative events. One complication after heminephrectomy occurred, which was urine leak. Five patients had stage I tumor and 3 had stage II. Seven patients had an intermediate-risk and one had a high-risk tumor. There were no local or distant relapses. All patients remained disease-free at a median follow-up of 71 months (range: 16-94). Conclusions: This report demonstrates the feasibility of LN in children with Wilms' tumors. The patients should be carefully selected and specific training in laparoscopy is necessary, particularly with regard to laparoscopic heminephrectomy.

Laparoscopic resection of liver tumors in children.

BACKGROUND: Laparoscopy for the resection of liver tumors in children has remained undeveloped in comparison to adults. Most of the indications for pediatric laparoscopic hepatic surgery have been limited to diagnostic laparoscopy (biopsy). Over the past ten years, however, laparoscopic liver resections for pediatric hepatic diseases have been performed successfully, and many case reports have been published. METHODS: The authors report 6 cases of laparoscopic hepatic resection of benign tumors in children. The most important aspects of surgical technique are presented. There were 3 boys and 3 girls, with age between 4 months and 16 years. The lesions were located in the following segments: II and III (4 patients), I (1), V (1). The maximum tumor size was 7 cm. RESULTS: One anatomical (left bisegmentectomy) and 5 nonanatomical resections were performed. Conversion to laparotomy was necessary in 1 patient owing to bleeding from the posterior branch of the right hepatic artery. There were no postoperative complications and patients were discharged on postoperative day 4, 5, 5, 5, 7 and 3 accordingly. The postoperative pathology of the specimens confirmed their benign nature: infantile hemangioendothelioma (1), nested stromal epithelial tumor (1), focal nodular hyperplasia (3), mixed benign tumor (hamartoma + vascular malformation) (1). CONCLUSIONS: This report demonstrates the feasibility of a laparoscopic hepatic resection in children. On the other hand, laparoscopic liver resection is challenging and teamwork and specific training are necessary.

[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.

A Prospective Randomized Controlled Trial of Single-Port and Three-Port Laparoscopic Appendectomy in Children.

Background: This study aimed to evaluate single-port laparoscopic appendectomy (SPLA) in comparison with three-port laparoscopic appendectomy (3PLA) in children about the extent of surgical trauma after SPLA and 3PLA measured by serum interleukin-6 (IL-6) and C-reactive protein (CRP) concentrations. Materials and Methods: A total of 50 patients with a median age of 11.5 were randomized to two groups. Experts in both methods performed the surgeries. The decision on the type of planned surgery was randomly determined. Serum IL-6 and CRP levels were measured using an enzyme-linked immunosorbent assay before, and at 12 and 36 hours after surgery. Furthermore, we compared operating time, hospital stay, postoperative pain, and complication rates. Results: The operative time in the 3PLA group was shorter than that in the SPLA group (P < .05). Preoperative IL-6 levels were not different between the two groups, but the rise (pre- versus postoperative) of IL-6 in the SPLA group was remarkably higher when compared with the 3PLA group (P < .05). Similar results were obtained for CRP; basal serum CRP levels were not different between the two groups, but the rise of CRP in the 3PLA group was significantly lower compared with that in the SPLA group. During the first 12 hours postoperative, the SPLA patients reported more severe postoperative pain and longer inpatient opiate usage was noted that after 3-PLA. Only one SPLA case was converted to 3PLA. There were no conversions to open surgery. The length of hospital stay and complication rate were not different between the two groups. Conclusions: SPLA in children is associated with longer operative times, increased pain level, and more severe surgical trauma as measured by postoperative CRP and IL-6 levels in comparison with a 3PLA. The two approaches were comparable regarding the length of hospital stay and complication rate.

[Evolving pattern of bladder dysfunction in boys with posterior urethral valves (PUV)]. / Przebieg zaburzen funcji pecherza moczowego u pacjentów z zastawka cewki tylnej na podstawie badan urodynamicznych.

UNLABELLED: Bladder dysfunction is present in 50-80% boys born with PUV. THE AIM OF THE STUDY: To assess the effect of age on the pattern of voiding dysfunction. MATERIAL AND METHODS: An analysis of urodynamic findings was performed in 62 boys with PUV divided into younger (mean 6.02 yrs) and older (mean 15.6 yrs) age groups. RESULTS: In younger boys a higher prevalence of decreased bladder compliance (p < 0.0001), detrusor instability (p < 0.001) and detrusor sphincter dyssynergy (p < 0.05) was noted. In older boys a higher prevalence of low detrusor pressure was observed (p < 0.01). CONCLUSION: The pattern of bladder dysfunction in boys with PUV evolves with age.

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.

[Chronic renal disease in boys with posterior urethral valve]. / Przewlekla choroba nerek u chlopców z zastawka cewki tylnej.

UNLABELLED: Posterior urethral valve (PUV) is the most common anatomical cause of bladder outlet obstruction with an incidence ranging from 1/3000 to 1/8000 births. In spite of early valve ablation deterioration of renal function is frequently reported during childhood and adolescence. AIM OF THE STUDY: To evaluate the frequency and progression of chronic renal disease in boys born with PUV. MATERIALS AND METHODS: The presence of chronic renal disease (CRD) in 58 boys with posterior urethral valve (PUV) was assessed by renal ultrasound and estimation of glomerular filtration rate (GFR). GFR was estimated by the method of Schwartz. Chronic renal disease was defined according to NFK K/DOQI Guidelines and classified into 5 stages. RESULTS: 48 (82%) patients with PUV had CRD. Among the patients observed from birth 89% already demonstrated CRD in the first year of life. CONCLUSIONS: 1. The vast majority of boys with PUV (83%) demonstrate symptoms of chronic renal disease. 2. CRD in boys with PUV is frequently evident from the first year of life. 3. PUV patients require regular nephrologic assessment from birth.

The role of laparoscopy in the management of pediatric appendicitis: a survey of Polish pediatric surgeons.

INTRODUCTION: Despite the increase in use of laparoscopic appendectomy (LA) in recent years, until now no nationwide survey on this issue has been performed in Poland. AIM: To determine current surgical practice patterns among Polish pediatric surgeons in the treatment of appendicitis in children. MATERIAL AND METHODS: The nationwide survey was conducted in the form of an internet questionnaire asking for information concerning treatment of children suspected of having acute appendicitis in the years 2007-2011. Twenty-seven major pediatric surgical departments in Poland were invited to participate in the study. RESULTS: The overall survey response rate was 70.37%. Laparoscopic appendectomy was offered in all departments except one. Laparoscopy was used in 33% of cases and was the standard procedure in 4 departments. In 1 center 100% of appendectomies were performed laparoscopically. The 3-port technique was used in all departments. Five centers offered transumbilical laparoscopic extracorporeal appendectomy and 2 centers single-port appendectomy. In LA the mesoappendix was mostly divided using bipolar or monopolar coagulation and the appendix was ligated using endoloops or was clipped. The mean hospitalization time was 3.31 days after laparoscopy and 5.47 days after open appendectomy. CONCLUSIONS: Despite an apparent consensus on some aspects of pediatric appendicitis among Polish pediatric surgeons, significant inconsistency exists in the operative approach. The low rate of LA suggests that LA still remains far from being a standard. Personal experience and preference have a major influence on the choice of operative approach. It is recommended that national guidelines be set up, which could standardize the care for children and training of pediatric surgery residents.

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.

One, two or three port appendectomy - a rational approach.

INTRODUCTION: Laparoscopic appendectomy is a safe and feasible technique accepted by many surgeons as the gold standard approach for the treatment of acute appendicitis in children. Traditionally laparoscopic appendectomy requires the use of three ports. However, surgical techniques with fewer ports have been reported. AIM: To evaluate the efficacy of laparoscopic appendectomy in children according to the proposed 3-step protocol using one, two or three ports. MATERIAL AND METHODS: A total of 100 children with the diagnosis of acute appendicitis underwent laparoscopic appendectomy. Patients were treated according to the following protocol: transumbilical access with one 10 mm port using the laparoscope with working channel. The appendix was mobilized and delivered through the umbilical port and tied extracorporeally and removed. If the appendix was placed retrocecally or had adhesions, a second port was introduced. The appendix was mobilized and finally retrieved from the abdominal cavity through the camera port, and resected extracorporeally. In the cases of very short and gangrenous appendix and immobile colon, a third port was introduced and totally intra-abdominal appendectomy was performed. Patients were evaluated regarding the duration of the operation, and operative and postoperative complications. RESULTS: During the study period 100 children (58 males, 42 females) had laparoscopic appendectomy: 48 children by one-port technique (group I), 27 children by two-port technique (group II) and 25 children by three-port technique (group III). The mean operative time was 33 min (20-55 min) in group I, 39 min in group II (23-60 min), and 49 min (30-75 min) in group III. There were no intraoperative complications. Wound infections were recorded in 4 (8.3%) patients in group I, three (11.1%) in group II and four (16.0%) in group III. One patient in group III developed an abdominal abscess managed conservatively. CONCLUSIONS: One-port laparoscopic appendectomy is a feasible technique in children. It allows 48% of children to have the operation. The addition of a second port allows one to mobilize the appendix and perform extracorporeal resection in an additional 27% of cases. These approaches have shorter operative time compared to 3-port technique. Laparoscopic extracorporeal appendectomy, especially one-port, is found to be cost effective and have excellent cosmetic results.

Laparoscopic single port surgery nephrectomy in a child - initial experience.

The aim of the stucdy was to present technical aspects and possible intraoperative complications based on the first single port surgery (SPS) laparoscopic nephrectomy performed in our center. The SPS laparoscopic nephrectomy of the right kidney was performed using a transperitoneal access in a 4-year-old child due to a small nonfunctional kidney complicated by hypertension. The intraoperative course was complicated by rupture of the gall bladder wall after grasper removal without leakage of its contents into the peritoneal cavity. No postoperative complications occurred. The SPS nephrectomy appears to be an advantageous method in children which offers a good cosmetic effect. Other benefits require confirmation in further studies. Fixation of the gall bladder is a very helpful but risky maneuver.

Laparoscopic management of giant ovarian cysts in adolescents.

A laparoscopic approach to giant ovarian cysts in girls, when the cyst's size exceeds the umbilicus, may be difficult regarding the risk of cyst rupture and limited working space. We present a series of three 12-year-old girls with giant ovarian cysts treated by percutaneous drainage under ultrasonographic guidance followed by laparoscopic resection. Clinical, laboratory and radiological diagnosis showed no signs of malignancy. After induction of general endotracheal anaesthesia, under ultrasonographic guidance, a 10 F vesicostomy catheter was placed into the cyst. Two to 4 l of serous (in one case mucinous) fluid were drained from the cyst. During laparoscopy, the abdominal cavity was inspected by the scope and no signs of malignancy were found. The laparoscopic procedure was completed with excision of the cyst and keeping some ovarian tissue in all cases. No intraoperative complications were observed. The mean operation time was 73 min. Pathological examination revealed a mature cystic teratoma in two cases and mucinous cystadenoma in one. The postoperative recovery was uneventful in all girls and they were discharged home on postoperative day 2-4 with minimal pain. The ultrasound scans and tumour markers were normal on follow-up after 3 and 6 months. Laparoscopic excision of giant ovarian cysts after ultrasound-guided drainage seems to be a safe and applicable treatment modality in pre-menarchal girls with a normal tumour marker profile and benign features of the cyst, excluding the possibility of malignancy.