The Prevalence of Redo-Ureteroneocystostomy and Associated Risk Factors inPediatric Vesicoureteral Reflux Patients Treated with Ureteroneocystostomy.

PURPOSE: The aim of the study was to examine the prevalence of redo-ureteroneocystostomy (redo-UNC) in pediatric vesicouretheral reflux (VUR) patients following open UNC and factors associated with redo-UNC. MATERIAL AND METHODS: Data on 122 patients who underwent open UNC for VUR were analyzed in this retrospective case-control study. The patients were divided into a successful initial UNC group (UNC group, control) and an unsuccessful initial UNC group (redo-UNC group, case). The following variables were analyzed: sex, age, dysfunctional voiding, laterality of VUR (unilateral or bilateral), VUR grade, etiology of VUR (primary or secondary), relative renal function on renal scintigraphy, and surgical technique. The use of the following procedures in the initial UNC was recorded: an endoscopic subureteric injection(ESI) and ureteral tapering. RESULTS: In our clinic, 122 patients (177 ureters), with an average age of 55.7 ± 41.2 months (range, 1-18 years) underwent open UNC for VUR between November 2005 and June 2014. Of these,67 (55%) had unilateral VUR, and 55 (45%) had bilateral VUR. There were 127 (71.8%) cases of grade 4-5 reflux. Postoperatively, hydronephrosis was noted in 19 (15.6%) patents. Ten (8.2%) patients underwent redo-UNC. In eight cases (6.5%), redo-UNC was performed because of ureterovesical (UV) junction obstruction.In the other two cases (1.7%), redo-UNC was dueto high-grade reflux. There were no statistically significant differences between the redo-UNC and UNC groups in any of the variables studied. CONCLUSION: Redo-UNC was required in 10 (8.2%) of cases after UNC. Age, sex, laterality of VUR, VUR grade,existence of primary or secondary VUR, relative renal function on renal scintigraphy, UNC technique, ESI procedure,and ureteral tapering were not risk factors for redo-UNC in our series.

Can Freehand Elastosonography Be an Alternative to Renal Scintigraphy in Pediatric Vesicoureteral Reflux Cases?

INTRODUCTION: Detecting renal scar is important in pediatric patients with vesicoureteral reflux (VUR) for deciding on treatment option. The aim of this study is to detect whether freehand elastosonography technique could be an alternative to dimercaptosuccinic acid (DMSA) scan in determining renal scar formation. MATERIALS AND METHODS: Between November 2015 and April 2016, 25 VUR patients, age ranging from 3 to 17 years admitted to our clinic, had urinary ultrasound and elastosonography, and data of approximately 147 renal region were recorded. Data were upper, middle, and lower pole renal parenchymal thickness and echogenicities obtained by ultrasound and these poles strain target (ST), strain reference (SR), and strain index (SI) values obtained by freehand elastosonography. DMSA scan data (differential function and upper, middle, and lower pole parenchymal scar formation) were recorded. RESULTS: Scar formation and more than 10% reduction in differential function in renal scan were statistically higher in renal regions in which parenchymal thinning and echogenicity increase was detected by ultrasound. There was no elastosonographic data difference between renal units with and without differential function decrease. Also, there was no elastosonographic data difference between renal units with and without scar formation. CONCLUSION: In this study, we could not find any significant difference in term of tissue tension values (ST and SI) measured by freehand elastosonography between renal units with and without scar formation in renal scan.

Pediatric bladder/prostate rhabdomyosarcoma: Eight cases from a single center.

In this study, clinical characteristics, treatment modalities and outcome of patients diagnosed with bladder/prostate rhabdomyosarcoma (BP RMS) were evaluated retrospectively. Files of 8 children diagnosed with BP RMS and treated between 2004-2014 were reviewed for clinical characteristics, treatment modalities and outcome. Seven males and one female were diagnosed with BP RMS between 2004-2014. Median age was 33.5 months (range, 2 to 176 months). At presentation the main clinical symptoms were hematuria in 5 patients, and constipation, oliguria and prolonged jaundice in 1 patient each. All patients were non-metastatic and only one had an embryonal histology. Primary resection before chemotherapy was performed on only one patient. Six patients were treated initially with VAC chemotherapy for 12 weeks, two patients were treated PIAV (ifosfamide, cisplatin, doxorubicin, vincristine). Local relapse or progressive disease occurred in 5 of 8 patients, and two of these patients underwent primary or secondary tumor resection without radiotherapy. Three patients developed a local relapse after combination of radiochemotherapy and tumor resection. Radical surgical treatment was performed in 3 patients with local relapse. Only one patient underwent partial cystectomy. Six of 8 patients were alive and under follow-up without disease at a median survival of 53 months (range, 13 to 78 months). BP RMS requires a multidisciplinary treatment approach. There is a general consensus that chemotherapy is the mainstay of treatment in BP RMS, but the method to be used for local control is controversial, and may vary from case to case in this heterogeneous disease.

Management of stone disease in infants.

Evaluating and treating renal stone disease in infants are technically challenging. In this study, we evaluated the surgical treatment of renal stones in children under 1 year of age. We retrospectively reviewed the records of patients under 1 year old who were treated with ESWL, endourological or open surgical procedures for renal stone disease between January, 2009 and December, 2012. The patients' age, gender, stone size, stone location and number, complications, stone-free status, and postoperative complications were recorded. 19 of 121 infants with a mean age of 10.2 ± 3.07 months were treated with surgical procedures. Six (75%) of eight cystinuria patients required a surgical intervention. Retrograde endoscopic management was performed in thirteen patients (63.4%) as an initial surgical approach. There were three major (15.7%) complications. The rate of open surgical procedures was 31.6% (6 of 19 infants). The cutoff value of stone size for open surgery was 10 mm. There was a significant relationship between the conversion to open procedures and stone size, stone location, and symptom presentation especially the presence of obstruction (p < 0.05). After repeated treatments, the stone clearance rate of RIRS reached 84.6%. Retrograde intrarenal surgery is an effective and safe treatment method for renal stones in infants and can be used as a first-line therapy in most patients under 1 year old. This is especially important if an associated ureteral stone or lower pole stone that requires treatment is present and for patients with cystinuria, which does not respond favorably to ESWL.

Teratoid Wilms' tumor exhibiting extensive squamous differentiation.

Teratoid Wilms' tumor is a rare renal tumor. Herein, we report an unusual variant of such tumor which simulated renal teratoma because of abundant keratinized squamous epithelium within the tumor.

Actinomycosis of the appendix in childhood- an unusual cause of appendicitis.

Actinomycosis is a rare chronic bacterial infectious disease in childhood. A 14-year-old boy admitted with cramping abdominal pain and vomiting. Physical examination revealed right lower quadrant tenderness. Appendectomy was performed. On the histological section, typical actinomycotic (sulfur) granules in the appendiceal lumen were observed.

Retrograde intrarenal surgery for the treatment of renal stones in children: factors influencing stone clearance and complications.

BACKGROUND: Retrograde intrarenal surgery (RIRS) is a known option for the treatment of upper tract calculi with an excellent success. However, the reports of RIRS in prepubertal children are limited. In this study, we evaluated the factors which affected the success rate and the complications of RIRS at renal stone treatment in childhood. METHODS: We retrospectively reviewed the records of children under 14 years old who underwent RIRS for renal stone disease between January 2009 and December 2012. Patients' age, gender, body mass index (BMI), stone size, stone location, stone number, intraoperative complications, stone free status, postoperative complications were recorded. RESULTS: There were 80 ureterorenoscopic procedures performed in 58 renal units of 47 children (23 males and 24 females). The patients' ages ranged from 8 months to 14 years (mean age 4.7 ± 3.4 years). There was a difference in the distribution of symptoms in age groups. UTI was higher in the 1-4 years age group, abdominal pain was seen mostly in children aged 5-14 years. Multiple stones (included staghorn stone) were noted in 60.4% of patients. In 27.6% of patients, ureteral stones were accompanied by renal stones in our series. In the infancy group, cystine and staghorn stones were more frequently seen, mostly bilateral. After a single ureteroscopic procedure for intrarenal stones in children, we achieved stone free status in 50.9% of the ureters (n=26). After the repeated sessions, the stone clearance rate reached to 85.1%. CONCLUSION: Retrograde intrarenal surgery can be used as a first line therapy to treat renal stones in children. This is especially important if an associated ureteral stone is present that requires treatment; or in patients with cystinuria, which is not favorably treated with ESWL. Complications were seen more frequently in patients with cystine stones. Extravasation was noted more frequently in patients admitted with UTIs. There was a significant relationship between the conversion to open procedures and the age groups, with most procedures occurring in infancy. The parents should be informed about the probability of multiple procedures to achieve stone free status.

Cytomegalovirus-related hemorrhagic cystitis in an immunocompetent child.

Cytomegalovirus (CMV) infections are mostly seen in immunocompromised patients. However, unusual manifestations or complications of acquired CMV infections in immunocompetent patients are rarely reported. CMV-related hemorrhagic cystitis is extremely rare but should be considered even in immunocompetent patients. We present a case of a 3-year-old immunocompetent boy with intermittent, terminal gross hematuria lasting for 1 month. There was no history of genitourinary trauma or stone disease. Urine analysis revealed hematuria with eumorphic red blood cells and no proteinuria. Urine culture was negative. Ultrasonography showed increased bladder wall thickness and irregularity at inferior of bladder. Cystoscopy revealed hyperemia and edema. Histopathological examination was consistent with CMV infection, viral DNA by polymerase chain reaction in peripheral blood and urine were positive. Clinical, laboratory, and imaging features pointed towards hemorrhagic cystitis due to CMV. He was followed-up with no treatment. After 1 month, repeated investigations showed complete resolution of finding. This is a rare description of an immunocompetent child with CMV-induced cystitis.

Utility of peritoneal dialysis in neonates affected by inborn errors of metabolism.

AIM: Some inborn errors of metabolism induce metabolic encephalopathy through accumulation of neurotoxic metabolites. Rapid elimination of these metabolites by peritoneal or extracorporeal dialysis is crucial to prevent neuronal damage or death. In this retrospective study, we evaluated the outcomes of nine neonates with metabolic crisis treated with peritoneal dialysis. METHOD: Six neonates with hyperammonemic coma (four with organic acidemias, two with urea cycle disorders) and three with leucine accumulation due to maple syrup urine disease (MSUD) were managed with peritoneal dialysis in conjunction with dietary and pharmacological therapy. RESULTS: Three patients with organic acidemia survived. One of the patients was normal; others had moderate and severe neurological impairments. One neonate with organic acidemia and both neonates with urea cycle disorders died. Two of the three patients with MSUD survived without neurological impairment; the other had severe neurological damage and died at 9 months of age due to sepsis. CONCLUSION: Theoretically, extracorporeal dialysis should be the first dialysis treatment of choice; however, this report demonstrates that peritoneal dialysis has a chance to prevent neurological damage in some patients. Therefore, in developing countries without extracorporeal dialysis opportunities, it can be still a life-saving procedure, if it is applied with skilled staff and standard procedures.

Ureteroscopy for treatment of ureteral stones in children: factors influencing the outcome.

OBJECTIVE: To evaluate the factors that affect the success and complication rate of ureteroscopy for ureteral stone treatment in children. MATERIALS AND METHODS: We retrospectively reviewed the records of children who were treated for ureteral stones at our institution from 2009 to 2011. The demographic data, stone size, stone location, number of stones, intraoperative complications, stone-free status, postoperative complications, and conversion to an open procedure were recorded. RESULTS: A total of 54 ureteroscopic procedures were performed in 32 children. The mean age was 5.91 ± 4.98 years. Conversion to an open surgical procedure was required in 6 patients (18.75%). A significant relationship was found between conversion to an open procedure and patient age. Although the initial complete clearance rate was 57% after the first session, overall, the stone-free rate was 92.68%. A significant relationship was found between stone-free status and patient age and stone composition. Our overall complication rate was 9.7%. Only 1 major complication (2.4%) occurred. A significant relationship was found between the occurrence of complications and patient age. CONCLUSION: Ureteroscopy is an effective method to treat ureteral stones in children. Cystine stones and lower patient age carry a risk of not achieving a stone-free status. The complication rate and conversion to an open procedure were greater in patients <5 years old. Parents should be informed before treatment that their children might require multiple treatment sessions.

Importance of anorectal manometry after definitive surgery for Hirschsprung's disease in children.

OBJECTIVES: The purpose of this investigation is to evaluate anorectal function after definitive surgery for Hirschsprung's disease (HD) by anorectal manometry. MATERIALS AND METHODS: We evaluated the anorectal manometric assessment of 18 children who were operated for HD. Functional outcomes were determined by a questionnaire. Rectoanal inhibitory reflex (RAIR) and maximum anal resting pressure (MARP) were monitored. The results were compared between obstructive patients and asymptomatic patients. RESULTS: The median age at definitive operation was 19 months (range 12-72 months). Anorectal manometry was performed in 14 male and 4 female patients. All the cases underwent three staged procedure for HD and modified Duhamel procedure was performed as definitive procedure for all the patients. Mean age was 4.3 months (range 25 days to 5 years) at time of diagnosis. Post-operative enterocolitis or severe constipation was observed in seven patients (38.8%). There were no patients with incontinence. Eighteen patients underwent anorectal manometry meanly 2 years after definitive operation. RAIR was absent in 14 (77.7%) patients and abnormal in 4 (22.2%). There were no significant differences in the MARP values between symptomatic and asymptomatic patients. CONCLUSION: The results of our study showed that the majority of the patients have impaired anorectal motility. There were no significant differences in the results of the functional studies for the seven patients with symptoms of obstruction or constipation when compared with asymptomatic patients after surgery for HD.

Prevalence and long-term follow-up outcomes of testicular adrenal rest tumours in children and adolescent males with congenital adrenal hyperplasia.

OBJECTIVE: There are a few studies regarding the prevalence of testicular adrenal rest tumours (TARTs) in boys and adolescent males with congenital adrenal hyperplasia (CAH), and there is little information regarding the treatment outcomes in patients with TARTs. The aim of this study was to determine the long-term treatment outcomes in boys and adolescent males with CAH. PATIENTS AND METHODS: Sixty boys and adolescent males with CAH, who were between 2 and 18 years of age, were included in the study. Fifty-five patients had 21-hydroxylase deficiency (21-OHD), and five patients had 11-ß hydroxylase deficiency (11ß-OHD). All patients were screened for TARTs by scrotal ultrasonography (US) performed by an experienced radiologist. RESULTS: TART prevalence was 18·3% in 2-18 years' of age; eight patients had 21-OHD, and three had 11ß-OHD. The youngest patient with TART was 4 years old, whereas eight patients with RTs were at puberty. Only two patients had tight metabolic control: eight patients had stage 2, one had stage 4, and two had stage five rest tumours. In four patients with stage 2 TARTs, tumours disappeared after high-dose steroid treatment and did not recur. Shrinkage of tumour was observed in two patients. Testis-sparing surgery was performed in one patient with stage five tumour. Gonadal functions were normal in patients with partially regressed tumours. Two patients became fathers of healthy male off-springs. CONCLUSIONS: Detection and treatment for TARTs in children with CAH at younger ages, earlier stages, may prevent infertility in adulthood. Therefore, we recommend that scrotal US screening should be performed in every 1-2 years starting from early childhood.

Calretinin immunohistochemistry in Hirschsprung's disease: An adjunct to formalin-based diagnosis.

AIM: This study was designed to assess the utility of calretinin immunohistochemistry in the diagnosis of Hirschsprung's disease. MATERIAL AND METHODS: Eleven definitive resection materials from 10 Hirschsprung's disease patients and 3 initial full-thickness rectal biopsies of these patients were retrieved from the pathology archives. Additionally, 15 distal colon and 13 proximal colon full-thickness samples from 23 non-Hirschsprung's disease patients were also evaluated as the control group. All material was reevaluated by light microscopy for the presence or absence of ganglion cells and immunostained with calretinin, including proximal surgical margins and aganglionic zone samples from each resection material. RESULTS: Immunohistochemistry for calretinin provided highly compatible results with hematoxylin-eosin findings in Hirschsprung's disease and non-Hirschsprung's disease patients, except in one Hirschsprung's disease patient with very rare nerve stainings at the distal surgical margin. CONCLUSIONS: Calretinin immunohistochemistry was found to be highly sensitive and specific in detecting aganglionic segments. New research should be conducted in order to clarify calretinin staining patterns of the transitional zone, rare Hirschsprung's disease types, pure hypoganglionosis patients, and the anorectal junction, and for the mapping of fetal and neonatal colonic specimens. The technique seemed very effective for lowering the need for excessive sectioning and practical regarding the erratic nature of the acetylcholinesterase staining.

The implementation of neonatal peritoneal dialysis in a clinical setting.

OBJECTIVE: To investigate etiology, outcome and complications related to neonatal peritoneal dialysis (PD). METHODS: Neonates treated with PD in our neonatal intensive care unit during 2007-2010 were analyzed retrospectively. RESULTS: Among 4036 hospitalized neonates; 20 neonates (0.5%) who underwent 21 cycles of PD [7 preterm, 13 term; 13 female, 7 male] were included. The mean birth weight was 2930.2 ± 720.6 g (1120-4570), mean gestational age was 37.5 ± 3.5 weeks (27-41). The etiologic disorders included inborn errors of metabolism (propionic acidemia, methylmalonic acidemia, citrullinemia, glutaric aciduria type 2, maple syrup urine disease, 10), or acute renal failure secondary to perinatal asphyxia (4), sepsis (2), prematurity (2), hypoplastic left heart syndrome (1), kernicterus (1). The complications included peritonitis (2), early leakage (4), hemorrhage (1), catheter removal (3) and occlusion (2). The mortality rate was 50%. The gestational ages and birth weights of surviving neonates were higher (p < 0.05). Among surviving neonates, chronic renal failure (1), severe (4) and moderate neuromotor impairment (2) developed within 4-43 months. CONCLUSION: PD, although invasive, is an effective therapy in neonates. The complexity and invasiveness of the procedure is probably responsible for high rate of complications and mortality. If appropriate catheter selection and technique in the placement should be done, PD might improve outcome.

A comparison of polymer clips and endoloop applications for securing the appendiceal stump during laparoscopic surgery in children.

INTRODUCTION: In this study, for the first time in children a polymer clip and endoloop (EL) for securing an appendiceal stump have been prospectively correlated and evaluated radiologically. METHODS: Forty-nine patients aged 1 to 15 years were operated upon by the same surgeon for acute or perforated appendicitis between May 2008 and May 2009. The appendiceal stump was ligated by an EL or polymer clip. Patients were radiologically evaluated during the postoperative period. RESULTS: In the EL group, the mean operating time for perforated appendicitis was recorded as 57.40 minutes and in nonperforated appendicitis as 39.37 minutes, respectively. In the clips-applied group, these periods were 48.23 and 34.72 minutes, respectively. Clip application is 3 times cheaper than EL. CONCLUSIONS: Polymer clip is an instrument that is cheaper, safe, easily applicable, and takes less time for securing appendiceal stumps compared with EL.

Sertoliform cystadenoma: a case with overlapping features.

The 1st pediatric case of sertoliform cystadenoma with unique features is described herein. The patient is a 6-year-old boy who presented with gynecomastia and a left testicular cystic mass. Histopathologically the tumor was found to originate from the rete channels, filling and distending them with areas of mural Sertoli cell proliferations reminiscent of large cell Sertoli cell tumor (noncalcifying form) and showing widespread intratubular Sertoli cell proliferation islands in the vicinity. Histopathologic and immunohistochemical features are described in light of the relevant literature.