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.

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.

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.

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.

Combination of tubularized island flap and ventral skin flap techniques in single-stage correction of severe proximal hypospadias.

OBJECTIVES: To evaluate the success of the combined tubularized island flap (Duckett technique) and Thiersch-Duplay techniques in one-stage correction of proximal hypospadias. METHODS: Thirty-four patients underwent surgical treatment for penoscrotal hypospadias in the last 9 years. Combined tubularized island flap (Duckett technique) and tubularized ventral skin (Thiersch-Duplay) procedures were performed in each case. The proximal urethra was repaired by the Thiersch-Duplay technique through the base of the phallus, and distal urethral repair was conducted via the tubularized island flap procedure. RESULTS: The follow-up lasted from 5 months to 6 years (mean 4.1 years). The overall complication rate was 26%, but no recurrent chordee was detected. Recurrent fistulae were noted in 7 patients (20.5%). Anastomatic stricture was noted in 3 patients (8.8%). CONCLUSION: A combination of Duckett's preputial tube and the Thiersch-Duplay procedure was employed to treat 34 cases of severe hypospadias, with a success rate of 74%. In our procedure, the island flap tube is relatively short. Oblique anastomoses of proximal and distal tubes were made, and the pedicule of the flap supported the neourethra following successful interventions. Because of the low complication and high success rates, this combination of two procedures is recommended for repair of severe proximal hypospadias.

Variables related to behavioral and emotional problems and gender typed behaviors in female patients with congenital adrenal hyperplasia.

We investigated the effects of type of congenital adrenal hyperplasia (CAH), treatment, endocrinological, surgical, and socio-demographic factors as well as patients' body perception on the gender-typed play and behavioral and emotional problems in female children with CAH. The sample included 28 females with CAH (mean age: 12.6 years). We compared patients with CAH to 28 age-matched patients with type 1 diabetes mellitus and 28 healthy controls. Patients with CAH had significantly higher externalization and total problems scores and were less interested in typically female behaviors. The behavioral and emotional problems in patients with CAH were associated with patient satisfaction with the appearance of their genitalia, the surgeons' assessment of the success of the surgical procedures, and mean testosterone level. Our results showed the severity of the behavioral and emotional problems was associated with severity of androgenization, patients' perception of their genitalia and the surgical outcome.

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.

Breast fibroadenoma in female adolescents.

Palpable breast masses in pediatric patients are uncommon. Fibroadenoma which must be distinguished from malignant tumors is the most frequent breast tumor in adolescent girl. Fine needle aspiration FNA biopsy is highly specific in breast masses. The final cosmetic result is important when planning the surgical excision. Therefore it is mandatory to know preoperative cytology of the mass. Five girls who had undergone operation with breast masses between 1999-2004 were evaluated retrospectively. The mean age at presentation was 13 years. Each patient underwent FNA biopsy before excision. Aspiration cytology of the lump showed fibroadenoma confirmed by subsequent histopathology. The breast masses can be handled operatively through a circumareolar incision without any complication. Protection of the developing breast bud, nipple and areola is as important as appropriate excision of the lesion. Cosmetic results have been satisfactory and there has been no evidence of recurrence.

Eventration of the diaphragm.

OBJECTIVE: Eventration of the diaphragm (ED) is defined as the abnormal elevation of the diaphragm. Although asymptomatic ED may be amenable to conservative treatment, symptomatic ED in children, either congenital or acquired, may require surgical treatment. This study evaluated the results of diaphragmatic plication in children with unilateral ED. METHODS: Fifteen patients who had undergone diaphragmatic plication for ED between 1997 and 2003 were evaluated retrospectively. The diagnosis of ED was established by routine chest radiographs and fluoroscopy. Patients who failed to respond to nonoperative treatment were referred for surgery. Nine patients underwent diaphragmatic fluoroscopy 1-5 years following plication to assess function. RESULTS: Indications for diaphragmatic plication were respiratory symptoms such as tachypnoea, dyspnoea, recurrent pneumonia and failure to thrive. In 14 patients, the position of the diaphragm was normal after plication, but the diaphragm was elevated without symptoms in one patient during postoperative follow-up. The motion of the diaphragm was investigated in nine patients. Fluoroscopic studies showed that the operated hemidiaphragm was immobile and there was no paradoxical motion. No return of symptoms was found during follow-up. CONCLUSION: ED is the abnormal elevation of the diaphragm as a result of paralysis or aplasia of the muscular fibres. The abnormally elevated diaphragm may compress the ipsilateral lung, and with respiratory effort the mediastinum may shift towards the normal side. Therefore, diaphragmatic plication is performed to restore normal pulmonary parenchymal volume by replacing the diaphragm in its normal location. After plication, there was immediate remission of symptoms in most patients and decreasing symptoms were observed for a year in others. During follow-up, the location of the diaphragm was normal and no paradoxical movement was observed. Relapse of symptoms was not noted in patients with immobile diaphragms.

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.

Conservative approach to the mediastinitis in childhood secondary to esophageal perforation.

The aim of this study was to evaluate the safety and efficacy of nonoperative treatment of esophageal perforation (EP) in children. Between 1999 and 2004, 13 episodes in 12 patients were evaluated. The treatment program consisted of broad-spectrum antibiotics, nasopharyngeal aspiration, parenteral and/or enteral nutrition by gastrostomy, and pleural effusion or mediastinal abscess drainage when required. Mean age of the patients was 3.75 +/- 1.13 (range 3-7 years). Two patients (16.7%) were girls and 10 patients (83.3%) were boys. Chest pain was found 76.9% of all EP episodes (10 of 13 perforations), followed by dyspnea in 69.2% (9 of 13), vomiting in 46.1% (6 of 13), fever in 46.1% (6 of 13), and epigastric pain in 7.6% (1 of 13). No deaths occurred. In children, in contrast with the adults, EP can be treated safely by nonoperative methods.

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.

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.

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.

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.

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.