ObjectivesDigital ulcers (DUs) are associated with a significant burden in systemic sclerosis (SSc) by leading to severe pain, physical disability, and reduced quality of life.This effort aimed to develop recommendations of the Turkish Society for Rheumatology (TRD) on the management of DUs associated with SSc. MethodsIn the first meeting held in December 2020 with the participation of a task force consisting of 23 rheumatologists the scope of the recommendations and research questions were determined.A systematic literature review was conducted by 5 fellows and results were presented to the task force during the second meeting.The Oxford system was used to determine the level of evidence.The preliminary recommendations were discussed, modified, and voted by the task force and then by members of TRD via e-mail invitation allowing personalised access to a web-based questionnaire [SurveyMonkey®]. Results A total of 23 recommendations under 7 main headings were formulated covering non-pharmacological measures for the prevention of DUs and pharmacological treatments including vasodilators, anti-aggregants, antibiotics, wound care, pain control, and interventions including sympathectomy, botulinum toxin, and surgery. Risk factors, poor prognostic factors,prevention of DU and adverse effects of medical treatments were reported as 4 overarching principles. ConclusionsThese evidence-based recommendations for the management of SSc-associated DUs were developed to provide a useful guide to all physicians who are involved in the care of patients with SSc, as well as to point out unmet needs in this field.

Introduction The management of neurogenic bladder mostly relies on urodynamic studies; however, several studies have shown low interobserver reproducibility. The aim of this study was to evaluate if a new objective cystometric parameter was superior to other cystometric measures. Patients and methods A new parameter (pressure adjusted area under curve ratio, PAUC) was formulated from the ratio of area under the cystometry curve to a DLPP-adjusted total area (A(T)). A(T) was figured from a rectangle designed on the cystometrogram curve with a fixed height of 200 cmH(2)O and a width defined by the filling phase (figure). Two different estimated pressure measures derived from this (upper mean static pressure, UMSP and theoretical end filling pressure TEFP) were then calculated. Medical records of myelomeningocele patients with ultrasonography and renal scintigraphy performed at the time of urodynamics (with an interval of >5 years) were reviewed. Hydronephrosis and new scars in scintigraphy were used as the outcome measures. Results The study group consisted of 115 subjects with a median age of 4 (0-23) years at the time of the first urodynamic study. The median follow-up was 6 (5-14) years. PAUC and its derivatives (UMSP and TEFP) had the best discriminative power in predicting high grade hydronephrosis (0.830, 95% CI:0.732-0.927, p < 0.001), worsening in hydronephrosis (0.827, 95% CI:0.723-0.931, p < 0.001), and new scar formation (0.704, 95% CI: 0.576-0.832, p = 0.002). PAUC>0.1 significantly correlated with urinary tract dilatation (p < 0.001) and new scar formation (p = 0.002). In the multivariate analysis, our three parameters and having scars at admission were the only independent risk factors for new scars (p = 0.001 and p = 0.002, respectively) and worsening in hydronephrosis (p < 0.001 and p = 0.001, respectively). Discussion Our results show that our three parameters derived from area under the urodynamic curve are more reliable than other urodynamic measures. Their major theoretical advantage is to incorporate all the pressure during filling phase giving a more accurate picture of what the intravesical pressure milieu is. Using these measures, we demonstrated their superiority in predicting clinical outcomes. Major limitations of this study are the retrospectively collected data and lack of longitudinal follow-up starting from infancy in each patient. Conclusion Our new parameters (PAUC, UMSP and TEFP) which incorporate the impact of entire filling phase pressure changes in the analysis, may be useful tools to identify those patients who are under the risk of kidney damage with neurogenic lower urinary tract dysfunction.

Aim: Standardization of an optimal treatment protocol for vesicoureteral reflux (VUR) correlated with duplex kidney systems remains controversial.In this study, we reviewed our experience in endoscopic subureteric injection to correct reflux in duplex systems with documented indications, in an aim to confirm its position as an alternative means of open surgery. Materials and Methods:The hospital records of complete renal duplex system patients who had experienced endoscopic subureteric injection for VUR between 2009 and 2018 were reviewed.The indications for the amelioration of VUR included breakthrough urinary tract infection (UTI) along with the presence of renal scarring.Results: A total of 24 patients (18 girls, 6 boys) with refluxing renal duplex systems were included in the study.The mean age at first injection was 46.4±28.8months (5-160) and the mean duration of follow-up was 47.25±27.7 months.The success rate with the initial injection was 83.3% (20/24).The mean volume of material injected was 0.68±0.49mL per duplex system. Conclusion:Management of reflux in duplex systems is still controversial with insufficient data.Our results suggest that endoscopic management should be considered as an alternative to open ureteral reimplantation in refluxing duplex renal collecting systems.Major open surgery and accompanying discomfort can be avoided by an endoscopic injection which can be performed as an outpatient procedure in most of the reflux patients with duplex system who have an indication for surgical management.

Introduction Neurogenic bladder with anorectal malformations (ARM) is a well-known yet understudied topic. Diffusion tensor imaging (DTI) is a special usage of MRI that can evaluate peripheric nerves. The aim of this study is to evaluate the lumbosacral plexuses of patients with ARM using DTI. Patients and methods Patients with ARM willing to participate were included. Patient files were reviewed, a questionnaire was made, and DTI was performed. Fractional anisotropy (FA) and apparent diffusion coefficient (ADC) were computed for the right and left lumbosacral plexuses. Results There were 18 patients and 12 controls. Groups were similar in terms of sex (p = 0.232) and age (p = 0.853). There was a significant difference only for ADC of the left plexus. There was visible asymmetry in tractographies and DTI parameters of two patients with severely deformed pelvises (image 1) but also in another patient with a normal sacrum. In addition, despite asymmetry, FA and ADC were similar to controls also in these patients. Conclusion This is the first study presenting quantitative data about the lumbosacral plexus in ARM. We think the most interesting finding of this study was observing the normal values even in the patients with severely malformed sacrum which is yet to be validated with further studies.

Acun Gökdemir was one of the first two pediatric surgery residents to receive formal modern training from the first official pediatric surgery department in Turkey, which was founded by Prof. Ihsan Numanoğlu at Ege University, Izmir in 1961. With his high surgical skills and enthusiasm, Acun Gökdemir was granted an academic position in the same department leading to his future as a full professor. His interest in pediatric urology was recognized and supported by Prof Numanoğlu. Acun Gökdemir started the division of pediatric urology in 1974, limiting his practice to pure pediatric urology from then on, and was the first to do soin Turkey. Like many pioneers in those days, he visited Sir David Innes Williams in London in 1976–1977. He returned to Izmir full of excitement to care for a large and helpless population of children with genitourinary conditions. He initiated the first official academic division of pediatric urology in Turkey in 1993, recognized by the Council of Higher Education of Turkey. He appointed two academic surgeons, Dr Ali Avanoglu in 1992 and Dr Ibrahim Ulman in 1993, to the division and supported them unconditionally (Fig. 1). He set the stage leading to the recognition of pediatric urology as a subspecialty and influenced formal training in pediatric urology in Turkey, which was started by his successors shortly after his retirement. Acun Gökdemir's contributions to pediatric urology as a pioneer is well-recognized by all pediatric urologists currently practicing in Turkey. He dedicated his whole professional life to treating children and teaching pediatric surgery and urology (Fig. 2). He was granted the first honorary member of the Society for Pediatric Urology in Turkey. He passed away in February 2022, after a rapidly progressive COVID19 infection at the age of 88 at his home with his beloved family by his side. He will always be remembered by Turkish pediatric urologists, his trainees, medical students, and children who received his care.Fig. 2Professor Gökdemir dedicated his career to children with urinary conditions.View Large Image Figure ViewerDownload Hi-res image Download (PPT)

Background In this study, we aim to present the experience of a tertiary center regarding penile epidermoid cysts over 15 years. Methodology Patient files of those who underwent surgical excision for penile epidermoid cysts between 2005 and 2019 were reviewed retrospectively. The demographics, clinical characteristics, etiological factors, cyst features, surgical techniques, complications, and follow-up data were analyzed. Results In total, 24 penile epidermoid cysts were excised in 21 boys. The median age at the time of surgery was 52 (15-204) months. The median duration between previous surgery and cyst excision was 40 (1-180) months. In total, 11 cases had a history of circumcision, and 10 had undergone hypospadias surgery. There was no significant difference between these two etiologic groups (p > 0.05). The main symptom was an asymptomatic penile mass. The average cyst size was 9.4 ± 6.7 mm. All cysts were completely excised with incisions made over old scars, except one. No complications were observed during a median follow-up period of 50 (12-120) months, and only one recurrence was noted. Conclusions Acquired penile epidermoid cysts may present as an early or late complication after penile surgery. Complete excision with an incision along the existing scars seems to be an effective solution for preventing new scars.

Purpose: The treatment of high-grade vesicoureteral reflux in infants is controversial. Subureteric injection has been tried recently with its advantage of easy application and less invasiveness. In this study, we aimed to assess the efficacy of endoscopic sub-ureteric injection to correct high-grade reflux in infants with documented indications for anti-reflux surgery. Materials and Methods: Hospital records of high-grade (grade 4-5) VUR patients who had undergone endoscopic sub-ureteric injection in the first year of life with documented breakthrough infections, between 2009-2016 were reviewed. Radiologic success was defined as complete resolution of reflux in VCUG obtained at least three months after the injection and clinical success was defined as the downgrading of reflux degree below three and absence of urinary infection. Results: A total of 23 patients with 34 high-grade refluxing units were included in the study. The mean age at first injection was 6.3±1.8 months (1-11 months). Radiologic success rate with initial injection was 61.7% and increased to 85.2% after repeated injections. The overall clinical success rate at first injection was 70.6% and 97.1% after repeated injections. Mean injected material volume was 0.34±0.27 (0.1-1) ml per ureter. Conclusion: Endoscopic treatment is a successful alternative in infants with high-grade vesicoureteral reflux suffering breakthrough infections.

Kidney parenchyma and collecting system arise from two different embryologic units as a result of a close interaction between them. Therefore, their congenital abnormalities are classified together under the same heading named CAKUT (congenital abnormalities of the kidney and urinary tract). The pathogenesis of CAKUT is thought to be multifactorial. Ureteropelvic junction obstruction (UPJO) is the most common and most investigated form of CAKUT. Despite years of experimental and clinical research, and the information gained on the embryogenesis of the kidney; its etiopathogenesis is still unclear. It involves both genetic and environmental factors. Failure in development of the renal pelvis, failure in the recanalization of ureteropelvic junction, abnormal pyeloureteral innervation, and impaired smooth muscle differentiation are the main proposed mechanisms for the occurrence of UPJO. There are also single gene mutations like AGTR2, BMP4, Id2 proposed in the etiopathogenesis of UPJO.

Objective: The aim of this study was to assess the quality of life (QoL) and psychological well-being in child and adolescent with disorders of sex development (DSD). Methods: Sixty-two cases, aged 2-18 years, who were followed by a multidisciplinary DSD team were included. All participants and their parents were requested the complete the Pediatric Quality Of Life Inventory (PedsQL) and the Strengths and Difficulties Questionnaire. The psychiatric diagnoses of the patients were evaluated according to Schedule for Affective Disorders and Schizophrenia for School-Age Children/Present and Lifetime Turkish Version. Results: There was no significant difference between the 46,XX DSD and 46,XY DSD groups for both child and parent in Total PedsQL score. In the subscale scores, the PedsQL Physical Functionality Score reported by children was significantly lower for the 46,XX DSD group than for the 46,XY DSD group (p=0.01). There was a psychiatric diagnosis in 25.8% of cases. The PedsQL School Functionality Score reported by children in the group with psychiatric diagnosis was significantly lower than the group without psychiatric diagnosis (p=0.018). In the group with psychiatric diagnosis, the PedsQL Total Score and the subscale scores (Emotional Functionality Score, Social Functionality Score, School Functionality) reported by parents were significantly lower than in parents of the group without psychiatric diagnosis. Conclusion: This study emphasized that psychiatric disorders in DSD patients negatively affect the QoL. Psychiatric support and counseling from a multidisciplinary team are very important for families affected by DSD.

Kidney parenchyma and collecting system arise from two different embryologic units as a result of a close interaction between them. Therefore, their congenital abnormalities are classified together under the same heading named CAKUT (congenital abnormalities of the kidney and urinary tract). The pathogenesis of CAKUT is thought to be multifactorial. Ureteropelvic junction obstruction (UPJO) is the most common and most investigated form of CAKUT. Despite years of experimental and clinical research, and the information gained on the embryogenesis of the kidney; its etiopathogenesis is still unclear. It involves both genetic and environmental factors. Failure in development of the renal pelvis, failure in the recanalization of ureteropelvic junction, abnormal pyeloureteral innervation, and impaired smooth muscle differentiation are the main proposed mechanisms for the occurrence of UPJO. There are also single gene mutations like AGTR2, BMP4, Id2 proposed in the etiopathogenesis of UPJO.

Background: Psychiatric consultation is important in the follow-up of disorders of sex development (DSD) patients. In this study, we aimed to present the 12-year psychiatric follow-up data of the patients who were referred by Ege University Medical Faculty DSD Multidisciplinary Team and followed up in Child and Adolescent Psychiatry. Methods: Psychiatric data of 118 patients, who were followed by the DSD multidisciplinary team between 2007 and 2019, were reviewed retrospectively. The psychiatric diagnoses of the patients were evaluated according to semi-structured interview form Schedule for Affective Disorders and Schizophrenia for School-Age Children/ Present and Lifetime Turkish Version. Results: The mean age of the 118 cases was 13.21 years (+/- 7.18). Endocrine diagnoses of the cases were 46 XX DSD in 35 (29.6%), 46 XY DSD in 81 (68.7%), and chromosome disorders in 2 (1.7%). There was at least psychiatric diagnosis in 36 (30.5%) cases. The most common psychiatric diagnosis was attention deficit and hyperactivity disorder (ADHD) (n=18, 15.3%). ADHD was most common in congenital adrenal hyperplasia (n =4, 22.4%) and androgen synthesis defects (ASD) (n= 4, 22.4%); depression was most common in complete gonadal dysgenesis and ASD n=3, 23.1%); and mental retardation was most common in ASD (n = 3, 37.5%). Conclusions: In order to provide a healthy perspective for cases with DSD, it is important to make a psychiatric evaluation and to share observations and clinical findings in regular team meetings.

Non-Hodgkin's lymphomas (NHL) are common malignant tumors in children and adolescents. Among them diffuse large B-cell lymphomas (DLBCL) are relatively rare as compared to non-cleaved small cell lymphoma (mostly Burkitt's-BL) and lymphoblastic lymphoma (LL). While BL has abdominal or cervical site predilection, LL (mostly T-cell) tends to have mediastinal involvement. However, diffuse large B-cell lymphomas may involve abdomen, peripheral lymph nodes, skin, bone, other rare sites. Ureteral NHLs are extremely rare in children; however, many cases have been reported in adults. In adults the histopathology is usually follicular lymphoma. Only one case of unilateral ureter DLBCL has been reported in an adolescent in the past. Here we report a case of bilateral ureteral DLBCL to highlight the unusual presentation of NHLs and that it should be considered in the differential diagnosis of acute renal insufficiency.

Purpose:Hinman syndrome is a rare disease with urodynamic findings and a clinical course resembling neuropathic bladder, without a neuropathic etiology. Diffusion tensor imaging is a special technique of magnetic resonance imaging that has recently been used to evaluate the peripheral nerves but has been demonstrated to be applicable for evaluation of the lumbosacral plexus. We examined the lumbosacral plexus using diffusion tensor imaging, which has not previously been reported in patients with Hinman syndrome.Materials and Methods:The study included 12 patients who fulfilled criteria for Hinman syndrome, with severe bladder dysfunction on urodynamics, renal scarring on scintigraphy and no pathological findings on magnetic resonance imaging. The 12 subjects serving as controls required pelvic or spinal magnetic resonance imaging for reasons other than spinal abnormalities. Evaluation was performed with a 3.0 Tesla magnetic resonance imaging system and 16-channel body coil. Tractography was done to examine the lumbosacral plexus. Fractional anisotropy and mean diffusivity were computed and compared between groups for the right and left plexuses.Results:Mean fractional anisotropy was 0.24 and 0.35 for the right plexus in patients and controls, respectively, and 0.24 and 0.36 for the left plexus. Mean diffusivity was 1.39 for the right and left plexuses in patients, and 1.28 for the right and left plexuses in controls (p <0.001 for all).Conclusions:Our study focusing on the lumbosacral plexus as a possible origin of neuropathy revealed abnormal findings in patients with Hinman syndrome resembling nerve injury series. This is the first known study to provide data showing that Hinman syndrome may have a neuropathic etiology.

Tekin A, Yağmur İ, Ergün O, Ayık MF, Atay Y, Ulman İ, Avanoğlu A. Excision of the atrial Wilms` tumor thrombus without sternotomy, atriotomy and cardiovascular By-pass. Turk J Pediatr 2019; 61: 436-439. The treatment of atrial-extention Wilms` tumor thrombus is surgical excision after chemotherapy. Atriotomy with cardiovascular by-pass is the one of the most common method for this procedure. Herein, we aimed to present a case of Wilms` tumor with a tumor thrombus extending into the right atrium totally excised with retrohepatic cavatomy. A 3.5 year-old girl was admitted with the symptom of dysuria. The examinations revealed a mass consistent with Wilms` tumor in the middle and lower poles of the left kidney. Doppler ultrasound and Echocardiographic examinations showed a tumor thrombus extending into the right atrium and some pulmonary nodules which were interpreted to be metastasis. Wilms` tumor was histopathologically diagnosed by an open biopsy. After three courses of chemotherapy imaging studies revealed that the atrial axtention of the tumor thrombus persisted. The tumor thrombus was found to be fibrotic on the magnetic resonance imaging scan of the patient. Therefore, nephroureterectomy along with the excision of the tumor thrombus from the inferior vena cava was done with intraoperative continuous trans-esophageal echocardiography (TEE). The suprarenal and retrohepatic vena cava were exposed by dissecting and ligating all short hepatic veins and completely mobilizing the right lobe of the liver. The thrombus was dissected out via Vertical cavatomy at the retrohepatic level. TEE confirmed complete removal of the thrombus from the atrium; Vena cava was then repaired. There was no need for a blood transfusion, or cardiovascular by-pass (CPB) during the operation. Total exposure of the retrohepatic and subdiaphragmatic vena cava using transplantation techniques is an effective method for the excision of a tumor thrombus without sternotomy, atriotomy and CPB, avoiding possible intra- and postoperative complications in selected cases of Wilms` tumor with intraatrial thrombus extension. The case emphasises the importance of multidisciplinary communication and collaboration.

Objective: Disorders of sex development (DSD) is a nomenclature intended to defeat the discomfort of families and patients and has found worldwide usage.The aim of this study was to address the perception and usage of terminology among the parents of DSD patients in a tertiary center in western Turkey.Methods: The records of the DSD council (multidisciplinary team where each patient with DSD is discussed) between years 2008-2015 were reviewed retrospectively.Data including details of the management process, patient characteristics and follow-up details were noted.Then inquiries reflecting parental perception about terminology were implemented during clinical visits.Results: In total, 121 patients were evaluated in monthly meetings of the DSD council and 79 inquiries were completed.Fifty-one percent of the families admitted knowing the terms DSD, ambiguous genitalia, "dubious genitals" and intersex.However, only 2% preferred using DSD, 6% intersex and 14% ambiguous genitalia.Fifty-two percent of the parents used a disease name in Latin (mostly hypospadias) addressing the disorder.The offspring of 69% of the parents who were familiar with the name "dubious genitals" were diagnosed in the neonatal period.The preferred terminology used by parents was strongly associated with the terminology used most commonly in the medical speciality their child most often attended.Conclusion: Each country has its own social norms.We suggest therefore that local committees including medical professionals, patients and families should be employed to develop proper terminology.

INTRODUCTION:Various bulking agents were utilized for endoscopic correction of VUR. A study reviewing multi-institutional data showed that the amount of injection material has increased over time with the purpose of improving success rates, which also resulted in costs. We noticed an opposite trend in our center since we started using a new bulking agent. The aim of this study was to evaluate evolution of our practice with different bulking agents.PATIENTS AND METHODS:Records of VUR patients who underwent subureteric injection with polyacrylate polyalcohol copolymer (PPC) and dextronomere hyaluronic acide (DxHA) between 2005 and 2014 were reviewed. Variation of different parameters throughout the study period was evaluated along with the success rate. Success was defined as complete resolution of reflux.RESULTS:A total of 260 patients with 384 refluxing units were included. The success rate was higher in PPC group compared to DxHA group. There was no statistically significant difference between years regarding distribution of VUR grade, body weight, patient height, and age in PPC group. Despite significant reduction in injection volume, success rate did not decrease through the years with PPC.CONCLUSION:Different bulking agents may require different injection volumes to achieve the same success rate in endoscopic treatment of vesicoureteral reflux. Habits gained with previous experience using other materials should be revised while using a new agent.