Outcomes and special techniques for treatment of penile amputation injury.

INTRODUCTION: Management of severe penile trauma presents great challenges for reconstructive urologists since these injuries vary from abrasions to total emasculation. A review of our case experience with penile amputation is presented, emphasizing techniques used to salvage or reconstruct the most difficult of penile injury cases. MATERIALS AND METHODS: A total of 13 patients with penile amputation injury referred to us between 2007 and 2016 were analyzed. Mean age at surgery was 16 years (ranged from 4 to 29 years). Etiology of penile amputation (partial or total) combined with management and outcomes were evaluated. Management included different surgical procedures with the aim to achieve good functional and esthetical outcomes. Postoperative questionnaire was used for assessment of patient's overall satisfaction. RESULTS: Follow-up ranged from 13 to 182 months (mean 53). Causes of penile injury were iatrogenic trauma (8), self-amputation (2), electrocution (1), intentional sexual assault (1) and mother's hair strangulation (1). Outcome criteria including aesthetic appearance, urinary function and ability to engage in satisfactory coitus, were noted in 11 cases (85%). Two cases with ensuing complications relating to the total phalloplasty required additional treatment due to urethral fistula. CONCLUSIONS: Severe penile injuries should be treated on a case by case basis utilizing the most propitious techniques. We respectfully propose that the needs of such patients are best served by referral centers with extensive experience.

Musculocutaneous latissimus dorsi flap for phalloplasty in female to male gender affirmation surgery.

PURPOSE: Despite a variety of free flaps that have been described for creation of the neophallus in gender affirmation surgery, none present an ideal solution. We evaluated our patients and outcomes after gender affirmation phalloplasty using musculocutaneous latissimus dorsi free flap. METHODS: Between January 2007 and May 2017, 129 female transsexuals, aged 20-53 years (mean 24 years) underwent total phalloplasty using latissimus dorsi free flap. Urethral lengthening was performed by combining a vaginal flap, labia minora flaps and a clitoral skin flap. Suitable sized testicular implants are inserted into the new scrotum. Penile prosthesis implantation, additional urethral lengthening and glans reshaping were performed in the following stages. RESULTS: The mean follow-up period was 43 months (ranged from 13 to 137 months). There were one partial and two total flap necrosis. The average size of the neophallus was 14.6 cm in length and 12.4 cm in girth. Total length of the reconstructed urethra during the first stage ranged from 13.4 to 21.7 cm (mean 15.8 cm), reaching the proximal third or the midshaft of the neophallus in 91% of cases. Satisfactory voiding in standing position was confirmed in all patients. Six urethral fistulas and two strictures were observed and repaired by minor revision. Malleable and inflatable prostheses were implanted in 39 and 22 patients, respectively. CONCLUSION: Musculocutaneous latissimus dorsi flap is a good choice for phalloplasty in gender affirmation surgery. It provides an adequate amount of tissue with sufficient blood supply for safe urethral reconstruction and penile prosthesis implantation.

Transcriptome-driven integrative exploration of functional state of ureter tissue affected by CAKUT.

AIMS: (1) to identify the most dysregulated genes in ureter tissue affected by congenital anomalies of the kidney and urinary tract (CAKUT) and to extract the biological meaning of these markers; (2) to describe the key molecular networks in CAKUT and to provide expression validation of the genes selected from these networks. MAIN METHODS: Transcriptome data was obtained from ureter samples of CAKUT patients and controls by Illumina iScan microarray. Identification of differentially expressed genes was coupled with subsequent bioinformatics analyses. Expression of candidate genes was validated by qRT-PCR. KEY FINDINGS: Analysis of the transcriptome led to the identification of 78 commonly dysregulated genes in CAKUT tissue compared to controls. Integrative bioinformatic analyses of differentially expressed genes identified 7 major networks. The targets for qRT-PCR validation were selected as members of the major molecular networks in CAKUT, which had both, the significant high fold change and biological relevance for CAKUT. By qRT-PCR the substantial increase of LCN2, PROM1, SOSTDC1, and decrease of INA, RASD1 and TAC3 mRNA levels was confirmed. SIGNIFICANCE: Since CAKUT is a leading cause of end-stage renal disease in children, the search for molecular targets for postnatal therapy is of particular interest. Data described in this study represents the gene expression profile and significant molecular networks specific to human ureter affected by CAKUT. The discovery of impaired molecular factors and processes is the step towards the uncovering of the key mechanisms that reflect CAKUT postnatally and could lead to the affected tissue deterioration and end organ damage.

Total Phalloplasty With Latissimus Dorsi Musculocutaneous Flap in Female-to-male Transgender Surgery.

OBJECTIVE: To present our technique of musculocutaneous latissimus dorsi (MLD) free-flap total phalloplasty. This technically demanding female-to-male gender reassignment surgery consists of creating a neophallus from extragenital tissue. METHODS: The presented technique included: removal of internal and/or external female genitalia, creation of neophallus using latissimus dorsi free flap, clitoral incorporation into the neophallus, urethral lengthening, and insertion of testicular implants into the newly created scrotum. The MLD flap with proper dimension is harvested from nondominant side and tubularized. Microvascular anastomosis is done between thoracodorsal vessels and femoral artery and saphenous vein. Neophallus is positioned in adequate place. Urethroplasty is performed by combining different genital flaps that are harvested from anterior vaginal wall, urethral plate, and both labia minora and clitoral skin. Scrotoplasty is done by joining both labia majora with implantation of testicular implants. RESULTS: Operative time was 427 minutes with minimal blood loss. Both donor site and graft healed well, and the patient reports voiding well while standing. Penile prosthesis implantation as well as neophallic urethroplasty are planned for the second stage. CONCLUSION: Total MLD flap phalloplasty with urethral lengthening is a challenging and complex surgical procedure. This technique presents good variant for female transgenders with acceptable cosmetic outcome and enables good volume of neophallus, sexual arousal, and voiding while standing.

Technetium-99m-dimercaptosuccinic acid renal scintigraphy can guide clinical management in congenital hydronephrosis.

OBJECTIVE: The purpose of this study was to evaluate damage of the kidney with technetium-99m-dimercaptosuccinic acid (99mTc-DMSA) scintigraphy in children with congenital hydronephrosis (CH) and the influence of other postnatal associated diagnoses on abnormal 99mTc-DMSA findings. SUBJECTS AND METHODS: 99mTc-DMSA scintigraphy in 54 children (17 girls and 37 boys), aged from 2 months to 5 years (median 11 months) with 66 congenital hydronephrotic renal units (RU) (42 unilateral hydronephrosis-29 boys and 13 girls; 12 bilateral hydronephrosis-8 boys and 4 girls) was performed. Male/female ratio was 2,2:1, unilateral/bilateral hydronephrosis ratio was 4:1. Hydronephrosis classified into three groups according to ultrasound measurement of the antero-posterior pelvic diameter APD): mild (APD 5-9.9mm) was present in 13/66RU, moderate (APD 10-14.9mm) in 25/66RU, and severe (APD≥15mm) in 28/66RU. Simple hydronephrosis was present in 15RU, and the postnatal associated clinical diagnosis were vesicoureteric reflux (VUR) in 21, pelviureteric junction (PUJ) obstruction in 7, pyelon et ureter duplex in 11, megaureter in 11 and posterior urethra valves in 1RU, respectively. Static renal scintigraphy was performed 2 to 3 hours after intravenous (iv) injection of 99mTc-DMSA using a dose of 50µCi/kg (1.85MBq/kg; minimal dose: 300µCi). Four views (posterior, left and right posterior oblique and anterior) were obtained with a head gamma camera "Orbiter" filtered with high resolution parallel whole collimator. All images were stored in an Pegasys computer with a matrix size of 256×256. The relative kidney uptake (RKU) between the left and right kidney was calculated as an average number counts from anterior and posterior view. Renal pathology was defined as inhomogenous or focal/multifocal uptake defects of radiopharmaceutical in hydronephrotic kidney or as split renal uptake of <40%, and poor kidney function was defined as split renal uptake <10%. Descriptive and analytical statistics (SPSS version 20.0) was performed. Analytical statistics implied the non-parametric Mann-Whitney test for determination of statistically significant difference between the normal and pathological findings on 99mTc-DMS scan. The default level of significance was P<.05. RESULTS: Our 99mTc-DMSA scintigraphy findings in children with ANH were: decreased or enlarged kidney with inhomogeneous kidney uptake radiopharmaceutical in 22, irregular shape kidney with inhomogeneous accumulation of radiopharmaceutical in 3, connected (fused) kidney in 1 patient, and poorly or nonvisual kidney in 14RU respectively (total 40/66RU with pathological 99mTc-DMSA finding, 60,6%). Relative accumulation in hydronephrotic kidney was less or equal to 40% in 17RU, less than 10% in 14RU and inhomogeneous radiopharmaceutical uptake with relative accumulation over 40% was detected in 9RU. Regular kidney morphology with homogeneous accumulation of radiopharmaceutical (normal DMSA scintigraphy finding) were found in 26/66RU (39,4%). Statistically significant correlation between the degree of the hydronephrosis (APD) and 99mTc-DMSA scan findings (P<0.001) and between the degree of the VUR and DMSA scan finding (P=0.002) was established. In our study, other associated diagnosis were not statistically correlated with pathological findings on 99mTc-DMSA scan due to low number of patients. CONCLUSION: On the basis of these results (60% pathological findings) we recommend DMSA scintigraphy in the evaluation renal pathology in children with congenital hydronephrosis. Greater number of patients is needed for the estimation of the associated diagnosis (other than VUR) influence on the renal parenchymal damage in children with CH.

Transcriptome-wide based identification of miRs in congenital anomalies of the kidney and urinary tract (CAKUT) in children: the significant upregulation of tissue miR-144 expression.

BACKGROUND: The genetic cause of most congenital anomalies of the kidney and urinary tract (CAKUT) cases remains unknown, therefore the novel approaches in searching for the common disease denominators are required. miRs regulate gene expression in humans and therefore have potentially therapeutic and biomarker properties. No studies thus far have attempted to explore the miRs in human CAKUT. We applied a new strategy to identify most specific miRs associated with CAKUT, in pediatric patients. METHODS: Data from the whole genome expression, gathered from ureter tissue samples of 19 patients and 7 controls, were used for the bioinformatic prediction of miRs activity in CAKUT. We integrated microarray gene expression data and miR target predictions from multiple prediction algorithms using Co-inertia analysis (CIA) in conjunction with correspondence analysis and between group analysis, to produce a ranked list of miRs associated with CAKUT. The CIA included five different sequence based miR target prediction algorithms and the Co-expression Meta-analysis of miR Targets. For the experimental validation of expression of miRs identified by the CIA we used tissue from 36 CAKUT patients and 9 controls. The results of gene ontology (GO) analysis on co-expressed targets of miRs associated with CAKUT were used for the selection of putative biological processes relevant to CAKUT. RESULTS: We identified 7 miRs with a potential role in CAKUT. The top ranked miRs from miRCos communities 4, 1 and 7 were chosen for experimental validation of expression in CAKUT tissue. The 5.7 fold increase of hsa-miR-144 expression in human tissue from CAKUT patients compared to controls (p = 0.005) was observed. From the GO we selected 7 biological processes that could contribute to CAKUT, which genes are potentially influenced by hsa-miR-144. The hsa-miR-200a, hsa-miR-183 and hsa-miR-375 weren't differentially expressed in CAKUT. CONCLUSIONS: This study shows that integrative approach applied here was useful in identification of the miRs associated with CAKUT. The hsa-miR-144, first time identified in CAKUT, could be connected with biological processes crucial for normal development of kidney and urinary tract. Further functional analysis must follow to reveal the impact of hsa-miR-144 on CAKUT occurrence.

Effectiveness of various surgical methods in treatment of Hirschsprung's disease in children.

BACKGROUND/AIM: Hirschsprung's disease is the most common identifiable developmental disorder of the enteric nervous system, characterized by a failure of its formation in a variable segment of distal bowel. Currently available surgical therapies for Hirschsprung's disease, although lifesaving, are associated with numerous complications. The aim of our study was to evaluate the effectiveness of different surgical methods and the incidence of serious complications after radical surgery of rectosigmoid Hirschsprung's disease. METHODS: A retrospective analysis, from June 1997 until May 2012 was carried out on 84 patients operated for Hirschsprung's disease of rectosigmoid colon. Transanal endorectal pull-through was performed in 30 (35.7%) patients (group I), while 54 (64.3%) patients were operated by other (Soave, Duhamel or Swenson) procedures (group II). The age at operation, the incidence and severity of postoperative complications, the need for previous colostomy and the number of reoperations are countered in order to evaluate the efficacy of surgical procedures. RESULTS: In the group I, the mean age at operation was 9.41 +/- 6.37 months and in the group II the mean age at operation was 16.8 +/- 13.9 months which was significantly higher (p < 0.01). In the group I there were only 3 (10%) patients with complications, one (3/6) of them was prone to only one redo procedure (1.00 +/- 0.00) and there was no need for previous colostomy in all patients (100%). In the group II there were 16 (30%) patients with significantly frequent complications (p < 0.05), about 2 reoperations on the average (1.94 +/- 1.84) in 4 of them (25%) and 22 (41%) redo procedures, which was, in total, significantly higher than in the group I (p < 0.01). Only Soave's procedure was performed without previous colostomy in 20 (37/6) patients. CONCLUSION: Transanal endorectal pull-through in surgical treatment of patients with Hirschsprung's disease is more effective than other procedures concerning earlier onset, low incidence and less severe complications, which would require further operations, and no scars.

Associated extrarenal vascular diseases may complicate the treatment and outcome of renovascular hypertension.

AIM: This studied reviewed renovascular hypertension (RVH) due to renal artery stenosis (RAS) in two Serbian paediatric centres from 2001 to 2013. METHODS: The patients' demographic data, underlying syndromes, blood pressure (BP), antihypertensive treatments and outcomes were reviewed. RESULTS: The incidence of RVH was 1.9 per million children per year during the study period, and there were 25 patients with RAS, aged 10.4 ± 5.2 years. At presentation, their mean blood pressure (BP) standard deviation scores were 6.9 ± 3.4 systolic and 5.2 ± 2.6 diastolic. BP loads on 24-hour ambulatory BP were 88 ± 14% systolic and 80 ± 29% diastolic. We found that 72% had fibromuscular dysplasia and 28% had underlying syndromes. RAS was unilateral in 64% and bilateral in 28%, and 8% had RAS of a single kidney. Antihypertensive treatment included antihypertensive drugs (100%), percutaneous transluminal angioplasty (92%), renal auto-transplantation (16%), surgical revascularisation (12%) and nephrectomy (12%). After 4.4 ± 3.6 years of follow-up, high BP was cured in 40% of the patients and 39.4% of the kidneys and improved in 48% (75.7%), with BP decreases of 20.3 ± 3.7% systolic and 16.3 ± 6.2% diastolic. CONCLUSION: Fibromuscular dysplasia was the most common cause of RVH in this study, and hypertension was cured or improved in 88% of the patients.

Management of Anesthesia in a Child with a Large Neck Rhabdoid Tumor.

OBJECTIVE: The aim of this paper was to report the management of anesthesia of a child with a large neck rhabdoid tumor. CLINICAL PRESENTATION AND INTERVENTION: A 9-month- old female patient underwent urgent neck tumor excision due to intratumoral bleeding from a large tumor that compressed and dislocated the trachea; therefore, intubation was expected to be difficult. Sevoflurane inhalation induction was utilized to maintain spontaneous respiration. Oral laryngoscopy revealed Cormack-Lehane grade 3 laryngeal view. The trachea was intubated using a reinforced tube on the third attempt. Fiberoptic bronchoscope-assisted intubation was planned as an alternative in case of conventional intubation failure. Anticipation of massive blood loss necessitated central venous catheterization. CONCLUSION: Establishing a safe airway, intubation during spontaneous breathing and invasive hemodynamic monitoring are crucial factors in the anesthetic management of pediatric patients with a large neck tumor.

Objective long-term evaluation after bladder autoaugmentation with rectus muscle backing.

PURPOSE: Bladder autoaugmentation with rectus muscle backing is an efficient surgical technique for bladder augmentation. We evaluated long-term outcomes to determine the value of this procedure. MATERIALS AND METHODS: Between August 1999 and June 2004 autoaugmentation was performed in 16 girls and 7 boys 4 to 13 years old (median age 8). The indication was neurogenic bladder with small capacity and poor compliance due to myelomeningocele in 18 patients, tethered cord in 3 and sacral agenesis in 2. Detrusorectomy usually involved the whole upper half of the bladder. The prolapsed bladder urothelium was hitched to the 2 rectus muscles to prevent retraction and provide easier bladder emptying with voluntary muscle contractions. RESULTS: At the median early followup of 27 months (range 9 to 49) bladder volume had increased significantly in all 23 patients (median 338 ml, range 190 to 462). At the current median long-term followup of 134 months (range 94 to 159) bladder volume continued to be significant compared to median bladder capacity preoperatively (median 419 ml, range 296 to 552). Voluntary voiding was achieved in 14 patients without post-void residual urine. Nine patients used clean intermittent catheterization, of whom only 4 could not empty the bladder voluntarily and relied only on clean intermittent catheterization. CONCLUSIONS: Detrusorectomy with a rectus muscle hitch and backing is a minimally invasive, completely extraperitoneal, simple and safe procedure. However, the technique is indicated only in select cases without anterior abdominal wall anomalies.

Diuretic 99mTc DTPA renography in assessment of renal function and drainage in infants with antenatally detected hydronephrosis.

BACKGROUND/AIM: The controversy over the postnatal management of infants with antenataly detected hydronephrosis (ANH) still exists. We presented the results of diuretic 99mTc diethylenetriamine pentaacetic acid (DTPA) renography in 30 infants with the antenatal diagnosis of unilateral renal pelvic dilatation. The aim of this study was to assess the renal function determined by the pattern of drainage and split renal function (SRF) on diuretic renography and to correlate these findings with anteroposterior pelvic diameter (APD) estimated by ultrasonography. METHODS: A total of 30 infants with 60 renal units (RU) (25 boys and 5 girls, median age 6.0 months, range 2-24) presented with unilateral hydronephrosis on ultrasound in the newborn period, underwent DTPA diuretic renal scintigraphy (F+15 protocol). The median APD evaluated on perinatal ultrasound was 15 mm (range 5-30). The postnatal associated clinical diagnosis were pelviureteric junction obstruction (PUJ), simple hydronephrosis, megaureter, vesicoureteral reflux (VUR) and posterior urethral valves in 11, 10, 6, 2 and 1 infant, respectively. Images and Tmax/2 after diuretic stimulation on the background subtracted renographic curves were used as the criteria for classifying the drainage as good, partial, and poor or no drainage. The SRF was calculated with the integral method. RESULTS: Good drainage was shown in 36/60, partial drainage in 13/60 and poor or no drainage in 11/60 RU. The SRF > 40% was observed in 55/60 RU, with no RU showing SRF lower than 23.5%. In infants with severe ANH the obstruction was not excluded in 94.1%. CONCLUSION: Diuretic renography in antenatally detected hydronephrosis should be a useful tool in postnatal follow up, especially in differentiating nonobstructive hydronephrosis from obstructive. It is also importanat to assess and monitor the SRF. Our results suggest that even in the presence of partial or no drainage, SRF may not be significantly impaired.

Post-transplant lymphoproliferative disorder--case reports of three children with kidney transplant.

INTRODUCTION: Post-transplant lymphoproliferative disorder (PTLD) is a heterogeneous group of diseases, characterized by abnormal lymphoid proliferation following transplantation. It is a disease of the immunosuppressed state, and its occurrence is mostly associated with the use of T-cell depleting agents, and also intensification of immunosuppressive regimens. In the majority of cases, PTLD is a consequence of Epstein-Barr virus (EBV) infection and is a B-cell hyperplasia with CD-20 positive lymphocytes. The 2008 World Health Organization classification for lymphoid malignancies divides PTLD into four major categories: early lesions, polymorphic PTLD, monomorphic PTLD and Hodgkin PTLD. The treatment and prognosis depend on histology. The cornerstone of PTLD therapy includes reduction/withdrawal of immunosuppression, monoclonal anti CD-20 antibody (rituximab) and chemotherapy. OUTLINE OF CASES: We reported here our experiences with three patients, two girls aged 7.5 and 15 and a 16-year old boy. They had different organ involvement: brain, combined spleen-liver and intestines, respectively. Even though EBV was a trigger of lymphoid proliferation as it was confirmed by histopathology or in cerebrospinal fluid, qualitative EBV-PCR was positive only in one patient at disease presentation. Reduction of immunosuppression therapy was applied in treatment of all three patients, while two of them received rituximab and ganciclovir. They had an excellent outcome besides many difficulties in diagnosis and management of disease. CONCLUSION: Qualitative EBV-PCR is not useful marker in pediatric transplant recipients. Our suggestion is that patients with the risk factors like T-cell depleting agents, immunosuppressant protocol or increasing immunosuppressive therapy and EBV miss-match with donor must be more accurately monitored with quantitative EBV PCR.

Intrapericardial immature teratoma in a newborn: a case report.

Intrapericardial teratomas are extremely rare and most often benign tumours. In this paper, we have described a case of intrapericardial teratoma diagnosed prenatally and successfully operated. The presented case is noteworthy as an example of potentially catastrophic cardiorespiratory distress caused by the space-occupying nature of the tumour. A multi-disciplinary approach is mandatory because the tumour most often arises from the ascending aorta and in some cases may require the use of cardiopulmonary bypass.

Severe penile injuries in children and adolescents: reconstruction modalities and outcomes.

OBJECTIVE: To review our experience with severe penile trauma, mechanism of injury, and their treatment modalities in 16 children younger than 18 years. Management of penile trauma poses diverse challenges to the reconstructive urologist, as injuries vary from abrasions to total emasculation. METHODS: Analysis of 16 patients with severe penile injuries referred to us between 2002 and 2011 was undertaken. The median age at surgery was 13 years (range, 5-17). Etiology of penile trauma and choice of treatment were evaluated. The management included a wide variety of surgical techniques that were tailored to the individual patient. Results were analyzed to define etiology, that is, mechanism of penile injury and to estimate modalities of surgical management and postoperative outcomes. Also, postoperative questionnaire was used, which included questions on functioning and esthetical appearance of participating patients and overall satisfaction. RESULTS: The causes of penile injury in these series were traffic accidents (2), iatrogenic trauma (5), self-amputation (1), electrocution (1), burns (3), dog bite (2), zipper injury (1), and mother's hair strangulation (1). The mean follow-up was 46 months (range, 14-122), and examinations were uneventful, except for 2 fistulae formation after neophallic urethral reconstruction. CONCLUSION: The main goal of reconstructive surgery is to have a penis with normal appearance and functions. Severe penile injuries should be treated on a case-by-case basis using the most propitious techniques.

Fast-track surgery concepts for congenital urogenital anomalies.

AIM: To investigate the possibility of fast-track surgery concepts in pediatric urology department as a single center study model of a developing country. MATERIAL OF STUDY: The study included 1620 patients surgically treated at the pediatric urology department, from 2009 to 2011. According to the congenital anomalies, all patients were classified in one of four groups: I - testicular anomalies (197 patients); II - external genital anomalies (453); III - upper urinary tract anomalies (801) and IV - associated anomalies (169). We analyzed the total duration of stay in the hospital of all patients among all treating doctors concerning the anomaly. RESULTS: Statistically significant difference in total length of hospitalization of all patients in Group I was noted in Doctors 1 and 5 (F=10.36** for F0.05;5;12=3.11 and F0.01;5;12=5.06), as well as in the Group II (F=17.01** for F0.05;5;12=3.11 and F0.01;5;12=5.06). Statistical analysis was not possible to be performed in groups III and IV because of lack of the patients. DISCUSSION: Analyzing the length of hospitalization of the patients treated at the urology department, all doctors showed the tendency to shorten the total length of hospitalization in patients of all groups. Majority of the studies carried out on pediatric urology departments in developed countries, showed that over 50% of children were successfully treated using fast-track surgery concept. CONCLUSIONS: Modern methods of surgical management and anesthesia allow decrease of hospitalization length, financial savings to the healthcare system and better comfort for patients.

Treatment for failed epispadias repair presenting in adults.

PURPOSE: We evaluated the results of 1-stage and multistage penile reconstruction in adults with complications after multiple failed epispadias repairs. MATERIALS AND METHODS: A total of 23 adults underwent penile disassembly for repeat epispadias repair from February 2006 to June 2011. Median age at surgery was 27 years (range 17 to 41). Surgical treatment included penile disassembly with complete straightening and lengthening of the penis, followed by urethral reconstruction. The corpora cavernosa were completely separated from the glans cap with the neurovascular bundles and from the urethra. The urethra, which was short in all cases, was divided at the glans level. Penile straightening and lengthening were achieved by tunica albuginea incision and grafting. The urethra was reconstructed 3 to 6 months later using combined buccal mucosa graft and genital skin flaps. Success was defined as a functional penis without urethral fistula or stricture, or residual chordee as well as a cosmetically acceptable penile appearance. RESULTS: Mean followup was 33 months (range 14 to 78). Of the patients 19 had a completely straightened and lengthened penis. Improved length of the erect penis was 2.7 to 6.6 cm. In 4 patients mild curvature developed without the need for additional correction. Urethral fistula in 3 cases was repaired 6 months after urethroplasty in 2, while it closed spontaneously in 1. All 21 sexually active patients reported good erectile function. CONCLUSIONS: Adults with complications after childhood epispadias repair are still a difficult population to treat. A radical surgical approach must be used to achieve a successful outcome.

Epispadias repair after failed surgery in childhood.

INTRODUCTION: Redo surgery in failed epispadias presents a great challenge. Our aim was to present a radical approach for correction of penile deformities as well as urethral reconstruction in patients after failed epispadias repair. MATERIALS AND METHODS: Between January 2006 and January 2011, 13 patients, aged 13 to 22 years, underwent redo surgery due to failed epispadias repair in childhood. All patients presented with severe dorsal curvature and short urethra. First stage included penile disassembly technique with complete separation of corporal bodies, urethral dissection, and transposition and subtotal glans mobilization. Residual dorsal curvature was corrected by tunical incision and grafting of the defect. Short urethra was dissected and transposed ventrally with opening at the base of the penis. Penile entities were reassembled in normal anatomical relationship. Penile body was covered using available vascularized skin flaps. After 6 months, second stage was performed and included reconstruction of the penile urethra using buccal mucosa graft and scrotal hairless skin flap. RESULTS: Follow-up ranged from 12 to 60 months (mean 33 months). Acceptable outcome is achieved in all the patients. Complete penile lengthening and straightening is obtained in 10 out of 13 patients. Mild curvature is noted in three patients without consequences. Satisfactory sexual activity was reported from nine patients. One patient developed fistula that was closed after 4 months, whereas all other patients reported normal voiding with no difficulties. CONCLUSIONS: Redo surgery of failed epispadias is very demanding procedure. Radical approach in these cases is necessary for complete repair of all penile deformities with satisfactory postoperative outcome.

Warren shunt combined with partial splenectomy for children with extrahepatic portal hypertension, massive splenomegaly, and severe hypersplenism.

PURPOSE: To establish the importance of shunt surgery combined with partial resection of the spleen for selected pediatric patients with extra-hepatic portal vein obstruction (EHPVO), enormous splenomegaly and severe hypersplenism. Severe hypersplenism is often refractory to treatment with endoscopic sclerotherapy or band ligation and shunt surgery; however, to our knowledge, this is the first such study to be published. METHODS: Distal splenorenal shunt with partial resection of the spleen was performed in 16 of 60 children treated for EHPVO in the Gastroenterology Department of our hospital. Upper gastrointestinal endoscopy had shown esophageal varices of varying grade in all patients and band ligation or endoscopic sclerotherapy had been done for children with a history of bleeding. The indications for surgery were pain and discomfort caused by a large spleen (5-15 cm below the costal margin) and symptomatic hypersplenism with leucopenia, thrombocytopenia, and anemia. Partial resection of the spleen was performed, starting with ligation of the branches and tributaries of the caudal two-thirds. When an ischemic line demarcated the splenic parenchyma, it was transected using electrocautery or LigaSure, leaving 20-30 % of splenic tissue. After the spleen resection, a Warren shunt was performed. Platelet and white blood cell counts and liver function tests were performed before and after the operation. Growth was assessed using SD scores (z scores) for height, weight, and body mass index at the time of surgery and 1 year later. RESULTS: Postoperative recovery was uneventful and the leukocyte and platelet counts normalized. The shunt patency rate was 100 %. Two cases of shunt stenosis were treated successfully with percutaneous angioplasty. There was no postoperative mortality. During the follow-up period, from 1 to 7 years, all 16 children were asymptomatic, with improved quality of life, growth, and nutrition. No episodes of variceal bleeding, sepsis or encephalopathy occurred. CONCLUSION: Our results demonstrate that shunt surgery with partial resection of the spleen is effective and safe for pediatric patients with massive splenomegaly and severe hypersplenism secondary to EHPVO.

[Possibilities of fast track surgery principles in the treatment of congenital urological anomalies].

INTRODUCTION: Urogenital congenital anomalies are among the most common congenital anomalies and very frequent pathology in paediatric urology. Health care systems strive to shorten the duration and reduce the costs of hospitalization, while maintaining treatment effectiveness. OBJECTIVE: To evaluate the duration of hospital stay of surgically treated patients with congenital urogenital anomalies and estimate the possibility of using fast track surgery principles in paediatric urology in the local settings of a developing country. METHODS: Retrospective non-randomized study included 552 patients who had been surgically treated at the Urology Department of the University Children's Hospital, during 2010. In line with their congenital anomalies, all patients were classified in one of four groups: I--upper urinary tract anomalies (252 patients); II--genital anomalies (164 patients); III--testicular anomalies (76 patients) and IV--associated anomalies (60 patients). We analyzed the total duration of stay as well as pre- and post-operative stay in the hospital. RESULTS: The average duration of hospitalization was 4.7 +/- 4.0 days. Patients with testicular anomalies stayed for the shortest period (2.3 +/- 1.9 days) (p < 0.01) and patients with associated anomalies stayed in the hospital the longest (6.5 +/- 4.7 days) (p < 0.01). CONCLUSION: Modern methods of surgical treatment allow reduction of hospitalization, financial savings to the healthcare system and greater comfort for patients. Our results showed that this is also possible to apply in our environment.

[Urethral reconstruction in severe hypospadias using buccal mucosa graft and penile skin flap].

INTRODUCTION: Hypospadias represents the most frequent penile anomaly. The most challenging part of hypospadias surgery is urethral reconstruction. Many various tissues are used (local skin flaps, bladder mucosa grafts, buccal mucosa grafts etc.) for the reconstruction of the neourethra. OBJECTIVE: Our aim was to evaluate advantages and disadvantages of combined buccal mucosa graft and penile skin flap in urethral reconstruction in severe hypospadias repair. METHODS: Between December 2005 and August 2009, 48 patients with severe hypospadias, aged from nine months to 12 years (mean age 23 months) underwent surgery. Thirty-four (71%) had penoscrotal and 14 (29%) scrotal hypospadias. Ventral penile curvature was present in all cases, and was corrected by incision of the short urethral plate. Urethroplasty was performed a buccal mucosa graft combined with dorsal penile skin flap. RESULTS: Mean follow-up was 22 months (range from 9 months to 3 years). Satisfactory functional and aesthetic results were achieved in 40 (83%) patients. Fistula was noted in six cases and was solved by minor revision. Urethral stenosis in two cases was resolved by simple dilatation. CONCLUSION: Urethral reconstruction using the buccal mucosa graft and dorsal penile skin flap in severe hypospadias repair could be the method of choice. Using this technique, single stage urethral reconstruction is possible with satisfactory esthetical and functional results.