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Purpose: The Uro Map application for smartphones was developed to help healthcare providers in the treatment of children with urological malformations, raising the possibility of graphically representing each urinary tract unit as an schematic illustration and associating it with relevant data from the patient’s medical history, producing an illustration representing the child’s urinary tract status that can be both archived and/or shared among caregivers. This app was submitted to a group of caregivers to test its accuracy and the results of this survey was the basis of the present report. Methods: Common pediatric urological situations were simulated through the application, generating ten images, which were attached to a form. For each figure, a multiple-choice question was assigned, with only one correct answer per question. The forms were sent to 875 Pediatric Surgeons and 400 Pediatric Nephrologists through their national scientific associations. Results: 174 Pediatric Surgeons, 53 Pediatric Nephrologists, 18 Pediatric Surgery Residents, 26 urologists and 12 other healthcare providers answered the questionnaire. The latter 2 groups were formed due to the sharing of the form by the participants themselves. The mean score in each group was: pediatric surgeons 9.51, pediatric nephrologists 9.52, pediatric surgery residents 9.88, urologists 9.69 and other participants 8.8. Conclusion: The Uro Map application was successfully developed and based on the participants’ scores, it can be considered effective to represent the most diverse pediatric urological disorders.

AimThe purpose of this study is to present data on the regional distribution of the pediatric surgical workforce and the expected local demand of pediatric operations in Brazil.MethodsWe collected data on the pediatric surgical workforce, surgical workload, GDP/capita and mortality for gastrointestinal tract malformations (MGITM) across the different regions of Brazil for 2019.FindingsData from CFM reported the existence of 1515 pediatric surgery registries in Brazil, corresponding to 1414 individual pediatric surgeons (some pediatric surgeons are registered in more than one State), or 2.4 pediatric surgeons per 100.000 children< 14 years. There were 828 male and 586 female with a M/F ratio of 1.14:1. Mean age was 51.5±12.8 years and mean time from graduation was 3,4±5,7years. Regional distribution showed a higher concentration of pediatric surgeons on the wealthier Central-West, South and Southeast regions. Individual workload ranged from 88 to 245 operations/year (average 146 operations/year) depending on the region. Only 9 (6.1%) of these were high complexity (including neonatal) operations. MGITM tended to be higher on the poorer North and Northeast regions of BrazilConclusionsWe found wide disparities in the surgical workforce and workload across Brazil, related to socioeconomic status. Areas of increased surgical workforce were associated with lower MGITM. The average number of complex operations performed yearly by the individual pediatric surgeon was considerably low. Strategic investment and definition of health policies may be needed to improve the quality of care in the different regions of Brazil.Level of EvidenceRetrospective review. Level III

Introduction: The rapid development of video surgery and minimally invasive surgical techniques prompted many studies on the methods of teaching these techniques to young surgeons in training. However, the characteristics of a short-term course that is both easily accessible and efficient for this group of surgeons remain controversial. To investigate this issue, a short-term training method was proposed for first year surgery residents, using inexpensive handmade wooden simulation boxes with the students smartphones as cameras . Its effectiveness was evaluated, as well as possible factors that could influence student performance, such as gender and previous experience with video games. Methods: 36 first-year General Surgery residents, entering in 2019 and 2020, participated in the study: 21 were males and 15 were females with ages between 22 and 29 years old, (mean 25.47 years). All participants performed a pre-established exercise (placing two simple stitches using a laparoscopic simulator), which was timed and scored. They then participated in a short theoretical-practical course, consisting of an initial lecture followed by 4 exercises on handcrafted wooden laparoscopic video surgery simulators. Afterwards, they were asked to repeat the same exercise from the first step. Finally, they answered a questionnaire that included questions on previous videogame experience. The data were tabulated and submitted to statistical analysis.Results: In the pre-training exercise, 15 (41.66%) participants were able to perform the two simple stitches in the simulator box within the maximum time limit of 5 minutes. After the short course, 22 (61.11%) of participants were able to perform the complete exercise. Improvement in the time to complete the practical exercise was statistically significant (p=0.0296) after participating in the theoretical-practical course. A better pre- and post-training performance was demonstrated by the 17 participants with experience with video games (p=0.0116), and a better post-training performance was demonstrated by female participants (p=0.0405). Conclusion: This short-term inexpensive theoretical-practical course in laparoscopic training for surgeons in training was effective in reducing the execution time of a laparoscopic stitch in a simulation box. Previous experience with video games and/or female gender appear to be associated with improved performance.

Background: Increased intra-abdominal pressure resulting from pneumoperitoneum can cause renal physiological changes, such as oliguria and anuria, in mammals. Although videolaparoscopic op...

Objective: To evaluate, in a sample of patients with disorders of sex development (DSD), data related to the age at referral and their correlation with the initial complaints, gender at referral, defined gender after diagnosis and etiological diagnosis. Methods: Retrospective review of the age at the first consultation and the reason for it, initial social gender and gender after the diagnosis, karyotype and etiological diagnosis of all cases treated at a DSD outpatient clinic between 1989 and 2016. Cases that did not involve DSD and DSD diagnoses that do not usually involve ambiguous genitalia, thus not requiring specialized monitoring, were excluded. Results: Of the 1793 treated cases, 1139 were diagnosed with some type of DSD. This study excluded 430 cases (272 with Turner's syndrome, 66 with Klinefelter syndrome, and 92 with pure gonadal dysgenesis), thus a total 709 individuals were included. Of these, 82.9% were referred due to ambiguous genitalia; only one-quarter were still in the first month of life, and 6.6% were referred due to pubertal delay, with most of them aged 10 years or older. Of these patients, 68.6% had a diagnosis of XY DSD, 22.4% of XX DSD, and 9% of sex chromosome abnormalities. Conclusions: This study presents the largest series in the literature of patients with DSD treated in a single center. The time of referral of the majority of patients with ambiguous genitalia fell short of the ideal, and milder cases of ambiguous genitalia and many with pubertal manifestations were referred even later. The results reinforce the importance of continuing education for professionals who will have the first contact with these patients, mainly pediatricians and neonatologists. Resumo Objetivo: Avaliar em uma amostra de pacientes com distúrbios da diferenciação do sexo (DDS), dados relacionados à idade, ao encaminhamento e sua correlação com as queixas iniciais, ao sexo ao encaminhamento e ao sexo final e diagnóstico etiológico. Métodos: Revisão retrospectiva da idade por ocasião da primeira consulta e motivo dela, sexo social inicial e após definição do diagnóstico, cariótipo e diagnóstico etiológico de todos os casos atendidos em um ambulatório especializado em DDS entre 1989 e 2016. Foram excluídos casos que não compreendiam DDS e diagnósticos de DDS que não cursam comumente com ambiguidade genital, não necessitam de acompanhamento especializado. Resultados: Dos 1.793 casos atendidos, 1.139 foram diagnosticados com algum DDS. Excluíram-se 430 (272 síndrome de Turner, 66 síndrome de Klinefelter e 92 disgenesia gonadal pura), totalizando 709. Desses, 82,9% foram encaminhados por ambiguidade genital, somente um quarto ainda no primeiro mês de vida e 6,6% por atraso puberal, a maioria com 10 anos ou mais; 68,6% tiveram diagnóstico de DDS XY; 22,4% DDS XX e 9% de anomalias dos cromossomos sexuais. Conclusões: Este estudo apresenta a maior casuística na literatura de pacientes com DDS atendidos em um único serviço. O momento de encaminhamento da maioria dos pacientes com ambiguidade genital foi aquém do ideal e casos mais leves de ambiguidade e muitos com manifestações puberais foram encaminhados ainda mais tardiamente. Os resultados reforçam a importância do ensino continuado a profissionais que terão o primeiro contato com esses pacientes, principalmente pediatras e neonatologistas.

Ovotesticular disorder of sex development (OT-DSD) is a rare condition characterized by coexistence of ovarian and testicular parenchyma, in separate gonads or in the same gonad (ovotestis), in individuals with variable degrees of genital ambiguity. Karyotype may be 46,XX (60%), 46,XY (10%), or there may be sex chromosomes abnormalities, including mosaicism, chimerism and structural anomalies (30%). A genetic origin may be identified in some subjects with normal XX or XY karyotype, but most cases are of unknown origin. Apparently balanced chromosome rearrangements (translocations, insertions and inversions) may cause truncation, deletion, inactivation or over-expression of specific genes. We report on a case of OT-DSD associated with an X chromosome inversion. Case report: A 3-month old girl was referred due to atypical genitalia. She was born at term to a 42 years old G3P2A1 mother and her 45 years old unrelated husband with normal weight, length and head circumference. She had normal development, no associated health problems, and family history was unremarkable. Physical examination revealed a 3.1-cm phallus with chordee, scrotal hypospadias, partial penoscrotal inversion and a 0.5 cm3 right gonad palpable in the inguinal region; there were no associated dysmorphic features. At 1.5 months there were normal levels of FSH (3.09 IU/L) and LH (3.67 IU/L), and testosterone (155 ng/dL) was in the normal male range. Ultrasound revealed normal uterus and gonads were in the inguinal regions. Urethra-cystoscopy and vaginoscopy at 9 months revealed a urogenital sinus with high vaginal confluence. Laparoscopy and gonadal biopsies were also performed; the left gonad was an ovotestis with multiple ovarian follicles, while the right gonad was a testis. In both gonads the seminiferous tubules had only Sertoli cells. Karyotype revealed a pericentric X chromosome inversion, 46,X,inv(X)(p22.1q26)dn[20]. FISH on peripheral blood and cultured cells from the right gonad with probes for X (DXZ1) and Y (DYZ3) centromeres and SRY (Yp11.3 - 122 Kb) showed only two X chromosome signals. Array GH analysis (Cytoscan 750K, Affymetrix) showed a 1.3 Mb deletion distal to the short arm breakpoint (Xp22.31), which was reported as VOUS, and a 9 Mb region of LOH on chromosome 9. Discussion: Several cases of X pericentric inversion with different breakpoints have been reported; though phenotypes of female heterozygotes are often normal, early menopause, irregular menses, gonadal dysgenesis or sterility have been described. In this case, it is plausible that the genomic rearrangement could have affected long-range regulation of SOX3 (located in Xq27.1) resulting in ectopic expression of this gene in the bipotential gonad. In addition, the features detected in array GH may have a role in the phenotype. Different methods to determine the exact chromosomal breakpoints and copy number variations in this region will be required.

COVID-19 is the disease caused by SARS-COV-2 coronavirus infection (Severe acute respiratory syndrome coronavirus 2). Although its most prevalent symptoms are respiratory, there are descriptions of gastrointestinal manifestations in children, but the presentation as an acute abdomen is rare. We report the case of a 6-month-old infant who was admitted with a diagnosis of intestinal obstruction and generalized peritonitis with no apparent cause, in whom a SARS-CoV-2 rt-PCR search was positive. We have not found descriptions of similar cases in the literature so far.

Embora seja usual a realização de cirurgias vídeo-laparoscópicas, a ocorrência de lesões renais decorrentes destes procedimentos ainda não foi precisamente documentada na literatura médica. Todavia, é sabido que a elevação da pressão intra-abdominal por meio do pneumoperitônio cirúrgico pode ocasionar oligúria ou anúria em mamíferos. O objetivo deste trabalho é avaliar o impacto do pneumoperitônio na função glomerular renal, ventilação pulmonar e estresse oxidativo em um modelo experimental de coelhos machos, através da perfusão renal com 99mTc-DTPA (diethylene triamine pentaacetic acid, marcado com tecnécio), análise de volumes e gases respiratórios, bem como o status inflamatório e estresse oxidativo resultantes, através de marcadores específicos. Serão utilizados cinco coelhos machos Nova Zelândia saudáveis, previamente anestesiados e ventilados mecanicamente.

Background: Ovotesticular disorder of sex development (OT-DSD) (true hermaphroditism) is an anatomopathological diagnosis based on the findings of testicular and ovarian tissues in the same subject, in the same gonad (ovotestis), or in separate gonads. OT-DSD is a rare cause of sex ambiguity, and the most common karyotype is 46,XX; mosaics and chimeras are found only in 10%–20%. Aim: To report a case of an OT-DSD patient with a rare karyotype constitution. Case report: A 2-month-old child with male sex assignment was referred to our clinic for investigation of sex ambiguity. He was the second child of healthy unrelated parents; pregnancy and labor were uneventful. On physical examination, he had a 2.3-cm phallus and perineal hypospadias (Prader grade III); the right gonad was in the labioscrotal fold and the left was found in the inguinal channel. Karyotype was 46,XX/47,XXY/48,XXYY. Anatomopathological examination of gonads revealed right testis and left ovotestis. The male sex assignment was maintained; the child underwent left gonadectomy, removal of Mullerian structures and urethroplasty. Conclusion: A thorough revision of literature revealed a single case of OT-DSD with the same chromosome constitution. Gonadal biopsy is necessary to establish diagnosis in cases of sex chromosome mosaicism.

Objective. To evaluate diagnosis, age of referral, karyotype, and sex of rearing of cases with disorders of sex development (DSD) with ambiguous genitalia.Methods. Retrospective study during 23 years at outpatient clinic of a referral center.Results. There were 408 cases; 250 (61.3%) were 46,XY and 124 (30.4%) 46,XX and 34 (8.3%) had sex chromosomes abnormalities. 189 (46.3%) had 46,XY testicular DSD, 105 (25.7%) 46,XX ovarian DSD, 95 (23.3%) disorders of gonadal development (DGD), and 19 (4.7%) complex malformations. The main etiology of 46,XX ovarian DSD was salt-wasting 21-hydroxylase deficiency. In 46,XX and 46,XY groups, other malformations were observed. In the DGD group, 46,XY partial gonadal dysgenesis, mixed gonadal dysgenesis, and ovotesticular DSD were more frequent. Low birth weight was observed in 42 cases of idiopathic 46,XY testicular DSD. The average age at diagnosis was 31.7 months. The final sex of rearing was male in 238 cases and female in 170. Only 6.6% (27 cases) needed sex reassignment.Conclusions. In this large DSD sample with ambiguous genitalia, the 46,XY karyotype was the most frequent; in turn, congenital adrenal hyperplasia was the most frequent etiology. Malformations associated with DSD were common in all groups and low birth weight was associated with idiopathic 46,XY testicular DSD.

We report the case of a 46,XX infant referred at 3 months of age for evaluation of ambiguous genitalia with no palpable gonads. The phallus was replaced by a homogeneous peduncular structure covered by skin, and a second peduncular structure covered by mucosa was located between the labia minora above the urethral meatus and the vaginal opening. There were no associated anomalies, and she had normal growth and neuromotor development. During surgery to remove these structures, a bifid clitoris was detected and cystoscopy and vaginoscopy revealed normal female urethra and vagina. The structure located on the phallic region was a superficial lipomatous nevus, and the other mass was a congenital urethral caruncle with ectopic migration/differentiation of colonic epithelium into ventral and anterior part of the urogenital sinus. Both genital lipomas and congenital caruncles of the female urethra are very rare and their etiology is unknown. There is no previous report of coincidence of these two rare findings in an otherwise healthy girl.

INTRODUCTION: Dilation of urinary tract occurs without the presence of obstruction. Diagnostic methods that depend on renal function may elicit mistaken diagnosis. Whitaker (1973) proposed the evaluation of urinary tract pressure submitted to constant flow. Other investigators proposed perfusion of renal pelvis under controlled pressure, making the method more physiological and reproducible. The objective of the present study was to evaluate the results of the anterograde pressure measurement (APM) of the urinary tract of children with persistent hydronephrosis after surgery suspected to present persistent obstruction. MATERIALS AND METHODS: Along 12 years, 26 renal units with persistent hydronephrosis after surgery (12 PUJ and 14 VUJ) were submitted to evaluation of the renal tract pressure in order to decide the form of treatment. Previous radionuclide scans with DTPA, intravenous pyelographies and ultrasounds were considered undetermined in relation to obstruction in 10 occasions and obstructive in 16. APM was performed under radioscopy through renal pelvis puncture or previous stoma. Saline with methylene blue + iodine contrast was infused under constant pressure of 40 cm H2O to fill the urinary system. The ureteral opening pressure was measured following the opening of the system and stabilization of the water column. RESULTS: Among the 10 cases with undetermined previous diagnosis, APM was considered non-obstructive in two and those were treated clinically and eight were considered obstructive and were submitted to surgery. Among the 16 cases previously classified as obstructive, nine confirmed obstruction and were submitted to surgery. Seven cases were considered non-obstructive, and were treated clinically, with stable DMSA and hydronephrosis. CONCLUSIONS: APM avoided unnecessary surgery in one third of the cases and was important to treatment decision in 100%. We believe that this simple test is an excellent diagnostic tool when selectively applied mainly in the presence of functional deficit.

Correction of gastroschisis may be accomplished by either primary or staged closure or even delayed primary closure after the use of a preformed silo. However, there is neither a consensus on the best approach nor established criteria to favor one method over the other. The aim of this paper was to investigate the role of intravesical pressure (IVP) as a tool to prevent abdominal compartment syndrome in newborns undergoing correction of abdominal wall defects.We retrospectively analyzed 45 newborns with gastroschisis in whom trans-operative intravesical pressure was used to choose between primary or staged closure. A threshold of 20 cm H(2)O was used and the outcomes between the two methods were compared.In 24 children delayed primary closure was achieved while the remaining 21 underwent staged reduction and closure. There was no difference in the frequency of complications, time to begin oral feeding, length of parenteral nutrition or length of hospital stay between the children of the two groups. The incidence of temporary oliguria or anuria, averaged 33% and it was similar in both groups of children.The data here presented suggests that monitoring intraoperative IVP during correction of gastroschisis may help to select children in whom staged closure is necessary, keeping their complication rate and overall outcome similar to that of children undergoing delayed primary closure. Further prospective studies should investigate more deeply the correlation between type of closure and the development of a compartment syndrome.

Preventing sudden changes in intraabdominal pressure (IAP) during surgical pneumoperitoneum may reduce adverse events. This study aimed to describe a valve system that stabilizes intraabdominal pressure, minimizing complications of erratic fluctuations in IAP. Five male Sprague-Dowley rats were submitted to pneumoperitoneum, with the insufflator set sequentially at 5, 10, and 15 mmHg for each rat. Measures of IAP were taken initially without the valve and then using the same insufflator levels with the valve system regulated to three different pressures (5, 10, and 15 mmHg). The mean of the three highest registered pressures during a 15-min observation was used as the maximal pressure, and the mean of the three lowest registered pressures was used as the minimal pressure for each experimental setting. Without the valve system, the pressure level set by the insufflator correlated poorly with the actual IAP. When the valve system was used, the IAP pressure was limited by the valve settings regardless of the insufflator settings. Also, the variability of IAP was significantly higher when no valve was used than in all situations that had implementation of the system. The valve system was very effective in stabilizing IAP, allowing a reproducible and reliable estimate of IAP and greatly reducing the variability resulting from the cycling mechanism of the insufflator. Due to the small dimensions of intracorporeal cavities in the newborn, this mechanism may help to improve safety when neonatal video-assisted surgery is performed.

Differentiation of testicular tissue in 46,XX individuals is seen either in XX males, the majority of them with SRY gene, or in individuals, usually SRY(-), with ovotesticular disorder of sex development (OT-DSD). Although they are sporadic cases, there are some reports on familial recurrence, including coexistence of XX maleness and OT-DSD in the same family.We report on a case of SRY(-) 46,XX monozygotic twins with genital ambiguity.Hormonal evaluation included testosterone, FSH, and LH measurements. SRY gene was investigated by PCR and two-step PCR in peripheral leukocytes and gonadal tissues, respectively. Direct DNA sequencing of the DAX-1 coding sequence was performed. Real-time PCR for SOX9 region on chromosome 17 was obtained.Both twins had a 46,XX karyotype. Twin A had a 1-cm phallus with chordee, penoscrotal hypospadias, and palpable gonads. Serum levels of FSH (2.34 mIU/ml), LH (8.8 mIU/ml), and testosterone (1.6 ng/ml) were normal, and biopsies revealed bilateral testes. Twin B had a 0.5-cm phallus, perineal hypospadias, no palpable gonad on the right, and a left inguinal hernia. Hormonal evaluation revealed high FSH (8.2 mIU/ml) and LH (15 mIU/ml) and low testosterone (0.12 ng/ml). Upon herniotomy, a right testis (crossed ectopia) and a small left ovotestis were found. SRY gene was absent in both peripheral leukocytes and gonadal tissue samples. Neither DAX-1 mutations nor SOX9 duplication was identified.This case provides evidence that both XX maleness and XX OT-DSD are different manifestations of the same disorder of gonadal development.

To report a series of children with pelviureteric junction obstruction (PUJO) due to lower polar crossing vessels who underwent laparoscopic vascular transposition. In order to confirm the relief of the obstruction and avoid unnecessary additional procedures, we suggest performing an intraoperative measure of the ureteral opening pressure.From January 2007 and January 2014, 11 children underwent laparoscopy to treat well-documented PUJO by polar vessels. In the first 7 cases, children underwent a careful dissection of the polar vessels that were transposed cranially in the pelvis. In the last 4 cases, a percutaneous needle was inserted into the renal pelvis, and the ureteral opening pressure was obtained intraoperatively, before and after the vascular hitch procedure, in 3 cases. No vascular relocation was necessary except in 1 case with a polar vessel unrelated to the obstruction.The laparoscopic procedure was feasible in all cases. Median operative time was 90 minutes without intraoperative complications. In the last 3 cases, a decrease in the renal pelvic pressure was demonstrated just after releasing the ureter from the polar vessels, confirming the extrinsic obstruction. In 1 case, the intraoperative pelvic pressure measurement showed that there was no vascular compression but that obstruction was due to renal rotation. During follow-up (range, 12-96 months) all patients reported resolution of their symptoms, nine children showed a decrease in the hydronephrosis grade, and all but one with poor function had improved drainage on diuretic renography.Intraoperative measurement of ureteral opening pressure may help to confirm that the vascular hitch procedure has relieved the pelvic obstruction, precluding the need for dismembered procedures. We believe that in some doubtful cases, with the addition of intraoperative pelvic pressure measurement, vascular hitch may be considered a safe procedure to treat selected cases of PUJO in children.

Background/Purpose Stenosis of the vaginal introitus is the most frequent complication after genital reconstruction for ambiguous genitalia associated with congenital adrenal hyperplasia (CAH). With the aim of enlarging the vaginal introitus, the authors present a technical modification of the introitoplasty that uses a bilateral cutaneous island flap based on the perineal superficial branches of the internal pudendal artery. Methods Eleven girls with CAH and Prader III to V genital ambiguity were included. Feminizing genitoplasty was performed in 1 stage. Bilateral cutaneous labioescrotal island flaps, based on the posterior labial artery, were included in the introitoplasty. The cosmetic results of the genitoplasty were evaluated by photographic analysis of the external genitalia. Results Integrity of the vaginal introitus as well as excellent integration of the flap and absence of additional scars in the donor area were assessed in all girls. Conclusions This modified island flap is technically feasible and reproducible producing no additional sequels in the donor area. It uses perineal skin that is usually excised in other techniques avoiding the use of harvesting skin from adjacent areas. Thus, it can be a useful additional procedure in the introitoplasty in association with the currently used techniques.

Background Congenital perineal lipoma is extremely rare and may lead to a misdiagnosis of ambiguous genitalia. Case reports We report on two girls referred to our service for ambiguous genitalia. Patient 1 (17 days old) and patient 2 (2 months old) had unremarkable gestational and perinatal histories. Both had normal female external genitalia and a 46,XX karyotype. Patient 1 had a polypoid, protruding 3.0 x 2.0 x 1.5-cm phallic-like mass arising at the inferior border of the left labium majora, and patient 2 had a similar mass of 1.5 x 1.5 x 1.0 cm at the same site and an imperforate anus. In both cases the mass was removed and found to be a lipoma. Discussion To our knowledge, perineal lipoma has been reported only in eleven girls, nine of them with associated anorectal malformation. Migration and fusion of the labioscrotal folds and formation of the urorectal septum are simultaneous developmental events occurring in the same region, which may explain the association of perineal lipoma and anorectal malformations.

Elevations of intraabdominal pressure during laparoscopic procedures may lead to oliguria or anuria in mammals. Despite this, previous research has not been able to confirm an associated kidney injury. This study aimed to investigate the occurrence of an early kidney lesion secondary to surgical pneumoperitoneum in a rat model using the expression of neutrophil gelatinase-associated lipocalin (N-GAL) as a biomarker for early kidney injury. In this study, 20 male Sprague-Dawley rats under general anesthesia and mechanically ventilated were allocated to one of five experimental time-dependent groups: group 1 (1-h control), group 2 (1-h pneumoperitoneum), group 3 (2-h control), group 4 (2-h pneumoperitoneum), and group 5 (positive kidney injury group induced by intravenous administration of cisplatin 7.5 mg/kg). To evaluate the renal expression of N-GAL 24 h after the procedure, all the rats underwent a 2-h urine output evaluation as well as laparotomy and bilateral nephrectomy performed sequentially to investigate the presence of renal injury using immunofluorescence qualification and western blotting. Urine output was reduced and N-GAL expression was increased in the animals from the cisplatin group. The animals undergoing 1- or 2-h pneumoperitoneum displayed urine output and N-GAL expression similar to that of the animals from the matching control groups. Under the experimental conditions of this study, the animals with normal preoperative renal function did not show any type of acute kidney injury associated with the presence of a stabilized surgical pneumoperitoneum.

OBJECTIVE: Report the results of laparoscopic upper-pole nephroureterectomy in infants. MATERIALS AND METHODS: Six consecutive infants underwent 7 laparoscopic upper-pole nephroureterectomy. Pre and postoperative evaluation included renal sonography, voiding cystourethrogram and renal scintigraphy. All infants showed upper-pole exclusion. Surgery was performed through a transperitoneal approach with full flank position in all infants. Three or 4 ports were used according to the necessity of retracting the liver. The distal ureter was ligated close to the bladder whenever reflux was present and the dysplastic upper-pole was divided with the help of an electrocautery. Data regarding operative time, postoperative use of analgesics, time to resume oral feeding, hospital stay and tubular function were collected and analyzed. RESULTS: All procedures were concluded as planned. Mean operative time was 135 min. One patient underwent staged bilateral upper-pole nephrectomy. There were no complications and the postoperative hospital stay was 48 hours in 5 procedures and 24 hours in 2 procedures. Pain medication was required only in the first day. Renal tubular function showed improvement in half of the cases. CONCLUSION: Laparoscopic partial nephrectomy is a safe and feasible procedure in infants. Due to the magnification provided by the lenses, a better vision of the structures is achieved, facilitating selective dissection of vascular upper-pole, renal parenchyma and distal ureter. This approach is less damaging to the lower pole, and is associated to low morbidity and a short hospital stay.

To evaluate the anatomical and cosmetic results of feminizing genitoplasty in girls with ambiguous genitalia due to Congenital Adrenal Hyperplasia (CAH).Twenty-seven females with genital ambiguity (Prader III-V) due to CAH underwent an one-stage feminizing genitoplasty and were followed for a mean time of 4 years. In twelve girls, two cutaneous labioscrotal island-flaps were associated with the conventional genitoplasty, to decrease the excess of labia majora and to enlarge the vaginal introitus. Urinary tract infections were observed in sixteen girls in the preoperative and in only seven in the postoperative period. The cosmetic results were obtained by the systematic anatomical evaluation of the different structures that compose the genitalia.The results of clitoroplasty were considered good in 63% of the cases; however, 4 girls developed clitoral atrophy. An excess of labia majora was present in 25% and persistence of the urogenital sinus occurred in 11% (three children without labioscrotal island flap). The labioscrotal island flap was considered technically feasible and reproducible. Five girls had to be re-operated. The cosmetic results were considered excellent or good in 63% of the cases, satisfactory in 18.5% and unsatisfactory or bad in 18.5%.The anatomical and cosmetic results were good in majority of the cases; however, some technical aspects should be adjusted, to avoid irreversible sequels and re-operations.

OBJETIVO: Avaliar os resultados anatômicos e cosméticos das genitoplastias feminizantes realizadas em meninas com genitália ambígua devido à Hiperplasia Congênita das Adrenais (HCA). CASUÍSTICA E MÉTODOS: Vinte e sete meninas com HCA e ambigüidade genital (Prader III-V) foram submetidas a genitoplastia feminizante em um estágio e acompanhadas por período médio de quatro anos. Em doze meninas, dois retalhos labioescrotais em ilha foram associados à genitoplastia convencional para adequar os grandes lábios e ampliar o intróito vaginal. Episódios de infecção urinária foram observados em dezesseis meninas no pré-operatório e em apenas sete no pós-operatório. Os resultados cosméticos foram avaliados por meio de sistematização da avaliação anatômica das diferentes estruturas RESULTADOS: Os da clitoroplastia foram considerados bons em 62,9% dos casos; houve atrofia clitoriana em quatro meninas. Excesso de grandes lábios ocorreu em 25%, e persistência do seio-urogenital em 11% (três meninas nas quais não havia sido associado retalho labioescrotal). O retalho labioescrotal em ilha foi considerado de simples confecção e reprodução. Cinco meninas precisaram ser re-operadas. De um modo geral, os resultados cosméticos foram considerados ótimos ou bons em 63% dos casos, satisfatórios em 18,5% e insatisfatórios ou ruins em 18,5%. CONCLUSÕES: Os resultados anatômicos e cosméticos foram classificados como bons na maioria dos casos, porém alguns aspectos técnicos devem ser ajustados com a intenção de evitar complicações irreversíveis e re-operações. OBJECTIVE: To evaluate the anatomical and cosmetic results of feminizing genitoplasty in girls with ambiguous genitalia due to Congenital Adrenal Hyperplasia (CAH). CASUISTIC AND METHOD: Twenty-seven females with genital ambiguity (Prader III-V) due to CAH underwent an one-stage feminizing genitoplasty and were followed for a mean time of 4 years. In twelve girls, two cutaneous labioscrotal island-flaps were associated with the conventional genitoplasty, to decrease the excess of labia majora and to enlarge the vaginal introitus. Urinary tract infections were observed in sixteen girls in the preoperative and in only seven in the postoperative period. The cosmetic results were obtained by the systematic anatomical evaluation of the different structures that compose the genitalia. RESULTS: The results of clitoroplasty were considered good in 63% of the cases; however, 4 girls developed clitoral atrophy. An excess of labia majora was present in 25% and persistence of the urogenital sinus occurred in 11% (three children without labioscrotal island flap). The labioscrotal island flap was considered technically feasible and reproducible. Five girls had to be re-operated. The cosmetic results were considered excellent or good in 63% of the cases, satisfactory in 18.5% and unsatisfactory or bad in 18.5%. CONCLUSIONS: The anatomical and cosmetic results were good in majority of the cases; however, some technical aspects should be adjusted, to avoid irreversible sequels and re-operations.

Prenatal diagnosis of myelomeningocele (MM) allows planning its management and, recently, a possible in utero repair.To describe the perinatal outcome of fetuses with MM, in a Fetal Medicine Unit, identifying possible candidates for the in utero surgical repair.Retrospective and descriptive study of 58 cases of prenatally diagnosed MM, at CAISM-UNICAMP, from January 1997 to December 2001, identifying possible fetal candidates for in utero repair.the diagnosis mean gestational age was 29 weeks (17-39); level of lesions was above sacral region in 85%, association with hydrocephaly in 86%. Surgical complications were present in 39% of the neonates. During follow-up, 98% presented neurogenic bladder and 60% neurological/mental handicap. Twenty eight fetuses (42%) could have indication of in utero repair.MM is associated with severe and frequent poor results. Almost one third of our cases could had fetal repair as a treatment choice.

INTRODUÇÃO: O diagnóstico pré natal da meningomielocele (MM) permite melhor planejamento de sua abordagem e, mais recentemente , um possível reparo intra-útero. OBJETIVO: Descrição da evolução perinatal de fetos com MM, acompanhados em um centro de referência em Medicina Fetal, identificando os possíveis fetos candidatos à cirurgia intra-uterina. MÉTODO: Análise retrospectiva descritiva de 58 casos de MM fetal, atendidos no CAISM-UNICAMP, de janeiro de 1997 a dezembro de 2001, identificando-se os casos cuja indicação de cirurgia fetal seria possível. RESULTADOS: Média da idade gestacional ao diagnóstico de 29 semanas (17-39); nível da lesão acima da região sacral em 85%; associação com hidrocefalia em 86%; taxa de complicações cirúrgicas de 39%. Na evolução, 98% apresentaram bexiga neurogênica e 60% deficiência neuro-mental. O potencial reparo intra-útero foi de 42%. CONCLUSÃO: MM está associada a graves e frequentes sequelas. Quase um terço dos nossos casos poderiam ter tido a cirurgia fetal como opção terapêutica. INTRODUCTION: Prenatal diagnosis of myelomeningocele (MM) allows planning its management and, recently, a possible in utero repair. OBJECTIVE: To describe the perinatal outcome of fetuses with MM, in a Fetal Medicine Unit, identifying possible candidates for the in utero surgical repair. METHODO: Retrospective and descriptive study of 58 cases of prenatally diagnosed MM, at CAISM-UNICAMP, from January 1997 to December 2001, identifying possible fetal candidates for in utero repair. RESULTS: the diagnosis mean gestacional age was 29 weeks (17-39); level of lesions was above sacral region in 85%, association with hydrocephaly in 86%. Surgical complications were present in 39% of the neonates. During follow-up, 98% presented neurogenic bladder and 60% neurological/mental handicap. Twenty eight fetuses (42%) could have indication of in utero repair. CONCLUSION: MM is associated with severe and frequent poor results. Almost one third of our cases could had fetal repair as a treatment choice.

OBJETIVO: Relatar a experiência inicial do Centro Infantil Boldrini com a esplenectomia laparoscópica (EL) em crianças e adultos jovens. MÉTODO: Foram revisados os prontuários de 40 pacientes (mediana da idade de 6,6 anos; 1 a 22,8) submetidos à EL entre Julho de 2000 e Maio de 2002. As principais indicações de acordo com a doença de base foram: doença falciforme (DF) em 20 pacientes (50%), esferocitose hereditária em 10 (25 %), púrpura trombocitopência idiopática em oito (20 %), doença de Hodgkin em um e anemia hemolítica a esclarecer em um. RESULTADOS: Trinta e oito esplenectomias foram completadas por via laparoscópica (duas conversões) e em doze foi realizada adicionalmente a colecistectomia. A mediana do tempo operatório foi de 127,5 minutos (90-240 min) e sete (17,5 %) baços acessórios foram encontrados. Sangramento intra-operatório foi significativo apenas nas duas conversões, mas não houve necessidade de transfusões. A mediana do peso dos baços foi de 250 g (106-1000; n=36). Complicações pós-operatórias ocorreram em sete (17,5 %) pacientes e, nos portadores de DF, 35% desenvolveram síndrome torácica aguda. A mediana da permanência hospitalar pós-operatória foi de dois dias (2 - 14). O seguimento variou de 23 dias a dois anos (mediana de 11 meses). CONCLUSÕES: A EL pode ser realizada de modo seguro mesmo em baços de grande tamanho e é opção atrativa que pode substituir o procedimento aberto. Em pacientes com DF, a taxa de complicações permanece alta, sugerindo mecanismos outros que vão além da escolha da via de acesso cirúrgica.

OBJECTIVE: Evaluate the results from the first 5 years of experience with laparoscopy for diagnosis and treatment of nonpalpable testes. MATERIALS AND METHODS: Medical records of 51 patients submitted to laparoscopic testicular exploration, during a 5-year period, were retrospectively analyzed. Patients' mean age was 65.7 months (median = 48) on the first procedure. The youngest patient was 10 months and the oldest was 14 years old on the first surgery. Twenty-four (47%) patients presented nonpalpable testes bilaterally, 7 (14%) only at the right side and 20 (39%) at the left, totaling 75 testicular units assessed. Patients who had their testes palpated after anesthetic induction were excluded from the study, and in all other cases, surgical management was based on the testicular position and viability. During the post-operative follow-up, surgical success was classified as palpable testis in scrotal sac, with adequate consistency and volume. RESULTS: Nine (12%) testes were not localized, but their vessels and deferent duct were atrophic. Two (3%) testes were intra-abdominal and atrophic, and 2 (3%) gonads, in the same patient, had a dysmorphic aspect. Nineteen (25%) testicular units were located close to the internal inguinal ring (peeping testes) and, in 22 (29%) units, the spermatic vessels and deferent duct penetrated the internal inguinal ring. Eight (10%) testes were located at a distance of less than 2 cm from the internal inguinal ring and 13 (17%) at a distance greater than 2 cm. The 2 intra-abdominal atrophic testes were removed. Inguinotomy was performed in a total of 41 (54%) cases, reaching a surgical success of 89%. Laparoscopic orchiopexy in one stage, without vascular ligation, was performed in 9 (12%) testes, which presented a distance of less than 2 cm from the internal inguinal ring, also with a surgical success index of 89%. Orchiopexy in 2 stages, with ligation of the spermatic vessels, was performed in 13 (17%) testicular units located at a distance greater than 2 cm from the internal inguinal ring, reaching 77% of good results. CONCLUSION: Videolaparoscopy is a safe and effective method for diagnosis and treatment of nonpalpable testis.

Purpose: Extended lymphadenectomy in bladder and prostate cancer represents an important step of the surgical treatment of these tumors. Some urological referral centers have been using the laparoscopic approach for these cases. The aim of this video is to demonstrate the surgical technique of laparoscopic extended lymphadenectomy for bladder and prostate cancer. Case 1: Patient with transitional cell carcinoma of the bladder invading the muscular layer underwent laparoscopic extended lymphadenectomy up to the aortic bifurcation. Total operative time was 100 minutes and intraoperative bleeding was 100cc. Thirteen nodes were retrieved with no neoplasic involvement. Case 2: Patient with prostate cancer clinical stage T2 was submitted to laparoscopic extended lymphadenectomy with dissection of the internal iliac artery and its branches. Operative time was 55 minutes and intraoperative bleeding was 100cc. Eleven nodes were retrieved with no neoplasic involvement. Conclusion: Extended lympadenectomy in bladder and prostate cancer is a challenging procedure that can be performed by using the laparoscopic approach following the oncologic concepts. Int Braz J Urol. 2010; 36 (Video #8): 648_9 Available at: www.brazjurol.com.br/videos/september_october_2010/Branco_648_649video.htm Correspondence address: Dr. Anibal W. Branco Cruz Vermelha Hospital Curitiba, Parana, Brazil E-mail: anibal@awbranco.com.br doi: 10.1590/S1677-55382010000500030

O presente estudo tem como objetivo analisar os métodos utilizados para o tratamento das complicações relacionadas ao cateter de diálise peritoneal , avaliando-se o uso das vias aberta e laparoscópica. Vinte pacientes apresentaram 35 complicações relacionadas ao cateter de diálise (obstrução 60%, peritonite 34,3% e infecção do trajeto 5,7%) e foram tratados cirurgicamente. Para o tratamento das complicações, foram realizados 22 procedimentos por via aberta (laparotomia) e 13 por via laparoscópica. Através do método laparoscópico, foi possível desobstruir e manter o cateter em 80% dos cateteres obstruídos, ao passo que 100% foram trocados ou retirados quando a via aberta foi utilizada. O tempo necessário para reutilização completa do mesmo após o procedimento foi maior pelo método aberto. Os dados apresentados sugerem que a laparoscopia é um método eficaz como opção terapêutica para o tratamento das complicações relacionadas ao cateter de diálise peritoneal, pela visualização direta da cavidade peritoneal e possibilidade de reposicionamento ou desobstrução do mesmo, não sendo portanto necessária a sua troca e diminuindo também o tempo necessário para reutilização completa do cateter. Peritoneal dialysis is a good and safe method to handle end-stage renal diseases while waiting arteriovenous fistula for hemodalysis or a renal transplantation. However, there are some catheter related complications such as obstruction and peritonitis. Those complications are usually treated by laparotomy, but with the increasing use of the minimally invasive surgery, laparoscopy became a therapeutic option. The aim of the present study is to evaluate the open vs. the laparoscopic approach in the management of the related catheter complications of peritoneal dialysis. Twenty patients developed 35 catheter related events treated surgically (obstruction 60%, peritonitis 34,3% and tunnel infection in 5,7%). The open procedure was used 22 times and the laparoscopic 13. Through laparoscopy, it was possible to remove the obstruction and keep the catheter in 80%, while when the open approach was used 100% had to be replaced or removed. Peritoneal dyalysis was restarted later in the open method. Our data suggest that laparoscopy is an effective and safe method for the management of the catheter related complications of peritoneal dialysis, allowing direct visualization and manipulation of the peritoneal cavity and decreasing of the time span until resuming full utilization of the cateter.

Although conservative treatment of multicystic dysplastic kidney is generally accepted, there are reports of complications when such a kidney is retained, particularly malignant degeneration.1.2 We report on a neonate with Wilms tumor in a prenatally diagnosed multicystic dysplastic kidney. To our knowledge this is the fifth case of Wilms tumor arising in a multicystic dysplastic kidney but only the second diagnosed prenatally.

SUMMARY A gonadal tumor was diagnosed in the first months of life in a patient with genital ambiguity, a 45,X/46,XY karyotype, and mixed gonadal dysgenesis. Gonadal biopsies at the age of 3 months revealed dysgenetic testes and a gonadoblastoma on the right testis. Even though gonadal tumors are rare in childhood, this case indicates that prophylactic removal of dysgenetic gonads should be performed as early as possible, especially when the female sex is assigned to a patient with a Y-chromosome sequence.

Deficiency of the enzyme P450 oxidoreductase is a rare form of congenital adrenal hyperplasia with characteristics of combined and partial impairments in steroidogenic enzyme activities, as P450 oxidoreductase transfers electrons to CYP21A2, CYP17A1, and CYP19A1. It results in disorders of sex development and skeletal malformations similar to Antley-Bixley syndrome. We report the case of a 9-year-old girl who was born with virilized genitalia (Prader stage V), absence of palpable gonads, 46,XX karyotype, and hypergonadotropic hypogonadism. During the first year of life, ovarian cyst, partial adrenal insufficiency, and osteoarticular changes, such as mild craniosynostosis, carpal and tarsal synostosis, and limited forearm pronosupination were observed. Her mother presented severe virilization during pregnancy. The molecular analysis of P450 oxidoreductase gene revealed compound heterozygosis for the nonsense p.Arg223*, and the novel missense p.Met408Lys, inherited from the father and the mother, respectively. Arq Bras Endocrinol Metab. 2012;56(8):578-85 A deficiência da enzima P450 oxidorredutase é uma forma rara de hiperplasia congênita da adrenal com características de inibição combinada e parcial de enzimas esteroidogênicas, pois a enzima P450 oxidorredutase participa da transferência de elétrons para as enzimas CYP21A2, CYP17A1 e CYP19A1. Essa deficiência causa um distúrbio do desenvolvimento do sexo e alterações esqueléticas semelhantes às da síndrome de Antley-Bixley. Relatamos o caso de uma menina, atualmente com 9 anos de idade, que apresentava ao nascimento genitais virilizados (Prader 5) sem gônadas palpáveis, com cariótipo 46,XX e hipogonadismo hipergonadotrófico. No primeiro ano de vida, foram observados cisto ovariano, insuficiência adrenal parcial e alterações osteoarticulares como leve craniossinostose, sinostose carpal e tarsal e limitação de pronossupinação dos membros superiores. Sua mãe apresentou intensa virilização durante a gestação. O estudo molecular do gene P450 oxidorredutase revelou a heterozigose composta das mutações nonsense p.Arg223* e da missense nova p.Met408Lys, herdadas do pai e da mãe, respectivamente. Arq Bras Endocrinol Metab. 2012;56(8):578-85