Predictors of surgical complications in boys with hypospadias: data from an internationa registry.

Background: Complications are frequently reported after hypospadias repair and there is a need to understand the factors that influence their occurrence. Methods: Data from boys with hypospadias born between 2000 and 2020 were obtained from the International Disorders of Sex Development (I-DSD) Registry. Logistic regressions, fisher's exact tests and spearman's correlation tests were performed on the data to assess associations between clinical factors and complication rates. Results: Of the 551 eligible boys, data were available on 160 (29%). Within the cohort, the median (range) External Masculinization Score (EMS) was 6 (2, 9). All presented with one or more additional genital malformation and 61 (38%) presented with additional extragenital malformations. Disorders of androgen action, androgen synthesis and gonadal development were diagnosed in 28 (18%), 22 (14%) and 9 (6%) boys, respectively. The remaining 101 (62%) patients were diagnosed as having non-specific 46,XY Disorders of Sex Development. Eighty (50%) boys had evidence of abnormal biochemistry, and gene variants were identified in 42 (26%). Median age at first hypospadias surgery was 2 years (0, 9), and median length of follow-up was 5 years (0, 17). Postsurgical complications were noted in 102 (64%) boys. There were no significant associations with postsurgical complications. Conclusions: Boys with proximal hypospadias in the I-DSD Registry have high rates of additional comorbidities and a high risk of postoperative complications. No clinical factors were significantly associated with complication rates. High complication rates with no observable cause suggest the involvement of other factors which need investigation.

Evaluation of the regional distribution of the pediatric surgery workforce and surgical load in Brazil.

Objective: The purpose of this study is to examine the regional distribution of the pediatric surgery workforce and the expected local demand for pediatric surgical procedures in Brazil. Methods: We collected data on the pediatric surgical workforce, surgical volume, Gross Domestic Product per capita, and mortality for gastrointestinal tract malformations (MGITM) across the different regions of Brazil for 2019. Results: Data from the Federal Medical Council reported 1515 pediatric surgery registries in Brazil, corresponding to 1414 pediatric surgeons (some pediatric surgeons are registered in more than one state), or 2.4 pediatric surgeons per 100 000 children 14 years of age and younger. There were 828 men and 586 women. The mean age was 51.5±12.8 years, and the mean time from graduation was 3.4±5.7 years. There is a higher concentration of pediatric surgeons in the wealthier Central-West, South, and Southeast regions. Individual surgical volume ranged from 88 to 245 operations/year (average 146 operations/year) depending on the region. Of these, only nine (6.1%) were high-complexity (including neonatal) operations. MGITM tended to be higher in the poorer North and Northeast regions than in other regions of Brazil. Conclusions: Our findings suggest significant disparities in the surgical workforce and workload across Brazil related to socioeconomic status. Regions with an increased surgical workforce were associated with lower MGITM. The average number of complex operations performed annually by each pediatric surgeon was considerably low. Strategic investment and well-defined health policies are imperative to enhance the quality of surgical care in the different regions of Brazil. Level of evidence: Retrospective review; level IV.

Analysis of the efficacy and safety of conservative treatment of blunt abdominal trauma in children: retrospective study. Conservative treatment of blunt abdominal trauma in children.

INTRODUCTION: in Brazil, trauma is responsible for 40% of deaths in the age group between 5 and 9 years old, and 18% between 1 and 4 years, and bleeding is the leading cause of preventable death in the traumatized child. Conservative management of blunt abdominal trauma with solid organs injury - started in the 60s - is the current world trend, with studies showing survival rates above 90%. The objective was to assess the efficacy and safety of conservative treatment in children with blunt abdominal trauma treated at the Clinical Hospital of the University of Campinas, in the last five years. METHODS: retrospective analysis of medical records of patients classified by levels of injury severity, in 27 children. RESULTS: only one child underwent surgery for initial failure of conservative treatment (persistent hemodynamic instability), resulting in a 96% overall success rate of the conservative treatment. Five other children (22%) developed late complications that required elective surgery: a bladder injury, two cases of infected perirenal collections (secondary to injury of renal collecting system), a pancreatic pseudocyst and a splenic cyst. Resolution of the complications was attained in all children, with anatomical and functional preservation of the affected organ. There were no deaths in this series. CONCLUSION: the conservative initial approach in the treatment of blunt abdominal trauma was effective and safe with high resolution and low rate of complications leading to a high preservation rate of the affected organs. Level of evidence III - prognostic and therapeutic study.

BISHOP-KOOP OSTOMY REVISITED: A "TEST-DRIVE" INTESTINAL DIVERSION FOR CHILDREN WITH SUSPECTED BOWEL DYSMOTILITY.

BACKGROUND: Bishop-Koop ileostomy has been widely used in pediatric patients with the intention of including as much bowel as possible in the intestinal transit early in the management of children with meconium ileus and intestinal atresia. In recent years, we have been using it as an alternative to test the distal bowel function before closure of a previously constructed ostomy in selected children with questionable distal bowel motility. AIMS: The aim of this study was to present our experience with this alternative use of the Bishop-Koop ostomy. METHODS: This is a cross-sectional retrospective review of hospital records, combined with a comprehensive literature review. RESULTS: Seven children were included: five had suspected aganglionosis, one had gastroschisis complicated with ileal atresia, and one had a colonic stricture secondary to necrotizing enterocolitis. In this short series of patients, motility of the distal bowel was correctly assessed in six patients and partially correctly assessed in one patient. One patient did not pass stools per anus after the Bishop-Koop, and he was later confirmed to have Hirschsprung disease. Four patients resumed normal evacuation pattern after closure of the Bishop-Koop. One patient had a Bishop-Koop colostomy because of recurrent enterocolitis after a transanal pull-through. Although he evacuated normally while having the colostomy, the diarrhea recurred after the ostomy was closed. An additional patient, with a severe behavioral problem, did not evacuate per anus after her colostomy was transformed in a Bishop-Koop-type ostomy, despite the apparent presence of normal ganglia in the bowel wall. CONCLUSIONS: Data from the present series allow us to affirm that Bishop-Koop-type ostomy is a safe and efficient procedure that can be used to assess distal bowel function before a definitive transit reconstruction, in children with uncertain motility issues.

Analysis of the efficacy and safety of conservative treatment of blunt abdominal trauma in children: retrospective study. Conservative treatment of blunt abdominal trauma in children / Análise de eficácia e segurança do tratamento conservador do trauma abdominal contuso em crianças: estudo retrospectivo. Tratamento conservador de trauma abdominal contuso em crianças

ABSTRACT Introduction: in Brazil, trauma is responsible for 40% of deaths in the age group between 5 and 9 years old, and 18% between 1 and 4 years, and bleeding is the leading cause of preventable death in the traumatized child. Conservative management of blunt abdominal trauma with solid organs injury - started in the 60s - is the current world trend, with studies showing survival rates above 90%. The objective was to assess the efficacy and safety of conservative treatment in children with blunt abdominal trauma treated at the Clinical Hospital of the University of Campinas, in the last five years. Methods: retrospective analysis of medical records of patients classified by levels of injury severity, in 27 children. Results: only one child underwent surgery for initial failure of conservative treatment (persistent hemodynamic instability), resulting in a 96% overall success rate of the conservative treatment. Five other children (22%) developed late complications that required elective surgery: a bladder injury, two cases of infected perirenal collections (secondary to injury of renal collecting system), a pancreatic pseudocyst and a splenic cyst. Resolution of the complications was attained in all children, with anatomical and functional preservation of the affected organ. There were no deaths in this series. Conclusion: the conservative initial approach in the treatment of blunt abdominal trauma was effective and safe with high resolution and low rate of complications leading to a high preservation rate of the affected organs. Level of evidence III - prognostic and therapeutic study.

RESUMO Introdução: no Brasil, o trauma é responsável por 40% dos óbitos na faixa etária entre 5 e 9 anos, e 18% entre 1 e 4 anos, e o sangramento é a principal causa de prevenção morte na criança traumatizada. O manejo conservador de trauma abdominal contuso com lesão de órgãos sólidos - iniciado na década de 60 - é a tendência mundial atual, com estudos mostrando taxas de sobrevivência acima de 90%. O objetivo do presente trabalho foi avaliar a eficácia e segurança do tratamento conservador em crianças com trauma abdominal contuso tratado no Hospital das Clínicas da Universidade de Campinas, nos últimos cinco anos. Métodos: análise retrospectiva de prontuários de pacientes classificados por níveis de gravidade da lesão, em 27 crianças. Resultados: apenas uma criança foi submetida a cirurgia por falha inicial do tratamento conservador (instabilidade hemodinâmica persistente), resultando em uma taxa de sucesso global de 96% do tratamento conservador inicial. Outras cinco crianças (22%) desenvolveram complicações tardias que exigiram cirurgias eletivas: lesão na bexiga, dois casos de coleção perirenal infectada (secundária à lesão de sistema de coleta renal), um pseudocisto pancreático e um cisto esplênico. Resolução da complicação foi atingida em todas as crianças, com preservação anatômica e funcional do órgão afetado. Não houve mortes nesta série. Conclusão: a abordagem inicial conservadora no tratamento de trauma abdominal contundente foi eficaz e segura com alta resolução e baixa taxa de complicações levando a uma alta taxa de preservação dos órgãos afetados. Nível de evidência III - estudo prognóstico e terapêutico.

Derivação intestinal à bishop-koop - um novo uso para uma velha técnica / Bishop-koop ostomy revisited: a "test-drive" intestinal diversion for children with suspected bowel dysmotility

ABSTRACT BACKGROUND: Bishop-Koop ileostomy has been widely used in pediatric patients with the intention of including as much bowel as possible in the intestinal transit early in the management of children with meconium ileus and intestinal atresia. In recent years, we have been using it as an alternative to test the distal bowel function before closure of a previously constructed ostomy in selected children with questionable distal bowel motility. AIMS: The aim of this study was to present our experience with this alternative use of the Bishop-Koop ostomy. METHODS: This is a cross-sectional retrospective review of hospital records, combined with a comprehensive literature review. RESULTS: Seven children were included: five had suspected aganglionosis, one had gastroschisis complicated with ileal atresia, and one had a colonic stricture secondary to necrotizing enterocolitis. In this short series of patients, motility of the distal bowel was correctly assessed in six patients and partially correctly assessed in one patient. One patient did not pass stools per anus after the Bishop-Koop, and he was later confirmed to have Hirschsprung disease. Four patients resumed normal evacuation pattern after closure of the Bishop-Koop. One patient had a Bishop-Koop colostomy because of recurrent enterocolitis after a transanal pull-through. Although he evacuated normally while having the colostomy, the diarrhea recurred after the ostomy was closed. An additional patient, with a severe behavioral problem, did not evacuate per anus after her colostomy was transformed in a Bishop-Koop-type ostomy, despite the apparent presence of normal ganglia in the bowel wall. CONCLUSIONS: Data from the present series allow us to affirm that Bishop-Koop-type ostomy is a safe and efficient procedure that can be used to assess distal bowel function before a definitive transit reconstruction, in children with uncertain motility issues.

RESUMO RACIONAL: A ileostomia Bishop-Koop foi amplamente utilizada em pacientes pediátricos com a intenção de incluir o máximo de intestino possível no trânsito intestinal no manejo inicial de recém-nascidos com íleo meconial e atresia intestinal. Nos últimos anos, temos usado-a como alternativa para testar a função intestinal distal antes do fechamento de uma ostomia, em algumas crianças com motilidade intestinal distal questionável. OBJETIVOS: Apresentar nossa experiência com este uso alternativo da ostomia Bishop-Koop. MÉTODOS: Revisão retrospectiva dos registros hospitalares, combinada com uma revisão abrangente da literatura. RESULTADOS: Sete crianças foram incluídas: cinco tinham suspeita de aganglionose, uma tinha gastrosquise complicada com atresia ileal e uma tinha estenose de colon secundária à NEC. Nesta pequena série de pacientes, a motilidade do intestino distal foi corretamente avaliada em 6 pacientes e parcialmente avaliada em um. Um paciente não evacuou por ânus após o Bishop-Koop e mais tarde foi confirmado que ele tinha doença de Hirschsprung. Seis pacientes retomaram o padrão normal de evacuação após o fechamento do Bishop-Koop. Um paciente que fez uma colostomia Bishop-Koop por causa de enterocolite recorrente após um abaixamento transanal, recidivou a enterocolite após o fechamento definitivo. CONCLUSÕES: A ostomia tipo Bishop-Koop é um procedimento seguro e eficaz que pode ser utilizado para avaliar a função intestinal distal antes de uma reconstrução definitiva do trânsito em crianças com problemas de motilidade intestinal.

Effectiveness of an inexpensive short-term theoretical-practical course on videosurgery for surgeons in training.

BACKGROUND: 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: Thirty-six 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.

Scintigraphic Evaluation of the Impact of Pneumoperitoneum on Renal Blood Flow: A Rabbit Model.

Background: Increased intra-abdominal pressure resulting from pneumoperitoneum can cause renal physiological changes, such as oliguria and anuria, in mammals. Although videolaparoscopic operations are common, the occurrence of renal lesions due to these procedures has not been precisely documented in the literature. The aim of this study was to evaluate the impact of pneumoperitoneum on renal blood flow using renal scintigraphy in a rabbit model. Methods: Six New Zealand male rabbits weighing 3 kg, previously anesthetized, were mechanically ventilated and underwent pneumoperitoneum. Each animal served as its own control and was analyzed in two different moments: [99mTc] diethylenetriaminepentaacetic acid (DTPA) renal blood flow evaluation in baseline conditions (T0) and 30 minutes after installation of 15 mmHg-pneumoperitoneum (T1). The animals were monitored throughout the study by capnography, oximetry, and arterial pressure median, and were euthanized at the end of the experiment. Results: The quantitative analysis of the scintigraphic images of renal uptake of the radiopharmaceutical evidence reduced renal arterial blood flow during pneumoperitoneum. Compared with baseline conditions, all animals presented a reduction of renal blood flow varying from 16% to 82%, with mean [±standard deviation] of 53% [±24%]. Conclusions: Pneumoperitoneum induces a significant reduction of the renal blood flow, as determined in this experimental method in rabbits and dynamic renal scintigraphy with [99mTc] DTPA is an adequate method to investigate this event in the experimental setting.

Early development of a gonadal tumor in a patient with mixed gonadal dysgenesis

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.

Early development of a gonadal tumor in a patient with mixed gonadal dysgenesis.

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.

408 Cases of Genital Ambiguity Followed by Single Multidisciplinary Team during 23 Years: Etiologic Diagnosis and Sex of Rearing.

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.

Lipomatous nevus and urethral caruncle mistaken for ambiguous genitalia in a female infant.

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.

Laparoscopic Transposition of Lower Pole Crossing Vessels (Vascular Hitch) in Children with Pelviureteric Junction Obstruction: How to Be Sure of the Success of the Procedure?

BACKGROUND: 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. PATIENTS AND METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Ovotesticular disorder of sex development with unusual karyotype: patient report.

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.

46,XX DSD and Antley-Bixler syndrome due to novel mutations in the cytochrome P450 oxidoreductase gene / DDS 46,XX e síndrome de Antley-Bixler causada por novas mutações no gene da enzima P450 oxidorredutase

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.

Antegrade pressure measurement of urinary tract in children with persistent hydronephrosis.

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.

Antegrade pressure measurement of urinary tract in children with persistent hydronephrosis

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.

Kidney safety during surgical pneumoperitoneum: an experimental study in rats.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

46,XX DSD and Antley-Bixler syndrome due to novel mutations in the cytochrome P450 oxidoreductase gene.

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.

Monitoring intravesical pressure during gastroschisis closure. Does it help to decide between delayed primary or staged closure?

INTRODUCTION AND OBJECTIVE: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.