RESUMEN
PURPOSE: Duplication enucleation (DE) has been described as an alternative to intestinal resection with primary anastomosis (IRA) for intestinal duplications, but no comparative study exists. The aim of this study was to compare both surgical procedures for intestinal duplication. METHODS: A retrospective study was performed, including all children treated for intestinal duplication (2005-2023). Patients that underwent DE were compared to those that underwent IRA. Statistical significance was determined using p < 0.05. Ethical approval was obtained. RESULTS: A total of 51 patients (median age: 5 months) were treated for intestinal duplication, including 27 patients (53%) that underwent DE and 24 IRA (47%). A cystic image was detected prenatally in 19 patients (70%) with DE and 11 patients (46%) with IRA (p = 0.09). Enucleation was performed using laparoscopy in 7 patients (14%). Patients that underwent DE had shorter time to first feed (1 vs 3 days, p = 0.0001) and length of stay (4 vs 6 days, p < 0.0004) compared to IRA. A muscular layer was identified in 68% of intestinal resection specimens. CONCLUSION: Compared to intestinal resection with anastomosis, duplication enucleation is associated with decreased postoperative length of stay and delay to first feeds without increasing post-operative complications. Regarding histological analysis, enucleation seems feasible in most cases.
Asunto(s)
Anastomosis Quirúrgica , Intestinos , Laparoscopía , Humanos , Estudios Retrospectivos , Anastomosis Quirúrgica/métodos , Femenino , Masculino , Lactante , Intestinos/cirugía , Intestinos/anomalías , Laparoscopía/métodos , Preescolar , Resultado del Tratamiento , Tiempo de Internación/estadística & datos numéricos , Recién Nacido , Procedimientos Quirúrgicos del Sistema Digestivo/métodos , NiñoRESUMEN
BACKGROUND: Among patients with nephroblastoma, those with bilateral disease are a unique population where maximising tumour control must be balanced with preserving renal parenchyma. METHODS: The SIOP 2001 protocol recommended surgery after neoadjuvant cycle(s) of Dactinomycin and Vincristine (AV) with response-adapted intensification, if needed. Adjuvant treatment was given based on the lesion with the worst histology. RESULTS: Three hundred and twenty seven patients with stage V disease were evaluable: 174 had bilateral Wilms tumour (BWT), 101 unilateral WT and contralateral nephroblastomatosis (NB) and 52 bilateral nephroblastomatosis. In these three groups, the estimated 5y-EFS was 76.1%, 84.6%, and 74.9%, respectively. AV chemotherapy alone was the successful chemotherapy for 58.7% of all the patients and 65.6% of the non-metastatic patients. Among the 174 patients with BWT, 149 (88.2%) had at least one nephron-sparing surgery. Twenty of 61 bilateral stage I patients were treated with four-week AV postoperatively achieving 94.4% 5y-EFS. At last follow-up, 87% of patients had normal renal function. CONCLUSIONS: This study demonstrates that AV without anthracyclines is sufficient to achieve NSS and good survival in the majority of patients. For patients with bilateral stage I WT and intermediate risk histology, only four weeks adjuvant AV seems to be sufficient. CLINICAL TRIAL REGISTRATION: NCT00047138.
RESUMEN
The surgical decision to attempt nephron-sparing surgery (NSS) in children with renal tumors can be difficult. In adults, nephrometric tools are used for decision-making. More than 90% of low-complexity tumors are eligible for NSS, and high-complexity tumors often require total nephrectomy. We retrospectively applied those nephrometric tools [Radius, Exophytic, Nearness to the sinus or collecting system, Anterior/posterior, Location relative to polar lines (RENAL), Preoperative Aspects and Dimensions Used for an Anatomical classification (PADUA), and Renal Tumor Invasion Index (RTII) scoring systems] to the preoperative imaging of children operated for renal tumors in our institution from 2015 to 2019 and correlated them with the type of surgery. The scores were assessed by 2 independent surgeons and 1 radiologist. Forty-four tumors were removed, including 16 NSS, 38 after neo-adjuvant chemotherapy, and 6 upfront surgeries, in 30 children. More than 50% of patients in the low and medium-risk population for RENAL, PADUA, and RTII scores, and ~15% in the high-complexity categories underwent NSS. Tumors removed through NSS were significantly less complex according to each score. Interobserver reliability was good for 3 scores. The application of the RENAL, PADUA, and RTII was able to accurately classify most of the pediatric tumors, according to their complexity. These scores could help increase the indications of NSS in renal tumor surgery.
Asunto(s)
Carcinoma de Células Renales , Neoplasias Renales , Adulto , Niño , Humanos , Estudios Retrospectivos , Reproducibilidad de los Resultados , Nefrectomía/métodos , Neoplasias Renales/cirugía , Neoplasias Renales/patología , Nefronas/cirugía , Nefronas/patología , Carcinoma de Células Renales/patologíaRESUMEN
Pre- and post-pubertal testicular tumors are two distinct entities in terms of epidemiology, diagnosis and treatment. Most pre-pubertal tumors are benign; the most frequent are teratomas, and the most common malignant tumors are yolk-sac tumors. Post-pubertal tumors are similar to those found in adults and are more likely to be malignant. Imaging plays a pivotal role in the diagnosis, staging and follow-up. The appearance on ultrasonography (US) is especially helpful to differentiate benign lesions that could be candidates for testis-sparing surgery from malignant ones that require radical orchidectomy. Some specific imaging patterns are described for benign lesions: epidermoid cysts, mature cystic teratomas and Leydig-cell tumors. Benign tumors tend to be well-circumscribed, with decreased Doppler flow on US, but malignancy should be suspected when US shows an inhomogeneous, not-well-described lesion with internal blood flow. Imaging features should always be interpreted in combination with clinical and biological data including serum levels of tumor markers and even intra-operative frozen sections in case of conservative surgery to raise any concerns of malignity. This review provides an overview of imaging features of the most frequent testicular and para-testicular tumor types in children and the value of imaging in disease staging and monitoring children with testicular tumors or risk factors for testicular tumors.
RESUMEN
BACKGROUND: Outcome of children with bladder-prostate rhabdomyosarcoma (RMS) has improved with multimodal therapies, including surgery and/or radiotherapy for local treatment. Our aim was to report long-term urological complications after a conservative approach combining conservative surgery and brachytherapy. PATIENTS AND METHODS: Eighty-six patients, free of disease, were retrospectively reviewed. Symptoms related to urinary tract obstruction, incontinence, infection, and lithiasis were reported and graded according to the Common Terminology Criteria for Adverse Events (CTCAE) classification. Only symptomatic patients underwent urodynamic studies. Risk factors for complications were analyzed. RESULTS: There were 76 males and 10 females. The median follow-up was 6.3 years (18 months to 24 years). Complications occurred after a median follow-up of 5 years (0-21). Twenty-two patients (26%) had long-term urological complications. Urinary tract obstruction was found in 15 patients (17%) and urinary incontinence in 14 patients (16%). Recurrent urinary tract infection occurred in four patients and urinary lithiasis in four (5%). The underlying physiopathology included bladder dysfunction in 15 patients (17%), urethral stenosis in six (7%), and ureterovesical junction stenosis in five (6%). On univariate analysis, posterior bladder wall dissection (p = .001), bladder neck trigone dissection (p = .010), and partial prostatectomy (p = .023) were significantly associated with an increased risk of bladder dysfunction; on multivariate analysis, only age ≤2 years (p = .028) at operation and posterior bladder wall dissection (p = .006) were found to be significant. CONCLUSION: The conservative surgical approach combined with brachytherapy for bladder-prostate RMS leads to long-term urological complications in 26% of survivors. Optimizing brachytherapy doses for young children and establishing a clear and long-term follow-up protocol could help to reduce these complications.
Asunto(s)
Braquiterapia , Neoplasias Pélvicas , Neoplasias de la Próstata , Rabdomiosarcoma , Neoplasias de la Vejiga Urinaria , Braquiterapia/efectos adversos , Braquiterapia/métodos , Niño , Preescolar , Humanos , Masculino , Próstata/patología , Neoplasias de la Próstata/tratamiento farmacológico , Estudios Retrospectivos , Rabdomiosarcoma/radioterapia , Rabdomiosarcoma/cirugía , Vejiga Urinaria/patología , Neoplasias de la Vejiga Urinaria/radioterapia , Neoplasias de la Vejiga Urinaria/cirugíaRESUMEN
INTRODUCTION: Necrotizing Enterocolitis (NEC) remained a dramatic complication leading to death or neonatal morbidities in preterms. For some, Intra-Abdominal Hypertension (IAH) and Abdominal Compartment Syndrome worsened the multi-organ failure. An open abdomen surgery could be an alternative to conventional surgical treatment to move beyond this stage. OBJECTIVES: To retrospectively describe the clinical course, pre- and post-operative features of preterms suffering from severe NEC with IAH treated by open abdomen surgery and referred to our center from October 2007 to September 2019. Our secondary objective is to identify various risk factors for mortality in this population. METHODS: Data on neonatal, clinical, biological, pre and post-operative features and outcome were collected. Univariate analyses were performed to compare their pre and post-operative features stratifying on outcome. RESULTS: Among 29 included patients, 14 (48%) survived to discharge without short bowel syndrome. Death was associated with an earlier postnatal age at NEC (16.3 ± 9.1 versus 31.3 ± 25.9 days; p = 0.004) and followed a withdrawal of treatment in 60% of cases. Surgery was associated with a significant improvement of respiratory and hemodynamic features (decrease of mean ventilator pressure from 13.1 ± 5.4 to 11.3 ± 4.0 cmH2O, p < 0.001), oxygen requirement (mean FiO2 decreased from 65.0% ± 31.2 to 49.0% ± 24.6, p < 0.001) and inotropic score (from 38.6 ± 70.1 to 29.9 ± 64.3, p < 0.001). In the survival group, pre and post-operative findings exhibited a significant increase of serum lactate concentrations from 2.7 ± 1.6 to 11.0 ± 20.3 mmol/L (p = 0.02) but a similar pH. CONCLUSION: Open abdomen surgery could be considered to rescue preterms with near fatal NEC. IAH and Abdominal Compartment Syndrome in these preterms should be investigated through further studies. LEVEL OF EVIDENCE: Level III.
Asunto(s)
Enterocolitis Necrotizante , Enfermedades del Recién Nacido , Hipertensión Intraabdominal , Abdomen , Estudios de Factibilidad , Humanos , Lactante , Recién Nacido , Recien Nacido Prematuro , Hipertensión Intraabdominal/etiología , Hipertensión Intraabdominal/cirugía , Estudios RetrospectivosRESUMEN
Botryoid growth pattern (BGP) is a polypoid mass growing into the renal pelvis, rarely seen in bilateral Wilms tumors where it represents a surgical challenge. We report our experience of nephron sparing surgery in 3 patients with BGP in bilateral Wilms tumor. Surgical en bloc removal was performed after calyx opening with no complications. The histology of the BGP was Intralobar Nephrogenic Rest in all cases while all Wilms tumors were of intermediate risk. One patient early recurred. At a follow-up of 9 months, 22 and 23 years, all patients were alive with a moderate renal insufficiency and hypertension.
Asunto(s)
Neoplasias Renales , Tumor de Wilms , Femenino , Humanos , Neoplasias Renales/patología , Neoplasias Renales/cirugía , Masculino , Recurrencia Local de Neoplasia/cirugía , Nefrectomía , Nefronas/patología , Nefronas/cirugía , Estudios Retrospectivos , Tumor de Wilms/patología , Tumor de Wilms/cirugíaRESUMEN
RATIONALE: Three-dimensional reconstruction (3DR) of preoperative images may improve the presurgical assessment of tumours prior to removal. We aimed to analyse the advantages and discrepancies of preoperative 3DR in paediatric tumours. METHODS: We conducted a prospective observational study from 2016 to 2019, including patients with thoraco-abdominal tumours having predictable surgical risks on preoperative images (encasement of vessels posing vascular risks, ie, neuroblastic and soft tissue tumours or parenchyma preservation of the invaded organ, ie, liver and kidney). A comparison of 2D/3DR and surgical findings was performed. RESULTS: Twenty-four patients, with a median age at surgery of 68.2 months (13 days-203 months), were operated on for neuroblastoma (n = 7), renal tumour (n = 7), hepatic tumour (n = 4) and others (n = 6; bone sarcoma of the iliac branch, abdominal lymph nodes of a recurrent testicular germ cell tumour, pseudoinflammatory tumour of the omentum, thoracic lipoblastoma, desmoplastic tumour, solid and pseudopapillar tumour of the pancreas). Reconstruction was of poor quality in two patients with renal tumours because computed tomography (CT) had no excretory phase. Discrepancies between 3DR and surgical findings occurred in two patients, one because of poor assessment of caliceal infiltration by renal nodules and the other because of inadequate reconstruction of renal vein thrombosis. For all the other tumours, 3DR improved the visualisation and precise location of vessels during surgery. CONCLUSION: High-quality preoperative images are mandatory to provide the best 3DR. In the majority of cases, 3DR is of significant help during surgery to better identify vascular structures within tumours and preserve parenchyma.
Asunto(s)
Procesamiento de Imagen Asistido por Computador/métodos , Imagenología Tridimensional/métodos , Neoplasias/patología , Cuidados Preoperatorios , Tomografía Computarizada por Rayos X/métodos , Adolescente , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Masculino , Neoplasias/diagnóstico por imagen , Neoplasias/cirugía , Pronóstico , Estudios ProspectivosRESUMEN
AIM: Definitions of nephron-sparing surgery (NSS) procedures in Wilms tumor (WT) are not clear. The new UMBRELLA protocol offers a formula (NSS(X)-SRM(n)-PRM(n)-RRP(n%)) to better define the different NSS parameters. We aimed to assess the advantages and limits of this new formula. METHODS: This retrospective monocentric study included patients operated by NSS for WT from 1975 to 2018. We reviewed the medical records and applied the NSS formula to all included patients. RESULTS: Eighty kidneys were operated on 56 patients at a mean age of 19.2 months (4 days-7.5 years), with 49 partial nephrectomies and 31 tumorectomies. The assessment of the surgical resection margins (SRM) showed a doubt in six cases and one tumor breach. An intact pseudocapsule along the resection margin with no renal parenchyma was found in four cases at pathological resection margins (PRM) assessment, whereas a tumor breach was described in seven cases. Among the six patients with a surgical doubt, only one had a pathological stage III. There were no surgical doubts in the seven patients with tumor breach at pathology. At a mean follow-up of eight years (15 days-28.6 years), eight patients had elevated blood pressure levels. Ten had proteinuria. These two parameters were significantly increased in patients with a remaining renal parenchyma (RRP) of less than half of the initial total renal parenchyma. The serum creatinine level was normal for all except two patients. CONCLUSION: The new NSS formula described all the crucial elements of NSS. RRP seemed essential for the evaluation of long-term renal function.
Asunto(s)
Algoritmos , Neoplasias Renales/cirugía , Nefrectomía/métodos , Nefronas/cirugía , Tratamientos Conservadores del Órgano/métodos , Tumor de Wilms/cirugía , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Lactante , Neoplasias Renales/patología , Masculino , Márgenes de Escisión , Pronóstico , Estudios Retrospectivos , Tasa de Supervivencia , Tumor de Wilms/patologíaRESUMEN
The umbilical hernia is common in children. Most of the cases have a spontaneous regression around the age of 3 years. Complications are very rare, and thus surgery is not routinely indicated before the age of 3 years. We report an exceptional case of spontaneous rupture of an umbilical hernia with emphasis on the management of this rare complication and a literature review of similar cases.
Asunto(s)
Hernia Umbilical/complicaciones , Rotura Espontánea/complicaciones , Prolapso Visceral/etiología , Preescolar , Femenino , Herniorrafia , Humanos , Rotura Espontánea/cirugía , Prolapso Visceral/cirugíaRESUMEN
The radiological distinction of Wilms tumor (WT) nodules from nephrogenic rests (NR) in patients with multifocal unilateral WT or bilateral disease is challenging. The study aims to compare the radiology assessment of kidney nodules with their final histology in 48 patients. The final histology of 118 nodules corresponded to the initial radiological diagnosis while 40 (25%) nodules were misdiagnosed, 20 being initially diagnosed WT on imaging were proved to be NR at histology. The size of nodules at diagnosis might help to distinguish WT from NR before surgery. Homogeneity did not seem to be a key feature.
Asunto(s)
Neoplasias Renales/diagnóstico por imagen , Neoplasias Renales/patología , Riñón/diagnóstico por imagen , Riñón/patología , Tumor de Wilms/diagnóstico por imagen , Tumor de Wilms/patología , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Estudios RetrospectivosRESUMEN
BACKGROUND: Liver metastases are rare in children with Wilms tumor (WT), and their impact on the outcome is unclear. PATIENTS AND METHODS: The French cohort of patients with WT presenting liver metastases at diagnosis and enrolled in the International Society of Pediatric Oncology (SIOP) 2001 study was reviewed. RESULTS: From 2002 to 2012, 906 French patients were enrolled in the SIOP2001 trial. Among them, 131 (14%) presented with stage IV WT and 18 (1.9%) had liver metastases at diagnosis. Isolated liver metastases were displayed in four of them. After preoperative chemotherapy, persistent liver disease was reported in 14/18 patients, and 13 of them underwent metastasectomy after nephrectomy. In resected liver lesions, the same histology of the primary tumor was reported for three patients, blastemal cells without anaplasia were identified in one patient with DA-WT, and post-chemotherapy necrosis/fibrosis was identified for the other 10 patients. For the four patients who had liver and lung surgery, both sites had nonviable cells with post-chemotherapy necrosis/fibrosis. Six patients had hepatic radiotherapy. Sixteen patients achieved primary complete remission and were alive at the last follow-up (median follow-up: 6.4 years). The only two deceased patients presented diffuse anaplasia histology. The five-year EFS and OS were 83% (60%-94%) and 88% (66%-97%), respectively. CONCLUSION: Liver involvement does not appear to be an adverse prognostic factor in metastatic WT. The role of hepatic surgery and radiotherapy remains unclear, and should be carefully considered in case of persistent liver metastases, according to histology and radiological response to other metastatic sites.
Asunto(s)
Hepatectomía/mortalidad , Neoplasias Renales/mortalidad , Neoplasias Hepáticas/mortalidad , Metastasectomía/mortalidad , Nefrectomía/mortalidad , Tumor de Wilms/mortalidad , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Lactante , Neoplasias Renales/patología , Neoplasias Renales/cirugía , Neoplasias Hepáticas/secundario , Neoplasias Hepáticas/cirugía , Masculino , Estudios Prospectivos , Inducción de Remisión , Estudios Retrospectivos , Tasa de Supervivencia , Resultado del Tratamiento , Tumor de Wilms/patología , Tumor de Wilms/cirugíaRESUMEN
PURPOSE: Human chorionic gonadotropin stimulates fetal testosterone production and contributes to normal development of male genitalia. Using population based data we hypothesized that differences in maternal free beta human chorionic gonadotropin may be associated with hypospadias. MATERIALS AND METHODS: Data were obtained from the Paris Registry of Congenital Malformations (REMAPAR) (2011 to 2016). The initial study population included 3,172 pregnant women who gave birth to a singleton live born male infant with a congenital malformation. After exclusion of cases with unknown beta human chorionic gonadotropin and those with chromosomal or genetic abnormalities, the study population included 194 boys with isolated hypospadias and 1,075 controls. For cases with operative notes (125) we obtained data on type (proximal/distal) of hypospadias. Using quantile regression we compared median values of multiple of median beta human chorionic gonadotropin measured for first trimester Down syndrome screening (10th to 13th gestational weeks) for overall as well as by type of hypospadias vs controls. We also considered possible effects of placental dysfunction (maternal age, intrauterine growth retardation and preterm births) as potential confounding factors. RESULTS: Overall the median beta human chorionic gonadotropin multiple of median was comparable for women who had an infant with hypospadias vs controls (0.99 vs 0.95, p=0.3). However, proximal hypospadias was associated with a statistically significant higher median multiple of median than distal hypospadias or unspecified (1.49 vs 0.92 vs 1.05, p=0.02). The estimates were comparable after adjustment for placental dysfunction. CONCLUSIONS: Our findings support the hypothesis that an alteration in maternal beta human chorionic gonadotropin levels is associated with hypospadias. However, this association appears to be limited to proximal hypospadias.
Asunto(s)
Gonadotropina Coriónica Humana de Subunidad beta/sangre , Hipospadias/sangre , Primer Trimestre del Embarazo/sangre , Adulto , Biomarcadores/sangre , Femenino , Estudios de Seguimiento , Francia/epidemiología , Humanos , Hipospadias/epidemiología , Incidencia , Recién Nacido , Masculino , Embarazo , Pronóstico , Estudios Retrospectivos , Adulto JovenRESUMEN
Circulating tumor DNA (ctDNA) is a powerful tool for the molecular characterization of cancer. The most frequent pediatric kidney tumors (KT) are Wilms' tumors (WT), but other diagnoses may occur. According to the SIOP strategy, in most countries pediatric KT have a presumptive diagnosis of WT if they are clinically and radiologically compatible. The histologic confirmation is established after post-chemotherapy nephrectomy. Thus, there is a risk for a small fraction of patients to receive neoadjuvant chemotherapy that is not adapted to the disease. The aim of this work is to perform molecular diagnosis of pediatric KT by tumor genetic characterization based on the analysis of ctDNA. We analyzed ctDNA extracted from plasma samples of 18 pediatric patients with KT by whole-exome sequencing and compared the results to their matched tumor and germline DNA. Copy number alterations (CNAs) and single nucleotide variations (SNVs) were analyzed. We were able to detect tumor cell specific genetic alterations-CNAs, SNVs or both-in ctDNA in all patients except in one (for whom the plasma sample was obtained long after nephrectomy). These results open the door to new applications for the study of ctDNA with regards to the molecular diagnosis of KT, with a possibility of its usefulness for adapting the treatment early after diagnosis, but also for disease monitoring and follow up.
Asunto(s)
Biomarcadores de Tumor/genética , ADN Tumoral Circulante/genética , Neoplasias Renales/genética , Tumor de Wilms/genética , Biomarcadores de Tumor/sangre , Niño , Preescolar , ADN Tumoral Circulante/sangre , Variaciones en el Número de Copia de ADN , Femenino , Humanos , Lactante , Neoplasias Renales/diagnóstico , Neoplasias Renales/terapia , Masculino , Terapia Neoadyuvante , Nefrectomía , Estudios Retrospectivos , Sensibilidad y Especificidad , Secuenciación Completa del Genoma/métodos , Tumor de Wilms/diagnóstico , Tumor de Wilms/terapiaRESUMEN
BACKGROUND/PURPOSE: To implement resident curriculum in France based on theoretical teaching and bed side training, the national council known as the "Collège Hospitalier et Universitaire de Chirurgie Pédiatrique" examined the relevance and feasibility of systematically introducing simulation program in the pediatric surgery resident training. MATERIAL AND METHODS: A national simulation training program was developed and took place in a 2-day session organized in 7 simulation centers in France. The program included technical (laparoscopic/suturing technique on low-fidelity models) and nontechnical (6 scenarios for standardized consultation, and a team work scenario based on errors prevention in the operative room) skills. Evaluation of the program (Likert scale from 1 (bad) to 5 (excellent) and notation on 20 points) concerned trainees and trainers. RESULTS: 40 residents (95% of all pediatric surgery French residents) attended with a ratio of trainees/trainer of ½. The training objectives earned a score of 4.46/5. The pedagogical value of the seminar scored 4.7/5, teaching quality 17.95/20, and the overall seminar score was 17.35/20. CONCLUSION: This program, unique nationally, was assessed very favorably by the participating residents and by the involved trainers. To our knowledge, it represents the first mandatory national simulation training program included within a surgical training model. LEVEL OF EVIDENCE: Level IV.
Asunto(s)
Cirugía General/educación , Internado y Residencia/métodos , Entrenamiento Simulado/métodos , Niño , Competencia Clínica/estadística & datos numéricos , Curriculum , Francia , Humanos , Modelos Anatómicos , Médicos , Evaluación de Programas y Proyectos de Salud/métodosRESUMEN
INTRODUCTION: Fistulas are a common complication of hypospadias surgery; they are more frequent after mid-shaft and posterior hypospadias repair. Surgical treatment of fistula still remains challenging with a significant failure rate. The basic principle is to add layers between skin and neourethra in order to decrease the incidence of recurrent urethrocutaneous fistula (UCF). We report our experience of UCF repair using a vascularized tunica vaginalis flap (TVF) after posterior and mid-shaft hypospadias surgery. MATERIAL AND METHODS: A retrospective review of all patients operated on using TVF for UCF in our institution between December 2005 and July 2017 was performed. RESULTS: Among 36 cases, TVF was used at a first attempt in 22 patients; 14 children had a prior attempt to close the fistula, and four of them had two surgeries before TVF repair. UCF was respectively penoscrotal (n = 3, 8%), posterior (n = 19, 53%), midshaft (n = 9, 25%) and anterior (n = 5, 14%). The size of the fistula was more than 5 mm in 26 patients. The UCF was treated successfully in every case after one single procedure. In the three children with two fistulas, both fistulas were successfully treated by the same TVF. After an average follow-up time of 45 months there was no recurrence of the initial UCF. In four cases of undescended testis, it was possible to dissect the flap through an inguinal incision and perform an orchydopexy in the same time. One patient presented a testicular atrophy after undescended testis surgery. DISCUSSION: Area review of published series shows excellent results in UCF repair including recurrent fistula (Table). TVF can aspire to some advantages with regard to a dartos flap (DF). First of all, a nearly 2.5-fold lower incidence of fistula after fistula repair with TVF than with DF (5.1% vs. 12.2%) has been shown. Secondly, TVF allows treating multiple fistulas, and can also be brought to the anterior part of the penis until the balano preputial furrow, allowing curing anterior fistula. Furthermore, it doesn't lead to aesthetic complications such as penile rotation or distal skin necrosis, which can occur during DF procedures. CONCLUSION: TVF is a simple and reproductive technique for UCF repair, with a high success rate. The risk of testicular atrophy has to be considered in case of associated undescended testis surgery, and careful attention must be given to the TVF dissection. This technique should be considered as first choice treatment for any UCF.
Asunto(s)
Fístula Cutánea/cirugía , Hipospadias/cirugía , Complicaciones Posoperatorias/cirugía , Colgajos Quirúrgicos , Enfermedades Uretrales/cirugía , Fístula Urinaria/cirugía , Adolescente , Niño , Preescolar , Humanos , Masculino , Estudios Retrospectivos , Testículo/cirugía , Factores de Tiempo , Procedimientos Quirúrgicos Urológicos Masculinos/métodos , Adulto JovenRESUMEN
PURPOSE: To report the results of a conservative strategy based on partial surgery combined with brachytherapy in a prospective cohort of children with bladder-prostate rhabdomyosarcoma (BP RMS). METHODS AND MATERIALS: We prospectively documented the outcome of children treated in our department between 1991 and 2015 for BP RMS and undergoing a multimodal approach combining conservative surgery (partial cystectomy and/or partial prostatectomy) and perioperative interstitial low-dose-rate or pulse-dose-rate brachytherapy. Before brachytherapy, children had received chemotherapy with modalities depending on their risk group of treatment. RESULTS: A total of 100 patients were identified, with a median age of 28 months (range, 5.6 months-14 years). According to the Intergroup Rhabdomyosarcoma Study (IRS) group, 84 were IRS-III, and 12 were IRS-IV tumors. Four patients were treated at relapse. The median number of chemotherapy cycles before local therapy was 6 (range, 4-13). After surgery, 63 patients had a macroscopic tumor residuum. Five patients underwent a brachytherapy boost before pelvic external beam radiation therapy because of nodal involvement, and 95 had exclusive brachytherapy. Median follow-up was 64 months (range, 6 months-24.5 years). Five-year disease-free and overall survival rates were 84% (95% confidence interval 80%-88%) and 91% (95% confidence interval 87%-95%), respectively. At last follow-up most survivors presented with only mild to moderate genitourinary sequelae and a normal diurnal urinary continence. Five patients required a secondary total cystectomy: 3 for a nonfunctional bladder and 2 for relapse. CONCLUSION: Brachytherapy is effective as part of a conservative strategy for BP RMS, with a relatively low delayed toxicity as compared with previously published studies using external beam radiation therapy. Longer follow-up is required to ensure that the functional results are maintained over time.
Asunto(s)
Braquiterapia/métodos , Tratamiento Conservador/métodos , Neoplasias de la Próstata/radioterapia , Neoplasias de la Próstata/cirugía , Rabdomiosarcoma/radioterapia , Rabdomiosarcoma/cirugía , Neoplasias de la Vejiga Urinaria/radioterapia , Neoplasias de la Vejiga Urinaria/cirugía , Adolescente , Antineoplásicos/uso terapéutico , Braquiterapia/efectos adversos , Niño , Preescolar , Terapia Combinada/métodos , Intervalos de Confianza , Tratamiento Conservador/efectos adversos , Cistectomía/efectos adversos , Cistectomía/métodos , Cistectomía/estadística & datos numéricos , Supervivencia sin Enfermedad , Femenino , Estudios de Seguimiento , Humanos , Lactante , Masculino , Neoplasia Residual , Estudios Prospectivos , Prostatectomía/efectos adversos , Prostatectomía/métodos , Prostatectomía/estadística & datos numéricos , Neoplasias de la Próstata/tratamiento farmacológico , Neoplasias de la Próstata/mortalidad , Rabdomiosarcoma/tratamiento farmacológico , Rabdomiosarcoma/mortalidad , Factores de Tiempo , Resultado del Tratamiento , Neoplasias de la Vejiga Urinaria/tratamiento farmacológico , Neoplasias de la Vejiga Urinaria/mortalidadRESUMEN
A heterozygous intragenic duplication within the repeated area (CTGCAGCTG)×2 of the NR5A1 gene was found in a 15-year-old 46,XY DSD (disorders/differences of sex development) patient with micropenis and severe proximal hypospadias. This heterozygous duplication has already been described twice in boys with a similar phenotype, whereas a deletion of 3 amino acids at the same position in the protein SF-1 has been described in a 46,XX patient with primary ovarian failure and short stature. These data suggest that this region within the NR5A1 gene has an important role for SF-1 protein function in gonads and is a hotspot for intragenic rearrangements.
Asunto(s)
Trastorno del Desarrollo Sexual 46,XY/genética , Hipospadias/genética , Factor Esteroidogénico 1/genética , Adolescente , Heterocigoto , Humanos , Hipospadias/metabolismo , Masculino , Mutación/genética , Factor Esteroidogénico 1/metabolismoRESUMEN
BACKGROUND: Patients with Beckwith-Wiedemann syndrome (BWS) or isolated hemihypertrophy (HH) treated for a Wilms tumor (WT) carry an increased risk of developing metachronous lesion. There are no guidelines on precise indications for nephron sparing surgery (NSS) in unilateral WT (UWT). The objective of this retrospective study was to delineate the indications of NSS in patients with BWS/HH treated for WT and to evaluate their outcome. PROCEDURE: All cases of BWS/HH treated for a WT according to SIOP protocols from 1980 to 2013 were reviewed. Patients were divided into two groups (G): isolated UWT (G1) and bilateral lesions (G2) with two subgroups: bilateral tumors suspected of malignancy (G2a), and unilateral tumor suspected of malignancy with contralateral nephroblastomatosis (G2b). RESULTS: Forty-six patients were included (34 G1, three G2a, and nine G2b). Nine NSS and 25 total nephrectomies (TN) were performed in G1, two bilateral NSS and one NSS with contralateral TN in G2a, and eight NSS and one TN in G2b. The 3-year event-free survival was 92.3% (95% CI [77.9-97.5%]). One death occurred after a local relapse following a TN for a stage III stromal WT (G1) and another after a combined local and distant relapse following a NSS for a stage I diffuse anaplastic WT (G2b). There were two metachronous WT (4%), 3 years after a TN (G1) and 12 years after a NSS (G2b). CONCLUSIONS: NSS is recommended in bilateral WT and may be an option in selected UWT patients with BWS/HH because it was not associated with an increased risk of local relapse.