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1.
Int. braz. j. urol ; 46(6): 1072-1074, Nov.-Dec. 2020. graf
Article in English | LILACS | ID: biblio-1134267

ABSTRACT

ABSTRACT Introduction We present an alternative procedure for distal hypospadias consisting of urethral mobilization and partial glandar disassembly, namely GUD (glandar urethral disassembly) technique. Materials and Methods A subcoronal circumcision exposes distal dysplastic urethra. We incise the Buck´s fascia on both sides of urethra releasing it partially from the corpora. We keep a thin bridge of urethral plate to the glans and disassembly almost completely the glans from the corpora, except for the bridge. The glans is incised creating two wide wings that are extremely mobile. The urethra is mobilized, advanced and sutured to the tip of the glans. The glans wings embrace the distal urethra producing a conical glans. Discussion The concept of urethral mobilization has been reported and popularized by Koff in the literature to correct distal hypospadias. One of the limitations of this procedure is the risk of urethral retraction due to extensive proximal dissection. We got inspiration from Mitchell and Bagli' s work of penile disassembly in epispadias to develop the GUD concept. We adopt minimal urethral mobilization mainly in glandar/proximal penile shaft and complete deconstruction of the glans, detaching the corpora from the glans and rotating the wide glans wings to embrace the urethra. Therefore we avoid suture urethroplasty and refurbish the glans to a better conical shape. Conclusion We are convinced that this operation can be regarded as a genuine alternative to distal hypospadias (coronal and subcoronal) but should not be addressed to midshaft forms.


Subject(s)
Humans , Male , Infant , Reconstructive Surgical Procedures , Hypospadias/surgery , Penis/surgery , Urologic Surgical Procedures, Male , Urethra/surgery
2.
Int. braz. j. urol ; 46(6): 1029-1041, Nov.-Dec. 2020. tab, graf
Article in English | LILACS | ID: biblio-1134266

ABSTRACT

ABSTRACT Introduction To assess the feasibility of vacuum physiotherapy meant to decrease graft contraction and recurrent penile curvature (PC), hence successful tubularization and a straight penis in patients underwent two-stage buccal mucosa graft (BMG) urethroplasty, in proximal hypospadias repair. Material and methods Between January 2014 and July 2018, 59 two-stage BMG urethroplasties performed at our referral center, were included in the study. The parents were counseled to use the vacuum device between the two stages. An internal, self-administered, semiquantitative, non-validated questionnaire was designed to record parent and patient adherence to the vacuum physiotherapy and parent satisfaction. Success rate of graft tubularization, curvature correction rates, and status of early (4 months) postoperative urinary stream were evaluated. Results Of 45/59 (76.3%) who returned the questionnaire, 77.8% followed the recommended physiotherapy protocol using the vacuum device. 93.3% of parents replied that the use of the vacuum was easy or moderately easy. None of the parents interrupted the physiotherapy because of perceived difficulty or intolerability. 100% of parents would have repeated the physiotherapy, if they had to. Overall, success rate of tubularization was 98.3% (58/59), complete curvature correction was achieved in 88.2% (52/59) of patients, and 79.7% (47/59) of patients showed a straight and powerful early post-operative urinary stream. Conclusions Physiotherapy with the vacuum device is safe, easy and practically feasible. Our vacuum physiotherapy protocol had high compliance rate. Vacuum physiotherapy should be considered for further assessment in patients undergoing two stage hypospadias repair using buccal mucosa.


Subject(s)
Humans , Male , Infant , Child, Preschool , Child , Hypospadias/surgery , Urologic Surgical Procedures, Male , Urethra/surgery , Vacuum , Physical Therapy Modalities , Mouth Mucosa
3.
Rev. cuba. anestesiol. reanim ; 19(3): e620, sept.-dic. 2020.
Article in Spanish | LILACS, CUMED | ID: biblio-1138887

ABSTRACT

Introducción: El laringoespasmo es una complicación temida por los anestesiólogos. Se asocia a broncoespasmo, hipoxia, arritmias, aspiración del contenido gástrico y paro cardiaco. Es más frecuente en neonatos y lactantes. Está estrechamente vinculado al tipo de cirugía y consiste en un cierre intenso y prolongado de la glotis en respuesta a la estimulación glótica directa o refleja. Objetivo: Describir el tratamiento del laringoespasmo parcial reflejo en un paciente pediátrico durante una intervención urológica. Presentación del caso: Paciente de 18 meses de edad al cual se le realiza meatotomía. Durante la cirugía presenta laringoespasmo parcial reflejo tratado con maniobras físicas. Sin la necesidad de usar medicamento ni vía área mecánica. Conclusiones: Las maniobras utilizadas son seguras y efectivas en el tratamiento del laringoespasmo reflejo sin la necesidad de abordar la vía aérea ni uso de medicamentos, se lograron resultados satisfactorios con una relación riesgo beneficio a favor del paciente(AU)


Introduction: Laryngospasm is a complication feared by anesthesiologists. It is associated with bronchospasm, hypoxia, arrhythmias, aspiration of gastric contents, and cardiac arrest. It is more common among neonates and infants. It is closely related to the type of surgery and consists in an intense and prolonged closure of the glottis in response to direct or reflex glottic stimulation. Objective: To describe the management of reflex partial laryngospasm in a pediatric patient during a urological intervention. Case presentation: 18-month-old patient who received meatotomy. During surgery, he presented reflex partial laryngospasm managed with physical maneuvers, without the need to use medication or the mechanic airways ventilation. Conclusions: The maneuvers used are safe and effective for the management of reflex laryngospasm without the need to address the airway or using of medications. Satisfactory outcomes were achieved with a risk-benefit ratio in favor of the patient(AU)


Subject(s)
Humans , Male , Infant , Laryngismus/therapy , Hypospadias/surgery
4.
Int. braz. j. urol ; 46(5): 772-777, Sept.-Oct. 2020. tab, graf
Article in English | LILACS | ID: biblio-1134224

ABSTRACT

ABSTRACT Introduction: Transverse preputial island flap urethroplasty (TPIFU) is one of the most frequently performed technique for single-stage repair in proximal hypospadias. It was reported that the subepithelial urethroplasty would obviously decrease urethrocutaneous fistula (UF) complication after proximal TIP. But in the process of TPIFU, it had not been reported yet. Objective: We reviewed our experience to evaluate and compare the effect of continuous eversion suture (CES) versus continuous inversion subepithelial suture (CIS) on complication rates in the TPIFU. Material and methods: A retrospective review of all patients operated with CES and CIS in our institution between January 2017 and Jun 2017 was performed. Results: A total of 161 patients were enrolled in the research. Patients were followed up for 12~17 months. Total success rate was 73.9% (119/161). No statistically difference was found between the two groups with regard to age of patients (P=0.097), catheter size (P=0.52), time of catheterization (P=0.47), length of neourethra (P=0.20), non-urethral comorbidity (P=0.44) and post-operative infection (P=1.0). The overall postoperative complications had no statistically difference between the two groups (P=0.067). There were no statistically significant differences in the incidence of urethra-cutaneous fistula (UF) (OR=0.07, 95% CI: -0.24~0.037, P=0.22), urethral diverticulum (UD) (OR=0.026, 95% CI: -0.16~-0.056, P=0.323), urethral stricture (US) (OR=0.081, 95% CI: -0.15~0.15, P=1.0) and breakdown of urethral repair (BU) (OR=0.02, 95% CI: -0.118~-0.044, P=1.0). Discussion: The comparison of two group's postoperative complications was feasible because there were no statistically differences among perioperative variables. It seemed as if continuous inversion subepithelial suture would promote healing. However, it indicated that the overall success rate and the incidences of UF, UD, US and BU complications had no statistically difference between groups. It might be accounted for the subtle differences of techniques changing the process of establishing prime and side branches vascularization. Conclusions: The CIS technique had no significantly different effect on the four complications rates when compared with CES in TPIFU. Thus, CES and CIS could be randomly adopted in TPIFU as personal preference.


Subject(s)
Humans , Male , Infant , Hypospadias/surgery , Postoperative Complications , Urologic Surgical Procedures, Male/adverse effects , Surgical Flaps , Sutures , Urethra/surgery , Retrospective Studies
6.
Int. braz. j. urol ; 46(3): 436-443, May-June 2020. tab, graf
Article in English | LILACS | ID: biblio-1090601

ABSTRACT

ABSTRACT Purpose: Spongioplasty (mobilization and midline approximation of the two branches of the bifid dysplastic distal corpus spongiosum) can form a covering layer for the neourethra to prevent urethrocutaneous fistula in hypospadias repair surgery. However, it remains unclear whether spongioplasty affects neourethral function. The objective of this study was to compare neourethral function after hypospadias repair with and without spongioplasty. Materials and Methods: Fourteen congenital hypospadiac New Zealand male rabbits were randomly allocated into two groups, seven animals underwent Duplay hypospadias repair and spongioplasty (experimental group), while seven underwent Duplay surgery alone (control group). Functional differences between groups were assessed by comparing neourethral compliance and flow rate. Two months after surgery, in vivo neourethral compliance was assessed by measuring intraluminal pressure with a digital pressure meter of an isolated neourethral segment, following progressive distension with 1, 2, and 3mL of air. Penises were harvested for uroflowmetry test using a simple device. Results: Postoperatively, fistula developed in one and zero rabbits in the control and experimental groups, respectively. Mean pressures tended to be higher in the experimental group than in the control group (82.14 vs. 69.57, 188.43 vs. 143.26, and 244.71 vs. 186.29mmHg for 1, 2, and 3mL of air, respectively), but the difference was not statistically significant. Mean flow rates also did not significantly differ between the experimental and control groups (2.93mL/s vs. 3.31mL/s). Conclusion: In this congenital rabbit model, no obvious functional differences were found between reconstructed urethras after hypospadias repair with and without spongioplasty.


Subject(s)
Humans , Animals , Male , Infant , Hypospadias , Penis , Rabbits , Urologic Surgical Procedures, Male , Surgical Flaps , Urethra , Random Allocation
7.
Article in Chinese | WPRIM | ID: wpr-828665

ABSTRACT

This article reported the clinical characteristics and SRD5A2 gene mutation pattern of a child with steroid 5-α reductase type 2 deficiency. The 2-month-old boy showed hypospadias and short penis shortly after birth. DNA was extracted from the peripheral blood of the child and his parents. The endocrine disease-related genes were captured and sequenced by high-throughput sequencing technology, and the family DNA samples were verified by Sanger sequencing. The results showed that c.680G>A(p.R227Q) and c.608G>A(p.G203D) compound heterozygous mutations existed in the SRD5A2 gene of the child. The c.680G>A mutation inherited from his father, which was a known pathogenic mutation. The c.608G>A mutation originated from his mother, which was a novel mutation discovered in this study. These results provide molecular evidence for the etiological diagnosis of the child and genetic counseling for the family, as well as extend the mutation spectrum of SRD5A2 gene.


Subject(s)
3-Oxo-5-alpha-Steroid 4-Dehydrogenase , Genetics , Base Sequence , Child , Female , Humans , Hypospadias , Infant , Male , Membrane Proteins , Genetics , Mutation
8.
Prensa méd. argent ; 105(11): 852-860, dic2019. fig, tab
Article in English | LILACS, BINACIS | ID: biblio-1050073

ABSTRACT

Background: The Tubularized Incised Urethral Plate (TIP) urethroplasty is a widely accepted technique among urologists and heavily used in their centers. This technique was developed by Snodgrass in 1994 for repairing varied degrees of hypospadias. Aim: This paper aims at analyzing and evaluating our early experience and the outcomes of using TIP urethroplasty technique. Therefore, the results have to be assessed in a large series. Methods: In this prospective study, 95 patients with varying hypospadiac meatus levels who experienced the procedure of TIP. During the entire sampling period, patients had varying degrees of hypospadias ranging from glanular to penoscrotal (glanular 2, coronal/subcoronal 37, distal shaft 30, mid-shaft 10, proximal shaft 5 and penoscrotal 11). Results: The overall success rate of TIP procedure repair of hypospadias was 87.4%; whereas the overall reoperation rate was 12.6%. The highest percentage was for those with failed previous repair (secondary). The average duration of the procedure was 58.6±18.4 minutes. Nearly a third of the patients developed one or more postoperative major complication. The total rate of fistula was 12.6%, with "10.6% in primary distal, 15.9% in primary proximal and 20% in secondary repair". Sixteen cases had a mild degree of "meatal stenosis" (16.8%), but all were managed by simple dilatation at the office or at home using 8 F feedingtube. Conclusion: The outcomes demonstrated that the TIP procedure is a quick, safe and reliable technique. Additionally, it can provide excellent cosmoses and function with few complications and acceptable reoperation rate.


Subject(s)
Humans , Male , Urethra/surgery , Urethral Stricture/pathology , Prospective Studies , Hypospadias/surgery , Reoperation/statistics & numerical data
9.
Int. braz. j. urol ; 45(6): 1238-1248, Nov.-Dec. 2019. tab, graf
Article in English | LILACS | ID: biblio-1056340

ABSTRACT

ABSTRACT Introduction: Pubic hypertrophy, defined as an abnormal and abundant round mass of fatty tissue located over the pubic symphysis, is frequently underestimated in patients with hypospadias. We examined the prevalence of this condition, as well as the outcomes associated with its surgical treatment. Material and methods: Within 266 hypospadias patients treated at our clinic, we assessed the prevalence of pubic hypertrophy, and we schematically described the surgical steps of pubic lipectomy. Multivariable logistic regression (MLR) tested for predictors of pubic hypertrophy. Finally, separate MLRs tested for predictors of fistula and any complications after pubic lipectomy. Results: Of 266 hypospadias patients, 100 (37.6%) presented pubic hypertrophy and underwent pubic lipectomy. Patients with pubic hypertrophy more frequently had proximal hypospadias (44 vs. 7.8%), disorders of sex development (DSD) (10 vs. 0.6%), cryptorchidism (12 vs. 2.4%), and moderate (30°-60°) or severe (>60°) penile curvature (33 vs. 4.2%). In MLR, the location of urethral meatus (proximal, Odds ratio [OR]: 10.1, p<0.001) was the only significant predictor of pubic hypertrophy. Finally, pubic lipectomy was not associated with increased risk of fistula (OR: 1.12, p=0.7) or any complications (OR: 1.37, 95% CI: 0.64-2.88, p=0.4) after multivariable adjustment. Conclusions: One out of three hypospadias patients, referred to our center, presented pubic hypertrophy and received pubic lipectomy. This rate was higher in patients with proximal hypospadias suggesting a correlation between pubic hypertrophy and severity of hypospadias. Noteworthy, pubic lipectomy was not associated with increased risk of fistula or any complications.


Subject(s)
Humans , Adolescent , Adult , Young Adult , Lipectomy/methods , Hypospadias/surgery , Hypospadias/epidemiology , Penis/surgery , Postoperative Complications , Pubic Bone/surgery , Logistic Models , Prevalence , Retrospective Studies , Risk Factors , Treatment Outcome , Statistics, Nonparametric , Serbia/epidemiology , Hypertrophy/surgery , Hypertrophy/epidemiology , Medical Illustration
10.
Medicina (Ribeiräo Preto) ; 52(3)jul.-set. 2019.
Article in Portuguese | LILACS | ID: biblio-1025590

ABSTRACT

Modelo de estudo: Estudo de prevalência. Objetivo do estudo: Avaliar o perfil epidemiológico dos casos de hipospádia atendidos no Hospital São Francisco na Providência de Deus da Universidade São Francisco (HUSF), Bra-gança Paulista/SP nos últimos 10 anos (2007-2017), relacionando os tipos de hipospádia com as malformações asso-ciadas e idade à primeira cirurgia corretiva. Metodologia: Foi realizado um levantamento de casos de hipospádia atendidos entre 2007 e 2017 no HUSF, com pacientes oriundos das cidades da Região Bragantina do estado de São Paulo. Os tipos de hipospádia foram relacionados com as malformações associadas. Foram pesquisados nos prontuários: tipo de hipospádia, idade à primeira cirurgia, laudo de exame ultrassonográfico, etnia, local de nascimento e malformação associada. Os dados foram analisados estatisticamente utilizando os testes Qui-quadrado, teste F e de Kruskal-Wallis. Resultados: Em 1401 atendimentos pediátricos no período avaliado, foram encontrados 45 casos de hipospádia com prevalência igual a 0,7/1000 nascidos vivos. Do total de casos, 29 foram classificados como do tipo distal, seis casos de hipospádia média e 10 casos do tipo proximal [F-test (2,8)=5,552; P=0,003; χ2=9,3117; P=0,009]. Nos casos de hipospádia distal, foram detectadas 16 malformações associadas e, nos tipos média e proximal, foram observadas sete malformações associadas em cada uma (KW=3,322, P=0,19). O maior número de casos foi abordado cirurgica-mente no intervalo entre os três e quatro anos de idade. Conclusão: A hipospádia distal foi a mais prevalente, com idade à primeira cirurgia em desacordo com o intervalo proposto para a correção da hipospádia. (AU)


Study model: a Prevalence study. Objective: Evaluating the epidemiological profile of hypospadias cases assisted in the Hospital São Francisco na Providência de Deus of the Universidade São Francisco (HUSF), Bragança Paulista, Brazil, in the last 10 years (2007-2017), relating the types of hypospadias with associated malformations and age at the first corrective surgery. Methodology: A survey of hypospadias cases assisted between 2007 and 2017 in HUSF was carried out with patients from the Bragantina region of the state of São Paulo. The types of hypospadias were related to the associated malformations. The following data were collected from the medical records: type of hypospadias, age at first surgery, ultrasound imaging, ethnicity, place of birth, associated malformation. Data were statistically analyzed using Chi-squared, F-test and Kruskal-Wallis tests. Results: In 1401 pediatric assistances, it was found 45 cases of hypo-spadias with a prevalence of 0.7/1000 births. From the total, 29 cases were classified as distal, six cases of medium, and 10 cases as proximal types [F-test (2.8)=5.552, P=0.003, χ2=9.3117, P=0.009]. Cases of distal hypospadias had 16 associated malformations, and for medial and proximal types there were seven associated malformations in each one (KW=3.322, P=0.19). The largest number of cases was surgically corrected between three and four years of age. Conclusion: Distal hypospadias were the most prevalent in the Bragantina region in the last 10 years. It was also ob-served that the age at first surgery is in disagreement with the range proposed for the corrective approach of hypospadia (AU)


Subject(s)
Humans , Male , Female , Child , Congenital Abnormalities , Health Profile , Hypospadias
11.
Rev. bras. anestesiol ; 69(1): 27-34, Jan.-Feb. 2019. tab, graf
Article in English | LILACS | ID: biblio-977427

ABSTRACT

Abstract Background and objectives: The combination of clonidine with local anesthetic administered for epidural anesthesia via caudal route seems to improve the quality of postoperative analgesia, but with conflicting results. This study compared the postoperative analgesia of three different doses of clonidine combined with bupivacaine in caudal epidural anesthesia in children undergoing hypospadias repair. Methods: Eighty children aged 1-10 years, candidates for surgical repair of hypospadias, were randomly divided into four groups of 20 patients to receive general anesthesia combined with caudal epidural anesthesia with bupivacaine 0.165% alone or in combination with 1, 2 or 3 µg.kg- 1 of clonidine. The primary outcome was morphine consumption in the first 24 h postoperatively. Mean arterial pressure, heart rate, end-tidal concentration of sevoflurane, time to awakening, pain severity (FLACC scale), level of sedation (RAMSAY), duration of analgesia, and occurrence of adverse effects were also compared. Results: Intraoperatively, there was no difference between groups regarding mean arterial pressure, heart rate, end-tidal concentration of sevoflurane, and time to awakening. Postoperative morphine consumption and pain severity were similar between groups, but the group receiving clonidine (3 µg.kg-1) had lower heart rate and higher sedation level than the group receiving bupivacaine alone. Conclusions: The combination of clonidine at doses of 1, 2 or 3 µg.kg-1 with bupivacaine 0.16% via caudal epidural route did not alter the consumption of morphine in the early postoperative period of children undergoing hypospadias repair.


Resumo Justificativa e objetivos: A associação de clonidina ao anestésico local administrado por via peridural caudal parece melhorar a qualidade da analgesia pós-operatória, mas com resultados conflitantes. Este estudo comparou a analgesia pós-operatória de três diferentes doses de clonidina associada à bupivacaína na anestesia peridural caudal em crianças submetidas à correção de hipospádia. Método: Oitenta crianças entre um e dez anos, candidatas à correção cirúrgica de hipospádia, foram divididas, aleatoriamente, em quatro grupos de 20 pacientes para receber anestesia geral associada à anestesia peridural caudal com bupivacaína 0,166% isolada ou associada a 1, 2 ou 3 µg.Kg-1 de clonidina. Como desfecho principal avaliou-se o consumo de morfina nas primeiras 24 horas de pós-operatório. Compararam-se também pressão arterial média, frequência cardíaca, concentração expirada de sevoflurano, tempo de despertar da anestesia, intensidade da dor pela escala FLACC, nível de sedação (Ramsay), tempo de duração da analgesia e ocorrência de efeitos adversos. Resultados: No transoperatório, não houve diferença entre os grupos quanto à pressão arterial média, frequência cardíaca, concentração expirada de sevoflurano e ao tempo de despertar. No pós-operatório, o consumo de morfina e a intensidade da dor foram similares entre os grupos, mas o grupo que recebeu 3 µg.Kg-1 de clonidina apresentou menor frequência cardíaca e maior sedação do que o grupo que recebeu somente bupivacaína. Conclusões: A associação de clonidina nas doses de 1, 2 ou 3 µg.Kg-1 à bupivacaína 0,166% por via peridural caudal não alterou o consumo de morfina no pós-operatório imediato de crianças submetidas à correção de hipospádia.


Subject(s)
Humans , Male , Infant , Child, Preschool , Child , Bupivacaine/administration & dosage , Clonidine/administration & dosage , Analgesics/administration & dosage , Hypospadias/surgery , Anesthesia, Epidural/methods , Anesthetics, Local/administration & dosage , Single-Blind Method , Prospective Studies , Drug Combinations
12.
Article in Chinese | WPRIM | ID: wpr-772079

ABSTRACT

OBJECTIVE@#To investigate the transcriptome profile of genital tubercles (GTs) in male SD rats and explore the mechanism of hypospadias induced by Di (2-ethylhexyl) phthalate (DEHP).@*METHODS@#Forty time-pregnant SD rats were randomly divided into 4 equal groups, namely GD16 group and GD19 group (in which the male GTs were collected on gestation day[GD]16 and GD19 for RNA-seq, respectively), control group and DEHP exposure group (with administration of oil and 750 mg/kg DEHP by gavage from GD12 to GD19, respectively).In the control and DEHP exposure groups, the GTs were collected from the male fetuses on GD19.5, and scanning electron microscopy and HE staining were used to observe the morphological changes.The differentially expressed genes (DEGs) in the GTs were screened using lllumina HiSeq 2000 followed by GO and KEGG enrichment analyses to characterize the transcriptome profile.Immunofluorescence assay was performed to verify the DEGs (Mafb) identified by RNA-seq results.Immunofluorescence assay and Western blotting were used to examine the expression levels of Mafb in the penile tissue.@*RESULTS@#A total of 1360 DEGs were detected in the GTs between GD16 group and GD19 group by RNA-seq.Among these genes, 797 were up-regulated and 563 were down-regulated.These DEGs were mainly enriched in the cell adhesion plaque signaling pathway, axon guidance signaling pathway, and extracellular matrix receptor signaling pathway.Compared with that in GD16 group, Mafb was significantly up-regulated in GD19 group, which was consistent with the sequencing results.Mafb and β-catenin were significantly down-regulated in DEHP-exposed group compared with the control group ( < 0.01).@*CONCLUSIONS@#Mafb expression increases progressively with the development of GTs in male SD rats.DEHP exposure causes significant down-regulation of Mafb and β-catenin, suggesting that β-catenin signaling pathway that affects Mafb is related to DEHP-induced hypospadias in SD rats.


Subject(s)
Animals , Diethylhexyl Phthalate , Female , Gene Expression Profiling , Humans , Hypospadias , MafB Transcription Factor , Male , Oncogene Proteins , Phthalic Acids , Pregnancy , Rats , Rats, Sprague-Dawley
13.
Article in Chinese | WPRIM | ID: wpr-781317

ABSTRACT

OBJECTIVE@#To carry out genetic testing for a boy presenting with mental retardation and hypoplasia.@*METHODS@#Conventional karyotyping, fluorescence in situ hybridization (FISH) and single nucleotide polymorphism based array (SNP-array) were used to analyze the boy and his parents.@*RESULTS@#SNP-array has detected a 25.7 Mb microduplication at 2q33.3q36.3 in the boy. Chromosomal karyotyping and FISH analysis indicated that his mother had a karyotype of 46,XX,ish ins(11;2) (p15;q33q36), and that the boy has carried an abnormal chromosome 11 derived from the maternal translocation. The karyotype of the boy was ascertained as 46,XY,ish der(11)ins(11;2) (p15;q33q36)mat.@*CONCLUSION@#SNP-array combined with G-banding and FISH can delineate the cryptic translocation and is valuable for the assessment of recurrence risk for subsequent pregnancies.


Subject(s)
Child , Chromosome Banding , Chromosome Duplication , Female , Genetic Testing , Humans , Hypospadias , Genetics , In Situ Hybridization, Fluorescence , Intellectual Disability , Genetics , Karyotyping , Male , Polymorphism, Single Nucleotide , Pregnancy , Translocation, Genetic
14.
Article in English | WPRIM | ID: wpr-762838

ABSTRACT

Hypospadias is a congenital malformation of the male genitalia. The reconstructive objectives are to obtain voiding with laminar flow and satisfactory sexual function. Several urethroplasty techniques have been described, but for perineal or revisional cases no single technique has shown robust success. In this study, we describe the expanded use of intestinal flaps for urethral reconstruction and report a peculiar request from a patient to undergo peno-urethral separation after successful hypospadias repair with a free ileum flap. A 51-year-old male patient with perineal hypospadias underwent several urethral reconstructive procedures with poor outcomes. A free ileum flap was attempted as a substitute for the urethra. Following successful reconstruction, separation of the neo-urethra (ileum) from the penile body was performed to address the patient's sexual expectations. A free ileum flap proved to be a reliable urethral substitute in perineal hypospadias reconstruction, with a successful outcome. The peno-urethral separation with the creation of an “accessory penis,” however peculiar, optimized the results in terms of both sexual and urinary function. Anatomical restoration of the urethra and patient-reported expectations are the key to successful hypospadias reconstructive procedures. Sexual function outcomes and the patient's perception of success should not be underestimated, even when urinary function has been restored.


Subject(s)
Female , Genitalia, Male , Humans , Hypospadias , Ileum , Male , Methods , Middle Aged , Penis , Urethra
15.
Ludovica pediátr ; 21(4): 4-7, dic. 2018.
Article in Spanish | LILACS | ID: biblio-969266

ABSTRACT

En la actualidad es frecuente la solicitud de ultrasonido renal pediátrico en pacientes con hipospadias. Si bien es necesario en hipospadias asociados con otras malformaciones, no parece ser útil en pacientes con hipospadias aislados


At present, the request for renal ultrasound is frequent pediatric in patients with hypospadias. Although it is necessary in hypospadias associated with other malformations, does not appear to be useful in patients with isolated hypospadias


Subject(s)
Humans , Ultrasonography , Hypospadias , Child
16.
Int. braz. j. urol ; 44(2): 409-410, Mar.-Apr. 2018.
Article in English | LILACS | ID: biblio-1040039

ABSTRACT

ABSTRACT Introduction and objective Urethral duplication is a rare congenital anomaly, with roughly 200 cases reported in the literature (1). It is more frequent in males, with few cases reported in females. The clinical presentation differs according to the anatomical variant present. The duplication most commonly occurs in the sagittal plane with one urethra located ventrally and the other dorsally (2). Usually the ventral urethra is the more functional of both. Duplications occurring in the coronal plane are quite rare and they are usually associated with bladder duplication (3). The purpose of this paper was to present a video of a boy with an unusual urethral duplication form. Materials and Methods Patient was born premature due to oligohydramnios at 7 months-gestational age and he has initial diagnosis of hypospadia. Since then, he presented at least 7 febrile UTI and mother complained of difficult micturition and a presence of a mass at lower abdomen. Patient was referred to our institution and we identified urethral duplication with a glandar and scrotal meatus, palpable bladder and left penile-hemiscrotum transposition. US and CT-scan showed left kidney agenesis and overdistended bladder. VCUG and retrograde urethrography showed duplication, presence of contrast in the seminal vesicles and complete catheterizing of both urethras was not possible. Results The topic urethra was dysplastic and not patent to a 4Fr plastic tube so we were unable to access it endoscopically. We performed initially a Mitrofanoff procedure to allow CIC and treat chronic retention. Six months later, we assessed both urethras surgically and concluded that dorsal urethra was dysplastic after 3cm still in the penile area and scrotal urethra was not possible to be catheterized. We excised the ventral urethra because of dribbling complaints up to bulbar area and reconstructed the scrotal transposition, keeping the topic urethra for cosmetic issues. Patient had excellent outcome, performs CIC every 4 hours and has not presented further UTI episodes. Discussion and conclusion The urethral duplication is an anomaly that has multiple anatomical presentations. There are several theories about the etiology, but none can explain all types of presentations. There is also more than one rating available, and the Effmann classification is the most detailed. The case exemplifies this varied spectrum of anatomic urethral duplication. It resembles the urethral duplication type IIa-Y, however, ventral urethra meatus was located in penoscrotal area and both urethras were at least partially hypoplastic/dysplastic associated with obstruction and bladder retention. In determining how to best manage a patient with Y-type urethral duplication, the caliber and quality of the orthotopic urethra must first be assessed. Published reports suggest that best outcomes are those using the ventral duplicated urethra for the reconstruction (4). In this case, none of urethras were functional and a supravesical outlet channel had to be provided. The treatment of this condition requires an individualized planning and a vast technical knowledge of reconstructive surgery.


Subject(s)
Humans , Male , Infant, Newborn , Urethra/abnormalities , Congenital Abnormalities/surgery , Urinary Retention/surgery , Hypospadias/surgery , Kidney/abnormalities , Kidney Diseases/congenital , Chronic Disease , Urinary Retention/complications , Hypospadias/complications , Kidney/surgery , Kidney Diseases/surgery , Kidney Diseases/complications
17.
Rev. inf. cient ; 97(4): i:860-f:867, 2018. ilus
Article in Spanish | LILACS | ID: biblio-1005680

ABSTRACT

Se presentó un caso intervenido en el servicio de Urología del Hospital Nacional "Guido Valadares" de Timor Oriental, con diagnóstico de retención completa de orina, insuficiencia renal aguda obstructiva posrrenal, hidronefrosis bilateral y estenosis uretral e hipospadia, con el objetivo de demostrar el uso de la mucosa prepucial para las hipospadias que concomitan con estenosis uretral. La conducta quirúrgica consistió en una uretroplastia con mucosa prepucial. Se logró la reconstrucción total de la uretra afectada, con buenos resultados finales estéticos y funcionales. El uso de la mucosa oral fue de gran utilidad en el manejo del paciente(AU)


A case was presented in the Department of Urology of Guido Valadares National Hospital in East Timor, diagnosed with complete retention of urine, post renal, obstructive and acute renal failure, bilateral hydronephrosis and urethral stricture and hypospadias, in order to demonstrate the use of prepucial mucosa for hypospadias that concomitan with urethral stricture. The surgical treatment consisted urethroplasty with preputial mucosa. Total reconstruction of the affected urethra was done with good aesthetic and functional outcomes. The use of oral mucosa was very useful in patient management(AU)


Foi apresentado um caso no Departamento de Urologia do Hospital Nacional "Guido Valadares" de Timor Oriental, com diagnóstico de retenção urinária completa, insuficiência renal aguda obstrutiva pós-renal, hidronefrose bilateral e estenose e hipospádia uretral, para demonstrar a utilização da mucosa prepucial para hipospádia que coincide com estenose uretral. O procedimento cirúrgico consistiu em uma uretroplastia com mucosa prepucial. A reconstrução total da uretra afetada foi alcançada, com bons resultados estéticos e funcionais finais. O uso da mucosa oral foi muito útil no manejo do paciente(AU)


Subject(s)
Humans , Male , Urethral Stricture/diagnosis , Hypospadias/surgery , Hypospadias/diagnosis , Timor-Leste
18.
Rev. argent. endocrinol. metab ; 54(4): 196-203, dic. 2017. tab
Article in Spanish | LILACS | ID: biblio-957986

ABSTRACT

Los niños con restricción del crecimiento intrauterino (RCIU) presentan en la vida posnatal una serie de alteraciones metabólicas y hormonales, y tienen predisposición al desarrollo de obesidad, hipertensión arterial, enfermedad cardiovascular, resistencia a la insulina y diabetes tipo 2. La exposición a un ambiente intrauterino desfavorable en fases críticas del desarrollo puede tener un efecto deletéreo sobre la gónada en formación. Se realizó una revisión bibliográfica y puesta al día sobre la posible asociación entre RCIU y alteraciones de la función gonadal en niños y adolescentes de ambos sexos. Para facilitar la actualización, se dividió por etapas en: 1, prenatal; 2, posnatal y prepuberal; 3, puberal, y 4, adulta. La mayoría de los niños que nacen muy prematuros o con muy bajo peso al nacer hacen una transición sin obstáculos desde la infancia a la edad adulta con respecto a la salud reproductiva. Sin embargo, en los varones se puede observar criptorquidia, hipospadias, cáncer testicular y menor fertilidad, y en las niñas, pubertad y menarca temprana, hiperandrogenismo y síndrome de ovario poliquístico. Existen datos controvertidos y se necesitan más estudios para aclarar la relación entre el RCIU y la función hipotálamo-hipófiso-gonadal.


Low birth weight due to intrauterine growth restriction (IUGR) is associated with an increased risk of obesity, hypertension, cardiovascular disease, insulin resistance, and type 2 diabetes during postnatal life. Exposure to an unfavourable intrauterine environment in critical phases of development may have a deleterious effect on the forming gonad. The objective was to carry out a bibliographic review and update on the possible association between IUGR and alterations of gonadal function in children and adolescents of both sexes. To facilitate the update, this was divided into stages: 1, prenatal; 2, postnatal and pre-pubertal; 3, puberal, and 4, adult. Most children born preterm or with low birth weight make a normal transition from childhood to adulthood with respect to reproductive health. However, cryptorchidism, hypospadias, testicular cancer and lower fertility could be observed in boys, and early puberty and menarche, hyperandrogenism and polycystic ovarian syndrome in girls. However, the data are controversial, and further studies are needed to clarify the relationship between IUGR and pituitary gonadal function.


Subject(s)
Humans , Male , Female , Infant, Small for Gestational Age/growth & development , Fetal Growth Retardation/physiopathology , Gonadal Disorders/etiology , Puberty, Precocious/embryology , Hyperandrogenism/embryology , Cryptorchidism/embryology , Hypospadias/embryology
19.
Int. braz. j. urol ; 43(2): 325-334, Mar.-Apr. 2017. tab, graf
Article in English | LILACS | ID: biblio-840835

ABSTRACT

ABSTRACT Objective To evaluate prevalence trends of hypospadias in South-America it is essential to perform multicenter and multinational studies with the same methodology. Herein we present systematic data as part of an international multicenter initiative evaluating congenital malformations in South America over a 24-year period. Materials and Methods A nested case-control study was conducted using the Latin American Collaborative Study of Congenital Malformations (ECLAMC), between January 1989 and December 2012. Cases were stratified as isolated (IH) and non-isolated hypospadias (NIH). Global prevalence was calculated and discriminated by country. Associations between birth weight and gestational age, and NIH distribution by associated abnormality and severity of hypospadias, were analyzed. Results A total of 159 hospitals from six countries participated, reporting surveillance on 4.020.384 newborns. A total of 4.537 hypospadias cases were detected, with a global prevalence of 11.3/10.000 newborns. Trend analyses showed in Chile, Brazil and Uruguay a statistically significant increase in prevalence. Analysis of severity and associated anomalies did not to find an association for distal cases, but did for proximal (RR=1.64 [95% CI=1.33-2.03]). Conclusion This is one of only a few Latin American multicenter studies reporting on the epidemiology of hypospadias in South America in the last two decades. Our data adds to evidence suggesting an increase in some countries in the region at different times. There were also variations in prevalence according to severity. This study adds to literature describing associated anomalies at a hospital-based level.


Subject(s)
Humans , Male , Infant, Newborn , Hypospadias/epidemiology , South America/epidemiology , Time Factors , Severity of Illness Index , Case-Control Studies , Population Surveillance , Prevalence , Regression Analysis , Gestational Age , Hypospadias/physiopathology
20.
National Journal of Andrology ; (12): 908-911, 2017.
Article in Chinese | WPRIM | ID: wpr-812858

ABSTRACT

Objective@#To investigate the clinical effect of modified Snodgrass surgical technique in the treatment of hypospadias.@*METHODS@#We retrospectively analyzed the clinical data about 212 cases of hypospadias treated by urethroplasty from January 2008 to October 2016, 94 with the modified Snodgrass technique, namely with a silk line in addition to the urethral suture to make easier postoperative removal of the suture (group A), and the other 118 with the conventional Snodgrass technique (group B). The urethral suture was removed at 10 days after surgery for the patients in group A. We compared the success rate of surgery and incidence of postoperative complications between the two groups.@*RESULTS@#Compared with group B, group A showed a significantly higher success rate of surgery (81.36% vs 91.49%, P 0.05).@*CONCLUSIONS@#The modified Snodgrass technique can improve the success rate of surgery and reduce the incidence rates of incisional infection and urinary fistula, which deserves wide clinical application.


Subject(s)
Child , Humans , Hypospadias , General Surgery , Incidence , Infant , Male , Postoperative Complications , Epidemiology , Retrospective Studies , Surgical Wound Infection , Epidemiology , Suture Techniques , Urethra , General Surgery , Urethral Stricture , Epidemiology , Urinary Fistula
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