A transposição penoescrotal (PST) é uma anomalia genital congênita rara em que o escroto está posicionado superior e anterior ao pênis. O PST pode apresentar um amplo espectro de anomalias que vão desde um simples escroto xale (escroto em forma de donut) até uma transposição extrema muito complexa com malformações craniofaciais, do sistema nervoso central, cardíacas, gastrointestinais, urológicas e outras malformações genitais (testículos que não desceram, hipospádia, cordee). Deficiência de crescimento e deficiência intelectual também podem ser observadas (60% dos casos).
Introdução
O que você precisa saber de cara
A transposição penoescrotal (PST) é uma anomalia genital congênita rara em que o escroto está posicionado superior e anterior ao pênis. O PST pode apresentar um amplo espectro de anomalias que vão desde um simples escroto xale (escroto em forma de donut) até uma transposição extrema muito complexa com malformações craniofaciais, do sistema nervoso central, cardíacas, gastrointestinais, urológicas e outras malformações genitais (testículos que não desceram, hipospádia, cordee). Deficiência de crescimento e deficiência intelectual também podem ser observadas (60% dos casos).
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 10 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 20 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Transposição peno-escrotal
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Publicações mais relevantes
First-stage procedure for proximal hypospadias with penile encasement/scrotal transposition (PEST) deformity.
We hereby report Curvature correction, Lateral foreskin Approximation & Scrotal Switch (CLASS) procedure as a first-stage of Penile encasement/scrotal transposition (PEST) deformity & proximal-hypospadias. At first stage, the encased penis was released by making two inverted "U" shaped incisions on either side of two hemi scrota; the dorsal foreskin was left attached to glans; lateral foreskin flaps, straightened at the coronal sulcus, were approximated in the midline after chordee correction. Fifteen patients with perineal hypospadias & PEST deformity underwent CLASS procedure and second stage with good success. Further large prospective studies are essential to validate this procedure.
Evaluation of penoscrotal transposition associated with complex hypospadias and concealed penis: Photographically controlled analysis of surgically treated patients and proposal of a novel classification (The Corrêa-Macedo classification).
This study aimed to propose and validate a novel classification for penoscrotal transposition and estimate the frequency of its different degrees in patients with complex hypospadias, buried penis, and in controls. This was a retrospective, descriptive, longitudinal study of consecutive cases, based on standardized photographic evaluation of patients operated between 2010 and 2021. The study included 23 patients with complex hypospadias and 50 patients with buried penis. Additionally, a prospective control group of 90 children without genital anomalies was analyzed. The proposed classification was compared to existing systems using appropriate statistical tests, validated by expert reviewers, and subsequently applied to the clinical subgroups and the control group. We demonstrated an accuracy of 87.1 %, significantly higher than that of previously established methods (p < 0.0001). Moderate penoscrotal transposition predominated among patients with buried penis (68 %), while severe transposition was more frequent in cases of complex hypospadias (60.9 %). In the control group, mild (41.1 %) and moderate (33.3 %) forms of transposition were observed, even in the absence of diagnosed genital anomalies. The predominance of moderate and severe forms among surgical patients reinforces the etiopathogenic association between penoscrotal transposition, complex hypospadias, and buried penis. The presence of varying degrees of transposition in children without genital anomalies highlights the need for systematic evaluation of scrotal positioning in children and question what is "the normal scrotal insertion" in the general population.
Single-stage Reconstruction of Bifid Scrotum and Penoscrotal Transposition without Hypospadias and Chordee with Modified Glenn-Anderson Technique: A Case Report.
Bifid scrotum and penoscrotal transposition are rare congenital scrotal anomalies. Cases diagnosed without hypospadias and chordee are uncommon. We present a rare case of bifid scrotum and incomplete penoscrotal transposition without hypospadias and chordee in a 10-year-old patient. Examination revealed an incomplete transposition of the external genitalia, with the scrotum bifid along the scrotal raphe. The hypoplastic penis was buried at the midpoint of the scrotum. Intraoperatively, no hypospadias or chordee was identified. Single-stage scrotal reconstruction was performed with the modified Glenn-Anderson technique. Post-operatively, the patient recuperated well, with minimal edema. Bifid scrotum and penoscrotal transposition may exhibit broad clinical variations. Different surgical techniques with single- or multiple-stage reconstruction may be performed to achieve good functional and cosmetic outcomes. The modified Glenn-Anderson technique is a feasible single-stage scrotal reconstruction procedure that enables the repair of hypospadias and chordee in the same setting. The post-operative swelling and aesthetic results are acceptable.
Commentary on "A case report of complete penoscrotal transposition and associated anomalies in a newborn" by Nsaho et al.
A case report of complete penoscrotal transposition and associated anomalies in a newborn.
Penoscrotal transposition is a rare congenital abnormality in which the scrotum is positioned above the penis. It results from the disrupted development of the genital tubercle and labioscrotal folds. The exact embryological errors are not fully understood, but they may arise from a failure in their migration and fusion around 12 weeks of gestation. A term male newborn was referred 4 h after birth due to respiratory distress, abnormal genitalia, and failure to pass meconium or urine. He had pulmonary hypertension, early-onset neonatal sepsis, complete penoscrotal transposition, and possibly an anorectal malformation. Evaluation showed elevated creatinine, hyperkalaemia, leucocytosis, and severe pulmonary hypertension. Despite supportive care-including antibiotics, fluids, correction of hyperkalaemia, and sildenafil-he deteriorated and died before surgery. The final diagnosis was a complex congenital anomaly, sepsis, and multiorgan failure leading to death. Persistent scrotal transposition is associated with midline defects, including anorectal, cardiac, and gonadal anomalies. Early prenatal imaging and thorough postnatal assessments-such as echocardiography, renal imaging, and genetic testing-are essential. The complexity of this anomaly requires organised, multidisciplinary management. By focusing on key investigations and prompt interventions, even with limited resources, outcomes can be enhanced and future diagnostic and prognostic strategies improved. A rare complete penoscrotal transposition with multiple anomalies and sepsis highlights the importance of multidisciplinary care and enhanced antenatal screening.
Publicações recentes
First-stage procedure for proximal hypospadias with penile encasement/scrotal transposition (PEST) deformity.
Commentary on "A case report of complete penoscrotal transposition and associated anomalies in a newborn" by Nsaho et al.
Evaluation of penoscrotal transposition associated with complex hypospadias and concealed penis: Photographically controlled analysis of surgically treated patients and proposal of a novel classification (The Corrêa-Macedo classification).
Single-stage Reconstruction of Bifid Scrotum and Penoscrotal Transposition without Hypospadias and Chordee with Modified Glenn-Anderson Technique: A Case Report.
A case report of complete penoscrotal transposition and associated anomalies in a newborn.
📚 EuropePMC70 artigos no totalmostrando 54
First-stage procedure for proximal hypospadias with penile encasement/scrotal transposition (PEST) deformity.
Journal of pediatric urologyCommentary on "A case report of complete penoscrotal transposition and associated anomalies in a newborn" by Nsaho et al.
International journal of surgery case reportsEvaluation of penoscrotal transposition associated with complex hypospadias and concealed penis: Photographically controlled analysis of surgically treated patients and proposal of a novel classification (The Corrêa-Macedo classification).
Journal of pediatric urologySingle-stage Reconstruction of Bifid Scrotum and Penoscrotal Transposition without Hypospadias and Chordee with Modified Glenn-Anderson Technique: A Case Report.
Journal of plastic and reconstructive surgeryA case report of complete penoscrotal transposition and associated anomalies in a newborn.
International journal of surgery case reportsA rare case of complex perineal anomaly in a male neonate: Multistage surgical management.
Urology case reportsPhenotypic Characterization of Seven Pediatric Patients Diagnosed With KAT6B -Related Disorders: Case Series and Review of the Literature.
American journal of medical genetics. Part AThe short-term efficacy of modified plate reconstruction and tubularization urethroplasty for posterior hypospadias.
Journal of pediatric urologyRepair of the Bulbospongiosus Muscle to Suspend the Penis to the Pubic Bones in Proximal Hypospadias.
Journal of pediatric surgeryPenoscrotal transposition: Long-term outcome in 29 patients.
Archivio italiano di urologia, andrologia : organo ufficiale [di] Societa italiana di ecografia urologica e nefrologicaNew classification of the penoscrotal positional anomalies.
Birth defects researchBurden of Delayed Circumcision on Healthcare Systems: a Retrospective Study.
Medical archives (Sarajevo, Bosnia and Herzegovina)An epidemiologic study of penoscrotal transposition by maternal characteristics using data from the Texas birth defects registry.
Birth defects researchPatient Perspectives and Outcomes in Adult Hypospadias Repair.
CureusKlinefelter syndrome with penoscrotal transposition and diphallia: A case study.
Congenital anomaliesThe Role of Tissue Expanders in the Reconstruction of Proximal Hypospadias.
UrologyEctopic scrotum and penoscrotal transposition: Case report and literature review.
Frontiers in pediatricsEvaluation of tunica vaginalis flap-covering combined with modified Glenn-Anderson in one-stage repair of proximal hypospadias with incomplete penoscrotal transposition.
Frontiers in pediatricsAnalysis of factors associated with postoperative complications after primary hypospadias repair: a retrospective study.
Translational andrology and urologyCloaca-Like Anomalies in the Male: A Report on Two Cases.
European journal of pediatric surgery reportsSpondylocostal dysplasia and brachydactyly associated with TBX6 and IHH variants: A case report.
Clinical case reportsClinical characteristics, socioeconomic factors and COVID-19 were associated with delayed surgery in children with hypospadias: a retrospective study of 4439 cases in a single center.
European journal of medical research[Effectiveness of penis up transfer method for penoscrotal transposition in children].
Zhongguo xiu fu chong jian wai ke za zhi = Zhongguo xiufu chongjian waike zazhi = Chinese journal of reparative and reconstructive surgery46 XY disorder of sex development (DSD) due to 5 alpha (SRD5A2) deficiency - Experience from a multidisciplinary Pediatric Gender Clinic.
Journal of pediatric urologySurgical Correction of True Diphallia in a Newborn Male.
UrologyProximal Hypospadias and Acquired Cryptorchidism: Incidence, Morphology and Potential Clinical Implications.
The Journal of urologyA different approach to distal hypospadias repair: The GUD (glandular urethral disassembly) technique.
Journal of pediatric urologyComplete Penoscrotal Transposition with Other Extragenital Anomalies in a Neonate Delivered at Term.
Case reports in urologyBladder Agenesis and Bilateral Ectopic Ureters in an Infant Male With Cystic Renal Dysplasia, Imperforate Anus, and Penoscrotal Transposition.
UrologyPrenatal Diagnosis and Classification of Fetal Hypospadias: The Role and Value of Magnetic Resonance Imaging.
Journal of magnetic resonance imaging : JMRITrue partial diphallia with associated penoscrotal transposition of two hemi-scrotums.
Radiology case reportsLong-Term Undesirable Consequences of Penile Skin Island Flap to Correct Penoescrotal Transposition: A Case Report and Review of Literature.
Case reports in urologyMegaprepuce: A New Surgical Technique with Excellent Cosmetic and Functional Results.
Journal of laparoendoscopic & advanced surgical techniques. Part AMEIS2 sequence variant in a child with intellectual disability and cardiac defects: Expansion of the phenotypic spectrum and documentation of low-level mosaicism in an unaffected parent.
American journal of medical genetics. Part AA simple way to treat penile concealing due to webbed penis.
International braz j urol : official journal of the Brazilian Society of UrologyPrenatal diagnosis of complete penoscrotal transposition with normal scrotum: Two case reports and review of the literature.
Journal of clinical ultrasound : JCUMid- to long-term outcomes of the 'anatomical approach' to congenital megaprepuce repair.
Journal of pediatric urologyEctopias of the kidney, urinary tract organs, and male genitalia.
Der PathologeIncidence and diagnoses of disorders of sex development in proximal hypospadias.
Journal of pediatric surgeryKeloid formation after pediatric male genital surgeries: an uncommon and difficult problem to manage.
Journal of pediatric urologyA Patient with Third Corpus Cavernosum, Severe Penile Ventral Deviation and Partial Peno-scrotal Transposition: First Worldwide Report.
Urologia internationalis[Ectopias of the kidney, urinary tract organs, and male genitalia. German version.].
Der PathologePenoscrotal Transposition to Achieve Urethral Continuity After Long-Segment Urethral Defect: A Case Report.
American journal of men's healthCan sacral development as a marker for caudal regression help identify associated urologic anomalies in patients with anorectal malformation?
Journal of pediatric surgeryProximal Hypospadias and a Novel WT1 Variant: When Should Genetic Testing Be Considered?
PediatricsA rare case of complete penoscrotal transposition with hypospadias in a newborn.
Turkish journal of obstetrics and gynecologySurgical correction of ectopic penis and scrotum associated with bilateral orchidopexy.
Einstein (Sao Paulo, Brazil)[Plasty method for penoscrotal transposition].
Zhonghua zheng xing wai ke za zhi = Zhonghua zhengxing waike zazhi = Chinese journal of plastic surgeryModification of the Koyanagi Technique for the Single-Stage Repair of Proximal Hypospadias.
Annals of plastic surgeryA New Modification of the Koyanagi Technique for the One-stage Repair of Severe Hypospadias.
UrologyThe contemporary role of 1 vs. 2-stage repair for proximal hypospadias.
Translational andrology and urologyPrenatal Features of Bladder Agenesis in a Female Fetus with Genital Transposition.
Fetal and pediatric pathologyPrepubertal diagnosis of Klinefelter syndrome due to penoscrotal malformations: Case report and review of literature.
Canadian Urological Association journal = Journal de l'Association des urologues du CanadaFurther analysis of the Glans-Urethral Meatus-Shaft (GMS) hypospadias score: correlation with postoperative complications.
Journal of pediatric urologyAssociações
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- First-stage procedure for proximal hypospadias with penile encasement/scrotal transposition (PEST) deformity.
- Evaluation of penoscrotal transposition associated with complex hypospadias and concealed penis: Photographically controlled analysis of surgically treated patients and proposal of a novel classification (The Corrêa-Macedo classification).
- Single-stage Reconstruction of Bifid Scrotum and Penoscrotal Transposition without Hypospadias and Chordee with Modified Glenn-Anderson Technique: A Case Report.
- Commentary on "A case report of complete penoscrotal transposition and associated anomalies in a newborn" by Nsaho et al.
- A case report of complete penoscrotal transposition and associated anomalies in a newborn.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:2842(Orphanet)
- MONDO:0017285(MONDO)
- GARD:4273(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q55786963(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
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