腹腔镜检查与超声波检查在评估性别分化障碍男性患者残余穆勒氏管方面的比较

IF 0.5 Q4 PEDIATRICS

Egyptian Pediatric Association Gazette Pub Date : 2024-05-27 DOI:10.1186/s43054-024-00256-4

Mohamed Sayed Abd El-Monsif, Noha Arafa, Mahmoud Marei Marei, Gamal Eltagy, Ahmed M. K. Wishahy

{"title":"腹腔镜检查与超声波检查在评估性别分化障碍男性患者残余穆勒氏管方面的比较","authors":"Mohamed Sayed Abd El-Monsif, Noha Arafa, Mahmoud Marei Marei, Gamal Eltagy, Ahmed M. K. Wishahy","doi":"10.1186/s43054-024-00256-4","DOIUrl":null,"url":null,"abstract":"The diagnosis of male differences of sex development is a challenging multidisciplinary team task, that requires external genital evaluation, karyotyping, hormonal profiling, radiological work up and frequently diagnostic laparoscopy and biopsy, for evaluation of internal duct system and nature of gonads. The debate still persists regarding the best diagnostic modality for accurate visualization of Müllerian duct remnants (MDRs) in those patients. The aim of the study was to compare between laparoscopy (L) and ultrasonography (US) regarding the diagnostic accuracy in detection of Müllerian duct remnants, in addition to describing their anatomical nature and relations with the male duct system, in patients with male DSD, with various karyotypes. We prospectively included 20 patients with male DSD, mostly due to 46 XY DSD or chromosomal DSD, over 2 years. The medical and radiological data were collected and analyzed. The age at the first diagnostic intervention ranged from 8 to 24 months (mean: 17 months). There were 14 patients with 46XY DSD with variable diagnoses (3 ovotesticular DSD, 3 partial gonadal dysgenesis, 6 persistent Müllerian duct remnants syndrome and 2 mixed gonadal dysgenesis). Two patients with 46XX DSD were included (one XX male, and one patient with ovotesticular DSD). One patient with chimerism (46XY/46XX) and three patients with 46XY/45XO mixed gonadal dysgenesis were also recruited. MDRs were evident in all cases (100%) by laparoscopy, only 25% (n = 5) were visualized by US. There was a statistically significant difference between laparoscopy and US regarding gonadal and MDR visualization, being higher with laparoscopy (p values, 0.0180 and 0.001). Ultrasonography failed to visualize Müllerian remnants in 75% of patients with complex DSD. On the other hand, laparoscopy provided optimum visualization of MDRs and gonads in those children.","PeriodicalId":43064,"journal":{"name":"Egyptian Pediatric Association Gazette","volume":null,"pages":null},"PeriodicalIF":0.5000,"publicationDate":"2024-05-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Laparoscopy versus ultrasonography for the evaluation of Müllerian duct remnants in male patients with disorder of sex differentiation\",\"authors\":\"Mohamed Sayed Abd El-Monsif, Noha Arafa, Mahmoud Marei Marei, Gamal Eltagy, Ahmed M. K. Wishahy\",\"doi\":\"10.1186/s43054-024-00256-4\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"The diagnosis of male differences of sex development is a challenging multidisciplinary team task, that requires external genital evaluation, karyotyping, hormonal profiling, radiological work up and frequently diagnostic laparoscopy and biopsy, for evaluation of internal duct system and nature of gonads. The debate still persists regarding the best diagnostic modality for accurate visualization of Müllerian duct remnants (MDRs) in those patients. The aim of the study was to compare between laparoscopy (L) and ultrasonography (US) regarding the diagnostic accuracy in detection of Müllerian duct remnants, in addition to describing their anatomical nature and relations with the male duct system, in patients with male DSD, with various karyotypes. We prospectively included 20 patients with male DSD, mostly due to 46 XY DSD or chromosomal DSD, over 2 years. The medical and radiological data were collected and analyzed. The age at the first diagnostic intervention ranged from 8 to 24 months (mean: 17 months). There were 14 patients with 46XY DSD with variable diagnoses (3 ovotesticular DSD, 3 partial gonadal dysgenesis, 6 persistent Müllerian duct remnants syndrome and 2 mixed gonadal dysgenesis). Two patients with 46XX DSD were included (one XX male, and one patient with ovotesticular DSD). One patient with chimerism (46XY/46XX) and three patients with 46XY/45XO mixed gonadal dysgenesis were also recruited. MDRs were evident in all cases (100%) by laparoscopy, only 25% (n = 5) were visualized by US. There was a statistically significant difference between laparoscopy and US regarding gonadal and MDR visualization, being higher with laparoscopy (p values, 0.0180 and 0.001). Ultrasonography failed to visualize Müllerian remnants in 75% of patients with complex DSD. On the other hand, laparoscopy provided optimum visualization of MDRs and gonads in those children.\",\"PeriodicalId\":43064,\"journal\":{\"name\":\"Egyptian Pediatric Association Gazette\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":0.5000,\"publicationDate\":\"2024-05-27\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Egyptian Pediatric Association Gazette\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1186/s43054-024-00256-4\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"PEDIATRICS\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Egyptian Pediatric Association Gazette","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1186/s43054-024-00256-4","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"PEDIATRICS","Score":null,"Total":0}

引用次数: 0

摘要

男性性发育差异的诊断是一项具有挑战性的多学科团队工作，需要进行外生殖器评估、核型分析、激素分析、放射学检查，并经常需要进行诊断性腹腔镜检查和活组织检查，以评估内导管系统和性腺的性质。关于如何采用最佳诊断方法准确观察这些患者的缪勒氏管残余（MDR），目前仍存在争议。本研究旨在比较腹腔镜检查（L）和超声波检查（US）在检测男性DSD患者残余缪勒管方面的诊断准确性，同时描述残余缪勒管的解剖性质及其与男性生殖管道系统的关系。我们对20名男性DSD患者进行了为期两年的前瞻性研究，其中大部分是46 XY DSD或染色体DSD患者。我们收集并分析了医学和放射学数据。首次接受诊断干预的患者年龄从 8 个月到 24 个月不等（平均：17 个月）。14 名 46XY DSD 患者的诊断结果各不相同（3 名卵巢 DSD、3 名部分性腺发育不良、6 名持续性穆勒氏管残留综合征和 2 名混合性性腺发育不良）。其中包括两名 46XX DSD 患者（一名 XX 男性，一名卵巢 DSD 患者）。此外，还招募了一名嵌合体（46XY/46XX）患者和三名 46XY/45XO 混合性性腺发育不良患者。所有病例（100%）的腹腔镜检查均可发现多发性生殖器发育不良，只有 25% 的病例（5 例）的腹腔镜检查可发现多发性生殖器发育不良。腹腔镜检查和超声检查在性腺和多发性生殖器发育不良的可视化程度上有显著的统计学差异，腹腔镜检查的可视化程度更高（P值分别为0.0180和0.001）。在75%的复杂DSD患者中，超声波检查未能观察到缪勒氏残余。而腹腔镜检查则能最佳地观察到这些儿童的MDR和性腺。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

查看原文本刊更多论文

Laparoscopy versus ultrasonography for the evaluation of Müllerian duct remnants in male patients with disorder of sex differentiation

The diagnosis of male differences of sex development is a challenging multidisciplinary team task, that requires external genital evaluation, karyotyping, hormonal profiling, radiological work up and frequently diagnostic laparoscopy and biopsy, for evaluation of internal duct system and nature of gonads. The debate still persists regarding the best diagnostic modality for accurate visualization of Müllerian duct remnants (MDRs) in those patients. The aim of the study was to compare between laparoscopy (L) and ultrasonography (US) regarding the diagnostic accuracy in detection of Müllerian duct remnants, in addition to describing their anatomical nature and relations with the male duct system, in patients with male DSD, with various karyotypes. We prospectively included 20 patients with male DSD, mostly due to 46 XY DSD or chromosomal DSD, over 2 years. The medical and radiological data were collected and analyzed. The age at the first diagnostic intervention ranged from 8 to 24 months (mean: 17 months). There were 14 patients with 46XY DSD with variable diagnoses (3 ovotesticular DSD, 3 partial gonadal dysgenesis, 6 persistent Müllerian duct remnants syndrome and 2 mixed gonadal dysgenesis). Two patients with 46XX DSD were included (one XX male, and one patient with ovotesticular DSD). One patient with chimerism (46XY/46XX) and three patients with 46XY/45XO mixed gonadal dysgenesis were also recruited. MDRs were evident in all cases (100%) by laparoscopy, only 25% (n = 5) were visualized by US. There was a statistically significant difference between laparoscopy and US regarding gonadal and MDR visualization, being higher with laparoscopy (p values, 0.0180 and 0.001). Ultrasonography failed to visualize Müllerian remnants in 75% of patients with complex DSD. On the other hand, laparoscopy provided optimum visualization of MDRs and gonads in those children.

求助全文

通过发布文献求助，成功后即可免费获取论文全文。去求助

来源期刊

Egyptian Pediatric Association Gazette PEDIATRICS-

自引率

0.00%

发文量

审稿时长

9 weeks

期刊介绍： The Gazette is the official journal of the Egyptian Pediatric Association. The main purpose of the Gazette is to provide a place for the publication of high-quality papers documenting recent advances and new developments in both pediatrics and pediatric surgery in clinical and experimental settings. An equally important purpose of the Gazette is to publish local and regional issues related to children and child care. The Gazette welcomes original papers, review articles, case reports and short communications as well as short technical reports. Papers submitted to the Gazette are peer-reviewed by a large review board. The Gazette also offers CME quizzes, credits for which can be claimed from either the EPA website or the EPA headquarters. Fields of interest: all aspects of pediatrics, pediatric surgery, child health and child care. The Gazette complies with the Uniform Requirements for Manuscripts submitted to biomedical journals as recommended by the International Committee of Medical Journal Editors (ICMJE).