To investigate the clinical course and management of congenital vaginal atresia. This retrospective analysis included patients with congenital vaginal atresia treated from March 2004 to August 2014 at the Obstetrics a...To investigate the clinical course and management of congenital vaginal atresia. This retrospective analysis included patients with congenital vaginal atresia treated from March 2004 to August 2014 at the Obstetrics and Gynecology Hospital of Fudan University. Thirty-nine patients were included in this study. Their average age was 16.87±2.2 years when they came to our hospital. Totally, 51% of the patients had isolated congenital vaginal atresia with a normal cervix, whereas the others had either cervical atresia or imperforate hymen. The primary presenting signs and symptoms included primary amenorrhea（71.8%）, periodic abdominalgia（41.0%）, abdominal pain（36.0%）, dyspareunia（10.3%）, menstrual disorders（5.1%）, and pelvic mass（5.1%）. Ultrasound and magnetic resonance imaging（MRI） were effective inspection methods for the screening of urogenital tract-associated anomalies. Vaginoplasty mainly included simple vagina reconstruction with insertion of a mold（n=22） and split-thickness skin grafting（n=4）. In 64% of surgical patients, normal menstrual bleeding was achieved. Four of the patients subsequently became pregnant and delivered at term. Primary amenorrhea, periodic abdominalgia and abdominal pain are the main reasons for the post pubertal patients to visit doctors. Surgical methods can successfully provide these patients an opportunity for subsequent conservative management, can result in normal menstrual bleeding, resolve cyclic pelvic pain, and provide some potential for fertility.展开更多
Importance:Type D esophageal atresia(EA)with tracheoesophageal fistula(TEF)is characterized by EA with both proximal and distal TEFs.It is a rare congenital anomaly with a very low incidence.Objective:To investigate d...Importance:Type D esophageal atresia(EA)with tracheoesophageal fistula(TEF)is characterized by EA with both proximal and distal TEFs.It is a rare congenital anomaly with a very low incidence.Objective:To investigate diagnostic and treatment strategies for this rare condition.Methods:We retrospectively reviewed the clinicopathological features of patients with EA/TEF treated at our institution between January 2007 and September 2021.Results:Among 386 patients with EA/TEF,14(3.6%)had type D EA/TEF.Only two patients were diagnosed with proximal TEF preoperatively.Seven patients were diagnosed intraoperatively.Five patients were missed for diagnosis during the initial surgery but was later confirmed by bronchoscopy.During the neonatal period,seven patients underwent a one-stage repair of proximal and distal TEF via thoracoscopy or thoracotomy.Due to missed diagnosis and other reasons,the other 7 patients underwent two-stage surgery for repair of the proximal TEF,including cervical incision and thoracoscopy.Ten of the 14 patients experienced postoperative complications including anastomotic leakage,pneumothorax,esophageal stricture,and recurrence.Patients who underwent one-stage repair of distal and proximal TEF during the neonatal period showed a higher incidence of anastomotic leak(4/7).In contrast,only one of seven patients with two-stage repair of the proximal TEF developed an anastomotic leak.Interpretation:Type D EA/TEF is a rare condition,and proximal TEFs are easily missed.Bronchoscopy may aim to diagnose and determine the correct surgical approach.A cervical approach may be more suitable for repairing the proximal TEF.展开更多
食管闭锁并气管食管瘘(esophageal atresia with or without tracheoesophageal fistula,EA/TEF)是一种需要手术修复的威胁生命的先天畸形,但不论是食管闭锁本身、可能存在的严重合并畸形还是手术的实施,仍是对患儿和医者的考验和挑战....食管闭锁并气管食管瘘(esophageal atresia with or without tracheoesophageal fistula,EA/TEF)是一种需要手术修复的威胁生命的先天畸形,但不论是食管闭锁本身、可能存在的严重合并畸形还是手术的实施,仍是对患儿和医者的考验和挑战.随着诊疗方法和技术的发展与提高,尤其是术前对EA的分型诊断、长段缺失型手术成功的积累增多及胸腔镜技术的应用等均取得进展,手术存活率已达到95%,但术后并发症及其处置仍需不断得到重视.展开更多
基金supported by the National Key R&D Program of China(No.2016 YFC1303100)
文摘To investigate the clinical course and management of congenital vaginal atresia. This retrospective analysis included patients with congenital vaginal atresia treated from March 2004 to August 2014 at the Obstetrics and Gynecology Hospital of Fudan University. Thirty-nine patients were included in this study. Their average age was 16.87±2.2 years when they came to our hospital. Totally, 51% of the patients had isolated congenital vaginal atresia with a normal cervix, whereas the others had either cervical atresia or imperforate hymen. The primary presenting signs and symptoms included primary amenorrhea（71.8%）, periodic abdominalgia（41.0%）, abdominal pain（36.0%）, dyspareunia（10.3%）, menstrual disorders（5.1%）, and pelvic mass（5.1%）. Ultrasound and magnetic resonance imaging（MRI） were effective inspection methods for the screening of urogenital tract-associated anomalies. Vaginoplasty mainly included simple vagina reconstruction with insertion of a mold（n=22） and split-thickness skin grafting（n=4）. In 64% of surgical patients, normal menstrual bleeding was achieved. Four of the patients subsequently became pregnant and delivered at term. Primary amenorrhea, periodic abdominalgia and abdominal pain are the main reasons for the post pubertal patients to visit doctors. Surgical methods can successfully provide these patients an opportunity for subsequent conservative management, can result in normal menstrual bleeding, resolve cyclic pelvic pain, and provide some potential for fertility.
基金Natural Science Foundation of Jiangxi,China:Grant/Award Number:81660092Beijing Municipal Science&Technology Commission:Grant/Award Number:Z2102921062。
文摘Importance:Type D esophageal atresia(EA)with tracheoesophageal fistula(TEF)is characterized by EA with both proximal and distal TEFs.It is a rare congenital anomaly with a very low incidence.Objective:To investigate diagnostic and treatment strategies for this rare condition.Methods:We retrospectively reviewed the clinicopathological features of patients with EA/TEF treated at our institution between January 2007 and September 2021.Results:Among 386 patients with EA/TEF,14(3.6%)had type D EA/TEF.Only two patients were diagnosed with proximal TEF preoperatively.Seven patients were diagnosed intraoperatively.Five patients were missed for diagnosis during the initial surgery but was later confirmed by bronchoscopy.During the neonatal period,seven patients underwent a one-stage repair of proximal and distal TEF via thoracoscopy or thoracotomy.Due to missed diagnosis and other reasons,the other 7 patients underwent two-stage surgery for repair of the proximal TEF,including cervical incision and thoracoscopy.Ten of the 14 patients experienced postoperative complications including anastomotic leakage,pneumothorax,esophageal stricture,and recurrence.Patients who underwent one-stage repair of distal and proximal TEF during the neonatal period showed a higher incidence of anastomotic leak(4/7).In contrast,only one of seven patients with two-stage repair of the proximal TEF developed an anastomotic leak.Interpretation:Type D EA/TEF is a rare condition,and proximal TEFs are easily missed.Bronchoscopy may aim to diagnose and determine the correct surgical approach.A cervical approach may be more suitable for repairing the proximal TEF.
文摘食管闭锁并气管食管瘘(esophageal atresia with or without tracheoesophageal fistula,EA/TEF)是一种需要手术修复的威胁生命的先天畸形,但不论是食管闭锁本身、可能存在的严重合并畸形还是手术的实施,仍是对患儿和医者的考验和挑战.随着诊疗方法和技术的发展与提高,尤其是术前对EA的分型诊断、长段缺失型手术成功的积累增多及胸腔镜技术的应用等均取得进展,手术存活率已达到95%,但术后并发症及其处置仍需不断得到重视.
