The aim of this study was to review the outcome of surgical management of various types of perineal masses encountered in patients with anorectal malformations (ARM). Retrospective review from 2 large pediatric anorec...The aim of this study was to review the outcome of surgical management of various types of perineal masses encountered in patients with anorectal malformations (ARM). Retrospective review from 2 large pediatric anorectal referral centers. Twentytwo patients with a perineal mass were identified in more than 2000 patients treated for an ARM over a 15-year period. The 22 patients (4 men) represented all levels of severity of ARMs. The lesions were of 3 types: lipomas (n = 10), vascular anomalies (n = 4), and hamartomas/ choristomas (n = 8). The lipomas were carefully removed from between the muscle fibers during the posterior sagittal anorectoplasty. The vascular anomalies (3 of 4 were hemangiomas) underwent magnetic resonance imaging preoperatively, but none were found to invade deeply and all were excised at the time of the posterior sagittal anorectoplasty. The hamartomas/choristomas all occurred in women, and 50%arose as a pedunculated mass from the vulva. The lesions contained tissues such as glia, osteoid, nephrogenic rests, and endocervical-type mucosa. One was initially misinterpreted as a teratoma, prompting awider excision. This and all subsequent patients have been correctly diagnosed pathologically as having either hamartomas or choristomas, which were not widely excised. Follow-up ranges from 5 months to 12 years. Six of the 10 lipoma patients are continent. One vascular anomaly was reexcised and there was minor wound separation in another. None of the hamartoma/choristoma lesions recurred. The presence of unusual perineal masses can add to the complexity of ARMs; however, most of these lesions can be carefully excised with preservation of the muscle complex and ultimate continence. Hamartomatous lesions can be mistaken for teratomas but do not require aggressive excision with clear margins.展开更多
Background: Magnetic resonance imaging (MRI) of the pelvic floor allows better assessment of pelvic pathology and has a potential as an adjunct for therapy planning. In complex congenital malformations of the pelvic f...Background: Magnetic resonance imaging (MRI) of the pelvic floor allows better assessment of pelvic pathology and has a potential as an adjunct for therapy planning. In complex congenital malformations of the pelvic floor and continence organs,it plays a major role in assessing urinary and fecal incontinence or constipation, especially when performed as a dynamic investigation such as MRI defecography. Patients and Methods:Twenty-three patients (3-21 years old) with urinary and/or feca lincontinence or constipation attributable to congenital malformations of the pelvic region presented at our institution. The diagnoses were anorectal malformations (18), bladder exstrophy(2), and cloacal exstrophy (3). All patients underwent static and dynamic MRI of the pelvic floor with rest, squeeze,and evacuation in supine position. Results: Morphology and function of the pelvic floor and pelvic organs could be demonstrated in each case. The reason for urinary incontinence, fecal incontinence, or constipation could be defined, and an individual therapeutic management concept was made based upon the data obtained by the investigation. Conclusions: The advantages of this method, in comparison to others, are direct visualization of the pelvic floor muscles and continence organs and their anatomical relationship during different functional actions.Pelvic floor dysfunction is often the reason for fecal and urinary incontinence and can be detected by MRI. Especially in children, minimizing radiation exposure is of major concern.Disadvantages are the costs and long investigation time.展开更多
溃疡性结肠炎常见,主要表现为腹痛、腹泻、脓血便.纤维结肠镜检为结肠粘膜弥漫性充血、水肿、糜烂和溃疡;大多数患者以左半结肠病变为主Reddy et al [1]报道,溃疡性结肠炎患者不仅存在粘膜病变,也存在结肠动力异常,表现为推进性小振幅...溃疡性结肠炎常见,主要表现为腹痛、腹泻、脓血便.纤维结肠镜检为结肠粘膜弥漫性充血、水肿、糜烂和溃疡;大多数患者以左半结肠病变为主Reddy et al [1]报道,溃疡性结肠炎患者不仅存在粘膜病变,也存在结肠动力异常,表现为推进性小振幅收缩增多.但直肠肛门动力和感觉功能有何改变尚不清楚。展开更多
文摘The aim of this study was to review the outcome of surgical management of various types of perineal masses encountered in patients with anorectal malformations (ARM). Retrospective review from 2 large pediatric anorectal referral centers. Twentytwo patients with a perineal mass were identified in more than 2000 patients treated for an ARM over a 15-year period. The 22 patients (4 men) represented all levels of severity of ARMs. The lesions were of 3 types: lipomas (n = 10), vascular anomalies (n = 4), and hamartomas/ choristomas (n = 8). The lipomas were carefully removed from between the muscle fibers during the posterior sagittal anorectoplasty. The vascular anomalies (3 of 4 were hemangiomas) underwent magnetic resonance imaging preoperatively, but none were found to invade deeply and all were excised at the time of the posterior sagittal anorectoplasty. The hamartomas/choristomas all occurred in women, and 50%arose as a pedunculated mass from the vulva. The lesions contained tissues such as glia, osteoid, nephrogenic rests, and endocervical-type mucosa. One was initially misinterpreted as a teratoma, prompting awider excision. This and all subsequent patients have been correctly diagnosed pathologically as having either hamartomas or choristomas, which were not widely excised. Follow-up ranges from 5 months to 12 years. Six of the 10 lipoma patients are continent. One vascular anomaly was reexcised and there was minor wound separation in another. None of the hamartoma/choristoma lesions recurred. The presence of unusual perineal masses can add to the complexity of ARMs; however, most of these lesions can be carefully excised with preservation of the muscle complex and ultimate continence. Hamartomatous lesions can be mistaken for teratomas but do not require aggressive excision with clear margins.
文摘Background: Magnetic resonance imaging (MRI) of the pelvic floor allows better assessment of pelvic pathology and has a potential as an adjunct for therapy planning. In complex congenital malformations of the pelvic floor and continence organs,it plays a major role in assessing urinary and fecal incontinence or constipation, especially when performed as a dynamic investigation such as MRI defecography. Patients and Methods:Twenty-three patients (3-21 years old) with urinary and/or feca lincontinence or constipation attributable to congenital malformations of the pelvic region presented at our institution. The diagnoses were anorectal malformations (18), bladder exstrophy(2), and cloacal exstrophy (3). All patients underwent static and dynamic MRI of the pelvic floor with rest, squeeze,and evacuation in supine position. Results: Morphology and function of the pelvic floor and pelvic organs could be demonstrated in each case. The reason for urinary incontinence, fecal incontinence, or constipation could be defined, and an individual therapeutic management concept was made based upon the data obtained by the investigation. Conclusions: The advantages of this method, in comparison to others, are direct visualization of the pelvic floor muscles and continence organs and their anatomical relationship during different functional actions.Pelvic floor dysfunction is often the reason for fecal and urinary incontinence and can be detected by MRI. Especially in children, minimizing radiation exposure is of major concern.Disadvantages are the costs and long investigation time.