Shaul Donald B, Monforte Hector L, Levitt Marc A, Hong Andrew R, Peña Alberto
Childrens Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, CA 90027, USA.
J Pediatr Surg. 2005 Jan;40(1):188-91. doi: 10.1016/j.jpedsurg.2004.09.027.
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.
Twenty-two 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 a wider 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 re-excised 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.
本研究的目的是回顾肛门直肠畸形（ARM）患者中遇到的各种类型会阴肿物的手术治疗结果。
对两家大型儿科肛门直肠转诊中心进行回顾性研究。
在15年期间接受ARM治疗的2000多名患者中，确定了22例患有会阴肿物。这22例患者（4例男性）代表了ARM的所有严重程度级别。病变有3种类型：脂肪瘤（n = 10）、血管畸形（n = 4）和错构瘤/迷芽瘤（n = 8）。在经会阴后矢状入路肛门直肠成形术中，脂肪瘤被小心地从肌纤维间切除。血管畸形（4例中有3例为血管瘤）术前行磁共振成像检查，但未发现有深部浸润，均在经会阴后矢状入路肛门直肠成形术时切除。错构瘤/迷芽瘤均发生于女性，50%为带蒂肿物，起源于外阴。病变包含神经胶质、类骨质、肾源性残余和宫颈内膜样黏膜等组织。1例最初被误诊为畸胎瘤，促使进行了更广泛的切除。此后所有患者经病理正确诊断为错构瘤或迷芽瘤，未进行广泛切除。随访时间为5个月至12年。10例脂肪瘤患者中有6例控便良好。1例血管畸形患者再次接受切除，另1例有轻微伤口裂开。错构瘤/迷芽瘤病变均无复发。
异常会阴肿物的存在会增加ARM的复杂性；然而，大多数这些病变可以小心切除，同时保留肌肉复合体并最终实现控便。错构瘤性病变可能被误诊为畸胎瘤，但不需要进行切缘清晰的广泛切除。
