小儿Currarino综合征的临床特点

笔者报告我院近年收治的 2例Cur rarino综合征的患儿 ,重点讨论其临床特点 ,以期引起临床医生的重视。临床资料一、文献资料Currarino综合征是一种先天性畸形 ,它包括肛门直肠畸形、骶骨畸形和骶骨前肿物 ;多数发病在 10岁以前 ,所有病例均存在骶骨畸形 ,骶骨前肿物多数为脊膜膨出和畸胎瘤 ,可伴有其他畸形[1 4] 。二、病例资料例 1:男 ,生后即发现无肛门 ,在我院诊断为高位肛门闭锁 ,生后 9d即行结肠造瘘术 ;生后 3个月 ,在我院行肛门成形术时 ,经骶尾部X线检查发现骶骨畸形 ,MRI检查显示骶骨前肿物、脊髓栓系 ,诊断为Currarino综合征 ,…     

Currarino综合征合并先天性巨结肠病二例报道及文献复习

目的 探讨儿童Currarino综合征合并先天性巨结肠病的诊断与治疗.方法 回顾性分析2009年9月至2014年12月我科收治的2例Currarino综合征合并先天性巨结肠病患儿的临床资料.检索Pubmed、Springer Link、Google Scholar、中国生物医学文献数据库(CBM)、中国知网(CNKI)、万方和维普数据库,对2016年3月10日前报道的Currarino综合征合并先天性巨结肠病例进行文献复习.结果 11岁女童生后发现低位肛门闭锁外院行肛门成形术,术后存在持续性便秘;3岁时经直肠壁组织学检查证实巨结肠病行根治术时发现骶前肿物一并切除;11岁时MRI检查示骶前肿物复发伴骶骨发育不全,明确Currarino综合征合并先天性巨结肠病诊断,复发骶前肿物再次切除.2个月大男童生后发现肛门狭窄,腰骶椎MRI示骶前畸胎瘤,伴骶骨发育不全.因持续腹胀,直肠壁活检证实合并先天性巨结肠,2.5个月时行骶前畸胎瘤,先天性巨结肠一期根治.通过文献检索,最终有16例纳入本次研究.其中,男5例,女9例,2例未描述;均有骶骨畸形,骶前肿物,包括脊膜膨出7例(其中合并畸胎瘤,表皮样囊肿各1例),畸胎瘤6例,皮样束肿、错构瘤、脂肪瘤各1例;6例有脊髓栓系,除未描述病例外其余8例均为短段型无神经细胞节症.结论 Currarino综合征合并先天性巨结肠病十分罕见.术前充分认识能明确诊断,手术治疗后预后较好.     

肛门闭锁术后再手术的原因及对策CSCD

目的 分析肛门闭锁术后再手术的原因,探讨如何避免及处理手术相关并发症。方法 回顾性分析2009年12月至2015年1月25例肛门闭锁行肛门成形术后再手术患儿的临床资料。其中,男23例,女2例;年龄12 d～9岁;合并大便失禁8例,存在大小便失禁2例。6例直肠黏膜脱垂患儿行脱垂直肠切除,1例同时行肛门重建术;12例巨直肠患儿中11例行巨直肠切除术,其中1例同时行Malone手术,1例同时行脱垂直肠切除和Malone手术,1例同时行尿道憩室切除和Malone手术,另外1例仅行Malone手术;2例直肠尿道瘘行经会阴瘘修补术;1例前置肛门行肛门重建术;2例骶尾部直肠切口瘘行肠造瘘术;1例肛周炎性肉芽肿行肉芽肿切除术;1例粘连性肠梗阻行肠粘连松解术。大便失禁者术前、术后行肛门功能评分并行配对t检验。结果 患儿平均术后3.48 d开始无渣饮食,平均术后住院时间6.76 d。术后出现切口感染1例。随访6个月～3年,除2例患儿仍存在排便功能障碍,余患儿无大小便功能障碍及远期并发症发生。大便失禁者术后肛门功能评分（7.75分）明显高于术前（4.20分）,差异有统计学意义（t＝－8.631,P〈0.01）。结论 首次手术合理的手术方案、术中精细操作可以减少术后并发症,降低再手术的发生率。直肠脱垂和巨直肠是肛门闭锁术后再手术的主原因;再次手术根据具体情况综合评估制定合理的手术方案可获得良好疗效。     

小儿Currarino综合征的诊治经验

目的总结小儿Currarino综合征的发病特点、诊断和手术方法。方法收集2008年1月至2020年6月首都医科大学附属北京儿童医院普外科收治的24例Currarino综合征患儿的相关资料。其中, 男7例, 女17例;年龄为(19.58±14.37)个月, 年龄范围为2～47个月。患儿肛门直肠畸形包括直肠会阴瘘16例、肛门狭窄2例、直肠前庭瘘3例、肛门闭锁1例、直肠尿道球部瘘1例及一穴肛1例。14例患儿因排便困难就诊, 45.8%(11/24)的患儿来本科就诊前存在诊治不当的情况。所有患儿均进行腰骶椎X线、骶尾部超声、骶尾部磁共振成像(magnetic resonance imaging, MRI)及下消化道造影检查。手术治疗采用经后矢状入路(经典后矢状入路8例、局限性后矢状入路13例)、肛门入路(1例)、前矢状入路(1例)和经骶后弧形切口切除臀部瘘管及包块(1例)。15例肠造瘘患儿术后3～6个月进行关瘘术。随访评估并记录每例患儿的排便和排尿功能, 排便功能评估采用Rintala评分。结果 2例患儿存在神经源性膀胱, 5例患儿存在生殖系统畸形(双子宫1例, 双角子宫1例, 双阴道2例, ...     

腹腔镜在高位肛门闭锁成形术中应用价值的探讨

目的 探讨腹腔镜辅助下对高位肛门闭锁患儿行Ⅰ期肛门成形术的价值。方法 先天性高位肛门闭锁患儿10例，男孩8例，年龄分别为1～3d的4例；年龄6个月并在新生儿期行乙状结肠造瘘的患儿4例；女孩2例，年龄分别为1d和3个月，为合并直肠闭锁的肛门闭锁和一穴肛畸形。对于6例无造瘘患儿，术中首先腹腔镜监视下，游离直肠，分离结扎断离直肠尿道(阴道)瘘管；然后通过盆底肌肉中心形成隧道，将直肠从中脱出，与会阴皮肤吻合。对于行乙状结肠造瘘手术后的4例患儿，腹腔镜下关闭断离瘘管后，切除造瘘口以远的直肠，将近端的结肠从盆底肌肉中心形成隧道脱出。结果 10例患儿均在腹腔镜辅助下Ⅰ期完成肛门成形术，腹腔镜手术操作时间平均67．8min(45～90min)，出血量平均8．3ml(5～20m1)，无需要输血者。未出现明显的术中并发症，所有患儿术后恢复顺利，于术后11d去除尿管和膀胱造瘘管，无尿道瘘憩室发生，1例乙状结肠造瘘手术后的患儿发生腹部切口感染。术后随访3至26个月，1例患儿手术后1个月出现肛门狭窄，于术后3个月行狭窄段切除术治愈。另外9例无并发症发生，目前患儿排便控制功能良好。结论 腹腔镜辅助肛门成形术是治疗高位肛门闭锁有效方法，有手术打击小、处理直肠泌尿系瘘方便、辨认盆底肌中心准确、对肌肉损伤小等优点，免除患儿分期手术之苦。     

腹腔镜下肛门成形术治疗直肠肛门畸形的并发症分析

目的 探讨腹腔镜下肛门成形术治疗直肠肛门畸形的并发症的产生原因和应对方法.方法 总结分析本中心2010年1月至2015年12月共腹腔镜手术治疗的直肠肛门畸形92例,全部为男性患儿,其中直肠膀胱颈瘘23例,直肠前列腺球部瘘35例,直肠尿道瘘34例.全部患儿均行横结肠造瘘或乙状结肠造瘘的分期手术.术后平均随访时间2.5年(4个月～5年),统计术后并发症和肛门排便情况,其中31例患儿随访大于3年.结果 术后直肠回缩11例,有1例再次手术缝合,另10例保守治疗.术后直肠黏膜外翻7例,均为早期病例,有4例再次手术切除局部外翻黏膜,有3例游离拖出结肠切除部分后再次肛门成形.腹腔残余感染4例,均经抗感染后好转.在已随访3年以上的31例患儿中术后排便失禁3例,其中有1例患儿由于严重瘢痕狭窄予扩肛治疗,另外有2例患儿伴发脊髓栓系.结论 虽然手术并发症难以完全避免,但注意围术期处理和手术技巧,能减少术后并发症的发生.     

治疗锁肛的术式-后矢状入路骶会阴肛门成形术（Pena术）

后矢状人路骶会阴肛门成形术即Pena术，是治疗锁肛的一种术式，适应于患儿已行结肠造瘘术或新生儿期的高位、中位肛门闭锁合并直肠尿道瘘或直肠阴道瘘的肛门闭锁、直肠闭锁。     

肛门直肠畸形-骶骨畸形-骶前肿物四例

近年来,由于对肛门直肠畸形疾病的诊断和治疗不断深入的研究,对其伴发畸形也有进一步了解和深入研究,尤其对肛门直肠畸形-骶骨畸形-骶前肿物(Currarino’s三联征)的认识有了较大提高,现将我院进行尸检的16例肛门直肠畸形病例中,有Currarino’s三联征3例和1例手术治疗的病例,现报道如下: 例1:男,高位肛门直肠发育不全、     

顽固性便秘 肛门直肠畸形 骶前肿物骶骨发育不良

患儿，女，3个月，因“无肛舟状窝瘘行结肠造瘘术后发现盆腔内肿物2月余”入院。患儿出生后家长即发现其肛门位置异常且较狭窄，未治疗，后渐出现顽固性便秘、最长排便时间每周1次。2个月前因先天性肛门闭锁并直肠舟状窝瘘在当地医院行乙状结肠造瘘，术中发现盆腔内有一脂肪性肿物，未行处理。母孕期身体健康，无孕期毒物及射线接触史，患儿出生无窒息。     

新生儿一期经脐单切口腹腔镜肛门成形术22例分析

目的 评估新生儿一期经脐单切口腹腔镜辅助肛门成形术(single-incision laparoscopic-assisted anorectoplaty,SILAARP)的安全性和疗效.方法 回顾性分析2013年6月至2015年12月间成功施行一期SILAARP新生儿22例的临床资料.其中,直肠尿道前列腺部瘘8例,直肠尿道球部瘘14例;平均年龄42.1 h.术中经脐部2 cm切口,提出乙状结肠近端,于系膜缘对侧沿纵轴切口1 cm,置入16号肛管,经肛管注入生理盐水反复冲洗抽吸远近端肠管至胎便完全清除.细针注射器穿刺扩张小肠抽吸积气,创造腹腔镜操作空间.经脐置入1个5mm腹腔镜和2个3mm传统腹腔镜手术器械,行肛门成形直肠尿道瘘修补术.并与本中心以往行三期SILAARP患儿22例行疗效对比.三期SILAARP患儿中,直肠尿道前列腺部瘘6例,直肠尿道球部瘘16例,与一期SILAARP患儿比较,差异无统计学意义(P=0.5).结果 22例患儿均成功施行一期SILAARP.平均手术时间2.4h,腹腔镜辅助远端直肠拖出平均时间1.7h.一期SILAARP患儿手术时间在肛门闭锁直肠尿道前列腺部瘘患儿为(1.7±0.3)h,在肛门闭锁直肠尿道球部瘘患儿为(1.9±0.4)h;与三期患儿肛门闭锁直肠尿道前列腺部瘘组1.8h和肛门闭锁直肠尿道球部瘘组1.9h比较,差异均无统计学意义(P=0.62,P=0.9).一期SILAARP患儿中位随访时间为18个月(3～33个月),无一例发生术后并发症.术后MR证实远端直肠置于盆底肌中心.20例患儿术后第1天即有排便,2例术后便秘患儿接受1～3个月间断洗肠治疗后可自主排便.结论 新生儿一期SILAARP安全有效,使肛门闭锁治疗达到生后立即根治,“视觉无瘢痕”的高级微创目标,能提高患儿远期生活质量.     

Anorectal malformations associated with a presacral tumor and sacral defect

The triad of anorectal malformation, presacral mass, and sacral bony defect is quite rare. During the past 18 years, five patients with this triad were treated in our institutions. Four had covered anal or anorectal stenosis, while one infant had anorectal agenesis. There was a central defect of the sacral bone in three and sacral hemivertebrae in two patients. An additional case that had an intact sacrum but was otherwise associated with the same anomalies is also reported. In the literature, we could find 51 cases (including 5 cases of our own) that fall under this symptom complex. All patients had a presacral tumor, the commonest being teratomas and meningoceles (20 cases each). Anal or anorectal stenoses were most frequently encountered (46 cases), while malformations such as anorectal agenesis (3) or anorectal stenosis plus rectovaginal fistula (2) were seen less frequently. We therefore contend that the spectrum of this symptom complex is broader than was previously considered. Among these patients, constipation was the most common presenting symptom. Barium enema and computer tomographic studies were helpful in identifying the presence, extent, and nature of the mass. The embryogenesis is discussed, reviewing the clinical and pathological characteristics of these patients.     

Anal canal duplication: a retrospective analysis of 12 cases from two European pediatric surgical departments

Anal canal duplication (ACD) represents an extremely rare intestinal congenital anomaly of unknown origin. Usually evidenced within 2 years of age, nearly 45% of reported cases present associated malformations such as presacral mass, anorectal malformation (ARM) and genitourinary anomalies. The confirmative diagnosis is istopathological, with evidence of an anal mucosal lining (squamous ± transitional epithelium), surrounded from a smooth muscle coat and anal glands. We review a conjoined experience from two European pediatric surgical departments. From 1970 to 2005, 12 patients were observed, seven in Pescara, Italy (1997–2005), five in Barcelona, Spain (1970–2004)—mean age at diagnosis 17.8 months, range 0–60; M:F = 1:11. Clinical presentation, diagnostic-surgical approach, and complications were reviewed. According to clinical presentation, patients could be divided in three age groups: asymptomatic (mean age 4.8 months, six patients—one with an associated complex genitourinary malformation, one with a presacral mature teratoma, one with ACD evidenced hysthologically on a retroanal mass removed during the correction of an ARM), mildly symptomatic—constipation, mucous discharge (mean age 29.2 months, four patients—one with associated presacral ependymoma and intestinal neuronal dysplasia type B, one with presacral mass) and complicated—perineal abscess, recurrent fistula (mean age 34 months, two patients). In 11 cases a perianal orifice was evident (ten posteriorly located). The pelvic-MRI was the preferred diagnostic tool in Pescara (5/7, with presacral mass in two patients), fistulography in Barcelona (5/5), where one presacral mass was discovered intraoperatively. Eleven patients underwent surgical removal of the ACD (five perineal approach, five posterior sagittal approach, and one PSARP). Histopathological findings confirmed the diagnosis in operated cases (11). The parents of the male patient denied the consent to surgical treatment. The only major post-operative complication was a sphincteric insufficiency (one case), surgically treated. When facing a perianal orifice, attention should be paid to ACD, particularly in female patients with coexistent genitourinary or intestinal malformations. Pelvic US and MRI are the gold standard to evidence the not rarely associated presacral mass. Surgical early removal (mucosectomy or perineal/posterior sagittal approach, depending on length of ACD and associated presacral mass) is warranted, also in asymptomatic patients, because of the risk of inflammatory complications and cancer (the latter reported in literature in adults).     

Familial triad of anorectal,sacrococcygeal, and presacral anomalies that includes sacrococcygeal teratomas

We present two Australian families with the triad of anorectal, sacrococcygeal, and presacral anomalies. In one family four members had sacral deformities, three of which were associated with presacral masses and anal stenosis. The presacral masses were: one meningocoele combined with a teratoma; one teratoma; and the third remains undiagnosed and unresected at the patient's request. In the second family, one child had an infected presacral teratoma with a small sacral defect and the father had a similar sacral defect with computed tomographic evidence of a presacral meningocoele and teratoma. The presence of anal stenosis and constipation with a sacral defect should raise the possibility of a presacral mass. The mass may take the form of a meningocoele, teratoma, enteric cyst, or any combination of these. Once detected, other family members should be investigated.     

睾丸鞘膜覆盖对减少尿道下裂再手术后尿道皮肤瘘的效果分析

目的比较在尿道下裂再手术病例中睾丸鞘膜与其他软组织覆盖对防止尿道皮肤瘘的疗效影响。方法回顾性分析2010年9月至2014年4月于本院进行尿道成形和尿瘘修补治疗的尿道下裂再手术患者226例,年龄2岁1个月至34岁9个月(平均年龄9岁5个月)。95例远段尿道裂开,107例尿瘘,28例尿道狭窄,24例尿道憩室,23例残留阴茎下曲,52例为分期手术一期术后。术中未采用软组织覆盖18例,阴囊肉膜瓣覆盖46例,阴茎皮下筋膜瓣覆盖74例,邻近软组织覆盖35例,睾丸鞘膜覆盖53例。术后留置尿管5~14d,随访24~67个月。结果无软组织覆盖组中,5例出现尿瘘(27.8%),阴囊肉膜瓣覆盖组7例出现尿瘘(15.2%),阴茎皮下筋膜瓣覆盖组7例尿瘘(9.5%),以邻近软组织覆盖组4例尿瘘(11.4%),睾丸鞘膜覆盖组中2例出现尿瘘(3.8%)。运用卡方检验对各组进行比较,尿瘘发生率的差异存在统计学意义(x^2=6.978,P=0.008),各组间两两比较,睾丸鞘膜组与无软组织覆盖组间(x^2=8.589,P=0.003)、睾丸鞘膜组与阴囊肉膜组间(x^2=3.863,P=0.049),带蒂筋膜组与无组织覆盖组间(x^2=4.237,P=0.004)差异有统计学意义,其余组间差异统计学意义。结论尿道下裂治疗中应强调对成形尿道进行软组织覆盖,睾丸鞘膜较其他常用软组织能够更有效减少尿道下裂再手术后尿道皮肤瘘的发生几率。     

肛门直肠畸形合并先天性巨结肠3例及文献复习

目的先天性肛门直肠畸形合并先天性巨结肠临床上罕见,常易漏诊。本文对先天性肛门直肠畸形合并先天性巨结肠患者的临床特点、诊疗及预后进行总结,以期提高对此类疾病的认识。方法2003年9月至2019年10月,上海交通大学医学院附属新华医院共收治3例肛门直肠畸形合并巨结肠男性患者,均为无肛直肠会阴瘘;检索万方、维普、中国知网、Pubmed、Medline数据库截至2019年10月已发表的关于肛门直肠畸形合并先天性巨结肠的相关文献,并进行分析。结果本研究中3例均于出生后诊断为肛门直肠畸形,行肛门成形术(2~11 d),后因持续便秘,确诊合并先天性巨结肠,行经腹经肛门Soave手术时患者月龄17~36个月。术后随访2~11年,3例术后污粪症状曾持续数年,经饮食调整、排便训练等保守治疗后排便状况逐渐得到改善,目前排便控制能力良好。通过检索数据库,检索到21篇相关文献(共报道71例患者),联合本中心3例患者,共有74例患者纳入本次研究。其中男42例,女32例,年龄2天至28岁,41例发现合并其他系统畸形。42例生后行结肠造瘘术,21例(21/42,50%)于肛门成形术前诊断合并先天性巨结肠。53例(53/74,71.6%)行肛门成形术后持续出现腹胀、便秘等症状,平均保守治疗8个月后确诊合并先天性巨结肠。74例均行巨结肠根治手术,40例(40/74,54.1%)采用Soave术,18例(18/74,24.3%)采用Duhamel术,8例(8/74,10.8%)采用Swenson术,8例(8/74,10.8%)采用Rehbein术,术后病理结果均证实无神经节细胞。38例进行了随访,排便状况良好。结论对于先天性肛门直肠畸形患者,如术后出现便秘持续并加重且保守治疗无效时,应警惕合并先天性巨结肠的可能;以直肠黏膜活检为依据的病理学诊断依然是诊断的金标准。Soave根治术安全有效,可作为优选的手术方式。     

肛门直肠畸形术后排尿功能障碍的评价和治疗

目的 评价肛门直肠畸形术后排尿功能障碍的原因及治疗对策.方法 肛门直肠畸形术后患儿10例,男7例,女3例,年龄1～12岁.肛门闭锁直肠尿道球部瘘4例,肛门闭锁直肠尿道前列腺部瘘3例,泄殖腔畸形1例(共同管＜3 cm),肛门闭锁并球形结肠1例,肛门闭锁直肠前庭瘘1例.10例患儿均有排尿困难,其中3例伴有尿失禁.MRI显示2例合并脊髓栓系.排泄行膀胱尿道造影显示3例合并左侧输尿管Ⅳ°反流及肾积水,其中1例存在后尿道憩室,无1例发现尿道狭窄.尿动力学检查显示9例膀胱容量及残余尿增加,充盈期逼尿肌压正常,无逼尿肌过度活动,尿流率下降,其中8例逼尿肌收缩力下降,1例逼尿肌收缩力正常.另外1例直肠前庭瘘合并脊髓栓系患儿膀胱容量减少、残余尿增多、尿流率下降,充盈期逼尿肌压升高,合并逼尿肌过度活动.直肠尿道瘘合并后尿道憩室患儿行后矢状入路尿道憩室切除,泄殖腔畸形和直肠尿道前列腺部瘘术后合并输尿管反流患儿行左侧输尿管再植,8例合并神经性膀胱的患儿坚持清洁间歇导尿.结果 随访6个月～5年,泄殖腔畸形患儿1年后仍存在左侧输尿管反流及肾积水,直肠尿道球部瘘合并尿道憩室患儿输尿管反流及肾积水消失,无排尿困难及残余尿,直肠尿道前列腺部瘘合并左侧输尿管Ⅳ°反流及肾积水患儿输尿管反流消失,仍需间歇导尿,其余7例患儿无1例出现上尿路损害.结论 肛门直肠畸形合并脊髓发育不良及手术损伤可导致神经性膀胱.术中直肠尿道瘘处理不当可能导致尿道憩室或尿道狭窄.清洁间歇导尿是神经性膀胱的首要治疗方法,对于后尿道憩室可行尿道憩室切除术.     

Post-operative strictures in anorectal malformation: trends over 15 years

Aim

For decades, paediatric surgeons have employed the standard posterior sagittal anorectoplasty (PSARP) approach to deal with patients with anorectal malformations (ARM). In recent years, we noted an apparent increase in the incidence of anal stricture after surgical repair of ARM following the introduction of laparoscopic pull-through and techniques aiming to preserve the internal sphincter—the internal sphincter sparing approach (ISSA). We decided to analyse our data to find out if these new trends had added to the problem of post-operative strictures.

Methods

All patients with ARM at our institution from January 2000 to December 2015 were identified. A retrospective case note review was carried out. Data collected included patient demographics, type of ARM, operative details, and post-operative outcomes.

Results

114 patients were identified. Ten patients were excluded. Of the remaining 104 children, 48 (46%) were female. Median age was 8.3 (range 1.2–16.8) years. Types of ARM were as follows: perineal fistula (15 patients), anterior stenotic anus (12), imperforate anus without fistula (10), vestibular fistula (32), rectourethral (bulbar) fistula (11), rectourethral (prostatic) fistula (14), rectovesical fistula (7), and cloaca (3). Twenty-seven patients with a perineal fistula or anterior stenotic anus underwent perineal procedures that were variably described by the different operating surgeons. The majority (15 patients) had an anoplasty, 5 had anal transposition, 5 had limited PSARP, and 2 patients had ISSA. Two patients with a cloacal anomaly underwent open cloacal reconstruction. Of the remaining 75 patients, 45 had a PSARP approach, 6 had a laparoscopic-assisted pull-through, and 18 had ISSA. Four girls with vestibular fistula had anal transposition and two boys with imperforate anus without fistula had anoplasty. 15 (14%) children developed anal stricture. Stricture incidence differed according to operation type. PSARP was the most commonly performed procedure, with only 6% developing a stricture. In contrast, 30% of ISSA patients and 50% of children who had laparoscopic pull-through developed a stricture. Strictures also occurred in 11 and 12% of children having anal transposition and anoplasty, respectively.

Conclusion

The laparoscopic-assisted pull-through involves tunnelling the sphincter muscle complex. We found that often the tunnels were not wide enough, resulting in narrowing not just at the ano-cutaneous junction but also at the deeper level. 50% developed strictures. We have modified our technique by ensuring that the tunnels are generous enough to allow the rectum to be pulled through without any resistance. ISSA unfortunately resulted in 30% of our patients developing strictures. This approach, started in 2004, was, therefore, abandoned in 2013. The standard Pena’s PSARP, with or without a laparotomy, has stood the test of time. Any modification of this approach must be carefully thought through and audited meticulously. Strictures can cause significant morbidity, which may need several revisions, and the resulting redo anoplasties run the risk of sphincter damage, ironically which the newer modifications of ISSA were trying to conserve.

     

分期Duplay术治疗合并阴茎阴囊转位的重度尿道下裂

目的：探讨分期原位皮瓣卷管（Duplay）成形尿道术治疗合并阴茎阴囊转位的重度尿道下裂的临床疗效。方法2013年10月至2016年2月我们采用分期 Duplay 术式修复重度尿道下裂21例,中位年龄12（9～24）个月,其中阴囊型9例,会阴型12例,均伴有阴茎阴囊转位。Ⅰ期完成 Byars 术＋Duplay 术＋阴茎阴囊复位术,术后半年以上行Ⅱ期 Duplay 术。患儿均获得随访,中位随访时间8（6～28）个月,均观察并处理并发症,对阴茎外观进行评分。结果重建尿道中位长度5.0（4.0～6.5）cm。Ⅰ期 Duplay 成形尿道中位长度1.5（1.0～2.0）cm,术后阴囊伤口感染2例,其中1例继发根部尿道口狭窄,后行狭窄切开造口。Ⅱ期 Duplay 成形尿道中位长度3.5（3.0～4.5）cm,术后尿道瘘4例（4／21）,其中根部瘘2例,体部瘘1例,冠状沟瘘1例;均已完成尿道瘘修补,术后无再次尿瘘发生;所有病例均无尿道狭窄、尿道憩室等并发症发生。Ⅱ期术后3个月对阴茎外观行改良 HOSE 评分,为17～20分,平均19分。结论分期 Duplay 术治疗合并阴茎阴囊转位的重度尿道下裂安全有效,并发症相对较少,也得到令医患满意的外观。