尿动力学检查对小儿神经源性膀胱手术方式选择的意义

目的 探讨尿动力学检查对小儿神经源性膀胱手术方式选择的意义.方法 回顾性分析我院手术治疗的神经源性膀胱患儿的资料41例.所有患儿术前完成B型超声、排尿性膀胱尿道造影(voiding cystourethrogram,VCUG)、99mTc-DTPA肾动态显像(DTPA)、尿动力学检查.其中34例行储尿期+排尿期膀胱压力描记.结果 尿动力学检查提示逼尿肌过度活动23例,活动低下11例;括约肌过度活动21例,活动低下13例.根据患儿不同的临床症状,参考尿动力学检查结果,选用不同的手术方式.具体术式包括:回肠代膀胱扩大+阑尾代可控性膀胱流出道(Mitrofanoff)+阑尾代顺行结肠灌洗造瘘(Malone)+膀胱颈悬吊(Sling)2例,回肠代膀胱扩大+Mitrofanoff+ Sling 5例,回肠代膀胱扩大+输尿管再植+ Mitrofanoff+ Sling 7例,回肠代膀胱扩大+Mitrofanoff+右肾切除+Sling 1例,回肠代膀胱扩大+输尿管再植+ Mitrofanoff 18例,回肠代膀胱扩大+Mitrofanoff 2例,输尿管再植+Mitrofanoff 3例,膀胱颈封闭+回肠代膀胱扩大+Malone+回肠卷管代可控性膀胱流出道(Monti)1例,Mitrofanoff 2例.术后39例患儿获得随访,随访时限3个月～5年,7例出现术后并发症,大部分患儿术后恢复满意.结论 小儿神经源性膀胱的手术方式选择主要依据其临床症状以及超声、影像、同位素检查结果,尿动力学检查可明确膀胱尿道功能障碍的类型,对于手术的方案制定也有重要参考意义.     

肾盂输尿管连接部梗阻合并同侧膀胱输尿管连接部梗阻的诊断与治疗

目的 肾盂输尿管连接部梗阻(ureteropelvic junction obstruction,UPJO)和膀胱输尿管连接部梗阻(ureterovesical junction obstruction,UVJO)这两个最常见的儿童泌尿系统病理状态同时存在较少见.本文对单侧UPJO合并同侧UVJO的诊断与治疗进行探讨.方法 回顾性分析上海交通大学医学院附属新华医院2012年1月至2015年7月间手术治疗的UPJO合并UVJO患儿.术前常规行泌尿系超声、同位素利尿肾图(DR)及排泄性膀胱尿道造影(VCUG)等检查.结果 我院共诊治单侧UPJO合并UVJO的43例患儿,失访2例.11例患儿术前明确诊断;41例患儿先行肾盂成形术+肾造瘘术,其中10例术后复查发现输尿管末端狭窄自行缓解,28例再次接受输尿管膀胱再植术,3例患儿行肾盂成形术+肾造瘘术后复查核素提示分肾功能低于10％,给予患肾切除;38例患儿术复查肾积水程度和肾脏功能均得到显著改善.结论 单侧UPJO合并UVJO的术前诊断非常困难,术中应仔细检查,避免遗漏同时合并存在的病理改变.术前尽量通过超声、DR及MR等影像检查作出明确诊断,进而制定个体化的治疗方案,能够获得满意的治疗效果.如术前明确有UPJO的存在,应选择首先进行肾盂成形术,术后根据检查结果决定是否行输尿管膀胱再植术.     

445例先天性尿道下裂的治疗

目的 探讨不同术式对445例不同类型先天性尿道下裂患儿的手术治疗效果。方法 回顾性分析我院1988年—2003年445例先天性尿道下裂病例资料，其中采用阴茎阴囊纵隔带蒂皮瓣尿道成型术311例，包皮内板带蒂皮瓣尿道成型术52例，包皮及阴茎阴囊联合皮瓣尿道成型术61例，膀胱粘膜代尿道术21例。每例均同时行耻骨上膀胱造瘘术。结果 治愈率90．6％，并发症为9．4％。其中尿道狭窄11例，尿瘘31例。尿道狭窄中膀胱粘膜法1例，包皮内板法2例，阴茎阴囊纵隔法5例，联合皮瓣法3例；尿瘘中膀胱粘膜法1例，包皮内板法3例，阴茎阴囊纵隔法24例，联合皮瓣法3例。结论 根据尿道下裂类型选择不同的术式 尿流改道，并严格遵守整形外科的原则是尿道下裂手术成功的关键。     

小儿前尿道瓣膜诊治11例

目的探讨小儿前尿道瓣膜的治疗方法及临床疗效。方法回顾性分析2003年1月至2013年5月作者收治的11例前尿道瓣膜患儿临床资料,患儿均为男性,年龄3个月至8岁,平均2岁8个月,其中年龄1岁以下6例。8例使用7.5F或9F膀胱尿道镜在尿道的4点、6点和8点处切开瓣膜,并留置导尿管10 d。对其中3例合并前尿道憩室的患儿,2例行憩室切除、尿道成形术;1例一期行膀胱造瘘术,二期行憩室切除、尿道成形术。结果 11例均手术顺利,术后均排尿通畅。无尿瘘及尿道海绵体损伤。随访3个月至4年,平均1.5年。尿白细胞消失,阴茎阴囊交界处包块消失。合并肾积水2例,膀胱输尿管反流3例,B超复查均有不同程度缓解。肾功能检查血清尿素、肌酐均正常。结论腔内冷刀切开治疗前尿道瓣膜症是一种可行的方法,疗效确切,术后并发症少,预后好。合并前尿道憩室患儿可选择憩室切除、尿道成形术。     

经肛门治疗Currarino综合征

目的 探讨Currarino综合征(Currarino syndrome,CS)的诊断和经肛门手术治疗的经验.方法 2013年1月至2015年5月,我院共收治11例CS患儿,男2例,女9例,平均年龄11个月.其中7例肛门位置正常,但肛门直肠严重狭窄;1例患儿反复出现肛周感染及肛瘘;3例患儿可见肛门直肠畸形.其中5例患儿术前已行结肠或回肠造瘘术.术前全部患儿行肛门指诊、骶尾部正侧位片、下消化道造影及骶部核磁共振检查.术中患儿经过肛门或瘘口游离直肠,肠管拖出后,向上牵开肠管.将切口后方向左右牵开,可暴露并切除骶前肿物.术后全部患儿给予抗感染治疗,术前已有造瘘患儿术后第1天恢复饮食,无造瘘患儿术后2d恢复饮水,术后4d恢复低渣进食,无并发症患儿术后7d出院,14d扩肛.术后每3个月复查.已有造瘘患儿术后3个月行造瘘还纳术.结果 肛门位置正常患儿全部有直肠肛门严重狭窄,小指不能进入.全部患儿影像学检查可见骶骨畸形和骶前肿物.11例患儿全部顺利完成手术,术后8例恢复良好,1例患儿出现骶前及中枢神经系统感染,1例高位肛瘘,1例出现术后骶前出血.全部患儿目前恢复良好.结论 对于年龄小于3岁,肿物最大径小于5 cm的CS患儿,经肛门一次完成肛门成型和骶前肿物切除是可行的.     

筋膜瓣技术在尿道下裂手术中的应用

目的探讨筋膜瓣技术在尿道下裂术中应用于创面修复的方法及可行性。方法收集先天性尿道下裂中需行尿道重建的患儿603例,年龄2～18岁。486例为首次手术病例,117例为再次手术病例。按尿道下裂病变特点分别采用Mathieu、带蒂岛状皮瓣法、Snodgrass、Duckett、Duckett+Duplay、Duplay、Thiersch术及膀胱黏膜法等重建尿道。按治疗年限及尿道创面覆盖方法分为筋膜瓣组360例(分别应用尿道自身筋膜瓣、阴茎旋转筋膜瓣、阴茎去上皮筋膜瓣、阴囊轴形筋膜瓣及任意筋膜瓣覆盖新建尿道,再行皮瓣转移错位缝合覆盖阴茎创面)和皮瓣组243例(直接应用皮瓣转移覆盖阴茎创面)。患儿均采用相同缝线及尿道支架材料。术后抗炎,10～14 d拔管。结果各种尿道重建术式中筋膜瓣组尿瘘的发生率均低于皮瓣组。除Duplay及膀胱黏膜法两术式外,其余术式2组尿瘘率比较差异均有统计学意义(Pa<0.05)。筋膜瓣组37例发生尿瘘,无尿道全程裂开等严重并发症病例,总尿瘘率为10.3%;皮瓣组77例发生尿瘘,其中17例新建尿道全程裂开,总尿瘘率为31.7%,2组总尿瘘率比较差异有统计学意义(P=0.000)。结论筋膜瓣技术适用性广、取材灵活,可为尿道提供良好的组织覆盖,有效避免尿瘘的发生,可作为尿道下裂术中创面修复的常规方法。     

间置尿道成形术治疗特殊形态阴茎头型尿道下裂和单纯性严重阴茎弯曲北大核心CSCD

目的探讨间置尿道成形术在特殊形态阴茎头型尿道下裂和单纯性严重阴茎弯曲中的应用价值。方法回顾性分析2008年11月至2019年12月在深圳市儿童医院实施间置尿道成形术治疗的特殊形态阴茎头型尿道下裂伴严重弯曲(>30°)或伴阴茎体段尿道菲薄者9例和单纯严重阴茎弯曲畸形(>30°)2例患儿的临床资料。患儿初次手术年龄25-109个月,中位年龄40个月。手术方法包括:不分期间置尿道成形术2例,分期间置尿道成形术9例。术后随访8个月-12年,随访内容包括阴茎形态、排尿情况,有无尿瘘、尿道狭窄和检测尿流率。尿流率数据以±s表示,组间比较采用t检验。结果术中间置尿道长度为2.2-4.2 cm,平均长度3.2 cm。术后随访阴茎无弯曲,排尿顺畅且尿线不分叉,阴茎外形满意。并发尿瘘2例,其中分期和不分期手术各1例,经再次手术修复成功;无尿道狭窄和尿道憩室患儿。最大尿流率术后3个月为(8.3±1.0)mL/s、6个月为(6.7±1.9)mL/s,与健康对照组[(12.3±3.0)mL/s]比较,差异有统计学意义(t=3.221、3.864,均P<0.05),而术后1年最大尿流率[(10.5±3.7)mL/s]与健康对照组比较,差异无统计学意义(P>0.05)。结论间置尿道成形术治疗儿童轻微的阴茎头型尿道下裂同时伴严重阴茎弯曲和/或阴茎体段尿道发育不良者及严重阴茎弯曲畸形是一种行之有效的手术方式。     

婴儿阑尾盲肠多重套叠并急性阑尾炎一例并文献复习

目的 探讨婴儿期阑尾盲肠多重套叠并急性阑尾炎的临床病理和诊治方法.方法 回顾性分析1例阑尾盲肠多重套叠并急性阑尾炎8月龄婴儿的临床资料,并通过PubMed、ProQuest、中国知网(CNKI)、万方和维普数据库中检索2016年5月前的报道并复习相关文献资料,对各类儿童阑尾套叠进行系统性综述.结果 本例阑尾盲肠多重套叠并急性阑尾炎经手术诊断,整复套叠后切除阑尾并经病理证实,术后顺利康复.共检索到215例阑尾套叠中儿童52例,阑尾盲肠套叠136例,仅3例阑尾自身套叠中有2例为儿童;检索到肠套叠并阑尾炎66例,儿童59例,其中小于1岁的患儿27例,回结型肠套叠43例,此外,检索到回肠盲肠型套叠7例和盲肠盲肠型套叠并阑尾炎1例均为成人患者,所有病例均行手术治疗而痊愈.儿童阑尾盲肠多重套叠并急性阑尾炎者未见报道.结论 婴儿阑尾盲肠多重套叠并急性阑尾炎罕见,如套叠未导致回盲瓣排空完全梗阻则无典型症状体征,诊断困难,易误、漏诊.因此,对经保守治疗不能解除肠梗阻且不能解释其原因时,只要B型超声提示“同心圆征”或“套筒征”就应果断行手术探查以明确诊断并及时治疗.     

“管中管”在尿道手术中的运用体会

1999年以来 ,我院对先天性尿道下裂 ,尿道手术后瘢痕、狭窄、缺损行尿道成形术及尿瘘修补术等 ,5 7例患儿不做耻骨上膀胱造瘘的尿流改道的手术 ,而是采用”管中管”引流膀胱尿液 ,取得非常满意的疗效 ,报告如下。一、临床资料1.一般资料　本组年龄 1岁 9个月～ 14岁。尿道瘢痕、狭窄松解后致尿道缺损 ,施行尿道成形术 4例。其中 1例缺损长度达 2 .6ｃｍ。尿瘘修补术 7例 ,瘘孔最长的达 1.2ｃｍ ,有 1例患儿有瘘口 3处。先天性尿道下裂行尿道成形术 46例。表 1　 46例尿道下裂的手术方法手术方法尿道下裂类型 (例数 )阴茎体型阴茎阴囊类型 会…     

游离尿道板卷管尿道成形治疗尿道上裂

目的 介绍和评价游离尿道板卷管尿道成形治疗男性尿道上裂.方法 2003年1月～2006年12月用游离尿道板卷管尿道成形治疗男性尿道上裂12例,年龄为1～20岁,其中单纯阴茎体尿道上裂10例,另2例为膀胱外翻曾在本院行骨盆截骨膀胱内翻关闭后.用Ransley法纠正阴茎背曲3例.结果 平均随访20个月(3～40个月),12例患儿阴茎头呈圆锥状,阴茎外观满意.1例患儿仍存在轻度阴茎背曲,1例患儿因术后尿瘘被再次手术修补.术后所有患儿阴茎勃起功能存在.结论 游离尿道板卷管尿道成形治疗尿道上裂方法简单,可达到恢复阴茎尿道正常的位置结构,使阴茎外观和功能接近正常.     

Hemorrhagic stroke associated with pulmonary edema and catastrophic cardiac failure

Cerebral arteriovenous fistula (AVF) is a vascular malformation that is rare in the pediatric population. Older children with cerebral AVF tend to present with neurologic problems related to intracranial venous hypertension or intracranial hemorrhage. Cardiac and pulmonary complications following acute neurologic injury such as subarachnoid hemorrhage are common in adults, but are rarely reported in children. However, complications have been reported in cases of enterovirus 71 rhombencephalitis in infants and children and can cause high morbidity and mortality. Here, we report a 14-year-old boy who presented with cardiac failure associated with pulmonary edema following cerebral hemorrhagic stroke due to AVF. After aggressive investigation and management, we intervened before significant hypoxia and hypotension developed, potentially reducing the risk of long-term adverse neurologic consequences in this patient.     

Fibroepithelial polyp of the bladder neck in children

Benign fibroepithelial polyps of the prostatic urethra and bladder are rare congenital lesions most commonly diagnosed in the paediatric population because of diverse urinary symptoms. We report two such cases: one, in a 2-year-old boy who presented with haematuria and voiding difficulties and the other in a 5-year-old boy with acute urinary retention. Imaging and endoscopic studies confirmed the presence of polypoid lesions. Following transurethral resection of these lesions, the diagnosis of a congenital fibroepithelial polyp was rendered by pathologic evaluation. These children are now free of the urinary symptoms and imaging studies are unremarkable 1 and 5 years later. Since these lesions have the potential for recurrence, long-term follow-up is warranted.     

Panic disorder in a child with recurrent chest pain

A 10-year-old boy was admitted to the Pediatric Cardiology Unit with complaints of chest pain and dizziness. Physical examination did not show any pathologic signs. Family history revealed no heart disease and diagnosis of panic disorder (PD) in one of his family members. On follow-up, he was admitted to the pediatric emergency department several times with the same complaint. Organic etiologies of chest pain were excluded by extensive diagnostic work-up. He was referred to the Child and Adolescent Psychiatry Department for further work-up, and PD was diagnosed. A few weeks after starting paroxetine therapy, the frequency and the intensity of the chest pain attacks began to diminish. Early diagnosis of PD will avoid unnecessary investigations and prevent utilization of expensive health services, especially those performed in the emergency department. Physicians should consider that chest pain may be related to psychiatric disorders and refer their patient to mental health professionals for further management. Pharmacological therapy and cognitive-behavioral interventions are successfully used in the management of PD in children and adolescents.     

Xanthogranulomatous pyelonephritis in children: diagnostic and therapeutic aspects.

BACKGROUND: Xanthogranulomatous pyelonephritis (XGP) is an uncommon chronic, inflammatory disease of the kidney. Etio-pathogenesis, diagnosis and management of pediatric XGP is still obscure due to the limited number of cases. Therefore, a retrospective clinical study was carried out to present an updated picture of the entire spectrum of pediatric XGP based on our 30 years' experience covering one of the largest non-collected series treated in a single medical center. METHODS: Records of children who were treated for XGP in our unit from 1970 to 1999, inclusive, were reviewed retrospectively.Information recorded for each patient included age, sex, past medical history, clinical characteristics, diagnostic procedures,treatment methods, histopathologic findings and outcome. RESULTS: 17 children with a mean age of 6.6 +/- 0.8 years, consisting of 15 males and 2 females, were treated for XGP. Eight patients had a urological disease history and 4 of them underwent surgery for urinary calculi and exstrophia vesica repair. The most common presenting symptoms were abdominal pain, fever,weight loss and anorexia. Palpable flank mass was the most common physical examination finding. Left and right kidneys were involved in 10 and 7 patients, respectively. Diagnostic procedures were intravenous pyelography, retrograde pyelography, ultrasound scan and computerized tomography (CT). Renal calculi and/or calcifications and non-functioning kidney were the most frequent findings in radiologic investigations. By combining all these investigations, the preoperative diagnoses were XGP (n = 2), renal and/or perirenal abscess (n = 5), psoas abscess(n = 2), non-functioning kidney due to recurrent pyelonephritis(n = 4), Wilms' tumor and/or renal clear-cell carcinoma (n = 4). XGP was correctly diagnosed in only two patients based on cr findings. Drainage of the renal or psoas abscess was performed in 8 patients as an initial procedure (surgical drainage in 6 and ultrasound-guided percutaneous drainage in 2 patients). Surgical procedures included nephrectomy (n= 13), partial nephrectomy (n=2), nephrectomy and diversion of reno-colic fistula(n= 1), and renal biopsy and nephrostomy (n= 1). Operative and postoperative complications were colonic perforation (n= 3) and wound infection (n = 3). Complications were noted only in patients who underwent nephrectomy without initial drainage procedures. Histopathologic examinations showed diffuse and focal XGP in 14 and 3 patients, respectively. CONCLUSION: XGP should be included in the differential diagnosis of all children presenting with perirenal or psoas abscess, renal mass and/or non-functioning kidney associated with/or without urolithiasis. Clinical awareness and a high index of suspicion is required to achieve the correct preoperative diagnosis and appropriate management. CT seems to be the most valuable imaging method for the diagnosis. We strongly recommend percutaneous drainage of the abscess and adjunctive antibiotic therapy prior to nephrectomy to avoid complications. Complete nephrectomy is the proper treatment for the diffuse form whereas frozen section biopsies followed by partial nephrectomy are mandatory for the proper treatment of focal disease.     

Spinal epidural abscess presenting with abdominal pain.

We report a case of spinal epidural abscess presenting as abdominal pain. An 7-year-old boy presented with abdominal pain. He was operated on under suspicion of appendicitis. During operation, no abnormalities were found. Postoperatively, the abdominal pain did not subside. Subsequently, the boy developed neurological abnormalities. MRI showed a spinal epidural abscess. A laminectomy was performed and the boy was treated with antibiotics; he recovered well. This case showed that it is important to consider a spinal epidural abscess as a cause of abdominal pain with fever in children.     

Extensive spondylodiscitis with epidural abscess causing fever and lower limbs pain in a child with sickle cell disease

Spondylodiscitis is an unusual diagnosis among children and consequent abscess formation is even rarer. A 6-year-old girl with fever, hip pain, and refusal to walk was evaluated. The neurologic examination was normal. Recurrent joint pain with cold weather, iron for anemia without improvement, and decreased intervertebral spaces raised the use of ceftriaxone, oxacillin, and external immobilization. Hemoglobin sickle cell disease, spondylodiscitis with paravertebral collections, and epidural abscess were documented. She was fully recovered. The treatment was conservative because there was no neurologic deficit. We add to the literature 1 case of spondylodiscitis with epidural abscess that was successfully treated with antibiotics alone.     

先天性胆管扩张症合并共同管内蛋白栓的诊断和处理

目的 探讨先天性胆管扩张症合并共同管蛋白栓的诊断和清除方法.方法 2001年6月至2009年1月,在收治的先天性胆管扩张症203例患儿中,经手术前超声、CT、MRCP和术中胆道造影检查发现22例合并胰胆合流异常患儿的共同管内有蛋白栓存在,年龄1岁8个月至12岁,平均6.1岁.对其临床表现、影像学特点及手术中蛋白栓的清除方法进行分析,并对其术后症状、生化检测和胆道改变进行随访.结果 22例患儿均以腹痛症状为主,并发呕吐,其中13例患儿合并黄疸,2例囊肿穿孔.19例患儿腹痛的发作期,血和尿中的胰淀粉酶升高.术中胆道造影22例均发现共同管内充盈缺损和扩张,胰管显影,其中9例合并胰管扩张.采用插管和尿道镜冲洗清除后,共同管的直径回缩,胰管不再显影.蛋白栓非常脆软,很容易被水流的冲力破碎成较小的颗粒.经再次共同管造影证实,蛋白栓清除干净.其中8例经小儿尿道镜直视下证实蛋白栓清除干净.患儿随访3个月至8年,22例患儿经血生化和超声检查,无胰淀粉酶升高和再发共同管和胰管内结石者.结论 1岁以上先天性胆管扩张症患儿,以腹痛为主,发作时血和尿中的胰淀粉酶升高,影像学共同管充盈缺损和扩张者,应怀疑共同管内蛋白栓.术中胆道造影是可靠的诊断方法.插管冲洗或尿道镜下清除蚩白栓安全有效,远期预后好.     

Subphrenic abscess due to ectopic appendicitis]

BACKGROUND: Idiopathic subphrenic abscesses are uncommon in children. Standard chest X-rays may provide the suspicion of this diagnosis. Initial percutaneous drainage of the collection is usually performed. Surgery is required when the underlying cause remains unknown. CASE REPORT: A 12-year-old boy presented signs of pulmonary abscess. Chest X-rays, ultrasonography and computed tomography established the diagnosis of a right subphrenic abscess, which was percutaneously drained. Surgery disclosed an ectopic appendiceal perforation. CONCLUSION: In children, appendicitis is the main etiology of primary or postoperative subphrenic suppurations. Ectopic appendicitis is an important predisposing factor to this complication.     

"Massive hemoptysis" and shock after fever and cough北大核心CSCD

11岁男性患儿,因间断发热15 d,咳嗽10 d,“咯血”7 d入院。患儿15 d前以发热咳嗽起病,伴左侧颈部疼痛,抗生素治疗有效。病程中突然出现大“咯血”致休克,后经纤维支气管镜检查发现左侧梨状窝内瘘口并持续渗血,结合颈部及血管影像学检查考虑为先天性梨状窝瘘感染并颈部脓肿致颈内静脉受损血栓形成,通过低温等离子消融梨状窝瘘封闭术治疗后好转,随访1年半未反复。检索文献未发现梨状窝瘘感染引起大出血休克报道,该文总结此例患儿的临床特点及诊治经过,为早期诊断该类疾病及其并发症防治提供参考依据。     

儿童后肾腺瘤五例报告

目的 探讨儿童后肾腺瘤临床病例特点,提高对儿童后肾腺瘤的认识.方法 回顾性分析2008年5月至2016年1月收治的5例儿童后肾腺瘤的临床资料,并进行文献复习.5例患儿中,男4例,女1例;年龄小于2岁2例,8～10岁3例;肿瘤位于左侧2例,右侧3例.以腹部包块就诊1例,超声偶然发现4例.术前均行超声和增强CT,2例考虑肾母细胞瘤,2例考虑肾细胞癌,1例考虑肾囊肿合并出血.结果 肿瘤直径小于5 cm者4例.行保留肾单位的肿瘤剜除术3例,行瘤肾切除术2例.病理检查报告:后肾腺瘤4例,后肾腺瘤合并乳头状肾细胞癌1例.术后平均随访4年5个月,均无复发和转移.结论 后肾腺瘤罕见,临床表现及影像缺乏特异性,术前诊断困难,确诊依靠病理及免疫组织化学.后肾腺瘤为良性病变,手术完整切除为治疗原则.有合并恶性成分的病例报道,术后需长期随诊复查.