小儿椎管内肠源性囊肿的诊断与治疗

目的探讨小儿椎管内肠源性囊肿的临床表现、MRI影像及显微手术治疗。方法回顾性分析1996年至今8年间5例椎管内肠源性囊肿的临床资料，并结合文献加以分析。结果全部病例采用显微外科手术治疗，其中2例全切，2例次全切除，1例大部切除。无一例死亡，经病理报告证实均为肠源性囊肿，术后神经根痛症状基本消失。随访0．5～2年，无一例复发；格拉斯哥预后评分，良好恢复4例，中残1例；脊髓损伤的Frankel分级，E级4例，D级1例。结论小儿椎管内肠源性囊肿有典型的临床表现和MRI特征，作为一种先天性病变，其最佳治疗方法是早期确诊并采取显微外科手术方法切除。     

儿童椎管内肠源性囊肿的诊治体会

目的 总结儿童椎管内肠源性囊肿的临床特点和治疗经验。方法 回顾性分析我院6例儿童椎管内肠源性囊肿的临床、影像学资料。结果 本组6例均有肢体活动障碍，4例伴有神经根性疼痛，5例感觉障碍。囊肿位于颈段4例，胸段2例。MRI检查表现为稍长T1、长T2均匀信号。5例作全切或大部切除术，1例行部分切除术。5例术后神经功能恢复良好。结论 儿童椎管内肠源性囊肿是少见的先天性疾患，多发生于颈段椎管硬膜下脊髓腹侧。MRI是有效的检查工具。早期诊断和显微手术是治疗的最佳方案。     

小儿椎管内肠源性囊肿11例

目的　探讨小儿椎管内肠源性囊肿的临床特点、诊断和治疗。方法　回顾性分析 10年来收治 11例椎管内肠源性囊肿的临床资料。结果　 11例小儿椎管内肠源性囊肿占同期小儿椎管内肿瘤的 16 % ,男女比3.7∶1,囊肿多位于颈段 ,占 5 5 % ,部分患儿并其他发育畸形 ,囊肿全切除率和近全切率达 73%。结论　椎管内肠源性囊肿为良性病变 ,早期诊断和手术全切或次全切除是治疗的最佳选择 ,预后良好     

先天性背部皮肤窦道与硬脊膜内脓肿

目的：探讨隐性脊柱裂中背部皮肤窦道引起硬脊膜内脓肿原因，以及如何早期诊断，及时治疗。方法：本组6例，年龄1－4岁，均为皮肤窦道伴椎客内皮样囊肿继发脓肿形成，诊断领先临床表现及影像学检查，手术行囊肿剥离及窦道切除，术后随访6－20个月，平均15个月。结果：6例患儿中，3例脊髓发育正常患儿，术后2例神经功能恢复正常，1例明显好转；3例脊髓低和，1例双下肢功能恢复，大小便失禁无明显好转，另2例神经功能无明显改善。结论：先天性脊部皮肤窦道可伴有硬脊膜下或髓内皮样囊肿，并感染形成脓肿，因此，对于中枢感染，应及时检查有否背部皮下窦道以及相应的椎管内病变，以利于及时手术，早期诊断，早期治疗有利于神经功能恢复。     

小儿纵隔肠源性囊肿的诊断与治疗

目的：探讨纵隔肠源性囊肿病因、临床诊断及治疗。方法：回顾性分析近20年17例纵隔肠源性囊肿患儿的临床资料。结果：1例为壁内型食道囊肿，16例为纵隔肠源性囊肿，16例中2例同时合并腹腔内的肠重复畸形；临床上多数患儿以呼吸系统症状就诊，12例合并椎体畸形，12例行胸部B超检查，4例行胸部MRI检查，均为边界清晰的囊性肿物，8例行^99TmTc扫描，后纵隔内可见核素^99TmTc浓集灶。结果：分析本组临床资料，得到如下结论：第一，小儿纵隔肠源性囊肿的临床特征有①具有呼吸系统压迫症状，患侧胸廓隆起，呼吸活动度差，呼吸音减弱或消失；②后纵隔囊肿合并椎体畸形；③胸部超声、CT和/或MRI检查，后纵隔存在边缘清楚，内壁光滑的单房性含液囊肿；④^99TmTc扫描发现后纵隔囊肿内有异位的胃肠粘膜。第二，本病的根治方法是手术治疗。     

婴幼儿椎管内肿瘤(附63例报告)

目的：分析和探讨婴幼儿椎管内肿瘤的临床特点,早期诊断和手术治疗,方法：对63例婴幼儿椎管内肿瘤的临床资料进行回顾性研究,结果：本组病例皮样囊肿和表皮样囊肿占41．3％,脂肪瘤12．7％,畸胎瘤7．9％,肠源性囊肿5．0％,神经母细胞瘤占4．8％,其他恶性肿瘤占4．8％,手术全切除率为76．7％,大部分切除率为18．3％,部分切除率为5．0％,肢体运动障碍者,术后随诊时78．9％,好转,感觉障碍64％改善,括约肌功能障碍63．3％,改善,全组无死亡。结论：婴幼儿椎管内肿瘤以先天性肿瘤为主,对其应早期诊断和早期手术治疗,大多能做到肿瘤全切除,术后症状缓解,能明显提高患儿生活质量。     

小儿先天性肝囊肿

目的 探讨小儿先天性肝囊肿的临床表现、诊断及鉴别诊断，正确选择治疗方法。方法 回顾性研究我院自1972年至今的14例小儿先天性肝囊肿病例，评价其治疗手段。结果 本组14例均行手术治疗，其中6例多囊肝病例中，1例术后呼吸衰竭死亡。8例孤立性肝囊肿中，2例行开窗引流，5例行不同形式的肝切除手术，1例行囊肿空肠Roux—Y吻合术。除3例多囊肝病例术后仍有轻度肝肿大外，其余患儿预后良好。结论 小儿先天性肝囊肿的治疗，应根据其不同表现，采取不同治疗手段，以达到满意疗效。     

纵隔肿瘤及囊肿的诊断与治疗

目的通过总结本院收治的20例小儿纵隔肿瘤及囊肿的临床资料,探讨小儿纵隔肿瘤及囊肿的临床诊断及治疗经验。方法回顾性分析2001年1月～2006年12月20例小儿纵隔肿瘤及囊肿的临床资料。结果男12例,女8例;就诊年龄为1个月～9岁。恶性膈瘤5例,占25%,畸胎瘤6例,肠源性囊肿4例,神经源性肿瘤2例,淋巴管瘤3例,支气管囊肿2例,淋巴肉瘤1例,胸腺瘤及增生1例。完整切除肿瘤16例,大部分切除3例,取活检1例。5例恶性肿瘤,4例术后常规化疗,良性肿瘤15例,术后随访10例,生长发育正常,预后良好。结论小儿纵隔肿瘤及囊肿诊断根据临床表现及辅助检查,尽早诊断,一旦确诊应尽早手术治疗,术后合理规范的化疗,可提高患儿的治愈率。     

小儿椎管内皮样和表皮样囊肿

目的总结小儿椎管内皮样和表皮样囊肿的临床特点和治疗经验。方法回顾分析16年来我科收治的17例椎管内皮样囊肿和6例表皮样囊肿的临床资料。结果本组23例平均年龄为3．7岁，男13例。女10例。囊肿位于胸段3例，腰段3例，腰骶段16例，骶段1例；17例皮样囊肿中有12例伴有背部皮肤窦道；6例表皮样囊肿中仅有1例伴有皮肤窦道。有皮肤窦道者均存在椎板或棘突的部分缺如。囊肿完整摘除10例。囊内容物剔除残留小部分囊壁3例，残留约一半囊壁的7例，髓内脓肿3例，纵向切开脊髓，吸除脓汁，皮肤窦道术中一并切除。结论小儿椎管内皮样和表皮样囊肿为良性病变，早期诊断和手术是最佳的治疗选择。     

儿童椎管内胚胎残余组织肿瘤——附66例报道

儿童椎管内胚胎残余组织肿瘤由胚胎发育期残存的胚层细胞发育而成，依组织结构不同可分为表皮样囊肿（epidermoid cyst）、皮样囊肿（dermoid cyst）、畸胎瘤（teratoma）、脂肪瘤（lipoma）和肠源性囊肿（enterogenous cyst）。北京天坛医院1995年至2004年共收治66例手术和病理证实的儿童椎管内胚胎残余组织肿瘤（年龄在15岁以下），现将其临床特点报道如下。     

胎儿腹腔囊性占位的生后治疗及转归

目的 分析产前诊断胎儿腹腔囊性占位的病因、随访、预后及转归,指导产前会诊咨询工作.方法 收集2006年4月至2009年4月产前发现胎儿腹腔囊性占位的病例32例,分析病因、就诊年龄、占位大小、手术治疗效果及随访情况,总结预后与转归.结果 32例患儿,男女比例为7:25,女性占绝对多数.新生儿期就诊23例,其余生后3个月内就诊.就诊时囊性占位较产前明显增大者7例(21.9%).病因包括卵巢囊肿、畸胎瘤、肠源性囊肿、肾上腺来源性占位及胆总管囊肿.27例经Ⅰ期手术预后良好.2例经Ⅱ期手术恢复良好.2例未行手术预后良好.1例因恶性肿瘤死亡.结论 胎儿腹腔囊性占位以女性多见,以卵巢囊肿最多见.肠重复畸形、囊性畸胎瘤亦是常见病因.多数患儿因产前发现肿块在新生儿早期就诊.巨大腹腔囊性肿块造成消化道梗阻症状是早期就诊的主要原因.腹腔囊性占位的胎儿多数预后良好,转归满意.

Abstract：

Objective To summarize our experience with prenatal diagnosis of the fetus with cystic abdominal mass, and retrospectively analyze their clinical outcome after birth. Methods From April 2006 to April 2009, 32 fetuses were diagnosed with cystic abdominal mass in prenatal tests.Their clinical data including etiology, age at diagnosis, mass size, surgical treatment and follow-up were retrospectively analyzed. Results The 32 cases included 7 males and 25 females. Twenty three (71.9%) patients visited doctors in the neonatal period. The other patients (71.9%) visited doctors in the first 3 months after birth. The chief compliant was intestinal obstruction caused by abdominal mass. Seven patients' (21.9%) cystic abdominal masses enlarged after birth. The origins of the cystic abdominal masses were ovarian cysts, teratorna, enterogenous cysts, adrenal tumors and common bile duct cysts. Among the 32 patients, 27 patients underwent one stage tumor resection, and 2 were performed 2 stage surgery. Two patients didn't receive any surgery. And 1 patient with malignant tumor died. All patients recovered eventually from surgery. Conclusions Cystic abdominal mass in female fetus is more common than that in male fetus. The most common origin of cystic abdominal mass in female fetus is ovarian cysts. Intestinal duplication and cystic teratoma is also the common origins. Most neonates visit doctors for prenatal diagnosis of cystic abdominal mass. The prognosis of the majority of fetus with abdominal cyst is good.     

Median raphe cysts of the perineum in childhood

Median raphe cysts of the perineum are uncommon congenital lesions. Congenital lesions rarely encountered in daily clinical practice. Their diagnosis in childhood is particularly rare. We report two children with these cysts requiring surgical removal. We review the embryological, diagnostic and therapeutic aspects of these cysts, which could be of interest to pediatricians and pediatric surgeons when faced with this pathology in children.     

Prognostic factors in pleuro-pulmonary blastoma

PURPOSE: To evaluate the prognostic factors in a series of children affected by pleuropulmonary blastoma (PPB). PATIENTS AND METHODS: Clinicopathological findings, treatment, and outcome of 22 PPB cases observed in 13 Italian Associations for Pediatric Hematology and Oncology centers are reported. Clinical data, surgical notes, pathologic findings, and summaries of treatment were taken from the charts and correlated with outcome by standard statistical methods. RESULTS: The series included 22 patients (14 males) with a median age of 30.5 months followed up for a median of 22 months (range 2-176 months). In nine patients the PPB developed with lung involvement only. Congenital lung cysts were recorded in five cases. Nine patients had recurrences. Gender, side, tumor size, pre-existing lung cysts, and extent of surgical resection at diagnosis did not significantly affect survival by univariate analysis. Achieving total resection of the tumor at any time of treatment resulted in a significantly better prognosis (P = 0.01), whereas extrapulmonary involvement at diagnosis resulted in a significantly worse prognosis (P = 0.01). Estimated 15-year event-free and overall survival rates were 44 and 49% for all patients, respectively. CONCLUSIONS: PPB is an aggressive neoplasm. Total resection of PPB performed at any time of treatment appears to provide a better outcome, whereas extrapulmonary involvement at diagnosis worsens the prognosis.     

Treatment of infantile fibrosarcoma with chemotherapy and surgery: results from the Dana-Farber Cancer Institute and Children's Hospital,Boston

PURPOSE: To retrospectively evaluate the treatment and outcome of patients diagnosed with infantile fibrosarcoma at the Dana-Farber Cancer Institute and Children's Hospital, Boston. PATIENTS AND METHODS: Between 1982 and 1998, a total of 11 infants were diagnosed pathologically with infantile fibrosarcoma. A retrospective chart review was conducted to determine the extent of surgical therapy and chemotherapy required for a favorable clinical outcome. Cytogenetic findings were reviewed and archived tumor specimens were analyzed, when available, for the presence of the TEL/TRKC fusion gene. RESULTS: Three patients had primary surgical resection with negative pathologic margins and have been lost to follow-up. Two patients received chemotherapy only after limited biopsy or subtotal resection and are alive with no evidence of disease 8 and 18 years from diagnosis. Four patients had limited biopsies followed by chemotherapy with delayed resection. One of these four patients had negative margins and received no further chemotherapy. The other three of these patients had positive microscopic margins; two of them received postoperative chemotherapy while the third did not. All four are currently alive with no evidence of disease. Two patients had progressive disease within 7 and 10 months of diagnosis while on chemotherapy after subtotal resections. One of these two patients is dead of disease; the other is alive after palliative radiotherapy. Seven patients had archived or frozen tissue available for molecular analysis. All seven had evidence of TEL gene rearrangement; six exhibited the TEL/TRKC fusion. Six patients had characteristic trisomies previously reported to be associated with infantile fibrosarcoma. CONCLUSIONS: Previously reported series of treatment outcomes in infantile fibrosarcoma have been limited to very few patients due to the rare occurrence of this tumor. In our experience, initial chemotherapy combined with surgery has been successful for most cases. When disease progression occurred, it was within one year of diagnosis. There was no development of distant metastases in the patients with progressive disease. The role of additional chemotherapy for microscopic margins after local control is not clear. We found a high incidence of the TEL/TRKC fusion gene, confirming its utility in diagnosis. We propose a uniform approach to treatment to gather clinical and biologic information about this rare and curable disease.     

小儿脾脏占位性病变的诊治

目的 总结小儿脾脏占位性病变的临床特点。方法 复习6例脾脏占位性病变患儿的临床资料及有关文献，分析其年龄特点、临床特征和诊治要点。结果 6例小儿脾脏占位性病变，其中良性肿瘤和囊肿各占一半，分别以血管瘤和真性囊肿为多发。平均年龄11岁，以男性儿童多见。患儿均手术治疗，其中脾脏切除术5例、脾脏部分切除术1例。术后恢复顺利。结论 影像学检查是诊断小儿脾脏占位性病变的主要手段，对不同类型的占位性病变需采用相应的治疗方法。     

小儿颅脊柱轴先天性皮肤窦道的外科处理

目的总结小儿颅脊柱轴先天性皮肤窦道的临床特点和诊治经验。方法回顾分析1990年7月-2005年7月经外科手术和病理证实43例。结果本组男24例，女19例，年龄2个月～11岁，平均年龄2．6岁。MRI是主要诊断方法。皮肤窦道位于枕后4例．颈段1例，胸段4例，腰骶段34例。枕后4例窦道穿过硬脑膜，合并小脑皮样囊肿继发脑脓肿，脊柱部位39例中合并皮样囊肿20例，表皮样囊肿2例，5例皮样囊肿位于髓内，其中2例继发感染形成脓肿。囊肿完全摘除17例，残留部分囊肿壁5例。结论颅脊柱轴皮肤窦道为先天性疾病。MRI是首选的诊断手段，早期诊断和显微外科手术治疗是最佳的选择。     

儿童椎管内硬脊膜外蛛网膜囊肿的外科治疗

目的 探讨儿童椎管内硬脊膜外蛛网膜囊肿的临床特点及其诊治.方法 回顾性分析2001年1月至2009年8月我科收治的4例儿童椎管内硬脊膜外蛛网膜囊肿.其中颈胸段1例,胸腰段3例.临床表现3例患儿出现运动障碍,1例有感觉障碍,1例有小便失禁,1例有根痛表现.4例患儿均采用外科手术治疗,其中2例囊肿节段小于3个椎体的采用椎板切除+囊肿切除术,2例囊肿节段大于3个椎体的采用椎板成形+囊肿切除术.结果 4例患儿术后均恢复良好,除1例患儿术后随访1年下肢仍有轻度乏力外,其余临床症状均消失,恢复学习.随访1～9年均未见囊肿复发,复查脊柱三维CT未见脊柱畸形的发生.结论 儿童椎管内硬脊膜外蛛网膜囊肿是一种少见疾病,外科手术治疗能获得满意的结果,椎板成形术能够减少囊肿节段过长的患儿术后脊柱畸形的发生.

Abstract：

Objective To review the clinical features and surgical treatment of spinal extradural arachnoid cysts (SEACs) in children. Methods Four children with SEAC from were admitted to our department between January,2001 to August,2009. One SEAC was located at the cervicothoracic spine whereas the other three were found in the thoracolumbar spines. All cases were treated by surgery.Two children underwent the laminectomy for resection of the SEAC, which occupied less than 3 segments. SEAC, spreading more than 3 segments in the remaining cases, were treated by laminoplasty and en bloc resection. Results Postoperatively, the children had good recovery and resumed school. Followup of 1 to 9 years revealed no recurrences. The three-dimensional CT spine did not demonstrate any spine deformity in follow-up study. Conclusions SEACs in children are rarely reported in the literature. Excision of the SEAC result in a favorable outcome. The larninoplasty can reduce spinal deformity in patients with the long segment SEAC.