新生儿梨状窝瘘的诊治特点

目的 阐述新生儿梨状窝瘘这一少见疾病的诊治特点.方法 收集我院2001年7月至2010年1月收治的9例新生儿梨状窝瘘病例,分析其发病年龄、临床表现、辅助检查及临床治疗经过,总结其诊治特点和预后.结果 9例梨状窝瘘均为左侧.均以颈部囊性肿块就诊.8例为新生儿时期出现症状,1例为产前发现.5例颈部增强CT提示肿块中含有气体.新生儿时期手术治疗7例.随访时间6个月至9年,患儿临床均无反复感染或复发迹象.结论 新生儿的梨状窝瘘临床表现不同于儿童,常因颈部无痛性、囊性肿块就诊,缺少反复脓肿切开引流史.超声或CT检查中常可发现囊肿中含有气体影.新生儿早期手术瘘管结扎率高,部分需要胃镜辅助.治疗安全性可靠,术后复发率低且预后良好.     

儿童梨状窝瘘反复发作的原因与胃镜辅助手术

目的分析儿童梨状窝瘘反复发作的原因，介绍胃镜辅助手术方法及其优越性。方法12例患儿。男8例，女4例，年龄2．5～9岁；曾行左颈部瘘管或囊肿切除术8例，左颈部反复感染者4例；吞钡造影、B超、CT、同位素扫描等检查诊断为梨状窝瘘。12例均行胃镜辅助瘘管切除术。取此前未经胃镜辅助而直接手术的8例临床资料作对照。结果经胃镜辅助切除瘘管，平均手术时间1h 35min，术后恢复顺利，随访无复发。对照组手术时间长，2例复发。结论缺乏对梨状窝瘘胚胎发生和病理过程的了解是治疗失误的主要原因，胃镜辅助手术是一种简便、有效的治疗手段。     

儿童梨状窝瘘41例

目的提高对儿童梨状窝瘘的认识，介绍相关诊断与治疗进展。方法报告41例梨状窝瘘病例，男16例，女25例，年龄9个月至12岁3个月；左侧40例，右侧1例；33例行颈部超声显像，20例行CT柃查，29例行甲状腺核素显像，39例行食管吞钡造影。28例手术治疗，其中20例应用胃镜辅助，并对37例进行随访，随访时间18个月至17年6个月。结果超声检查显示：32例病变侧甲状腺实质不均质占位或软组织实质不均质占位，与甲状腺关系密切；CT检查提示：病变侧颈部炎性肿块或合并甲状腺内炎性占位性病变，其中5例直接诊断为梨状窝瘘；核素显像提示：26例甲状腺左叶、特别是上极放射性稀疏，2例呈“冷结节”；食管吞钡检查显示：左侧或右侧梨状窝底部有垂直下行的细小瘘管。10例单纯切除术中8例治愈，2例复发，经胃镜辅助治愈；20例经胃镜辅助切除病例中，2例复发；13例未手术，其中9例获随访，5例自愈或未发作，4例仍反复发作。结论儿童梨状窝瘘炎症消退后食道吞钡检查可明确诊断，CT、B超及同位素检查在梨状窝瘘的诊断中也起着非常重要的作用，手术切除瘘管是主要的治疗方法，关键在于完整切除或消灭瘘管；胃镜辅助检查有助于瘘管的寻找，从而保证瘘管的完整切除，是一种简便、有效的治疗手段。     

儿童颈部梨状窝瘘诊治进展

梨状窝瘘多以小儿反复发作的单侧（左侧为主）颈部炎性脓肿或急性化脓性甲状腺炎等症状就诊,以往在治疗上多为反复颈部脓肿切开引流及联合多种抗生素对症缓解症状或是开放性常规分离瘘管手术寻求根治,但复发率较高。该文通过总结近年梨状窝瘘相关诊断和治疗进展,旨在强调对于疑似患儿需及时行超声、CT、上消化道钡餐等检查,必要时做内镜检查,治疗上在非急性期以内镜辅助下导管置入或亚甲蓝内口注射行梨状窝瘘切除术可明显减少复发率。     

经口内镜下低温等离子微创治疗儿童先天性梨状窝瘘

目的:探讨经口内镜下低温等离子封闭内瘘口治疗儿童梨状窝瘘的手术方法及预后。方法:收集2018年1月至2020年4月河南省儿童医院收治的31例先天性梨状窝瘘患儿的临床资料。其中,男17例,女14例,年龄范围为21 d至9岁,中位年龄为3岁2个月。31例患儿均为左侧发病,16例患儿有脓肿切开引流史。术前CT检查显示脓肿内有...     

"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。     

个体化术式选择治疗儿童梨状窝瘘的效果分析

目的:探讨个体化术式选择治疗儿童梨状窝瘘的效果。方法:选取2015年6月至2019年11月河南省人民医院小儿外科收治的43例梨状窝瘘患儿,左侧41例,右侧2例;炎症控制期27例,急性炎症期16例;根据个体病情选择低温等离子射频消融、梨状窝瘘切除、颈部脓肿切开引流等术式或联合术式,观察术后并发症及复发情况。结果:43例梨...     

Differential diagnosis and treatment of neonatal suprarenal mass

目的 分析我院28例新生儿肾上腺肿块的临床资料,探讨新生儿肾上腺肿块的鉴别诊断与治疗方法.方法 回顾性分析2004年5月至2010年8月我院收治的28例新生儿肾上腺肿块患儿的临床资料、治疗方法及预后.结果 28例新生儿肾上腺肿块的患儿中,男17例,女11例,年龄2h至24 d,中位年龄3.5d.右侧23例,左侧肾上腺病变5例,产前B超检查发现肿块7例,占所有病例的25.0％.临床表现以黄疸、窒息、阴囊血肿及腹部包块为主.肾上腺出血22例,肿瘤患儿6例,包括成熟性畸胎瘤1例;神经母细胞瘤5例.肾上腺肿块B超及增强CT均可表现为囊性或实质性肿块,但肿瘤与出血病例相比,B超检查肿块内有血流信号(P=0.02)、增强CT后肿块内有强化(P=0.007),差异有统计学意义.尿VMA出血患儿均正常,肿瘤患儿部分升高(3例).所有出血患儿均保守治疗,失访3例,其余随访1～6个月后血肿消失.5例神经母细胞瘤患儿中,Ⅰ期2例,Ⅲ期1例,Ⅳs期2例,所有患儿完整切除肿瘤,除1例Ⅳs和Ⅲ期患儿化疗外,其余患儿仅单纯手术.除1例Ⅳs期神经母细胞瘤患儿带瘤存活外,其余肿瘤患儿均无瘤存活.结论 新生儿肾上腺肿块的诊断主要依靠产前产后B超、临床表现、尿VMA检测、CT等手段,并且CT和B超检查更具有鉴别诊断的价值,对于鉴别困难的肾上腺肿块可随访观察1个月,并不影响肿瘤患儿的治疗效果及预后.     

小儿胃重复畸形的诊治

目的 探讨小儿胃重复畸形在临床表现、诊断及治疗上的特点,以期减少误诊,提高诊治水平.方法 回顾分析2010年3月至2015年3月我院收治的4例胃重复畸形患儿诊治过程.其中女3例,男1例.年龄9个月至11岁,术前B型超声或CT检查均发现上腹部囊性肿块,但不能明确肿块来源,经腹腔镜探查及开腹探查术中确诊.不同类型胃重复畸形可行不同方式切除.结果 腹腔镜辅助脐孔拖出切除2例,完全腹腔镜下切除1例,中转开腹手术切除1例.手术顺利,术后无并发症出现,分别随访1个月、1年、2年、3年,术前症状完全消失,恢复良好.结论 小儿胃重复畸形较为少见,无特异性临床表现.腹部B型超声及CT易于发现病变,常为上腹部囊性肿块,但难以分辨病变的来源,常误诊为胰腺囊肿、肠重复畸形等病变.外科手术探查是诊断胃重复畸形的有效手段,不复杂的囊状胃重复畸形可选择腹腔镜辅助或单纯腹腔镜切除,单纯重复畸形体切除能达到较好治疗效果.     

子宫外产时处理胎儿颈部巨大占位的临床初探

目的 探讨子宫外产时处理(EXIT)技术治疗胎儿颈部巨大肿块的临床应用.方法 2007年9月至2010年2月,选择性对4例产前诊断颈部巨大占位合并羊水过多、气管受压/受压不明确的胎儿实施EXIT技术.分析其病因、生后气管受压、术前依赖机械通气等情况,初步评估EXIT技术对胎儿颈部巨大占位预后的影响.结论 男女比例3:1,足月,择期分娩.3例术前依赖机械辅助通气,增强CT提示气管明显受压及推移,最严重者出现口咽腔受压变狭窄.3例患儿新生儿期手术治疗,1例未手术.诊断肠源性囊肿1例、淋巴管瘤2例及梨状窝瘘1例.随访6~36个月预后良好.结论 胎儿颈部占位以淋巴管瘤、畸胎瘤多见,其他如肠源性囊肿、梨状窝瘘或食管重复畸形等少见.多数颈部占位可对气道产生压迫及推移,严重者影响呼吸.EXIT技术可为胎儿出生即刻提供良好通气状况,最大程度减少缺氧发生.颈部占位胎儿接受EXIT技术分娩,多数预后良好,转归令人满意.

Abstract：

Objective To discuss the role of EXIT(ex-utero intrapartum treatment) procedure in the treatment of fetal neck mass.Methods Form 2007,9 to 2010,2,4 fetus were selected,those diagnosed neck huge mass with polyhydramnios in prenatal period.After term pregnancy,they were delivered by EXIT procedure in Obstetrics & Gynecology Hospital and follow-up in our Children's hospital.All information of the neck mass,mass of air pressure on the circumstances,the preoperative on mechanical ventilation was recorded,and the effect and prognosis of these patients were evaluated.Results Boy: girl: 3:1.They all delivered by EXIT procedure after full term.Three cases depended on the mechanical ventilation before the operation.Enhance CT showed trachea obviously compressed and lapsed.The most serious patient was found the pharynx under pressure.Those 3 cases had operations in neonatal period.The other one case had no operation and follow-up in clinic department.Four cases were diagnosed as enterogenous cyst 1 case,lymphangioma 2 cases and pyriform sinus fistula 1 case.Conclusions EXIT procedure would provide a ventilation pathway for baby with reduced hypoxia.Fetal with neck mass delivered by EXIT procedure will get good prognosis,the treatment results will be satisfactory.     

新生儿腹腔囊性肿块108例病理与临床结果

目的分析新生儿期腹腔囊性肿块患儿的病因、病理、预后及转归。方法收集2008年8月至2013年7月我院新生儿外科术前诊断腹腔囊性肿块的病例,分析其性别、入院日龄、病因、治疗效果、病理及随访,总结预后及转归。结果 108例患儿中男女比例为40∶68;入院日龄3 h至28天,平均12天;住院日8～23天,平均11.2天;105例为足月儿,3例为早产儿。产前检查发现腹腔肿块42例（36例择期剖宫产）,新生儿期发现66例（27例剖宫产分娩）。91例患儿在新生儿期完成手术,15例患儿在生后1～3个月实施手术,均痊愈出院,另2例未行手术治疗。术后病理证实：胆总管囊肿51例,卵巢囊肿25例,肠源性囊肿16例,肠系膜淋巴管瘤6例,其他8例。2例开腹胆总管囊肿根治术患儿术后因粘连性肠梗阻再次行肠粘连松解术,恢复良好,其余手术患儿均健康成长。结论新生儿腹腔囊性肿块以女性稍多见,胆总管囊肿和卵巢囊肿占大部分,肠源性囊肿和肠系膜淋巴管瘤也是常见原因。新生儿腹腔囊性肿块患儿绝大多数预后良好,转归满意。     

Branchial fistula: CT manifestations

A patient with a recurrent left neck abscess was found to have a branchial fistula from the pyriform sinus. This uncommon anomaly was demonstrated by CT and barium swallow. The embryology, pathology and clinical features of this lesion are discussed and contrasted with those of the branchial cyst.     

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.     

Imaging studies of pyriform sinus fistula

BACKGROUND: Pyriform sinus fistula (PSF) refers to a persistent embryologic third or fourth pharyngeal pouch, which typically presents as a congenital sinus tract that originates from the pyriform sinus. The sinus tract is often diagnosed by a barium study or direct endoscopic inspection. Utilization of advanced imaging studies, including ultrasound (US), computed tomography (CT), and magnetic resonance imaging (MRI), may aid in the diagnosis of this disease entity. OBJECTIVES: To review the imaging findings of PSF and demonstrate the value of various cross-sectional imaging (US, CT, and MRI) in the diagnosis of PSF. MATERIALS AND METHODS: PSF in five children was verified surgically. Preoperative barium esophagography, US, CT, and MRI were performed selectively in these patients. The clinical and imaging findings are reviewed retrospectively. RESULTS: Barium studies demonstrated the sinus tract in all five patients. US, CT, and MRI demonstrated an associated inflammatory process. By utilizing the trumpet maneuver, the presence of sinus tract was evident in two patients during US. The sinus tract is also demonstrated by CT in another patient. CONCLUSIONS: Although barium esophagography is advantageous in demonstration of the sinus tract in PSF, US and CT are also capable of showing the sinus tract. The extent of inflammatory process related to PSF is better delineated by US, CT, and MRI.     

新生儿枫糖尿症二例报告并文献复习

目的 探讨新生儿枫糖尿症的临床特点及诊治经验,以提高认识,更好地预防和治疗该病.方法 报道新生儿枫糖尿症2例,并结合国内近15年来已报道的15例进行文献复习.结果 新生儿枫糖尿症的报道有增多趋势.17例发病年龄3 h～8 d,均有喂养困难,7例家族中有类似患儿,14例出现神经系统表现,8例伴特殊气味,13例血中支链氨基酸明显增高,6例利用串联质谱检测,12例尿中支链氨基酸及其代谢产物排泄增加,5例利用尿气相色谱-质谱分析,10例MRI/CT发现异常信号,12例死亡或放弃治疗,1例并发脑瘫,4例经特殊配方奶粉治疗者病情有不同程度好转.结论 新生儿枫糖尿症(maple syrup urine disease,MSUD)早期常出现喂养困难、反应差、嗜睡等非特异性症状,临床需要注意有无特殊气味和神经系统症状,疑诊者应及时进行血、尿氨基酸检测以便早期诊断,MRI(DWI)特异性水肿改变信号也有助于诊断,诊断后应立即干预治疗,同时应加强家长的配合,从而改善其预后.