Sternal cleft associated with enterogenous cyst treated during the newborn period.

We present the case of a newborn with sternal cleft (SC) and presternal enterogenous cyst operated on during the neonatal period. SC is an uncommon congenital malformation of the thoracic wall which can occur as an isolated form or in association with other malformations. To our knowledge, the presence of SC and enterogenous cyst has not been described to date. Early surgical repair of SC gives good aesthetic and functional results and is usually the preferred approach.     

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。     

Cervico-dorsal spinal enterogenous cyst

A case of a histologically proven ventrally situated spinal enterogenous cyst of cervico-dorsal region is reported with magnetic resonance (MR) imaging features. A brief review of literature on the subject is discussed.     

Ileocolic intussusception with enterogenous cyst: ultrasonic diagnosis

Conclusion Ultrasonography is becoming a common imaging modality for diagnosis of intus-susception in children. Its value depends on the strict observation of the ultrasonographic semiology: pseudokidney or doughnut configuration. The detection of other findings, especially if there is fluid, have to make one suspect a lead point whatever the age of the child. As our case has shown, the association of pseudokidney appearance with cystic liquid mass must be considered as an intussusception with an enterogenous cyst.     

Perineal lipoma in a newborn boy--a case report.

We report the case of a newborn presenting with a pediculated mass arising from the anal margin. Antenatal sonogram and magnetic resonance imaging were unable to diagnose the precise nature of the lesion. Sacrococcygeal teratoma, an enterogenous cyst, a polyp, a prolapse or other perineal tumors were all proposed as possible entities. At birth, no other anatomic anomaly than this homogenous 2 cm para-anal lesion was seen. Excision of the mass was performed under general anesthesia. The postoperative histological exam showed mature fat cells. Reviewing the literature, there have been few previously reported cases of congenital perineal lipoma. It is a rare, benign and easy-to-treat condition that can be evocated by morphological sonography or magnetic resonance imaging (MRI).     

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。     

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。     

Congenital arachnoid cyst mimicking meningocele

The subject of this report is a rare case of a 5-year-old girl who developed an arachnoid cyst with a bony and dural defect in the parietal convexity. She had no history of head trauma or infection. Surgical exploration revealed the bulging lesion to consist of cerebrospinal fluid-containing spongy subcutaneous tissue and to extend into the bony and dural defect. The arachnoid cyst cavity was found beneath the subcutaneous lesion and was not connected to the adjacent subarachnoid space. Histologically, the subcutaneous tissue contained a complex of sinusoidal channels formed by an abundance of migrating arachnoidal cells, thus mimicking meningocele.     

椎管内肠源性囊肿

目的：报告6例椎管内肠源性囊肿的临床表现，诊断及治疗，方法：分析6例椎管内肠源性囊肿的病史，临床表现及诊断，治疗情况，结合文献加以分析讨论。结果：6例皆在儿童期发病，5例以神经根性疼痛起病，6例均有脊髓受压表现，2例症状有反复，3例有脊柱畸形。5例手术全切，1例大部切除，均取得较好疗效。结论：椎管内肠源性囊肿是良性先天性发育异常疾病，手术治疗效果好，MRI检查及微创手术的应用是提高诊断和治疗的关键。     

Intrapericardial Bronchogenic Cyst Overriding the Pulmonary Artery

We describe a case of a 3-year-old child with an intrapericardial bronchogenic cyst arising from the pericardium overlying the pulmonary artery found incidentally at the time of elective repair of a sinus venosus atrial septal defect with partial anomalous pulmonary venous return. A cystic mass overriding the right pulmonary artery was resected and diagnostic studies revealed multiple loculated cysts lined by respiratory mucosa with cartilage and smooth muscle in the wall consistent with a bronchogenic cyst. The cyst was completely resected and postoperative recovery was uneventful.     

Spinal cord compression caused by extradural arachnoid cysts. Clinical examples and review

Most spinal arachnoid cysts are asymptomatic and detected incidentally during magnetic resonance imaging or myelography. The etiology of intraspinal arachnoid cyst is not yet clear. We present two children with three spinal extradural arachnoid cysts and each cyst protruded from a separate dura defect. In both patients, plain radiographs demonstrated widening of the interpedicular distance, which suggested progressive widening of the spinal bony canal. Limited laminectomy was performed to remove the intraspinal cysts. Separate dura defects, the apparent predisposing factors, were also found and repaired. The patients completely recovered neurologically. Radical cyst removal and dura defect closure are the surgical intervention of choice in patients with symptomatic extradural arachnoid cyst.     

Lateral posterior fossa encephalocele with associated migrational disorder of the cerebellum in an infant

Encephaloceles are acquired or congenital defects in which intracranial contents protrude through a defect in the calvaria. The embryogenesis of these lesions is incompletely understood. The vast majority of lesions occur at or near the anatomical midline. The authors present an extremely rare case of a laterally oriented, pathologically proven encephalocele associated with a posterior fossa cyst and cerebellar migrational defect in an infant. The authors review past and current theories of encephalocele formation as it relates to this case.     

Diagnosis of Chiari III malformation by second trimester fetal MRI with postnatal MRI and CT correlation

We report a case of Chiari III malformation diagnosed by fetal MRI. Ultrasound (US) performed at a gestational age of 18 weeks demonstrated a posterior skull base cyst. Repeat US at 19 weeks demonstrated neural tissue in the cyst, consistent with an encephalocele. MR imaging at 23 weeks confirmed the presence of an occipital encephalocele, demonstrated additional bony defect in the upper cervical spine, and identified abnormal morphology and position of the brainstem consistent with the diagnosis of Chiari III. Postnatal MRI and CT confirmed the fetal MRI findings and demonstrate the utility of fetal MRI in the early evaluation of songraphically detected posterior fossa abnormalities.     

Mediastinal esophageal duplication cyst associated with a partial pericardial defect

A two-year-old girl with a mediastinal esophageal duplication cyst is the twelfth example of a thoracic cyst of foregut origin or pulmonary "sequestration" (bronchopulmonary foregut malformation according to the expanded definition of Gerle et al.) that has been associated with a partial pericardial defect on the same side, suggesting that anomalous thoracic masses of several morphologic types have a similar pathogenesis.     

Body stalk anomaly in monozygotic twinning: a case report

We describe a case of concordant body stalk anomaly in a monozygotic twin. Autopsy of the fetus showed abnormalities compatible with the maldevelopment of embryonic folding. Abdominal viscera were in a sac covered by the amnion and were attached directly to the placenta. The anus was not visible and no discernible external genitalia were noted. Other findings included a neural tube defect and a rectal duplication as an enteric cyst. Umbilical cord had only one vein and an artery. No abnormalities were found on pathologic examination of the placenta. Although we encountered cases previously with gastroschsis and omphalocele, this was the first case of body stalk anomaly that we recognized as an enteric cyst, which is extremely rare in twins.     

Coexistent neurenteric cyst and enterogenous cyst

The authors describe common embryological pathways responsible for coexistent neurenteric and enterogenous cysts in a patient with spinal dysraphism. Received: 29 January 1998 Accepted: 3 December 1998     

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.     

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.     

Cockayne syndrome in two brothers

We report the clinical history of two brothers with the classical Cockayne syndrome. The main manifestations consisted of cachectic dwarfism, mental retardation, intracranial calcifications, microcephaly, enophthalmos, senile appearance, joint hypomotility and skin photosensitivity. In one of these children, who died at 10 years of age of bronchopneumonia, necropsy studies revealed a variety of anomalies, mainly encephalic,which included an arachnoidal cyst at the base of the cerebellum, a defect apparently previously undescribed inpatients with this syndrome.