Cystic spinal dysraphism of the cervical and upper thoracic region

Background Cystic dysraphic lesions of the cervical and upper thoracic region are rare and only a few series have been published about the topic. These malformations can be divided into categories that include both myelocystoceles and the so-called cervical meningoceles or myelomeningoceles.Methods A retrospective study of 18 patients was conducted.Results In 17 patients a squamous or a cicatricial epithelium of variable thickness covered the dome of the lesions, while the base was covered with full-thickness skin. In one case the skin was entirely normal. Four patients displayed associated CNS malformations and three more had systemic congenital anomalies. All patients underwent surgical exploration and the length of time between birth and surgery ranged from 6 h to 9 months. The most frequent surgical finding, seen in 14 patients, was a stalk connecting the dorsal surface of the spinal cord to the cyst. In three patients the findings were consistent with myelocystocele. Only in one case was a true meningocele found. Hydrocephalus and Chiari II malformation were not as consistently associated as in myelomeningoceles. Neurological signs and symptoms were not so marked as in myelomeningoceles and were found in the follow-up of four patients. In two of them there was a non-progressive deficit, probably expressing an imperceptible involvement of the nervous system in the first year of life. The histopathological findings were of three types: neuroglial stalks, fibrovascular stalks and myelocystoceles.Conclusions Cystic dysraphisms of the cervical and upper thoracic region differ clinically and structurally from meningomyelocele and have a more favorable outcome. We believe that these malformations have not been properly labeled and propose a classification based on the structures found inside the cyst.     

经鼻内镜手术治疗鼻部脑膜脑膨出

目的　探讨经鼻内镜手术治疗鼻部脑膜脑膨出的可行性并确立临床处理的基本原则。方法　经鼻内镜下采用剪除、电切、切割等方式切除鼻内型脑膜脑膨出 5例 ,并根据颅底缺损的不同情况选择使用肌肉、筋膜、软骨、骨等一期修补颅底。结果　 5例均一次手术治愈 ,除 1例术后颅内感染 (脑膜炎 )外 ,无并发症发生 ,随访 1～ 5年无复发。结论　位于鼻腔内的脑膜脑膨出可以经鼻内镜手术完成 ,直径 0 5cm以下的颅底缺损可使用肌肉填塞和筋膜覆盖的方法修补 ,直径超过 0 5cm的颅底缺损应先使用软骨或骨封闭缺损 ,然后再用肌肉填塞和筋膜覆盖 ,以免复发。     

CT和MRI在诊断鼻内型脑膜脑膨出中的作用

目的：评价CT和MRI在诊断鼻内型脑膜脑膨出中的价值。方法：回顾性分析7例经手术和病理证实鼻内型脑膜脑膨出患者的CT与MRI表现；4例均行CT和MRI扫描,2例仅行CT扫描，1例仅行MRI检查。结果：7例鼻内型脑膜脑膨出患者中有1例并发脑积水。冠状位CT扫描可见筛骨骨质缺损，MRI见鼻腔内囊状异常脑脊液信号，并向上与颅内蛛网膜下腔直接相通，其内有少量脑组织信号。结论：CT和MRI能正确诊断该型脑膜或脑膜脑膨出；CT显示颅底骨质缺损大小、部位优于MRI，MRI可清楚显示疝出物及其与脑底的关系。     

儿童鼻内型脑膜脑膨出的临床特征及手术治疗

目的探讨儿童鼻内型脑膜脑膨出的临床特征及手术治疗。方法对1995年1月～2005年1月本科收治的9例鼻内型脑膜脑膨出患儿病例资料回顾性分析。结果经颅硬膜外手术4例、硬膜内手术2例，眶上锁孔入路手术3例。9例患儿均1次手术治愈。1例术后出现脑脊液漏，经保守治疗治愈。随访时间6个月～9年4个月，无复发及脑脊液鼻漏、脑膜炎等并发症。结论鼻内型脑膜脑膨出手术的关键是严密的硬脑膜缝合，避免术后脑脊液漏；确凿的颅骨缺损修补，避免术后复发；妥善处理疝囊，改善鼻腔通气功能。经前额入路修补是治疗鼻内型脑膜脑膨出安全有效的手段，眶上锁孔入路手术创伤小，疗效可靠。     

Tracheal intubation with size 1 Airtraq in a 3 month old child with occipital meningocele: A novel experience

Meningocele is a neural tube defect where meninges protrude through a skeletal defect. Occipital meningocele compounded with the problems of paediatric airway itself pose challenges to anaesthesiologist in securing and maintaining airway. We present a case report of a 3 month-old-child with occipital meningocele posted for ventriculo-peritoneal shunt for hydrocephalus, who was successfully intubated using a size 1 Airtraq optical laryngoscope.     

重型颅脑损伤术中急性脑膨出的原因及治疗方案探讨

目的 探讨颅脑损伤术中脑膨出的原因及治疗对策.方法 对颅脑损伤术中发生脑膨出患者55例的临床资料进行回顾性分析.结果 55例术中发生急性脑膨出的原因包括迟发性颅内血肿（28例）,广泛严重性脑挫裂伤、急性脑肿胀（19例）,晚期脑疝、血肿压迫时间过长、脑疝后脑梗死（4例）,创伤性休克和术中低血压导致脑组织缺血、缺氧（4例）.出院时按GOS评定预后：良好3例,中残5例,重残8例,植物生存5例,死亡34例.结论 迟发性颅内血肿、急性脑肿胀是术中急性脑膨出的主要原因.明确颅脑损伤术中发生急性脑膨出的原因,尽早采取相应措施,可降低患者病死率.     

蝶窦外侧隐窝脑膜脑膨出鼻内镜下经翼突径路手术

目的探讨经鼻内镜下翼突径路处理蝶窦外侧隐窝脑膜脑膨出的手术方式及其相关问题。方法总结2001-2006年住院诊断为蝶窦外侧隐窝脑膜脑膨出并脑脊液鼻漏9例患者，男6例，女3例；年龄27—56岁。其中2例患者有前期经鼻内镜手术修补史。术前CT扫描和磁共振脑池造影检查定位诊断脑脊液鼻漏和脑膜脑膨出。在全身麻醉下行经鼻内镜下翼突径路切除蝶窦外侧隐窝脑膜脑膨出并修补颅底手术。结果9例患者均一次手术修补成功。1例术后并发颅内高压和脑积水，2例术后合并患侧面部、硬腭和上唇麻木，1例干眼。术后6个月症状逐渐缓解。随访6—58个月，平均25．6个月，无复发。结论蝶窦外侧隐窝脑膜脑膨出伴脑脊液鼻漏采用鼻内镜下经翼突进入蝶窦外侧隐窝术式，为一微创技术和简便手术径路。     

An encephalocele replacing nose completely

Congenital encephalocele presenting in the nasal region is extremely rare. We present here a case ot tncephaloce lecompletely replacing the nose of a five year old boy and duscuss about similar swellings in this region.     

Cervical myelocystocele: prenatal diagnosis and therapeutical considerations

Background  Cervical myelocystocele (CMC) is a very rare congenital malformation and belongs to the spectrum of skin-covered (occult) dysraphisms. Only 15 cases have been so far reported throughout the literature. We report the first case of CMC whose diagnosis was established prenatally by ultrasound imaging (US) followed by fetal magnetic resonance imaging (MR). Case History  A 35-year-old woman was referred for further investigations following prenatal assessment of a fetal cervical mass observed on routine US during pregnancy. Fetal karyotype was normal. Fetal MR confirmed the ultrasonographic findings and led us to strongly suspect the diagnosis of CMC. The newborn was operated on 2 months after birth. The goal of surgical procedure was to remove the malformation and to obtain an untethering of the spinal cord. Twelve months later, the child is still neurologically intact. Discussion  We discuss embryogenesis, different subtypes, associated malformations, and surgical strategy associated with myelocystoceles. Conclusions  This case adds to the existing literature in that it shows for the first time antenatal images of this rare condition and discusses treatment and follow-up implications.     

Transsphenoidal meningocele with hypothalamic insufficiency

Summary A 16-year-old girl with transsphenoidal meningocele and signs of hypothalamic insufficiency is presented. Hormonal disturbances have been reported in only three similar cases in the literature. The radiological criteria for the diagnosis are discussed with emphasis on the differential diagnosis of a persistent craniopharyngeal canal. Based on the present case and studies of the pertinent literature we propose that all patients exhibiting signs of dysraphism of the base of the skull should be thoroughly investigated for hormonal disturbances. Children with cleft palate should be examined for possible concomitant dysraphism of the skull.