Transsellar transsphenoidal encephalocele: a series of four cases

Transsellar transsphenoidal encephalocele is the least common type of basal encephalocele. We present a series of four cases of transsellar transsphenoidal encephalocele. Clinical findings, imaging reviews, surgical repair techniques and postoperative morbidity are discussed with the relevant literature. Non contrast CT scan head with 3D reconstruction and magnetic resonance imaging should be done in all patients of transsphenoidal encephalocele. Endocrine assessment is also essential. Repair of a transsphenoidal encephalocele should be coordinated between a team of neurosurgeons and ENT surgeon. Our surgical outcome supports the transpalatal/ transnasal approach over the transcranial approach.     

蝶窦内脑膨出并自发性脑脊液鼻漏1例及文献复习

目的探讨蝶窦内脑膨出并自发性脑脊液鼻漏的发病机制和手术治疗经验。方法收集我科收治的l例蝶窦内脑膨出并自发性脑脊液鼻漏患者资料,结合文献复习进行临床分析。结果患者因左侧鼻腔反复流出清亮液体就诊;头颅MRI示左侧蝶窦侧壁骨质缺损,蝶窦内含脑组织信号影及脑脊液信号影;入院后行左侧翼点入路硬脑膜修补术;术后患者症状消失。术后随访6个月,未见复发。结论对于蝶窦内脑膨出发病机制目前并不明确,与侧颅咽管形成、蝶窦过度气化、颅内压增高等多个因素有关;术前准确诊断瘘口位置非常重要,同时应选择个体化的手术方式,术后长期随访。     

Anterior encephaloceles: a series of 103 cases over 32 years.

Anterior encephalocele is a rarely reported CNS malformation with a geographical predilection for South-East Asia. The paucity of literature prompted us to analyse our results with hemiorbital advancement and classical Tessier's operation in 103 cases over 32 years (1971-2002). In our series, the frontoethmoidal subtype was the most common (80%), followed by the orbital (8%), transethmoidal (8%), transsellar (3%) and interfrontal types (1%). All patients with classical frontoethmoidal encephalocele had swelling over the nose or inner canthus since birth, with varying degrees of hypertelorism; and those with the nasopharyngeal subtype commonly presented with nasal obstruction and cerebrospinal fluid (CSF) rhinorrhoea. Neurofibromatosis was present in seven patients, all of whom had an orbital encephalocele. The diagnostic workup included a computerised tomography scan of the head in 96 patients and magnetic resonance imaging of the brain in 16 patients. The associated findings on imaging were hydrocephalus (15%), corpus callosum agenesis (7%), arachnoid cyst (3%), porencephalic cyst (3%) and single ventricle (3%). A classical Tessier's operation was performed in the initial 30 cases. However, since 1988, we have been performing a single stage hemiorbital advancement and repair of the encephalocele. There were three deaths, one due to pneumonia and two due to fulminant meningitis. Twenty-two patients (22%) had postoperative CSF leak, of which five required lumboperitoneal shunt placement. We believe that hemiorbital advancement offers satisfactory results with less morbidity than the Tessier's operation.     

Modern treatment of 84 newly diagnosed craniopharyngiomas

There is debate regarding the appropriate treatment for craniopharyngiomas, which often present symptomatically given their proximity to critical brain structures, and pose significant surgical challenges. The goal of this study is to identify which patient and tumor characteristics are associated with specific preoperative symptoms, surgical complications, patient outcomes, and tumor recurrence in order to guide craniopharyngioma treatment. We retrospectively identified 84 patients with newly diagnosed craniopharyngiomas treated at our institution from 1986–2010. We used binary logistic regression and survival analysis to determine the effect of several variables (including sex, age, tumor size, location, surgical approach, and extent of resection) on preoperative symptoms and postoperative outcomes, including complication rates and tumor recurrence. Age and tumor location were associated with increased rates of preoperative symptoms, with children being more likely than adults to present with endocrine dysfunction, and intraventricular tumors being more likely than extraventricular tumors to present with headaches and hydrocephalus. A transcranial surgical approach was associated with 1.5 times higher rate of surgical complications than transsphenoidal surgery, while only intraventricular tumor location was associated with a poorer patient outcome. The main factor significantly associated with tumor recurrence was extent of resection. We conclude that intraventricular tumor location is most highly correlated with preoperative symptoms. If feasible, transsphenoidal approaches are preferred, as they result in fewer surgical complications, and gross total resections are optimal because they lead to lower rates of recurrence. When gross total resection is not possible, we favor multimodal treatment approaches.     

An endoscopic endonasal approach for the surgical repair of transsphenoidal cephalocele in children

Transsphenoidal cephalocele is a rare lesion traditionally managed by either an intracranial approach or by a combined intracranial and extracranial approach. A two-year-old boy presented with a transsphenoidal cephalocele manifesting as apneic-cyanotic spells accompanied by breathing difficulties due to nasal obstruction after birth. Neuroimaging revealed a cystic mass extending from the pituitary fossa into the nasopharynx through the sphenoid sinus. Nasal endoscopy confirmed the diagnosis of transsphenoidal meningocephalocele. The lesion was managed with an endoscopic endonasal approach. The cephalocele and its contents were passively reduced by applying bipolar cautery to the overlying mucosa. Once reduced, the mucosa was incised and removed from the underlying dura to allow further reduction of the dural sac and its contents. The dural sac was amputated partially using microscissors, followed by repair of the dural defect. The patient developed mild diabetes insipidus after surgery. Follow-up evaluation demonstrated resolution of the preoperative symptoms without lasting morbidity. This procedure is safe, less time consuming than the transcranial approach, and efficacious.     

From above or below: The controversy and historical evolution of tuberculum sellae meningioma resection from open to endoscopic skull base approaches

In the early 20th century, the first successful surgical removal of a tuberculum sellae meningioma (TSM) was performed and described by Harvey Cushing. It soon became recognized that TSM pose a formidable challenge for skull base surgeons because of their deep and sensitive location, proximity to critical neurovascular elements, and often dense and fibrous nature. Because of this, over the next several decades controversy transpired regarding their optimal method of resection. Early attempts involved utilization of open transcranial routes. This included classic bilateral and unilateral frontal approaches, followed by pterional or frontotemporal approaches, which have evolved to incorporate skull base modifications, such as the supraorbital, orbitozygomatic, and orbitopterional approaches. Minimally invasive supraorbital keyhole approaches through eyebrow incisions have also been adopted. Over the past 25 years, the microsurgical transsphenoidal approach, classically used for pituitary and parasellar tumors, was modified to resect suprasesllar TSM via the extended transsphenoidal approach. More recently, with the evolution of endoscopic techniques, resection of TSM has been achieved using purely endoscopic endonasal transplanum transtuberculum approaches. Although each of these techniques has been successfully described for the treatment of TSM, the question still remains: is it better to access and operate on these lesions via a traditional, transcranial avenue, or are they better treated via endoscopic endonasal techniques? We outline the surgical management of TSM through history, from early transcranial and transsphenoidal approaches to modern extended endoscopic endonasal procedures. We briefly explore the arguments favoring each of the methods and the advancements which have emerged to further optimize surgical resection.     

33例垂体卒中的临床分析

目的探讨和观察垂体卒中的临床特点及最佳治疗方案。方法回顾性分析2000—2006年我科收治的33例垂体卒中患者的临床资料，根据患者手术方式分为开颅组及经蝶组，比较术后患者视力改善情况及垂体功能低下的发生率。结果本组经蝶手术23例，其中视力完全恢复13例，明显恢复7例，略恢复3例，无视力障碍加重；10例术后发生垂体功能低下，其中6例需长期激素替代治疗。开颅手术10例，其中视力完全恢复3例，明显恢复3例，略恢复2例，视力障碍加重2例；8例术后发生垂体功能低下，其中5例需长期激素替代治疗。结论经蝶入路手术治疗垂体卒中创伤小、并发症少、疗效确实，术后垂体功能低下发生率低，可作为垂体卒中的首选手术入路。     

Endoscope-Assisted Trans-Sphenoidal Approach for Treatment of Sternberg's Canal

We report an uncommon case of a 45-year-old woman who presented with spontaneous rhinorrhea. A computed tomography (CT) scan of the head revealed an abnormally large sphenoid sinus associated with a parasellar bony defect (Sternberg''s canal) through which magnetic resonance imaging could detect an encephalocele of the right temporal lobe. An endoscope-assisted trans-sphenoidal approach was performed and, with the aid of image guided surgery, reduction of the encephalocele was obtained and followed by surgical repair of the dural and bony defects. The postoperative course was uneventful and the cerebrospinal fluid fistula was closed as confirmed by the postoperative CT scan and by the absence of rhinorrhea. After three years of monitoring the patient remained asymptomatic.     

Complications of the transsphenoidal approach to sellar lesions.

Transsphenoidal surgery is currently performed extensively for lesions of the sella turcica. The mortality and morbidity of this surgical approach are minimal, and the results satisfactory overall. Only a few studies have addressed the complications of this approach. The authors present a retrospective study of 200 transsphenoidal procedures in 196 patients over an eleven year period. There was one death due to meningitis. Major morbidity consisted of intraventricular hemorrhage in three patients, false aneurysm of the internal carotid artery in one patient and thalamic infarction in another patient. Minor morbidity details are presented. The pathogenesis of the most serious of these complications along with possible preventive measures are discussed.     

Large supra- and infra-tentorial occipital encephalocele encompassing posterior sagittal sinus and torcular Herophili

Occipital encephaloceles are the most common type of encephalocele encountered in the Western Hemisphere. The occipital encephalocele is typically classified according to the relationship of the herniated tissue to the torcular. We report the unusual case of a premature infant with a rare, large occipital encephalocele which encompassed the posterior superior sagittal sinus and torcular. We discuss the variable venous anatomy with occipital encephaloceles and review the different options to employ in their surgical repair.     

ChiariⅢ型畸形一例报告并文献复习

目的 报告1例ChiariⅢ型畸形病例成功治疗经验并复习文献.方法 通过神经系统检查和MRI检查确诊,行枕下后正中入路脑膨出修补术,延颈髓栓系松解术,第四脑室出口再造术.结果 患儿术前症状改善,术后1年随访恢复良好.结论 选择合适的手术时机,手术方法得当,Chiari Ⅲ型畸形可取得较好预后.     

垂体腺瘤经蝶切除术后发生迟发性视功能减退原因及处理探讨

目的 探讨经蝶切除垂体腺瘤术后继发迟发性视功能减退的原因，尤其是其与空蝶鞍的关系，提出积极防治的意义。方法 分析了北京协和医院诊治过的4例经蝶手术切除垂体腺瘤术后继发不同程度空蝶鞍的迟发性视功能减退患者，并结合文献加以讨论。结果 这4例患者中2例再次经颅手术，1例再次经蝶手术，1例接受保守治疗，治疗后其视功能均有不同程度地改善或恢复正常。结论 结合文献复习，认为经蝶术后合并空蝶鞍的患者发生迟发性视功能减退与视神经和视交叉局部的手术后瘢痕组织机械性牵拉以及血运改变有关。综合治疗后4例患者的视功能均有不同程度的改善或恢复正常，这既说明了上述原因的存在，又显示了积极防治的意义。     

Craneofaringiomas: experiencia y resultados

IntroductionCraniopharyngiomas are a big challenge in the neurosurgical field. Because these lesions involve important systems, surgeons must weigh the risks of aggressive resection against the long-term challenges of recurrence. We present the outcomes of our patients based on clinical results, degree of resection, recurrence and disease-free survival.Materials and methodsWe reviewed medical records in all patients who had undergone surgical resection for craniopharyngioma at (Hospital Italiano de Buenos Aires) between 2007 and 2019. We considered ophthalmological examinations, imaging studies, endocrinological studies and surgical complications. Radical resections were planned in all of the patients. To help choose the correct surgical approach, craniopharyngiomas were classified based on tumor location.ResultsThirty cases of craniopharyngioma were analysed. 12.5% were classified as intrasellar, 12.5% as prechiasmatic, 43.75% as retrochiasmatic, and 31.25% as intraventricular. Overall, 38 cases involved a transcranial surgery (15 orbitozygomatic approach; 19 pterional approach and 4 transcallosal approach), seven involved a transsphenoidal approach, two microscopic transnasal approach and one ventricular endoscopy for emptying the craniopharyngioma cyst. Gross-total resection was achieved in 43.7% and near-total resection (more than 90%) in 25%. The mean follow-up period after resection was 4.7 years. Tumor recurrence occurred in 48%, with an average of 42.7 disease-free months.ConclusionTotal tumor resection is the best treatment for craniopharyngioma. Due to its high morbidity and mortality, a multidisciplinary team is necessary for the management of these tumors.     

老年性垂体腺瘤50例临床分析

目的探讨老年性垂体腺瘤的临床诊断和手术治疗特点。方法回顾性分析8年间50例老年垂体腺瘤患者的手术资料,其中11例采用经额手术,39例采用经蝶手术。结果肿瘤全切23例,次全切除15例,大部切除12例。结论对于有视力障碍的老年垂体腺瘤病例应采用手术治疗,尤其是经蝶入路切除垂体腺瘤,具有良好的耐受,高龄已经不再是禁忌证。     

Severe cardiovascular complications due to the pituitary adenoma with acromegaly. An interdisciplinary approach to the treatment. A case report

Acromegaly reduces life expectancy and leads to 3-5-fold increase in mortality. The main causes are cardiovascular, pulmonary and enhanced prevalence of deaths from malignancy. Successful therapy ought to normalize GH, IGF-I secretion, remove the adenoma mass and its local pressure effects and preserve pituitary functions intact to improve systemic morbidity and normalize mortality. The primary therapy for most patients with acromegaly is still transsphenoidal adenomectomy. The authors present a 64-year-old woman with diagnosed GH-secreting pituitary macroadenoma suffering from severe coronary heart disease and diabetes mellitus. Somatostatin analogue therapy was ineffective in our patient. She was unfit for transsphenoidal adenomectomy. The patient was qualified for coronary artery bypass grafting after cardiological investigation. We have decided to carry out the bypass grafting and transsphenoidal adenomectomy during one anaesthesia. Both surgical procedures and postoperative time were uncomplicated. Our patient feels well and she is in outpatient follow-up.     

大型和巨大型垂体腺瘤经蝶显微外科治疗的疗效及处理策略

目的 探讨大型和巨大型垂体腺瘤手术入路的选择和处理策略。方法 回顾性总结1985—2001年收治的302例大型和巨大型垂体腺瘤临床资料和经蝶手术切除的疗效。结果 显微镜下全切除188例(62．3％)，次全切除68例，部分和大部分切除46例。手术并发症多为一过性，死亡5例(1、66％)。术后动态随诊CT(MRI)173例，无肿瘤残余92例(53．2％)。随诊期(平均22．5个月)视力、视野改善190例(95．5％)，激素分泌性垂体腺瘤相应激素水平大部分正常或不同程度下降。结论 绝大部分本类型肿瘤均可首选经蝶手术切除。术后定期随诊，如残余肿瘤明显或再生长、复发，根据具体情况经颅或再次经蝶手术和(或)辅以放疗和溴隐亭等药物治疗。     

340例垂体腺瘤显微手术治疗的经验

目的:为了总结垂体腺瘤显微手术治疗的经验,回顾性分析了10年来施行手术的340例病例。方法:234例经颅切除肿瘤,106例经蝶切除肿瘤。结果:经颅手术术后无一例复发,手术死亡2例(0.8％):经蝶切除肿瘤者。术后复发3例(2.8％),其中2例经开顿切除肿瘤,1例放疗治愈,手术死亡2例(1.9％)。结论:作者认为,经项切除肿瘤术野宽阔,有利于肿瘤全切:经蝶入路损伤小,但术野狭小,适合于鞍内肿瘤及年老体弱者。     

大型垂体瘤不同手术入路治疗的比较研究

本文回顾性比较106例大型垂体瘤的开颅和经蝶手术治疗的比较。开颅组58例,经蝶组48例。开颅组肿瘤次全切除9例,大部切除48例,部分切除1例;经蝶组全切除35例,大部切除12例,部分切除1例。开颅组术后视力好转率71%,无变化12例,恶化3例。手术后偏盲好转率67%;经蝶组手术后视力好转率77%,无变化者11例,无1例恶化。手术后偏盲好转率93%。开颅组20例有额叶挫伤,其中1例并发脑内血肿,死亡4例。作者认为垂体腺瘤不长向鞍旁、海绵窦、前颅窝底和脚间池和肿瘤不呈瓶颈样生长者,都应优先考虑经蝶窦入路手术。     

Anaesthesia for pituitary surgery.

The anaesthetic care of patients undergoing pituitary surgery involves an understanding of the varied presentations of pituitary disease and their implications for the patient's perioperative condition and management. The neuroanaesthetist must also have an appreciation of the issues relevant to the surgical approach (either transsphenoidal or, less commonly, transcranial) and be able to anticipate and manage them accordingly.