表现为动眼神经麻痹的垂体腺瘤卒中的诊治(附15例分析)

目的探讨垂体腺瘤卒中所致颅神经麻痹的手术疗效,提高术前诊断率。方法总结从2002年4月到2012年6月,15例合并动眼神经麻痹的垂体腺瘤患者的临床资料。男10例,女5例,年龄18~68岁,平均49.6岁;病程从6 d到7年;临床症状表现为头痛,视力下降,视野缺损,动眼神经麻痹,外展神经麻痹,垂体前叶功能低下等内分泌功能紊乱等;术前均行内分泌激素测定、垂体增强磁共振;所有患者均行经蝶入路手术切除垂体肿瘤。结果术后病理结果证实15例患者均存在垂体卒中,无手术死亡病例。术后动眼神经麻痹完全恢复9例,部分恢复3例,另外3例合并外展神经麻痹术后眼肌麻痹均未恢复。66.7%的患者视力改善(6/9),83.3%的视野缺损术后改善(5/6)。所有患者均获随访,平均随访时间12.4个月(6个月至2年)。结论垂体卒中合并动眼神经麻痹需尽快手术治疗,经蝶窦入路手术减压,肿瘤切除是治疗垂体卒中所致颅神经麻痹的安全有效的治疗方法。     

Surgical outcome of oculomotor nerve palsy in pituitary adenoma

Oculomotor nerve palsy is a relatively rare symptom in pituitary adenoma compared to visual compromise or endocrine deficiency. The causes and recovery remain unclear. A total of 23 patients with pituitary adenomas presenting with oculomotor nerve palsy were reviewed. Patients were treated immediately with glucocorticoid therapy. Elective pure-endoscopic transsphenoidal surgery was used for decompression and histopathological confirmation. The clinical differences of patients with apoplectic (hemorrhage or infarction) (20 patients) and non-apoplectic tumors (three patients) were compared. In the apoplectic group, hemorrhage was noted in 13 patients and infarction in seven. Most patients presented with ptosis, followed by limited gaze and diplopia. In the long-term follow-up, the overall complete recovery rate was 19/23 (82.6%): 18/20 in the apoplectic group (90%), and one in three patients in the non-apoplectic group (33%). The median recovery time was 9 days after surgical decompression; and early treatment resulted in early recovery (p = 0.03). Patients with pupil-sparing pituitary adenoma recovered more rapidly than those with pupil involvement (p = 0.012). Patients with minor symptoms recovered earlier than patients with complete palsy (p = 0.003). MRI revealed that the tumor had invaded the interclinoid ligament region in all patients. We conclude that oculomotor nerve palsy usually occurs in patients with apoplectic adenomas, especially those with hemorrhage. Early treatment, pupil-sparing, and minor oculomotor symptoms are factors indicating a good recovery. Endoscopic transsphenoidal surgical decompression achieved good results in this study.     

Clinical Outcome of Cranial Neuropathy in Patients with Pituitary Apoplexy

Objective

Pituitary apoplexy (PA) is described as a clinical syndrome characterized by sudden headache, vomiting, visual impairment, and meningismus caused by rapid enlargement of a pituitary adenoma. We retrospectively analyzed the clinical presentation and surgical outcome in PA presenting with cranial neuropathy.

Methods

Twelve cases (3.3%) of PA were retrospectively reviewed among 359 patients diagnosed with pituitary adenoma. The study included 6 males and 6 females. Mean age of patients was 49.0 years, with a range of 16 to 74 years. Follow-up duration ranged from 3 to 20 months, with an average of 12 months. All patients were submitted to surgery, using the transsphenoidal approach (TSA).

Results

Symptoms included abrupt headache (11/12), decreased visual acuity (12/12), visual field defect (11/12), and cranial nerve palsy of the third (5/12) and sixth (2/12). Mean height of the mass was 29.0 mm (range 15-46). Duration between the ictus and operation ranged from 1 to 15 days (mean 7.0). The symptom duration before operation and the recovery period of cranial neuropathy correlated significantly (p = 0.0286). TSA resulted in improvement of decreased visual acuity in 91.6%, visual field defect in 54.5%, and cranial neuropathy in 100% at 3 months after surgery.

Conclusion

PA is a rare event, complicating 3.3% in our series. Even in blindness following pituitary apoplexy cases, improvement of cranial neuropathy is possible if adequate management is initiated in time. Surgical decompression must be considered as soon as possible in cases with severe visual impairment or cranial neuropathy.     

Ischemic pituitary apoplexy associated with the lupus anticoagulant

PURPOSE: To report a case of ischemic pituitary apoplexy secondary to a hypercoagulable state following elective orthopedic surgery. DESIGN: Observational case report. METHODS: A 48-year-old Caucasian man underwent an uneventful, left-sided total hip replacement and corrective osteotomy of the proximal femur. Two days post-operative, he developed a frontal headache and blurred vision in both eyes. RESULTS: Ophthalmic examination revealed right VIth cranial nerve palsy. Imaging studies indicated pituitary apoplexy with a large hypodense pituitary lesion with widening of the sella turcica. Laboratory findings included positive lupus anticoagulant, a normal prothrombin time (PT), and an elevated activated partial thromboplastin time (aPTT). Five days after the initial symptoms, a transsphenoidal pituitary resection was performed. At a 6 months follow-up, the VIth nerve paresis was completely resolved. CONCLUSIONS: This case describes ischemic pituitary apoplexy associated with a pre-operatively elevated aPTT and positive lupus anticoagulant. This is the first report, to our knowledge, of atypical apoplexy concurrent with antiphospholipid (aPL) antibodies following an uneventful surgery. The results we obtained on this patient suggest that an associated hypercoaguable state may increase the risk of thrombosis following elective orthopedic surgery.     

急性垂体腺瘤卒中的临床研究

目的　对急性垂体卒中的临床表现、诱发因素、辅助检查、治疗和结果进行分析讨论。方法　回顾性分析1996年1月至2001年12月经手术治疗的35例急性垂体卒中病人。男17例,女18例。年龄19～69岁,平均46.5岁。所有患者术前、术后均行内分泌激素及头颅CT和/(或)MRI检查。结果　头痛是急性垂体卒中最常见的临床症状,其次是视觉障碍,再次为眼肌麻痹。急性垂体卒中的可能诱发因素为高血压、糖尿病。CT和MRI检查对急性垂体卒中确诊率分别为26%和90%。经蝶手术22例,开颅手术13例,无手术死亡。无功能性垂体腺瘤最多见,占65.7%。在急性垂体卒中后7天内手术,术后视力、视野改善率较高(P<0.05)。术后随访时间平均为3.2年,均恢复良好,肿瘤复发2例。术后4例放疗。术后长期需要甲状腺激素、皮质类固醇激素、睾酮和抗利尿激素替代治疗者分别为51%、56%、34%(男)、和3%。结论　突发头痛是急性垂体卒中最常见的症状。高血压和糖尿病是急性垂体卒中的重要诱发因素,无功能腺瘤在急性垂体卒中最多见。MRI是急性垂体卒中首选的检查方法。经蝶入路手术减压是治疗垂体卒中安全有效的方法。在垂体卒中后7d内手术视力、视野改善更明显。术后放疗仅适用于肿瘤残留或复发者。     

Sudden death from pituitary apoplexy in a patient presenting with an isolated sixth cranial nerve palsy.

A 68-year-old diabetic, hypertensive man presented with a left sixth cranial nerve palsy. MRI demonstrated an inhomogeneous sellar mass encroaching on the left cavernous sinus. Two days later, a left third cranial nerve palsy developed. Within 24 hours, the patient went into cardiac arrest and died. An autopsy showed hemorrhage within a pituitary macroadenoma ("pituitary apoplexy"). Pituitary apoplexy should be considered a cause of acute isolated sixth cranial nerve palsy and may represent a life-threatening emergency that can be averted with emergent hormonal replacement and hypophysectomy.     

垂体瘤卒中诊断和治疗的再探讨

目的探讨典型和不典型垂体瘤卒中的发病机制,从而为合理制定治疗方案和正确选择手术时机提供帮助。方法经临床表现和/或手术治疗及病理学诊断为垂体瘤卒中病例28例中,24例符合垂体瘤卒中的典型临床表现,另4例符合不典型垂体瘤卒中的诊断。分析垂体瘤卒中的临床表现、影像学表现、手术及病理发现与病程之间的关系及其在病程中各时期的影像学表现、手术发现及病理诊断的特征。结果根据典型垂体瘤卒中病程中不同阶段的临床症状体征、影像学、手术及病理特点,将病程分为早期梗死出血期和后期坏死期,手术全切率分别为87.5%(7/8)和100%(14/14)。不典型垂体瘤卒中的影像学、手术和病理均证实为瘤内陈旧性出血,手术全切率为75.0%(3/4)。结论典型垂体瘤卒中多因肿瘤梗塞后坏死并继发出血所致,急诊手术治疗目的是为了缓解症状。后期坏死期的手术效果明显优于早期梗死出血期,症状不显著者可采用激素替代保守治疗。不典型垂体瘤卒中以慢性瘤内出血为主要特征,无急诊手术治疗指征。     

Acute hemorrhage and ischemic necroses in hypophyseal tumors: hypophyseal apoplexy

Acute enlargement of pituitary adenomas due to haemorrhage or ischaemic necrosis in the tumour was described as "pituitary apoplexy" by Brougham et al. in 1950. Since then, more than 200 cases have been reported, but--especially in the German literature--the syndrome has caught only little attention. Therefore, in a series of 12 own patients, typical findings and clinical characteristics are demonstrated and the literature is discussed. 9 patients had a haemorrhage into the tumour, 3 an acute ischaemic necrosis. The guiding symptom was the acute onset with ophthalmoplegia (11 of 12 patients). Only in one case the adenoma was known before the apoplexy. Other symptoms were headache, blurred vision, drowsiness and, in severe cases, hemiparesis, coma, and hypothalamic disorder. Most important is the acute endocrinological substitution with hydrocortisone; this may be life-saving. Neuroophthalmological recovery depends on early operation: cases of oculomotor palsy require an operation within the first 2 weeks after the acute event. An emergency operation is required only by an acute amaurosis. In general there will be enough time for careful clinical endocrinological and radiological investigations.     

Pituitary Apoplexy Following Mitral Valvuloplasty

Pituitary apoplexy is a rare but potentially life-threatening clinical syndrome caused by the sudden enlargement of a pituitary adenoma secondary to hemorrhage or infarction. Pituitary apoplexy after cardiac surgery is a very rare perioperative complication. Factors associated with open heart surgery that may lead to pituitary apoplexy include hemodynamic instability during cardiopulmonary bypass and systemic heparinization. We report a case of pituitary apoplexy after mitral valvuloplasty with cardiopulmonary bypass. After early pituitary tumor resection and hormonal replacement therapy, the patient made a full recovery.     

Cushing’s disease presenting with pituitary apoplexy

Pituitary tumour apoplexy is a rare but life threatening condition. Cushing’s disease usually presents with clinical features of Cushing’s syndrome. We report a 30-year-old male patient with Cushing’s disease who presented with severe headache and right third nerve palsy. MRI of the pituitary gland revealed a pituitary adenoma with infarction suggestive of apoplexy. After a transsphenoidal surgery he developed pan-hypopituitarism with diabetes insipidus. We also review the relevant literature.     

Radiological Apoplexy and Its Correlation with Acute Clinical Presentation, Angiogenesis and Tumor Microvascular Density in Pituitary Adenomas

Objective

Pituitary apoplexy is life-threatening clinical syndrome caused by the rapid enlargement of a pituitary tumor due to hemorrhage and/or infarction. The pathogenesis of pituitary apoplexy is not completely understood. We analyzed the magnetic resonance imaging (MRI) of pituitary tumors and subsequently correlated the radiological findings with the clinical presentation. Additionally, immunohistochemistry was also performed to determine whether certain biomarkers are related to radiological apoplexy.

Methods

Thirty-four cases of pituitary adenoma were enrolled for retrospective analysis. In this study, the radiological apoplexy was defined as cases where hemorrhage, infarction or cysts were identified on MRI. Acute clinical presentation was defined as the presence of any of the following symptoms: severe sudden onset headache, decreased visual acuity and/or visual field deficit, and acute mental status changes. Angiogenesis was quantified by immunohistochemical expression of fetal liver kinase 1 (Flk-1), neuropilin (NRP) and vascular endothelial growth factor (VEGF) expression, while microvascular density (MVD) was assessed using Endoglin and CD31.

Results

Clinically, fourteen patients presented with acute symptoms and 20 for mild or none clinical symptoms. Radiologically, fifteen patients met the criteria for radiological apoplexy. Of the fifteen patients with radiologic apoplexy, 9 patients presented acute symptoms whereas of the 19 patient without radiologic apoplexy, 5 patients presented acute symptoms. Of the five biomarkers tracked, only VEGF was found to be positively correlated with both radiological and nonradiological apoplexy.

Conclusion

While pituitary apoplexy is currently defined in cases where clinical symptoms can be histologically confirmed, we contend that cases of radiologically identified pituitary hemorrhages that present with mild or no symptoms should be designated subacute or subclinical apoplexy. VEGF is believed to have a positive correlation with pituitary hemorrhage. Considering the high rate of symptomatic or asymptomatic pituitary tumor hemorrhage, additional studies are needed to detect predictors of the pituitary hemorrhage.     

Ectopic pituitary adenoma of the clivus presenting with apoplexy: case report and review of the literature

Ectopic pituitary adenomas usually occur within sphenoid sinus or nasopharynx, and seldom within the clivus. There is only a single reported example of ectopic adenoma with clinical apoplexy, albeit not from clivus. We report a 78-year-old male with known prostate carcinoma admitted with acute onset of blurred vision, suggestive of apoplexy. Work-up revealed unilateral cranial nerve VI palsy and neuroimaging showed a mass confined to the clivus; sellar region was normal. Preoperative considerations included chordoma, chondrosarcoma, or metastatic prostate carcinoma to bone. Resection was via endoscopic transsphenoidal approach to the clivus. An ectopic null cell pituitary adenoma with bland infarction was identified as the cause of the patient's clinical apoplexy. No antecedent precipitating factors for apoplexy were present; specifically the patient had not received leuprolide preoperatively, a known precipitant of pituitary apoplexy in prostate cancer patients who receive drug. We review the literature on ectopic clival pituitary adenomas, apoplexy in ectopic adenomas, and the link between apoplexy and leuprolide usage.     

Blindness following pituitary apoplexy: Timing of surgery and neuro-ophthalmic outcome

The aim of this study is to report the neuro-ophthalmic outcome in patients who underwent transsphenoidal decompression for unilateral or bilateral blindness that was due to pituitary apoplexy. Four patients who were rendered blind (with an absence of light perception) unilaterally or bilaterally as a result of pituitary apoplexy were studied. Neuro-ophthalmic evaluation was performed pre- and post-operatively. Patients underwent CT and MRI pre-operatively and CT post-operatively. All four patients underwent transethmoidal decompression of the pituitary adenoma. One patient underwent a second transcranial procedure to remove the remaining suprasellar component of the tumor. Visual acuity, visual fields and extra-ocular movements were documented during the follow-up period. There were three males and one female. Ages ranged from 40 to 68 years. Three patients had unilateral blindness and one was blind in both eyes. Two of the four patients had associated ophthalmoplegia. All the four patients presented within one week of ictus. One patient underwent surgery within the first week, two patients underwent surgery two and three weeks after ictus and the other patient underwent surgery two months after ictus. The patient who was operated on within the first week recovered from bilateral blindness to a visual acuity of 6/9 and 6/12 with superior quadrantic field defects. The two patients who were operated on two and three weeks after ictus improved to 6/60 in the affected eyes and the patient who was operated on after two months improved to 1/60 in the affected eye. Both the patients with ophthalmoplegia improved completely even though the surgery was delayed by two months for one patient. Although blindness following pituitary apoplexy is rare, visual acuity improves in most patients following transsphenoidal surgery. Early surgery within the first week after ictus leads to excellent visual outcome when compared with surgery that is performed at a later stage. In contrast to visual outcome, ophthalmoplegia improves even if surgical decompression is delayed.     

Surgical treatment of jugular foramen meningiomas

ObjectWe present our experience with surgery of jugular foramen meningiomas with special consideration of clinical presentation, surgical technique, complications, and outcomes.MethodsThis retrospective study includes three patients with jugular foramen meningiomas treated by the senior author between January 2005 and December 2010. The initial symptom for which they sought medical help was decreased hearing. In all of the patients there had been no other neurological symptoms before surgery. The transcondylar approach with sigmoid sinus ligation at jugular bulb was suitable in each case.ResultsNo death occurred in this series. All of the patients deteriorated after surgery mainly due to the new lower cranial nerves palsy occurred. The lower cranial nerve dysfunction had improved considerably at the last follow-up examination but no patient fully recovered. Two of three patients with preoperatively impaired yet functional hearing deteriorated after surgery with no subsequent cranial nerve VIII function improvement. In one case postoperative stereotactic radiosurgery was performed due to non-radical tumour resection (Simpson Grade IV) and tumour remnant proved stable in the 4-year follow-up. None of the patients have shown signs of tumour recurrence in the mean follow-up period of 56 months.ConclusionsJugular foramen meningiomas represent one of the rarest subgroups of meningiomas and their surgical treatment is associated with significant risk of permanent cranial nerve deficits.     

Surgical treatment of jugular foramen schwannomas

ObjectiveWe present our experience with surgery of jugular foramen schwannomas with special consideration of clinical presentation, surgical technique, complications, and outcomes.MethodsThis retrospective study includes ten patients with jugular foramen schwannomas treated by the senior author between January 2007 and December 2012. Three patients had undergone partial tumour resection elsewhere. The initial symptom for which they sought medical help was hearing loss, dysphagia, hoarseness, and shoulder weakness. Preoperative glossopharyngeal and vagal nerve deficits were the most common signs. In our series, tumour extension was classified according to Kaye-Pellet grading system. In two cases the tumours were classified into type A and 8 patients presented with type D tumours. A retromastoid suboccipital craniotomy was performed for type A tumours and modifications of cranio-cervical approach were suitable for type D.ResultsNo death occurred in this series. Four patients deteriorated after surgery: in two patients preoperative cranial nerve deficits deteriorated after surgery while new cranial nerve palsy occurred in 2 other patients. In four patients, the cranial nerve dysfunction had improved at the last follow-up examination. In all other patients, the cranial nerve dysfunction remained the same. One patient experienced tumour recurrence over a follow-up period of 40 months. This patient underwent a successful second surgery without further evidence of tumour growth.ConclusionsJugular foramen schwannomas can be radically managed with the use of skull base surgery techniques. However, the surgical treatment of jugular foramen schwannomas carries a significant risk of the lower CN deficits.     

Pituitary apoplexy after cardiac surgery in a patient with subclinical pituitary adenoma: case report with review of literature

We report a case of pituitary apoplexy occurring in a 74-year-old patient 6 hours after cardiac surgery. The patient presented with confusion, unilateral ptosis and ophthalmoplegia. Neurological examination revealed right oculomotor nerve palsy and decreased level of consciousness. Magnetic resonance imaging showed a hemorrhagic and necrotic pituitary macroadenoma. After prompt endocrinological replacement therapy with hydrocortisone and levothyroxine, the confusion of the patient resolved. Removal of a non-functional macroadenoma with large necrotic areas resulted in full recovery. The physician should be aware of pituitary adenoma infarction after open cardiac surgery and should remember that it can be fatal or cause permanent neurological or endocrine damage without proper treatment. Surgical and endocrine treatment can be life-saving procedures.     

Visual Outcome after Transsphenoidal Surgery in Patients with Pituitary Apoplexy

Objective

Pituitary apoplexy is one of the most serious life-threatening complications of pituitary adenoma. The purpose of this study is to investigate the visual outcome after early transsphenoidal surgery for the patients with pituitary apoplexy.

Methods

We retrospectively reviewed the 31 patients with pituitary apoplexy who were admitted due to acute visual acuity or field impairment and treated by transsphenoidal surgery. Five patients were excluded because of the decreased conscious level. The visual acuity of each individual eye was evaluated by Snellen''s chart. Visual fields were also checked using automated perimetry. To compare the visual outcome according to the surgical timing, we divided the patients into 2 groups. The first group, 21 of the patients have been undertaken transsphenoidal approach (TSA) within at least 48 hours after admission. The second group included 8 patients who have been undertaken TSA beyond 48 hours. All patients were monitored at least 12 months after surgery.

Results

Patients were 21 males and 8 females (M : F=2.6 : 1) with the mean age of 42.4 years. Among the enrolled 29 patients, 26 patients presented with decreased visual acuity and 23 patients revealed the defective visual field respectively. Postoperatively, improvement in the visual acuity was seen in 15 patients (83.3%) who underwent surgery within the first 48 hours of presentation, as compared to those in whom surgery was delayed beyond 48 hours (n=5; 62.5%) (p=0.014). Improvement in the visual field deficits was observed in 15 (88.2%) of patients who had been operated on within the first 48 hours of presentation, as compared to those in whom surgery was delayed beyond 48 hours (n=3; 50.0%) (p=0.037).

Conclusion

This study suggests that rapid transsphenoidal surgery is effective to recover the visual impairment in patients with pituitary apoplexy. If there are associated abnormalities of visual acuity or visual fields in patients with hemorrhagic pituitary apoplexy, early neurosurgical intervention within 48 hours should be also required to recover visual impairment.     

扩大经蝶窦入路治疗侵袭性垂体腺瘤(附117例病例分析)

目的 探讨扩大经蝶窦入路治疗侵袭性垂体腺瘤的疗效.方法 回顾性分析采用扩大经蝶窦入路治疗117例侵袭性垂体腺瘤病例,对其疗效进行分析.结果 肿瘤向前方及额叶底部生长14例,向侧方生长包绕海绵窦103例,向后方生长破坏斜坡27例,突破鞍底向蝶窦生长45例,向两个方向以上的呈侵袭性生长者57例.手术显微镜下肿瘤全切除73例,次全切除40例,大部切除4例.手术并发症包括短暂性脑脊液鼻漏7例;脑神经不全麻痹5例;垂体功能低下5例;颈内动脉损伤2例;单眼失明2例;水久性尿崩症1例;无死亡病例.随访3个月-8年,2例患者出现肿瘤复发而予γ-刀治疗,无再手术病例.结论 采用扩大经蝶窦入路切除侵袭性垂体腺瘤,肿瘤显露满意,全切除率高,手术并发症少;但对于肿瘤未能完全切除或激素分泌型侵袭性腺瘤患者,仍需密切随访,必要时联合放射、药物等综合治疗.     

颅咽管瘤显微手术的疗效分析

目的 探讨颅咽管瘤手术入路的选择及手术疗效。方法 2004年1月至2015年5月手术治疗颅咽管瘤126例,采用翼点入路54例、前纵裂入路21例、经胼胝体入路19例、经鼻蝶入路32例。结果 肿瘤全切除87例,近全切除28 例,部分切除 11 例。术后颅内压增高症状均得到缓解;41例内分泌异常患者中改善18例;65例视力障碍患者中52例缓解。术后发生钠代谢紊乱117例、尿崩症82例、高热47例、同侧动眼神经瘫痪4例、意识障碍37例、癫痫8例（经药物治疗后均控制良好）。肿瘤复发12例,经伽玛刀治疗,效果良好。结论 术前依据肿瘤的分型选择合适的入路,术中保护好垂体柄、下丘脑及其周围重要神经结构,术后积极处理并发症,对提高患者的生存质量至关重要。     

经鼻蝶入路显微手术治疗垂体卒中的临床分析

目的总结55例垂体卒中患者的临床表现特征，分析经鼻蝶入路显微手术的疗效。方法回顾性分析经鼻蝶入路显微手术治疗的55例垂体卒中患者的临床和影像学资料。结果本组55例垂体腺瘤中，48例全切除，7例次全切，肿瘤全切率为87．3％。51例视力障碍、视野缺损的患者中46例术后恢复正常（90．2％），5例恢复欠佳；32例伴有内分泌症状的患者中29例明显缓解（90．6％），3例无明显变化。术后一过性尿崩症7例，脑脊液漏5例。无死亡病例5例。结论根据突发头痛及视路症状等相关症状、体征下并结合影像学检查一般可确诊垂体卒中；经鼻蝶入路显微手术和／或内窥镜手术可做为治疗垂体卒中的首选方法。