Imaging of the Extra-axial Tumors and Tumor-like Lesions Involving both Middle and Posterior Cranial Fossae: Classification and Diagnosis

Objective: To study the imaging features of extra-axial tumors and tumor-likelesions involving both middle and posterior cranial fossae and to make a classification. Methods:Sixty cases of pathologically confirmed extra-axil tumors and tumor-like lesions involving bothmiddle and posterior cranial fossae were analyzed. They were divided into central and lateral types,the latter of which were subdivided into three types: middle cranial fossae type, posterior cranialfossae type and the over-riding type. The constitution and imaging features of each type wereanalyzed. Results: There were 12 cases of central type, including chordoma (n=5), pituitary adenoma(n=3), nasopharyngeal carcinoma (n=2), craniopharyn-gioma (n=1) and meningioma (n=l). 48 cases oflateral type including trigeminal nerve tumors (n=14), meningioma (n=12), epidermoid cyst (n=11),dural cavernous hemangioma (n=4), dermoid cyst (n=2), metastasis (n=2), hemangiopericytoma (n=1),paraganglioma of glonius jugular (n=1) and nasopharyngeal carcinoma (n=1). Each type of the lesionshad its own shape features, some of which were characteristic for some specific tumors. Most of thetumors and tumor-like lesions could be qualitatively diagnosed according to their imagingcharacteristics and the extent of the lesions could be defined definitely. Conclusion: It is helpfulto categorize extra-axial tumors and tumor-like lesions involving both middle and posterior cranialfossae according to their location for qualitative diagnosis and description of the extent of theselesions. It is of great clinical value in providing more precise and thorough imaging informationfor planning therapeutic methods and route of operation.

Inflammatory Myofibroblastic Tumor Mimicking Malignant Meningioma in the Middle Cranial Fossa:a Case Report

INFLAMMATORY myofibroblastic tumor（IMT）,alsoknown as inflammatory pseudotumor or plasma cellgranulomas,is an uncommon fibro-inflammatorylesion which is composed of inflammatory cells andmyofibroblastic spindle cells.1Its pathogenesis is still un-known.The tumor commonly occurs in the lung,upperrespiratory tract,live,orbit,abdominal membrane,retro-peritoneum,and genitourinary tract.2-4It rarely involvesthe central nerve system,5,

Comparative Study on Two Surgical Procedures for Middle Cranial Fossa Arachnoid Cysts

In this study, we explored the operation options for middle cranial fossa arachnoid cysts （MCFAC）. One hundred and forty-nine patients who were operated for a symptomatic MCFAC between 1993 and 2006 in our hosptial were analyzed. Follow-up time ranged from 1 y to 14 y （mean=5.4 y）. All these patients were divided into three subgroups according to Galassi classification. Long-term outcome and complications were studied respectively. Fenestration （F） resulted in a more favorable long-term outcome and less complication for cysts of types I and Ⅱ, whereas a favorable outcome was noted in type Ⅲ patients who underwent cysto-peritoneal shunting （S）. We are led to conclude that Fenestration is suitable for cysts of types Ⅰ and Ⅱ （Galassi classification）, cysto-peritoneal shunting is better for cysts of type Ⅲ.

Calcium pyrophosphate deposition disease of the temporomandibular joint invading the middle cranial fossa:Two case reports

BACKGROUND Pseudogout is a benign joint lesion caused by the deposition of calcium pyrophosphate dihydrate crystals,but it is invasive.Pseudogout of the temporomandibular joint(TMJ)is uncommon,and it rarely invades the skull base or penetrates into the middle cranial fossa.The disease has no characteristic clinical manifestations and is easily misdiagnosed.CASE SUMMARY We present two cases of tophaceous pseudogout of the TMJ invading the middle cranial fossa.A 46-year-old woman with a history of diabetes for more than 10 years was admitted to the hospital due to swelling and pain in the right temporal region.Another patient,a 52-year-old man with a mass in the left TMJ for 6 years,was admitted to the hospital.Maxillofacial imaging showed a calcified mass and severe bone destruction of the skull base in the TMJ area.Both patients underwent excision of the lesion.The lesion was pathologically diagnosed as tophaceous pseudogout.The symptoms in these patients were relieved after surgery.CONCLUSION Tophaceous pseudogout should be considered when there is a calcified mass in the TMJ with or without bone destruction.A pathological examination is the gold standard for diagnosing this disease.Surgical treatment is currently the recommended treatment,and the prognosis is good after surgery.

Trigeminal Ganglioneuroma in the Middle-posterior Cranial Fossa： a Case Report

GANGLIONEUROMA is considered as the most mature and noninvasive form of neuroblastic tumors. It derives from neural crest cells, and can arise from wherever sympathetic tissue exists, including neck, posterior mediastinum, adrenal gland, retroperitoneum and pelvis. The two most common locations for this tumor are retroperitoneum and posterior mediastinum; infrequently it occurs in the intracranial re-gion,2-8 with only three cases has been reported arising from trigeminal nerve.2-4 The current paper presents a 49-year-old male patient with a ganglioneuroma arising from right trigeminal ganglion and extending to the mid-dle-posterior cranial fossa. We summarized the clinical and diagnostic characteristics of this extremely rare tumor, in comparison with the three reported cases in literatures.

Primary Endodermal Sinus Tumor in the Posterior Cranial Fossa:Clinical Analysis of 7 Cases

Objective To clarify the clinical features,therapeutic method and outcomes of the primary endodermal sinus tumors(ESTs)in the posterior cranial fossa.Methods The English literatures on EST in the posterior cranial fossa were retrieved from PubMed and reviewed.And a 4-year-old boy diagnosed with EST in our hospital was reported.The clinical manifestations,therapy,pathologic features,and prognosis of these cases were analyzed.Results Only seven cases of the ESTs in the posterior cranial fossa were enrolled in this review,including six cases searched from the PubMed and one case from our hospital.Six patients were boy and one patient’s gender was not available from the report.Ages ranged from 1 to 5 years(mean 3.14 years).The mean tumor size in our cohort was 4.4 cm.Six cases came from East Asia.Schiller-Duval bodies were found in all seven neoplasms.All tumors were positive for alpha-fetoprotein.The alpha-fetoprotein level in serum was increased to a very high level before therapy and depressed quickly after the effective chemotherapy.The mean follow-up time was 24.4 months(range 5-52 months).Six tumors were totally removed,and four of them recurred.Three cases died including one whose tumor was partially removed.Conclusions The serum alpha-fetoprotein level is well correlated with the severity of the tumor.A combination of operation and chemotherapy might be the effective management for EST in the posterior cranial fossa.The prognosis of extragonadal intracranial EST is poor.

The Microscopic Surgical Treatment for Tumor of Posterior Cranial Fossa in Children

OBJECTIVE To analyze and discuss about the clinicalcharacteristics, pathological types, surgical modalities andtechniques, and postoperative complications in children withtumor of posterior cranial fossa .METHODS Retrospective study was conducted on 102 cases ofpediatric tumor of posterior cranial fossa, admitted and treated inour hospital during the period of January 1996 to January 2007.All patients underwent microscopic surgical treatment. Fifty-eight were male and 44 cases were female. The age ranged from 9months to 14 years old, with an average of 6.1 ± 0.5 of age. CranialCT or MRI examination was conducted before and after thesurgery on all patients.RESULTS The primary manifestations for this group of patientswere increased intracranial pressure and/or ataxia. Postoperativepathological diagnoses showed: 46 cases of medulloblastoma, 43cases of astrocytoma, 11 cases of ependymoma (including 1 caseof degenerative ependymoma), 1 case of dermoid cyst, and 1 caseof teratoma. In this group of the patients, radical surgery wasused in 68 cases and subtotal surgical removal used in 31 cases,while surgical removal of large section was performed on 3 cases.There were no deaths from surgery reported. Ninety-one casesshowed significant symptomatic improvement when comparedwith preoperative conditions, while 11 cases showed either noimprovement or more severely affected afterward. For 6 cases,postoperative ventriculoperitoneal shunt was performed within7 days to 2 months after the surgery. Sixty-three patients gainedfollow-up for 3 to 60 months in duration. Thirty-nine patientsregained normal life and were able to learn well, while there were7 patients who could not live normally on their own. During thefollow-up period, there were 17 cases of recurrence and 7 casesof death. In 23 cases of medulloblastoma in children with age of3 years old or above, 2 cases who underwent surgical removal ofintracranial ependymoma received small dosage of postoperativeX-ray radiotherapy on the the brain and spinal cord. Nine casesof medulloblastoma in children under age of 3 and 17 cases ofastrocytoma diagnosed after the surgery received chemotherapyof Carmustine.CONCLUSION Medulloblastomas and astrocytomas werethe most common types of pediatric tumor of posterior cranialfossa, right followed by ependymoma, and dermoid cysts andteratomas were rare. Early correct diagnosis, proper selection ofappropriate surgical modality and the surgical margin, propertreatment of postoperative complications, and the selecting rightradiotherapy or chemotherapy were the key factors in influencingthe prognostic outcome of children with tumor of posterior cranialfossa.

Schwannoma of the facial nerve involving the middle cranial fossa:case report

Facial nerve schwannoma involving the middle cranial fossa is quite rare, and its accurate diagnosis is very difficult before surgery. Here we present a case of schwannoma of the facial nerve at the middle cranial fossa that was misdiagnosed previously at a local hospital and then cured in our hospital.

Traumatic extradural hematoma in the middlecranial fossa base:clinical analysis of 14cases

Toexploreclinicalcharacteristics ,CT manifestationandoperativeindicationsoftraumatic extraduralhematomainthemiddlecranialfossabase (EHMCFB) . Methods: Atotalof 14caseswithtraumatic EHMCFBfromJanuary 1997toMay 2 0 0 0were retrospectivelyanalyzed . Results : Of 14cases ,12cases (85 .7% )maintained formchangeofcisternaambiensforbeingpressed ;11cases whoreceivedoperationswereallcured ;3received conservativetherapy ,ofwhom 2becamebetterand 1died . Conclusions: IntraumaticEHMCFB ,theform changeofcisternaambiensoccurseasily .Therefore ,if thereappearsprogressivelyaggravatedformchangeof cisternaambiensafterthecisternaambiensbeingpressed , anearlyandactiveoperationisnecessaryandcrucial.

Undiagnosed Anterior Cranial Fossa Dural Arteriovenous Fistula with Intracranial Hematoma: Case Report and Review of the Literature about Its Natural History

Dural arteriovenous fistulas (dAVFs) of an anterior cranial fossa are rare. Because of the high risk of intracranial hemorrhage and relatively easy access for direct surgery, aggressive treatment has been recommended. The natural history of anterior cranial fossa dAVFs (ACF dAVFs) is unclear in spite of many reports for the natural history of general dAVFs. To treat ACF dAVFs, direct surgery has traditionally been performed and endovascular surgery has recently been introduced. A 74-year-old man was transferred with severe consciousness disturbance and presented with devastating intracerebral hemorrhage on the CT scan. Digital subtraction angiography revealed the ACF dAVFs with a large venous pouch. The patient received direct surgery, nevertheless he became vegetative state. Later on, a smaller venous pouch was recognized on the CT scan when he had suffered from the thalamic hemorrhage sixteen months before. There are twelve cases including our case which was treated for a certain period and documented in detail. Eleven of twelve cases were asymptomatic. Three of the six cases with a venous pouch had some events possibly related to the disease, though none of the six cases without a venous pouch had any events during observation. In conclusion, an ACF dAVF with a venous pouch should be treated by direct surgery or endovascular surgery even if it is incidentally found. By contrast, careful observation might be a possible therapeutic option for an ACF dAVF without a venous pouch if there is mild reflux flow.

Retrospective Cohort Study Laparoscopic anatomical SVIII resection via middle hepatic fissure approach:Caudal or cranio side

BACKGROUND Laparoscopic hepatectomy is a proven safe and technically feasible approach for liver tumor resection,but laparoscopic anatomical SVIII resection(LASVIIIR)remains rarely reported due to poor accessibility,difficult exposure,and the deep-lying Glissonean pedicle.This study examined the safety,feasibility,and perio-perative outcomes of LASVIIIR via a middle hepatic fissure approach at our in-stitution.AIM To investigate the safety,feasibility,and perioperative outcomes of LASVIIIR via a middle hepatic fissure approach at our institution.METHODS From November 2017 to December 2022,all patients with a liver tumor who underwent LASVIIIR were enrolled.The perioperative outcomes and postope-rative complications were evaluated.RESULTS Thirty-four patients underwent LASVIIIR via a middle hepatic fissure approach from the side or cranio side and were included.The mean operation time was 164±54 minutes,and the intra-operative blood loss was 100 mL(range:20-1000 mL).The mean operative times were,respectively,152±50 minutes and 222±29 minutes(P=0.001)for the caudal side and cranial side approaches.In addition,the median blood loss volumes were 100 mL(range:20-300 mL)and 250 mL(range:20-1000 mL),respectively,for the caudal and cranial sides(P=0.064).Three patients treated using the cranial side approach experienced bile leakage,while 1 patient treated using the caudal side approach had subphrenic collection and underwent percutaneous drainage to successfully recover.There were no differences regarding postoperative hospital stays for the caudal and cranial side approaches[9(7-26)days vs 8(8-19)days](P=0.226).CONCLUSION LASVIIIR resection remains a challenging operation,but the middle hepatic fissure approach is a reasonable and easy-to-implement technique.

Transmastoid resurfacing versus middle fossa plugging for repair of superior canal dehiscence:Comparison of techniques from a retrospective cohort

Objective:To compare and contrast our experience with middle cranial fossa approach (MFR) and transmastoid approach with capping of the dehiscence (TMR) of superior semicircular canal dehiscence and to determine guidelines to help guide management of these patients. Methods:All patients from 2005 to 2014 with symptomatic superior semicircular canal dehis-cence syndrome with dehiscence demonstrated on CT scan of the temporal bone who under-went surgical repair and had a minimum 3 months of follow up. Surgical repair via the MFR or TMR, preoperative CT temporal bone, preoperative, and postoperative cervical vestibular evoked myogenic potential (cVEMP) testing and anterior canal video head thrust testing (vHIT). Success of repair was stratified as complete success, moderate success, mild success, or failure based on resolution of all symptoms, the chief complaint, some symptoms, or no improvement, respectively. Results:A total of 29 ears in 27 patients underwent surgical repair of canal dehiscence. Com-plete or moderate success was seen in 71% of the MFR group compared to 80% of the TMR group. There were zero failures with the MFR group and no major intracranial complications.There were 2 failures out of 15 ears that underwent the TMR. Residual symptoms were most commonly vertigo or disequilibrium in the MFR and aural fullness or autophony in the TMR groups, respectively. MFR hospital stay was approximately 2 days longer. Average cVEMP threshold shifted 18 dB with surgical correction in the MFR group. A 29 dB average shift was seen in the TMR group. The MFR group had a significant reduction in their anterior canal gain compared to the TMR group. Conclusions:TMR is a less invasive alternative to MFR. However, in our series, we have not seen any intracranial complications (aphasia, stroke, seizures, etc.) in our MFR patients. Interest-ingly, vestibular symptoms were better addressed than audiological symptoms by the TMR sug-gesting its usefulness as a less invasive option for patients with primarily vestibular complaints. Residual auditory symptoms in TMR patients may be due to the flow of acoustic energy from the superior canal to the mastoid cavity through an incompletely sealed third window.

Endoscopic endonasal transclival removal of tumors of the clivus and anterior region of the posterior cranial fossa (results of surgical treatment of 140 patients)

Background: Until recently, tumors of the clivus and the anterior region of the posterior cranial fossa were considered extremely difficult to access and often inoperable using standard transcranial approaches. With the introduction into the neurosurgical practice of minimally invasive methods utilizing endoscopic techniques, it became possible to effectively remove hard-to-reach tumors, including central tumors of the anterior region of the posterior cranial fossa. Methods: From 2008 to the present time, the inpatient institution has operated on 140 patients with various tumors of the base of the skull, localized to the clivus and anterior region of the posterior cranial fossa (65 men and 75 women). The age of patients ranged from 3 to 74 years. Tumor distribution according to the histopathological features was as follows: chordomas, 103 (73.57%);meningiomas, 12 (8.57%);pituitary adenomas, 9 (6.43%);fibrous dysplasia, 4 (2.86%);cholesteatoma, 3 (2.14%);craniopharyngiomas, 2 (1.43%);plasmacytomas, 2 (1.43%);and other tumors (giant cell tumor, neurohypophyseal glioma, osteoma, carcinoid, chondroma), 5 (3.57%). The tumors had the following size distribution: giant (more than 60 mm), 35 (25%);large (35–59 mm), 83 (59.3%);medium (21–35 mm), 21 (15%);and small (less than 20 mm), 1 (0.7%). In 11 cases, intraoperative monitoring of the cranial nerves was performed (21 cranial nerves were identified). Results: Upper, middle, and lower transclival approaches provide access to the anterior surface of the upper, middle, and lower neurovascular complexes of the posterior cranial fossa. The chordoma cases were distributed as follows according to extent of removal: total removal, 68 (66.02%);subtotal removal, 25 (24.27%);and partial removal, 10 (9.71%). The adenomas of the pituitary gland were removed totally in 6 cases, subtotally in 1 case and partially in 2 cases. The meningiomas were removed totally in 1 case, subtotally in 5 cases, and partially in 5 cases, with less than 50% of the tumor removed in 1 case. Other tumors (cholesteatoma, craniopharyngioma, fibrous dysplasia, giant cell tumor, glioma of the neurohypophysis, osteoma, plasmacytoma, carcinoid, and chondroma) were removed totally in 9 cases and subtotally in 7 cases. Postoperative CSF leaks occurred in 9 cases (6.43%) and meningitis in 13 cases (9.29%). Oculomotor disorders developed in 19 patients (13.57%), 12 of which regressed during the period from 4 to 38 days after surgery, and 7 of which were permanent. In 2 cases, surgical treatment had a lethal outcome (1.43%). (Continued on next page) (Continued from previous page) Conclusion: The endoscopic endonasal transclival approach can be used to obtain access to the centrally located tumors of the posterior cranial fossa. It is an alternative to transcranial approaches in the surgical treatment of tumors of the clivus. The results of using this approach are comparable with the results of transcranial and transfacial approaches and, in some cases, surpass them in effectiveness. The extended endoscopic endonasal posterior (transclival) approach, considering its minimally invasive nature, allows fora radical and low-risk (in terms of postoperative complications and lethality) removal of various skull base tumors of central localization with the involvement and without the involvement of the clivus, which, until recently, were considered to be almost inoperable.

Concurrent chemotherapy and reduced - dose cranial spinal irradiation followed by conformal posterior fossa tumor bed boost for average - risk medulloblastoma: efficacy and patterns of failure

PURPOSE:To review the efficacy and patterns of failure in average-risk medulloblastoma patients treated withconcurrent chemotherapy and reduced-dose cranial spinal irradiation and a conformal tumor bed boost.METH-ODS AND MATERIALS:Thirty-three patients with average risk(defined as<==1.5 cm(2)of residual tumorafter resection,age>3 years,and no involvement of the cerebrospinal fluid or spine)medulloblastoma werediagnosed at our institution between January 1994 and December 2001.They were enrolled in an institutional

多模态图像融合及三维重建技术在后颅窝肿瘤手术中的应用

目的旨在探讨多模态图像融合及三维重建技术在后颅窝肿瘤手术治疗中的应用效果。方法回顾性分析2022年1月至2023年9月在江苏大学附属人民医院神经外科接受手术治疗的19例后颅窝肿瘤患者的临床资料。所有患者在术前均接受头部CT和MRI检查,并将影像数据输入影像融合工作站进行图像融合和三维重建。医生利用这些融合后的影像进行肿瘤空间评估和模拟手术入路。术后统计肿瘤全切除率和术后并发症,并对其应用价值进行评估。结果多模态图像融合及三维重建技术能清晰显示后颅窝肿瘤与周围结构的解剖关系,19例患者中肿瘤全切除15例(78.9%),次全切除4例,无围手术期死亡患者。术后并发症包括脑水肿2例,颅内感染1例,面瘫2例,吞咽困难1例。根据医生的反馈,多模态图像融合及三维重建技术在手术中表现出显著价值的案例有16例,辅助价值的案例有3例。结论多模态图像融合及三维重建技术可精准、清晰地显示后颅窝肿瘤与周围重要组织的空间关系,有助于医生设计更精准的手术切口和选择更合理的手术入路,对手术顺利完成有较高的辅助价值。

颅脑超声对宫内窘迫新生儿脑损伤的早期诊断价值

目的探讨颅脑超声对宫内窘迫新生儿脑损伤的诊断价值。方法对146例宫内窘迫新生儿行颅脑超声及常规MRI检查,并将超声检查结果与MRI结果进行对比分析。根据脑损伤情况分为脑损伤组(n=46)和无脑损伤组(n=100),分析比较2组新生儿大脑中动脉和前动脉血流动力学指标[收缩期血流速度(PSV)、舒张末期血流速度(EDV)及阻力指数(RI)],采用受试者工作特征(ROC)曲线分析其对脑损伤的诊断价值。结果146例宫内窘迫新生儿中46例存在影像学异常征象,阳性检出率31.74%(46/146),其中MRI的检出率为91.30%(42/46),明显高于颅脑超声的65.22%(30/46,χ^(2)=9.200,P=0.002)。缺氧缺血性脑病(12.33%比5.48%,χ^(2)=4.222,P=0.040)、硬膜或蛛网膜下腔出血(2.74%比0,χ^(2)=4.056,P=0.044)的MRI检出率明显高于颅脑超声(P<0.05),脑室周围-脑室内出血的颅脑超声检出率明显高于MRI(11.64%比4.79%,χ^(2)=4.540,P=0.033)。脑损伤组新生儿大脑中动脉和前动脉PSV、EDV小于无脑损伤组,RI大于无脑损伤组(P<0.05)。ROC曲线显示,大脑中动脉和前动脉PSV、EDV和RI诊断脑损伤的曲线下面积分别为0.710、0.786、0.625和0.740、0.819、0.613。结论颅脑超声可早期发现宫内窘迫新生儿脑组织结构改变及血流动力学变化,在脑室周围-脑室内出血方面的检出率优于MRI,但脑损伤诊断效果不如MRI,临床可根据患儿情况选择合适检查方案。

后颅窝室管膜瘤临床病理分析

目的探讨后颅窝室管膜瘤临床病理特征。方法收集2022年10月-2023年10新疆医科大学第一附属医院病理科存档的后颅窝室管膜瘤共6例。调阅组织切片,分析免疫组化法检测相应指标表达情况,并收集随访信息,查阅相关文献总结其临床病理学表现。结果6例后颅窝室管膜瘤中PFA组4例,PFB组2例。临床表现为头疼,呕吐,继发性脑积水等。PFA组患者均为儿童,年龄10个月-11岁,其中女性3例,男性1例;肿瘤3例位于四脑室,1例位于脑干。2例PFB组患者均为成年女性,年龄分别为32岁和45岁,肿瘤均位于四脑室。肿瘤组织学形态呈片状生长模式,出现血管周围假性菊形团,真性室管膜菊形团,组织学分级CNS WHO 2级1例,3级5例。6例后颅窝室管膜瘤免疫组化染色结果显示肿瘤细胞GFAP、S-100均弥漫性强阳性表达,EMA、D2-40表现为核旁点状及腔缘阳性,P53大部分为野生型,oligo-2、Neu-N均阴性。PFA组出现特征性H3 K27Me3细胞核表达缺失,Ki-67增殖指数20%-60%。PFB组H3 K27Me3细胞核未缺失,Ki-67增殖指数4%-15%。结论后颅窝室管膜瘤PFA组好发于儿童且预后较差,通过H3 K27me3免疫组化染色初筛并分组,对于明确诊断及指导临床辅助治疗具有重要临床意义。

前庭神经鞘瘤三种不同手术入路的听力保留效果分析

随着显微神经外科技术的发展,前庭神经鞘瘤治疗目标由最初的生命保全转变为神经功能保留。继面神经成功保留后,保留听力(保听)成为新的目标。为实现这一目标,有多种治疗方案可供选择,但手术治疗仍然是当前最根本的治疗方式。保听手术入路包括乙状窦后入路、中颅窝入路和迷路后入路3种,每种入路都有其适用条件与优势,这些手术入路在近年得到广泛的拓展与进步。我们在了解前庭神经鞘瘤听力变化过程与治疗方案的基础上,比较了3种不同手术入路的保听效果。

儿童后颅窝肿瘤手术后小脑性缄默综合征研究进展

小脑性缄默综合征是儿童后颅窝肿瘤手术后的常见并发症,在不同病理类型肿瘤中的发生率存在较大差异(髓母细胞瘤最高,占24%~30%)。发病机制尚未阐明,认为与小脑⁃大脑回路损害、小脑顶核⁃中脑导水管周围灰质损害和脑网络改变相关。基于小脑性缄默综合征危险因素构建的预测模型尚未展现出预期的稳定性,仍未推广至临床。药物治疗主要基于临床经验,但疗效有待验证,故目前缺乏明确的有效治疗方法;非药物治疗如物理治疗、作业疗法和言语治疗,对改善远期生活质量有一定作用。本文综述小脑性缄默综合征的发病机制及治疗进展,未来研究应致力于探究小脑性缄默综合征的病理生理学机制、构建更准确的预测模型、制定个性化治疗方案,改善患儿远期预后。