Immunotherapy in combination with chemotherapy for Peutz-Jeghers syndrome with advanced cervical cancer:A case report

BACKGROUND Peutz-Jeghers syndrome(PJS)is a rare autosomal dominant disorder,and female patients may develop gynecologic tumours.The prognosis for such patients is poor and the specific pathogenesis remains uncertain.Therefore,there are currently no uniform treatment options.CASE SUMMARY Herein,we introduce the case of a 45-year-old female who was diagnosed with PJS for 45 years and cervical cancer for 3 years.Postoperative pathological examination showed metastases in the right external iliac lymph nodes.The patient was initially treated with a combination of doxorubicin and carboplatin chemotherapy and pelvic magnetic resonance showed that the metastases had grown.Subsequently,we performed whole exome sequencing in this patient and identified the relevant causative gene.In addition to the chemotherapy regimen,sindilizumab was administered and the patient was followed up.After 4 cycles of treatment,the metastases were substantially reduced and were not enlarged after six months of follow-up.This case report suggests that patients with PJS combined with cervical cancer may have a sustained response to immunecombination chemotherapy regimens.CONCLUSION Clinicians should be aware of the importance of immunotherapy in patients with PJS combined with advanced cervical cancer.

Vaginal clear cell adenocarcinoma in Herlyn-Werner-Wunderlich syndrome:A case report

BACKGROUND Herlyn-Werner-Wunderlich(HWW)syndrome is a rare Müllerian duct anomaly,characterized by a combination of urogenital abnormalities.The occurrence of primary cervico-vaginal carcinomas in patients with HWW syndrome is excep-tionally rare,posing significant challenges for screening,early diagnosis,and effective management.CASE SUMMARY We report a rare case of primary clear cell carcinoma of the vagina complicated in a 40-year-old woman with HWW syndrome.The patient presented with irregular vaginal bleeding for 4 years.On gynecological examination,an oblique vaginal septum was suspected.Surgical resection of the vaginal septum revealed a com-municating fistula and a tumor on the left vagina and the left side of the septum,which was confirmed as clear cell carcinoma.One month later,she underwent a radical hysterectomy,vaginectomy,bilateral salpingo-oophorectomy,and pelvic lymph node dissection.Due to significant side effects,she completed only one course of chemotherapy.A year later,lung metastasis was detected and continued to grow.A thoracoscopic wedge resection of the right upper lobe was performed 4 years after the initial surgery.We also conducted a systemic review of the lite-rature on primary cervical or vaginal carcinoma in HWW syndrome to explore this rare entity.CONCLUSION Cervico-vaginal adenocarcinomas in patients with HWW syndrome are occult,and require early surgical intervention and regular imaging surveillance.

Assessing the Validity of a Novel Model of Vertebral Artery Type of Cervical Syndrome Induced by Injecting Sclerosing Agent Next to Transverse Process of Cervical Vertebra

The efficacy of injecting sclerosing agent next to transverse process of cervical vertebra to induce vertebral artery type of cervical syndrome(CSA)was observed.Twenty rabbits were randomly divided into two groups:the model group and the control group.The rabbits in the model group were injected with sclerosing agent next to transverse process of cervical vertebray,on the contrary,the rabbits in the control group were injected with nothing.Transcranial Doppler(TCD)was used to detect the average speed of blo...

Anterior cervical corpectomy decompression and fusion for cervical kyphosis in a girl with Ehlers-Danlos syndrome:A case report

BACKGROUND Spinal deformities in Ehlers-Danlos syndrome(EDS; type VI) are generally progressive and severe. Surgical treatment has been described for kyphoscoliosis in the thoracolumbar spine. However, there are few studies describing the consequences of an anterior approach in cervical kyphosis. An anterior approach may not be able to fully decompress the spinal canal and restore the normal curvature of the cervical spine. Therefore, the anterior approach for cervical kyphosis in young children is hard. We describe the first case in an EDS girl with cervical kyphosis who received satisfactory anterior cervical corpectomy decompression and fusion.CASE SUMMARY The chief complaints of a 16-year-old girl with EDS were double upper limb weakness for 7 years and double lower limb walking instability for 2 years.Moreover, the imaging results revealed that the degree of kyphosis from cervical vertebra 2 to 4 accompanying with spinal cord compression was 30°. An anterior cervical corpectomy involving cervical vertebra 3 and a titanium mesh implant were performed with internal fixation. The results at 3 mo after surgery demonstrated that the anterior fusion was solid, and the kyphosis of the cervical spine was corrected. Additionally, the power of all four extremities was significantly improved.CONCLUSION The incidence rate of cervical kyphosis in EDS is rare. The surgical treatment for these patients, especially an anterior approach, is challenging. Therefore, to develop safer and more effective strategies to treat cervical kyphosis in EDS,there is still much work to do.

Klippel-feil:A syndrome in the occipital-cervical spine field and its dentofacial manifestations

Klippel-Feil syndrome(KFS) is defined by congenital cervical vertebral spine fusion and is seen with a wide spectrum of dental manifestations and craniofacial profiles. Previous studies on lateral cephalograms have documented an association between fusion of the cervicalvertebrae and deviations in the craniofacial profile in non-syndromic patients with severe malocclusion. To our knowledge, no previous studies have described the craniofacial profile including the cranial base of KFS patients on lateral cephalograms. Therefore KFS and its craniofacial and dental manifestations were described according to existing literature and additionally the craniofacial profile and cranial base was analysed on lateral cephalograms of two patients with KFS. According to the literature the dental manifestations of KFS-patients included oligodontia, overjet, cross bite, open bite and deep bite. The craniofacial profile was clinically described as reduced lower facial height, midfacial hypoplasia, and mandibular prognathia. The analyses of the two lateral cephalograms showed increased mandibular inclination, increased vertical jaw-relationship, increased jaw angle and maxillary retrognathia. The cranial base was normal in both cases. The sagittal jaw relationship and mandibular prognathia varied between the two cases. The literature review and the analyses of the two lateral cephalograms have shown that deviations in the occipital and cervical spine field as KFS were associated with deviations in the teeth and craniofacial profile.

CLINICAL STUDY ON TREATMENT OF CERVICAL SPONDYLOTIC SYNDROME WITH BEETLE NEEDLE

The authors treated 68 cases of cervical spondylotic syndrome with beetle nee-dle,a new needle apparatus(made by themselves)which is not inserted into the skin of the humanbody.A total of 15 sessions of treatment were given to every patient.Results showed that 56 cases(82％)were cured,7 cases(10.3％)improved and 5 cases(7.4％)invalid.Just as its name implies,the beetle is instead of acupuncture needle.All the diseases being able to be treated with acupunctureneedle could also be treated with beetle needle.In addition,the beetle needle therapy has no violentpain,is safe,effective and easy to be popularized. It also has no harm to viscerae,no cross infection,no dangers of broken needle and of failing during treatment,so,the patient needs not to be in fear.The therapy is preferable for patients to accept.

Interventional pain therapy in cervical post-surgery syndrome

Fifteen percent to forty percent of patients present with persistent disabling neck pain or radicular pain after cervical spine surgery. Persistent pain after cervical surgery is called cervical post-surgery syndrome(CPSS). This review investigates the literature about interventional pain therapy for these patients. Because different interventions with different anatomical targets exist, it is important to find the possible pain source. There has to be a distinction between radicular symptoms(radicular pain or radiculopathy) or axial pain(neck pain) and between persistent pain and a new onset of pain after surgery. In the case of radicular symptoms, inadequate decompression or nerve root adherence because of perineural scarring are possible pain causes. Multiple structures in the cervical spine are able to cause neck pain. Hereby, the type of surgery and also the number of segments treated is relevant. After fusion surgery, the so-called adjacent level syndrome is a possible pain source. After arthroplasty, the load of the facet joints in the index segment increases and can cause pain. Further, degenerative alterations progress. In general, two fundamentally different therapeutic approaches for interventional pain therapy for the cervical spine exist: Treatment of facet joint pain with radiofrequency denervation or facet nerve blocks, and epidural injections either via a transforaminal or via an interlaminar approach. The literature about interventions in CPSS is limited to single studies with a small number of patients. However, some evidence exists for these procedures. Interventional pain therapies are eligible as a target-specific therapy option. However, the risk of theses procedures(especially transforaminal epidural injections) must be weighed against the benefit.

A novel mouse model of central cord syndrome

Patients with potential spinal stenosis are susceptible to central cord syndrome induced by blunt trauma.Suitable animal models are helpful for studying the pathogenesis and treatment of such injuries.In this study,we established a mouse model of acute blunt traumatic spinal cord injury by compressing the C6 spinal cord with 5 and 10 g/mm~2 compression weights to simulate cervical central cord syndrome.Behavioral testing confirmed that this model exhibited the characteristics of central cord syndrome because motor function in the front paws was impaired,whereas basic motor and sensory functions of the lower extremities were retained.Hematoxylin-eosin staining showed that the diseased region of the spinal cord in this mouse model was restricted to the gray matter of the central cord,whereas the white matter was rarely affected.Magnetic resonance imaging showed a hypointense signal in the lesion after mild and severe injury.In addition,immunofluorescence staining showed that the degree of nerve tract injury in the spinal cord white matter was mild,and that there was a chronic inflammation reaction.These findings suggest that this mouse model of central cord syndrome can be used as a model for preclinical research,and that gray matter is most vulnerable to injury in central cord syndrome,leading to impaired motor function.

Neuroanatomy and clinical analysis of the cervical sympathetic trunk and longus colli

Anterior cervical surgery is commonly used for cervical vertebral body lesions. However, the structure of blood vessels and nerve tissues along the route of anterior cervical surgery is complex. We aimed to measure the data of the longus colli, the sympathetic trunk and the cervical sympathetic trunk （CST） ganglia in Chinese cadaver speci- mens. A total of 32 adult cadavers were studied. We delineated the surgical anatomy of the CST. The superior and inferior/cervicothoracic ganglia of the sympathetic trunk consistently appeared. The middle ganglion was observed in 28.1% of the specimens and there were 2 cases of unilateral double middle cervical ganglia. The inferior ganglion was observed in 25.0% of the specimens and the cervicothoracic ganglion was observed in the remaining speci- mens. The distance between the CST gradually decreased from the top to the bottom, and the distance between the medial edges of the longus colli gradually broadened from the top down. The average angle between the bilat- eral CST and the midline of the vertebra was 11.2°± 1.8° on the left side and 10.3°± 1.4° on the right side. The average angle between the medial margins of longus colli of both sides was 11.1°± 1.9°. The CST is at high risk when LC muscle is cut transversely or is dragged heavily, especially at the levels of C6 and C7. Awareness of the regional anatomy of the CST could help surgeons to identify and preserve it during anterior cervical surgeries.

Midtrimester pregnancy loss and cervical incompetence—Is there an association with connective tissue laxity?

Objectives: The basic mechanisms of cervical incompetence remain unknown although preliminary histological, biochemical and DNA studies suggest connective tissue pathology may be a contributing factor. Certain connective tissue disorders are known to be associated with obstetric complications. Utilising a standardised established scoring system for connective tissue laxity, this study aimed to test the relationship between clinical evidence of connective tissue laxity and cervical incompetence. Methods: This case-control study involved pregnant and non-pregnant women with a history of mid-trimester pregnancy loss in the absence of major bleeding, infection and uterine abnormalities and a control group of women with uncomplicated obstetric histories. Relevant medical details were obtained. Connective tissue laxity was assessed utilizing the Beighton scoring system. Potential confounding factors, including age, pregnancy and gestation were explored. Results: The frequency of connective tissue laxity between the cases [n = 29] was not statistically different from the controls [n = 58] [p = 0.391] suggesting a lack of association between cervical incompetence and clinical evidence of connective tissue laxity. Conclusion: Although no clear evidence of connective tissue laxity was demonstrated, it is possible that cervical incompetence is associated with abnormal connective tissue. But this abnormality is different from the defect that underlies joint hypermobility and skin elasticity.

Klippel-Feil syndrome

男性患者,33岁。主因交通事故后右肩、左前臂疼痛伴颈后触痛入院。颈椎X线检查可见C4~5椎体融合及其椎弓根融合。临床诊断:Klippel Feil综合征。未予治疗,建议患者避免颈部损伤,如出现上肢感觉异常或感觉缺失,立即复诊。

Establishment of an animal model of extra-vertebral foramen cervical nerve entrapment by local electrical stimulation and selection of suitable stimulation parameters

A rat model of extra-vertebral foramen cervical nerve entrapment was established according to the following parameters： stimulation intensity 20 V; frequency 50 Hz; pulse width 200 μs; duration 333 ms/s for a total of 8 hours. After the electrical stimulation, rats exhibited mild muscle fiber atrophy, mild inflammatory exudates, connective tissue local fibrosis and chondrocyte metaplasia. Mean muscle fiber cross-sectional area was reduced. The nerve myelin sheath continuity was partially demyelinated. The microstructure of nerve cells was disrupted and these symptoms worsened with prolongation of the stimulation. The shoulder, neck and upper extremity muscles on the tested side demonstrated positive sharp waves and fibrillations. The severity increased with continuation of the stimulation. High amplitude and polyphasic motor unit potentials gradually appeared. Similar findings were seen in the contralateral side, but at a less severe level.

White Cord Syndrome: The Terrible Nightmare

Introduction: Reperfusion injury of spinal cord “white cord syndrome” refers to acute neurological deterioration after decompressive spinal surgery without any reported intraoperative events. The postoperative appearance of diffuse hyperintense intramedullary areas in T2-weighted MRI is characteristic without any other pathological or compressive signs. The etiology may be due to sudden expansion of the cord after reperfusion of chronically ischemic areas leading to disruption of blood-spinal cord barrier. The incidence and risk factors of the syndrome are not well documented in the literature. Methods: This is a retrospective case series that included 150 patients with cervical spondylotic myelopathy who had undergone decompression surgery in our institution from 2017 to 2019. Patients’ demographics, clinical, imaging characteristics, intraoperative and postoperative data were collected and analysed. Results: In this study, we documented 7 cases of this syndrome. We found that advanced age, chronic hypertension, pre-existing cord signal in MRI and long history of symptoms may be risk factors for this syndrome. Conclusion: Early diagnosis and prompt treatment of this syndrome with steroids and physical therapy may lead to initial improvement. Awareness of the surgeons about this syndrome should be increased and identifying its risk factors is very important for patients counseling.

腹部摩法结合颈椎旋转定位扳法治疗痰湿阻络型椎动脉型颈椎病患者的疗效观察

目的探讨腹部摩法结合颈椎旋转定位扳法治疗椎动脉型颈椎病痰湿阻络证的效果。方法选取2022年1月—2022年12月期间黑龙江中医药大学附属第二医院收治的90例椎动脉型痰湿阻络证患者,采用随机数字表法分为腹摩组、扳法组、联合组,每组各30例。3组患者均给予西药与常规推拿治疗,在此基础上,腹摩组施以腹部摩法,扳法组施以颈椎旋转定位扳法,联合组给予腹部摩法+颈椎旋转定位扳法,3组患者均治疗2周。观察比较3组患者临床疗效、安全性以及治疗前后中医证候积分、眩晕症状评分(Evaluation scale for cervical vertigo,ESCV评分)、颈椎功能评分[颈椎功能障碍指数(Neck disability index,NDI)及日本骨科协会(Japanese orthopaedic association scores,JOA)]、椎-基底动脉血流动力学[平均血流速度(Velocitymean,Vm)、收缩期峰值血流速度(Velocity systolic,Vs)、舒张期末血流速度(Vend of diastolic velocity,Vd)、阻力指数(Resistance index,RI)、搏动指数(Pulsation index,PI)值]。结果治疗后联合组临床总有效率96.67%(29/30)高于腹摩组70.00%(21/30)与扳法组80.00%(24/30),差异有统计学意义(P<0.05)。治疗后3组患者中医证候积分均较治疗前降低,差异有统计学意义(P<0.05);且联合组中医证候积分明显低于腹摩组与扳法组,差异有统计学意义(P<0.05)。治疗后3组患者ESCV评分均较治疗前降低,差异有统计学意义(P<0.05);且联合组ESCV评分明显低于腹摩组与扳法组,差异有统计学意义(P<0.05)。治疗后3组患者NDI评分较治疗前降低,JOA评分较治疗前升高,差异有统计学意义(P<0.05);且联合组NDI评分明显低于腹摩组与扳法组,JOA评分明显高于腹摩组与扳法组(P<0.05)。治疗后3组患者椎-基底动脉Vm、Vs、Vd参数均较治疗前升高,RI、PI参数均较治疗前降低,差异有统计学意义(P<0.05);且联合组Vm、Vs、Vd参数明显高于腹摩组与扳法组,RI、PI参数明显低于腹摩组与扳法组,差异有统计学意义(P<0.05)。治疗期间,3组患者安全性分级与不良反应发生率比较,差异无统计学意义(P>0.05)。结论腹部摩法结合颈椎旋转定位扳法治疗椎动脉型颈椎病痰湿阻络证安全、有效,有利于改善颈椎功能,调节椎-基底动脉血流动力学,进而缓解眩晕症状。

基于“筋、节、骨、髓”分型探讨神经根型颈椎病的手法治疗

神经根型颈椎病(CSR)是一种由于颈椎间盘和椎间关节退变引起的疾病,主要表现为颈部、肩部及上肢神经支配区域的疼痛、感觉异常和运动障碍。传统中医的筋骨辨证在治疗该病时存在概念不清、辨证分型不一等问题。岐黄学者林定坤教授在筋骨辨证的基础上,结合现代解剖学提出了颈椎“筋”“节”“骨”“髓”4大分型。该文通过总结林定坤教授对CSR筋骨辨证学术思想,总结手法治疗该病的作用靶点,提出基于筋劳、节错、骨变和髓损等4种病损分型,采用理筋、调节、护髓和治骨4种手法治疗,以期完善骨伤科手法治疗CSR的辨证施治体系,为临床提供参考。

桂枝葛根汤联合循经走罐法治疗神经根型颈椎病的效果观察

目的 分析桂枝葛根汤联合循经走罐法对神经根型颈椎病(风寒阻络证)的治疗价值。方法 回顾性分析126例于2021年5月至2023年6月天津市南开区中医医院诊治的神经根型颈椎病患者资料,均属风寒阻络证,以2021年5月至2022年3月诊治的63例患者为对照组,以2022年4月至6月诊治的63例患者为观察组,对照组行常规治疗,观察组在对照组基础上加桂枝葛根汤联合循经走罐法治疗。经4周治疗后,比较两组病情程度相关评分,颈椎活动度,血流动力学及临床疗效。结果 治疗后,观察组颈椎病临床评价量表(CASCS)评分明显较对照组高,简化McGill疼痛问卷量表(SF-MPQ)及颈椎功能障碍指数(NDI)评分明显较对照组低(P<0.05);治疗后,观察组颈椎活动角度明显较对照组大(P<0.05);治疗后,观察组基底动脉阻力指数(RI)、搏动指数(PI)明显较对照组低,平均血流速度(Vm)明显较对照组高(P<0.05);治疗后,观察组、对照组总有效率分别为92.06%、77.78%,观察组明显较高(P<0.05)。结论 桂枝葛根汤联合循经走罐法治疗神经根型颈椎病(风寒阻络证),能促进病情改善,增大颈椎活动范围,调节血流动力学指标。

NT与血清β-hCG、PAPP-A、ADAM12单独及联合检测对孕早期DS的诊断价值

目的:探讨颈部透明层厚度(NT)与血清人绒毛膜促性腺激素(β-hCG)、妊娠相关蛋白A(PAPP-A)及解整合素金属蛋白酶12(ADAM12)单独及联合检测对孕早期唐氏综合征(DS)的诊断价值。方法:选取2019年1月—2023年1月在厦门大学附属第一医院杏林分院经羊膜穿刺确诊的47例DS孕妇作为DS组,另选取同期49例产前检查正常的孕产妇作为对照组,检测并比较两组NT及血清β-hCG、PAPP-A、ADAM12水平,并采用受试者工作特征(ROC)曲线分析NT与血清β-hCG、PAPP-A、ADAM12单独及联合检测对DS的诊断价值。结果:DS组NT及血清β-hCG水平高于对照组,PAPP-A、ADAM12水平均低于对照组,差异有统计学意义(P<0.05)。ROC曲线分析显示,NT与血清β-hCG、PAPP-A、ADAM12联合检测诊断DS的曲线下面积为0.993,预测敏感度、特异度分别为95.7%、98.0%,均高于上述指标单独检测。结论:NT与血清β-hCG、PAPP-A、ADAM12检测对孕早期DS均具有一定的诊断价值,且联合应用诊断效果更高,可作为孕早期DS筛查的有效指标。

葛根汤联合中药热敷治疗神经根型颈椎病风寒阻络证临床观察

目的 观察葛根汤联合中药热敷治疗神经根型颈椎病风寒阻络证的临床效果。方法 选取吉林省吉林中西医结合医院骨伤科2021年7月—2022年7月收治的90例神经根型颈椎病风寒阻络证患者,运用掷硬币法分为2组,各45例。对照组用葛根汤治疗,观察组使用葛根汤加中药热敷治疗,比较2组颈椎功能障碍评分、临床疗效、颈肩疼痛程度评分。结果 治疗后,对照组总有效率低于观察组(P<0.05);观察组的颈椎功能障碍评分与颈肩疼痛程度评分更低(P<0.05)。结论 葛根汤联合中药热敷治疗神经根型颈椎病风寒阻络证临床效果较好,优于单一使用葛根汤治疗的方式,有较高临床应用价值。

妊娠合并宫颈大细胞神经内分泌癌和低钠血症一例

回顾分析1例妊娠合并宫颈大细胞神经内分泌癌ⅢC1(r)期剖宫产术后行静脉化疗发生低钠血症的患者,经放化疗治疗原发肿瘤,对症治疗纠正低钠血症达完全缓解后6个月,出现疾病复发,通过化疗联合靶向及免疫治疗,患者因出现分布性休克、电解质紊乱,患者家属放弃治疗,随访至2023年5月,患者死亡。宫颈大细胞神经内分泌癌是一种恶性程度高、预后差的恶性肿瘤,患者出现低钠血症可能与肿瘤的不良预后相关,临床上容易被化疗的不良反应所掩盖。临床医生应掌握低钠血症的临床表现,提高早期识别的能力,做到早诊断、早治疗,积极改善患者预后。

宫颈大细胞神经内分泌癌伴抗利尿激素分泌异常综合征一例

宫颈大细胞神经内分泌癌(large cell neuroendocrine carcinoma,LCNEC)是一种具有内分泌功能的恶性肿瘤,是抗利尿激素分泌异常综合征(syndrome of inappropriate secretion of antidiuretic hormone,SIADH)的病因之一。报告1例妊娠合并宫颈LCNEC患者,入院时存在轻度低钠血症,剖宫产终止妊娠后行化疗,化疗过程中出现了重度低钠血症伴抽搐、意识不清,诊断为SIADH。给予镇静镇痛、补钠、补钾、降颅压、纠正酸碱失衡等抢救措施后,患者血Na+水平恢复正常,随后行3个周期顺铂+依托泊苷方案化疗以及同步放化疗。最终患者肿瘤复发,因Ⅳ度骨髓抑制、体能状况差,无法耐受抗肿瘤治疗而终止治疗后死亡。SIADH早期识别与诊断困难,诊治不当可导致认知障碍、癫痫、昏迷和渗透性脱髓鞘综合征等,影响患者预后。