Synovial cyst at the craniovertebral junction

A case of synovial cyst of the upper cervical spine that resulted in spastic quadriparesis and sensory loss is reported. Radiographic evaluation included a computed tomography scan after myelography and a magnetic resonance imaging scan. The synovial cyst was removed by a laminectomy at C-1 and C-2, combined with a foramen magnum craniectomy. The patient had a good recovery.     

枕颈结合部肿瘤诊断和外科治疗

枕颈结合部位于颅底和颈椎交界区域,位置深在,解剖结构复杂.该区域一旦发生肿瘤,早期诊断往往困难,而手术治疗更具风险性和挑战性.随着近年影像诊断技术的不断提高,特别是枕颈部外科技术的不断发展,枕颈结合部肿瘤的诊断和外科治疗水平正逐步提高.该文就诊断步骤和治疗策略作一综述.     

Cervical intramedullary schwannoma: complete excision using the KTP laser

Intramedullary cervical spinal cord schwannomas are rare tumours and complete excision is often an elusive goal. The use of the KTP laser to accomplish complete excision has not been reported previously. Postoperatively, our patient had no additional deficit and after 1 year has made a good recovery. This case further highlights the difficulty in interpretation of intraoperative biopsy specimens.     

Tuberculosis of the craniovertebral junction

Purpose

Tuberculosis (TB) of craniovertebral junction (CVJ) occurs in 1–5 % of cases of TB spondylitis. This can be a life-threatening condition due to mass effect of infective process or resultant instability. Surgical indications for TB of CVJ are not clear from literature.

Methods

We have reviewed all the patients with TB of CVJ admitted at our center between 2005 and 2010.

Results

There were 15 patients including 10 males and 5 females. Average age was 38 years and average duration of symptoms was 8 months. All patients were started on multidrug antituberculous chemotherapy and skull traction. Those patients who failed to respond in 4–6 weeks and had persistent instability or neurological deficit were offered surgery. Rest was treated conservatively by immobilisation or traction. All five patients who were surgically treated had occipitocervical fusion (OCF) with titanium screws and plate/rod construct combined with posterior decompression if needed. Only one patient needed anterior surgery in addition to OCF at a later stage. All patients improved neurologically whether they were treated surgically or conservatively. Only difference was that surgically treated patients had earlier pain relief, mobilisation, neurological improvement and lesser complications.

Conclusion

We recommend that all patients with TB of CVJ with instability and neurological compromise, who fail to respond to 4–6 weeks of antituberculous chemotherapy and skull traction should be offered occipitocervical fusion with or without posterior decompression. Anterior surgery will be needed only in those few cases who do not improve neurologically after OCF.

     

Intradural epithelial cyst at the craniovertebral junction. Case report

A case of an intradural epithelial cyst at the craniovertebral junction is reported in a 37-year-old man. The classification of these rare lesions is discussed.     

Infantile myofibromatosis of the craniovertebral junction

Infantile myofibromatosis (IM) is a rare pathological entity characterized by solitary or multiple nodular skin, soft tissues or bony lesions. Craniovertebral (CV) junction lesions are rare. We report the successful management of a solitary IM involving the posterior elements of the CV junction in a 6-month-old child.     

Management of tuberculomas of the craniovertebral junction

Tuberculosis of the craniovertebral junction is extremely rare. However, recent evidence suggests that the incidence of this condition may be increasing in the United Kingdom. The diagnosis is often difficult despite advances in imaging using MRI. CT guided biopsy of lesions often yields inconclusive results. The transoral approach to the anterior craniovertebral junction provides excellent access to this region with a low operative morbidity and mortality, enabling biopsy of lesions and decompression of the neuraxis. Management of secondary atlantoaxial instability, regarding both timing and method of stabilization, is controversial. We report two cases of tuberculomas of the craniovertebral junction, that illustrate the role of transoral surgery in both diagnosis and treatment of this condition. Previous management strategies are reviewed and future recommendations are presented.     

Factors predicting growth of vestibular schwannoma in neurofibromatosis type 2

We retrospectively reviewed characteristics of patients with neurofibromatosis type 2 to identify factors predicting further growth of bilateral vestibular schwannomas. Subjects comprised 27 neurofibromatosis type 2 patients with 54 vestibular schwannomas, followed for 24–204 months (mean, 86 months). This study investigated factors predictive of vestibular schwannoma growth in neurofibromatosis type 2. Features distinguishing actively growing from quiescent VS were determined for untreated course (28 vestibular schwannomas) and posttreatment course (including either resection or radiosurgery; 33 vestibular schwannomas). A general estimation equation was used to identify factors affecting tumor growth. During the untreated course, 19 vestibular schwannomas showed growth and 9 vestibular schwannomas were stable. No factors predictive of growth were shown. During the posttreatment course (23 surgical resections, ten radiosurgeries), ten treatments were followed by growth and 23 were followed by stability, with growth showing an association with onset at an early age (p = 0.007). Multivariate analysis identified no factors predictive of growth. After treatment, close follow-up is warranted for patients with onset at an early age.     

Magnetic resonance imaging of the normal craniovertebral junction

Sagittal magnetic resonance images of the normal craniovertebral junction in 25 patients were examined for visualization of bony, synovial, and ligamentous structures. The excellent delineation of soft tissue by magnetic resonance imaging enabled recognition of the joint space between the dens and anterior arch of C1 in 14 out of 25 patients. High-signal-intensity tissue was noted immediately superior to the dens in all patients; an anatomic specimen confirmed the fibrofatty nature of this tissue. The medullary space of the dens had lower signal intensity than did the marrow in the body of C2 in more than one half of the cases. Additional thin-section images suggested that this was a partial-volume artifact. Understanding of the normal appearances of structures in this region is necessary to assess correctly the presence or absence of disease.     

寰椎枕骨化畸形患者的椎动脉走行

目的:观察寰椎枕骨化畸形患者椎动脉第=三段的走行情况,探讨其分型方法.方法:对连续收治的36例诊断为寰椎枕骨化畸形患者进行椎动脉的CT血管造影检查,观察椎动脉的走行情况.结果:根据椎动脉进入椎管和颅腔的位置由远到近分为Ⅰ、Ⅱ、Ⅲ、Ⅳ型,36例患者中5例6侧椎动脉从枢椎横突孔穿出,横行向内,于枕骨化寰椎后弓下方进入椎管,再向上进入枕骨大孔,椎动脉经过寰椎侧块的下方走行,占8.3%,为Ⅰ型;16例18侧椎动脉从枢椎横突孔穿出,横行向内,于枕骨化寰椎后弓下方进入椎管,再向上进入枕骨大孔,椎动脉走行经过寰椎侧块的表面,或在其表面转弯,占25%,为Ⅱ型;25例44侧椎动脉从枢椎横突孔穿出,向上、外方走行,沿寰椎与枕骨之间的骨性孔道进入颅内,占61.1%,为Ⅲ型;4例4侧椎动脉缺如,占5.6%,为Ⅳ型.结论:寰椎枕骨化畸形患者的椎动脉第三段有四种解剖形态,对寰椎枕骨化患者置入寰椎侧块螺钉时,Ⅱ、Ⅲ型椎动脉走行者损伤椎动脉的风险较高.对单侧椎动脉缺如者,对侧手术操作应更加谨慎.     

Plexiform schwannoma of the esophagus in a child with neurofibromatosis type 2

Schwannoma is a benign neoplasia of the peripheral nerve sheath. Its localization in the gastrointestinal tract, and in particular in the esophagus, is very rare. According to the existing literature esophageal schwannoma has been reported so far only in adult patients. We report the case of an 11 year old patient with neurofibromatosis, type 2, who underwent surgical excision of a plexiform schwannoma of the esophagus.     

Calcium pyrophosphate dihydrate crystal deposition in the craniovertebral junction

OBJECT: Calcium pyrophosphate dihydrate (CPPD) deposition is a rare cause of retroodontoid mass lesions in elderly individuals. However, this condition may be severely underdiagnosed if sufficient attention is not paid to imaging characteristics and clinical presentation. The authors sought to evaluate the decision-making process in both the diagnosis and surgical treatment of CPPD. METHODS: A retrospective review of University of Iowa medical records and radiographs accumulated between 1977 and 2006 was performed. The inclusion criterion was histopathological findings consistent with pseudogout at the craniovertebral junction (CVJ). Twenty-one patients with a mean age of 70.3 years and a mean symptom duration prior to presentation of 17.5 months were identified and included in this study. RESULTS: The patients presented most frequently with occipital and neck pain (85%) and numbness or paresthesias (61%). Lower cranial nerve deficits were seen in 29%. Calcification of the mass or transverse ligament was seen on computed tomography in all included patients. Gross-total resection was achieved in all patients: 19 of 21 patients underwent transoral-transpalatopharyngeal resection, with only 16 requiring concomitant dorsal occipital-cervical fusion. The mean follow-up duration was 15 months. Eighteen patients (86%) had improvement or resolution of symptoms after treatment, and 3 were lost to follow-up. CONCLUSIONS: Although rare, CPPD deposition at the CVJ should be suspected on finding calcification of and around the transverse ligament on neuroimaging. Transoral-transpalatopharyngeal resection is preferred to halt the progression of neurological deterioration; dorsal fusion is not always mandatory as concomitant ligamentous calcification and atlantoaxial joint ankylosis may provide added stability.     

Predictors of vestibular schwannoma growth in patients with neurofibromatosis Type 2

OBJECT: The results of two longitudinal studies of growth rates of vestibular schwannomas (VSs) in patients with neurofibromatosis Type 2 (NF2) differ as to whether VS growth rates decrease or increase with increasing patient age. The authors undertook this study to assess the relationship between VS growth rates and patient age and type of constitutional NF2 mutation; they also examined variability in VS growth rates among multiple patients in families with NF2. METHODS: Gadolinium-enhanced magnetic resonance images obtained in 18 patients with inherited NF2 from 11 unrelated families were retrospectively analyzed. The patients had been observed for a median of 4 years. Volumes of the VSs were measured using a two-component box model (intrameatal and extrameatal parts measured separately). Single-strand conformation polymorphism analysis and Southern blot analysis were used to identify constitutional NF2 mutations. Growth rates of the VSs were highly variable, but tended to decrease with increasing patient age both at onset of signs or symptoms of NF2 (r2 = 0.35, p = 0.026) and at diagnosis (r2 = 0.33, p = 0.012). The VS growth rates did not vary significantly with the type of constitutional NF2 mutation or the number of non-VS cerebral or spinal tumors. The VS growth rates were highly variable within families and did not correspond to clinical indices of NF2 disease severity, such as patient age at symptom onset and the number of non-VS cerebral and spinal tumors. CONCLUSIONS: The growth rates of VSs in patients with NF2 are highly variable, but tend to decrease with increasing patient age. Clinical treatment of multiple patients in families with NF2 cannot be based on the expectations of similar VS growth rates, even when other clinical aspects of disease severity are similar.