Endoscopic repair technique for traumatic penetrating injuries of the clivus

Unlike basilar skull fractures, penetrating traumatic injuries to the clivus are uncommon. We present two novel and interesting cases of traumatic crossbow arrow injury and penetrating screwdriver injury to the clivus. A review of the literature describing methods to repair these injuries was performed. A careful, systematic approach is required when working up and treating these injuries, as airway preservation is critical. An adaptation to the previously described “gasket-seal” method for skull base repair was utilized to repair the traumatic cerebrospinal fluid (CSF) fistulas. This repair technique is unique in that it is tailored to a much smaller defect than typical post-surgical defects. Two patients are presented, one with a post-traumatic CSF fistula after penetrating crossbow injury to the clivus and one with a penetrating screwdriver injury to the clivus. The patients were treated successfully with transnasal endoscopic repair with fascia lata graft and a nasoseptal flap, a novel adaptation to the previously described “gasket-seal” technique of skull base repair.     

Bone invasiveness is associated with prognosis in clivus chordomas

Clivus chordomas present a great challenge for neurosurgeons, and the prognosis is poor. To investigate bone invasiveness characteristics in regard to the prognosis of clivus chordomas, a retrospective study of 19 patients with primary clivus chordoma was performed. Clinical data, MRI, CT scans and follow-up data were examined, and the bone invasiveness of the chordoma was classified into two growth patterns (endophytic and exophytic) which were analyzed with regard to prognosis. The overall survival rate was 78.9% with radical surgery and adjuvant radiation, with a mean follow-up of 44.5 months. There were 12 patients in the endophytic group and seven in the exophytic group, and the exophytic group exhibited a higher recurrence rate than the endophytic group (p = 0.006). Chordomas with an exophytic growth pattern were more likely to recur than those with an endophytic growth pattern, and the surgical approach can be tailored according to each growth pattern.     

Lower clivus and foramen magnum anterolateral meningiomas: Surgical strategy

Abstract

Lower clivus and anterolateral foramen magnum meningiomas are rare but challenging tumors. Indeed, all access routes to their intradural anterolateral implantation appear unsafe since highly-functional local structures may not be sacrified or even retracted. Anterior and posterior surgical approaches have specific advantages and limitations. Different lateral extensions of the posterior approach have been described. They include a transposition of the vertebral artery, a partial occipital condylectomy and even the exposure and the section of the sigmoid sinus. Such posterolateral approaches offer the optimal access to anterolateral foramen magnum meningiomas and allow a total removal with a minimal morbidity. We report the surgical strategy that we applied for the last 6 anterolateral foramen magnum meningiomas in our department. [Neural Res 1998; 20: 577–584]     

Clivus metastases: Report of seven patients and literature review

Background  Tumours of the clivus are rare and metastases involving this area have been previously described only as single case reports or included in series with other skull base tumours. Here, we describe seven such examples and review the pertinent literature. Method  Clinical, radiological and follow-up data of patients who had undergone surgery for clivus tumours at our Institution between January 1995 and December 2007 were retrospectively collected. A literature review was performed using PubMed. Findings  Of 46 patients who underwent surgery for clivus bone tumours, seven proved to harbour a metastasis. This figure represents 0.18% and 0.42% respectively of intracranial and skull base tumours which were treated in our Institution in the study period. The primary tumours associated were lung adenocarcinoma (n = 2), prostate carcinoma (n = 2), skin melanoma (n = 1), hepatocarcinoma (n = 1) and lung squamous cell carcinoma (n = 1). All patients presented with a sixth nerve palsy as the symptom. Histopathology was obtained via a trans-sphenoidal biopsy. In spite of radiotherapy and chemotherapy, the mean survival was 12 months. On literature review, 27 examples of metastases located in the clival bone were found. Including our series, the most common primary tumours were prostate cancer (26.4%), thyroid carcinoma (11.7%) and hepatocarcinoma (11.7%). Conclusion  Though exceedingly rare, metastases involving the clivus should be considered in the differential diagnosis with clivus chordoma. The metastatic lesion might be a late and single expression of the primary tumour. The trans-sphenoidal approach is the ideal procedure to establish a histopathological diagnosis. Prognosis is poor.     

Infantile clivus chordoma without clivus involvement: case report and review of the literature

Background We present a giant clival chordoma with disseminated disease but without involvement of the clivus. To our knowledge, this is the youngest child and only the second case, presenting without base of skull involvement, in paediatric literature and the fourth reported case of a chordoma in a patient with tuberous sclerosis.Discussion We discuss the subtle presentation, difficulties in diagnosis and management and also review the literature.     

Variability in the enhancement of the normal central skull base in children

Summary We studied the signal and enhancement characteristics of the central skull base prospectively in 40 children aged 13 days to 8 years, on a 1.5 T MRI system. Identical standard short TR/TE spin echo sequences in the sagittal plane were performed before and after intravenous gadolinium-DTPA. The sequences used for comparison were filmed at identical window and level settings. Three independent observers assessed (1) the intensity of contrast enhancement of the basisphenoid, basiocciput and presphenoid, (2) the signal intensity of the spheno-occipital synchondrosis, (3) the degree of pneumatization of the sphenoid sinus and (4) the uniformity of signal intensity reflecting fatty replacement of the marrow of the basisphenoid, basiocciput and presphenoid. In 16% and 28% respectively of cases there was no enhancement of the basisphenoid and basiocciput after gadolinium administration; in 42% and 44% there was mild enhancement, and moderate or intense enhancement was observed in 42% and 28% of cases. Even when there was irregular fatty replacement, residual hemopoietic clements could enhance intensely. When skull base neoplasms are being investigated, the normal signal irregularity and enhancement of the central skull base in children must not be confused with pathologic invasion of the marrow.     

Endoscopic endonasal transsphenoidal surgery for sellar tumors in children

Objective

Endoscopic endonasal transsphenoidal surgery (EETS) is still rarely used in pediatric subjects compared with adults. Reports on EETS in children appeared only recently in the literature, usually regarding small series. The aim of the study is to assess the actual role and the limits of EETS in children with sellar tumors by reporting a two-centers experience.

Methods

Twenty-seven children (mean age: 12.2 years) were operated on during the last decade. Seventeen patients harbored a sellar mass, 7 a suprasellar tumor, and 3 a clival mass. Laboratory investigations revealed hypopituitarism in 6 children and hormone hypersecretion in 9. All the operations were carried out by a team including both ENT surgeon and neurosurgeon using a dedicated 18-cm long rigid endoscope (2.7 mm and 4 mm diameter) through the direct paraseptal or the transethmoidal or the transpterygoid route.

Results

Overall, 29 operations were performed. Gross total tumor resection was obtained in 22 children (81.5%) while a subtotal and a partial removal in 2 (7.5%) and 3 cases (11%), respectively. Pituitary adenoma was the most common histotype (12 cases), followed by craniopharyngioma (5) and Rathke's cleft cyst (4). No surgical mortality or neurological morbidity or late nasal complications were observed. Postoperative CSF fistula occurred in 3 patients. All children are alive at current follow-up (average: 8.6 years). Preoperative hypopituitarism disappeared or improved in 4 cases and was stable in the remaining 2 (no new hormone deficits appeared).

Conclusion

EETS is a safe and effective surgical option also in children. As for adults, it allows to manage most of the tumor lesions of the sellar region with stable long-term results.     

Lymphocytic panhypophysitis in a young man with involvement of the cavernous sinus and clivus

Lymphocytic hypophysitis is an unusual inflammatory lesion that is caused by autoimmune destruction of the pituitary gland. We report a case of 42-year-old man who presented with a 6-month history of severe headache, blurred vision in the right eye, hearing loss, polyuria, polydipsia, and impotence. Medical history showed that he and his mother had osteopetrosis. The results of the physical examination and laboratory tests showed that secondary hypothyroidism, hypogonadism, and hypocortisolism had developed. Central diabetes insipidus was diagnosed by water deprivation test. MRI of the sella showed pituitary enlargement with symmetrical suprasellar expansion, compression of the chiasma, thickened infundibulum, and involvement of both bilateral cavernous sinuses and clivus. Hormonal substitution with hydrocortisone, levothyroxine, and DDAVP resulted in rapid improvement of all symptoms and signs. Transsphenoidal biopsy was diagnostic of lymphocytic hypophysitis. In spite of extensive literature reviewing, we have not been aware of any case of lymphocytic hypophysitis with clivus involvement. The present case represents a variant of lymphocytic hypophysitis which has progressed to involve bilateral cavernous sinuses and the clivus.     

经鼻蝶入路行斜坡区手术蝶窦斜坡隐窝的CT评价

目的：通过对经鼻蝶人路行斜坡区手术斜坡隐窝的CT评价,为术前手术入路的设计提供影像学依据。方法：93例患者行头颅螺旋CT扫描,多平面重建后,在正中矢状面上,根据蝶窦、斜坡和垂体的位置关系,评价斜坡隐窝并对其进行分型,比较不同分型患者斜坡不同位置厚度的差异。结果：本组斜坡隐窝出现率为65％（60／93）。该隐窝位于蝶鞍下方（鞍下型）、伸入鞍后（鞍后型）或枕骨基底部（枕型）者分别占13％（12／93）、29％（27／93）和23％（21／93）。无斜坡隐窝型蝶窦共33例（35％）,其斜坡各部分均较其它类型蝶窦厚;枕型和鞍后型斜坡隐窝的斜坡上部、中部较其它类型蝶窦薄,枕型蝶窦的斜坡下部较其它类型蝶窦薄。结论：术前利用螺旋CT对斜坡隐窝进行评价有助于经鼻蝶入路行斜坡区手术人路的设计。     

斜坡肿瘤的MRI诊断

目的评价颅底区域肿瘤的MRI诊断价值。材料与方法经手术或穿刺活检病理证实的颅底肿瘤58例（如鼻咽癌、脊索瘤、侵袭性垂体瘤等），全部病例进行了MRI检查。结果侵袭性垂体瘤、脑膜瘤等少数肿瘤的MR信号具有特征性，肿瘤的中心部位对定性诊断有所帮助，当病变破坏广泛时鉴别比较困难。结论MRI可充分显示肿瘤的范围，部分肿瘤可依据其信号、部位与侵犯特征给予定性诊断。