Double-level cervical total disc replacement for adjacent segment disease: is it a useful treatment? Description of late onset heterotopic ossification and review of the literature.

We report a rare case of double-level adjacent segment disease (ASD), occurring ten years later an anterior cervical discectomy (ACD) without fusion, treated by cervical arthroplasty, highlighting the outcome at long-term follow-up and focusing on heterotopic ossification. In 1995 a 25-year-old man satisfactorily underwent ACD at C4/C5. At that time MRI also showed signs of degenerative disc disease (DDD) at C3/C4 and C5/C6. Ten years later, a new MRI scan showed a large C3/C4 and a smaller C5/C6 soft disc hernia together with spondylotic changes at the level above and below the site of the first surgery. At C4/C5 imaging revealed a kyphotic stable "pseudoarthrosis" with anterior bridging osteophyte. The patient underwent double-level arthroplasty with ProDisc-C. Clinical and radiological outcome was satisfactory. 3 and 5 years after surgery, X-rays and CT scan documented the progressive development of heterotopic ossification, with gradual reduction of range of motion. A late onset heterotopic ossification can neutralize the theoretical advantages of cervical arthroplasty, which should be considered an effective surgical option only in selected cases. ACDF and restoration of normal lordosis can be a viable alternative in cervical revision surgery, as motion preservation can not be always mantained for a long time.

Anterior, extracanalar, cervical spine osteochondroma associated with DISH: description of a very rare tumor causing bilateral vocal cord paralysis, laryngeal compression and dysphagia. Case report and review of the literature.

INTRODUCTION: Osteochondromas are common benign bone tumors, rarely involving the spine. BACKGROUND: Osteochondroma account for about 35% of bone benign tumors and 9% of all bone tumors. Spinal involvement is described in 1%-4% of cases and their origin from the anterior surface of cervical vertebral bodies is exceedingly rare. AIM: We describe the rare case of an osteochondroma arising from the anterior surface of the C4 and C5 vertebral bodies, and not involving the spinal canal, in a 68-year-old male patient suffering from Diffuse Idiopathic Skeletal Hyperostosis (DISH). MATERIALS AND METHODS: The patient presented with acute onset of respiratory distress due to laryngeal compression exerted by the lesion, dysphagia and paralysis of left vocal cord. Imaging revealed the unusual lesion compressing and dislocating the air ways. An anterior approach to cervical spine was performed to remove the tumor. RESULTS: Postoperatively, the patient sustained a progressive improvement of respiratory function and recovery of the vocal cord paralysis. DISCUSSION: We believe that this case holds some interesting peculiarities: firstly, the anterior location of a cervical osteochondroma could be considered exceptional; yet, the resulting clinical picture should be remembered for differential diagnosis. Secondly, we hypothesized a possible correlation between the tumorigenesis of osteochondroma and the co-existence of DISH. Indeed, the abnormal bone turnover in cervical segments due to DISH could explain the unusual occurrence of osteochondromas in adult age. CONCLUSIONS: The occurrence of an osteochondroma should be considered in patients suffering from DISH and harbouring large osteophytes.

Prognostic value of EGFR expression in de novo and progressed atypical and anaplastic meningiomas: an immunohistochemical and fluorescence in situ hybridization pilot study.

AIM: The aim of this study was to assess both the epidermal growth factor receptor (EGFR) protein expression by immunohistochemistry and the EGFR gene amplification by fluorescence in situ hybridization in meningiomas of different grade, in order to evaluate their possible role in the development of the disease. EGFR protein belongs to the family of tyrosine kinase growth factor receptors, which also includes HER2, HER3 and HER4. Elevated expression or activity of EGFR has been reported in several cancers, including brain tumours. EGFR activation can enhance the malignant potential of epithelial tissues. METHODS: We investigated whether there was a difference in the EGFR protein expression and the EGFR gene amplification between the so called de novo malignant meningiomas and recurrent meningiomas with or without malignant progression from a previously lower grade tumor. Our goal was to evaluate if EGFR expression was a useful marker to select patients affected by meningioma with a major risk of recurrences. We also assessed the prognostic value of the EGFR expression on overall survival. RESULTS: Progression from benign meningiomas to atypical or anaplastic meningiomas correlated with an increase in the expression of EGFR protein. Our study shows that EGFR immunostaining in meningiomas directly correlates to the tumor's grade. The EGFR expression did not correlate with the overall survival and the recurrence-free survival of the patients affected by meningioma (de novo, recurrent and progressed). CONCLUSION: We submit that the EGFR expression is not a useful prognostic element to identify patients with a major risk of meningioma recurrence.

A rare location of benign osteoblastoma: case study and a review of the literature.

Osteoblastoma is a solitary, benign bone tumor that is rarely localized in the frontal sinus. It consists of hypocellular mineralized tissue that may form large masses or irregular trabeculae. A 31 year old man came to our attention with a 7 month history of diplopia, photophobia, frontal headhaches and progressive exophthalmos with proptosis of the left eye. The patient was submitted to computed tomography (CT) which allowed to appraise the extension of the lesion. The mass expanded inside the left frontal sinus and the upper ethmoidal cells invading the left orbital roof. Considering the extension of the tumor, the site and the connections with contiguous structures, a combination of endoscopic endonasal technique with intraorbital approach was performed. At histological examination typical features of benign osteoblastoma were observed. The sites of predilection for the tumor are the long bones, vertebral column, and small bones of hands and feet. Its occurrence in the skull and jaw bones is relatively rare and represents only 15% of all osteoblastomas. To our knowledge, only 5 cases of osteoblastoma of the frontal sinus have been previously reported in the English-language literature. This report describes a case of benign osteoblastoma in a rare site, namely, the frontal sinus with particular attention about the differential diagnosis and the treatment.    

Cervical spine chondroma arising from C5 right hemilamina: a rare cause of spinal cord compression. Case report and review of the literature.

Chondromas are rare primary spine tumors. Only 12 cases of chondromas located in the cervical spine have been reported in the literature up to now. The authors report a case of a cervical periosteal chondroma in a 38-year-old man presenting with neck pain and a syndrome of spinal cord compression. Magnetic resonance imaging revealed a lesion that was hypo- or isointense on T1-weighted images and iso-hyperintense on T2-weighted images. Administration of Gd-DPTA resulted in margin enhancement on T1-weighted images. CT scans revealed a hyperdense calcified lesion arising from the internal surface of C5 right hemi-lamina. The patient underwent a right C5 hemi-laminectomy, with complete en-bloc removal of the lesion. The authors emphasize that early identification of the initial lesion should be coupled with total surgical resection, as a definitive treatment, in order to prevent malignant transformation.

Earliest clinical manifestations and natural history of neurofibromatosis type 2 (NF2) in childhood: a study of 24 patients.

BACKGROUND: Neurofibromatosis type 2 (NF2) is an autosomal dominant disease characterised by the development of multiple nervous system tumours, ocular abnormalities, and skin tumours. Although classically considered a disease of adults, initial signs and/or symptoms may be evident in childhood and are often unrecognised. OBJECTIVES: The aim of this study was to identify the earliest clinical presentations of NF2 and to characterise the clinical course and outcome in children with NF2. METHODS: We have performed a retrospective (years 1990-1998) and prospective (years 1998-2004) study of 24 patients (10 males, 14 females; currently aged 4 to 22 years) fulfilling the revised (Manchester) NF2 criteria seen at the Universities of Catania and Rome, Italy. RESULTS: Causes of referral prior to a definitive diagnosis of NF2 were: 1) Ophthalmologic problems: early onset lens opacities (n = 3); strabismus (n = 3) and amblyopia (n = 3) (due to underlying cranial nerves and/or brain tumours); 2) Otolaryngology problems: hearing loss and tinnitus (n = 2) in early teens disregarded or treated as ear infections; hoarse (n = 1) or bitonal (n = 1) voice; 3) Neurological dysfunction: seizures secondary to intracranial meningioma (n = 1) or vestibular schwannomas (VS) (n = 1), neurological dysfunction related to brainstem and/or spinal cord tumours (n = 7), isolated and multiple cranial nerve deficits (n = 10), and peripheral neuropathy secondary to schwannomas (n = 4); 4) Skin manifestations: schwannomas misdiagnosed as neurofibromas because of associated café-au-lait spots (n = 2); café-au-lait spots (n = 8) and skin tumours (n = 3). A family history was relevant in 20 % of the patients. Molecular genetic analysis of the NF2 gene revealed typical truncating mutations in all the 5 familial cases and in 2/10 sporadic cases analysed. CONCLUSIONS: Children with NF2 often first come to medical attention because of ocular, subtle skin, or neurological problems the significance of which is realised when they later present with more classical symptoms due to bilateral VS or other intracranial tumours. The clinical course at this young age is highly variable, depending on tumour burden, early surgical intervention, surgical outcome after tumour resection, and complications.

Potential indications for somatostatin analogues: immune system and limphoproliferative disorders.

Among hormones and neuropeptides influencing the immune system, somatostatin seems to play a key role not only in inhibiting specific immune cell activities, but also in promoting selected functions of particular immune cell subsets. Indeed, controversial effects have been observed in experimental conditions where somatostatin seems to stimulate certain cell functions, such as secretion of specific products (immunoglobulin, cytokines), cell migration and adhesion to extracellular matrix components. However, interestingly, cortistatin (CST), a neuropeptide that strongly resembles somatostatin, from both the structural and functional points of view, seems to have potential roles in regulating immune responses, as well as other lymphoid cell functions. The unexpected wide distribution of CST in a number of human organs, but particularly in immune cells, points to a broader physiological role of CST than previously presumed. The actions of somatostatin and its synthetic analogs (SSA) are mediated by five membrane G protein-coupled receptors subtypes (SSTR1-5), displaying a tissue specific distribution. The majority of somatostatin-target tissues, including lymphoid tissues, may co-express multiple somatostatin receptor (SSTR). The number of SSTRs in lymphoid cells is significantly lower compared to neuroendocrine tissues. However, the presence of receptors allowed the localization by in vivo SSTR scintigraphy of lymphoproliferative disorders, as well as granulomatous and autoimmune diseases. In specific cases, this technique may contribute to establishing the diagnosis and staging the disease. Recent studies evaluating the specific and quantitative SSTR distribution in lymphoid organs and cells, in both normal conditions and immune disorders, have largely contributed to better understand the phenomenology of in vivo receptor imaging and also the involvement of the different SSTR in determining the uptake of radiolabeled SSAs. Moreover, since lymphomas are highly radiosensitive malignancies, a promising approach in refractory patients with malignant lymphomas may be represented by radionuclide-targeted therapy with radioactive-coupled SSAs combined with gene therapy. This latter technique seems effective in inducing the expression or increasing the number of given SSTR in order to ameliorate the impact of radionuclide-targeted therapy. Medical treatment of lymphoproliferative diseases with currently available synthetic analogs have produced unsatisfactory and conflicting results. This might be due to the affinity of the current available SSAs for specific SSTR. However, the synthesis of new compounds with distinct properties has reopened a challenge in this field. The application of receptor-based localization and anti-tumor strategies should also be taking into account the new knowledge recently emerged on the physiopathology of neuropeptide receptors: firstly, neuropeptide receptor homo- and heterodimerization, which may involve different subtypes of SSTRs, as well as other neuropetide receptors, and secondly, the role of endogenous SSTR ligands, such as CST.

Neurological deterioration after head trauma in patients with colloid cysts of the 3rd ventricle. Two case histories.

The authors report 2 cases of colloid cyst of the 3(rd) ventricle (both patients previously asymptomatic) diagnosed after head trauma. Neurological deterioration due to biventricular hydrocephalus, rendering necessary an urgent bilateral CSF shunting, was observed. The possible relationship between clinical deterioration and head injury in these cases is discussed.

Cryopreservation of autogeneous bone flap in cranial surgical practice: what is the future? A grade B and evidence level 4 meta-analytic study.

AIM: Autogenous bone flap adequately preserved can be used successfully for repair of iatrogenic cranial bone defects because of its biological and economic advantages. The commonly used techniques of bone preservation are freezing at temperature ranging from -16 to -40 inverted exclamation mark C or subcutaneous pocketing. METHODS: We searched on NLM database querying as subject headings "cranioplasty and autologous bone" and found 7 papers meeting our criteria. For every series we considered the frequency of bone resorption and infection and performed statistical analysis by means of contingency tables carrying out for the chi2 test according to Pearson and considering to be statistically significant p(chi2) <0.05 values. RESULTS: Compared series presented no statistically significant difference with regard to frequency of the complications (chi2=6.43 for critical value of 11.07 (k-1=5) (P>0.25) and chi2=5.48 for critical value of 7.81 (k-1=3) (P>0.10) respectively for infection and resorption. CONCLUSION: Although we did not find any significative difference among the frequencies of the complications of the series, it is our opinion that freezing the bone flap is preferable to abdominal pocketing because it needs no double surgical abdominal time and the bone flap frozen is stronger to mechanical loading than fresh flap. Cryopreservation of autologous bone flap should be the standard technique of preservation for delayed cranioplasty but a multicentric prospective trial is advisable to obtain in short time statistically significant results focusing better on the rate of marginal resorption of the bone flap using new cryoprotectant solutions.

Nuchal fibrocartilaginous pseudomotor. Case report and review of the literature.

The case of a 30-year-old woman who, many years after a trauma to the neck, developed a hard tissue mass in the posterior midline at C5-C6 level is reported. The patient underwent surgical excision of the mass. Nucal fibrocartilaginous pseudotumor (NFP) was diagnosed. This is a very rare non-neoplastic lesion, probably arising throug a post-traumatic metaplasia of the nuchal ligament, and only 14 cases have been reported to date in the literature. The clinicopathologic and neuroradiologic features of NFP are described.

Spinal meningeal mesenchymal chondrosarcoma. Report of a new case and review of the literature.

A case of T12-L2 intraspinal extradural meningeal mesenchymal chondrosarcoma is described in a patient presenting with a clinical history of low-back pain and weakness of the lower limbs. Magnetic resonance T1-weighted Gadolinium enhanced imaging, showed an intraspinal extradural mass, extending from T12 to L2, located anterolaterally into the spinal canal and pushing posteriorly the conus medullaris. The mass was completely removed and postoperative histological diagnosis was of mesenchymal chondrosarcoma. The patient was completely symptoms-free after surgery. Intraspinal meningeal mesenchymal chondrosarcoma and treatment options are reviewed.

Transverse carpal ligament reconstruction with polyesterurethane patch for prevention of recurrence in therapy of failed primary carpal tunnel surgery.

A small group of patients with carpal tunnel syndrome (CTS) can present recurrences and persistent symptoms of CTS after its surgical release. If recurrence is due to scarring between median nerve and surrounding tissue (true recurrence of CTS, transverse carpal ligament [TCL] reconstruction with transposition flap technique is available but it presents poor results: This 48-year-old woman presented a recurrence of CTS 3 months after open standard incision. At operation, a scar was detected that did not permit normal median nerve gliding during wrist movements. External neurolysis restored normal gliding and non-absorbable polyesterurethane patch was used to reconstruct TCL. At 2-year follow-up the patient was in good health and returned to her manual usual job without pain and sensitive and motor deficits. Implantation of unresorbable poliesterurethane patch for TCL reconstruction after external neurolysis appears to be more advantageous than TCL reconstruction with transposition flap technique because it takes little time, causes minimal adhesion formation, does not need of wide incision and provides the same favourable conditions of the transposition flap as mechanical stabilization of the tranverse carpal arch, prevention of bowstringing of the flexor tendons, increase of postoperative grip strength and good protection of the median nerve. However, more cases should be studied before considering TCL reconstruction with poliesterurethane patch as a useful option in secondary surgery of true recurrence of CTS.

Spinal intradural extramedullary tumors. Personal experience.

BACKGROUND: Spinal intradural extramedullary tumors account for 2/3 of all intraspinal neoplasms and are mainly represented by meningiomas and schwannomas, with the former accounting for the 25-46% of all primary intraspinal tumors. Technical advances in imaging technique, magnetic resonance imaging (MRI) and surgical procedures have brought about significant better clinical results in the last 2 decades. Neverthless a small percentage of patients still present poor postoperative outcome mainly related to the duration of clinical history, the severity of preoperative neurological deficits and to some specific anatomo-surgical aspects. METHODS: In an effort to clarify the influence of these factors on patient's outcome, the authors analyze the clinical, surgical and prognostic data of 41 patients with intradural extramedullary spinal tumor surgically treated between January 1990 and December 1999. The follow-up period ranged from 1 to 9 years. The clinical history until admission and treatment was 3-48 months for meningiomas and 1-72 months for schwannomas. RESULTS: Morbidity and mortality rate was 5 and 0% for meningiomas and 0 and 6% for schwannomas. Almost all the patients experienced a significant neurological improvement after surgery, with a percentage of Nurick's grade 1 and 2 of 68% among patients with meningiomas and 66% among patients with schwannomas. Removal of the tumor was complete in 90% of meningiomas and 94% of schwannomas. CONCLUSIONS: The authors address radical surgery as the ideal goal in these neoplasms, as it gives the patients the best long-terms results, pointing out the importance of systematic early identification of any main radiculomedullary artery during debulking and dissection of the tumor to avoid any risk of severe postoperative neurological worsening of the patients.

Trigeminal palsy caused by triventricular hydrocephalus. A case report.

A case of left trigeminal palsy caused by triventricular hydrocephalus and completely recovered after ventriculo peritoneal shunt is reported. The case was studied by MR before and after the operation. Preoperative MR showed triventricular hydrocephalus with marked reduction of the left Meckel's cave. Following ventriculo peritoneal shunt the patient showed a complete recovery of the left 5th nerve palsy and NR showed the expansion of the left Meckel's cave. The authors suggest that the trigeminal palsy was due to intracranial hypertension probably associated to a weakness of the lateral wall of the left Meckel's cave. No similar case have been previously reported in the literature.

[Telangiectasias associated with reticular veins. Sclerotherapy or combined sclerotherapeutic and surgical approach?]. / Teleangectasie associate a varici reticolari: scleroterapia o approccio combinato chirurgico e scleroterapico?

BACKGROUND: We compare the results obtained with sclerotherapy or combined surgical and sclerotherapic approach in two groups of patients, affected by reticular veins-related telangiectasias without incompetence of the saphenous systems or perforating veins. METHODS: 44 female patients were evaluated at our Institution. The first group (21 patients) was treated with sclerotherapy (polidocanol at a concentration of 1% for the reticular veins and 0.25% for the telangiectasias); the second group (23 patients) was treated with a combined approach (ambulatory phlebectomy according to Müller and sclerosis using polidocanol at a concentration of 0.25%). Six and twelve months after surgery or sclerotherapy, we evaluated the appearance of hyperpigmentation or telangiectatic matting, and the recurrence of the treated vessels. RESULTS: In the first group, the hyperpigmentations and the appearance of telangiectatic matting showed, after 6 months, an incidence rate of 14.3% after 12 months, a recurrence of the treated vessels was observed in 4 patients (19%). In the second group, the hyperpigmentations and the appearance of telangiectatic matting showed, after 6 months, an incidence rate of 4.3% at 12 month follow-up no recurrence of treated vessels was observed. CONCLUSIONS: The combined approach might be considered, in selected patients, a valid alternative to sclerotherapy in the treatment of reticular veins-related telangiectasias, particularly in preventing side effects such as hyperpigmentations and telangiectatic matting.

Oculomotor palsy and supraclinoid internal carotid artery aneurysms. Personal experience and review of the literature.

Oculomotor palsy related to the presence of an intracranial aneurysm arising from the supraclinoid internal carotid artery (ICA) is a well known and described clinical condition. Recent microanatomical and clinical evidences seem to demonstrate that the pathophysiology of the aneurysm-related III nerve palsy could be interpreted as that of any other cranial nerve's neurovascular compression syndrome. The authors review their personal experience with supraclinoid ICA aneurysms related to oculomotor palsy and the data of the literature, aiming to elucidate a better clinical, therapeutic and prognostic correlation to these factors.

Symptomatic subdural hygroma after petroclival meningioma surgery. Case report of a intractable complication.

Although subdural hygroma development after craniotomy or aneurysm surgery is a well-known complication and subdural peritoneal or V-P shunt are commonly successful procedures, there are situations that cannot be treated by available surgical options. We reported a case of a 28-year-old young man who developed a symptomatic subdural hygroma after removal of petroclival meningioma. This hygroma increased in size and became bilateral in spite of implantation of V-P with programmable valve at open pressure variable from 30 to 180 mmH2O, placement of subdural-peritoneal shunt with low-low valve or without valve and external diversion. The occurrence of this case showed that there other factors can play role in hygroma development as disturbance of normal CSF dynamic with shunt of CSF from basal arachnoidal to subdural spaces secondary to surgery and/or slow growth of petroclival meningioma as well as postoperative progressive cerebral atrophy. Actually there are not effective diagnostic tools to detect causes and therefore, there are restricted therapeutic possibilities. This potential and serious complication should be always considered when planning petroclival meningioma surgery because of the severe consequences on functional outcome.