"Klingon headache" - a case report of mimic new daily persistent headache associated to primary essential cutis verticis gyrata.

BACKGROUND: Primary essential cutis verticis gyrata is a condition that usually affects healthy subjects associated to convoluted folds and furrows formed from thickened skin of the scalp resembling cerebriform pattern. CASE: we describe a case of association between primary essential cutis verticis gyrata and new daily persistent headache. DISCUSSION/CONCLUSIONS: In our knowledge this is the first description of new daily persistent headache associated with primary essential cutis verticis gyrata. We think that primary essential cutis verticis gyrata could be considered as a possible trigger factor, never described before, for the development of new daily persistent headache.

Post laminoplasty cervical kyphosis-Case report.

INTRODUCTION: Cervical kyphosis is a progressive cervical sagittal plane deformity that may cause a reduction in the ability to look horizontally, breathing and swallowing difficulties, sense of thoracic oppression and social isolation. Moreover, cervical kyphosis can cause myelopathy due to a direct compression by osteo-articular structures on the spinal cord or to a transitory ischaemic injury. The treatment of choice is surgery. The goals of surgery are: nervous structures decompression, cervical and global sagittal balance correction and vertebral stabilization and fusion. PRESENTATION OF CASE: In October 2008 a 35 years old woman underwent surgical removal of a cervical-bulbar ependymoma with C1-C5 laminectomy and a C2-C5 laminoplasty. Five months after surgery, the patient developed a kyphotic posture, with intense neck and scapular girdle pain. The patients had a flexible cervical kyphosis. Therefore, we decided to perform an anterior surgical approach. We performed a corpectomy C4-C5 in order to achieve the anterior decompression; we placed a titanium expansion mesh. DISCUSSION: Cervical kyphosis can be flexible or fixed. Some authors have reported the use of anterior surgery only for flexible cervical kyphosis as discectomy and corpectomy. This approach is useful for anterior column load sharing however it is not required for deformity correction. CONCLUSION: The anterior approach is a good surgical option in flexible cervical kyphosis. It is of primary importance the sagittal alignment of the cervical spine in order to decompress the nervous structures and to guarantee a long-term stability.

Microsurgical excision without fusion as a safe option for resection of synovial cyst of the lumbar spine: long-term follow-up in mono-institutional experience.

Spinal synovial cysts are cystic dilatations of the synovial membrane that may arise at all levels of the spine. We describe our experience, paying attention to diagnosis, surgical treatment, and long-term follow-up. Between 1995 and 2007, 18 patients were surgically treated. Of these, three patients were excluded from the study because they presented spinal instability at pre-operative assessment. All patients were evaluated pre-operatively with CT, MRI, and dynamic X-rays, and underwent surgery for removal of the cyst by hemilaminectomy and partial arthrectomy. All patients were evaluated with early MRI and had a minimum 2-year follow-up by dynamic X-rays. None of the patients required instrumented fusion due to the absence of radiological signs of instability on the pre-operative dynamic tests. In all patients, there was an immediate resolution of the symptoms, with evidence of complete removal of the cysts on post-operative MRI. At 2-year follow-up, all patients underwent dynamic X-rays and responded to a questionnaire for evaluation of outcome. None of them showed signs of relapse. The gold standard for treatment is surgery, even though other conservative treatment regimens have been proposed. Correct surgical strategy relies on pre-operative assessment of biomechanical stability for deciding whether patients need instrumented fusion during cyst removal. Patients with no instability signs are suitable for hemilaminectomy with partial arthrectomy, preserving 2/3 of the medial portion of the articular facet, because this represents a valid option of treatment with a low risk of complications and a low rate of relapse.

The use of platelet gel in postero-lateral fusion: preliminary results in a series of 14 cases.

Over the last few years, some hemocomponents have been used advantageously in clinical neurosurgical practice, not systemically via transfusion but topically as a sealant (fibrin glue). This has diverted the attention of many authors to the role of platelets in the healing process. The combination of hyper-concentrated platelets and fibrin glue (fibrinogen, XIII factor, fibronectin) with activated thrombin produces a platelet gel that can be easily applied to "difficult" wounds. This topical use of hemocomponents has gained an important role in regenerative medicine. The authors have considered the possibility of using a preparation with a high autologous platelet concentration applied in addition to autologous bone during vertebral postero-lateral fusion. The aim of the procedure is to induce a higher rate of vertebral fusion. Between November 2007 and November 2008, 14 patients (9 men and 5 women, mean age 58.9) underwent laminectomy, vertebral stabilization and postero-lateral fusion. The number of vertebral levels involved in stabilization was: 1 in 2 patients, 2 in 5 patients, 3 in 5 patients, 4 in 1 patient and 5 in 1 patient. Platelet gel was obtained by taking 16 ml of peripheral venous blood from the patient. For this procedure two patented test tubes were used for each patient, with a capacity of 8 m each. These make up the REGEN-THT(®) (Thrombocyte Harvesting Tube) system that makes it possible to obtain 8 ml of autologous platelet gel in 40-45 min. The addition of Ca gluconate and ethanol at 95% makes it possible to obtain a preparation of plasma rich in platelets and activated thrombin with a platelet concentration five times superior to the haematic one. The platelet gel is combined with fragments of autologous bone and synthetic bone during surgical operation. To allow a comparative assessment of the degree of fusion achieved with and without application of the platelet preparation in each patient, it was arbitrarily decided to use it in only one half of the operative field. All patients underwent serial CT scans 3 and 6 months after surgery as well as plain X-rays to evaluate bone fusion. The reconstructed CT images, especially in sagittal and axial planes, permitted an evaluation of the degree of vertebral fusion and "bone growth". The fusion rate was calculated measuring the increment of bone density on CT images, by means of an evaluation of the ROI (HU) in the newly formed bone, and comparing bone density within the bone callus formed by autologous and synthetic bone alone in the one to which the platelet preparation had been added. A good rate of fusion was observed in all patients. Furthermore, a comparative analysis of ROI at 3 and 6 months after surgery demonstrated a high increase in the fusion rate during the first 3 months after surgery. After 6 months the differences in ROI between the two sides had balanced out. However, at 6-month follow-up examination, bone density in the half of the surgical field in which platelet gel had been added to autologous-heterologous bone was higher in comparison to the contralateral one. Bony neoformation after posterior-lateral arthrodesis is well-evident 3 months after surgery and usually continues gradually for the following 18-24 months. The autologous platelet preparation used seems to accelerate bony deposition and to promote tissue healing, increasing bone density at the level of posterior-lateral arthrodesis. Moreover, this preparation has low production costs and is easy to apply.

[Registry of genetic and malformative diseases in the province of Macerata]. / Registro delle malattie genetiche e malformative della provincia di Macerata.

OBJECTIVES: The aim of this epidemiological research is to evaluate the prevalence of genetic diseases and malformative syndromes in paediatric population living in the Macerata county. MATERIAL AND METHODS: All the data were collected through a careful analysis of a specific questionnaire sent to all the family paediatricians. RESULTS: 23,379 children living in Macerata county, aged 0 to 9 years, were evaluated (93.8% of all this paediatric population). Among those were found N 400 cases of genetic diseases and malformative syndromes: Malformations Tot.N. 255 cases (63.3% of the reported cases); Malformative Syndromes Tot. N. 55 cases (27.8% of the reported cases); Endocrinology and Metabolic Diseases Tot. N. 41 cases (10.3% of the reported cases); Osteochondrodysplasia Tot. N. 22 cases (5.7% of the reported cases); Other Tot. N. 28 cases (7.0% of the reported cases); Male population was found more affected than female: M/F ratio = 1.4. The analysis of the data showed an increasing trend in detecting these pathological conditions, consistent with the increase in geographic altitude (3 areas considered): 0-100 meter = 0.88%; 100-600 m.a.s. = 1.34%; over 600 m.a.s. = 1.88%. CONCLUSION: The knowledge of the number of children affected by genetic and malformative diseases in the Macerata county is relevant in order to establish a Genetic Service with the aim to better support the medical assistance of these patients and counselling service for the families.