Cauda equina syndrome from intradural metastasis of a non-neural tumor: case report and review of literature.

BACKGROUND: Cauda equina syndrome (CES) is a challenging condition and it can be caused by variable entities. Leptomeningeal carcinomatosis (LC) is a multifocal seeding of the leptomeninges by malignant cells and it is observed in 1-8% of patients with solid tumors. Diagnosis of intradural metastases of the cauda equina is often delayed due to the non-specific characteristics of this condition but also to the delay of presentation of many patients. Cauda equina metastases usually occur in advanced cancers, but rarely can be the first presentation of disease. CASE DESCRIPTION: A 63-year-old man presented with 6 months history of low back pain and 20 d history of bilateral sciatica, hypoesthesia of the legs and the saddle, flaccid paraparesis and bowel incontinence determine by multiple nodular small lesions on the entire cauda equina with contrast-enhancement. Total-body CT showed a millimetric lesion at the lung. The patient underwent L2-L5 laminectomy and subtotal removal and histological examination showed a small cell lung carcinoma metastasis. CONCLUSIONS: In the literature, 54 cases of CES from non-CNS tumor metastasis are described. The diagnosis is challenging, back pain, with or without irradiation to the lower limbs, is the most frequently reported disturbance. In about 30% of patients there is no known malignancy and CES is the first clinical presentation. Treatment of choice is surgery, followed by radiotherapy and less frequently adjuvant chemotherapy. The surgical removal is almost always incomplete and functional outcome is often not satisfactory. Prognosis is poor.

Abrupt foramen magnum syndrome due to shunt malfunction in a previously asymptomatic chiari I malformation: the hidden predictable risk in long-lasting shunted patients.

Cerebrospinal fluid overdrainage in long-lasting shunted hydrocephalus is a well recognized complication. It may lead to different clinical syndromes which may occur over time, namely cranioencephalic disproportion and hindbrain herniation. Among the latter, Chiari malformation type 1 classically refers to one of the mildest form. When symptomatic, the patients generally exhibit a chronic, slowly progressive disease course. Although well documented in Chiari type II malformation and hydrocephalus, ventriculo-peritoneal shunt malfunction causing acute neurological deterioration in acquired Chiari malformation type 1 has been described only in three cases. All these patients underwent urgent shunt revision and suboccipital decompression in two of them, achieving good clinical improvement. We report on a 20-year-old woman harboring a previously asymptomatic Chiari malformation type 1, who experienced an acute foramen magnum syndrome with ventriculo-peritoneal shunt malfunction. We performed an endoscopic third-ventriculo-cisternostomy and definite removal of the shunt, obtaining the complete resolution of symptoms and shunt independence at 2 years follow-up. The phisiopathogenetic mechanisms and surgical management are discussed.

Chylous fistula: management of a rare complication following right anterior cervical spine approach.

PURPOSE: Chylorrhea resulting from injury of the lymphatic system during neck dissection is a well-known complication. It is an uncommon occurrence in spinal surgery, and only one case after right anterior cervical spine surgery has been described so far. Despite its rarity, chylous leakage deserves a particular attention since it may become a serious and occasionally fatal complication if not detected early and managed appropriately. METHODS: We report the case of a 42-year-old man who underwent a standard anterior cervical discectomy and fusion according to Cloward approach for a C6-C7 disk herniation. The patient developed a delayed prevertebral chyle collection on postoperative day 5, presenting with mild breathing and swallowing difficulties. RESULTS: He was managed with conservative care, including bed rest, low-fat diet and drainage pouch positioning, which led to the complete resolution of the fluid collection. CONCLUSIONS: Knowledge of the normal anatomy of the lymphatic system and of its variations is essential when planning an anterior spinal procedure, and represents the first measure to be adopted in order to avoid such complication. The prompt identification of a postoperative chylous fistula and the applicability of an individually based management's protocol may help in the majority of the cases to reduce the potential morbidity, without significant long-term effects.

Prolactin Secreting Pituitary Microadenoma: Results of Transsphenoidal Surgery After Medical Therapy With Dopamine Agonist.

Medical therapy with dopamine agonist is very effective in controlling prolactin serum levels and it usually represents the first therapeutic choice for prolactin secreting pituitary adenomas. However, many patients present increase of prolactinemia after withdrawal of medical therapy which consequently should be taken for long time; other present intolerance to medical therapy; women with pregnancy need to withdraw dopamine agonists with consequent potential related problems: in these patients transsphenoidal surgery can be requested. The presented study concerns the efficacy of transsphenoidal surgery in patients affected by microprolactinoma after treatment with medical therapy for different periods of time. Different postsurgical results were achieved in 2 groups of 24 (group 1) and 25 (group 2) patients affected by microprolactinoma who had taken medical therapy for a period of time respectively longer or shorter than 1 year. In summary, the authors observed in group 1 a rate of hormonal remission of 33.3% and an overall prolactinemia improvement with possibility of medical therapy withdrawal in 49.9% of patients. In group 2, the authors observed a rate of hormonal remission of 84% and an overall rate of improvement with no necessity of medical therapy of 92%. Therefore, the authors' experience showed that the surgical option for the therapy of microprolactinomas should be indicated within 1 year from the beginning of medical therapy with dopamine agonist, to achieve a high rate of hormonal remission and possibility to withdraw medical therapy. The authors' protocol for microprolactinoma treatment is presented.

Ethmoidal encephalocele associated with cerebrospinal fluid fistula: indications and results of mini-invasive transnasal approach.

Anterior skull base defects with encephalocele in adults are quite rare and can be a cause of spontaneous rhinoliquorrhea; however, cerebrospinal fluid (CSF) fistula can be not rarely misdiagnosed for several months or years. Five adult patients affected by ethmoidal encephalocele with CSF fistula were treated in our institute from 2006 through to 2011. Onset of clinical history was represented by rhinoliquorrhea, which was precociously recognized in only 1 patient; in the other 4, it was misdiagnosed for a period ranging from 11 months to 5 years. After clinical diagnosis of CSF fistula and after brain magnetic resonance imaging, ethmoidal encephalocele was evident in all patients; preoperative study was completed by spiral computed tomography scan, to clearly identify the skull base bone defect. All patients were operated on by transsphenoidal endonasal endoscope-assisted microsurgical approach through 1 nostril. The herniated brain was coagulated and removed, and reconstruction of cranial base was performed. Postoperative rhinoliquorrhea or other complications did not occur in any patient at short and late follow-up. All patients were discharged after a few days. Endonasal endoscope-assisted microsurgical approach was effective in exposing and repairing the ethmoidal bone defect; tridimensional vision and wide lateral and superior exposition of the operative field were possible in each patient, thanks to the use of microscope and angulated endoscope.

Spinal giant cell tumor in tuberous sclerosis: case report and review of the literature.

BACKGROUND: Patients affected by tuberous sclerosis (TS) have a greater incidence of tumors than the healthy population. Spinal tumours in TS are reported very rarely and consist mainly of sacrococcygeal and cervical chordomas. METHOD: Case report. FINDINGS: A 21-year-old man, affected by TS, presented a spinal dorsal T2 tumor that caused medullary compression. He underwent decompressive laminectomy and microsurgical excision of a giant cell tumor and an associated aneurysmal bone cyst. Postoperative hypofractionated radiotherapy was performed on the surgical field. At 2.4 years of follow-up the patient reported total recovery of neurological deficits and was free from tumor recurrence. CONCLUSION: Considering this association, which is the first reported in the literature, spinal magnetic resonance imaging with gadolinium should be performed at the onset of spinal pain in patients affected by TS.

Multidisciplinary treatment of giant invasive prolactinomas in paediatric age: long-term follow-up in two children.

BACKGROUND: Invasive giant prolactinomas are rare tumours, still representing a therapeutic challenge due to their characteristics of invasiveness and variable clinical course. Giant prolactinomas in childhood are extremely rare. Only single case reports have been described in the literature. CASE PRESENTATION: We report on two children who presented a progressive visual deterioration. Magnetic resonance imaging showed extensive intra-suprasellar tumour invading the anterior skull base. The laboratory investigations detected markedly elevated prolactin levels. The patients were firstly started on dopamine agonist therapy with partial reduction in size of the tumours. The debulking of the residual lesions through a transsphenoidal approach was then performed in both patients, one of whom requiring a second surgical procedure for tumour regrowth. The complete shrinkage of the residual adenomas was achieved after the treatment with conventional radiotherapy, with a follow-up of 13 and 14 years, respectively. DISCUSSION: Multidisciplinary therapeutic approach for giant prolactinomas in paediatric patients can be an effective treatment; despite the invasiveness of these tumours, the efficacy of this combined treatment can reach a satisfactory control of the disease at long term, assuring a good quality of life as well.

Cystic craniopharyngioma: trans-sphenoidal surgery and intra-cystic apposition of "bleomycin wax".

BACKGROUND: The current therapeutic approach to craniopharyngioma is multidisciplinary. Sub-total removal, followed by adjuvant treatments, especially in large cystic tumours, is an accepted regime reported by many authors. CASE REPORT: A young patient with an intra- and suprasellar cystic craniopharyngioma was operated on via a microsurgical trans-sphenoidal approach, achieving sub-total removal and bleomycin mixed with bone wax ("bleomycin wax") applied to the capsular remnant. RESULTS: Pre-operative neurological and endocrinological deficit improved after surgery. There was no evidence of tumour recurrence after a follow-up period of 5.4 years. CONCLUSIONS: The intra-operative use of "bleomycin-wax" should be limited to those patients in whom intra-operative CSF fistula does not occur.

Validity of percutaneous controlled radiofrequency thermocoagulation in the treatment of isolated third division trigeminal neuralgia.

BACKGROUND: Of 756 percutaneous controlled radiofrequency thermocoagulations of gasserian ganglion and/or retrogasserian rootlets for idiopathic trigeminal neuralgia or for trigeminal neuralgia in multiple sclerosis, the results in 158 patients who had isolated third division trigeminal neuralgia were reviewed. METHODS: In 129 patients, percutaneous thermocoagulation was fluoroscopic guided, whereas in the last 29 the procedure was performed under CT control. In all cases, the goal was to achieve selective anesthesia limited to the third trigeminal division. RESULTS: Complete pain relief was obtained immediately after the procedure in all patients and selective anesthesia in the third division was achieved in all of them, except for 2 patients in whom unwanted first and second division anesthesia/hypoesthesia also occurred. Other significant complications were transient sixth cranial nerve palsy in 1 patient and masseter muscle dysfunction, which improved during follow-up, in another one. The selected sensory impairment was well tolerated in all patients. During an average follow-up period of 8.8 years, recurrences occurred in 12 patients, together with a partial regression of the anesthesia: in 10 patients the procedure was repeated successfully. In 2 patients with multiple sclerosis, the procedure was repeated twice and 3 times, respectively. CONCLUSIONS: Percutaneous controlled radiofrequency thermocoagulation is quite easy to perform under fluoroscopic or CT control, and it involves no mortality and very low morbidity; it is an immediately effective procedure and the rate of recurrence is low. Last, but not least, if the anesthesia is limited to the third division, no anesthesia dolorosa occurs and patients are very grateful.

Lateral transmaxillosphenoidal approach to the lateral compartment of the cavernous sinus: technical case report.

This article outlines the surgical technique and the indications for the lateral transmaxillosphenoidal approach, which is illustrated by an index case. A 27-year-old woman presented with a trigeminal sensory deficit caused by a dermoid tumor occupying the lateral compartment of her right cavernous sinus. A lateral transmaxillosphenoidal approach was performed, and the tumor was removed lateral to the intracavernous carotid artery (ICA) and medial to intracavernous cranial nerves. The lateral transmaxillosphenoidal approach is similar to the transmaxillosphenoidal approach used for the removal of pituitary adenomas invading the medial compartment of the cavernous sinus. By opening the lateral wall of the sphenoid sinus just above and laterally to the carotid artery, tumor can be removed medial to the intracavernous cranial nerves and lateral to the ICA.

Fibrous dysplasia of the clivus with a second T8 bone lesion: case report.

BACKGROUND: We present a rare case of a probably poliostotic fibrous dysplasia of the clivus in a young woman with a clival and vertebral involvement. We also compare our case with data reported in tables that include all cases of clival fibrous dysplasia present in literature. CASE DESCRIPTION: Thirty-year-old women presented with headache. We reviewed radiological and clinical literature before surgery. It was possible to perform a preoperative diagnosis of fibrous dysplasia of the clivus so it was possible to adopt a minimally invasive surgical approach on the clivus. The diagnosis of fibrous dysplasia of the clivus was confirmed after the operation. As the patient was asymptomatic, we did not operate on the vertebral lesion which was stable after 1 year. CONCLUSION: We draw the conclusion that it is possible to obtain a correct preoperative diagnosis of fibrous dysplasia based on magnetic resonance imaging and computed tomographic scans; this makes it possible to avoid major surgical operations. The reported case is particularly interesting because apart from the clival fibrous dysplasia, a vertebral involvement of the same type can be assumed; this association has never been reported in literature.

Percutaneous computer tomography-guided technique to close postsurgical cerebrospinal fluid fistulas of the frontal sinus.

BACKGROUND: The occurrence of cerebrospinal fluid fistulas of the frontal sinus after anterior skull base surgery is not rare. The extracerebral techniques to repair cerebrospinal fluid fistulas are often used, especially because they avoid open-air surgical operations. METHODS: A percutaneous CT-guided technique to close postsurgical cerebrospinal fluid fistulas of the frontal sinus in three patients after anterior skull base surgery is presented in this report. Ten millimeters of human fibrin glue was injected into the frontal sinus through one of the burr holes of the bone flap by an 18-gauge spinal needle. RESULTS: After an average follow-up period of 2.8 years, all three patients are in excellent general and neurological conditions and have not shown any further signs of rhinoliquorrhea. CONCLUSIONS: The presented percutaneous CT-guided technique can be considered a valid and harmless solution to closer small or moderate cerebrospinal fluid fistula that occurred after anterior skull base surgery.

Intrasphenoidal encephalocele associated with cerebrospinal fluid fistula and subdural hematomas: technical case report.

OBJECTIVE AND IMPORTANCE: Intrasphenoidal encephalocele is a rare clinical entity that is often complicated by rhinorrhea, recurrent meningitis, and headache, but in no case has the association of rhinorrhea with subdural hematomas been described. A surgical procedure to stop persistent cerebrospinal fluid leakage is reported. CLINICAL PRESENTATION: A 59-year-old man sought care for intractable rhinoliquorrhea of 6 months' duration. Cranial computed tomographic and magnetic resonance imaging scans revealed a basal posterior frontal bony defect and an evocative image suggesting intrasphenoidal encephalocele. INTERVENTION: A transnasal transsphenoidal surgical procedure was performed; the encephalocele was removed, and the sphenoid sinus was filled with an inflatable pouch made of synthetic dura mater containing abdominal fat. Postoperative reduction of the rhinoliquorrhea, but not its total disappearance, was observed. Total disappearance was achieved only after endonasal, transmucosal inflation of the pouch with human fibrin glue. One of the subdural hematomas disappeared spontaneously, and the other was treated by a surgical procedure. CONCLUSION: The possible role of the presented technique in the treatment of cerebrospinal fluid leakage is discussed.

Spinal intramedullary arachnoid cyst: case report and literature review.

BACKGROUND CONTEXT: Intramedullary arachnoid cysts are extremely rare; only 14 cases have been reported in the literature so far. PURPOSE: We report on the case of a 31-year-old woman who presented with back pain and progressive paraparesis secondary to a dorsal intramedullary arachnoid cyst detected on magnetic resonance imaging (MRI): the surgical planning and clinico-radiological outcome are discussed along with a review of the relevant literature. STUDY DESIGN: Case report and literature review. PATIENT SAMPLE: One patient affected by intramedullary arachnoid cyst. OUTCOME MEASURES: Magnetic resonance imaging and pathological findings from operative specimens were used to confirm the diagnosis. METHODS: A 31-year-old woman presented with a 7-year history of back pain that had worsened 3 months before admission to our department; for this reason, the patient had undergone a spinal MRI revealing the presence of a 1-cm cystic intramedullary lesion at the level T11-T12, with no contrast enhancement. After 2 months, the patient presented with a worsening of clinical symptoms complaining of severe back pain radiating to the lower extremities associated with a progressive paraparesis, urinary incontinence, and abdominal pain. Referred to our department, at the time of admission the patient was bedridden because of the impossibility of maintaining a standing position. The patient underwent a T11-T12 laminectomy with fenestration of the cyst. RESULTS: She experienced an immediate relief of pain symptoms, and by the seventh postoperative day she was able to stand without help and walk a few meters with assistance. By the sixth postoperative month, the patient had significantly improved, having gained the ability to walk alone without assistance with complete resolution of the bladder dysfunctions, with no cyst recurrence after approximately 2 years of follow-up. CONCLUSIONS: Intramedullary arachnoid cysts should be considered in the differential diagnosis for intramedullary cystic lesions. A particular consideration deserves their occurrence in asymptomatic patients, who should be adequately informed on the possible natural evolution: when symptomatic, surgical therapy should be promptly offered, considering that a postoperative complete recovery is usually observed, regardless of the surgical technique.

Spinal hydatidosis relapse: a case report.

Human cystic echinococcosis (CE) is a zoonosis caused by the larval stage of the Echinococcus granulosus and the most common sites affected are the liver and lung in approximately 80-90% of cases. The hydatid bone represents the 0.5-2.5% of all cases and localization cord is present about 50% of the time. This benign and commonly asymptomatic disease may simulate an aggressive malignancy because of osseous destruction and aggressive extension. We report a case of a 42-year-old male patient, presented with an unusual spinal hydatidosis relapse, related to anthelmintic drug therapy withdrawal after 10-year treatment. The man had previous excision of chest and hepatic hydatid cysts (resp., 10 and 3 years ago) and after primary mediastinal and spinal involvement (3 years ago) he was lost to follow-up and discontinued drug therapy. The patient underwent surgery and the postoperative histopathology confirmed the diagnosis. The patient recovered with no complications. Despite significant progress in diagnostic imaging, pharmacological and surgical therapy, spinal CE remains associated with high morbidity.

Transsphenoidal removal of huge pituitary adenomas with marked suprasellar extension: results with modified surgical technique.

OBJECTIVE: A modified transsphenoidal technique to remove huge pituitary adenomas with marked suprasellar extension (4.5-8 cm of maximum diameter) is presented. METHODS: The technique allowed to avoid the occurrence of a precocious descent of the suprasellar cisternal plane into the sellar plane during tumour removal and its related consequences (incomplete tumour removal, occurrence of cerebrospinal fluid leak, prolonged time of postoperative stay in hospital). Technique is performed opening at the beginning only the lateral parts of peritumoral dura mater, leaving the central part of the dura mater in support of the central part of tumour and suprasellar cisternal plane. After removal of lateral parts of the tumour, the central part of peritumoral dura mater is opened and the central intra- and supra-sellar parts of the tumour are removed. RESULTS: Total removal was accomplished in 64% and 45% in groups two and one respectively. Intraoperative CSF leak occurred in 2.4% and 22.5% respectively in groups two and one. Postoperative CSF fistula did not occur in group two, while it occurred in 7.4% of patients of group one. Average time of postoperative stay in hospital was 4.3 and 8.2 days in groups two and one respectively. CONCLUSION: The presented modified transsphenoidal microsurgical technique for removal of huge pituitary adenomas allowed to achieve better results than patients operated by standard transsphenoidal surgery.

Preliminary results of 45 patients with trigeminal neuralgia treated with radiosurgery compared to hypofractionated stereotactic radiotherapy, using a dedicated linear accelerator.

Radiosurgery (RS) and hypofractionated stereotactic radiotherapy (HSRT) were performed in 23 and 22 patients respectively for the treatment of trigeminal neuralgia. RS and HSRT were performed with a dedicated linear accelerator (LINAC): an invasive frame (for RS) or a relocatable stereotactic frame fitted with a thermoplastic mask and bite blocks (HSRT) were used for positioning patients. The RS treatment delivered 40 Gy in a single fraction, or for HSRT, the equivalent radiobiological fractionated dose - a total of 72 Gy in six fractions. The target (the retrogasserian cisternal portion of the trigeminal nerve) was identified by fusion of CT scans with 1-mm-thick T2-weighted MRI, and the radiant dose was delivered by a 10-mm-diameter cylindrical collimator. The results were evaluated using the Barrow Neurological Institute pain scale during follow-up (mean 3.9 years). The 95% isodose was applied to the entire target volume. After RS (23 patients), Class 1 results were observed in 10 patients; Class II in nine, Class IIIa in two, Class IIIb in one, and Class V results in one patient. Facial numbness occurred in two (8.7%) patients, and the trigeminal neuralgia recurred in two patients (8.7%). Following HSRT (22 patients), Class I results were achieved in eight patients, Class II in eight, Class IIIa in four, and Class IIIb in two patients; recurrence occurred in six (27.5%), and there were no complications. Thus, both RS and HSRT provided effective and safe therapy for the treatment of trigeminal neuralgia. Patients who underwent RS experienced better pain relief and a lower recurrence rate, whereas those who underwent HRST had no side effects, and in particular, no facial numbness.