Operative Treatment of Bertolotti Syndrome: Resection Versus Fusion.

OBJECTIVE: To compare the outcomes of joint resection versus fusion in patients who undergo operative treatment for Bertolotti syndrome. METHODS: A chart review identified patients with Bertolotti syndrome who underwent operative treatment, consisting of either Bertolotti joint decompression/resection or fusion across the abnormal transitional lumbosacral vertebrae. Patients with other symptomatic operative spinal disease were excluded. RESULTS: Twenty-seven patients (9 men, 18 women) were identified for inclusion in the study with an average age of 40 ± 16 years, body mass index of 27 ± 5, and follow-up of 39 ± 48 months. Most patients presented with back pain (74%) or leg pain (48%) for an average duration of 61 ± 54 months. Nineteen (70%) presented with a Castellvi subtype 2a Bertolotti joint with computed tomography as the most common method for radiographic diagnosis (56%). When comparing long-term pain improvement (>12 months) after fusion (n = 9) versus joint resection (n = 18), more fusion patients reported improvement in their pain (78%) compared to joint resection (28%, P = 0.037). There was not a statistically significant difference in the short-term pain improvement (<6 months) between the fusion (100%) and resection (78%) patients (P = 0.27). There was no statistically significant difference between the two groups in terms of age, sex, body mass index, presenting symptoms, symptom duration, Bertolotti injection response, follow up, Castellvi subtype, and complications. CONCLUSIONS: Patients with Bertolotti syndrome who underwent surgical fusion across the transitional lumbosacral vertebrae had a higher rate of long-term pain improvement compared to patients who had resection of the abnormal pseudoarticulation.

Thoracic vertebral osteomyelitis following catfish spine impalement.

Although rare, thoracic osteomyelitis can occur when toxins from a catfish spine impalement to the hand spread via the blood. This article describes diagnosis and management of this condition in a man who required hospitalization for debilitating back pain that developed 3 weeks after the initial injury.

Management of hypoglossal schwannomas: single institutional experience of 14 cases.

Background Hypoglossal schwannomas are rare intracranial neoplasms. Microsurgical resection with the goal of cure is the aim of management but is associated with a high rate of postoperative morbidity. Objective The objective of the study was to outline the clinical presentation, radiologic characteristics, surgical techniques, postoperative morbidity, and long-term follow-up results for hypoglossal schwannomas. Methods Patients treated for hypoglossal schwannoma at the Department of Neurosurgery of a tertiary-level referral institution from January 2001 until December 2010 were analyzed retrospectively using hospital records. Results There were 14 patients who were treated in the study period. Tongue atrophy and swallowing difficulties were the most common presenting symptoms. Surgery done in 12 patients using a variety of approaches (retromastoid retrosigmoid suboccipital in 9, midline suboccipital in 2, and far lateral in 1). Five patients having small residual tumors received gamma knife (GK) subsequently. two patients received primary GK stereotactic radiosurgery. Three patients had permanent morbidity in the form of cranial nerve paresis. Immediate postoperative complications like cerebrospinal fluid leak and pneumonia were present in three patients. Conclusion Complete microsurgical resection is often associated with a high rate of morbidity. Subtotal and near-total resection followed by stereotactic radiosurgery or observation now offers an alternative approach.

Cubitus valgus and tardy ulnar nerve palsy due to an intracapsular ulnar nerve.

BACKGROUND: We describe a patient with tardy ulnar neuropathy and cubitus valgus deformity found to have an intracapsular ulnar nerve. METHODS: An 89-year-old woman presented with severe neuropathic pain in the ulnar digits of the hand, advanced degenerative arthritis of the elbow, and tardy ulnar nerve palsy. Her pain was exacerbated with elbow movement, particularly flexion. She had paralysis of ulnar nerve innervated muscles, hypersensitivity with absence of two-point discrimination in her ulnar 1-1/2 digits, and a fixed ulnar claw deformity. She also had a grossly unstable elbow. RESULTS: Plain films revealed a cubitus valgus deformity (38°), an absent radial head, a dislocated proximal radioulnar joint and advanced arthritic changes. Ultrasonography revealed an indistinct ulnar nerve within the cubital tunnel which penetrated the joint. Electrophysiological studies revealed evidence of a severe ulnar neuropathy at the level of the elbow. Intraoperatively, an attenuated 2 cm length of the retrocondylar ulnar nerve was observed to be incorporated into the joint capsule tethered by a fibrous/synovial band which was released. A large effusion was drained. The ulnar nerve was transposed subcutaneously. The capsular rent was repaired in layers. She noted immediate and sustained (2 year follow-up) pain relief and regained moderate function in her interossei. CONCLUSIONS: We believe that the chronic cubitus valgus deformity and secondary degenerative elbow joint changes led to an altered course of the nerve and attenuation of the medial joint capsule such that the ulnar nerve spontaneously buttonholed itself intra-articularly.

Thoracic extraosseous, epidural, cavernous hemangioma: Case report and review of literature.

A 40-year-old male presented with mid-thoracic backache and progressive, ascending, spastic, paraparesis for one year. Magnetic resonance imaging demonstrated an extraosseous, extradural mass, without any bone invasion at the T2-T4 vertebral levels, located dorsal to the thecal sac. The spinal cord was compressed ventrally. The lesion was totally excised after a T2-T4 laminectomy. Histopathological examination revealed a cavernous hemangioma. The authors reported this case and reviewed the literature, to explain why extraosseous, extradural, cavernous hemangiomas should be considered in the differential diagnosis of extradural thoracic compressive myelopathy.

Analysis of changing paradigms of management in 179 patients with spinal tuberculosis over a 12-year period and proposal of a new management algorithm.

OBJECTIVE: To describe management and outcome in a large cohort of patients with spinal tuberculosis (TB). METHODS: Of 212 patients with spinal TB treated between January 1999 and June 2011, 179 patients were included in the study (≥6 months follow-up; mean age, 34.8 years; age range, 10-75 years). The cohort was divided into two groups (n = 89 and n = 90); group I was treated from 1999-2003, and group II was treated from 2004-2011. RESULTS: The study cohort comprised 93 male patients. Mean age was 34.8 years ± 7.2 (range, 10-75 years). Mean duration of symptoms was 2.4 months. Sensorimotor deficits were present in 167 patients (93.5%; 74 patients were paraplegic), pain was present in 156 patients (87%), bladder involvement was present in 127 patients (71.7%), and extraspinal TB was present in 36 patients (22.3%). Of patients, 92% were receiving prior chemotherapy; one fifth of these patients were on second-line chemotherapy. Thoracic spine involvement was most common (n = 86; 57%), followed by cervical spine (n = 50; 29%), craniovertebral junction (n = 22; 15%), and lumbosacral spine (n = 20; 10.5%). Surgery was performed in 146 patients (68% instrumented fusions and 16% circumferential fusions). Mean follow-up was 20.2 months (range, 6-60 months). Sensorimotor deficits improved in 89% of patients, pain improved in 71%, bladder symptoms improved in 88%, and paraplegia improved in 77%. Patients in group II had a higher incidence of cord compression (P < 0.01), severe vertebral body collapse (P < 0.001), and paraplegia (P < 0.001). Group II patients underwent more instrumented surgeries (P < 0.01), especially circumferential fusions (P < 0.001). The improvement in paraplegia was better after 2004 (group II). Bladder symptoms correlated with the timing of surgery (P < 0.1). CONCLUSIONS: Medical treatment of spinal TB is the mainstay; however, radical, instrumented surgeries should be offered when indicated. The presence of paraplegia should not preclude surgery. A practical management paradigm is also suggested.

L5-S1 extraforaminal intraneural disc herniation mimicking a malignant peripheral nerve sheath tumor.

BACKGROUND CONTEXT: An extraforaminal disc herniation may present as a retroperitoneal mass and is thus a differential diagnosis for a malignant lesion. PURPOSE: To highlight the difficulty in the accurate preoperative differentiation between an extraforaminal disc herniation and a malignant retroperitoneal mass despite the use of advanced anatomical and metabolic imaging. STUDY DESIGN: Case report. METHODS: The authors present a case of a 55-year-old man, with a family history of neurofibromatosis, who presented to us 2 months after the insidious occurrence of severe, unrelenting, right-sided, L5 dermatomal pain. He subsequently developed rapidly progressive motor weakness in the same myotome, which caused a foot drop. On examination, his straight-leg raise test was normal. Magnetic resonance imaging demonstrated a peripherally enhancing, complex, cystic mass continuous with the L5 nerve, displacing its fascicles medially, as it descended over the right sacral ala. A positron emission tomography scan demonstrated increased radiotracer uptake. The clinicoradiologic presentation was that of a malignant peripheral nerve sheath tumor. RESULTS: At surgery, an intraneural disc herniation was seen. CONCLUSIONS: Several pitfalls exist in establishing the uncommon diagnosis of an extraforaminal disc herniation. This entity occurs ventrally and must remain a consideration when elaborating the differential diagnosis of a retroperitoneal mass in close proximity to the vertebral column.

Cushing's disease: results of treatment and factors affecting outcome.

OBJECTIVE: To analyze the therapeutic results of intervention and the factors affecting the outcome of patients with Cushing's disease (CD) cared for at the All Indian Institute of Medical Science (AIIMS), New Delhi. DESIGN: Patients with Cushing's disease treated at a teritiary care centre from January 2000 to December 2009 were prospectively studied. RESULTS: Ninety-seven patients received treatment for CD during this period. Mean duration of follow-up was 3.4 ± 2.2 (mean ± SD) years. Eighty-one patients (83.5%) underwent transsphenoidal surgery (TSS) as the primary treatment modality. Fifty-four patients (66.7%) went into remission after initial TSS; ten (18.5%) of them relapsed after a mean follow-up period of 2.9 ± 2.1 (mean ± SD) years. Histopathologic examination of resected tissue showed corticotroph adenoma in 48 of the 54 (88.9%) who went into remission and 17 of the 27 (63.0%) who did not go into remission after the initial TSS. Sixteen patients with severe hypercortisolism underwent bilateral adrenalectomy (BA) as a life-saving measure which was followed by pituitary surgery 6 to 12 months later. Five patients including one with a large macroadenoma required three or more procedures to achieve eucortisolism. CONCLUSION: Fifty-four out of 81 (66.7%) of our patients with CD had remission following initial TSS, ten of whom relapsed later on. Sixteen patients unerwent BA as a life-saving procedure. Factors affecting outcome were, age, gender, low dose dexamethasone suppression test cortisol value and histologic confirmation of corticotroph adenoma.

Effect of risk-stratified, protocol-based perioperative chemoprophylaxis on nosocomial infection rates in a series of 31 927 consecutive neurosurgical procedures (1994-2006).

OBJECTIVE: Although the use of prophylactic antibiotics has been shown to significantly decrease the incidence of meningitis after neurosurgery, its effect on extra-neurosurgical-site infections has not been documented. The authors explore the effect of risk-stratified, protocol-based perioperative antibiotic prophylaxis on nosocomial infections in an audit of 31 927 consecutive routine and emergency neurosurgical procedures. METHODS: Infection rates were objectively quantified by bacteriological positivity on culture of cerebrospinal fluid (CSF), blood, urine, wound swab, and tracheal aspirate samples derived from patients with clinicoradiological features of sepsis. Infections were recorded as pulmonary, wound, blood, CSF, and urinary. The total numbers of hospital-acquired infections and the number of patients infected were also recorded. A protocol of perioperative antibiotic prophylaxis of variable duration stratified by patient risk factors was introduced in 2000, which was chosen as the historical turning point. The chi test was used to compare infection rates. A P value of <0.05 was considered significant. RESULTS: A total of 31 927 procedures were performed during the study period 1994-2006; 5171 culture-proven hospital-acquired infections (16.2%) developed in 3686 patients (11.6%). The most common infections were pulmonary (4.4%), followed by bloodstream (3.5%), urinary (3.0%), CSF (2.9%), and wound (2.5%). The incidence of positive tracheal, CSF, blood, wound, and urine cultures decreased significantly after 2000. Chemoprophylaxis, however, altered the prevalent bacterial flora and may have led to the emergence of methicillin-resistant Staphylococcus aureus. CONCLUSION: A risk-stratified protocol of perioperative antibiotic prophylaxis may help to significantly decrease not only neurosurgical, but also extra-neurosurgical-site body fluid bacteriological culture positivity.

Spinal meningioma containing bone: a case report and review of literature.

Meningiomas constitute about 25% of primary spinal tumours and 1% to 5% of them are calcified. Ossification is a rare event and is rarely reported. Here, the case of a 40-year-old woman who had dorsal spinal cord meningioma (globular variety) at the T(6) vertebral level is reported; the meningioma showed a nidus of T2 weighting hypointensity on MRI as well as a bony chip inside the tumour intraoperatively. The tumour was successfully resected. Though the aetiology of ossification in the meningioma is not well known, metaplasia of arachnoid cells/dystrophic calcification may be the cause. Ossified meningiomas are more difficult to resect than the usual variety. Hypointensity inside tumour in T2-weighted images of MRI should make the surgeon suspicious of this condition, which may in some cases complicate tumour resection.

Radiation tolerance limits of the brainstem.

OBJECTIVE: One of the key limitations of gamma knife surgery arises from the radiation safety tolerance limit of the brainstem. The authors conducted an analysis of patients with intra-axial brainstem lesions and documented the incidence of adverse radiation imaging effects (ARIE) and new neurological deficits after gamma knife surgery. METHODS: Thirty-eight patients (39 lesions) with intra-axial brainstem astrocytomas or vascular malformations underwent gamma knife surgery during a 6-year interval. Brainstem exposure volume was calculated by subtracting the volume within the 12-Gy isodose line (12 Gray volume) from the prescription volume. ARIE was defined as a new parenchymal signal alteration on follow-up magnetic resonance imaging sequences. RESULTS: The average prescription volume was 1.46 cm, 12 Gy volume was 2.03 cm, and brainstem exposure volume was 0.57 cm. Seven (18.4%) patients developed ARIE. ARIE correlated only with the presence of new neurological deficits and age younger than 40 years. Three (7.9%) patients developed minor residual deficits without any ARIE. There was no mortality. CONCLUSION: Exposure of the brainstem to more than 12 Gy at volumes as low as 0.1 cm can produce ARIE and new neurological deficits. The tolerance of the brainstem to radiosurgery is related to patient age, lesion volume, and pathology. Analysis of the exposed volume of brainstem tissue may be useful in radiosurgical planning for individual patients.

Capnography as an aid in localizing the phrenic nerve in brachial plexus surgery. Technical note.

BACKGROUND: To determine whether monitoring end- tidal Carbon Dioxide (capnography) can be used to reliably identify the phrenic nerve during the supraclavicular exploration for brachial plexus injury. METHODS: Three consecutive patients with traction pan-brachial plexus injuries scheduled for neurotization were evaluated under an anesthetic protocol to allow intraoperative electrophysiology. Muscle relaxants were avoided, anaesthesia was induced with propofol and fentanyl and the airway was secured with an appropriate sized laryngeal mask airway. Routine monitoring included heart rate, noninvasive blood pressure, pulse oximetry and time capnography. The phrenic nerve was identified after blind bipolar electrical stimulation using a handheld bipolar nerve stimulator set at 2-4 mA. The capnographic wave form was observed by the neuroanesthetist and simultaneous diaphragmatic contraction was assessed by the surgical assistant. Both observers were blinded as to when the bipolar stimulating electrode was actually in use. RESULTS: In all patients, the capnographic wave form revealed a notch at a stimulating amplitude of about 2-4 mA. This became progressively jagged with increasing current till diaphragmatic contraction could be palpated by the blinded surgical assistant at about 6-7 mA. CONCLUSION: Capnography is a sensitive intraoperative test for localizing the phrenic nerve during the supraclavicular approach to the brachial plexus.

Gamma knife radiosurgery for glomus jugulare tumors: therapeutic advantages of minimalism in the skull base.

CONTEXT: Glomus jugulare (GJ) tumors are paragangliomas found in the region of the jugular foramen. Surgery with/without embolization and conventional radiotherapy has been the traditional management option. AIM: To analyze the efficacy of gamma knife radiosurgery (GKS) as a primary or an adjunctive form of therapy. SETTINGS AND DESIGN: A retrospective analysis of patients who received GKS at a tertiary neurosurgical center was performed. MATERIALS AND METHODS: Of the 1601 patients who underwent GKS from 1997 to 2006, 24 patients with GJ underwent 25 procedures. RESULTS: The average age of the cohort was 46.6 years (range, 22-76 years) and the male to female ratio was 1:2. The most common neurological deficit was IX, X, XI cranial nerve paresis (15/24). Fifteen patients received primary GKS. Mean tumor size was 8.7 cc (range 1.1-17.2 cc). The coverage achieved was 93.1% (range 90-97%) using a mean tumor margin dose of 16.4 Gy (range 12-25 Gy) at a mean isodose of 49.5% (range 45-50%). Thirteen patients (six primary and seven secondary) were available for follow-up at a median interval of 24 months (range seven to 48 months). The average tumor size was 7.9 cc (range 1.1-17.2 cc). Using a mean tumor margin dose of 16.3 Gy (range 12-20 Gy) 93.6% coverage (range 91-97%) was achieved. Six patients improved clinically. A single patient developed transient trigeminal neuralgia. Magnetic resonance imaging follow-up was available for 10 patients; seven recorded a decrease in size. There was no tumor progression. CONCLUSIONS: Gamma knife radiosurgery is a safe and effective primary and secondary modality of treatment for GJ.

Metachronous pheochromocytoma metastasis to the upper dorsal spine-6-year survival.

BACKGROUND CONTEXT: Malignant pheochromocytoma is a rare neoplasm of chromaffin tissue. Very few cases of malignant adrenal pheochromocytoma metastatic to vertebrae exist. PURPOSE: To determine the prognosis of a patient with an excised adrenal pheochromocytoma and a single metachronous metastasis to the upper dorsal spine. STUDY DESIGN: Case report METHODS: The authors report a patient who underwent total excision of an adrenal pheochromocytoma of the left adrenal gland in 2000 who developed a single metastasis to the second dorsal vertebra in 2002 with no evidence of abdominal recurrence. RESULTS: Four-year survival is documented after the spinal metastasis was first detected after two attempts at excision and radiotherapy. CONCLUSIONS: Patients with adrenal pheochromocytomas must be screened periodically with whole body imaging despite normal abdominal imaging as there is a definite risk of metachronous metastasis. Aggressive therapy may result in improving survival significantly in a subset of patients with isolated spinal metastases.

Synchronous endometrial carcinoma and a macroprolactinoma: exploring a causal relationship.

BACKGROUND: While unopposed estrogen hormone secretion is most commonly implicated in the pathogenesis of endometrial carcinoma, the role of prolactin has only recently been highlighted. The authors present a case of a synchronous endometrial carcinoma in a patient with a prolactin-secreting macroadenoma. METHODS: A 29-year-old woman presented with a 4-year history of primary infertility, irregular periods and intermittent galactorrhea. Hormonal evaluation revealed elevated prolactin and subnormal luteinizing hormone and follicle-stimulating hormone (FSH) serum concentrations. An ultrasound of the pelvis revealed endometrial thickening. The MRI of the brain confirmed a pituitary macroadenoma. The patient underwent a resectoscopic polypectomy and dilation and curettage followed by transnasal transsphenoidal excision of the pituitary macroadenoma. RESULTS: The biopsy of the endometrium revealed a well-differentiated endometrioid carcinoma while that of the pituitary tumor confirmed a prolactinoma. CONCLUSION: An indirect causal mechanism can be postulated to explain this association. Hyperprolactinemia inhibits gonadotropin-releasing hormone leading to subnormal FSH and luteinizing hormone levels. Though the patient is hypoestrogenic, chronic anovulation with unopposed estrogen secretion can increase the risk of endometrial carcinoma. Patients with prolactinomas and irregular menstrual bleeding should undergo endometrial sampling to rule out this possibility.