Mirror image of bilateral DACA aneurysm with its successful surgical management.

BACKGROUND: Among various locations of intracranial aneurysms reported in the literature, two different aneurysms situated symmetrically opposite on bilateral distal anterior cerebral arteries (DACA) are very rare. CASE DESCRIPTION: Here, we report a rare case of mirror image distal anterior cerebral aneurysm in a middle-aged male patient. The patient presented with severe headache and loss of consciousness. Angiography was done which suggested mirror imaging of two aneurysms located over both DACA. It was treated through microsurgical approach with a successful outcome. CONCLUSION: Careful analysis of intracranial vasculature should be done using angiography, particularly in different views and stages to rule out multiple aneurysms at different locations in the same artery or at different arteries. Mirror images of bilateral DACA aneurysms are very rare. Fundamental surgical strategy of securing the parent artery and clipping the neck after meticulous dissection should be followed.

A large primary orbital lymphoma with proptosis: A case report and review.

BACKGROUND: Primary orbital lymphomas are a rare subset of tumors constituting 1-2% of non-Hodgkin's lymphoma. They are mostly indolent B-cell lymphomas presenting with gradual progressive proptosis, decreased visual acuity, restricted ocular mobility, and diplopia. The role of surgery is mainly for obtaining a biopsy. Most of these tumors require multimodality treatment including chemotherapy, radiation, or both, which have major role. CASE DESCRIPTION: We report one such case of marginal zone lymphoma of the orbit in a female with significant proptosis who was treated with multimodality treatment, including surgical excision as a major treatment modality. Decompression of symptomatic proptosis was followed by chemotherapy and radiation. CONCLUSION: Primary orbital lymphoma is a rare clinical entity with diverse clinical outcomes. It can be successfully managed with surgical excision for decompression of mechanical proptosis followed by chemotherapy, radiation, or both.

Surgical management of acromegaly: Long term functional outcome analysis and assessment of recurrent/residual disease.

CONTEXT: Functional growth hormone producing adenomas have long-term deleterious effects on the visual apparatus, the cardiovascular and musculoskeletal systems, and often predispose to malignancies. Since persistence of acromegaly affects outcome and quality of life, therapeutic interventions become mandatory. AIM: This study represents an analysis of long-term clinical and endocrinal outcome of 115 patients of acromegaly after surgical management. SETTING AND DESIGN: Tertiary care retrospective study. MATERIALS AND METHODS: One hundred and fifteen patients (male:female ratio: 1:1.09) with acromegalic features were studied. Apart from acromegalic features, their main clinical presentation also included headache, diminution of vision, field defects, ptosis, irregular menstruation, diabetes insipidus, diabetes mellitus and hypertension. Six of them presented with apoplexy. Their preoperative endocrinal evaluation included basal and suppressed growth hormone (GH), prolactin and thyroid levels. On the basis of axial and coronal CT scan or multiplanar MR imaging or both, the tumors were classified according to their suprasellar and parasellar extension (Hardy's grade). Transnasal trans-sphenoidal surgery (TSS) (n = 37) and sublabial, rhinoseptal TSS (n = 72) were the preferred approaches. Six patients with significant parasellar extensions underwent trans-cranial explorations. The patients were followed up at 6 and 12 weeks and then at 6 monthly intervals. Hormonal and CT/MR evaluation were also done. Attainment of random GH value less than 2.5 µg/L, and the nadir GH value after oral glucose tolerance test (OGTT) less than 1 µg/L were used as the criteria of cure. FINDINGS: The patients were preoperatively in Hardy's tumor grade 0 (29), A (21), A+E (3), B (21), B+E (5), C (9), C+E (10), D (1) D+E (11), E (5), respectively. One hundred and one patients were available for follow-up (FU; median FU duration: 84 months; range: 6 to 132 months). Surgical cure was achieved in 73 patients following the first surgery; and, in 10 additional patients following a second intervention. No patient with a preoperative grade 0, A, B, C had a recurrence after attaining the initial remission. Recurrence after an initial cure occurred in 7 patients (overall remission rate following surgery: 75.24%). The preoperative grade of the latter patients was A+E:1, B+E:1, C+E:1, D:1, D+E:2, E:1, respectively. All these patients underwent subsequent radiotherapy (RT). The twelve patients with persistent symptoms and high GH levels following surgery underwent RT; six others with improved symptoms despite high GH levels were kept under strict observation. There was no surgical mortality. CONCLUSIONS: A high remission rate without significant morbidity could be achieved following surgical intervention in acromegalic patients. Following surgery, tumors with greater than or equal to 3 cm suprasellar height and without parasellar extension had no clinical recurrence at FU. A continuous monitoring is mandatory to pick up relapsed cases as well as those who develop delayed signs of hypopituitarism. A subset of patients who show clinical improvement following surgery but still have higher GH levels may be followed up without additional therapy unless clinical signs reappear or the serum GH levels progressively increase.

Posterior inferior cerebellar artery aneurysms: Anatomical variations and surgical strategies.

CONTEXT: Posterior inferior cerebellar artery (PICA) aneurysms are associated with multiple anatomical variations of the parent vessel. Complexities in their surgical clipping relate to narrow corridors limited by brain-stem, petrous-occipital bones, and multiple neurovascular structures occupying the cerebellomedullary and cerebellopontine cisterns. AIMS: The present study focuses on surgical considerations during clipping of saccular PICA aneurysms. SETTING AND DESIGN: Tertiary care, retrospective study. MATERIALS AND METHODS: In 20 patients with PICA aneurysms, CT angiogram/digital substraction angiogram was used to correlate the site and anatomical variations of aneurysms located on different segments of PICA with the approach selected, the difficulties encountered and the final outcome. STATISTICAL ANALYSIS: Comparison of means and percentages. RESULTS: ANEURYSMS WERE LOCATED ON PICA AT: vertebral artery/basilar artery (VA/BA)-PICA (n=5); anterior medullary (n=4); lateral medullary (n=3); tonsillomedullary (n=4); and, telovelotonsillar (n=4) segments. The Hunt and Hess grade distribution was I in 15; II in 2; and, III in 3 patients (mean ictus-surgery interval: 23.5 days; range: 3-150 days). Eight patients had hydrocephalus. Anatomical variations included giant, thrombosed aneurysms; 2 PICA aneurysms proximal to an arteriovenous malformation; bilobed or multiple aneurysms; low PICA situated at the foramen magnum with a hypoplastic VA; and fenestrated PICA. The approaches included a retromastoid suboccipital craniectomy (n=9); midline suboccipital craniectomy (n=6); and far-lateral approach (n=5). At a follow-up (range 6 months-2.5 years), 13 patients had no deficits (modified Rankin score (mRS) 0); 2 were symptomatic with no significant disability (mRS1); 1 had mild disability (mRS2); 1 had moderately severe disability (mRS4); and 3 died (mRS6). Three mortalities were caused by vasospasm (2) and, rupture of unclipped second VA-BA junctional aneurysm (1). CONCLUSIONS: PICA aneurysms may present with only IV(th) ventricular blood without subarachnoid hemorrhage. PICA may have multiple anomalies and its aneurysms may be missed on CT angiograms. Surgical approach is influenced by VA-BA tortuosity and variations in anatomy, location of the VA-BA junction and the PICA aneurysm relative to the brain-stem, and the pattern of collateral supply. The special category of VA-PICA junctional aneurysms and its management; and, the multiple anatomical variations of PICA aneurysms, merit special surgical considerations and have been highlighted in this study.

Atlantoaxial dislocation.

Atlanto-axial dislocations (AADs) may be classified into four varieties depending upon the direction and plane of the dislocation i.e. anteroposterior, rotatory, central, and mixed dislocations. However, from the surgical point of view these are divided into two categories i.e. reducible (RAADs) and irreducible (IAADs). Posterior fusion is the treatment of choice for RAAD. Transarticular screw fixation with sub-laminar wiring is the most stable& method of posterior fusion. Often, IAAD is due to inadequate extension in dynamic X-ray study which may also be due to spasm of muscles. If the anatomy at the occipito-atlanto-axial region {O-C1-C2; O: occiput, C1: atlas, C2: axis} is normal on X-ray, the dislocation should be reducible. In case congenital anomalies at O-C1-C2 and IAAD are seen on flexion/extension studies of the cervical spine, the C1-C2 joints should be seen in computerized tomography scan (CT). If the C1-C2 joint facet surfaces are normal, the AAD should be reducible by cervical traction or during surgery by mobilizing the joints. The entity termed "dolichoodontoid" does not exist. It is invariably C2-C3 (C3- third cervical vertebra) fusion which gives an appearance of dolichoodontoid on plain X-ray or on mid-saggital section of magnetic resonance imaging (MRI) or CT scan. The central dislocation and axial invagination should not be confused with basilar invagination. Transoral odontoidectomy alone is never sufficient in cases of congenital IAAD, adequate generous three-dimensional decompression while protecting the underlying neural structures should be achieved. Chronic post-traumatic IAAD are usually Type II odontoid fractures which get malunited or nonunited with pseudoarthrosis in dislocated position. All these dislocations can be reduced by transoral removal of the offending bone, callous and fibrous tissue.

The "moustache" sign: localized intervertebral disc fibrosis and panligamentous ossification in ankylosing spondylitis with kyphosis.

Localized intervertebral disc and ligamentous ossification may precipitate neurological deficits at any time during the natural course of ankylosing spondylitis (AS). We report 2 patients with AS with "moustache' sign (localized intervertebral disc fibrosis and panligamentous ossification) and neurological deficits. One patient had syndesmophytosis (with paraparesis secondary to thoracic spinal canal stenosis), and the other had "bamboo spine" above and below the involved level. A laminectomy in the former relieved paraparesis, and posterior element excision in both the patients relieved focal tenderness and pain; and both of them could lie supine following surgery.

Os odontoideum with "free-floating" atlantal arch causing C1-2 anterolisthesis and retrolisthesis with cervicomedullary compression.

BACKGROUND: Os odontoideum (OO) with C1-2 anterolisthesis and retrolisthesis may cause cervicomedullary injury both from anterior and posterior aspects. We analyzed fourteen such patients for biomechanical issues, radiological features and management of OO with free-floating atlantal arch and review pertinent literature. MATERIALS AND METHODS: Fourteen patients having nonsyndromic, reducible atlantoaxial dislocation (AAD) with orthotopic OO were analyzed. During neck flexion, their C1 anterior arch-os complex displaced anteriorly relative to remnant odontoid-C2 body. The posteriorly directed hypoplastic remnant odontoid sliding below the atlas and forward translation of the C1 posterior arch caused concomitant cervicomedullary compression. During neck extension, there was retrolisthesis of the "free-floating" C1 arch-os complex into spinal canal. Spinal stenosis and lateral C1-2 facet dislocation; Klippel-Feil anomaly; and posterior circulation infarcts were also present in one patient each, respectively. Posterior C1-2 (n=10) or occipitocervical fusion (n=3) was performed in neutral position to stabilize atlantoaxial movements. RESULTS: Follow-up (mean, 3.9 years) assessment revealed improvement in spasticity and weakness in 13 patients. One patient had neurological deterioration following C1-2 posterior sublaminar fusion, requiring its conversion to occipitocervical contoured rod fusion. One patient with posterior circulation stroke died prior to any operative intervention. Follow-up lateral view radiographs showed a bony union or a stable construct in these 13 patients. CONCLUSIONS: OO with free-floating atlantal arch may precipitate cord injury both during neck flexion and extension. This condition may be overlooked unless lateral radiographs of craniovertebral junction are undertaken in neck extension, along with the usual ones in neutral and flexed positions. Etiological factors include C1 ring-OO unrestrained movements above the hypoplastic odontoid; upward pull on OO by alar and apical ligaments; lax C1-2 facet joint ligaments; and congenital presence of horizontal facet joint surfaces that facilitates C1-2 translation.

Postauricular, transpetrous, presigmoid approach for extensive skull base tumors in the petroclival region: the successes and the travails.

PURPOSE: Postauricular, transpetrous, presigmoid approach combines a supra/infratentorial exposure with partial petrosectomy to access third to the twelfth cranial nerves and extraaxial lesions situated anterolateral to brainstem. It provides a shorter working distance to large petrosal, petroclival, cerebellopontine, and cerebellomedullary cisternal lesions and their extensions to the subtemporal-infratemporal areas. This study reviews the surgical technique, corridors of extension, and complications encountered utilizing this approach for excising extensive lesions in these locations. METHODS: The lesions (n = 14) included petroclival meningiomas [(n = 5), including three recurrent lesions], dumbbell lower cranial nerve schwannomas (n = 2), giant acoustic schwannomas (n = 2), recurrent giant trigeminal nerve schwannoma (n = 1), glomus jugulare (n = 3), and recurrent petrous aneurysmal bone cyst (n = 1). The approach was combined with a retrosigmoid suboccipital craniectomy (n = 3), with an infratemporal approach (n = 2), and with an extreme lateral transcondylar approach and a translabyrinthine approach in one patient each, respectively. External auditory canal was not ligated in nine patients, superior petrosal sinus and tentorial division was performed in all patients, and sigmoid sinus-internal jugular vein was excised in three patients (with a glomus jugulare (n = 1) and petroclival meningioma (n = 2), respectively). Repair was performed with fat-fascia, pedicled pericranium, and temporalis muscle. Lumbar drain was placed for three to five postoperative days. RESULTS: Total excision was performed in nine patients. Small tumor remnants were left attached to the brainstem (n = 3, petroclival meningioma), carotid canal and cavernous sinus (n = 1, glomus jugulare), and sigmoid sinus-jugular bulb (n = 1, recurrent trigeminal schwannoma). A two-staged procedure was performed in three patients. Two patients with recurrent giant petroclival meningiomas died: one with lower cranial nerve paresis due to aspiration pneumonitis and the other with cerebrospinal fluid otorrhoea and secondary meningitis. CONCLUSIONS: The approach facilitates direct tumor decompression and its retraction away from the brainstem without initially encountering the intracisternal cranial nerves and neuraxis. It provides multiple corridors for excising extensive posterior fossa tumors. Preoperative assessment of sigmoid sinus dominance, jugular bulb height, labyrinth, vein of Labbe, and space available through Trautman's triangle considerably helps in complication avoidance.

Pediatric colloid cysts of the third ventricle: management considerations.

PURPOSE: Pediatric colloid cysts (CC) have a congenital origin, and yet, there are very few studies focussing exclusively on their occurrence in the pediatric population. Pediatric CC has been associated with more aggressive clinical and radiological patterns than their adult counterparts. In this study, undertaken on children with anterior third ventricular CC, excised using the interhemispheric transcallosal approach, the characteristic clinicoradiological features and management options are studied. METHODS: Five pediatric patients (aged 16 years or less; mean age 13.8 years; mean duration of symptoms:7.6 months) out of 38 patients with CC operated between 1995 to 2009 were included. The clinical manifestations included those of raised intracranial pressure (n = 4); exacerbation of occipital headache on reading (n = 1); secondary optic atrophy (n = 3); and, drop attacks (n = 1). On computed tomography scan, the cyst was hyperdense, enhancing in two patients and not enhancing in three patients. All had bilateral lateral ventricular dilatation with periventricular lucency. On magnetic resonance imaging (n = 3), the cyst was T1 hypointense and T2 isointense in one, hyperintense on both T1 and T2 with a hypointense capsule and nonenhancing on contrast in one (with a giant colloid cyst), and T1 hyperintense and T2 hypointense in one patient. An interhemispheric, transcallosal trajectory combined with transforminal approach (n = 3); combined transforminal and subchoroidal approaches (n = 1); and, interforniceal approach (n = 1) were used. RESULTS: Total excision was performed in four patients. In one patient, a small part of capsule was left attached to thalamostriate vein. Symptoms of raised intracranial pressure showed improvement in all the patients with resolution of hydrocephalus. There was no tumor recurrence at follow-up. CONCLUSIONS: Pediatric colloid cysts are rarer than their adult counterparts due to their late detection only after manifestations of raised intracranial pressure, visual or cognitive dysfunction or drop attacks occur. Their radiological appearance varies depending upon the amount of mucoid content, cholesterol, proteins, and water content. The fast development of clinical manifestations in children may be related to rapid enlargement of cyst due to higher water content within them. The transcallosal approach is the "gold standard" of surgery and usually ensures gratifying and lasting results.

Effect of spinal cord signal intensity changes on clinical outcome after surgery for cervical spondylotic myelopathy.

OBJECT: The presence of intramedullary T2 high signal intensity changes in patients with cervical spondylotic myelopathy (CSM) indicates the existence of a chronic spinal cord compressive lesion. However, the prognostic significance of signal intensity changes remains controversial. The purpose of this study was to evaluate the effect of spinal cord T2 signal intensity changes on the outcome after surgery for CSM. METHOD: In a prospective study, 64 patients with CSM who underwent surgical treatment between October 2006 and April 2008 using an anterior approach were included. Based on the clinical symptoms and signs present, the severity of neurological deficits of all patients was scored according to a modified Japanese Orthopaedic Association scale score for CSM just before the surgery and at 6 months follow-up. Recovery rates were calculated at 6 months. RESULTS: There were 22 patients who did not have spinal cord intensity changes on MR imaging and 44 who demonstrated high-intensity signal changes on T2-weighted images (focal or segmental). No statistically significant differences were found in recovery rates between cases with T2 signal intensity changes and those with no signal intensity changes. However, the postoperative modified Japanese Orthopaedic Association scale scores and the recovery rates were much lower in patients with multisegmental signal intensity changes compared with those without these changes or those with focal signal intensity change, and ANOVA demonstrated this difference to be statistically significant (p < 0.05). CONCLUSION: Multisegmental spinal cord signal intensity changes on T2-weighted MR imaging are predictors of a poor outcome in terms of functional recovery rate in patients undergoing operations for CSM.

Fronto-temporo-orbitozygomatic craniotomy and "half-and-half" approach for basilar apex aneurysms.

BACKGROUND: Basilar apex aneurysms (BAA) are located in interpeduncular cistern surrounded by eloquent neurovascular structures. Surgical access is difficult due to narrow surgical corridors and requires traversing through a depth of 6-8 cm of subarachnoid space. AIM: Surgical management of BAAs clipped using frontotemporal craniotomy, orbitozygomatic osteotomy with combined subtemporal and transylvian (half and half) approach is discussed. SETTING AND DESIGN: Tertiary care referral institute; prospective study. MATERIALS AND METHODS: Five patients with BAA rupture causing subarachnoid hemorrhage presented in modified Hunt and Hess (Hand H) grades II (n=1), III (n=1) and IV (n=3), respectively. In 4 patients, the aneurysms were 0.8-1.2 cm in diameter, situated 7 mm-1 cm above dorsum sellae. Two of them had posteriorly projecting aneurysms. One patient had a giant, high BAA with a left parietooccipital arteriovenous malformation. Vasospasm of posterior cerebral/proximal basilar artery was seen in 2 patients. In one patient, internal carotid artery was mobilized by intradural anterior clinoid drilling with carotid collar division. Triple-H therapy was administered following surgery. RESULTS: There was no intraoperative rupture or temporary clipping. Follow up angiography showed complete aneurysmal obliteration with preservation of posterior cerebral and superior cerebellar arteries. Follow up (mean: 8.7+/-3.5 months) H and H grades were II (n=2) and III (n=3), respectively. The morbidity include caudate and thalamic region infarct, transient III rd nerve palsy and cerebrospinal fluid otorrhoea (n=1, respectively). CONCLUSIONS: This simple approach provides a wide surgical corridor from 5 mm below to greater than 1 cm above dorsum sellae with adequate proximal control of basilar artery. It is an option to endovascular embolization especially with large and giant, or wide-necked BAA, vertebrobasilar tortuosity, coil compaction or postcoiling re-rupture and an associated large haematoma.

Tentorial meningiomas: operative nuances and perioperative management dilemmas.

PURPOSE: Tentorial meningiomas, comprising approximately 3-6% of all intracranial meningiomas, are complex entities with an intricate relationship to surrounding structures and require multiple surgical approaches. In the present study, the rationale for deciding the approaches for TMs and the perioperative management dilemmas were evaluated. METHODS: Thirty-seven patients (28 primary [supratentorial (2), infratentorial (20) and both (6)] and nine complex [cerebellopontine (CP) angle (5) and petroclival (4)] underwent surgery using the occipital transtentorial, supracerebellar infratentorial, subtemporal transtentorial, bioccipital suboccipital, midline suboccipital, retrosigmoid, and combined pre and retrosigmoid approaches. The extent of excision was categorized according to Simpson's grade. RESULTS: Simpson's grade of excision was I in six, II in 11, III in nine and IV in 11 patients, respectively. Follow-up assessment (2 months to 9 years) in 27 patients (72.9%) showed that 23 patients returned to their previous activity level with either no or minimal symptoms, three returned to previous activity level with major cranial nerve palsy, and one patient required permanent assistance. One patient had recurrence and four others underwent resurgery for residual tumor. Two patients with petroclival lesions died due to aspiration pneumonitis and meningitis, respectively; one with CP angle TM presented in a poor general condition and expired following emergency ventriculoperitoneal shunt and subsequent definite surgery. Pseudomeningocele, cerebrospinal fluid leak, and cranial nerve palsy were the major morbidities. CONCLUSIONS: Classifying TM into medial and lateral, supra and infratentorial groups helps in deciding an appropriate and safe approach. Meticulously preserving venous sinuses is important since the risk of venous infarction cannot be predicted even with radiological good venous collaterization and apparent venous sinus blockade by tumor. Laterally situated tumors carry a better prognosis when compared to the medially situated ones. Leaving a small residual tumor in an effort to preserve important neurovascular structures does not obviate the expectation of a good long-term prognosis with minimal morbidity and low recurrence rates.

Choroid plexus papilloma in children: Diagnostic and surgical considerations.

BACKGROUND: Choroid plexus papilloma (CPP) is a benign neoplasm that arises from the ventricular choroid plexus. The clinical features, radiological characteristics, and treatment have been discussed in this study for a pediatric population. METHODS: Over an eight-year period, seven pediatric (≤12 years) CPP patients were treated. Tumors were located in the lateral ventricle (n = 4), IVth ventricle (n = 2), and in both the lateral and IIIrd ventricles (n = 1). The patients presented predominantly with features of raised intracranial pressure. Total microsurgical excision was carried out in all cases. RESULTS: There was complete relief of symptoms at follow-up in six patients. A 2.5 year-old child with a large trigonal CPP with hydrocephalus leading to complete visual impairment, died due to postoperative hypokalemia that caused ventricular fibrillation. One of our patients required a postoperative, permanent CSF diversion procedure while another required a subduroperitoneal shunt for persisting postoperative subdural CSF collection. CONCLUSIONS: Coagulation of the tumor under constant irrigation to shrink and excise it in toto, avoids excessive bleeding during surgery. The vascular pedicle supplying the tumor should be adequately dealt with during the last part of tumor removal as retraction of a bleeding pedicle may result in ventricular hemorrhage and brain edema. Following surgery, an external ventricular drain for three days helps in preventing the development of acute hydrocephalus in lateral ventricular lesions, and the color of the drained CSF gives an estimate of the ventricular hemostasis achieved. Total excision is usually possible in these cases with excellent postoperative outcomes.

Surgical strategies for giant medial sphenoid wing meningiomas: a new scoring system for predicting extent of resection.

BACKGROUND: Surgical management of giant medial sphenoid meningiomas (> or =5 cm in maximum dimension) is extremely challenging due to their intimate relationship with vital neural structures like the optic nerve, cranial nerves of the cavernous sinus and the cavernous internal carotid artery. Their surgical management is presented incorporating a radiological scoring system that predicts the grade of tumour excision. MATERIALS AND METHODS: 20 patients of giant medial sphenoidal wing meningioma (maximum tumour dimension range: 5.2 to 9.5 cm; mean maximum dimension = 6.12 +/- 1.06 cm) with mainly visual and extraocular movement deficits, and raised intracranial pressure, underwent surgery. A preoperative radiological scoring system (range 1-12) was proposed considering tumour volume (using Kawamoto's method); extension into the surrounding surgical corridors; extent of cavernous sinus invasion (based on the tumour relationship to the cavernous internal carotid artery); associated hyperostosis and/or >50% calcification; and, associated brain oedema. Both the conventional frontotemporal craniotomy (n = 13) and its extension to orbitozygomatic osteotomy (n = 7) were utilized. The cavernous sinus was explored in 4 patients and the hyperostotic sphenoid ridge drilled in five patients. FINDINGS: Total excision was achieved in nine patients; small tumour remnants within the cavernous sinus, interpeduncular fossa or suprasellar cistern were left in eight patients; and less than 10% of tumour was left in three patients. A patient with a completely calcified meningioma died due to myocardial infarction. When the preoperative radiological score was > or =7, there was considerable difficulty in achieving total tumour excision. A mean follow of 17.58 +/- 15.05 months revealed improvement in visual acuity/field defects in three, stabilisation in 11, and deterioration of ipsilateral visual acuity in five patients. Symptoms of raised pressure, cognitive dysfunction, aphasia and proptosis showed improvement. CONCLUSION: A relatively conservative approach to these extensive lesions resulted in good outcome in a majority of our patients. Both the standard as well as skull base approaches may be utilized for successful removal of giant medial sphenoidal wing meningiomas. A preoperative radiological score of > or =7 predicts a greater degree of difficulty in achieving complete surgical extirpation.

Taste dysfunction in vestibular schwannomas.

BACKGROUND: Gustatory dysfunction associated with vestibular schwannomas (VS) is a poorly represented clinical presentation. MATERIALS AND METHODS: One hundred and forty-nine cases operated from 1997 to 2005 where at least six-month follow-up was available were included. All patients were tested for taste sensations using four modalities of standard taste solutions. Apart from the taste sensations, any altered or abnormal taste perceptions were recorded both in the preoperative and postoperative period. RESULTS: After applying the exclusion criteria, the taste dysfunction was studied in 142 patients. The evidence of decreased taste sensation was found in 58 (40.8%) patients prior to surgery. Preoperatively, taste disturbance was found in 29 (37.2%) giant, 28 (45.9%) large and one (33.3%) medium-sized tumors, respectively. There were no significant age or sex-related differences. The postoperative taste disturbances were found in 65 (45.8%) patients. Among patients with anatomically preserved facial nerve, postoperative taste disturbances were found in 55 (42.3%) patients whereas nine (6.9%) patients reported improvement in taste sensations. CONCLUSIONS: Taste dysfunction is common following vestibular schwannoma surgery. Patient counseling prior to surgery is necessary to avoid any distress caused by taste dysfunction. Taste dysfunction should be included in the facial nerve functional grading system while assessing outcome.

Association of methylenetetrahydrofolate reductase genetic polymorphisms with atlantoaxial dislocation.

OBJECT: Genetic mechanisms of atlantoaxial dislocation (AAD) have not previously been elucidated. The authors studied association of polymorphisms in the methylenetetrahydrofolate reductase (MTHFR) gene, which encodes enzymes of the folate pathway (implicated in causation of neural tube defects [NTDs]), in patients with AAD. METHODS: Molecular analysis of MTHFR polymorphisms (677C-->T, cytosine to thymine and, 1298A-->C, adenine to cytosine, substitutions) was carried out using polymerase chain reaction and restriction enzyme digestion in 75 consecutive patients with AAD and in their reducible (nine patients, 12%) and irreducible (66 patients, 88%) subgroups. Controls were 60 age- and sex-matched patients of the same ethnicity. Comparisons of genotype and allele frequencies were performed using a chi-square test (with significance at p < 0.05). RESULTS: The CT genotype frequency of MTHFR 677C-->T polymorphism was significantly increased in the full group of patients with AAD (odds ratio [OR] 3.00, 95% confidence interval [CI] 1.28-7.14, p = 0.005) as well as in the irreducible subgroup (OR 2.81, 95% CI 1.17-6.86, p = 0.01). The frequency of T alleles was also higher in the AAD group (25.3%) than in controls (15%). The comparison of the combined frequency of CT and TT genotypes with the frequency of the CC genotype again showed significant association in AAD (OR 2.63, 95% CI 1.98-5.90, p = 0.009) and the irreducible (OR 2.5, 95% CI 1.1-5.74, p = 0.016) subgroup. There was, however, no significant association of MTHFR 1298A-->C polymorphism with AAD. CONCLUSIONS: Both MTHFR 677C-->T polymorphism and higher T allele frequency have significant associations with AAD, especially the irreducible variety. Perhaps adequate supplementation of periconceptional folic acid to circumvent effects of this missense mutation (as is done for prevention of NTDs) would reduce the incidence of AAD.

Occipitocervical contoured rod stabilization: does it still have a role amidst the modern stabilization techniques?

BACKGROUND: The occipitocervical contoured rod (CR) stabilization for use in craniovertebral junction (CVJ) pathologies is an effective and economical technique of posterior fusion (PF). AIMS: The various indications for CR in CVJ pathologies are discussed. SETTINGS AND DESIGN: Retrospective analysis. MATERIALS AND METHODS: Fifty-four patients (mean age: 31.02+/-13.44 years; male: female ratio=5.75:1) who underwent CR stabilization are included. The majority had congenital atlantoaxial dislocation (AAD; n=50); two had CVJ tuberculosis; one each had rheumatoid arthritis and C2-3 listhesis, respectively. The indications for CR fusion in congenital AAD were associated Chiari 1 malformation (C1M) (n=29); occipitalized C1 arch and/or malformed or deficient C1 or C2 posterior elements (n=9); hypermobile AAD (n=2); and, rotatory AAD (n=3). Contoured rod as a revision procedure was also performed in seven patients. Most patients were in poor grade (18 in Grade III [partial dependence for daily needs] and 15 in Grade IV [total dependence]); 15 patients were in Grade II [independent except for minor deficits] and six in Grade I [no weakness except hyperreflexia or neck pain]. RESULTS: Twenty-four patients improved, 18 stabilized and six deteriorated at a mean follow-up (FU) of 17.78+/-19.75 (2-84) months. Six patients were lost to FU. In 37 patients with a FU of at least three months, stability and bony union could be assessed. Thirty-one of them achieved a bony fusion/stable construct. CONCLUSIONS: Contoured rod is especially useful for PF in cases of congenital AAD with coexisting CIM, cervical scoliosis, sub-axial instability and/or asymmetrical facet joints. In acquired pathologies with three-column instability, inclusion of joints one level above the affected one by using CR, especially enhances stability.

Intraventricular meningiomas: a surgical challenge.

BACKGROUND: Primary intraventricular meningiomas (IVM) pose a surgical challenge as they often remain asymptomatic until they become very large; have close proximity to vital intraventricular structures; and, their vascular supply is often encountered only after significant tumor debulking. In this study, the surgical management of IVM is discussed. METHODS: Between 1989 and 2003, nine patients (7 with lateral and 2 with fourth ventricular) IVM, were operated upon. Raised intracranial pressure, gait ataxia, memory impairment, seizures (motor and sensory) were the main presentations. Four had hydrocephalus while five had either a sequestered temporal or occipital horn. All were large tumors with an average size more than 5.3 cm. The lateral ventricular IVM were accessed by the posterior middle temporal gyrus (n=5) or the superior parietal lobule (n=2) approach. A midline suboccipital craniectomy was used for the fourth ventricular IVM. RESULTS: Total excision was achieved in eight and partial excision in one. Patients with lateral ventricular tumors required a postoperative intraventricular drain for a minimum period of 48 hours. The main morbidity included visual field defects and transient hemiparesis, seizure and meningitis. The one patient with partial excision died 6 weeks later due to transtentorial herniation as a result of intratumoral bleed and lateral ventricular sequestration. CONCLUSIONS: An IVM, being initially asymptomatic, usually attains a large size before detection. During surgery, its devascularisation is usually achieved only after significant tumor debulking. Development of hydrocephalus or ventricular sequestration should be constantly monitored and may be avoided in the postoperative period by continuous external ventricular drainage.