Clinical and technical factors in endoscopic skull base surgery associated with reconstructive success.

BACKGROUND: In this study, we identified key discrete clinical and technical factors that may correlate with primary reconstructive success in endoscopic skull base surgery (ESBS). METHODS: ESBS cases with intraoperative cerebrospinal fluid (CSF) leaks at four tertiary academic rhinology programs were retrospectively reviewed. Logistic regression identified factors associated with surgical outcomes by defect subsite (anterior cranial fossa [ACF], suprasellar [SS], purely sellar, posterior cranial fossa [PCF]). RESULTS: Of 706 patients (50.4% female), 61.9% had pituitary adenomas, 73.4% had sellar or SS defects, and 20.5% had high-flow intraoperative CSF leaks. The postoperative CSF leak rate was 7.8%. Larger defect size predicted ACF postoperative leaks; use of rigid reconstruction and older age protected against sellar postoperative leaks; and use of dural sealants compared to fibrin glue protected against PCF postoperative leaks. SS postoperative leaks occurred less frequently with the use of dural onlay. Body-mass index, intraoperative CSF leak flow rate, and the use of lumbar drain were not significantly associated with postoperative CSF leak. Meningitis was associated with larger tumors in ACF defects, nondissolvable nasal packing in SS defects, and high-flow intraoperative leaks in PCF defects. Sinus infections were more common in sellar defects with synthetic grafts and nondissolvable nasal packing. CONCLUSIONS: Depending on defect subsite, reconstructive success following ESBS may be influenced by factors, such as age, defect size, and the use of rigid reconstruction, dural onlay, and tissue sealants.

Evolution of Radiographic Changes of a Vascularized Pedicled Nasoseptal Flap after Endonasal Endoscopic Skull Base Surgery.

BACKGROUND AND PURPOSE: There is active research involving the radiographic appearance of the skull base following reconstruction. The purpose of this study was to describe the radiographic appearance of the vascularized pedicle nasoseptal flap after endoscopic skull base surgery across time. MATERIALS AND METHODS: We performed chart and imaging review of all patients with intraoperative nasoseptal flap placement during endoscopic skull base surgery at a tertiary academic skull base surgery program between July 2018 and March 2021. All patients underwent immediate and delayed (>3 months) postoperative MR imaging. Primary outcome variables included flap and pedicle enhancement, flap thickness, and flap adherence to the skull base. RESULTS: Sixty-eight patients were included. Flap (P = .003) enhancement significantly increased with time. Mean nasoseptal flap thickness on immediate and delayed postoperative scans was 3.8 and 3.9 mm, respectively (P = .181). The nasoseptal flap adhered entirely to the skull base in 37 (54.4%) and 67 (98.5%) patients on immediate and delayed imaging, respectively (P < .001). CONCLUSIONS: Our findings demonstrate heterogeneity of the nasoseptal flap appearance after skull base reconstruction. While it is important for surgeons and radiologists to evaluate variations in flap appearance, the absence of enhancement and lack of adherence to the skull base on immediate postoperative imaging do not appear to predict reconstructive success and healing, with many flaps "self-adjusting" with time.

Design and Performance Characterization of a Novel, Smartphone-Based, Portable Digital Slit Lamp for Anterior Segment Screening Using Telemedicine.

Purpose: Telemedicine-enabled, portable digital slit lamps can help to decentralize screening to close-to-patient contexts. We report a novel design for a portable, digital slit lamp using a smartphone. It works on an advanced optical design and has the capability of instantaneous, objective photodocumentation to capture anterior segment images and is telemedicine-enabled. Methods: The device is constructed keeping its usability and the importance of design ergonomics for nonspecialized field personnel in mind. The optical design is described, and the resolution and magnification are compared with traditional desktop-based slit lamps. A Health Insurance Portability and Accountability Act (HIPAA)-compliant, patient management software is integrated to synchronize the captured images with a secure cloud server along with a sharpness algorithm to extract the best focused frames of the cornea, iris, and lens, from videos. We demonstrate its photodocumentation ability and teleophthalmology feasibility by capturing images in a pilot study from nine subjects. Results: Images were obtained in various illumination, magnification, and filter settings. Synchronous and asynchronous teleophthalmology consults were conducted. The performance of the device was shown to be limited by the smartphone sensor resolution and not the optical design, because the Air Force target resolution was found to be the same on smartphone-mounted traditional slit lamps despite a lower magnification. Conclusions: The novel, portable, digital slit lamp with advanced optical design using smartphones has the ability to screen for anterior segment pathologies using telemedicine. Translational Relevance: A portable, telemedicine-friendly, ergonomically designed, slit lamp used by nonspecialist personnel allows for both synchronous and asynchronous modes of consultation at remote locations, facilitating mass screening programs.

Do MRI features distinguish Wilson's disease from other early onset extrapyramidal disorders? An analysis of 100 cases.

Magnetic resonance imaging (MRI) is frequently used in the evaluation of various extrapyramidal disorders. Among the plethora of MRI features in Wilson's disease (WD), only "face of the giant panda" sign has been recognized to distinguish WD from other early onset extrapyramidal disorders (EOEPD). To ascertain the value of various MRI features in differentiating neuropsychiatric form of WD from other EOEPD. This retrospective analysis included 100 patients (M:F = 56:44) of EOEPD (5-40 years), who had undergone MRI during Jan'03 to Nov'08. Their clinical features were recorded and the following MR sequences were analyzed: T1WI, T2WI, FLAIR. Fifty-six patients had WD (M:F = 28:30, age at onset: 14 +/- 6.8 years) and 44 had other EOEPD (M:F = 27:17, age at onset: 19 +/- 9.8 years) that included Huntington's disease--4, young-onset Parkinson's disease--7, mitochondrial disorders--2, Hallervorden-Spatz disease--8, non-Wilsonian hepatolenticular degeneration--2, toxic/metabolic disorder--1, and others--20. The duration of illness at the time of MRI was comparable (WD: 3.1 +/- 4.9 years; Other EOEPD: 2.8 +/- 2.4 years). MR signal characteristics varied in topography and severity in both the groups. All the patients of WD had signal abnormalities in MRI, as against 16/44 of the other EOEPD group. The following MR observations were noted exclusively in WD: "Face of giant panda" sign (14.3%), tectal plate hyperintensity (75%), central pontine myelinolysis (CPM)-like abnormalities (62.5%), and concurrent signal changes in basal ganglia, thalamus, and brainstem (55.3%). Besides "Face of giant panda" sign, hyperintensities in tectal-plate and central pons (CPM-like), and simultaneous involvement of basal ganglia, thalamus, and brainstem are virtually pathognomonic of WD.

Wilson's disease: (31)P and (1)H MR spectroscopy and clinical correlation.

INTRODUCTION: Proton ((1)H) magnetic resonance spectroscopy (MRS) changes are noted in Wilson's disease (WD). However, there are no studies regarding membrane phospholipid abnormality using (31)P MRS in these patients. We aimed to analyze the striatal spectroscopic abnormalities using (31)P and (1)H MRS in WD. METHODS: Forty patients of WD (treated, 29; untreated,11) and 30 controls underwent routine MR image sequences and in vivo 2-D (31)P and (1)H MRS of basal ganglia using an image-selected technique on a 1.5-T MRI scanner. Statistical analysis was done using Student's t test. RESULTS: The mean durations of illness and treatment were 6.2 ± 7.4 and 4.8 ± 5.9 years, respectively. MRI images were abnormal in all the patients. (1)H MRS revealed statistically significant reduction of N-acetyl aspartate (NAA)/choline (Cho) and NAA/creatine ratios in striatum ((1)H MRS) of treated patients compared to controls. The mean values of phosphomonoesters (PME) (p < 0.0001), phosphodiesters (PDE) (p < 0.0001), and total phosphorus (TPh) (p < 0.0001) were elevated in patients compared to controls. Statistically significant elevated levels of ratio of PME/PDE (p = 0.05) observed in the striatum were noted in treated patients as compared to controls in the (31)P MRS study. The duration of illness correlated well with increased PME/PDE [p < 0.001], PME/TPh [p < 0.05], and PDE/TPh [p < 0.05] and decreased NAA/Cho [p < 0.05] ratios. There was correlation of MRI score and reduced NAA/Cho ratio with disease severity. The PME/PDE ratio (right) was elevated in the treated group [p < 0.001] compared to untreated group. CONCLUSIONS: There is reduced breakdown and/or increased synthesis of membrane phospholipids and increased neuronal damage in basal ganglia in patients with WD.

Cognitive profile and structural findings in Wilson's disease: a neuropsychological and MRI-based study.

BACKGROUND: Systematic studies on neuropsychological profile in patients with Wilson's disease (WD) are far and few. AIM: To examine the profile of cognitive deficits and their magnetic resonance imaging (MRI) findings in patients with WD. PATIENTS AND METHODS: Twelve confirmed patients of WD (age at onset and evaluation, 13.7±11.2 and 21.7±5.3 years, respectively; M-F ratio, 7:5) on de-coppering therapy constituted the study sample. Battery of neuropsychological tests measuring mental speed, motor speed, sustained attention, focused attention, verbal category fluency, verbal working memory, response inhibition, planning, concept formation, set-shifting ability, verbal and visual learning and memory were administered. Phenotypic details and observations on MRI of brain carried out within six months of neuropsychological assessment were documented. RESULTS: Neuropsychological assessment elicited cognitive deficits in multiple domains in all but one patient, who had normal MRI. Percentage of patients in the deficit range in various domains included: motor speed: 73%; verbal working memory, sustained and focused attention: 50%; verbal learning: 42%; visuo-constructive ability, verbal memory, mental speed: 33%-34%; verbal fluency, set-shifting ability, visual memory, verbal memory: 25%-27%; and verbal recognition: 17%. MRI was normal in three patients, and revealed variable abnormalities in the remaining: cerebral atrophy in 3; brainstem atrophy in 2; signal changes in basal ganglia in 9; and brainstem signal changes in 5. None had subcortical white matter changes. Two patients with normal MRI showed cognitive deficits. CONCLUSION: This study provides insight into the complex cognitive and brain changes observed on MRI in WD. Use of advanced MRI techniques in a larger cohort may improve understanding regarding functional and structural brain changes observed in similar disorders.

Intrathecal gadolinium-enhanced magnetic resonance cisternography in cerebrospinal fluid rhinorrhea: road ahead?

Accurate localization of cerebrospinal fluid (CSF) fistula in a patient with CSF rhinorrhea is challenging and often involves multiple imaging studies with associated expense and patient discomfort. Intrathecal contrast-enhanced computed tomography (CT) and unenhanced magnetic resonance (MR) cisternography using constructive interference in steady state (CISS-3D) sequences are currently being used in attempt to localize the leakage site but with varying degrees of success. Our purpose is to evaluate the utility of intrathecal gadolinium-enhanced MR cisternography (GdMRC) in patients with CSF rhinorrhea. Ten consecutive patients of CSF rhinorrhea (five spontaneous, four post-traumatic, and one post-operative) were evaluated with GdMRC. Nine of the patients underwent intrathecal contrast CT and CISS-3D examinations in addition. Each of studies was reviewed independently by three neuroradiologists blinded to results of other modalities. Surgery was planned after consensus and surgical correlation obtained in nine cases. The fistulous site was clearly demonstrated by using GdMRC in eight patients, CISS-3D in six, and intrathecal CT in three patients. The site of leakage was confirmed surgically in all the patients. One patient was found to be false negative both by intrathecal CT and GdMRC, and in one patient no fistulous site was demonstrated by any of the modalities and surgery was not offered. No adverse reaction was seen in any of the patients. GdMRC is a novel method of confirmation and localization of CSF fistula with potential for routine clinical application. Diagnosis and localization of fistulous site is better demonstrated due to its high-contrast resolution, absence of bony artifacts, and direct multi-planar imaging.

Prognostic factors in patients presenting with severe neurological forms of Wilson's disease.

BACKGROUND: Wilson's disease (WD), a metabolic disorder, is believed to be potentially reversible, even in its severe form. However, some patients do not respond to treatment. AIM: To analyse prognostic factors in severe WD. DESIGN: Retrospective audit. METHODS: A total of 140 patients were regularly followed from February 2002 to May 2004. Twenty-nine (18 males, 11 females) had severe disease, as defined by Modified Schwab and England Activities of Daily Living score (MSEADL) of < or=50% or Chu stage of 3. We analysed their clinical, laboratory and MRI features with respect to prognosis. RESULTS: For the severe form, mean age at symptom onset was 11.5 +/- 6.4 years, and at diagnosis, 13.3 +/- 7.0 years. Mean Neurological Symptom Score (NSS), Chu stage, and MSEADL were 26.5 +/- 8.2, 2.7 +/- 0.5 and 24.8 +/- 17.4, respectively. Twenty-one patients underwent MRI; 14 had repeat MRI. Following treatment, 14 (group A) had progressive worsening, including death in two, while 15 (group B) had sustained clinical improvement. Baseline demographic, clinical and laboratory features and MRI scores did not significantly differ between the two groups. However, diffuse white-matter abnormalities were more extensive in group A. Full-dose initial penicillamine therapy could have contributed to worsening in four patients. Drug compliance was poor in both groups but resumption of treatment did not benefit patients in group A. Serial MRI showed regression of lesions only among patients with clinical improvement. DISCUSSION: Severe WD remains a therapeutic challenge, with early diagnosis and treatment are essential. Specific MRI observations, a 'start low-go slow' regimen for penicillamine, and compliance may have prognostic significance. In absence of clinical predictors, genetic attributes need to be explored.

Spinal intramedullary cysticercosis.

OBJECTIVE: Spinal intramedullary cysticercosis is an uncommon manifestation of neurocysticercosis. We review our experience with eight cases of intramedullary cysticercosis. METHODS: Eight patients who were surgically treated for spinal intramedullary cysticercosis between 1982 and 1991 were retrospectively reviewed, and the final outcomes were assessed. RESULTS: In six patients, the cysticercosis involved the thoracic cord, whereas in the other two, the cysticercosis was cervical in location. Only one patient had multiple soft tissue calcifications, as revealed by plain radiography. Myelography indicated an intramedullary lesion in each of seven patients; two of the seven patients had partial myelographic block, suggesting the segmental nature of the lesion. Cerebrospinal fluid studies were noncontributory. One patient had three cysts, whereas the other seven had one cyst each. Four patients had adjacent soft purulent materials, which were revealed by histopathological examination to be granulation tissue. The neurological statuses of seven patients improved after surgery. Six patients were followed up for a mean of 30.6 months (3 mo-5 yr). Three could resume their previous occupations, two others could manage their daily activities, and one required only minimal assistance for daily activities. CONCLUSION: The outcome of intramedullary cysticercosis is not as dismal as reported earlier, and patients with paraplegia also have favorable outcomes. A preoperative diagnosis of cysticercosis can be suspected in an endemic area in the presence of multiple soft tissue calcifications and segmental lesions revealed by myelography or magnetic resonance imaging studies.

Extradural haematoma complicating lumbar puncture following a craniotomy. A case report.

BACKGROUND: This case report illustrates the development of an intracranial extradural haematoma (EDH) as an uncommon complication of a lumbar puncture. CLINICAL PRESENTATION: A 10-year-old girl operated for intra-third ventricular cysticercosis developed postlumbar puncture headache after a drainage lumbar puncture (LP) on the 7th postoperative day. CT scan revealed a right frontal EDH away from the operative site. INTERVENTION: The child was managed conservatively in view of her preserved sensorium. RESULTS: The child had an uneventful recovery. Follow-up CT scans showed resolution of the haematoma. CONCLUSIONS: LP, though considered to be a safe procedure, may rarely be associated with a potentially lethal intracranial haematoma, which can be managed successfully if diagnosed at an early stage.

Ossification of the posterior longitudinal ligament of the cervical spine in Asian Indians--a multiracial comparison.

Ossification of the Posterior longitudinal ligament (OPLL) has been thought to be a predominantly Japanese disease with few reports among the non-orientals and Caucasians. A prospective clinicoradiological study of 47 symptomatic Caucasoid Indians is reported. Twenty-seven patients presented with myelopathy, twelve with myeloradiculopathy and six with radiculopathy. 'Total' configuration of OPLL was the most frequent (70%). The most frequent location was at C3 and C4 levels. The thickness of OPLL ranged from 2.5 mm to 11.5 mm. Canal stenosis was most severe in patients with 'total' type of OPLL. Ossification of other spinal ligaments were seen in 50% of the patients. Twenty-one patients underwent decompressive laminectomy and twenty of them improved. The difference in the cervical canal diameters and the configuration of OPLL between the Japanese and our population (Caucasoid Indians) may be of therapeutic relevance.

Role of stereotactic aspiration in the management of tuberculous brain abscess.

BACKGROUND: Intracranial tuberculous abscesses are uncommon clinical entities, even in countries where tuberculosis is endemic. Surgical excision and antituberculous treatment is the treatment of choice. The role of stereotactic aspiration in the management of these lesions is highlighted in this communication. METHODS: Three patients, all receiving treatment for pulmonary tuberculosis, presented with symptoms of raised intracranial pressure and neurological deficits. Computed tomography (CT) scans revealed cystic lesions with enhancing rim in the thalamus in two patients and multiple coalescing cystic lesions in the deep temporal region in another. RESULTS: Stereotactic aspiration of the cyst and biopsy of the cyst wall were performed in all. In two, the pus revealed acid fast bacilli (AFB). In another, though the pus did not reveal any AFB, the wall showed tuberculous granuloma. Antituberculous treatment was continued in all the patients. Follow-up CT revealed resolution of the lesions in all patients. CONCLUSION: Stereotactic aspiration is an useful alternative modality of management of tuberculous abscesses in a selected group of patients.

Diagnostic efficacy of stereotactic biopsies in intracranial tuberculomas.

BACKGROUND: Stereotactic biopsies for diagnosing intracranial tuberculomas are often reported to be inconclusive, being confirmatory in only 28% of cases. We analyzed the role and diagnostic efficacy of stereotactic biopsies in the management of intracranial tuberculomas. METHODS: Twenty patients diagnosed with intracranial tuberculomas based on neuroimaging procedures underwent computerized tomography (CT) guided stereotactic biopsies for histological confirmation. In 10 patients (Group A), biopsies were obtained from the center of the lesion; in the other 10 (Group B), biopsies were obtained from both the enhancing rim and the isodense center and examined separately. The tissues were processed for paraffin sections and hematoxylin eosin staining. In addition, in the first five patients a squash smear from a small portion of the biopsy was prepared, and the cytological features of the cells were examined. All the patients received antitubercular medication and CT scans were repeated 4 months posttreatment. RESULTS: Histopathological features of the paraffin sections in 17 patients (Group A: 8, Group B: 9) were diagnostic of tuberculoma, whereas the other three revealed only chronic nonspecific inflammation. Based on the cytological features in squash smears, diagnosis could be made in one of five. In two, although the smear was not diagnostic, the histological section from the same specimen confirmed the tuberculous pathology. There was no procedural morbidity or mortality. One patient had a small asymptomatic hematoma at the biopsy site. Three patients died during the follow-up period, due to secondary complications of tuberculosis. Repeat CT scans during follow-up after antituberculous treatment confirmed the reduction in the size of the lesion in 15/17 patients. CONCLUSION: The high positive yield of stereotactic biopsies in diagnosing tuberculomas argues for their inclusion in the management protocol for tuberculous mass lesions.

Posterior circulation abnormalities in moyamoya disease : a radiological study.

Moyamoya disease (MMD) is an uncommon entity outside Japan. Though the clinical and radiological features are well described, involvement of the posterior circulation has not been highlighted. Out of 10 patients of MMD studied, the posterior circulation was involved in 9 (3 bilateral, 6 unilateral). The P1 segment was most commonly affected. Interestingly, no infarcts were seen in the territory of the posterior circulation in any patient. Five patients showed recent haemorrhages on scan. It was thalamic haemorrhage in four and subarachnoid in one patient. The posterior circulation is frequently involved in MMD as evident on angiography. However, ischaemic events of the posterior circulation are not frequent, as the posterior circulation acts as collateral pathway for the diseased anterior circulation till later stages of the disease.

Laminoplasty: an evaluation of 24 cases.

Cervical expansive laminoplasty has been advocated as an alternative procedure to laminectomy for the decompression of the cervical spine. It provides favourable cord decompression and stabilisation of the cervical spine and is a simpler and safer alternative to anterior fusion and laminectomy for myelopathy and myeloradiculopathy, due to multisegmental cervical spondylosis and ossified posterior longitudinal ligament. We report our experience in 24 patients with this procedure, 12 of whom had myelopathy and another 12 had myeloradiculopathy. The earliest symptom to improve was radicular pain or paraesthesia (75%). A reduction in spasticity was seen in 21 of the 24 patients (87.5%). Eleven patients had improvement in their motor power during a follow up period ranging from 1 month to 14 months. One patient deteriorated following the procedure and developed Brown Sequard features due to under riding of the lamina on the hinged side, another had severe post operative paraesthesias, while one patient had a CT scan evidence of 'closing of the door', without being symptomatic for it. The technique of the procedure is discussed and the pertinent literature reviewed.

Open and minimally open lips schizencephaly.

Two patients with isolated schizencephaly, a very rare congenital anomaly of the brain, who presented with epilepsy are presented. According to imaging morphology, there are two types of schizencephaly, 'open lip' and 'minimally open lip'. These two cases emphasize that while MRI is superior to CT in the diagnosis of congenital brain anomalies, schizencephaly can be diagnosed by its characteristic CT features.

Tuberculous radiculomyelitis : a myelographic and cmputed tomographic study.

Tuberculous spinal arachnoditis is one of the commonest causes of paraplegia in India. Clinical and laboratory diagnosis is difficult and radiological evaluation often remains an important component of diagnosis and management The role of water soluble contrast medium myelography and CT features have been described. The most common features observed on myelography include long lengths of irregular contrast medium, nodular filling defects, thickened nerve roots and empty thecal sac. The CT features include irregular subarachnoid contrast medium, extradural and intradural filling defects with the spinal cord of variable dimension. Adherence of the nerve roots to one another and to the meninges was not an uncommon finding. Given the clinical setting,myelographic and CT myelographic features are reasonably characteristic to suggest a diagnosis of tuberculous spinal arachnoiditis.

MRI in subacute combined degeneration of spinal cord: a case report and review of literature.

A 56 year old man presented with acute onset posterior column and lateral spinothalamic tract dysfunction over a period of 15 days. MRI showed diffuse hyperintensity on T2WI involving the posterior columns. A diagnosis of subacute combined degeneration (SCD) of the spinal cord was considered and confirmed by laboratory findings. The patient showed complete recovery on B12 therapy. MRI lesion also compeletely resolved.