Suyash Singh

Spinal Dysraphism: Trends in Northern India

Objective: To evaluate the clinical profiles, image findings and surgical outcome of 155 cases of spinal dysraphism. Methods: 155 patients were studied prospectively (143) or retrospectively (12). The male to female ratio was 1.5:1. Mean age at presentation was 5.7 years. Out of 155 cases of spinal dysraphism, 119 had open spina bifida [meningomyelocele (MMC) in 113 (72%), meningocele in 3 (2%) and myelocystocele in 3 (2%)] and 36 had occult spina bifida [split cord malformation (SCM) without overt MMC sac (pure SCM) in 29 (19%) and midline dermal sinus in 7 (4.5%)]. Lipomeningomyelocele constituted 73 of the 113 cases of MMC (65%). Twenty cases of MMC (18%) had associated SCM (complex spina bifida). The total number of cases with SCM was 49 (32%). Twenty-four children with MMC presented with an operative scar from previous surgery, performed at the periphery. After a detailed clinical evaluation of all patients, craniospinal MRI was the preferable mode of investigation. All patients underwent surgery and were clinically assessed over a mean follow-up period of 3.6 years. Results: MMC sac was the commonest skin manifestation seen in 89/155 cases (57%). Hypertrichosis and previous operative scar were noticed in 24 cases each (15%). Cutaneous hemangioma, skin tag and multiple neurofibroma were the other common superficial skin manifestations. Clinically, 103 patients (66.5%) had variable weakness of the lower limbs, and muscle atrophy was noticed in 56 cases (41%). Graded sensory loss, sphincteric dysfunction, trophic ulcer and backache were present in 89 (57%), 64 (36%), 17 (11%) and 9 cases (6%), respectively. Significant scoliosis in 56 cases (36%) and congenital talipes equinovarus in 51 cases (33%) were the most frequent neuroorthopedic deformities apart from high-arched foot, leg length discrepancy and flat foot. Common image findings were low-lying cord in 101 (65%), neural placode in 76/113 (49%), hydrosyrinx in 42 (27%), hydrocephalus in 71 (46%) and Chiari malformation in 62 cases (45%). The lumbosacral region in 56 cases (38%), followed by the lumbar region in 47 (30%), were the most common sites of occurrence of spina bifida. Of 71 hydrocephalic patients, 39 (55%) needed shunting before definitive surgery and 21 (30%) after the surgery. Eleven patients (15%) did not require shunting at all. CSF leak in 51 patients (33%), pseudomeningocele in 26 (17%), wound infection in 22 (14%) and meningitis in 13 (8%) were the most common postoperative complications. Two patients died in the postoperative period. During an average follow-up of 3.6 years (range 1.5–8 years), motor weakness improved in 47 children (45%) and remained static in 53 (52%), and 3 children showed deterioration in motor power. Sensory dysfunction improved in 43 (48%), remained static in 42 (47%) and deteriorated in 4 patients (4%). Sphincteric function clinically improved in 26 patients (41%) and was static in 38 (59%), and 6 patients (4%) had deterioration. Backache was dramatically relieved in all 9 patients with this complaint (100%). In 13 children with trophic ulcer (76%), it completely healed. Conclusion: A significant proportion of patients with open spinal dysraphism harbor an underlying SCM, an entity coined complex spina bifida (in our previous study). The incidence of hydrocephalus in spinal dysraphism is low in India in comparison to the Western world. Some patients with mild hydrocephalus on scan may not require a shunt operation. Surgical intervention should be early after entire neural axis screening by MRI.

Comparative Study of Complex Spina Bifida and Split Cord Malformation

Objective: To see the difference in clinical profiles, radiological findings and surgical outcome of the group 1 split cord malformation and meningomyelocele (SCM with MMC) from group 2 (SCM without MMC).Methods: 46 patients of SCM were selected from a total of 138 cases of spinal dysraphism. They were divided into two groups, based on presence or absence of MMC. Group I (SCM with MMC) n=19 patients and Group II (SCM without MMC) n=27 patients. A detail clinical evaluation and MR screening of whole spine of all cases was performed. All patients underwent surgical detethering of cord. After an average follow-up of 1.7 years, the operative results were clinically assessed and statistical significance was calculated.Results: Male to female ratio was 1:09. Mean age of presentation was 3.6 years. Cutaneous markers like tuft of hair, cutaneous haemangioma, etc, had a higher incidence in group II in comparison to group I (50% vs 10.5%). The incidence of motor deficits was significant in group I in comparison to group II (63% vs 40%). The incidences of sensory loss, trophic ulcers, sphincteric dysfunction and muscle atrophy were relatively more common in group I patients, while neuro-orthopedic deformities such as congenital telepes equinovarus (CTEV), scoliosis and limb shortening were more frequent (67%) in group II children as compared to group I (53%). Type I SCM has higher incidence in group I children. Low lying conus were found in 47% patient of group I, while in group II it was noticed in 69%. The associated cranial anomalies like hydrocephalus, ACM and syrinx, were slightly higher in group I patients. At surgery, dysgenetic nerve roots, neural placode, arachnoid bands and atrophic cord were seen mainly in group I. Postoperative complications like, CSF leak, pseudomeningocele and meningitis were more commonly encountered in group I patients. The patients of group II showed better operative outcome compared to group I cases.Conclusion: Incidence of SCM with MMC amount to 41% of total SCM cases. Progressive neurological deficit was higher in this group (SCM with MMC) in comparison to the group harboring SCM without MMC. In view of a significant association of SCM in MMC cases, associated with other craniospinal anomalies, a thorough screening of neuraxis (by MRI) is recommended to treat all treatable anomalies simultaneously for desired outcome.

Spinal intradural hydatid cyst causing arachnoiditis: A rare etiology of cauda equina syndrome

This study aims to focus on a rare presentation of spinal hydatid cyst as cauda equine syndrome and misdiagnosed as intradural extramedullary (IDEM) benign lesion on magnetic resonance imaging. In this article, we report a case of spinal hydatid cyst masquerading as IDEM tumor, and intraoperatively, we accidently find clumped granuloma with severe arachnoiditis and hydatid cyst in lumber region, which was present as bilateral S1 radiculopathy with cauda equina syndrome. An 11 year old boy who presented with symptoms and signs of cauda equina syndrome and planned for surgical excision. His radiological impression was IDEM possibly neurofibroma. To our surprise, we found multiple intradural cystic lesions with arachnoiditis. Dissecting in plane cyst was flushed out, and surgical cavity was irrigated with 3% saline. Postoperatively histopathology and serum tests confirmed the diagnosis of hydatid cyst. Hydatid disease is rare cause of cauda equine syndrome which can be miss diagnosed on radiological investigations. A high index of suspicion should be kept especially in a young patient from the Indian subcontinent.

Hyperostotic paraganglioma of occipitotemporal bone

A 25-year-old male presented with off-and-on vertigo of 10-year duration. He had left-sided cerebellar signs, left vocal cord paresis, and minimal left-sided hearing impairment. Computed tomography of the head revealed significant hyperostosis of squamous occipital, mastoid, and petrous temporal bone with no adjacent soft tissue mass. On excision, it turned out to be paraganglioma. Paraganglioma as a pure bony mass is not reported in the literature. The site of origin of such a tumor could not be ascertained, even on generous screening. The tumor remained nonsecretory on clinical and biochemical investigations.

Exophytic cerebral hemispheric low grade glioma: An unusual growth pattern of a common central nervous system tumour

BACKGROUND Exophytic growth (EG), wherein the tumor mass grows beyond the neuraxial boundary formed by pia mater, remains a rare pattern of glioma growth. It has been described in gliomas at several locations like the brainstem, cerebellum, suprasellar area, spinal cord, and insula. However, EG in hemispheric grade 2 astrocytomas, particularly affecting the convexity subarachnoid space, as against the more spacious basal subarachnoid space, is exceedingly rare. To the best of our knowledge, there is only 1 such case reported in the English literature. Due to the extreme rarity, little is known about these tumors, particularly the mechanisms underlying the EG and their possible clinical implications. CASE DESCRIPTION A 32-year-old woman presented with partial motor seizures without any neurologic signs. On magnetic resonance imaging of the head, a nonenhancing, T2-hyperintense, right frontal lobe lesion was noted. The majority of the lesion was occupying the adjacent subdural space while still remaining in continuity with the intra-axial globular component. During surgery, the exophytic nature of the lesion was confirmed. We performed maximal resection of the exophytic portion with subtotal excision of the intra-axial component. She recovered uneventfully after surgery and subsequently received external beam radiotherapy for the residual tumor. CONCLUSION Although extremely rare, cerebral hemispheric grade 2 astrocytomas may have an EG pattern. The exact mechanisms underlying this are not clearly known. More such cases need to be reported so that we can decipher the true nature of these tumors.

Technical pearls and surgical outcome of early transitional period experience in minimally invasive lumbar discectomy: A prospective study

Background: There is growing indications of minimally invasive spine surgery. The inherent attitude and institutive learning curve limit transition from standard open surgery to minimally invasive surgery demanding understanding of new instruments and correlative anatomy. Materials and Methods: In this prospective study, 80 patients operated for lumbar disc prolapse were included in the study (between January 2016 and March 2018). Fifty patients (Group A) operated by various minimally invasive spine surgery (MISS) techniques for herniated disc disease were compared with randomly selected 30 patients (Group B) operated between the same time interval by standard open approach. Surgical outcome with Oswestry Disability Index (ODI) and patient satisfaction score was calculated in pre- and postoperative periods. Results: Mean preoperative ODI score in Group A was 31.52 ± 7.5 standard deviation (SD) (range: 6–46; interquartile range [IQR]: 8; median: 32.11) and postoperative ODI score was 9.20 ± 87.8 SD (range: 0–38; IQR: 11; median: 6.67). Mean preoperative ODI score in Group B was 26.47 ± 4.9 SD (range: 18–38; IQR: 4; median: 25) and postoperative ODI score was 12.27 ± 8.4 SD (range: 3–34; IQR: 12; median: 10.0). None of the patients was unsatisfied in either group. On comparing the patient satisfaction score among two groups, no significant difference (P = 0.27) was found. Discussion: On comparing the change in ODI and preoperative ODI among both groups, we found a significant difference between the groups. It is worth shifting from open to MISS accepting small learning curve. The satisfaction score of MISS in early transition period is similar to open procedure. Conclusion: The MISS is safe and effective procedure even in transition period for the central and paracentral prolapsed lumbar intervertebral disc treatment. The results are comparable, and patient satisfaction and symptomatic relief are not compromised.

Ischemic stroke associated with ankylosing spondylitis: an integral part of disease spectrum, or a natural consequence of progressive infirmity?

Ankylosing spondylitis (AS), a chronic rheumatic disease with a prevalence ranging from 0.2 to 1.1% [1, 2], is a manifestation of autoimmunity against the axial skeleton (often leading to spinal immobility) that also affects peripheral joints and extra-articular structures (causing uveitis, inflammatory bowel disease (IBD), psoriasis, and enthesitis). Chronic inflammation, bone destruction, and aberrant bone repair may cause progressive disability. The 3E Initiative in Rheumatology has stated that AS is likely to be present, if along with chronic back pain of at least 3-month duration, the presence of several of the following features are present: inflammatory back pain, alternating buttock pain, response to non-steroidal anti-inflammatory drugs (NSAIDs), onset of symptoms before the age of 45 years, the presence of peripheral disease manifestations (arthritis, dactylitis, or enthesitis), the presence of confirmed acute anterior uveitis, a positive family history of the disease, the presence of HLA-B27 positivity in the patient, and the diagnosis of sacroiliitis/ spondylitis by imaging [1]. Reports exist regarding the association of AS with an increased risk of ischemic heart disease [3, 4], but whether or not this association also translates into a higher incidence of cerebrovascular stroke remains uncertain [5–7]. In a study based inWales, UK, published in 2012, comparing 1686AS patients with 1,206,621 control subjects, there was no increase in the incidence of cerebrovascular disease/stroke rates in patients with AS compared to those without AS, despite there being higher rates of hypertension prevalent in patients with AS [8]. On the other hand, in a study from Sweden, patients with AS (n = 5358), rheumatoid arthritis (n = 37,245), and those with matched general population subjects (n = 25,006) were compared. The study revealed that patients with AS are at a 30–50% increased risk of incident cardiovascular events [9]. A retrospective cohort study from Quebec, Canada, that included 8616 individuals with AS compared with 50,699 individuals in the general population also revealed that patients with AS are at an increased risk for cardiovascular and cerebrovascular diseases. The risk is much higher in younger patients [2]. Another study from Taiwan, using the database obtained from the complete National Health Insurance (NHI) claim database, showed a 1.9-fold increased risk of developing an ischemic stroke in young patients with AS [10]. The increased propensity of patients with AS to develop a stroke has been attributable to several etiopathological factors. Yet, none of them have an unequivocally proven role in the causation of stroke. The Trial of Org 10172 in Acute Stroke Treatment (TOAST) classification denotes five subtypes of ischemic stroke: (1) large artery atherosclerosis, (2) cardioembolism, (3) small vessel occlusion, (4) stroke of other determined etiology, and (5) stroke of undetermined etiology. All five subtypes of ischemic stroke may occur in patients with AS [11]. Large artery atherosclerosis may be causally related to inflammation that forms an integral component of the pathogenesis of AS. Increased levels of inflammatory markers, such as interleukin-6, C-reactive protein, and tumor necrosis factor alpha, increase atherogenesis and may lead to intimal and media thickening of largeand medium-sized blood vessels [10, 12]. Rheumatological states, such as AS, may cause endothelial injury by immune complex formation and complement activation. The disproportionate frequency of methylenetetrahydrofolate reductase (C677T) gene polymorphism in patients with AS may also be a potential etiopathological factor for the development of stroke in AS. Cardioembolic stroke may be the consequence of cardiomyopathy, valvular heart disease, or aortic insufficiency, which constitute the extra-articular manifestations of AS. Small vessel occlusion may be the result of end artery embolic phenomenon or may be the consequence of inflammation and vasculitis associated with AS [10]. Stroke * Sanjay Behari sbehari27@yahoo.com

Split cord malformation

In 1992 Pang revolutionalized the concept and nomenclature of split cord malformation. His unified theory of embryogenesis unravelled the long-lasting conundrums in understanding why some septum is bony and other fibrous. After this concept, words like “diplomyelia” and “diastematomyelia” became history. According to Pang’s unified theory, persistent accessory neuroenteric canal leads to formation of septum. Actually, it was Bremer et al. [1] who first proposed the term accessory neuroenteric canal. He said that the persistent adhesion of the ectoderm-endoderm and cleft notochord favours the formation of neuroenteric canal. Later on, Pang added concept that the failure of “anlagen” to integrate in midline and formations of endomesenchymal tract contribute to septum in split cord malformation. Further, incorporation of “menix primitiva” into the tract leads to bony septum and non-incorporation leads to fibrous septum.

Is it the monster “teratoma” or simply meningomyelocele: Our experience of “histological surprise”

Teratomas are one of the most common tumors in newborn with excellent prognosis arises from totipotent primordial germ cells harboring two or three germ cell layers. The tumor has been titled “Great masquerade.” The teratomas of sacrococcygeal region present with lower limb weakness, urinary or bowel obstruction, and swelling at lower back or intrauterine mass in ultrasound or complicated delivery. A 2-month-old male child presented with complaints of swelling over lumbosacral region with discharging punctum since birth. Sagittal T2-weighted magnetic resonance imaging (MRI) of the spine showed myelocele at L5 level forming placode with central defect at L4-S1 and low-lying tethered cord up to L4–L5. The patient was operated, and histopathology surprisingly came to be mature teratoma. We followed the patient with serum beta human chorionic gonadotropin and alpha-fetoprotein markers and MRI. Literature supports complete surgical removal, including coccyx and tumor base. Mature teratoma is considered as benign disease thus even subtotal excision is appropriate but with aggressive follow-up. The difference in recurrence following total compared to subtotal resection is considered insignificant. In this article, we have discussed the management of teratoma in detail. Teratoma with meningomyelocele is a rare entity. There is still dilemma in managing cases and prognosticating parents in such patients. The provisional diagnosis of teratoma should also be considered when child presents as midline sacrococcygeal mass.

Endoscopic repair of CSF rhinorrhea through persistent Sternberg’s canal in an elderly patient with ankylosing spondylitis

Received: November 10, 2016; Accepted: November 16, 2016; Published: November 18, 2016 A 69-year-old gentleman with ankylosing spondylitis presented with spontaneous clear watery discharge from his right nostril for 2 months without anosmia. There was no history of preceding trauma or features suggesting benign intracranial hypertension. Neurological examination was essentially normal. Considering a diagnosis of spontaneous CSF rhinorrhea, we ordered plain computed tomography of the head including a CT cisternogram. A bony defect in the lateral wall of the sphenoid sinus was unravelled. Endoscopic endonasal transethmoidal trans-sphenoidal approach was taken striking a balance of optimum surgical position and the fixed neck and trunk deformity of the patient. The defect was repaired with fat and fascia lata graft harvested from the thigh. CSF leak completely subsided after the repair. The patient recovered uneventfully after surgery and is doing well at follow-up.