indicative of spinaldysraphism are dermal changes such as areas with marked hirsutism or pigmentation on and surrounding the area and level of the spinaldysraphism, however, they are not as common in adults as they are in children, accounting for 35.6% in adult cases [1,5,10]. Based on these findings and the mild pain which improved with analgesia, either conservative treatment was determined in combination with clinical symptoms or further diagnostic measure such as Somatosensory evoked potentials was Ordered and the result was. Presenting complaints of TCS are non-specific, encompassing lower back pain, sensory deficits, motor weakness, and sphincter incompetence [1,3,5-8]. Adult onset TCS is usually precipitated by physical exertion, such as back flexion, washing dishes, or receiving trauma to the back [9,10]. Furthermore, spine abnormalities such as degenerative spinal stenosis are an aggravating factor for the emergence of TCS symptoms . Suspicion of TCS as a differential diagnosis is low, due to the subtle and non-specific presenting complaint, insidious and chronic onset, and deficits not respecting the dermatomoal and myotomal distribution pattern; hence they are initially diagnosed as one of the common orthopedic, neurological, or genitourinary complaint such as lumbosacral degenerative disc herniation . The initial complaint is usually lower back pain, which radiated to the lower limbs in a non-dermatomal pattern chronically; radicular pain distribution is
Aims: After initial primary repair by inexperienced hands for the spectrum of pathological conditions in spinaldysraphism (SD), a few percentage of patients present with recurrent symptoms and worsening neurological status especially when primarily pathology is not identified and dealt properly. When the primary intradural tethering element is left untouched, worsening of symptoms is common. In this retrospective study, we tried to analyze the symptomatology, functional outcome at 1–2 months after the second surgery and associated complications. Subjects and Methods: All patients underwent second surgery at author’s institution. Pre and post-operative data were evaluated using Necker –Enfants Malades (NEM) neurological and modified Hoffer ambulatory scale. Results: The main presenting complaints were bladder incontinence and limb weakness. Preoperative mean scores for motor and bladder were 3.56 and 2.78 out of 5, 2.67 out of 4, and 2.11 out of 3 for bowel and sensory function, respectively. Postoperative mean score for motor, sensory, bladder, and bowel function revealed good neurological improvement. Statistically neurological improvement in bladder and bowel function was significant. More than 60% of patients had normal ambulation at follow-up. Conclusions: Patients presenting with recurrent symptoms in an operated case of SD need to be investigated, cause of recurrence has to be identified, and if needed repeat surgery is recommended at the earliest. Long-standing neurological deficits can potentially improve, especially bladder and bowel function which gives a good quality of life to the patients. Furthermore, we want to stress the fact that since it is an intradural pathology, these cases should be operated by experienced neurosurgeons, and this fact should be made aware among referring doctors.
Approximately 50-70% of patients with spinaldysraphism have Orthopedic symptoms and signs are usually progressive deformities. They may be asymptomatic to start and later affect the lower limbs and spine as the child begins to grow. Lower limb deformities include pes cavus, .varus, .valgus and equinus deformities, tight Achilles tendons, clubbing, toe clawing, hammertoe, talipes and leg length discrepancy. Spinal anomalies include scoliosis, sacral dysgenesis segmental spinal dysgenesis. The complications arising out of orthopaedic lesions are low bone density, respiratory dysfunction, immobility, poor quality of life, spasticity. Sometimes the orthopaedic problems are managed surgically without suspecting the underlying neurological problem. If a patient has combination of orthopaedic and anorectal malformation, an ultrasound of the spine followed by confirmation with MRI is important.
searched our data base for all examinations performed in the set- ting of spinaldysraphism assessment, as well as for appropriate gestational age–matched controls. The image quality had to be good, consisting of at least 3 orthogonal T2-weighted HASTE se- ries of the fetal brain with limited fetal motion, allowing adequate performance of the outcome measurements. The primary selec- tion and review of images was performed by a single pediatric radiologist (M.A.) with ⬎ 3 years of experience in fetal MR imag- ing. The main exclusion criteria were twin pregnancy, syndromal pathology, fetal hydrops, or anhydramnios. The number of pa- tients and individual reasons for exclusion are shown in On-line Table 2. This exclusion left data from 349 MR imaging examina- tions of a total of 1006, including 274 examinations in 246 control fetuses. These data illustrate that some fetuses were scanned more than once. Additionally, we included 52 MR imaging examina- tions in fetuses with OSD, of whom 23 had a repeat MR imaging examination after the operation. The eligibility criteria for fetal surgery were those used in the MOMS trial. 12
Cases of spinaldysraphism are rare, even in newborns with cutaneous stigmas. In three prospective studies in the literature, the highest incidence reported, in an evaluated population of 2,010 patients, was 7.2%, that is, 144 newborns who presented cutaneous stigmas. Of these, only 5.5% (8) were diagnosed with occult spinaldysraphism . Some stig- mas have been proven to present greater risk of occult spinaldysraphism, such as deep or atypical sacrococcygeal dimples, hemangiomas, cutaneous aplasia, subcutaneous masses, and exophytic skin lesions such as tails and hair tufts. Cases of multiple stigmas comprise another group at risk . In our case, the neonate presented exophytic skin lesions in the lumbar region and hands (postaxial polydactyly).
Isoflurane had always been known to have neuroprotective properties. Sevoflurane by virtue of its low solubility provides rapid onset and offset of an aesthesia, thus facilitating early postoperative evaluation of the patients especially in the neurosurgical procedures. However superior role of sevoflurane to isoflurane in pediatric patients requiring intermediate lasting anaesthesia remains controversial. Therefore the aim of this prospective comparative randomized study was to sevoflurane and isoflurane in paediatric patients undergoing surgery for spinaldysraphism on the basis of early post recovery outcomes, pain, intraoperative and postoperative hemodynamics, and postoperative side effects of both the agents. Materials and Methods: 60 patients scheduled for spinaldysraphism surgery of ASA physical status I & II, of either sexes, aged 6 years, were divided into two groups. After I.V. induction maintenance gent as isoflurane for Group I (n=30), and sevoflurane for Group II (n=30). The primary end points being post-anaestheisa recovery, extubation time, discharge readiness in PACU and emergence agitation, While the secondary end points included and postoperative Heat Rate, noninvasive blood pressure and SpO 2 and postoperative
We constructed a decision-analytic Markov model to compare the costs and health outcomes of 4 diagnostic strategies in children who are suspected of having occult spinaldysraphism: 1) MRI, 2) plain radiographs followed by MRI for positive results (radio- graphs), 3) ultrasound (US) followed by MRI for positive results (US), and 4) no imaging with close clinical follow-up. We analyzed 3 risk groups based on the prevalence (pretest probability) of disease: 1) low (intergluteal dimple and infants of diabetic moth- ers; prevalence: 0.3%), 2) intermediate (low and intermediate ano- rectal malformation; prevalence: 27%–33%), and 3) high risk (high anorectal and cloacal malformation; prevalence: 44%– 46%). Figure 1 shows a simplified representation of the decision tree.
Dr. Ram Manohar Lohia Hospital, New Delhi from 1st November 2012 to 31st March 2014. Approval from hospital and Institutional Ethical Committee was obtained prior to initiation of the study. Patients who were diagnosed or provisionally diagnosed as cases of spinaldysraphism, irrespective of age and sex, based on the Clinical profile and imaging profile as preliminary findings on radiographs/Ultrasonography and incidentally detected cases on either Radiographs, CT, USG or MRI were included in our study. A written informed consent was taken from all patients. A detailed history was taken with complete physical and systemic examination of the patient. Relevant biochemical investigations were done wherever required.
Spina bifida aperta is most common type of spinaldysraphism representing a serious congenital anomaly with severe Neurologic, Musculoskeletal, Genitourinary, and Bowel anomalies. It encompasses three forms namely Myelomeningocele, Myelocele and rarely Meningocele. Females show a higher incidence than Males and most of them present at birth and are immediately taken for surgical repair and hence are rarely imaged in unoperated cases.
Occult spinaldysraphism should be suspected in case of poor PEG responder constipation, even when evident sa- cral abnormalities on the physical examination are not detected. In this case, instrumental tests (i.e. abdominal x-ray/MRI) should be performed. Particularly abdominal x-ray is a first level test, cost-effective and easy to per- form which can give useful information about rachis morphology.
90 patients aged >65 years undergoing surgery for hip fracture under hyperbaric tetra caine spinal anaesthesia were divided as follows. 45 normotensive pts (15=1.5mg PE) (15=3mg PE) (15=normal saline) 45 hypertensive patients (15=1.5mg PE) (15=3mg PE) (15=normal saline). All the patients received the drug prophylatically. The incidence of hypotension ( >25% decrease in MAP from base line) was significantly lower in the patients who received phenylephrine 1.5 or 3 mg than the control groups. Both in normotensive and hypertensive group (p < 0.01). the normotensive patient who received 3mg and 1.5mg and hypertensive patients who received 3mg had significant lower percentage reduction MAP (p<0.05) and required smaller doses of rescue IV ephedrine than with other groups.
Removal of the metallic bullet was easily accomplished under C-arm control before MRI examination on the fol- lowing day. Actually the bullet entry was through the left flank and it had passed through the posterior elements of T12-L1 vertebral bodies, contusing but not passing the spinal cord. The patient was paraplegic at T12 sensory level (AIS=A). On T2 weighted MRI, the rostrocaudal length of signal change area was about 20 mm (Figure 1). Written informed consent was obtained for both intrathe- cal MSCs injection and subcutaneous G-CSF administra- tion from the patient.
MR Imaging is the modality of choice technique to diagnose all spinal extradural angiolipomas , show- ing hyperintenses signals on T1-weighted images without contrast which gives hint of their fatty content. In study conducted by Provenzale and Mclendon  it showed that the hypo-intense area noticed in the lesion on non- contrasted T1-weighted imaging study are indicative of their high vascularity whereas in T2-weighted images may be changeable, but are generally hyper-intense. Finally, most tumors enhance with gadolinium contrast administration . Non-infiltrative type of spinal angi- olipomas are most likely present at the posterior part of the extradural space with well demarcation lines from the surrounding tissues.
bitors primarily inhibit the production of pros- taglandin E2, which is a prostaglandin believed to play an important role in the inflammatory cascade. In addition, specific COX-2 inhibitors are reported to have a less inhibitory effect on bone healing or bone union after a spinal fusion compared with conventional nonspecific NSAIDs both in vivo and vitro. 25,26
Spinal muscular atrophy is a disorder of lower motor neurons, most commonly caused by recessive mutations in SMN1 on chromosome 5q. Cases without SMN1 mutations are subclassiﬁed according to phenotype. Spinal muscular atrophy, lower ex- tremity-predominant, is characterized by lower limb muscle weakness and wasting, associated with reduced numbers of lumbar motor neurons and is caused by mutations in DYNC1H1, which encodes a microtubule motor protein in the dynein-dynactin complex and one of its cargo adaptors, BICD2. We have now identiﬁed 32 patients with BICD2 mutations from nine different families, providing detailed insights into the clinical phenotype and natural history of BICD2 disease. BICD2 spinal muscular atrophy, lower extremity predominant most commonly presents with delayed motor milestones and ankle contractures. Additional features at presentation include arthrogryposis and congenital dislocation of the hips. In all affected individuals, weakness and wasting is lower-limb predominant, and typically involves both proximal and distal muscle groups. There is no evidence of sensory nerve involvement. Upper motor neuron signs are a prominent feature in a subset of individuals, including one family with exclusively adult-onset upper motor neuron features, consistent with a diagnosis of hereditary spastic paraplegia. In all cohort members, lower motor neuron features were static or only slowly progressive, and the majority remained ambulant throughout life. Muscle MRI in six individuals showed a common pattern of muscle involvement with fat deposition in most thigh muscles, but sparing of the adductors and semitendinosus. Muscle pathology ﬁndings were highly variable and included pseudomyopathic features, neuropathic features, and minimal change. The six causative mutations, including one not previously reported, result in amino acid changes within all three coiled-coil domains of the BICD2 protein, and include a possible ‘hot spot’ mutation, p.Ser107Leu present in four families. We used the recently solved crystal structure of a highly conserved region of the Drosophila orthologue of BICD2 to further-explore how the p.Glu774Gly substitution inhibits the binding of BICD2 to Rab6. Overall, the features of BICD2 spinal muscular atrophy, lower extremity predominant are consistent with a pathological process that prefer- entially affects lumbar lower motor neurons, with or without additional upper motor neuron involvement. Deﬁning the phenotypic features in this, the largest BICD2 disease cohort reported to date, will facilitate focused genetic testing and ﬁltering of next generation sequencing-derived variants in cases with similar features.
intermittent encephalopathy. Biopsy samples of the meninges of the posterior fossa, obtained on two separate occasions, re- vealed a fibrocellular proliferation of unclear origin. MR im- aging studies obtained 1 month before the patient’s death showed intra- and extraaxial cystic lesions involving the basal ganglia and cerebellum as well as persistent enhancement of the subarachnoid spaces (Fig 2). The cysts had signal intensity similar to CSF on all sequences, and did not enhance. At this time, the diagnosis of gelatinous pseudocysts secondary to cryptococcosis was entertained, but the biopsy results did not support this hypothesis. A contrast-enhanced MR study of the cervical spine showed diffuse enhancement of the subarach- noid space and a small enhancing nodule within the spinal cord at the C6–C7 level (Fig 3A and B). The patient’s final hospital admission was complicated by pneumonia and he died of sep- sis and multiple organ failure. An autopsy revealed extensive necrotizing pneumonia caused by Legionella pneumophilia. Neuropathologic examination of the brain disclosed diffuse leptomeningeal oligodendrogliomatosis.
In published series of spinal nerve sheath schwan- noma, extradural tumors have not been systematically addressed and they are only mentioned in association with more frequent intradural and intradural–extradural tumors. The disease is more prevalent in the fifth decade of life and more common in females. Extradural cervical spinal cord schwannoma in the pediatric population has never been reported in the literature [12, 13].
The predominant mechanism in most cases of trau- matic brain injury (TBI) is diffuse axonal injury . While axonal injury is common in all TBI regardless of severity, a shearing of the axons occurs in human diffuse axonal injury (DAI) leading to progressive changes that ultimately may result in the loss of connections between nerve cells. The slow progression of events in DAI con- tinues for up to several weeks after injury creating a window of opportunity for therapeutic intervention. There are approximately 500,000 new cases of TBI in the U.S. each year , and the incidence requiring hospitali- zation is estimated to be approximately 200-225/100,000 population. Currently, it is estimated that brain injuries account for 12% of all hospital admissions in the United States . When compared to spinal cord injury, which accounts for less than 1% of hospital admissions, it is clear that TBI is a medical care problem which has a sig- nificant impact financially within the United States. Ap- proximately 30,000 - 44,000 people will survive a severe TBI with GCS score TBI (GCS#10). Yet with new med- ical management techniques, less than 10% will remain in a persistent vegetative state. A GCS score of eight or less generally reflects a state of unconsciousness in which the patient demonstrates no eye opening, does not follow simple commands to move muscles, and has vo- calizations which are limited to sounds. Such signs are indicative of severe brain injury . Approximately 52,000 to 56,000 people die each year from TBI , re- sulting in direct costs approximated at more than $50 billion annually . The costs of severe TBI to the indi- vidual and family are extremely high . Acute medical and rehabilitation bills are often around $100,000 with
Returning back to the more accurate physiological reasoning of having only one gain per subject across all conditions, allowed for an assessment of the model from the perspective of the effects of trunk angle on the predicted internal moments. Because a single mean maximum muscle stress value from all trials was used for each subject to predict the internal moment, this mean value was larger than the maximum muscle stress values for the some of the trials involving full trunk flexion angles, and less than the ones in the trials involving only small trunk flexion angles. The model without passive components was only accurate at the medium trunk flexion angles. As shown in Figure 16 to Figure 19, the model without passive components tended to over predict at small trunk flexion angles, and under predict at near full trunk flexion angles. This finding that including passive components in the model would affect the prediction of the maximum muscle stress value at different trunk flexion angles had significant implication to the previous EMG-assisted biomechanical models. McGill and Kippers (1994) introduced a model included the passive components. They studied the distributions of the spinal load to trunk tissues in flexion-relaxation phenomenon. Their focus was the static full flexed posture so they made no quantitative comparison between external moment and internal moment during the full range of trunk flexion. Thus the effect of trunk flexion angle on the prediction of maximum muscle stress value (in their study it was the maximum muscle force) was not addressed.