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1.  “Giant” terminal myelocystocele: A rare variant of spinal dysraphism 
Asian Journal of Neurosurgery  2015;10(4):350-352.
Terminal myelocystocele (TMC) is a rare form of spinal dysraphism. They constitute approximately 5% of skin covered lumbosacral masses. The TMC is composed of a low-lying conus medullaris with cystic dilatation of caudal central canal and a surrounding meningocele that extends from the conus to the skin. A 2-year-old female child presented with a large lumbosacral mass and weakness of both lower limbs since birth. Magnetic resonance imaging revealed a giant TMC. The child was operated on successfully. This is an interesting and rare case of “giant” TMC with partial sacral agenesis.
doi:10.4103/1793-5482.162730
PMCID: PMC4558822  PMID: 26425175
Giant terminal myelocystocele; good outcome; sacral agenesis; spinal dysraphism; surgery
2.  Terminal myelocystocele--a case report. 
Journal of Korean Medical Science  1996;11(2):197-202.
Terminal myelocystocele is a rare form of occult spinal dysraphism in which the hydromyelic caudal spinal cord and the subarachnoid space are hemiated through a posterior spina bifida. A 1.5 month old boy presented with a large lumbosacral mass and urinary incontinence. The magnetic resonance imaging, operative findings and pathological findings revealed a low lying conus with a dilated central canal dorsally attached to the subcutaneous tissue. Ventral subarachnoid space was enlarged and herniated through the laminar defect of the sacrum. The lesion was typical of a terminal myelocystocele. The clinical features are different from those of myelomeningocele in many aspects. Though the incidence is low, terminal myelocystocele should be included in the differential diagnosis of congenital lesions presenting as a lumbosacral mass.
PMCID: PMC3053929  PMID: 8835771
3.  Imaging of terminal myelocystoceles. 
This article presents a retrospective analysis of the presentation, imaging studies, and associated findings in 20 children with surgically and histologically proven terminal myelocystoceles. All 20 children presented at birth with a black mass; 13 had cloacal extrophy. The patient population was comprised of 15 girls and 5 with ambiguous genitalia: Of the imaging studies, 8 had plain radiographs, 6 myelography-computed tomography, 11 ultrasound, and 14 magnetic resonance. The associated findings included Chiari I (eight patients), Chiari II (one patient), hydromyelia (three patients), hydrocephalus (three patients), and vertebral segmentation anomalies (six patients). Magnetic resonance imaging was the best imaging modality to diagnose and evaluate children with a myelocystocele. Magnetic resonance imaging demonstrated the classic findings: a terminal cyst of the central canal of the spinal cord that is tethered and herniated with arachnoid and cerebrospinal fluid through an area of spinal dysphria onto the back as a mass.
Images
PMCID: PMC2608070  PMID: 8803433
4.  Cervical myelocystocele: Case report and review of literature 
Dysraphisms involving cervical region are very rare and there are very few series describing their follow-up in literature. Here, we report a 6-year-old boy who underwent postnatal “cosmetic” repair of posterior cervical cystic lesion and presented to us with a large recurrence with syringohydromyelia and tethering. Tethered cord should be suspected in the presence of meningocele and intact neurology. Treatment protocols of such complicated cervical spinal dysraphisms should include intradural exploration and detethering, with an aim to prevent neurological deterioration in future.
doi:10.4103/1817-1745.84410
PMCID: PMC3173918  PMID: 21977091
Cervical myelomeningocele; recurrence; tethered cord
5.  An unusual case of 4 level spinal dysraphism: Multiple composite type 1 and type 2 split cord malformation, dorsal myelocystocele and hydrocephalous 
The authors here have reported a rare case of a child with a complex spina bifida with two different levels of split cord malformation (SCM) type 1 and single level type 2, a non terminal myelocystocele, coccygeal dermal sinus, bifid fatty filum and hydrocephalus, which substantiates the neuroenteric canal theory and have further tried to highlight the importance of complete Magnetic resonance imaging (MRI) screening of the whole spine and brain with SCM to rule out other associated conditions. The patient was admitted with a leaking myelocystocele with bilateral lower limb weakness. MRI of whole spine with screening of brain was done. Patient underwent 5 operations in the same sitting- (According to classification given by Mahapatra et al.) removal of SCM type 1a at D7-8; removal of SCM type1c at L2-3; removal of SCM type 2 at D10; repair of non terminal myelocystocele at D6-D10; low pressure ventriculoperitoneal shunt on right side with excision of dermal coccygeal sinus; and, excision of bifid fatty filum. The clinicoradiological findings in our patient further substantiate the multiple accessory neuroenteric canal theory in the development of composite type of SCM. The physical and neurological signs of SCM and nonterminal myelocystocele should prompt the neurosurgeon to consider performing the screening MRI of whole spine with brain to rule out other composite types of SCM and hydrocephalus.
doi:10.4103/1817-1745.84411
PMCID: PMC3173919  PMID: 21977092
4 level spinal dysraphism; non terminal myelocystocele; one stage surgery; spinal cord malformation
6.  A rare case of thoracic myelocystocele associated with type 1 split cord malformation with low lying tethered cord, dorsal syrinx and sacral agenesis: Pentad finding 
Myelocystocele is a rare form of spinal dysraphism. Thoracic myelocystocele is still rarer. The occurrence of thoracic myelocystocele associated with type-1 split cord malformation, low lying tethered cord, dorsal syrinx and spina bifida is extremely rare. Clinical presentation of such a rare case and an early surgical management is discussed briefly.
doi:10.4103/0976-3147.143209
PMCID: PMC4244798  PMID: 25552859
Low lying tethered cord; spinal dysraphism; split cord malformation; syringomyelia; thoracic myelocystocele
7.  Spondylocostal dysostosis (Jarcho-Levine syndrome) associated with occult spinal dysraphism: Report of two cases 
Spondylocostal dysostosis, also known as Jarcho-Levine syndrome, is a rare disorder characterized by multiple vertebral and rib anomalies at birth. The association of occult spinal dysraphic lesions with this entity is rare. Two patients with spondylocostal dysostosis and occult spinal dysraphic lesions, one with type I split cord malformation and another with spinal dermal sinus are being reported. A 7-month-old female child who was operated at birth for imperforate anus was noted to have a dimple at the low back with altered skin color around the dimple. Examination revealed the right lower extremity was slightly thinner than the left. Plain radiographs showed features of spondylocostal dysostosis with scoliosis. Magnetic resonance imaging (MRI) showed a type I split cord malformation at the lumbosacral junction with low-lying conus and terminal syringomyelia. Patient underwent excision of the bony spur uneventfully. A 14-month-old male child was noted to have a small swelling in the low back along with deformity of the right lower chest since birth. Plain radiographs revealed features of spondylocostal dysostosis. MRI showed a spinal dermal sinus at the lumbosacral junction with a low-lying conus. The patient underwent excision of the spinal dermal sinus and untethering of the cord uneventfully. Although rare, spondylocostal dysostosis can be associated with occult spinal dysraphic lesions like type I split cord malformations or spinal dermal sinus. Physicians should be aware about the possibility of children with spondylocostal dysostosis harboring occult spinal dysraphic lesions so that these patients receive appropriate treatment.
doi:10.4103/1817-1745.159204
PMCID: PMC4489055  PMID: 26167215
Jarcho-Levine syndrome; spinal dermal sinus; spondylocostal dysostosis; spondylothoracic dysostosis; tethered cord; type I split cord malformation
8.  Gluteal pseudophallus in a male child: A rare cutaneous marker of occult spinal dysraphism 
Congenital midline paraspinal cutaneous markers have been practically linked to the location and nature of neural-tissue lesions. One of the most interesting congenital midline paraspinal cutaneous markers has been the human tail in the lumbosacral region, with underlying spinal dysraphism. Human tails have many shapes and sizes and are usually localized to the lumbosacral region. After a complete neurological examination, the MRI is the most sensitive diagnostic modality to reveal the underlying occult spinal dysraphic state. Surgical excision is aimed at untethering of the spinal cord in symptomatic children and for aesthetic reasons in asymptomatic patients. Here we report an asymptomatic male child with normal external genitilia, whose tail is attached to the gluteal region like an adult phallus and investigations revealed an underlying spinal dysraphic state.
doi:10.4103/1817-1745.57343
PMCID: PMC3162781  PMID: 21887197
Gluteal pseudophallus; male child; occult spinal dysraphism
9.  The lumbosacral angle does not reflect progressive tethered cord syndrome in children with spinal dysraphism 
Child's Nervous System  2010;26(12):1757-1764.
Purpose
Our goal was to validate the hypothesis that the lumbosacral angle (LSA) increases in children with spinal dysraphism who present with progressive symptoms and signs of tethered cord syndrome (TCS), and if so, to determine for which different types and/or levels the LSA would be a valid indicator of progressive TCS. Moreover, we studied the influence of surgical untethering and eventual retethering on the LSA.
Methods
We retrospectively analyzed the data of 33 children with spinal dysraphism and 33 controls with medulloblastoma. We measured the LSA at different moments during follow-up and correlated this with progression in symptomatology.
Results
LSA measurements had an acceptable intra- and interobserver variability, however, some children with severe deformity of the caudal part of the spinal column, and for obvious reasons those with caudal regression syndrome were excluded. LSA measurements in children with spinal dysraphism were significantly different from the control group (mean LSA change, 21.0° and 3.1° respectively). However, both groups were not age-matched, and when dividing both groups into comparable age categories, we no longer observed a significant difference. Moreover, we did not observe a significant difference between 26 children with progressive TCS as opposed to seven children with stable TCS (mean LSA change, 20.6° and 22.4° respectively).
Conclusions
We did not observe significant differences in LSA measurements for children with clinically progressive TCS as opposed to clinically stable TCS. Therefore, the LSA does not help the clinician to determine if there is significant spinal cord tethering, nor if surgical untethering is needed.
doi:10.1007/s00381-010-1281-0
PMCID: PMC2981732  PMID: 20857121
Lumbosacral angle; Spinal dysraphism; Tethered cord syndrome; Untethering
10.  A rare case of giant terminal lipomyelocystocele with partial sacral agenesis 
Terminal myelocystoceles (TMCs) are a rare form of occult spinal dysraphism. They constitute approximately 5% of skin covered lumbosacral masses. The TMC is composed of a low-lying conus medullaris with cystic dilatation of caudal central canal, a surrounding meningocele and a lipoma that extends from the conus to a subcutaneous fat collection. A 6-month-old female child presented with a large lumbosacral mass (14 cm × 12 cm × 10 cm) and weakness at the ankle joints since birth. Magnetic resonance imaging and computed tomography scan revealed a low-lying terminal lipomyelocystocele with holocord syrinx and partial sacral agenesis. The child was operated on successfully. This is an interesting and rare case of giant terminal lipomyelocystocoele with syrinx and a partial sacral agenesis. Terminal lipomyelocystocele should be included in the differential diagnosis of congenital lesions presenting as a lumbosacral mass and operated early.
doi:10.4103/1817-1745.111425
PMCID: PMC3680899  PMID: 23772247
Giant terminal lipomyelocystocoele; sacral agenesis; syrinx
11.  Extensive Neuronal Differentiation of Human Neural Stem Cell Grafts in Adult Rat Spinal Cord 
PLoS Medicine  2007;4(2):e39.
Background
Effective treatments for degenerative and traumatic diseases of the nervous system are not currently available. The support or replacement of injured neurons with neural grafts, already an established approach in experimental therapeutics, has been recently invigorated with the addition of neural and embryonic stem-derived precursors as inexhaustible, self-propagating alternatives to fetal tissues. The adult spinal cord, i.e., the site of common devastating injuries and motor neuron disease, has been an especially challenging target for stem cell therapies. In most cases, neural stem cell (NSC) transplants have shown either poor differentiation or a preferential choice of glial lineages.
Methods and Findings
In the present investigation, we grafted NSCs from human fetal spinal cord grown in monolayer into the lumbar cord of normal or injured adult nude rats and observed large-scale differentiation of these cells into neurons that formed axons and synapses and established extensive contacts with host motor neurons. Spinal cord microenvironment appeared to influence fate choice, with centrally located cells taking on a predominant neuronal path, and cells located under the pia membrane persisting as NSCs or presenting with astrocytic phenotypes. Slightly fewer than one-tenth of grafted neurons differentiated into oligodendrocytes. The presence of lesions increased the frequency of astrocytic phenotypes in the white matter.
Conclusions
NSC grafts can show substantial neuronal differentiation in the normal and injured adult spinal cord with good potential of integration into host neural circuits. In view of recent similar findings from other laboratories, the extent of neuronal differentiation observed here disputes the notion of a spinal cord that is constitutively unfavorable to neuronal repair. Restoration of spinal cord circuitry in traumatic and degenerative diseases may be more realistic than previously thought, although major challenges remain, especially with respect to the establishment of neuromuscular connections.
When neural stem cells from human fetal spinal cord were grafted into the lumbar cord of normal or injured adult nude rats, substantial neuronal differentiation was found.
Editors' Summary
Background.
Every year, spinal cord injuries, many caused by road traffic accidents, paralyze about 11,000 people in the US. This paralysis occurs because the spinal cord is the main communication highway between the body and the brain. Information from the skin and other sensory organs is transmitted to the brain along the spinal cord by bundles of neurons, nervous system cells that transmit and receive messages. The brain then sends information back down the spinal cord to control movement, breathing, and other bodily functions. The bones of the spine normally protect the spinal cord but, if these are broken or dislocated, the spinal cord can be cut or compressed, which interrupts the information flow. Damage near the top of the spinal cord can paralyze the arms and legs (tetraplegia); damage lower down paralyzes the legs only (paraplegia). Spinal cord injuries also cause many other medical problems, including the loss of bowel and bladder control. Although the deleterious effects of spinal cord injuries can be minimized by quickly immobilizing the patient and using drugs to reduce inflammation, the damaged nerve fibers never regrow. Consequently, spinal cord injury is permanent.
Why Was This Study Done?
Scientists are currently searching for ways to reverse spinal cord damage. One potential approach is to replace the damaged neurons using neural stem cells (NSCs). These cells, which can be isolated from embryos and from some areas of the adult nervous system, are able to develop into all the specialized cells types of the nervous system. However, because most attempts to repair spinal cord damage with NSC transplants have been unsuccessful, many scientists believe that the environment of the spinal cord is unsuitable for nerve regeneration. In this study, the researchers have investigated what happens to NSCs derived from the spinal cord of a human fetus after transplantation into the spinal cord of adult rats.
What Did the Researchers Do and Find?
The researchers injected human NSCs that they had grown in dishes into the spinal cord of intact nude rats (animals that lack a functioning immune system and so do not destroy human cells) and into nude rats whose spinal cord had been damaged at the transplantation site. The survival and fate of the transplanted cells was assessed by staining thin slices of spinal cord with an antibody that binds to a human-specific protein and with antibodies that recognize proteins specific to NSCs, neurons, or other nervous system cells. The researchers report that the human cells survived well in the adult spinal cord of the injured and normal rats and migrated into the gray matter of the spinal cord (which contains neuronal cell bodies) and into the white matter (which contains the long extensions of nerve cells that carry nerve impulses). 75% and 60% of the human cells in the gray and white matter, respectively, contained a neuron-specific protein six months after transplantation but only 10% of those in the membrane surrounding the spinal cord became neurons; the rest developed into astrocytes (another nervous system cell type) or remained as stem cells. Finally, many of the human-derived neurons made the neurotransmitter GABA (one of the chemicals that transfers messages between neurons) and made contacts with host spinal cord neurons.
What Do These Findings Mean?
These findings suggest that human NSC grafts can, after all, develop into neurons (predominantly GABA-producing neurons) in normal and injured adult spinal cord and integrate into the existing spinal cord if the conditions are right. Although these animal experiments suggest that NSC transplants might help people with spinal injuries, they have some important limitations. For example, the spinal cord lesions used here are mild and unlike those seen in human patients. This and the use of nude rats might have reduced the scarring in the damaged spinal cord that is often a major barrier to nerve regeneration. Furthermore, the researchers did not test whether NSC transplants provide functional improvements after spinal cord injury. However, since other researchers have also recently reported that NSCs can grow and develop into neurons in injured adult spinal cord, these new results further strengthen hopes it might eventually be possible to use human NSCs to repair damaged spinal cords.
Additional Information.
Please access these Web sites via the online version of this summary at http://dx.doi.org/doi:10.1371/journal.pmed.0040039.
The US National Institute of Neurological Disorders and Stroke provides information on spinal cord injury and current spinal cord research
Spinal Research (a UK charity) offers information on spinal cord injury and repair
The US National Spinal Cord Injury Association Web site contains factsheets on spinal cord injuries
MedlinePlus encyclopedia has pages on spinal cord trauma and interactive tutorials on spinal cord injury
The International Society for Stem Cell Research offers information on all sorts of stem cells including NSCs
The US National Human Neural Stem Cell Resource provides information on human NSCs, including the current US government's stance on stem cell research
doi:10.1371/journal.pmed.0040039
PMCID: PMC1796906  PMID: 17298165
12.  Two Faces of Chondroitin Sulfate Proteoglycan in Spinal Cord Repair: A Role in Microglia/Macrophage Activation 
PLoS Medicine  2008;5(8):e171.
Background
Chondroitin sulfate proteoglycan (CSPG) is a major component of the glial scar. It is considered to be a major obstacle for central nervous system (CNS) recovery after injury, especially in light of its well-known activity in limiting axonal growth. Therefore, its degradation has become a key therapeutic goal in the field of CNS regeneration. Yet, the abundant de novo synthesis of CSPG in response to CNS injury is puzzling. This apparent dichotomy led us to hypothesize that CSPG plays a beneficial role in the repair process, which might have been previously overlooked because of nonoptimal regulation of its levels. This hypothesis is tested in the present study.
Methods and Findings
We inflicted spinal cord injury in adult mice and examined the effects of CSPG on the recovery process. We used xyloside to inhibit CSPG formation at different time points after the injury and analyzed the phenotype acquired by the microglia/macrophages in the lesion site. To distinguish between the resident microglia and infiltrating monocytes, we used chimeric mice whose bone marrow-derived myeloid cells expressed GFP. We found that CSPG plays a key role during the acute recovery stage after spinal cord injury in mice. Inhibition of CSPG synthesis immediately after injury impaired functional motor recovery and increased tissue loss. Using the chimeric mice we found that the immediate inhibition of CSPG production caused a dramatic effect on the spatial organization of the infiltrating myeloid cells around the lesion site, decreased insulin-like growth factor 1 (IGF-1) production by microglia/macrophages, and increased tumor necrosis factor alpha (TNF-α) levels. In contrast, delayed inhibition, allowing CSPG synthesis during the first 2 d following injury, with subsequent inhibition, improved recovery. Using in vitro studies, we showed that CSPG directly activated microglia/macrophages via the CD44 receptor and modulated neurotrophic factor secretion by these cells.
Conclusions
Our results show that CSPG plays a pivotal role in the repair of injured spinal cord and in the recovery of motor function during the acute phase after the injury; CSPG spatially and temporally controls activity of infiltrating blood-borne monocytes and resident microglia. The distinction made in this study between the beneficial role of CSPG during the acute stage and its deleterious effect at later stages emphasizes the need to retain the endogenous potential of this molecule in repair by controlling its levels at different stages of post-injury repair.
Michal Schwartz and colleagues describe the role of chondroitin sulfate proteoglycan in the repair of injured tissue and in the recovery of motor function during the acute phase after spinal cord injury.
Editors' Summary
Background.
Every year, spinal cord injuries paralyze about 10,000 people in the United States. The spinal cord, which contains bundles of nervous system cells called neurons, is the communication superhighway between the brain and the body. Messages from the brain travel down the spinal cord to control movement, breathing, and other bodily functions; messages from the skin and other sensory organs travel up the spinal cord to keep the brain informed about the body. All these messages are transmitted along axons, long extensions on the neurons. The spinal cord is protected by the bones of the spine but if these are displaced or broken, the axons can be compressed or cut, which interrupts the information flow. Damage near the top of the spinal cord paralyzes the arms and legs (tetraplegia); damage lower down paralyzes the legs only (paraplegia). Spinal cord injuries also cause other medical problems, including the loss of bowel and bladder control. Currently there is no effective treatment for spinal cord injuries. Treatment with drugs to reduce inflammation has, at best, only modest effects. Moreover, because damaged axons rarely regrow, most spinal cord injuries are permanent.
Why Was This Study Done?
One barrier to recovery after a spinal cord injury seems to be an inappropriate immune response to the injury. After an injury, microglia (immune system cells that live in the nervous system), and macrophages (blood-borne immune system cells that infiltrate the injury) become activated. Microglia/macrophage activation can be either beneficial (the cells make IGF-1, a protein that stimulates axon growth) or destructive (the cells make TNF-α, a protein that kills neurons), so studies of microglia/macrophage activation might suggest ways to treat spinal cord injuries. Another possible barrier to recovery is “chondroitin sulfate proteoglycan” (CSPG). This is a major component of the scar tissue (the “glial scar”) that forms around spinal cord injuries. CSPG limits axon regrowth, so attempts have been made to improve spinal cord repair by removing CSPG. But if CSPG prevents spinal cord repair, why is so much of it made immediately after an injury? In this study, the researchers investigate this paradox by asking whether CSPG made in the right place and in the right amount might have a beneficial role in spinal cord repair that has been overlooked.
What Did the Researchers Do and Find?
The researchers bruised a small section of the spinal cord of mice to cause hind limb paralysis, and then monitored the recovery of movement in these animals. They also examined the injured tissue microscopically, looked for microglia and infiltrating macrophages at the injury site, and measured the production of IGF-1 and TNF-α by these cells. Inhibition of CSPG synthesis immediately after injury impaired the functional recovery of the mice and increased tissue loss at the injury site. It also altered the spatial organization of infiltrating macrophages at the injury site, reduced IGF-1 production by these microglia/macrophages, and increased TNF-α levels. In contrast, when CSPG synthesis was not inhibited until two days after the injury, the mice recovered well from spinal cord injury. Furthermore, the interaction of CSPG with a cell-surface protein called CD44 activated microglia/macrophages growing in dishes and increased their production of IGF-1 but not of molecules that kill neurons.
What Do These Findings Mean?
These findings suggest that, immediately after a spinal cord injury, CSPG is needed for the repair of injured neurons and the recovery of movement, but that later on the presence of CSPG hinders repair. The findings also indicate that CSPG has these effects, at least in part, because it regulates the activity and localization of microglia and macrophages at the injury site and thus modulates local immune responses to the damage. Results obtained from experiments done in animals do not always accurately reflect the situation in people, so these findings need to be confirmed in patients with spinal cord injuries. However, they suggest that the effect of CSPG on spinal cord repair is not an inappropriate response to the injury, as is widely believed. Consequently, careful manipulation of CSPG levels might improve outcomes for people with spinal cord injuries.
Additional Information.
Please access these Web sites via the online version of this summary at http://dx.doi.org/10.1371/journal.pmed.0050171.
The MedlinePlus encyclopedia provides information about spinal cord injuries; MedlinePlus provides an interactive tutorial and a list of links to additional information about spinal cord injuries (in English and Spanish)
The US National Institute of Neurological Disorders and Stroke also provides information about spinal cord injury (in English and Spanish)
Wikipedia has a page on glial scars (note: Wikipedia is a free online encyclopedia that anyone can edit; available in several languages)
doi:10.1371/journal.pmed.0050171
PMCID: PMC2517615  PMID: 18715114
13.  Isolated thoracic (D5) intramedullary epidermoid cyst without spinal dysraphism: A rare case report 
Spinal epidermoid cyst, congenital or acquired, is mainly congenital associated with spinal dysraphism, rarely in isolation. Intramedullary epidermoid cysts (IECs) are rare with less than 60 cases reported so far; isolated variety (i.e., without spinal dysraphism) is still rarer. Complete microsurgical excision is the dictum of surgical treatment. A 14-year-old boy presented with 4-month history of upper backache accompanied with progressive descending paresthesia with paraparesis with early bladder and bowel involvement. His condition deteriorated rapidly making him bedridden. Neurological examination revealed upper thoracic myeloradiculopathy probably of neoplastic origin with sensory localization to D5 spinal level. Digital X-ray revealed no feature suggestive of spinal dysraphism. Contrast magnetic resonance imaging (MRI) characteristics clinched the presumptive diagnosis. Near-total microsurgical excision was done leaving behind a small part of the calcified capsule densely adhered to cord. Histopathological features were confirmative of an epidermoid cyst. Postoperatively, he improved significantly with a gain of motor power sufficient to walk without support within a span of 6 months. Spinal IECs, without any specific clinical presentation, are often diagnosed based upon intraoperative and histopathological findings, however early diagnosis is possible on complete MRI valuation. Complete microsurgical excision, resulting in cessation of clinical progression and remission of symptoms, has to be limited to sub-total or near-total excision if cyst is adherent to cord or its confines.
doi:10.4103/1817-1745.159206
PMCID: PMC4489056  PMID: 26167216
Diffusion weighted imaging; epidermoid; intramedullary; spinal dysraphism; thoracic vertebra
14.  True Cervicothoracic Meningocele: A Rare and Benign Condition 
Neurology International  2015;7(3):6079.
Cervical meningoceles are rare spinal dysraphism, accounting for approximately 7% of all cystic spinal dysraphism. In spite of the rarity, the clinical course is most of the times benign. The surgical treatment includes resection of the lesion and untethering, when presented. We present a 14-day-old female child with true meningoceles who underwent to surgical excision and dura-mater repair. Retrospect analysis of the literature concerning true cervical meningocele is performed. By reporting this illustrative case, we focus on its classification and its differentiation from other types of cervical spinal dysraphism, such as myelocystocele and myelomeningocele. Although its course is benign, it is mandatory a continuum follow up with periodic magnetic resonance imaging of spinal cord, since late neurological deterioration has been described.
doi:10.4081/ni.2015.6079
PMCID: PMC4704472  PMID: 26788266
Cervical meningocele; spinal dysraphism
15.  Multiple neural tube defects in the same patient with no neurological deficit 
Congenital deformities involving the coverings of the nervous system are called neural tube defects (NTDs). NTD can be classified as neurulation defects, which occur by stage 12, and postneurulation defects. Cervical meningocele and myelomeningocele are rare spinal dysraphic lesions. Unlike lumbosacral dysraphic lesions, there is often no neurologic deficits and thus the subtle features of cervical cord tethering may be overlooked on imaging. The presence of meningomyelocele and/or encephaloceles at multiple (two or more) sites along the vertebral axis is a very rare event occurring in <1% of cases. Less than 10 cases have been described in the published literature. We are reporting a case of multiple NTD in same patient with no neurological deficit.
doi:10.4103/1817-1745.66677
PMCID: PMC2964786  PMID: 21042511
Dermal sinus; meningomyelocele; neral tube defect; tethered cord
16.  Limited access surgery for 360 degrees in-situ fusion in a dysraphic patient with high-grade spondylolisthesis 
European Spine Journal  2011;21(3):390-395.
Progressive high-grade spondylolisthesis can lead to spinal imbalance. High-grade spondylolisthesis is often reduced and fused in unbalanced pelvises, whereas in-situ fusion is used more often in balanced patients. The surgical goal is to recreate or maintain sagittal balance but if anatomical reduction is necessary, the risk of nerval damage with nerve root disruption in worst cases is increased. Spinal dysraphism like spina bifida or tethered cord syndrome make it very difficult to achieve reduction and posterior fusion due to altered anatomy putting the focus on anterior column support. Intensive neural structure manipulation should be avoided to reduce neurological complications and re-tethering in these cases. A 26-year-old patient with a history of diastematomyelia, occult spina bifida and tethered cord syndrome presented with new onset of severe low back pain, and bilateral L5/S1 sciatica after a fall. The X-ray demonstrated a grade III spondylolisthesis with spina bifida and the MRI scan revealed bilateral severely narrowed exit foramina L5 due to the listhesis. Because she was well balanced sagittally, the decision for in-situ fusion was made to minimise the risk of neurological disturbance through reduction. Anterior fusion was favoured to minimise manipulation of the dysraphic neural structures. Fusion was achieved via isolated access to the L4/L5 disc space. A L5 transvertebral hollow modular anchorage (HMA) screw was passed into the sacrum from the L4/L5 disc space and interbody fusion of L4/L5 was performed with a cage. The construct was augmented with pedicle screw fixation L4–S1 via a less invasive bilateral muscle split for better anterior biomechanical support. The postoperative course was uneventful and fusion was CT confirmed at the 6-month follow-up. At the last follow-up, she worked full time, was completely pain free and not limited in her free-time activities. The simultaneous presence of high-grade spondylolisthesis and spinal dysraphism make it very difficult to find a decisive treatment plan because both posterior and anterior treatment strategies have advantages and disadvantages in these challenging cases. The described technique combines several surgical options to achieve 360° fusion with limited access, reducing the risk of neurological sequelae.
doi:10.1007/s00586-011-1994-0
PMCID: PMC3296860  PMID: 22008862
High-grade spondylolisthesis; In-situ fusion; HMA screw; Tethered cord; Spina bifida
17.  Long Term Outcome of Non-Dysraphic Intramedullary Spinal Cord Lipomas in Adults: Case Series and Review 
Asian Spine Journal  2014;8(4):476-483.
Study Design
It is a case series involving clinical presentation, radiological findings, surgical technique and long term outcome of Non-dysraphic intramedullary spinal cord lipomas in adults along with the review of the literature.
Purpose
The purpose of the study is to find out from our series as well as from literature what determines the long term outcome and how it can be improved in patients diagnosed to have intramedullary spinal cord lipomas.
Overview of Literature
Non-dysraphic spinal intramedullary lipomas in adults are extremely rare. Majority of cases occur in children and in cervico-dorsal regions. Only eight cases of dorso-lumbar spinal lipomas without spinal dysraphism in adults have been reported in the English literature till 2013.
Methods
Here we report our experience with three such cases in the dorsolumbar region and discuss the surgical technique and the long term outcome of such cases.
Results
Review of literature and from our own cases we conclude that long term outcome after surgery is determined by the preoperative neurological status.
Conclusions
Earlier surgical intervention with preserved neurological status results in better outcome. Radical subtotal excision without producing iatrogenic postoperative neurological deficit should be the goal of the surgery and it stabilizes the disease process in the long run. When early clinico-radiological signs of recurrence develop, such patient's to be reoperated immediately to prevent them from developing a fixed neurological deficit.
doi:10.4184/asj.2014.8.4.476
PMCID: PMC4149991  PMID: 25187865
Spinal lipomas; Intramedullary tumors; Spinal cord tumor; Lipomas
18.  Extraspinal Type I Dural Arteriovenous Fistula with a Lumbosacral Lipomyelomeningocele: A Case Report and Review of the Literature 
Seven cases of adult spinal vascular malformations presenting in conjunction with spinal dysraphism have been reported in the literature. Two of these involved male patients with a combined dural arteriovenous fistula (DAVF) and lipomyelomeningocele. The authors present the third case of a patient with an extraspinal DAVF and associated lipomyelomeningocele in a lumbosacral location. A 58-year-old woman with rapid decline in bilateral motor function 10 years after a prior L4-5 laminectomy and cord detethering for diagnosed tethered cord underwent magnetic resonance imaging showing evidence of persistent cord tethering and a lipomyelomeningocele. Diagnostic spinal angiogram showed a DAVF with arterial feeders from bilateral sacral and the right internal iliac arteries. The patient underwent Onyx embolization of both feeding right and left lateral sacral arteries. At 6-month follow-up, MRI revealed decreased flow voids and new collateralized supply to the DAVF. The patient underwent successful lipomyelomeningocele exploration, resection, AV fistula ligation, and cord detethering. This report discusses management of this patient as well as the importance of endovascular embolization followed by microsurgery for the treatment of cases with combined vascular and dysraphic anomalies.
doi:10.1155/2015/526321
PMCID: PMC4407406  PMID: 25949837
19.  Split cord malformation – A study of 300 cases at AIIMS 1990–2006 
Journal of Pediatric Neurosciences  2011;6(Suppl1):S41-S45.
Background:
Split cord malformation (SCM) is a rare condition. With decreasing incidence of neural tube defect (NTD) in the West, the reports of SCM are getting lesser and lesser. However, in India, spinal dysraphism is still a major problem encountered by the neurosurgeons.
Objective:
Our aim was to analyze 300 patients of SCM for their clinical features, radiological findings and outcome of surgery, which can throw light on the subject to others, who have less scope of finding these cases frequently.
Materials and Methods:
Over a 16-year period, we encountered 300 cases of SCM at AIIMS. Over the same period, more than 1500 cases of NTD were managed. SCM was noticed in 20% of cases with NTD. Skin stigmata were noted in two-third of the cases, and scoliosis and foot deformity were observed in 50% and 48% cases, respectively. Motor and sensory deficits were observed in 80% and 70% cases, respectively. Commonest site affected was lumbar or dorsolumbar (55% and 23%, respectively). In 3% cases, it was cervical in location. Magnetic resonance imaging (MRI) scan revealed a large number of anomalies like lipoma, neuroenteric cyst, thick filum and dermoid or epidermoid cysts. All the patients were surgically treated. In type I, bony spurs were excised, and in type II, bands tethering the cord were released. Associated anomalies were managed in the same sitting. Patients were followed up from 3 months to 3 years.
Results:
Overall improvement was noticed in 50% and stabilization in 44% cases and deterioration of neurological status was recorded in 6% cases. However, 50% of those who deteriorated improved to preop status prior to discharge, 7-10 days following surgery.
Conclusions:
SCM is rare and not many large series are available. We operated 300 cases and noticed a large number of associated anomalies and also multilevel and multisite splits. Improvement or stabilization was noted in 94% and deterioration in 6% cases. We recommended prophylactic surgery for our asymptomatic patients.
doi:10.4103/1817-1745.85708
PMCID: PMC3208912  PMID: 22069430
Good outcome; prophylactic surgery; role of magnetic resonance imaging; split cord malformation; surgery
20.  Infiltrating Blood-Derived Macrophages Are Vital Cells Playing an Anti-inflammatory Role in Recovery from Spinal Cord Injury in Mice 
PLoS Medicine  2009;6(7):e1000113.
Using a mouse model of spinal injury, Michal Schwartz and colleagues tested the effect of macrophages on the recovery process and demonstrate an important anti-inflammatory role for a subset of infiltrating monocyte-derived macrophages that is dependent upon their expression of interleukin 10.
Background
Although macrophages (MΦ) are known as essential players in wound healing, their contribution to recovery from spinal cord injury (SCI) is a subject of debate. The difficulties in distinguishing between different MΦ subpopulations at the lesion site have further contributed to the controversy and led to the common view of MΦ as functionally homogenous. Given the massive accumulation in the injured spinal cord of activated resident microglia, which are the native immune occupants of the central nervous system (CNS), the recruitment of additional infiltrating monocytes from the peripheral blood seems puzzling. A key question that remains is whether the infiltrating monocyte-derived MΦ contribute to repair, or represent an unavoidable detrimental response. The hypothesis of the current study is that a specific population of infiltrating monocyte-derived MΦ is functionally distinct from the inflammatory resident microglia and is essential for recovery from SCI.
Methods and Findings
We inflicted SCI in adult mice, and tested the effect of infiltrating monocyte-derived MΦ on the recovery process. Adoptive transfer experiments and bone marrow chimeras were used to functionally distinguish between the resident microglia and the infiltrating monocyte-derived MΦ. We followed the infiltration of the monocyte-derived MΦ to the injured site and characterized their spatial distribution and phenotype. Increasing the naïve monocyte pool by either adoptive transfer or CNS-specific vaccination resulted in a higher number of spontaneously recruited cells and improved recovery. Selective ablation of infiltrating monocyte-derived MΦ following SCI while sparing the resident microglia, using either antibody-mediated depletion or conditional ablation by diphtheria toxin, impaired recovery. Reconstitution of the peripheral blood with monocytes resistant to ablation restored the lost motor functions. Importantly, the infiltrating monocyte-derived MΦ displayed a local anti-inflammatory beneficial role, which was critically dependent upon their expression of interleukin 10.
Conclusions
The results of this study attribute a novel anti-inflammatory role to a unique subset of infiltrating monocyte-derived MΦ in SCI recovery, which cannot be provided by the activated resident microglia. According to our results, limited recovery following SCI can be attributed in part to the inadequate, untimely, spontaneous recruitment of monocytes. This process is amenable to boosting either by active vaccination with a myelin-derived altered peptide ligand, which indicates involvement of adaptive immunity in monocyte recruitment, or by augmenting the naïve monocyte pool in the peripheral blood. Thus, our study sheds new light on the long-held debate regarding the contribution of MΦ to recovery from CNS injuries, and has potentially far-reaching therapeutic implications.
Please see later in the article for Editors' Summary
Editors' Summary
Background
Every year, spinal cord injuries paralyze about 11,000 people in the US. The spinal cord, which contains bundles of nervous system cells called neurons, is the communication highway between the brain and the body. Messages from the brain travel down the spinal cord to control movement, breathing and other bodily functions; messages from the skin and other sensory organs travel up the spinal cord to keep the brain informed about the body. The bones of the spine normally protect the spinal cord but, if these are broken or displaced, the spinal cord can be cut or compressed, which interrupts the information flow. Damage near the top of the spinal cord paralyzes the arms and legs (tetraplegia); damage lower down paralyzes the legs only (paraplegia). Spinal cord injuries also cause other medical problems, including the loss of bladder and bowel control. Currently, there is no effective treatment for spinal cord injuries, which usually cause permanent disability because the damaged nerve fibers rarely regrow.
Why Was This Study Done?
After a spinal cord injury, immune system cells called macrophages accumulate at the injury site. Some of these macrophages—so-called monocyte-derived macrophages—come into (infiltrate) the spinal cord from the blood in response to the injury, whereas others—microglia—are always in the nervous system. Although macrophages are essential for wound healing in other parts of the body, it is unclear whether they have good or bad effects in the spinal cord. Many experts believe that immune system cells hinder healing in the spinal cord and should be suppressed or eliminated, but other scientists claim that macrophages secrete factors that stimulate nerve regrowth. Furthermore, although some macrophages elsewhere in the body have proinflammatory (potentially deleterious) effects, others have anti-inflammatory (beneficial) effects. So do the infiltrating monocyte-derived macrophages and the resident microglia (which are proinflammatory) have different functions at spinal cord injury sites? In this study, the researchers try to answer this important question.
What Did the Researchers Do and Find?
The researchers bruised a small section of the spinal cord of adult mice and then investigated the effect of infiltrating monocyte-derived macrophages on the recovery process. Monocyte-derived macrophages and microglia cannot be distinguished using standard staining techniques so to study their behavior after spinal cord injury the researchers introduced labeled monocyte-derived macrophages into their experimental animals by using adoptive transfer (injection of genetically labeled monocytes into the animals) or by making bone marrow chimeras. In this second technique, the animals' monocyte-derived macrophages (but not their microglia) were killed by irradiating the animals before injection of genetically labeled bone marrow, the source of monocytes. Using these approaches, the researchers found that monocyte-derived macrophages collected at the margins of spinal cord injury sites whereas microglia accumulated throughout the sites. When the pool of monocyte-derived macrophages in the mice was increased by adoptive transfer or by using a technique called “CNS-specific vaccination,” more monocyte-derived macrophages infiltrated the injury site and the animals' physical recovery from injury improved. Conversely, removal of the infiltrating monocyte-derived macrophages from the injury site reduced the animals' physical recovery. Other experiments indicated that the infiltrating monocyte-derived macrophages have a beneficial, local anti-inflammatory effect that is dependent on their expression of interleukin-10 (an anti-inflammatory signaling molecule).
What Do These Findings Mean?
These findings provide new information about the contribution of monocyte-derived macrophages to spontaneous recovery from spinal cord injury, a contribution that has long been debated. In particular, the findings suggest that this subset of macrophages (but not the resident microglia) has a beneficial effect on spinal cord injuries that is mediated by their production of the anti-inflammatory molecule interleukin-10. The findings also show that the effect of these monocyte-derived macrophages can be boosted, at least in mice. Although results obtained in experiments done in animals do not always accurately reflect what happens in people, this new understanding of the different functions of microglia and infiltrating monocyte-derived macrophages after injury to the spinal cord may eventually lead to the development of better treatments for spinal cord injuries.
Additional Information
Please access these Web sites via the online version of this summary at http://dx.doi.org/10.1371/journal.pmed.1000113.
The MedlinePlus encyclopedia provides information about spinal cord injuries (in English and Spanish)
The US National Institute of Neurological Disorders and Stroke provides detailed information about spinal cord injury, including information on current research into the problem (in English and Spanish)
MedlinePlus provides an interactive tutorial on spinal cord injury and a list of links to additional information (in English and Spanish)
doi:10.1371/journal.pmed.1000113
PMCID: PMC2707628  PMID: 19636355
21.  Mind the gap: an unusual case of a cervical lipomyelocele 
Radiology Case Reports  2016;11(3):266-270.
Cervical dysraphism is rare, and the 3 recognized subtypes manifest as cystic, skin-covered masses. To our knowledge, no case of cervical lipomyelocele has been reported in the literature so far. We present a case of surgically and pathologically confirmed cervical lipomyelocele in a patient with spondylocostal dysostosis and multiple other congenital anomalies and a brief review of the literature. In this case, magnetic resonance imaging demonstrates fat extension into a dysraphic cervical spinal canal, allowing for preoperative diagnosis. Computed tomography using 3-dimensional reconstruction serves to more clearly characterize the extensive spine malsegmentation characteristic of spondylocostal dysostosis. The use of this technique is suggested to benefit the orthopedic or neurologic surgeon confronted with such complex malformations.
doi:10.1016/j.radcr.2016.04.008
PMCID: PMC4996908  PMID: 27594964
Cervical dysraphism; Lipomyelocele; Spondylocostal dysostosis
22.  Spinal dysraphism: A challenge continued to be faced by neurosurgeons in developing countries 
Asian Journal of Neurosurgery  2014;9(2):68-71.
Objectives:
The incidence of spinal dysraphism has significantly decreased over the last few decades, all over the world; however, still the incidence is much higher in developing countries with poor socioeconomic status.
Materials and Methods:
The present study includes all patients managed for spinal dysraphism over a period of one year (January 2011-December 2011). Details including demographics, antenatal care history, site and type of lesion, neurological examination, imaging finding, associated congenital anomalies, management offered, and outcome were recorded.
Results:
A total of 27 children were operated for spinal dysraphism during the study period (17 males and 11 females). Median age was 120 days (age range, 1 day to 6 years). Mothers of 15 children did not seek any regular antenatal checkup and only 13 mothers received folic acid supplementation during pregnancy. Fourteen children were delivered at home and 13 were at hospital. The most common site was lumbosacral region (67.8%). Seven patients had rupture of the sac at the time of presentation, one child had local infection, and four patients had hydrocephalus (requiring shunt before surgical repair). Two patients developed hydrocephalus at follow up, needing shunt surgery. The mean hospital stay was 7 days (range, 5 days to 31 days; median, 10 days).
Conclusion:
Spinal dysraphism is still a major public health problem in developing countries. Management of patients with spinal dysraphism is complex and needs close coordination between pediatrician, neurologist, neurosurgeon, and rehabilitation experts. A large number of factors influence the outcome.
doi:10.4103/1793-5482.136713
PMCID: PMC4129580  PMID: 25126121
Meningocele; myelomeningocele; spinal dysraphism
23.  Surgical Treatment of a Patient with Human Tail and Multiple Abnormalities of the Spinal Cord and Column 
Advances in Orthopedics  2010;2011:153797.
The dorsal cutaneous appendage, or so-called human tail, is often considered to be a cutaneous marker of underlying occult dysraphism. The authors present a case of human tail occurring in a 9-month-old infant with multiple abnormalities of the spinal cord and spine. Examination revealed unremarkable except for a caudal appendage and a dark pigmentation area in the low back. Neuroradiological scans revealed cleft vertebrae and bifid ribbon, split cord malformations, block vertebrae, and hemivertebra. Surgical excision of the tail and untethering the spinal cord by removal of the septum were performed. The infant had an uneventful postoperative period and was unchanged neurologically for 18 months of followup. To our knowledge, no similar case reports exist in the literature. The specific features in a rare case with a human tail treated surgically are discussed in light of the available literature.
doi:10.4061/2011/153797
PMCID: PMC3170723  PMID: 21991405
24.  Recurrent ‘universal tumour’ of the spinal cord 
BMJ Case Reports  2012;2012:bcr1220115284.
Lipoma is popularly known as the ‘universal tumour’ because of its ubiquitous presence anywhere in the body. This is the first documented case of recurrent thoracic spinal cord intramedullary lipoma in a 44-year-old man, with a background of spinal dysraphism, which recurred 15 years after initial surgery. He was followed up every 2 years and currently presented with an 8-month history of progressive weakness in his lower limbs. An MRI of the spine confirmed recurrence of lipoma. He underwent redo laminectomy and partial resection and spinal cord decompression with duroplasty. Lipoma, although a low-grade tumour, can cause significant neurological deficits because of its location. Surgical exploration and removal of lipoma is recommended. However, to preserve the functionality of the spinal cord, one may resort to partial resection and aim for spinal cord decompression. The literature on spinal cord lipoma is reviewed and the aetiopathogenesis of this rare occurrence is described.
doi:10.1136/bcr.12.2011.5284
PMCID: PMC4543125  PMID: 22675149
25.  The Indian Basket Trick: a case of delayed paraplegia with complete recovery, caused by misplaced thoracic pedicle screw 
SpringerPlus  2016;5(1):944.
Introduction
Pedicle screw fixation allows purchase of all three spinal columns without encroaching into the spinal canal improving fracture fixation, as well as deformity correction. Fortunately, neurologic injury associated with pedicle screw malposition is rare.
Case presentation
A 19-year-old boy was surgically treated for severe right thoracic scoliosis associated with a Chiari Type 1 malformation and a C6 to T7 syringomyelia. Six months after the initial surgery, the patient was referred to our institution after three weeks of gait disturbances and repeated falls. Imaging showed the gross misplacement of the left T5 pedicle screw, which crossed the center of the vertebral canal. The initial surgery used a freehand technique of pedicle screw insertion, with anteroposterior and lateral postoperative X-ray control. During the surgery, no SEP modifications were noted during pedicle screw placement. However, after insertion of the second rod and scoliosis correction by posterior translation technique, SEP responses decreased considerably. Revision surgery was performed to remove the misplaced screw. During the first three months after screw removal, repeated clinical examinations showed progressive recovery of the neurological deficits. Gait and bladder functions were normal six months after screw removal, and clinical signs of spasticity disappeared. SEP explorations performed at final follow-up showed similar responses to those performed before the initial surgery for scoliosis correction
Discussion and evaluation
Neurologic injury associated with pedicle screw malposition is rare. In early or delayed neurological status worsening, intraoperative or postoperative imaging must be done to detect pedicle screw misplacement. In the current case, thanks to cobalt-chromium and titanium use, MRI and CT scan allowed good visualization of the spinal canal and spinal cord. Experimental studies have shown that neurophysiological monitoring of the spinal cord does not detect moderate compression. In that way, neurophysiological monitoring is an all-or-nothing technique which can misdiagnose early stage of spinal cord injuries. Major penetration of the spinal canal by pedicle screw may conduct to hardware removal.
Conclusions
In early or delayed neurological status worsening, intraoperative or postoperative imaging must be done to detect pedicle screw misplacement. In the current case, thanks to cobalt-chromium and titanium use, MRI and CT scan allowed good visualization of the spinal canal and spinal cord. Major penetration of the spinal canal by pedicle screw may conduct to hardware removal.
doi:10.1186/s40064-016-2334-y
PMCID: PMC4929096  PMID: 27386388
Misplaced pedicle screw; SEP monitoring; Scoliosis; Late paraplegia

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