Perioperative Halo-Gravity Traction in the Treatment of Scoliosis with Intraspinal Anomalies.

OBJECTIVE: To investigate the efficacy and safety of preoperative halo-gravity traction and 1-stage posterior surgery for the treatment of scoliosis with intramedullary anomalies. METHODS: A total of 11 patients with scoliosis with intramedullary anomalies were evaluated. All patients were treated with preoperative halo-gravity traction and 1-stage posterior surgery. The average age was 11.4 years (range, 7-21 years). All patients were followed-up for at least 2 years (mean, 3.5 years; range, 2-5 years). Their radiologic presentations and complications were reviewed. RESULTS: The operating time was 7.9 hours, and the intraoperative bleeding amount was 1890 mL. Both the Cobb angle of scoliosis and kyphosis were significantly improved after halo-gravity traction and the operation (P < 0.05). Tethered cords were released, and intraspinal masses (neurofibromatosis and lipoma) were excised. Syringomyelia and split spinal cord malformations were left untreated. None of the patients experienced deterioration in their neurologic status after surgery. No severe complications, such as infection, cerebrospinal fluid leakage, failed internal fixation, or fractured pedicle screws or rods occurred after the operation. There was no deterioration of neurologic function, delayed infection, or pseudoarthrosis during the follow-up visits. CONCLUSIONS: Preoperative halo-gravity traction and 1-stage posterior surgery provided patients who had scoliosis and intramedullary anomalies an effective and safe treatment option with few complications.

Split spinal cord malformations in 4 Holstein Friesian calves.

BACKGROUND: The split spinal cord malformation (SSCM) is an uncommon congenital malformation of the vertebral canal in which parts of the spinal cord are longitudinally duplicated. In SSCM Type I, each spinal cord has its own dura tube. In the SSCM Type II, both parts of the spinal cord are surrounded by a common dura tube. CASES PRESENTATION: During the clinical examination one calf showed ambulatory paresis and 3 calves non-ambulatory paraparesis. Calf 4 additionally had a congenital tremor. The examination of calf 4 using magnetic resonance imaging (MRI) showed a median hydrosyringomyelia at the level of the 4th lumbar vertebra. The caudal part of this liquid-filled cavity was split longitudinally through a thin septum. From there, the spinal cord structures duplicated with an incomplete division, so that the transverse section of the spinal cord appeared peanut-shaped and in each half a central canal could be observed. The pathological-anatomical examination after euthanasia showed a duplication of the spinal cord in the area of the lumbar vertebral column in all calves. The histopathological examination revealed two central lumbar vertebral column channels. The two spinal cord duplicates were each surrounded by two separate meninges in calf 2 (SSCM type I); in the other calves (1, 3, 4, and) the two central canals and the spinal cord were covered by a common meninx (SSCM type II). A pedigree analysis of calves 2, 3 and 4 showed a degree of relationship suggestive of a hereditary component. This supports the hypothesis of a possible recessive inheritance due to common ancestors, leading to partial genetic homozygosity. CONCLUSIONS: The clinical appearance of SSCM can vary widely. In calves with congenital paralysis SSCM should always be considered as a differential diagnosis. A reliable diagnosis intra vitam is possible only with laborious imaging procedures such as MRI. Further studies on the heritability of this malformation are necessary to confirm a genetic cause of this disease.

The Managment of cervical spine abnormalities in children with spondyloepiphyseal dysplasia congenita: Observational study.

Spondyloepiphyseal dysplasia congenita (SEDC) is an autosomal dominant disorder, characterized by disproportionate dwarfism with short spine, short neck associated with variable degrees of coxa vara. Cervical cord compression is the most hazardous skeletal deformity in patients with SEDC which requires special attention and management.Ten patients with the clinical and the radiographic phenotypes of spondyloepiphyseal dysplasia congenita have been recognized and the genotype was compatible with single base substitutions, deletions or duplication of part of the COL2A1 gene (6 patients out of ten have been sequenced). Cervical spine radiographs showed apparent atlantoaxial instability in correlation with odontoid hypoplasia or os-odontoideum.Instability of 8 mm or more and or the presence of symptoms of myelopathy were the main indications for surgery. Posterior cervical fusion from the occiput or C1-3, decompression of C1-2 and application of autorib transfer followed by halo vest immobilization have been applied accordingly.Orthopedic management of children with spondyloepiphyseal dysplasia congenita (SEDC) should begin with the cervical spine to avoid serious neurological deficits and or mortality.

Rare Hereditary Klippel-Feil Syndrome and Arnold-Chiari Malformation Caused by Cervical Spondylotic Myelopathy.

BACKGROUND: A rare case of familial genetic disorder Klippel-Feil syndrome and Arnold-Chiari malformation caused by cervical spondylotic myelopathy was reported here. CASE DESCRIPTION: The reconstruction of stability and spinal cord decompression was achieved by anterior cervical discectomy, fusion, and internal fixation. CONCLUSIONS: Although the disease genetic characteristics have been studied, operation is necessary when it leads to abnormal neurologic symptoms and the surgery of surgery can have a beneficial outcome.

Huge multiple spinal extradural meningeal cysts in infancy.

BACKGROUND: Multiple spinal extradural meningeal cysts (SEMCs) are rare lesions. SEMCs communicate with the subarachnoid space through multiple dural defects and expand into the extradural space with progressive spinal cord compression. CASE PRESENTATION: We report a 5-month-old boy with hydronephrosis involving nine huge SEMCs that were distributed from the T1-L5 levels. Eight SEMCs, except for one small noncommunicating cyst, were exposed through laminoplastic laminotomy at the T10-L5 and T3-5 levels. Five transdural communications with dural defects were packed with a piece of autologous muscle and fibrin glue. Tenting sutures to lift up the dura to the vertebral arch were added to minimize the extradural dead space. Postoperatively, cord compression was relieved and hydronephrosis improved. CONCLUSION: In conclusion, packing of all dural defects and dural tenting sutures at a one-staged operation is useful in the surgical management of huge and multiple SEMCs in infancy.

Cervicothoracic spine duplication: a 10-year follow up of a neurological intact boy.

PURPOSE: Spine duplication is a very rare condition with the literature being composed of only case reports. All previously reported cases were thoracolumbar spine duplications. Here, we report cervicothoracic spine duplication in a neurological intact male. According to our knowledge, it is the first case in the literature of cervicothoracic spine duplication. CLINICAL PRESENTATION: A 3-year-old patient presented to a primary physician with a complaint of short stature. He was referred to our department with suspected spinal deformity. Computerized tomography imaging revealed anterior bony structure duplication and posterior dysmorphic elements at the C5-T9 levels. Magnetic resonance imaging revealed a syrinx cavity which splits cord at the duplication level and the relation of the syrinx with posterior mediastinum through anterior bone defect. He was followed up for 10 years. CONCLUSION: In the literature, spine duplication has been classified as a severe form of split cord malformation because of the concurrence of bone duplication with split spinal cord malformation (SCM). This case presents a distinct form of SCM which shows non-duplicated dural tube as unclassified and cervicothoracic duplication level without neurological deficitis. Treatment of SCM was based on removal of splitting fibrous/osseous process. Neurologic intact spine duplication could be followed up without surgical intervention.

Compound heterozygous RYR1 mutations in a preterm with arthrogryposis multiplex congenita and prenatal CNS bleeding.

RYR1 mutations, the most common cause of non-dystrophic neuromuscular disorders, are associated with the malignant hyperthermia susceptibility (MHS) trait as well as congenital myopathies with widely variable clinical and histopathological manifestations. Recently, bleeding anomalies have been reported in association with certain RYR1 mutations. Here we report a preterm infant born at 32 weeks gestation with arthrogryposis multiplex congenita due to compound heterozygous, previously MHS-associated RYR1 mutations, with additional signs of prenatal hemorrhage. The patient presented at birth with multiple joint contractures, scoliosis, severe thoracic rigidity and respiratory failure. He continued to depend on mechanical ventilation and tube feeding. Muscle histopathology showed a marked myopathic pattern with eccentric cores. Interestingly, the patient had additional unusual prenatal intraventricular hemorrhage, resulting in post-hemorrhagic hydrocephalus as well as epidural hemorrhage affecting the spinal cord. This report adds to the phenotypic variability associated with RYR1 mutations, and highlights possible bleeding complications in affected individuals.

Congenital Zika Virus Infection Induces Severe Spinal Cord Injury.

We report 2 fatal cases of congenital Zika virus (ZIKV) infection. Brain anomalies, including atrophy of the cerebral cortex and brainstem, and cerebellar aplasia were observed. The spinal cord showed architectural distortion, severe neuronal loss, and microcalcifications. The ZIKV proteins and flavivirus-like particles were detected in cytoplasm of spinal neurons, and spinal cord samples were positive for ZIKV RNA.

Congenital Malformations of Vertebral Articular Processes in Dogs.

Articular process anomalies are considered congenital. Their occurrence in specific breeds may be indicative of undetermined genetics. Clinical significance varies and is interdependent upon location, function and anatomy. Etiology, uniform nomenclature and classification of vertebral articular process anomalies in the dog are lacking; however recent efforts are beginning to address this deficit. This author proposes that the term articular process dysplasia appropriately encompasses the spectrum of anomalies in severity as well as including those affecting both the cranial and caudal articular processes. The general category description of articular process dypslasia doesn't preclude, but rather allows for more specific designations.

Cystic Abnormalities of the Spinal Cord and Vertebral Column.

Cystic lesions of the vertebral column and spinal cord are important differential diagnoses in dogs with signs of spinal cord disease. Synovial cysts are commonly associated with degenerative joint disease and usually affect the cervical and lumbosacral regions. Arachnoid diverticulum (previously known as cyst) is seen in the cervical region of large breed dogs and thoracolumbar region of small breed dogs. This article reviews the causes, diagnosis, and treatment of these and other, less common, cystic lesions.

Atlantoaxial Instability.

Atlantoaxial instability is a congenital neurologic condition predominantly affecting toy breed dogs. Neurologic signs of a cranial cervical myelopathy typically present at a young age and can range from cervical pain (hyperesthesia) to paralysis. Diagnosis is often based on survey radiographs, although advanced diagnostic imaging can facilitate surgical planning, allow evaluation of spinal cord parenchyma, and rule out concurrent neurologic conditions. Treatment options consist of medical or surgical management, with surgical management being preferable in patients with neurologic deficits or those with unresolved cervical pain despite medical management. The prognosis for surgery is generally favorable.

Comparison of Outcome Between Surgical and Conservative Management of Symptomatic Spinal Cord Cavernous Malformations.

BACKGROUND: Intramedullary cavernous malformations (CMs) are rare lesions with unclear natural history. OBJECTIVE: To compare the functional outcomes of spinal CMs managed surgically and conservatively. METHODS: We performed a retrospective study of patients diagnosed with intramedullary CMs seen at our institution from 2006 to 2013. Functional outcomes of patients were assessed by treatment modality with the Modified McCormick Scale and Karnofsky Performance Status. RESULTS: We identified a total of 85 study-eligible patients; 51 (60.0%) were male. Mean age of patients was 40.5 years. Fifty-eight patients underwent microsurgical removal, and 27 patients underwent conservative management. All patients except 1 harbored a single symptomatic intramedullary CM. Mean follow-up time was 42.8 months. For the surgical group (n = 58), 51 CMs were completely resected. During the follow-up period, 40 patients (69.0%) within the surgical group had improvement in neurological state, 16 patients (27.6%) remained unchanged, and 2 patients (3.4%) experienced deteriorated functional status. In the conservative group, 4 patients (14.8%) had improvement of their symptoms, 19 patients (70.4%) remained in baseline, and 4 patients (14.8%) deteriorated. No significant statistical difference was observed in follow-up Karnofsky Performance Status assessment (odds ratio = 0.89; 95% confidence interval = 0.73-1.08; P = .15) or Modified McCormick Scale assessment (odds ratio = 0.90; 95% confidence interval = 0.74-1.10; P = .30) after adjustment for preoperative lesion size and location. Annual hemorrhagic risk was 3.9% in conservatively managed patients. In contrast, no patients experienced subsequent hemorrhages after surgical resection. CONCLUSION: Surgical resection of intramedullary CMs eliminates the risk of subsequent hemorrhagic and may achieve satisfactory outcome when patients are carefully selected. Although conservative management is recommended in patients at high surgical risk, they should be closely monitored because of persistent hemorrhagic risk.

Dural ectasia in a child with Larsen syndrome.

INTRODUCTION: We present a case of an incidental finding of dural ectasia in a child diagnosed with Larsen syndrome. Larsen syndrome is a rare inherited disorder of connective tissue characterized by facial dysmorphism, congenital joint dislocations of the hips, knees and elbows, and deformities of the hands and feet. Dural ectasia is as an abnormal expansion of the dural sac surrounding the spinal cord and may result in spinal morphologic changes, instability, and spontaneous dislocation. To the best of our knowledge, the presence of dural ectasia in Larsen syndrome has not previously been reported. CASE STUDY: A 6-year-old boy diagnosed with Larsen syndrome presented with an upper thoracic curve measuring 74 degrees, a right thoracic curve measuring 65 degrees, and significant cervicothoracic kyphosis with 50% anterior subluxation of C6 on C7 and C7 on T1. Advanced imaging studies showed dural ectasia (evidenced by spinal canal and dural sac expansion), thinning of pedicles and lamina, and C4 and C6 pars defects with cervical foramen enlargement. The patient received growing rod instrumentation (attached to cervical spine fixation) by a combined anterior/posterior surgical approach using intraoperative halo. Complications included intraoperative medial breach (fully resolved), wound dehiscence, 2 instances of bilateral broken rods, and a broken cervical rod. Following 7 lengthening procedures, the patient underwent definitive fusion. DISCUSSION: Surgeons should be aware of the potential for dural ectasia in patients with Larsen syndrome. Its presence will cause difficulties in the surgical intervention for spinal deformity. Multiple factors must be considered, and surgical approach and technique will require modification to avoid complications. Although dural ectasia confounds surgical intervention in these patients, surgery still appears to outweigh the risks associated with delayed intervention. The presence of dural ectasia should not preclude surgical decompression and stabilization. This report adds to the body of knowledge on the treatment of Larsen syndrome by demonstrating the potential existence of dural ectasia and highlights the importance of careful and thorough preoperative evaluation and diagnostic imaging.

Segmental spinal cord hypoplasia in a Holstein Friesian calf.

An 8-day-old female Holstein Friesian calf was examined because of congenital spastic paresis of the hind limbs. Myelography revealed deviation and thinning of subarachnoid contrast medium columns in the lumbar segment. Upon magnetic resonance imaging, the 'hour-glass' subdural compression appeared as a T1-hypointense, T2-hyperintense ovoidal area suggestive of cerebral spinal fluid collection, compatible with hydrosyringomyelia. The calf was euthanized and the necropsy confirmed the diagnosis of segmental spinal cord hypoplasia of the lumbar tract associated to hydromyelic and syringomyelic cavities.

Inherited myelopathies.

Inherited myelopathies are a small, but important subset of diseases that cause dysfunction of the spinal cord. Manifestations can include various combinations of signs and symptoms, including disturbance of gait, spasticity, paraplegia, amyotrophy, sensory loss, and urinary sphincter dysfunction. These diseases can be divided into classes that include (1) distal axonopathies-exemplified by hereditary spastic paraplegia, (2) motor neuron diseases including familial amyotrophic lateral sclerosis and spinal muscular atrophy, (3) inborn errors of metabolism such as adrenomyeloneuropathy, and (4) other inherited diseases with myelopathy as part of their spectrum of manifestations. Although the inherited myelopathies are relatively rare diseases, knowledge of them and their manifestations is important for the physician faced with a patient with myelopathy, particularly if there are similarly affected individuals in the patient's family. In addition, understanding the pathophysiologic underpinnings of these diseases provides insight into the molecular biology of the nervous system and provides a gateway toward developing treatments for these diseases.

Intramedullary neurenteric cyst of the conus medullaris without associated spinal malformation: a case report and review of the literature.

Spinal neurenteric (NE) cysts are rare congenital anomalies that may occur either alone or in the context of a complex malformative disorder. They are usually intradural-extramedullary lesions. Intramedullary NE cysts not associated with other congenital anomalies are very rare and only a few cases have been reported in the conus medullaris region. Intramedullary neurenteric cysts not associated with other spinal anomalies are very rare especially in the conus medullaris region. MRI is useful to define the cyst and the osseous anomalies associated with this lesion. The goal of treatment of an intramedullary neurenteric cyst is total excision at the first operation, if possible. Life-long follow-up with annual MRI is recommended due to the risk of cyst recurrence. We report an intramedullary NE cyst of the conus medullaris without associated malformation and the relevant literature is briefly reviewed.