Spinal Kyphoscoliosis Associated with Chiari Formation and Syringomyelia 'Recovery' Following Atlantoaxial Fixation: A Preliminary Report and Early Results Based on Experience with 11 Surgically Treated Cases.

OBJECTIVE: Immediate postoperative and early clinical outcome of treatment of spinal kyphoscoliosis (SKS) associated with Chiari formation (CF) and syringomyelia (SM) and treated by atlantoaxial fixation is described. METHODS: During the period April 2016 to March 2018, 11 patients with SKS as a major presenting symptom in association with CF and SM were surgically treated and are analyzed retrospectively. The patients were 6 boys and 5 girls, and their ages ranged from 11-17 years (average 14 years). Six patients (54.5%) had associated neurologic symptoms and 9 patients had neck pain. All patients were treated by atlantoaxial fixation. No manipulation of foramen magnum bone or dura was performed. No surgery was performed on the subaxial cervical or dorsal spine. Patient Satisfaction Score was based on a specially designed questionnaire. Other outcome measures included clinical and radiologic assessment data, clinical photographs and video observation, and a Scoliosis Research Society Questionnaire. RESULTS: Apart from improvement in neurologic condition, more particularly in breathing capability and voice volume restoration, all patients and their relatives noticed recovery in SKS in the immediate postoperative phase. During the average follow-up of 14 months, all patients continued to have progressive symptomatic recovery and recovery from SKS. Patient Satisfaction Score suggested that all patients were 'highly' satisfied with the surgical outcome. CONCLUSIONS: Atlantoaxial fixation in patients with CF and SM associated with SKS results in reversal of several musculoskeletal and neural abnormalities that includes recovery from spinal deformity.

Facetal Fixation Arthrodesis as Treatment of Cervical Radiculopathy.

OBJECTIVE: We assessed the efficacy of "only fixation" as treatment for cervical radiculopathy. METHODS: From 2012 to December 2017, 21 patients who had presented with primary symptoms related to cervical radiculopathy, including radiating pain, tingling paresthesia, numbness, weakness, and wasting were treated by facetal stabilization surgery aimed at achieving segmental arthrodesis. No decompression by removal of bone, disc material, or osteophyte was performed. The age of the patients ranged from 27 to 59 years. Of the 21 patients, 19 were men and 2 were women. The imaging findings showed a herniated disc in 4, a disc bulge in 12, and osteophyte-related foraminal stenosis in 5 patients. Transarticular screw fixation was deployed for surgery. The levels of cervical fixation were guided by the clinical and radiological information and determined by direct observation of the facet morphology and stability by manual manipulation of the bones in the region. RESULTS: All the patients experienced "remarkable" relief of the presenting radicular symptoms in the "immediate" postoperative period. The visual analog scale and neck disability index scores were used to assess the patients before and after surgical treatment. During the follow-up period, which ranged from 6 to 64 months, all the patients continued to experience satisfactory symptomatic relief. CONCLUSIONS: Instability of the spinal segment is the nodal point of pathogenesis and the primary cause of symptoms related to degenerative spondylotic radiculopathy. The treatment is spinal stabilization. No direct bone or soft tissue decompression is necessary.

Voice Quality Affection as a Symptom of Chiari Formation.

OBJECTIVE: Voice alteration as a presenting symptom in cases with Chiari formation is analyzed, as well as outcome after atlantoaxial fixation. METHODS: During the period January 2016 to June 2017, 25 cases of Chiari formation presented with associated or a major presenting symptom of voice or speech quality alteration related to inadequate breathing efforts. All patients underwent atlantoaxial fixation. No foramen magnum decompression was done. RESULTS: In all patients, the voice quality and volume and breathing disturbances improved in the immediate postoperative period after recovering from anesthesia. During the period of follow-up that ranged from 12 to 25 months, all patients continued to improve in their speech function. CONCLUSIONS: Such critical symptoms of voice disturbance and inadequate breathing and the possibility of rapid postoperative recovery have not been recorded earlier in the literature.

Extraspinal-Interdural Surgical Approach for C2 Neurinomas-Report of an Experience with 50 Cases.

OBJECTIVE: We report our experience with surgery in 50 patients with C2 neurinomas. The anatomic subtleties of these discrete forms of tumors and their surgical implications are analyzed. METHODS: During the period 2006-2016, we operated on 50 patients with 55 C2 neurinomas. Type A tumors were located within the spinal canal, type B tumors were located in the lateral gutter, and type C tumors had a paraspinal extension. By working within the dural confines of the tumor and appropriately angulating the microscope, the entire tumor bulk extending into 1 or all 3 compartments was resected. Follow-up duration ranged from 3 months to 10 years (mean 68 months). RESULTS: This series included 36 male and 14 female patients. Age range of patients was 14-70 years (mean age 36 years). Progressive symptoms of myelopathy were present in 41 patients. There were 16 type A + B tumors, 27 type B tumors, 10 type B + C tumors, and 2 type A + B + C tumors. All patients experienced symptom improvement after surgery and were able to resume their normal lifestyle. CONCLUSIONS: C2 neurinomas arise in the region of the C2 ganglion, and despite the fact that some achieve a large size, they remain confined within the dura. Radical tumor resection can be achieved by working within the layers of the dural cover. Bone removal and opening of spinal dura for tumor exposure and resection can be avoided.

Atlantoaxial Fixation for Chiari 1 Formation in Pediatric Age-Group Patients: Report of Treatment in 33 Patients.

OBJECTIVE: The role of atlantoaxial instability in the pathogenesis of Chiari 1 formation (Chiari formation) in pediatric age-group patients is evaluated. MATERIAL AND METHODS: During the period of January 2010 to June 2017, 33 pediatric patients having Chiari formation were treated with atlantoaxial fixation. Twenty-four patients had basilar invagination, and 9 patients had no bone abnormality at the craniovertebral junction. Sixteen patients had syringomyelia, and 9 patients had both basilar invagination and syringomyelia. Considering the type of facet alignment and atlantoaxial instability, the patients were divided into 3 groups. Type 1 dislocation (13 patients) was anterior atlantoaxial instability wherein the facet of the atlas was dislocated anterior to the facet of the axis. Type 2 dislocation (5 patients) was posterior atlantoaxial instability wherein the facet of the atlas was dislocated posterior to the facet of the axis. Type 3 dislocation (15 patients) was the absence of demonstrable facet malalignment. Type 2 and 3 atlantoaxial facet instability were labeled as central atlantoaxial dislocation. In 14 patients, dynamic images showed mobile and at least partially reducible vertical atlantoaxial dislocation. RESULTS: All patients were treated with atlantoaxial plate and screw fixation using techniques described in 1994 and 2004. Foramen magnum decompression or syrinx manipulation was not performed in any patient. Occipital bone and subaxial spinal elements were not included in the fixation construct. All patients had gratifying and sustained clinical improvement. CONCLUSIONS: The outcome further confirms the cause-effect relationship of Chiari formation and atlantoaxial instability.

Subtemporal "Interdural" Surgical Approach for "Giant" Facial Nerve Neurinomas.

OBJECTIVE: The management issues of 15 cases of giant and dumbbell-shaped facial neurinomas that extended both in the middle and posterior cranial fossa are reported. MATERIAL AND METHODS: During the period 2002 to June 2017, we surgically treated 15 cases of giant and dumbbell shaped facial neurinomas: 10 males and 5 females ranging from 17-59 years (average 34.2 years). Average duration of facial nerve weakness before seeking surgical relief was 49.46 months. Fourteen patients had varying degrees of hearing disturbance. Seven patients had ataxia. The sizes of the tumor ranged from 5.2-8 cm (average being 6.2 cm). The tumors were in an "interdural" location, both in the middle and in the posterior cranial fossa. An extradural subtemporal "interdural" approach was used to resect the tumor in both compartments. The dural cover of the tumor provided a well-defined surgical plane of dissection. Tumor recurrence was observed in 3 cases. The tumor was interdural even at the time of recurrence. CONCLUSIONS: Understanding the fact that the facial nerve neurinomas are interdural in nature and soft and necrotic in character can allow quick and safe surgery with a relatively small surgical exposure.

Pial Arteriovenous Fistula: A Brief Review and Report of 14 Surgically Treated Cases.

OBJECTIVE: The authors report their successful experience of treating 14 cases of pial arteriovenous fistula (PAVF) by direct surgery. METHODS: During the period January 2010 to April 2017, 14 patients with PAVF were treated by surgery. Only those patients were selected who had a single arterial feeding channel. There were 9 male patients and 5 female patients, and their ages ranged from 5 to 53 years (average, 19 years). Ten patients were younger than 20 years of age. Five patients presented clinical and radiologic features that suggested hemorrhage from the PAVF. Ten patients had seizures. Two patients had hemispheric symptoms or neurologic deficits at the time of presentation. In 12 patients, there were no gross neurologic deficits. The diagnosis was made on the basis of digital subtraction angiography in all patients and computed tomographic angiography in 8 patients. Angiography revealed that the PAVFs in 8 patients were supplied by the middle cerebral artery, in 5 patients by the anterior cerebral artery, and in 1 patient by branches of the basilar artery. Surgical procedures involved identification of the site of fistula, obliteration of the feeding artery, and resection of the entire venous varix. RESULTS: The PAVF was successfully excluded from circulation in all patients. There were no neurologic deficits related to the surgical procedure. CONCLUSIONS: Direct surgical resection of the entire PAVF is a safe, effective, and probably curative method of treatment.

Only spinal fixation as treatment of prolapsed cervical intervertebral disc in patients presenting with myelopathy.

AIM: An alternative form of surgical treatment of prolapsed cervical intervertebral disc in patients presenting with symptoms related to myelopathy is discussed. The treatment involved fixation of the affected spinal segments and aimed at arthrodesis. No direct manipulation or handling of the disc was done. MATERIALS AND METHODS: During the period August 2010 to June 2017, 16 patients presenting with symptoms attributed to myelopathy and diagnosed to have prolapsed cervical intervertebral disc were surgically treated by spinal stabilization. There were 11 males and 5 females and their ages ranged from 20 to 66 years (average: 40.6 years). Apart from clinical and radiological indicators, the number of spinal segments that were stabilized depended on direct observation of facetal morphology, alignment, and stability. Surgery involved distraction-fixation of facets using Goel facet spacer (8 patients), transarticular facetal fixation (5 patients) using screws or a combination of both facetal spacer, and transarticular screws (3 patients). RESULTS: All patients had "remarkable" clinical improvement in the immediate postoperative period as assessed by visual analog scale, Goel's clinical grading, and Japanese Orthopedic Association scores. Follow-up ranged from 3 to 84 months (average: 50 months). The herniated disc regressed or disappeared at follow-up radiological assessment that ranged from 24 h to 3 months after surgery. CONCLUSIONS: Spinal segmental fixation aiming at arthrodesis with or without distraction of facets and without any direct surgical manipulation in the disc space or removal of the prolapsed portion of the disc can be considered in the armamentarium of the surgeon.

Brain Herniation in Neurofibromatosis with Dysplasia of Occipital Bone and Posterior Skull Base.

A 22-year-old female, a known case of neurofibromatosis 1 (NF1), presented with a congenital swelling in the left occipital region. She had developed recent onset dysphagia and localized occipital headache. Neuroradiology revealed a left occipital meningoencephalocele and a left parapharyngeal meningocele. This was associated with ventriculomegaly. She was advised on cranioplasty along with duraplasty which she denied. She agreed to a lumbar-peritoneal shunt. She described a dramatic improvement in her symptoms following the lumbar-peritoneal shunt. Occipital dysplasias, though uncommon, have been reported in the literature. We review this case and its management and discuss relevant literature on occipital dysplasias in NF1.