Cervical kyphosis correction in Marfan syndrome: our experience and literature review.

BACKGROUND: Spinal deformities are common in Marfan syndrome (MFS). They usually involve the thoraco-lumbar spine but rarely involves the cervical spine. Kyphosis is the common spine deformity of the cervical spine and mandates surgical correction as they are at risk of neurological deterioration since they are refractory to conservative management. Few studies of surgical correction of spine deformity included cervical deformity. OBJECTIVES: To analyze the challenges faced during surgery, clinical and radiological outcome, and complications following surgical correction for cervical kyphosis in Marfan syndrome. METHODS: We identified that 5 patients with a diagnosis of MFS with cervical kyphosis who underwent fusion surgery between the years 2010 and 2022 were reviewed, retrospectively. We analyzed the demographic details, radiological parameters, operative variables (blood loss and nuances), perioperative complications, length of stay, clinical and radiological outcome, and complications following fusion surgery for cervical kyphosis in MFS. RESULTS: The mean age of patients was 16.6 ± 4.72 years (range, 12-23 years). The average kyphotic vertebra involved is 3 ± 0.7 bodies (range 2-4) with 2 patients with thoracic deformity. All patients underwent surgical deformity correction. All patients improved clinically with Nurick grade (pre vs. post: 3.4 vs. 2.2) and mJOA (pre vs. post: 8.2 vs. 12.6). There was significant deformity correction from 37.48° to 9.1°. Mean blood loss encountered was 900 ± 173.2 ml. Perioperative complications: wound complication with CSF leak (1). Late complications: ventilator dependence (1) and junctional kyphosis (1). Mean length of hospital stay was 103 ± 178.9 days. All patients were doing symptomatically better after mean follow-up of 58 ± 28.32 months. One patient is bedridden and hospitalized. CONCLUSION: Cervical kyphosis is a rare spine deformity in patients with MFS, and they usually present with neurological deterioration mandating surgical correction. Multidisciplinary approach (pediatrics, genetics and cardiology) is required for systematic evaluation of these patients. They should be evaluated with necessary imaging to rule out associated spinal deformity (atlanto-axial subluxation, scoliosis, and intraspinal pathology like ductal ectasia). Our results suggest better surgical outcome in terms of low operative complications with neurologic improvement in MFS patients. These patients require regular follow-up to identify late complications (instrument failure, non-union, and pseudarthrosis).

A Rare Triad of Lateral Rectus Palsy, Sylvain Fissure Bleed, and Bilateral SDH Shrouding a Benign Pathology.

Background: Arachnoid cysts are benign extra-cerebral congenital lesions that are thought to arise from the splitting of the arachnoid membrane. Although most arachnoid cysts remain static with advancing age, occasionally they can become symptomatic due to cyst enlargement or hemorrhage. Hemorrhage into the arachnoid cysts (intra-cystic hemorrhage) with or without associated subdural hematoma is of rare occurrence. Case Discussion: A 23-year-old male presented with a history of sudden onset severe headache while sleeping. The patient also noticed double vision, especially when looking towards the left side. Non-contrast computed tomography (NCCT) scan of the head showed left temporal pole hematoma with left Sylvian fissure bleed and bilateral fronto-temporoparietal (FTP) subacute SDH. Conclusion: Every patient with incidentally detected AC, especially in the middle cranial fossa, should be counseled regarding the risk of possible complications including hemorrhage and regular follow-up.

Single-Stage Posterior Only Approach for Unilateral Atlantoaxial Spondyloptosis with type-II odontoid Fracture in Pediatric Patients.

Objective: When there is a complete slippage of facet joints of C1 over C2 such that there is no contact between the articulating surfaces of C1 and C2, the condition is known as atlantoaxial spondyloptosis (AAS). AAS represents an extremely rare manifestation of atlantoaxial instability. This study was performed to highlight the presentation, radiological features, and management of unilateral AAS in pediatric patients. Material and Methods: We retrospectively identified four pediatric patients with AAS from our hospital records in the last 6 years (2014-2019). Results: Among the four patients with unilateral AAS, three were posttraumic and one was diagnosed with craniovertebral junction tuberculosis (CVJ TB). All the patients had a varying degree of spastic quadriparesis on presentation. One patient with CVJ TB presented with neck tilt. All patients with traumatic unilateral AAS were associated with an odontoid fracture. These patients underwent C1-C2 fixation with complete reduction of spondyloptosis using the techniques of joint manipulation and joint remodeling with a posterior only approach. Complete reduction of AAS in patients with trauma was also associated with the realignment of the odontoid fracture. All patients improved neurologically after surgery and achieved excellent correction of the deformity on a follow-up imaging. Conclusion: Pediatric unilateral AAS is an extremely rare phenomenon. A single-stage posterior approach with C1-C2 fixation is a feasible technique for the treatment of this seemingly difficult to correct deformity in pediatric patients and the clinical outcomes are excellent.

Surgical Outcome in Spinal Operation in Patients Aged 70 Years and Above.

INTRODUCTION: The world is seeing a growth of the aging population and the number of surgical treatments in this age group which is also true for spinal conditions. The greatest increase in spinal fusion surgery has been observed in patients aged 65 years and above. Only a few works of literature were available on the issue, especially in India. MATERIALS AND METHODS: An observational study in which 70 patients aged 70 years and above who underwent spinal surgery for degenerative and traumatic spinal injury, from January 2013 to July 2017 in the neurosurgery department of a single institute, were reviewed. Around 53 patients were assessed for disability/functional outcome and their health-related quality of life (HRQOL) using the Oswestry disability index (ODI) and RAND 36-item health survey 1.0 scoring method (SF-36) comparing the preoperative and postoperative status. RESULT: The mean age was 74.19 years (range 70-91 years). Laminectomy-19 (27.14%) was the most common surgical procedure performed. Overall there were nine (12.85%) major complications with mortality of five (7.14%) patients. There was a significant reduction of crippled patients (14-9, P = 0.009) in the ODI score. SF-36: There was significant improvement in degenerative patient (P = 0.000 to P = 0.012). In traumatic patient, only pain had significant improvement (P = 0.045). CONCLUSION: This study showed that the age of the patient should not be the limiting factor for the surgical management of a patient with a degenerative or a traumatic spinal condition.

Primary spinal atypical teratoid/rhabdoid tumour presenting with hematomyelia and subarachnoid haemorrhage-a case report.

Atypical teratoid/rhabdoid tumours (AT/RTs) are highly aggressive and uncommon malignant tumours of the central nervous system (CNS) affecting children younger than 3 years of age. Primary spinal cord involvement is an extremely rare presentation. AT/RTs show necrosis and haemorrhages on histopathology frequently. However, spinal atypical teratoid/rhabdoid tumour (AT/RT) with hematomyelia and spinal subarachnoid haemorrhage (SAH), as seen in our case, has never been reported in the literature in the paediatric age group. We report a case of primary spinal AT/RT in a 3-year-old male child presenting acutely with hematomyelia and spinal SAH and try to elucidate its pathophysiological basis.

Management of Pediatric Posterior Circulation Aneurysms-12-Year Single-Institution Experience.

BACKGROUND: Pediatric posterior circulation aneurysms are rare, complex, poorly understood lesions on which only limited literature is currently available. We report our 12-year experience of managing this condition to enhance knowledge of this rare entity. METHODS: Patients <18 years old with posterior circulation aneurysms managed at our institution from January 2005 to April 2017 were included. Demographic, clinical, radiologic and management details were retrieved from hospital records and characteristics of the aneurysms and treatment were analyzed. RESULTS: During this period, 20 pediatric patients (male-to-female ratio 15:6; mean age, 13.1 years) with posterior circulation aneurysms were treated. Most of the patients (75%) presented with subarachnoid hemorrhage. The most common location was the vertebrobasilar junction and vertebral artery (31.81%) followed by the basilar artery and the posterior cerebral artery (27.72% each). Dissecting (81.8%) and large (63.63%) aneurysms were the most common types noted. Of the15 patients with 22 aneurysms treated, 13 underwent endovascular management (parent vessel sacrifice in 8 aneurysms and parent vessel preservation in 5 aneurysms), 1 patient underwent surgery, and 1 patient received medical management for central nervous system tuberculosis. During follow-up, 1 patient had recurrence of aneurysm, and 1 patient died after discharge from the hospital. Overall good outcome was recorded in 90% of patients (Glasgow Outcome Scale score 4-5). CONCLUSIONS: Vertebrobasilar junction and vertebral artery was the most common location for posterior circulation aneurysms and most were dissecting aneurysms. Endovascular treatment was the mainstay of management. Overall good outcome was observed at long-term follow-up.

Evaluation of Correction of Radiologic Parameters (Angulation and Displacement) and Accuracy of C2 Pedicle Screw Placement in Unstable Hangman's Fracture with Intraoperative Computed Tomography-Based Navigation.

BACKGROUND: Opinions vary regarding optimal treatment of unstable hangman's fractures, including rigid orthosis and internal fixation. The anatomy of upper cervical spine is complex. The advent of intraoperative 3-dimensional navigation systems facilitates safe and accurate instrumentation. OBJECTIVE: To evaluate radiologic parameters of fracture morphology in unstable hangman's fracture in preoperative and postoperative period and accuracy of inserting axis pedicle screws by using intraoperative computed tomography-based navigation. METHODS: Fifteen patients with unstable hangman's fractures with age ranging from 17 years to 81 years were operated using computed tomography-based navigation from September 2011 to march 2016. Patient's age, sex, mechanism of injury, associated injuries, and neurologic status were noted. Clinical outcome, accuracy of screw insertion, preoperative and postoperative displacement, and angulation of C2 over C3 and bony fusion were assessed. RESULTS: Overall, 76 screws were inserted including 30 screws in C2 pedicle with 2 (2/60; 6.7%) malplaced screws in C2 pedicle. Mean follow-up period was 34 ± 18 months (range 7-80 months). Mean hospital stay was 12.8 ± 2.4 days. Mean preoperative and postoperative displacements were 4.09 mm ± 1.78 mm and 1.82 mm ± 1.14 mm respectively with a mean reduction of 2.27 mm ± 1.49 mm. Mean preoperative angulation was 7.23° ± 11.96° and postoperative angulation was 2.32° ± 4.77° with a mean reduction of 5.11° ± 11.96°. Bony fusion was achieved and rotation was preserved at C1-C2 joint in all cases. CONCLUSIONS: Intraoperative O-arm-based navigation is a safe, accurate, and effective tool for screw placement in patients with unstable hangman fracture and achieves good anatomical reduction.