Multiple spinal extradural arachnoid cysts presenting as compressive myelopathy in a teenager: case report and literature review with special emphasis on postoperative spine deformity in the current minimally invasive era.

INTRODUCTION: Multiple synchronous spinal extradural arachnoid cysts are extremely rare in children and can lead to cord compression and disability. Multiple spinal extradural arachnoid cysts (SEDACs) in children with immature and growing spine need to be studied separately from multiple SEDACs in adults with mature and fully grown spine because of multiple surgical limitations in children (blood loss, surgery duration, long-term spinal stability after long segment exposure, etc.). MATERIAL AND METHODS: We planned a non-systematic literature review of similar cases described in literature to analyse the pattern of presentation, management, and outcome of this surgically curable disease. RESULTS: A total of 28 cases of paediatric multiple SEDACs in the age range of 5 months to 17 years and mean age of 11.54 years were analysed. Exposure was achieved by laminectomy in 9, laminoplastic laminotomy in 13, laminectomy in first surgery followed by laminoplasty in second surgery (re-exploration) in 1, hemilaminectomy in 2, and technique "not reported" in 3 children. Complete/near-complete recovery was seen in 27 (96.42%) children. Postoperative spine deformity was reported in 7 (25%) children and was progressive in 2 children during serial follow-up. CONCLUSIONS: Symptomatic multiple synchronous SEDACs in children is a rare surgically curable condition. Sincere attempt to find out a dural defect in all cysts is a very important step in multiple SEDAC surgery. Total cyst wall excision with closure of all dural defects is the gold standard treatment for symptomatic cases. Laminoplasty is preferred for excision of multiple SEDACs in children to prevent postoperative spine deformity during long-term follow-up. Long serial follow-up for postoperative spine deformity is necessary.

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).

Correction of pediatric cervical kyphosis with standalone posterior approach-a single-center experience of seven cases.

PURPOSE: To study clinical and radiological outcomes of pediatric cervical kyphosis correction with a standalone posterior cervical approach. Cervical spine kyphotic deformity in pediatric age group is a distinct entity and the management is challenging. Pediatric cervical kyphosis is less often encountered, and literature is sparse with only few case series. Management algorithms are devised keeping the flexibility of the deformity at the core of decision making. Circumferential fusion is mostly recommended for non-flexible (rigid) kyphosis. METHODS: Authors present a single center retrospective analysis of cases of pediatric cervical kyphosis managed by a standalone posterior approach. Pre- and post-operative clinical and radiological parameters were recorded and analyzed. Changes in neurological status, kyphosis correction and bony fusion were assessed. Surgical and implant related complications were noted. RESULTS: Seven cases (6 male, 1 female) were included. Mean age was 13.9±2.9 years, ranging from 8-17 years. Etiology was traumatic in 2 cases, developmental in 2, and syndromic, Hirayama disease and post-laminectomy in 1 case each. Mean kyphosis correction was 36.80±19.30 (87%±21%) with a mean pre-operative kyphosis angle of 37.80±15.30 and mean immediate post-operative kyphosis angle of 3.70±8.70. Mean hospital stay duration was 10±6 days. Median follow-up duration was 36 months. Myelopathy improved in 5 cases at last follow-up. Six cases demonstrated bony fusion at a mean follow-up of 8.4±1.5 months. CONCLUSION: Significant immediate correction in pediatric cervical kyphosis may be achieved with a standalone posterior approach with proper planning and technique in selected cases. Inserting pedicle screws at strategic locations of implant construct offer better corrections and pull-out strength and maintain long-term stability resulting in higher arthrodesis rates. Larger studies with longer follow up are needed to further ascertain the role of standalone posterior cervical approaches in pediatric cervical kyphosis.

Primary spinal cord glioblastoma multiforme: a single-center experience.

INTRODUCTION: Primary spinal glioblastoma (GBM) are very rare tumors of the spinal cord, with dismal prognosis and their exact management is controversial. We attempt to formulate treatment guidelines for these extremely rare tumors based on our institutional experience and a comprehensive review of the literature. MATERIALS AND METHODS: In this retrospective study from 2008 to 2020, all the patients diagnosed with primary spinal GBM who underwent surgery at our institution were included. Clinical data were retrieved from case files, outpatient records and telephonic follow-up. Data on postoperative chemoradiation was noted in all the patients. The final diagnosis of spinal GBM was confirmed as per the histopathology reports. Patients who could not be followed up and those with prior history of cranial GBM were excluded from the study. RESULTS: Nine patients were followed up and a median survival of 11 months was noted. Chemotherapy with TMZ and radiotherapy to the whole craniospinal axis significantly improved survival in these patients. The extent of surgical resection was not shown to be significant. Intracranial metastasis was the leading cause of mortality in such patients. Three patients developed documented intracranial metastasis during the course of the disease. CONCLUSIONS: Low threshold must be kept in mind in diagnosing patients with high-grade spinal cord intramedullary tumors in view of the rapidly progressing nature of the disease. In case of positive histopathological diagnosis of spinal GBM, the whole craniospinal axis should be imaged and any cranial metastasis which was originally missed during initial workup could be given appropriate radiotherapy.

Posterior fixation for paediatric and adolescent unstable hangman's fracture: evolution to C1 sparing techniques.

PURPOSE: The aim was to study surgical outcomes in hangman's fractures in paediatric and adolescent patients and to demonstrate evolution in posterior surgery from C1-C2-C3 fusion to C1 sparing techniques. METHODS: Patients (aged ≤ 18 years) operated at a tertiary level centre between September 2011 to February 2018 with more than 1 year of follow-up were included. Neurological status, type of fracture, operating time, blood loss, follow-up, and complications were assessed. RESULTS: Nine patients were included, with mean age mean of 16.45 years, with a mean follow-up of 42.78 months. Six patients having neurological deficit showed improvement. Two patients, one having undergone C1-C3 lateral mass screw rod fixation (LMSF) and other had C2 pedicle screw with C3 LMSF, developed kyphosis for which fixation was further extended caudally. One patient with an old hangman's fracture with reabsorbed axis pedicle underwent C2 body screw along with C3-C4 pedicle screw rod fixation and C2 pedicle reconstruction. All patients showed evidence of postoperative fusion. CONCLUSION: Hangman's fractures in young patients can be successfully managed via posterior fixation. In our centre, we have evolved in the direction of motion preservation at C1 C2 joint, along with 3 column stable fixation of the C2 pedicle. C2 pedicle reformation has allowed motion preserving surgery in complex fracture types. Extension of construct till C4 in selected cases is important to prevent postoperative kyphosis.

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.

Cavernous malformations of central nervous system in pediatric patients: our single-centered experience in 50 patients and review of literature.

PURPOSE: Cavernous malformations (CMs) are rare developmental cerebrovascular malformations of the central nervous system with a childhood prevalence of 0.3 to 0.53%. Our purpose was to assess the clinical features and microsurgical outcome in pediatric central nervous system (CNS) CMs. MATERIAL AND METHODS: We retrospectively enrolled all the CM patients admitted to our institute from 1 January 2001 to 31 December 2014. Data was analyzed for their clinical features and surgical outcome. RESULTS: A total of 50 patients with CMs (30 supratentorial, 14 infratentorial, and 6 spinal) with a mean age of 14 years (3-18 years, SD ±4.64) were enrolled into the study. Most of these patients (78%) were male. Size varied from 1.2 to 6 cm. Three patients had multiple CMs. Symptoms of CMs were site specific. Seizure was the most common symptom (63.3%) of CMs at supratentorial location followed by headache (46%) and neurodeficiency (26%), while all brainstem and spinal CMs presented with neurodeficiencies. History of clinically significant acute hemorrhage was present in 19.2% of supratentorial (ST) superficial CMs, 50% of ST deep CMs, 25% of cerebellar CMs, 44.4% of brainstem CMs, and 50% of spinal CMs. Forty-five CMs in 44 patients were surgically excised. Their follow-up ranged from 6 to 162 months (mean 47.2 months, SD ±53). All supratentorial CM patients showed improvement in their symptoms. Patients with preoperative seizure showed good seizure control with Engel scale I in 16 (94.1%) and Engel scale II in 1 (5.9%). In infratentorial (IT) and spinal CM patients, 92.3 and 66.7% had improvement in their neurodeficiencies, respectively. There was no mortality in our series. CONCLUSION: Microsurgical excision of CNS CM results in excellent neurological outcome in pediatric patients. Early intervention is necessary in spinal CMs for better outcome.

Endoscopic pituitary surgery: Techniques, tips and tricks, nuances, and complication avoidance.

Endoscopic pituitary surgery is useful in all micro- and macro-pituitary adenomas including those with suprasellar and cavernous sinus extension. The endoscope provides a panoramic close-up, a multi-angled view with excellent illumination and magnification, permitting complete excision of the tumor with preservation of normal pituitary. However, surgeons need to learn altogether different skills unique to endoscopy and the learning curve is steep. The learning curve can be shortened by proper selection of cases, gradual transition from the microscopic to the endoscopic approach, adequate sphenoethmoidal recess widening, identification of important landmarks during each stage of surgery, and use of neuronavigation. Results and long term outcomes can be improved with bimanual dynamic dissection and sequential tumor excision, preservation of normal pituitary, avoidance of arachnoidal tear and use of extended approach for tumors with large suprasellar extension. The gradual transition from microscopic to endoscopic approach, adherence to step by step technique and learning 'tips and tricks' of the endoscopic pituitary surgery reduce complications.

Medial sphenoid wing meningiomas: Experience with microsurgical resection over 5 years and a review of literature.

BACKGROUND: Medial sphenoid wing meningiomas are medially located tumors on the sphenoid wing with attachment over the anterior clinoid process. They represent a distinct entity. These medial sphenoid wing meningiomas present a more difficult problem for the neurosurgeons because in a majority of cases, they involve the anterior visual pathways and arteries of the anterior circulation and may invade the cavernous sinus (CS). Higher morbidity, mortality and recurrence rates have been observed in these tumors compared with meningiomas in other locations. The rate of recurrence for medial sphenoid wing meningiomas is reported as being one of the highest amongst intracranial meningiomas. MATERIAL AND METHODS: The authors retrospectively analyzed 78 consecutive patients with the diagnosis of medial sphenoid wing meningioma who were operated in our department from January 2008 to December 2012. RESULTS: These patients, having a meningioma of the medial sphenoid ridge, were divided into two types depending on the involvement of CS. Diplopia, internal carotid artery encasement, and postoperative visual deterioration were more common in Type 2 tumors. Similarly, extent of resection and postoperative morbidity were greater in Type 2 patients. CONCLUSIONS: CS invasion confers an added risk to the surgical morbidity and outcomes. However, with proper surgical techniques, optimum outcomes can be achieved and overall surgical results at our center are found to be comparable to that of the current literature.

Neurosurgery at All India Institute of Medical Sciences, a center of excellence: A success story.

The department of neurosurgery at All India Institute of Medical Sciences (AIIMS) started its humble beginning in 1965. With the untiring and selfless hard work of Prof. P N Tandon and Prof. A K Banerji, the department progressed over time to become a center of excellence in the subcontinent. To establish a neurosciences center at AIIMS was an uphill task, which was accomplished with great efforts. The department has established itself as one of the highest centers of learning in the country with its vast infrastructure and diversity in all fields of neurosurgery. AIIMS, New Delhi was established by an act of the parliament in 1956. It was started with a grant from the Government of New Zealand under the "Colombo Plan." It was the vision of Rajkumari Amrita Kaur, the first Health Minister of India, that led to the establishment of a medical institute of international repute in India. AIIMS, New Delhi is an autonomous institute and is governed by the AIIMS Act, 1956. The department of neurosurgery at AIIMS was started in March 1965 with Prof. P.N. Tandon as the Head of the Department. Prof. A.K. Banerji joined him a few months later. The Department celebrated its golden jubilee in the year 2015, and has tremendously grown in stature from its humble beginnings to being a center of excellence with world-wide recognition.

Surgical management of odontoid fractures at level one trauma center: a single-center series of 142 cases.

INTRODUCTION: Odontoid fractures constitute 9-20% of all adult cervical spine fractures. The present study was carried out to focus on the nuances involved in the surgical management of odontoid fractures. MATERIALS AND METHODS: Patients with an odontoid fracture, admitted for surgical stabilization, between January 2008 and March 2014, were included in the study. RESULTS: Among 142 patients [127 male and 15 female patients; median age: 28 years range 4-75 years], type II odontoid fractures were present in 111, type IIA fractures in 8, and type III fractures in 23 patients. 58.5% patients had been involved in a motor vehicular accident while 38.7% had sustained a fall. Eighty-five patients (59.9%) with a well-reduced fracture and an intact transverse ligament underwent anterior odontoid screw (OS) placement; the other 57 patients (40.1%) underwent posterior fixation (PF). The mean follow-up duration was 22 months (range: 6 months-5.4 years). OS placement was successful in 82 patients (96.5%) with a fusion rate of 95% (95.8% in type II, 100% in type III, and 75% in type IIA odontoid fractures). The procedure-related morbidity was 11.7%. One patient died of sub-arachnoid hemorrhage (SAH) that occurred during OS placement. The PF procedures had a better fusion rate (96.5%). The latter patients, however, had significant restriction of their neck movements and an overall morbidity of 8.7%. The revision surgery rates after OS placement and PF fixation were 7% and 3.5%, respectively. CONCLUSIONS: Anterior OS fixation shows excellent fusion rates and should be the first-line management in reduced/non-displaced acute type II (including type IIA) and high type III odontoid fractures as it preserves cervical motion. PF, that has also been associated with an excellent fusion rate, should be reserved for patients where OS fixation has either failed or has not been feasible.

Correction of pediatric cervical kyphosis: our experience and systematic-literature review.

BACKGROUND: Pediatric cervical kyphosis is a distinct entity with diverse etiology (congenital, syndromic, traumatic, metabolic or neoplastic). Surgical correction in pediatric population is challenging due to their growing spine and low blood volume. PURPOSE: To analyse their presentation, surgical techniques and outcome of pediatric cervical kyphosis and systematically review the pertinent literature. DESIGN: Retrospective study. PATIENT SAMPLE: 16 patients aged ≤ 18 years who underwent correction for cervical kyphosis between 2009 and 2021. OUTCOME MEASURES: Nurick's grading, mJOA score and Global cobb's angle. METHODS: Clinical parameters (Nurick grading and mJOA score) were noted from database on admission and on follow-up at 6 months. Radiological parameters of assessment included Global Cobb's angle. The C2-C7 Cobb angle was the angle of C2 vertebra lower end plate and C7 vertebra lower end plate. For C1-2 kyphosis, anterior border of C1 and anterior border of C2 angle was taken. Radiographic parameters were studied on CT and radiographs of cervical spine to assess for stability, the degree of deformity correction and fusion status at 6 months follow-up. RESULTS: 16 patients with mean age of 14.2 ± 3 years (9 syndromic, 4 post-traumatic, 2 metabolic and 1 post-laminectomy). All underwent surgical correction, 6 underwent Antero-posterior spinal fusion, 6 underwent Posterior spinal fusion and 4 underwent Anterior spinal fusion. There was significant clinical improvement postoperatively with-Nurick grade (pre vs. post: 2.8 vs. 1.8, p = 0.004), mJOA score (pre vs. post: 11.3 vs. 14, p = 0.003). There was significant deformity correction of Cobb's angle from 40.7 ± 26.5° to 14.9 ± 10° (p = 0.001). Early complications included intraoperative hemodynamic instability (3) and wound complication (1). Mean follow-up was 76.9 ± 59.3 months. CONCLUSION: Pediatric cervical kyphosis is a debilitating condition which are managed surgically. Approach has to be individualized to the pathology and good results can be achieved. Patients should be screened for syndromic association and followed-up regularly.

Incidence of Rebleed Following Cerebrospinal Fluid (CSF) Drainage in Poor Grade Subarachnoid Hemorrhage: An Institutional Experience.

BACKGROUND: The outcome of poor grade subarachnoid hemorrhage (SAH) is dismal. Some of these patients need cerebrospinal fluid (CSF) drainage procedure for the hydrocephalus and intraventricular hemorrhage (IVH) which may precipitate rebleeding. However, aneurysmal rebleed following CSF drainage procedure is controversial. OBJECTIVE: Our study aimed at analyzing the effect of CSF drainage procedure on aneurysmal rebleeding. MATERIAL AND METHODS: We retrospectively analyzed the records of all the consecutive patients diagnosed with poor grade aneurysmal SAH over three year period. Patients initially requiring either external ventricular drainage (EVD) or lumbar drain (LD) were included in the study group, and the rest (not requiring drainage) were included in the control group. Rebleeding was confirmed on computed tomography. The factors affecting rebleeding were analyzed. RESULTS: Overall 194 patients with poor grade SAH were enrolled in the study (91 males: 103 females; mean age: 50.6 years). The study group had 91 patients (83 EVD and 8 LD) while 103 patients were in the control group. Posterior circulation aneurysms, poor grade SAH, hydrocephalus, and IVH were more common in the study group P < 0.001. The rebleeding rate was 7.6% in the study group and 8.7% in the control group. On univariate analysis size >1 cm, multiplicity, multilobularity, vasospasm, and CSF drainage were significant risk factors for rebleeding (P < 0.001). On multivariate analysis aneurysm size >1 cm, CSF overdrainage >250 ml/day were significantly associated with risk of rebleeding. CONCLUSION: Ventricular drainage is essential to relieve acute hydrocephalus and drain IVH in SAH and we found no significant association between CSF drainage and rebleeding. However, rapid overdrainage of CSF can lead to aneurysm rupture, hence controlled controlled CSF drainage should be undertaken.

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.

Country-Made Firearm (Desi-Katta)-Related Cranial Injuries: An Institutional Experience.

Background: Approximately, 80% of civilian cranial gunshot injuries in India are mainly due to unlicensed mostly country-made guns, called the "Desi-Kattas," manufactured by the traditional gunsmiths. These injuries constitute a unique subgroup, with respect to the make and design along with the unique wound ballistics compared to the factory-made firearms. Objective: This study is aimed at defining the prognostic factors in predicting the outcomes related to cranial gunshot injuries due to these desi-kattas. Methods: Ten patients with kata-related cranial gunshot injury underwent surgical intervention at our institution, between 2014 and 2018. Their clinical status, imaging features, and outcomes were retrospectively analyzed. Results: Ten patients with kata-related gunshot injuries to the head were identified. Homicidal injuries were documented in six cases. Five had a GCS of 13-15 at presentation. Exit wounds were noted in 50% of the suicidal cases. Multilobar involvement was seen in 70% of cases, with the bullet crossing the midline in only 40% cases. Decompressive craniectomy was required in five cases. Eight patients had a Glasgow Outcome Score of four or more at 6 months of follow-up. There was one death during the follow-up period, while the other patient remains to be in vegetative state. Conclusion: The Katta-related cranial injuries constitute low-muzzle velocity injuries with the majority of the patients sustaining nonfatal injuries, especially homicidal. The presence of positive CT findings and the crossing of the bullet across the midline on CT brain predicts dismal prognosis. Optimal neurosurgical approach should consist of minimal local debridement and attain water seal dural closure.

Use of Intraoperative Imaging to Preserve C1-C2 Mobility in Complex Atlas-Hangman Fractures.

Background: Combination fractures of the C1-C2 complex especially atlas and hangman are relatively uncommon and management usually compromises C1-C2 mobility. Objective: To evaluate the treatment of combined C1- hangman's fracture with and without intraoperative O- arm based navigation system, and its outcome in terms of preserving C1-C2 mobility. Methods: This was a case series of patients with combined C1 and hangman's fracture, managed at a tertiary care hospital during February 2009 to December 2016. Neurological function assessed with American Spine Injury Association (ASIA) impairment scale. Radiological fusion of the operated segment assessed with computed tomographic scan, criteria used for successful fusion included formation of callus across the fracture. Preservation of rotational motion between C1 and C2 was assessed by cervical flexion rotation (CFR) test. Results: We included 10 patients (male/female: 9/1; mean 47.7 ± 17.5 years) in our study. Operative intervention was performed in 9 patients. We used intraoperative computed tomogram (CT) scan with navigation in 5 patients. The mean follow-up period was 28.7 months (range 6 to 70 months). Neurological recovery occurred in all 4 patients with preoperative neurological deficits. Radiological fusion occurred in all cases. Rotation at C1-2 was preserved in all 5 cases operated under O-arm guidance and in one patient with type 1 fracture who was managed conservatively. Conclusions: The goals in treating these complex fractures are to achieve early maximum stability and preserving maximum range of motion. These are often competing phenomena, which can be achieved by using intra operative CT scan and navigation system.

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.