MR documented craniocervical ligamentous injury at age 18 months: delayed formation of OS odontoideum. Complex management issues. Case-based review.

OBJECTIVE: There are two separate theories regarding the genesis of os odontoideum: congenital and post-traumatic. Trauma documentation in the past has been the presence of a normal odontoid process at the time of initial childhood injury and subsequent development of the os odontoideum. True MR documentation of craniocervical injury in early childhood and subsequent os odontoideum formation has been very rare. METHODS: An 18-month-old sustained craniocervical ligamentous injury documented on MRI with transient neurological deficit. Chiari I abnormality was also recorded. Subsequent serial imaging of craniocervical region showed the formation of os odontoideum and instability. He became symptomatic from the os odontoideum and the Chiari I abnormality. The patient underwent decompression and intradural procedure for Chiari I abnormality and occipitocervical fusion. Postoperative course was complicated by the failure of fusion and redo. He later required transoral ventral medullary decompression. He recovered. RESULTS: This is an MR documented craniocervical ligamentous injury with sequential formation of os odontoideum with accompanying changes in the atlas. Despite a subsequent successful dorsal occipitocervical fusion, he became symptomatic requiring transoral decompression. CONCLUSIONS: Os odontoideum here is recognized as a traumatic origin with the presence of congenital Chiari I abnormality as a separate entity. The changes of the anterior arch of C1 as well as the os formation were serially documented and give credence to blood supply changes in the os and atlas as a result of the trauma. The recognized treatment of dorsal occipitocervical fusion failed in this case requiring also a ventral decompression of the medulla.

Pediatric cervical kyphosis in the MRI era (1984-2008) with long-term follow up: literature review.

OBJECTIVE: Cervical kyphosis is rare in the pediatric population. It may be syndromic or acquired secondary to laminectomy, neoplasia, or trauma. Regardless, this should be avoided to prevent progressive spinal deformity and neurological deficit. Long-term follow-up is needed to evaluate fusion status, spine growth, potential instability, and neurological function. METHODS AND MATERIALS: A retrospective review of 27 children (6 months to 16 years) with cervical kyphotic deformity was performed and limited to the MRI era until 2008, to provide a long-term follow-up after which complex instrumentation was available. There were 27 patients, 19 syndromic (average age 5.36 years), and 8 non-syndromic (average age 14 years). Syndromes encountered were spondyloepiphyseal dysplasia (SED) 4, spondylometaphyseal dysplasia 1, unnamed collagen abnormality syndrome 1, osteogenesis imperfecta (OI) 2, Aarskog syndrome 1, Weaver syndrome 1, Larsen syndrome 1, multiple cervical level disconnection syndrome 1, Klippel-Feil 3, congenital absence of C2 pars 4. Non-syndromic cases; 2 with neurofibromatosis (NF1) and prevertebral tumors, fibromatosis 1, spontaneous kyphosis 1, and postlaminectomy 4. Factors considered were age, pathology, flexibility on cervical spine dynamic films, reduction with traction and spinal cord compression. Patients with flexible kyphosis underwent dorsal fixation. Children with non-flexible ventral compression/kyphosis had crown halo traction. Irreducible kyphosis had ventral decompression and fusion as well as dorsal fusion. Eleven of 19 syndromic children with flexible and reducible kyphosis underwent dorsal fixation alone. Four of 8 non-syndromic (2 NF1) needed ventral and dorsal approaches. RESULTS: The preoperative deformity (global and local Cobb angles) as well as neurological status improved. Growth during follow-up was not impaired, and we did not encounter instability or junctional kyphosis. The only complications were seen in syndromic patients. One patient with SED showed delayed cantilever bending of the ventral fusion mass requiring reoperation, and 1 other OI child had left C5 and C6 nerve root weakness after anterior C4 and C5 decompression which resolved over 1 year. One child with SED developed cervicothoracic junction scoliosis 18 years later after thoracic scoliosis surgery. CONCLUSIONS: Syndromic pathology presented early with neurological dysfunction and 24% had rigid kyphosis. An attempt at traction/reduction was successful as in Tables 1 and 2. The majority exhibited long-term improvement in kyphosis and function. A treatment algorithm and literature review is presented. Table 1 Motor function of the modified Japanese Orthopedic Association (JOA) score in children [24, 37] Score Upper extremity •Unable to move hands or feed oneself 0 •Can move hands; unable to eat with spoon 1 •Able to eat with spoon with difficulty 2 •Able to use spoon; clumsy with buttoning 3 •Healthy; no dysfunction 4 Lower extremity •Unable to sit or stand 0 •Unable to walk without cane or walker 1 •Walks independently on level floor but needs support on stairs 2 •Capable to walking, clumsy 3 •No dysfunction 4 Table 2 Pediatric cervical kyphosis-preoperative evaluations Case ID, year presented Age Sex Diagnosis Presentation Imaging Apex Cobb angle degree Reducibility Preop traction Syndromic #1 2003 4 years M SED Progressive quadriparesis Bladder incontinence Severe C2-4 kyphosis with cord compression C3-4 85° No No #2 2001 3 years M SED Progressive quadriparesis C2-3 kyphosis. No dorsal C2. Buckled cord C2-3 25° No No Recurrent weakness after recovery 2 years later Kyphosis at fusion site C2-3 33° No No #3 1997 13 years M SED Neck pain. Hand weakness. Thoracic scoliosis C1-3 kyphosis Os odontoideum C2-3 30° Yes No #4 2006 6 years F SED Tingling in hands Bladder incontinence Deformed C2 body and odontoid C1-2 instability C2-3 27° Yes No #5 1997 4 years M SMD Quadriparesis. Previous C2-3 kyphosis with O-C3 dorsal fusion elsewhere Fixed C1-2 dislocation. C2-3 kyphosis. O-C4 fusion C2 35° Partial Yes 4 days #6 2007 13 years F Syndromic collagen abnormality Neck pain. Leg length discrepancies. T-L scoliosis. Quadriparesis Bilateral C2 and partial C3 spondylolysis C-T levoscoliosis C2-3 35° Partial Yes 4 days #7 2003 14 years F Osteogenesis imperfecta (OI) Only able to use right upper extremity C3-5 kyphosis. Canal diameter 4 mm at C4 C4 25° No No #8 1989 3 years F OI - Bruck's syndrome Quadriparesis age 9 months. Had C1-C3 posterior decompression and fusion elsewhere Progressive kyphosis Worse weakness Bend in fusion C1-2 40° No No #9 1996 11 years M Aarskog syndrome Neck pain with limited neck motion Cervical myelopathy Psychomotor delay C4-5 spondylolysis C5-6 kyphosis C5 30° No Yes 3 days #10 1989 3½ years F Weaver syndrome Quadriparesis age 2 years. Elsewhere C1-C3 dorsal rib fusion and wires Fusion failure C2-3 subluxation Cord compression C2-3 3° Yes Yes 1 day #11 1986 11 years F Larsen syndrome Neck pain in extension Quadriparesis C2-3 kyphosis. Deformed bodies C2-5 Os odontoideum C1-2 instability C2-3 28° Yes Yes 1 day #12 1996 5 years M Multilevel cervical disconnect syndrome Horner pupil on right Small right arm Quadriparesis C4, C5 vertebral bodies behind C5 C5 body in canal Left vertebral artery in C5 body C4-5 35° No No #13 1985 3 years F Klippel-Feil Neck pain. Weak hands Atlas assimilation C3-4 kyphosis No posterior bony arches C3, C4 C3-4 40° Yes No #14 1994 3 years F Klippel-Feil Unable to sit. Floppy. Quadriparesis C2-3 kyphosis No posterior arches C2-3 and L4 C2-3 45° Yes No #15 1993 11 months F Tuberous sclerosis Spondylolysis C2 Salam seizures Quadriparesis No pars C2 C2-3 kyphosis C2-3 30° Yes No #16 1998 2 years M C2 spondylolysis Quadriparesis, arms worse than legs C2 spondylolysis C2-3 kyphosis C2-3 35° Yes No #17 1998 6 months M C2 spondylolysis Failure to thrive Apneic spells Weak in arms after endoscopy C2-3 kyphosis No C2 lamina Cord compression C3-4 on MRI C2-3 45° Yes No #18 1990 4 years F C2 spondylolysis Developmental delay Quadriparesis C2 spondylolysis C2-3 kyphosis C3 45° Yes No #19 1994 4 years F Klippel-Feil No posterior C2 Torticollis age 6 mo Quadriparesis C2-3 kyphosis No posterior arch C2 Fused C3-4 bodies C2-3 45° Yes No Non-syndromic #20 1996 15 years M NF1. Ventral prevertebral plexiform neurofibroma Neck pain Weak arms Cervical myelopathy C4-5 kyphosis Cord draped over C4-5 Enhanced prevertebral tumor C4-5 60° Partial Yes 4 days #21 1996 6 years M NF1 Age 6 mo had C1-3 laminectomies elsewhere Progressive kyphosis Quadriparesis C3-5 plexiform neurofibromas C2-4 kyphosis C3-4 45° No No #22 1993 11 years M "Fibromatosis" Neck pain Gag ↓ Right hemiparesis C2 body and odontoid curved dorsally C2-3 kyphosis C2 40° No Yes 3 days #23 2007 13 F Mid-cervical kyphosis Neck pain Unable to move neck C3-4 kyphosis C3-4 45° Yes Halo vest elsewhere 6 weeks Repeat traction on referral #24 1998 12 years M Chiari I Syringohydromyelia Difficulty swallowing Quadriparesis Previous posterior fossa and C1-3 decompression Basilar invagination C3-4 kyphosis C3-4 50° Yes Halo traction 3 days #25 1994 16 years M Chiari I. SHM Difficult speech Quadriparesis Previous posterior fossa and C1-4 laminectomies C3-4 kyphosis Basilar invagination C3-4 55° Yes Halo traction 3 days #26 2002 11 years M Chordoma C3-5 Initial quadriparesis improved after posterior decompression then worse Dorsal and lateral tumor C3-4 C3-4 20° Yes Traction 3 days #27 2006 13 years M C4 lamina Aneurysmal bone cyst Neck and shoulder pain C4 laminectomy for tumor resection Worse 4 months later C4-5 kyphosis C3-4 40° Yes No Table 3 Pediatric cervical kyphosis-postoperative evaluations Case ID Diagnosis Treatment-operation Complication PO orthosis F/U time Fusion status Preop Cobb Postop Cobb Preop JOA Postop JOA Comments Syndromic #1 SED Crown halo traction 1. Median mandibular glossotomy. Resection C2-3 bodies with rib graft fusion 2. Dorsal O-C3 rib graft fusion None Halo vest 3 months Soft collar 3 months 8 years Complete anterior and posterior fusion 85° 10° 2 8 Complete neurological recovery #2 SED Crown halo traction 1. Median mandibular glossotomy. C2-4 corpectomies. C2-5 anterior rib graft fusion Recurrent weakness 2 years s later Halo vest 3 months 2 years Fused 25° 20° 4 5 T. scoliosis. Cardiac abnormalities. Walking then quadriparesis Redo ventral resection and C1-4 iliac bone graft Worsening quadriparesis Minerva brace 1 year 18 years Fused 33° 15° 3 5 Much improved in 6 months #3 SED Crown halo traction Dorsal O-C4 fusion with loop and rib graft None Miami J collar 3 months 10 years Fused 30° 13° 4 7 Works in bookstore #4 SED Crown halo traction Dorsal O-C3 fusion with loop and rib graft 4 years later developed C-T scoliosis after T. scoliosis surgery Miami J collar 3 months 14 years Fused 27° 5° 5 7 C-T scoliosis developed after thoracic scoliosis correction #5 SMD Crown halo traction Transoral C2 odontoid resection None Minerva brace 6 months 20 years No from preop status 35° 10° 1 4 In wheelchair. Works as programmer #6 Collagen abnormality Crown halo traction C2-5 ACDF C2-5 plate with C3-4 lag screws Junctional kyphosis 7 years later after scoliosis correction Miami J collar 6 weeks 12 years Fused 36° 5° 4 7 Abnormal vertebral arteries. Thoracic outlet syndrome May-Thurner syndrome #7 OI Crown halo traction C3-5 corpectomies C2-6 Orion plate with iliac crest graft None Soft collar 4 years Fused 25° 30° 1 5 Restrictive lung disease. Multiple fractures Expired #8 OI - Bruck syndrome 1. Redo C1-2 dorsal rib graft fusion No change Molded Minerva brace 4 years Fused 40° 35° 3 4 Increased weakness age 7 2. 11 years age anterior C3-7 decompression and plate C3-7 Worsening left deltoid and biceps function Molded Minerva brace 30 years Fused 52° 34° 3 5 Lives alone. Wheelchair. Computer technologist Uses hands well #9 Aarskog syndrome Crown halo traction C2-6anterior cervical fusion with iliac crest graft None Molded Minerva brace 20 years Fused 30° 14° 4 7 Works on a farm. No myelopathy. Syndrome in family #10 Weaver syndrome Crown halo traction Redo C1-4 dorsal rib graft fusion None Miami J collar 2 years Fused 3° 10° 2 5 Neuroblastoma age 3 months. Chemotherapy Stable #11 Larsen syndrome Crown halo traction O-C5 dorsal fusion None Halo vest 6 weeks Miami J 3 months 6 years Fused 28° 10° 3 7 Doing well #12 Multilevel cervical disconnect syndrome Crown halo traction C5 corpectomy C4-6 iliac bone fusion anteriorly Dorsal C4-6 fusion None Halo vest 3 months 5 years Fused 35° 5° 3 7 Persistent Horner pupil #13 Klippel-Feil Crown halo traction C2-6 posterior rib graft fusion None Halo vest 3 months 19 years Fused 40° 12° 3 7 Hearing loss Genitourinary abnormalities Sprengel's deformity #14 Klippel-Feil Crown halo C2-5 dorsal rib graft fusion None Halo vest 3 months 35 years Fused 45° 10° 1 6 Hearing loss Genitourinary abnormalities #15 Tuberous sclerosis Spondylolysis C2 C1-4 dorsal interlaminar rib fusion None Halo vest 3 months 6 years Fused 30° 5° 1 6 Psychomotor delay #16 C2 spondylolysis C1-4 dorsal interlaminar fusion None Halo vest 3 months 4 years Fused 35° 10° 2 6 Recovered full function in one year #17 C2 spondylolysis Tracheostomy Molded cervicothoracic brace None Mold brace 4 years 6 years Formed C2 posterior arches 45° 20° 1 3 Reformed C2 at 4 years on CT Parents did not wish surgery #18 C2 spondylolysis Intraoperative traction C1-3 dorsal rib graft fusion None Neck brace 4 months 8 years Fused 45° 12° 2 5 Developed C2 posterior elements #19 Klippel-Feil Intraoperative traction O-C4 fusion with rib graft None Molded brace 6 months 1 years Fused O-C2 dorsally 45° 16° 1 4 Able to sit and use hands Non-syndromic #20 NF1 Resection of ventral tumor C3-6 C4-5 corpectomies; C4-5 iliac graft; C3-7 Orion plate None Halo vest 6 weeks 14 years Fused 60° 15° 3 7 Recovered in 6 weeks. Works on a farm #21 NF1 Intraoperative traction Resect prevertebral tumor C2-5 kyphectomies; C2-6 anterior fusion iliac crest None Halo vest 3 months 2 years Fused 45° 20° 3 5 Initial C1-3 decompression done elsewhere #22 Fibromatosis 1. Transoral C2 decompression 2. Dorsal O-C3 fusion with loop None Brace 3 months 12 years Fused 40° 12° 4 6 Age 2 years had neck mass resected. Diagnosis "fibromatosis" #23 Mid-cervical kyphosis Traction C2-5 lateral mass fusion with screws, rods and rib grafts Worse after removal of initial traction Brace 3 months 8 years Fused 45° 15° 7 8 Doing well #24 Chiari I SHM Intraoperative traction O-C5 rib graft fusion None Halo vest 3 months 21 years Fused 50° 7° 2 6 Facets atrophied C2, C3 at surgery #25 Chiari I SHM Intraoperative traction O-C5 dorsal fusion with loop and rib None Miami J brace 4 months 22 years Fused 55° 10° 3 6 Facets atrophied C2-4 at surgery #26 Chordoma C3-4 1. Dorsal lateral C3-6 fusion 2. C2-5 anterior fusion with iliac bone None Miami J brace 6 months 18 years Fused 20° 12° 5 8 Weak in hands after initial surgery elsewhere #27 ABC tumor C4 Anterior C3-5 fusion with plate and bone None Miami J brace 4 weeks 12 years Fused 40° 15° 5 8 No recurrence SED spondyloepiphyseal dysplasia, SMD spondylometaphyseal dysplasia, JOA Japanese Orthopedic Association, MRI magnetic resonance imaging, SHM syringohydromyelia, NF1 neurofibromatosis type 1, f/u follow up, OI osteogenesis imperfecta, CT computed tomography, JK junctional kyphosis.

Neurenteric cysts at foramen magnum in children: presentation, imaging characteristics, and surgical management-case series and literature review.

INTRODUCTION: Neurenteric cysts (NEC) are rare benign tumors of the central nervous system. Even more rare are the NEC at the ventral foramen magnum in children that are misdiagnosed. They cause distortion of the vertebrobasilar vascular system and cervicomedullary neural structures and are adherent to the latter. The inferior portion lies ventral to the upper most dentate ligament. This has implications in the surgical approach for complete excision. METHODS AND CASE SERIES: We reviewed our series of five children with ventral foramen magnum NEC in the MRI era and analyzed presentation, radiological findings and surgical management. A literature review is also presented. RESULTS: There were two females and five males. Headache and neurological deficit was present in all five; one child presented with recurrent meningitis. The NEC was present ventral to the cervicomedullary junction in all cases. The posterolateral transcondylar approach was used for complete excision with no recurrences. CONCLUSIONS: Neurenteric cysts at the ventral foramen magnum can be confused with other cystic lesions. Headaches are the most common presentation. The posterolateral transcondylar approach to the ventral cervicomedullary junction provides the most reliable avenue for entire resection.

Atlas assimilation: spectrum of associated radiographic abnormalities, clinical presentation, and management in children below 10 years.

OBJECTIVE: To analyze the varied presentation and management of atlas assimilation with associated radiographic abnormalities in children in the MRI era METHODS: Database analysis of 313 children (less than 10 years) RESULTS: Atlas assimilation (AA) was associated with atlantoaxial dislocation in 12, abnormal skull base and Chiari I abnormality in 42, C2-C3 segmentation failure and instability and Chiari I abnormality in 74, and condylar hypoplasia and basilar invagination in 74. Proatlas segmentation failures were 54, atlantoaxial rotary dislocation in 26 with Goldenhar's syndrome, abnormal C1 atlas posterior arch causing dynamic compression of cord in 31 children. Vascular compromise was documented in 26 children. The study encompassed ages 6 months to 10 years. Cranial nerves commonly affected were glossopharyngeal, vagal, and hypoglossal nerves. Children below 2 years presented with torticollis, failure to thrive, difficulty swallowing, and motor and sensory deficits. Craniovertebral junction instability associated with AA was treated with custom-built craniocervical orthosis below 5 years. Closed reduction of instability or basilar invagination was attempted with neuromuscular blockade under anesthesia and traction above age 5 years. Successful reduction was treated with dorsal foramen magnum and atlas decompression with occiput-C2 dorsal fusion using rib grafts below the age of 5 years and instrumentation after that. Follow-up was 2 to 32 years. Neurological recovery was seen in nearly all patients. CONCLUSIONS: Children with atlas assimilation and associated abnormalities may be symptomatic in early childhood. The treatment depends on the age and tailored to the abnormalities present. The long-term results have been successful.

Current management of juvenile idiopathic arthritis affecting the craniovertebral junction.

PURPOSE: Craniovertebral instability is a rare and serious problem. While previously treated surgically, better understanding of disease processes has permitted the field to move towards conservative management. Juvenile idiopathic arthritis (JIA) is one cause of pediatric craniovertebral instability. Early recognition and institution of appropriate medical therapy and bracing in a multidisciplinary fashion is critical to avoid long-term instability, joint abnormalities, or morbid surgical procedures. We seek to highlight cases of this rare problem and provide a principled approach to management decisions. METHODS: We review 6 cases that have presented over the last 6 years and highlight 3 cases in particular regarding craniovertebral instability as a presentation of JIA. We reviewed the clinical records and radiographic features with particular emphasis of the stability of the craniovertebral junction. RESULTS: Age range of the subjects was from 5 to 12. All patients presented with neck pain and abnormal head rotation. Four of the patients responded to medical management and/or cervical bracing with no long-term sequelae or instability. Two patients had refractory rotary subluxation, one that responded to manual reduction under pharmacological paralysis and bracing; the other had an incompetent transverse ligament requiring surgical reduction and fixation. CONCLUSIONS: Neck pain and abnormal head rotation in an older child is rare finding but should prompt suspicion as a manifestation of JIA to the general pediatrician or initial provider. Appropriate serologic studies and MRI studies with contrast at the craniovertebral junction is necessary for evaluation. Early institution of medical management and cervical bracing under a multidisciplinary team of pediatric rheumatology and neurosurgery is key to avoiding surgical intervention and long-term abnormalities at the craniovertebral junction.

Intracranial extradural arachnoid cyst in a child.

INTRODUCTION: Arachnoid cysts are benign developmental anomalies of arachnoid membrane origin that can occur anywhere along the neuro-axis. They are believed to develop from the splitting or duplication of the arachnoid membrane by CSF that is trapped by a ball-valve mechanism. Intracranial arachnoid cysts have only been described as intradural lesions while spinal arachnoid cysts can be both intradural or extradural. CASE REPORT: After an extensive literature review, we report the first case of an intracranial, extradural arachnoid cyst in a 5-yearold girl. The child presented with a 2-week history of suspected seizure-like activity and imaging revealed a large midline extradural CSF-containing arachnoid cyst causing severe compression of the superior sagittal sinus and underlying brain. Venous flow through the sagittal sinus was nearly obliterated. Osseous changes and bone growth adjacent to the cyst was also noted on imaging and intraoperatively. She underwent a bifrontal craniotomy and cyst excision with decompression of underlying brain and reestablishment of venous flow through the sagittal sinus.

Infantile cranial fasciitis: case-based review and operative technique.

BACKGROUND: Cranial fasciitis (CF) is an uncommon benign primary lesion of the skull that typically affects the pediatric age group. Due to the rarity of CF, no prospective studies exist. Earliest description of this condition dates to 1980. The limited scientific and clinical literature regarding CF is dominated by case reports. For these reasons, questions pertaining to the true incidence, genetic risk factors, prognosis, and long-term outcome remain unanswered. DISCUSSION: Clinically, CF presents as a firm, painless, growing scalp mass that is typically not considered in the differential diagnosis. Preoperative pathognomonic signs and symptoms are absent, and imaging features are often nonspecific. Treatment is typically through complete surgical resection, at which time histopathological examination confirms the diagnosis of CF. Reconstruction of the skull defect in the child is critical. Autograft techniques help maintain a rigid construct that integrates with the native skull while preserving its continued ability to grow. Generally, a good outcome is observed with complete resection. EXEMPLARY CASE: We report a case of CF in an infant with emphasis on operative nuances and early follow-up results. CONCLUSION: CF is a rare fibroproliferative disease that has a poorly defined incidence and long-term follow-up. Due to its locally invasive nature and nonspecific presentation, CF is often difficult to differentiate from malignancies and infections. Complete surgical resection is the best approach for diagnosis and cure. Its occult clinical presentation often allows it to achieve considerable growth, leaving a sizeable skull defect following resection. Since CF presents in the pediatric population, allograft reconstruction is preferred over titanium mesh or other synthetic materials to allow osseous integration and continued uninterrupted skull growth.

Evolution of transoral approaches, endoscopic endonasal approaches, and reduction strategies for treatment of craniovertebral junction pathology: a treatment algorithm update.

The craniovertebral junction (CVJ), or the craniocervical junction (CCJ) as it is otherwise known, houses the crossroads of the CNS and is composed of the occipital bone that surrounds the foramen magnum, the atlas vertebrae, the axis vertebrae, and their associated ligaments and musculature. The musculoskeletal organization of the CVJ is unique and complex, resulting in a wide range of congenital, developmental, and acquired pathology. The refinements of the transoral approach to the CVJ by the senior author (A.H.M.) in the late 1970s revolutionized the treatment of CVJ pathology. At the same time, a physiological approach to CVJ management was adopted at the University of Iowa Hospitals and Clinics in 1977 based on the stability and motion dynamics of the CVJ and the site of encroachment, incorporating the transoral approach for irreducible ventral CVJ pathology. Since then, approaches and techniques to treat ventral CVJ lesions have evolved. In the last 40 years at University of Iowa Hospitals and Clinics, multiple approaches to the CVJ have evolved and a better understanding of CVJ pathology has been established. In addition, new reduction strategies that have diminished the need to perform ventral decompressive approaches have been developed and implemented. In this era of surgical subspecialization, to properly treat complex CVJ pathology, the CVJ specialist must be trained in skull base transoral and endoscopic endonasal approaches, pediatric and adult CVJ spine surgery, and must understand and be able to treat the complex CSF dynamics present in CVJ pathology to provide the appropriate, optimal, and tailored treatment strategy for each individual patient, both child and adult. This is a comprehensive review of the history and evolution of the transoral approaches, extended transoral approaches, endoscopie assisted transoral approaches, endoscopie endonasal approaches, and CVJ reduction strategies. Incorporating these advancements, the authors update the initial algorithm for the treatment of CVJ abnormalities first published in 1980 by the senior author.

Nosographic identification and management of pediatric craniovertebral junction anomalies: evolution of concepts and modalities of treatment.

The clinical significance of abnormalities of the craniocervical junction has gained tremendous momentum with the increasing interest in the area as a result of better neurodiagnostic imaging. Abnormalities of the bone, soft tissue, and neural structures are easily recognized and there is a better understanding of the biomechanics of this complex region as well as the embryology. A database of symptomatic patients, children, and adults with neurological symptoms and signs secondary to abnormalities at the craniocervical junction have been evaluated by the author at the University of Iowa Hospitals and Clinics. This large database comprises 6,000 patients and provides a better awareness of the natural history of abnormalities as well as diseases affecting this area.A surgical physiological approach to pathology at this region was proposed in 1977 and still holds true. However, with the advent of better imaging as well as surgical approaches and instrumentation, treatment of problems in this region can be divided into the clinical syndromes, surgical approaches, and techniques of stabilization. A brief outline of the evolving nature of this is provided in this manuscript.

The role of occipital condyle and atlas anomalies on occipital cervical fusion outcomes in Chiari malformation type I with syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium.

OBJECTIVE: Congenital anomalies of the atlanto-occipital articulation may be present in patients with Chiari malformation type I (CM-I). However, it is unclear how these anomalies affect the biomechanical stability of the craniovertebral junction (CVJ) and whether they are associated with an increased incidence of occipitocervical fusion (OCF) following posterior fossa decompression (PFD). The objective of this study was to determine the prevalence of condylar hypoplasia and atlas anomalies in children with CM-I and syringomyelia. The authors also investigated the predictive contribution of these anomalies to the occurrence of OCF following PFD (PFD+OCF). METHODS: The authors analyzed the prevalence of condylar hypoplasia and atlas arch anomalies for patients in the Park-Reeves Syringomyelia Research Consortium database who underwent PFD+OCF. Condylar hypoplasia was defined by an atlanto-occipital joint axis angle (AOJAA) ≥ 130°. Atlas assimilation and arch anomalies were identified on presurgical radiographic imaging. This PFD+OCF cohort was compared with a control cohort of patients who underwent PFD alone. The control group was matched to the PFD+OCF cohort according to age, sex, and duration of symptoms at a 2:1 ratio. RESULTS: Clinical features and radiographic atlanto-occipital joint parameters were compared between 19 patients in the PFD+OCF cohort and 38 patients in the PFD-only cohort. Demographic data were not significantly different between cohorts (p > 0.05). The mean AOJAA was significantly higher in the PFD+OCF group than in the PFD group (144° ± 12° vs 127° ± 6°, p < 0.0001). In the PFD+OCF group, atlas assimilation and atlas arch anomalies were identified in 10 (53%) and 5 (26%) patients, respectively. These anomalies were absent (n = 0) in the PFD group (p < 0.001). Multivariate regression analysis identified the following 3 CVJ radiographic variables that were predictive of OCF occurrence after PFD: AOJAA ≥ 130° (p = 0.01), clivoaxial angle < 125° (p = 0.02), and occipital condyle-C2 sagittal vertical alignment (C-C2SVA) ≥ 5 mm (p = 0.01). A predictive model based on these 3 factors accurately predicted OCF following PFD (C-statistic 0.95). CONCLUSIONS: The authors' results indicate that the occipital condyle-atlas joint complex might affect the biomechanical integrity of the CVJ in children with CM-I and syringomyelia. They describe the role of the AOJAA metric as an independent predictive factor for occurrence of OCF following PFD. Preoperative identification of these skeletal abnormalities may be used to guide surgical planning and treatment of patients with complex CM-I and coexistent osseous pathology.

Os odontoideum: database analysis of 260 patients regarding etiology, associated abnormalities, and literature review.

Introduction: Since the first description of os odontoideum in 1886, its origin has been debated. Numerous case series and reports show both a possible congenital origin and origin from the secondary to craniovertebral junction (CVJ) trauma. We conducted a detailed analysis of 260 surgically treated cases to document the initial symptoms, age groups, radiographic findings, and associated abnormalities, aiming to enhance the confirmation of the etiology. A literature search (1970-2022) was performed to correlate our findings. Methods and materials: A total of 260 patients underwent surgical management of a referral database of 520 cases (1978-2022). All patients were examined by plain radiography and myelotomography as needed until 1984, and since then, CT and MRI have been employed. History of early childhood (aged below 6 years) CVJ trauma was investigated, including obtaining emergency department's initial radiographs from the referral and subsequent follow-up. Associated radiographic and systemic abnormalities were noted, and the atlas development was followed. Results: The age of the patients ranged from 4 to 68 years, mostly between 10 and 20 years. There were 176 males and 86 females. Orthotopic os odontoideum was identified in 24 patients, and 236 patients had dystopic os odontoideum. Associated abnormalities were found in 94 of 260 patients, with 73 exhibiting syndromic abnormalities and 21 having Chiari I malformation. Two sets of twins had spondyloepiphyseal dysplasia. Of 260 patients, 156 experienced early childhood trauma /. Among these, 54 initially presented with normal radiographs but later demonstrated anterior atlas hypertrophy. In addition, a smaller posterior C1 arch was observed, leading to the development of os odontoideum. Two children had initial CVJ trauma as documented by MRI, with subsequent classical findings of os odontoideum and atlas changes. Syndromic patients had an earlier presentation. The literature reviewed confirms the multifactorial etiology. Conclusions: The early presentation and associated abnormalities (such as Down syndrome, Klippel-Feil syndrome, Chiari I malformation, spondyloepiphyseal dysplasia, Morquio syndrome, and others) along with case reports documenting familial, hereditary, and twin presentations strongly support a congenital origin. Likewise, surgical complications are more prevalent in syndromic patients (40%) compared to 15% in other cases, as reported in the literature. The documentation of normal odontoid in early childhood trauma cases followed by the later development of os odontoideum provides evidence supporting trauma as an etiological factor. This process also involves vascular changes in both the atlas and the formation of os odontoideum. Associated abnormalities exhibit an earlier presentation and are only seen in cases with a non-traumatic origin.

Management of Ventral Brainstem Compression in Chiari Malformation Type I.

Craniovertebral junction (CVJ) abnormalities are associated with the Chiari malformation type I. These abnormalities may lead to ventral brainstem compression which can be reduced with traction and posterior reduction using instrumentation. In other cases, the irreducible CVJ pathology with persistent ventral brainstem compression requires ventral decompression. In all cases, a posterior extradural or extra-intradural decompression is required along with an occipitocervical fusion to maintain the reduced and realigned CVJ or stabilize the CVJ after a ventral decompression.

Ventriculus terminalis cyst in an infant: a case report.

BACKGROUND: Filar cysts are frequently found on neonatal ultrasound and are physiologically involuting structures with natural resolution. Hence, there has been no previous histologic correlation. Ventriculus terminalis is a focal central canal dilation in the conus medullaris and usually not clinically significant. Extra-axial cyst at the conus-filum junction connected to ventriculus terminalis is extremely rare, especially when associated with tethered lipomatous filum terminale and with progressive cyst enlargement. CASE PRESENTATION: A Caucasian female neonate with abnormal gluteal cleft had ventriculus terminalis cyst with an extra-axial cyst at the conus-filar junction and taut lipomatous filum on ultrasound examination and magnetic resonance imaging. This persisted at 6-month follow up imaging. In light of the nonresolving extra-axial mass and thick taut lipomatous filum, the child underwent L1-L3 osteoplastic laminectomies. The extra-axial cyst expanded after bony decompression and furthermore on dural opening; visualized on ultrasound. It communicated with the central canal and was documented with intraoperative photomicrographs. It was excised and filum sectioned. Histological immunostaining of the cyst wall showed neuroglial and axonal elements. The child did well without deficits at 4-year follow up with normal urodynamics. CONCLUSION: Progression dilation of ventriculus terminalis and extra-axial conofilar cyst with tethered lipomatous filum will likely progress to clinical significance and require surgical intervention. The embryologic basis for this pathology is discussed, with literature review.

Surgical outcomes and risk factors for recurrence of myxopapillary ependymoma: a single-center experience.

OBJECTIVE: Myxopapillary ependymomas (MPEs) are low-grade, well-circumscribed tumors that often involve the conus medullaris, cauda equina, or filum terminale. They account for up to 5% of all tumors of the spine and 13% of spinal ependymomas, with a peak incidence between 30 and 50 years of age. Because of the rarity of MPEs, their clinical course and optimal management strategy are not well defined, and long-term outcomes remain difficult to predict. The objective of this study was to review long-term clinical outcomes of spinal MPEs and identify factors that may predict tumor resectability and recurrence. METHODS: Pathologically confirmed cases of MPE at the authors' institution were identified and medical records were reviewed. Demographics, clinical presentation, imaging characteristics, surgical technique, follow-up, and outcome data were noted. Two groups of patients-those who underwent gross-total resection (GTR) and those who underwent subtotal resection (STR)-were compared using the Mann-Whitney U-test for continuous and ordinal variables and the Fisher exact test for categorical variables. Differences were considered statistically significant at p ≤ 0.05. RESULTS: Twenty-eight patients were identified, with a median age of 43 years at the index surgery. The median postoperative follow-up duration was 107 months (range 5-372 months). All patients presented with pain. Other common presenting symptoms were weakness (25.0%), sphincter disturbance (21.4%), and numbness (14.3%). GTR was achieved in 19 patients (68%) and STR in 9 (32%). Preoperative weakness and involvement of the sacral spinal canal were more common in the STR group. Tumors were larger and spanned more spinal levels in the STR group compared with the GTR cohort. Postoperative modified McCormick Scale grades were significantly higher in the STR cohort compared with the GTR group (p = 0.00175). Seven of the 9 STR patients (77.8%) underwent reoperation for recurrence at a median of 32 months from the index operation, while no patients required reoperation after GTR, for an overall reoperation rate of 25%. CONCLUSIONS: Findings of this study emphasize the importance of tumor size and location-particularly involvement of the sacral canal-in determining resectability. Reoperation for recurrence was necessary in 78% of patients with subtotally resected tumors; none of the patients who underwent GTR required reoperation. Most patients had stable neurological status postoperatively.

Natural history and management of cervical spine disease in chondrodysplasia punctata and coumarin embryopathy.

PURPOSE: Chondrodysplasia punctata (CDP) is a group of skeletal dysplasias manifesting with progressive cervical instability that leads to neurological deficits and eventual death. The major clinical features of CDP also present in a phenocopy known as coumarin embryopathy (CE) which results from coumarin exposure during pregnancy. The objective of this study was to assess treatment strategies employed for children affected by CDP or CE with cervical instability and to determine a strategy on how best to diagnose and treat affected neonates. METHODS: We performed a systematic review of the English literature for cases reporting cervical spine involvement in CDP and CE and identified 44 such patients. We extracted clinical information on these disorders and identified two patients from our craniovertebral junction database of over 6,000 patients evaluated at our institution. RESULTS: Patients most frequently present with hyperreflexia (21%) and weakness (21%), and there were various conservative treatment strategies. Twenty-one percent of patients who were treated conservatively had neurological complications in their clinical course. There were two deaths reported, one resulting from conservative treatment and one from surgical treatment. We also report long-term follow-up analysis for a patient treated at our institution for the last 30 years and agree with all other reports that suggest that monitoring patients for neurological changes is essential to prevent further neurological injury. CONCLUSIONS: This study emphasizes the need for careful neurological and surgical evaluation of pediatric patients with cervical spine abnormalities affected by CDP or CE in order to prevent progressive instability.

Spinal Meningioma in Adults: Imaging Characteristics, Surgical Outcomes, and Risk Factors for Recurrence.

OBJECTIVE: Although spinal meningiomas (SMs) are associated with overall long tumor-free survival, SMs can recur. This study analyzed factors associated with complications, misdiagnosis, and recurrence of SMs. METHODS: We reviewed patient demographics; radiographic characteristics of patients with SMs, including level, location within the canal, and size; surgical resection; pathology; and recurrence. RESULTS: The study included 64 women and 10 men (74 SMs). Of patients, 64 showed no recurrence after surgery with a median (range) follow-up of 17 (1-99) months. Recurrence was identified in 10 patients (13.5%) during a median (range) follow-up of 66 (25-230) months. There was no significant difference in sex between the recurrence and no recurrence cohorts. Patients in the recurrence cohort were significantly younger (median [range] age 58 [35-70] years) than patients in the no recurrence cohort (median [range] age 69 [18-93] years; P = 0.0091). There was significant predilection for foraminal locations in the recurrence cohort (P < 0.001) compared with the no recurrence cohort. SM was correctly identified on preoperative magnetic resonance imaging or computed tomography myelography in 62 of 64 tumors (96.9%) in the no recurrence cohort, but in only 6 of 10 tumors (60%) in the recurrence cohort (P < 0.001). CONCLUSIONS: In 74 patients with SMs, a preponderance of female patients and a predilection of tumors for the thoracic spine were shown. Recurrence was significantly more common in younger than older patients. Risk factors for recurrence included larger tumors, foraminal location, and en plaque lesions. Patients who developed recurrence were significantly more likely to have been misdiagnosed on preoperative imaging with nerve sheath tumors or lymphoma.

Complications and outcomes of posterior fossa decompression with duraplasty versus without duraplasty for pediatric patients with Chiari malformation type I and syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium.

OBJECTIVE: The aim of this study was to determine differences in complications and outcomes between posterior fossa decompression with duraplasty (PFDD) and without duraplasty (PFD) for the treatment of pediatric Chiari malformation type I (CM1) and syringomyelia (SM). METHODS: The authors used retrospective and prospective components of the Park-Reeves Syringomyelia Research Consortium database to identify pediatric patients with CM1-SM who received PFD or PFDD and had at least 1 year of follow-up data. Preoperative, treatment, and postoperative characteristics were recorded and compared between groups. RESULTS: A total of 692 patients met the inclusion criteria for this database study. PFD was performed in 117 (16.9%) and PFDD in 575 (83.1%) patients. The mean age at surgery was 9.86 years, and the mean follow-up time was 2.73 years. There were no significant differences in presenting signs or symptoms between groups, although the preoperative syrinx size was smaller in the PFD group. The PFD group had a shorter mean operating room time (p < 0.0001), fewer patients with > 50 mL of blood loss (p = 0.04), and shorter hospital stays (p = 0.0001). There were 4 intraoperative complications, all within the PFDD group (0.7%, p > 0.99). Patients undergoing PFDD had a 6-month complication rate of 24.3%, compared with 13.7% in the PFD group (p = 0.01). There were no differences between groups for postoperative complications beyond 6 months (p = 0.33). PFD patients were more likely to require revision surgery (17.9% vs 8.3%, p = 0.002). PFDD was associated with greater improvements in headaches (89.6% vs 80.8%, p = 0.04) and back pain (86.5% vs 59.1%, p = 0.01). There were no differences between groups for improvement in neurological examination findings. PFDD was associated with greater reduction in anteroposterior syrinx size (43.7% vs 26.9%, p = 0.0001) and syrinx length (18.9% vs 5.6%, p = 0.04) compared with PFD. CONCLUSIONS: PFD was associated with reduced operative time and blood loss, shorter hospital stays, and fewer postoperative complications within 6 months. However, PFDD was associated with better symptom improvement and reduction in syrinx size and lower rates of revision decompression. The two surgeries have low intraoperative complication rates and comparable complication rates beyond 6 months.

Socioeconomic and demographic factors in the diagnosis and treatment of Chiari malformation type I and syringomyelia.

OBJECTIVE: The goal of this study was to assess the social determinants that influence access and outcomes for pediatric neurosurgical care for patients with Chiari malformation type I (CM-I) and syringomyelia (SM). METHODS: The authors used retro- and prospective components of the Park-Reeves Syringomyelia Research Consortium database to identify pediatric patients with CM-I and SM who received surgical treatment and had at least 1 year of follow-up data. Race, ethnicity, and insurance status were used as comparators for preoperative, treatment, and postoperative characteristics and outcomes. RESULTS: A total of 637 patients met inclusion criteria, and race or ethnicity data were available for 603 (94.7%) patients. A total of 463 (76.8%) were non-Hispanic White (NHW) and 140 (23.2%) were non-White. The non-White patients were older at diagnosis (p = 0.002) and were more likely to have an individualized education plan (p < 0.01). More non-White than NHW patients presented with cerebellar and cranial nerve deficits (i.e., gait ataxia [p = 0.028], nystagmus [p = 0.002], dysconjugate gaze [p = 0.03], hearing loss [p = 0.003], gait instability [p = 0.003], tremor [p = 0.021], or dysmetria [p < 0.001]). Non-White patients had higher rates of skull malformation (p = 0.004), platybasia (p = 0.002), and basilar invagination (p = 0.036). Non-White patients were more likely to be treated at low-volume centers than at high-volume centers (38.7% vs 15.2%; p < 0.01). Non-White patients were older at the time of surgery (p = 0.001) and had longer operative times (p < 0.001), higher estimated blood loss (p < 0.001), and a longer hospital stay (p = 0.04). There were no major group differences in terms of treatments performed or complications. The majority of subjects used private insurance (440, 71.5%), whereas 175 (28.5%) were using Medicaid or self-pay. Private insurance was used in 42.2% of non-White patients compared to 79.8% of NHW patients (p < 0.01). There were no major differences in presentation, treatment, or outcome between insurance groups. In multivariate modeling, non-White patients were more likely to present at an older age after controlling for sex and insurance status (p < 0.01). Non-White and male patients had a longer duration of symptoms before reaching diagnosis (p = 0.033 and 0.004, respectively). CONCLUSIONS: Socioeconomic and demographic factors appear to influence the presentation and management of patients with CM-I and SM. Race is associated with age and timing of diagnosis as well as operating room time, estimated blood loss, and length of hospital stay. This exploration of socioeconomic and demographic barriers to care will be useful in understanding how to improve access to pediatric neurosurgical care for patients with CM-I and SM.

Congenital Cervical Tethered Spinal Cord in Adults: Recognition, Surgical Technique, and Literature Review.

OBJECTIVE: Recognition of congenital tethered cervical cord in adults and literature review. METHODS: Retrospective review of adult onset tethered cervical cord patients (age >20 years). RESULTS: Three adults were identified; 2 women and 1 man, average age 47 years. The presenting symptoms were neck pain with restricted movement (3), quadriparesis (2), and sensory changes (2). Hyperreflexia was present in all three. Bony abnormalities were mainly bifid cervical spinous processes (3), with Klippel-Feil abnormalities in 1. The neurocutaneous stigmata was seen in 2. Magnetic resonance imaging revealed "limited dorsal myeloschisis" in all 3 patients. The dorsal aspect of the cervical cord extruded into the tract leading to the surface. CONCLUSIONS: Neurocutaneous stigmata should not be considered benign. A missed clinical diagnosis was apparent in all 3 patients; 2 of whom underwent surgery with excellent results. Magnetic resonance imaging can identify the abnormal cervical cord protruding towards the "sinus tract" and allow planning to avert injury to the spinal cord during release.

Fourth ventricle roof angle as a measure of fourth ventricle bowing and a radiographic predictor of brainstem dysfunction in Chiari malformation type I.

OBJECTIVE: Chiari malformation type I (CM-I) is a congenital and developmental abnormality that results in tonsillar descent 5 mm below the foramen magnum. However, this cutoff value has poor specificity as a predictor of clinical severity. Therefore, the authors sought to identify a novel radiographic marker predictive of clinical severity to assist in the management of patients with CM-I. METHODS: The authors retrospectively reviewed 102 symptomatic CM-I (sCM-I) patients and compared them to 60 age-matched normal healthy controls and 30 asymptomatic CM-I (aCM-I) patients. The authors used the fourth ventricle roof angle (FVRA) to identify fourth ventricle "bowing," a configuration change suggestive of fourth ventricle outlet obstruction, and compared these results across all three cohorts. A receiver operating characteristic (ROC) curve was used to identify a predictive cutoff for brainstem dysfunction. Binary logistic regression was used to determine whether bowing of the fourth ventricle was more predictive of brainstem dysfunction than tonsillar descent, clival canal angle, or obex position in aCM-I and sCM-I patients. RESULTS: The FVRA had excellent interrater reliability (intraclass correlation 0.930, 95% CI 0.905-0.949, Spearman r2 = 0.766, p < 0.0001). The FVRA was significantly greater in the sCM-I group than the aCM-I and healthy control groups (59.3° vs 41.8° vs 45.2°, p < 0.0001). No difference was observed between aCM-I patients and healthy controls (p = 0.347). ROC analysis indicated that an FVRA of 65° had a specificity of 93% and a sensitivity of 50%, with a positive predictive value of 76% for brainstem dysfunction. FVRA > 65° was more predictive of brainstem dysfunction (OR 5.058, 95% CI 1.845-13.865, p = 0.002) than tonsillar herniation > 10 mm (OR 2.564, 95% CI 1.050-6.258, p = 0.039), although increasing age was also associated with brainstem dysfunction (OR 1.045, 95% CI 1.011-1.080, p = 0.009). A clival canal angle < 140° (p = 0.793) and obex below the foramen magnum (p = 0.563) had no association with brainstem dysfunction. CONCLUSIONS: The authors identified a novel radiographic measure, the FVRA, that can be used to assess fourth ventricular bowing in CM-I and is more predictive of brainstem dysfunction than tonsillar herniation. The FVRA is easy to measure, has excellent interrater variability, and can be a reliable universal radiographic measure. The FVRA will be useful in further describing CM-I radiographically and clinically by identifying patients more likely to be symptomatic as a result of brainstem dysfunction.