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Childs nervous system. November 22, 2021. doi: 10.1007/s00381-021-05409-z. Online before printing.
Purpose: Cervical kyphosis is rare in the pediatric population. It may be syndrome or acquired secondary to laminectomy, neoplasia, or trauma. In any case, this situation should be avoided to prevent progressive spinal deformities and neurological deficits. Long-term follow-up is required to assess fusion status, spinal growth, potential instability, and neurological function.
Methods and materials: A retrospective study was conducted on 27 children with cervical kyphosis (6 months to 16 years), limited to the MRI era, until 2008, to provide long-term follow-up, after which complicated equipment can be used. There are 27 patients, 19 syndrome patients (average age 5.36 years) and 8 non-syndromic patients (average age 14 years). The syndromes encountered are spine epiphyseal dysplasia (SED) 4, spinal metaphyseal dysplasia 1, unnamed collagen abnormal syndrome 1, osteogenesis imperfecta (OI) 2, Aarskog syndrome 1, Weaver syndrome 1, Larsen syndrome 1, multiple cervical segmental disconnection syndrome 1, Klippel-Feil 3, congenital deficiency C2 Part 4. Non-syndromic cases; 2 and neurofibromatosis (NF1) and prevertebral tumors, fibromatosis 1. Spontaneous kyphosis 1 and after laminectomy 4. The factors considered are age, pathology, flexibility on cervical dynamic films, traction reduction and spinal cord compression. Patients with soft kyphosis receive dorsal fixation. Children with inflexible ventral compression/kyphosis have coronal traction. Unreducible kyphosis includes ventral decompression fusion and dorsal fusion. 11 of the 19 children with flexible and reducible kyphosis syndrome received only dorsal fixation. Four of the eight non-syndromic (2 NF1) require ventral and dorsal methods.
Results: The preoperative deformities (global and local Cobb angle) and neurological status were improved. The growth was not impaired during the follow-up, and we did not experience instability or kyphosis at the junction. The only complication occurs in patients with the syndrome. A SED patient showed delayed cantilever bending of the ventral fusion block and required another operation. Another OI child had C5 and C6 nerve root weakness after C4 and C5 anterior decompression, which was resolved within 1 year. A child with SED developed scoliosis at the cervical-thoracic junction 18 years after thoracic scoliosis surgery.
Conclusion: The early pathology of the syndrome is neurological dysfunction, and 24% have rigid kyphosis. The attempt to pull/reset was successful, as shown in Table 1 and Table 2. Most people show long-term improvements in kyphosis and function. The treatment algorithm and literature review are proposed. Table 1 Modified Japanese Orthopaedic Association (JOA) Children's Motor Function Score [24, 37] Score Upper limbs • Cannot handle or eat 0 • Can handle; Cannot eat with a spoon 1 • Can eat with a spoon with difficulty 2 • Can eat with a spoon; button Buckle clumsy 3 • Healthy; no dysfunction 4 Lower limbs • Cannot sit or stand 0 • Cannot walk without crutches or walkers 1 • Walk independently on a level floor, but need support on stairs 2 • Can walk, Clumsy 3 • No dysfunction 4 Table 2 Children with cervical kyphosis-preoperative assessment case ID, year, age, gender, diagnosis, display image, Apex Cobb angle, reducible preoperative traction syndrome #1 2003 4 years M SED Progressive quadriplegic bladder Severe incontinence C2-4 Kyphosis with umbilical cord compression C3-4 85° No No #2 2001 3 years M SED Progressive quadriplegia C2-3 Kyphosis. There is no dorsal C2. Umbilical cord buckle C2-3 25° No No Recurrent weakness after recovery after 2 years C2-3 33° No No #3 1997 13 M SED Neck pain. Weak hands. Thoracic scoliosis C1-3 Kyphosis Os odontoideum C2-3 30° Yes No #4 2006 6 years F SED Hand tingling bladder incontinence C2 Body deformation and odontoid process C1-2 Instability C2-3 27° Yes No # 5 1997 M SMD quadriplegia in 4 years. The previous C2-3 kyphosis was fused with the dorsal O-C3 elsewhere to fix the C1-2 dislocation. C2-3 Kyphosis. O-C4 fusion C2 35° part is 4 days #6 2007 13 F Syndrome Collagen Abnormal Neck Pain. Differences in leg length. TL Scoliosis. C2 and C3 on both sides of tetraplegia, CT spinal dissolution, C2-3, 35°, part of left scoliosis, 4 days #7 2003 14 F Osteogenesis imperfecta (OI) Only the right upper limb C3-5 kyphosis can be used. C4 C4 25° No No #8 1989 3 years F OI-Brooke's syndrome quadriplegia for 9 months. C1-C3 posterior decompression and fusion have been performed elsewhere. Progressive kyphosis. More severe weakness and bending fusion. C1-2 40° No No #9 1996 11 M Aarskog syndrome Neck pain and limited neck movement Cervical myelopathy psychomotor delay C4-5 spondylolysis C5-6 kyphosis C5 30° no yes 3 days#10 1989 3½ years old F Weaver syndrome quadriplegia 2 years old. In other places C1-C3 dorsal rib fusion and wire fusion failed C2-3 subluxation umbilical cord compression C2-3 3° Yes Yes 1 day #11 1986 11 F Larsen Syndrome Neck Stretching Pain Quadriplegia C2- 3 Kyphosis. Deformed body C2-5 Os odontoideum C1-2 Instability C2-3 28° Yes Yes 1 day#12 1996 5 years M Multi-grade cervical spondylolisthesis syndrome Right Horner pupil quadriplegia of small right arm C4, C5 vertebrae Body C5 Back C5 The left side of the body in the spinal canal C5 The vertebral artery in the body C4-5 35° No No #13 1985 3 Years F Klippel-Feil Neck pain. Weak hand Atlas Assimilation C3-4 Kyphosis without posterior arch C3, C4 C3-4 40° Whether #14 1994 3 F Klippel-Feil cannot sit down. floppy disk. Tetraplegia C2-3 kyphosis without posterior arch C2-3 and L4 C2-3 45° Whether #15 1993 11 months F Tuberous sclerosis spondylolysis C2 Salam seizure Quadriplegia C2 C2-3 kyphosis C2-3 30° Whether #16 1998 2 years M C2 spondylolysis quadriplegia, arms are more severe than legs C2 spondylolysis C2-3 kyphosis C2-3 35° whether #17 1998 6 months M C2 spondylolysis failed to grow Arm weakness after apnea endoscopy C2-3 Kyphosis without C2 Laminar line C3-4 MRI C2-3 45° Yes No #18 1990 4 years F C2 spondylolysis Quadriparesis C2 spondylolysis C2-3 Kyphosis C3 45° Yes No #19 1994 4 years F Klippel-Feil no posterior C2 torticollis age 6 months tetraplegia C2-3 kyphosis no posterior arch C2 fusion C3-4 body C2-3 45° is it non-syndrome #20 1996 15 years M NF1. Ventral prevertebral plexiform neurofibroma neck pain weak arm cervical spondylotic myelopathy C4-5 kyphosis spinal cord covered on C4-5 enhanced prevertebral tumor C4-5 60° part is 4 days #21 1996 6 years M NF1 6 months with C1-3 laminectomy, laminectomy elsewhere, tetraplegia C3-5 plexiform neurofibroma C2-4 kyphosis C3-4 45° No No #22 1993 11 years old M "Fibromatosis" Neck pain sore throat↓ Right hemiplegia C2 Body and odontoid back bend C2-3 Kyphosis C2 40° No Yes 3 days# 23 2007 13 F Middle cervical kyphosis Neck pain cannot move neck C3-4 Kyphosis C3-4 45° is a halo vest elsewhere and repeated traction during 6 weeks of referral #24 1998 12 M Chiari I Spinal cord edema Dysphagia Quadriplegia and previous C1 posterior fossa-3 decompression base Inverted segment C3-4 Kyphosis C3-4 50° is halo traction for 3 days #25 1994 16 years old M Chiari I. SHM Difficulty in speech quadriplegia Previous posterior fossa and C1-4 laminectomy C3-4 Kyphosis deformity basal ganglia invagation C3-° is halo traction for 3 days #26 2002 11 M Chordoma C3-5 Initial tetraplegia improved after posterior decompression and then worsened dorsal and lateral tumors C3-4 C3-4 20° is traction for 3 days #27 2006 13 year M C4 laminar aneurysm bone cyst neck and shoulder pain C4 laminectomy tumor resection worse 4 months later C4-5 kyphosis C3-4 40° yes Table 3 Children with cervical kyphosis-postoperative evaluation case ID diagnosis and treatment-surgical complications PO orthosis F/U time fusion status Preop Cobb Postop Cobb Preop JOA postoperative JOA review syndrome #1 SED Corona traction 1. Median mandibular tongue Incision. Resection of C2-3 body and rib graft fusion 2. Dorsal O-C3 rib graft fusion no halo vest 3 months soft collar 3 months 8 years complete anterior and posterior fusion 85° 10° 2 8 complete nerve function recovery #2 SED crown Halo traction 1. Median mandibular tongue incision. C2-4 Resection. C2-5 Recurrent weakness halo vest after 2 years of anterior rib graft fusion, 3 months 2 years fusion 25° 20° 4 5 T. Scoliosis. Heart abnormality. Walk and re-do the tetraplegia with C1-4 iliac bone graft and re-do abdominal resection and C1-4 iliac bone graft. Minerva brace for 1 year and 18 years fusion 33° 15° 3 5 6 months, there is a great improvement #3 SED Coronary traction Dorsal O-C4 fusion with loop and rib grafts without Miami J collar 3 months 10 years Fused 30° 13° 4 7 Working in a bookstore #4 SED Corona traction dorsal O-C3 fusion with loops and rib grafts 4 years Later T. Scoliosis developed into CT scoliosis after surgery. Miami J collar 3 months and 14 years fusion 27° 5° 5 7 CT scoliosis developed into thoracic scoliosis after correction #5 SMD Coronary traction via mouth C2 teeth Process resection without Minerva brace 6 months 20 years preoperative status 35° 10° 1 4 Sit in a wheelchair. As a programmer #6 Collagen abnormal coronal traction C2-5 ACDF C2-5 plate and C3-4 lag screw scoliosis correction 7 years later kyphotic deformity Miami J collar 6 weeks 12 years Fusion 36° 5° 4 7 The vertebral artery is abnormal. Thoracic outlet syndrome May-Thurner syndrome #7 OI Corona traction C3-5 Osteotomy C2-6 Orion plate and iliac crest graft without soft collar 4 years fusion 25° 30° 15 Restrictive lung disease. Multiple fracture expiration #8 OI-Brooke syndrome 1. Rework C1-2 dorsal rib graft fusion unchanged Molded Minerva brace 4 years fusion 40° 35° 3 4 age 7 2. C3-7 reduction before 11 years old Compression and plate C3 -7 Deterioration of left deltoid and biceps function Molded Minerva brace 30 years fusion 52° 34° 3 5 Living alone. wheelchair. Computer technology expert hand is very good #9 Aarskog syndrome Corona traction C2-6 Anterior cervical fusion and iliac crest graft moldless Minerva brace fusion 30° 14° 4 7 Working on the farm. There is no myelopathy. Syndrome in the family #10 Weaver syndrome Corona traction Redo C1-4 dorsal rib graft fusion without Miami J collar 2 years fusion 3° 10° 25 Neuroblastoma 3 months. Chemotherapy Stable #11 Larsen Syndrome Coronary Traction O-C5 Dorsal Fusion Halo-free Vest 6 weeks Miami J 3 months 6 years Fusion 28° 10° 3 7 Good performance#12 Multi-stage cervical vertebral disconnection syndrome Coronary traction C5 Vertebral resection C4-6 Anterior iliac fusion dorsal C4-6 Fusion without Halo vest 3 months 5 years Fused 35° 5° 3 7 Persistent Horner pupil#13 Klippel-Feil Corona traction C2-6 Posterior rib graft Fusion without Halo vest 3 months 19 years Fused 40° 12 ° 3 7 Hearing loss Urogenital system abnormality Sprengel malformation #14 Klippel-Feil Corona C2-5 Dorsal rib graft fusion no halo vest 3 months 35 years Fusion 45° 10° 1 6 Hearing loss Urogenital system abnormality#15 Tuberous sclerosis Spondylolysis C2 C1 Dorsal interlaminar rib fusion without halo vest 3 months 6 years fusion 30° 5° 1 6 Psychomotor delay#16 C2 Spinal lysis C1-4 Dorsal interlaminar fusion non-faint vest 3 months 4 years fusion 35° 10° 2 6 Fully functional within one year #17 C2 Spondylolysis tracheostomy Molded cervical thoracic brace No molded brace 4 Formation of C2 posterior arch 45° 20° at 6 years 1 3 4 years with modified C2 on CT. Parents do not want surgery #18 C2 Traction during spondylolysis C1-3 Dorsal rib graft fusion without neck brace 4 months 8 years fusion 45° 12° 2 5 Development of C2 posterior element #19 Klippel-Feil intraoperative traction O-C4 fusion rib grafting without molding brace 6 months 1 year fusion O-C2 dorsal 45° 16° 1 4 Able to sit and use Hand non-syndrome #20 NF1 ventral tumor resection C3-6 C4-5 resection; C4-5 iliac bone graft; C3-7 Orion plate no halo vest 6 weeks 14 years fusion 60° 15° 3 7 within 6 weeks recover. Work on the farm #21 NF1 intraoperative traction removal of prevertebral tumors C2-5 kyphosis; C2-6 anterior fusion iliac crest without Halo vest 3 months 2 years fusion 45° 20° 3 5 Initial completed elsewhere C1-3 Decompression #22 Fibromatosis 1. Oral C2 decompression 2. Dorsal O-C3 and loop fusion without stent 3 months 12 years Fused 40° 12° 46 2 years old I have removed the neck mass. Diagnosis of "Fibromatosis" #23 Cervical kyphosis deformity Traction C2-5 Lateral mass fusion screw, rod and rib grafts. Worse after initial traction. Bracket 3 months 8 years fusion 45° 15° 7 8 Good performance #24 Chiari I SHM intraoperative traction O-C5 rib graft fusion without Halo vest 3 months 21 years fusion 50° 7° 2 6 small joints C2 and C3 atrophy during surgery #25 Chiari I SHM 22 years fusion 55° 10° 3 6 Small joints atrophy during surgery C2-4 #26 Chordoma C3-4 1. Dorsal C3-6 fusion 2. C2-5 Anterior fusion of ilium without Miami J brace 6 months 18 years fusion 20 ° 12 ° 5 8 Hand weakness after initial surgery elsewhere #27 ABC Tumor C4 Front C3-5 Fusion with plate and bone without Miami J brace 4 weeks 12 years fusion 40° 15° 5 8 No recurrence SED Spine epiphyseal dysplasia, SMD Spinal metaphyseal dysplasia, JOA Japan 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.
PMID: 34806157 | DOI: 10.1007/s00381-021-05409-z