Реабилитация пациентов в отдаленном периоде травмы спинного мозга: метаанализ литературных данных

Objective. To perform validation study of the E. Pola classification (2017) and to assess expert consensus on the diagnosis and treatment of infectious lesions of the spine in the national professional community.Material and Methods. A clinical and radiological database on 15 cases of infectious spondylitis, as well as the information about original article by Pola and a Russian translation of the classification and tactical tables from this article, were distributed to 408 orthopedic traumatologists, neurosurgeons and radiologists who have experience in treating patients with spinal pathology and whose data are available in the registers of the relevant professional associations of the Russian Federation. The coincidence/difference in the responses concerning the definition of lesion types and the choice of treatment tactics, as well as proposals for the use of classification were assessed.Results. Answers were obtained from 37 respondents from 11 regions of the Russian Federation. The general interobserver agreement index (Fleiss kappa) for all types of spondylodiscitis was 0.388 (95 % CI 0.374–0.402), including for lesion types: type A – 0.480 (95 % CI 0.460–0.499, type B – 0.300 (95 % CI 0.281–0.320), and type C – 0.399 (95 % CI 0.380–0.419). Agreement levels were higher among radiologists (type A – 0.486, type B – 0.484, and type C – 0.477), orthopedic traumatologists (type A – 0.474, type B – 0.380, and type C – 0.479), and specialists with clinical experience less than 10 years (type A – 0.550, type B – 0.318, and type C – 0.437). The pooled data for all 12 lesion subtypes showed general poor agreement (k = 0.247, CI 0.240–0.253), satisfactory level was found for B3.2 type (k = 0.561, CI 0.542–0.581), good agreement (k > 0.61) was achieved between orthopedic traumatologists for type B3.2 and between radiologists for B3.1 and B3.2 lesion types. Respondents refused to use basic treatment options for type A in 15.1 %, type B in 7.5 % and type C in 3.2 % of answers, while indicating the need for interventions through anterior approach in 24.7 %, 43.0 % and 46.2 %, respectively. Limitations of the classification use depending on the localization and etiology of spondylitis were noted. Authors recommended taking into account the presence of systemic inflammatory response syndrome, mandatory CT scanning, clarification of spinal instability criteria, and the addition of anterior surgical interventions to the treatment algorithm.Conclusion. The Pola classification of spondylodiscitis is currently considered the most successful for tactical algorithms and implementation in broad clinical practice for spondylodiscitis. However, at the stages of its clinical application, there is an unsatisfactory interobserver expert consensus on the types of lesions, and there are limitations related to the etiology, localization and severity of the disease. A modified classification taking into account the identified limitations and including anterior procedures in the tactical options is advisable.

Objective. To analyze feasibility and safety of C1, C2 fixation for craniocervical stabilization in patients of different age groups.Material and Methods. Study design is a retrospective multicenter cohort analysis. Level of evidence – II. The study was based on the diagnosis and treatment data of 43 patients aged 5–74 years who underwent C1, C2 screw fixation.Results. In most cases, atlantoaxial instability was due to traumatic injuries. Screws were implanted in C1 in 81 cases, and in C2 in 80. Postoperative MSCT data were evaluated in 41 patients. The position of the screws in C1 was defined as ideal in 63,0 % of cases, in the remaining cases there were malpositions, with 6 screws having double malpositions. Out of 80 screws inserted in C2, 64 were implanted transpedicularly. In 59.5 % of cases, a good position was revealed; in other cases different malpositions were noted. In none of the cases neurovascular damage or clinical manifestation occurred.Conclusion. Analysis of screw fixation of C1, C2 showed that this technique is feasible in patients of different age groups. Duration of surgery and blood loss did not go beyond the conventional values. Postoperative malpositions are not accompanied by neurological disorders, which allows to consider this fixation quite safe.

Background: We describe patients who were recognized via conventional radiographs of the skull as manifesting wormian bones. Wormian bones are not a specific diagnostic entity and can be seen in variable forms of syndromic disorders. Materials and Methods: Seven children and three adults (of 10–28 years) were seen and diagnosed in our departments. The principal complaints for the pediatric and adult group were: ligamentous hyperlaxity, a history of delayed walking and occasional fractures, which later in life started to manifest a constellation of neurological symptoms such as nystagmus, persistent headache, and apnea. Conventional radiographs were the first traditional tools used to recognize wormian bones. We performed 3D reconstruction CT scans to further understand the precise etiology and the nature of these wormian bones and attempted to connect them with a broad spectrum of unpleasant clinical presentations. Our group of patients was consistent with the phenotypic and genotypic diagnoses of osteogenesis imperfecta type I and type IV as well as patients with multicentric carpotarsal osteolysis syndrome. Results: Three-dimensional reconstruction CT scan of the skulls confirmed that these worm-like phenotypes are in fact stemmed from the progressive softening of the sutures. The overall phenotype of the melted sutures is akin to overly stretched pastry. The most concerning sutures in this pathological process are the lambdoid. The overstretching of the lambdoid sutures was responsible for the development of sub-clinical basilar impression/invagination. Patients with certain forms of skeletal dysplasia such as osteogenesis imperfecta type I and IV manifested the heterozygous mutation of COL1A1/COLA2, shown as typical overstretching of the sutures. Similarly, patients with multicentric carpotarsal osteolysis syndrome with a heterozygous missense mutation of MAFB also manifested the phenotype of overly stretched pastry along the skull sutures. Conclusion: What we encountered via 3D reconstruction CT scan in our group of patients was entirely different than the traditional description that can be found in all relevant literature of the last decades. The worm-like phenomenon is in fact a pathological sequel occurring as a result of a progressive softening of the sutures, which results in the overstretching of the lambdoid sutures, a pathological process roughly similar to an overly stretched soft pastry. This softening is totally connected to the weight of the cerebrum (the occipital lobe of the cerebrum). The lambdoid sutures represent the weight-bearing zone of the skull. When these joints are loose and soft, they adversely alter the anatomical structures of the skull and lead to a highly hazardous derangement of the craniocervical junction. The latter causes the pathological upward invasion of the dens into the brain stem, leading to the development of morbid/mortal basilar impression/invagination.

Background: The diagnostic process for children and adults manifesting a constellation of ectodermal abnormalities requires a conscientious and highly structured process. Material and Methods: Six girls (aged 6-month–8 years) and two older girls (aged 13 and 16 years) were born with variable skin lesions of varying intensities associated with noticeable cranial and skeletal malformation complexes. Cleft palate, abnormal dentition, and multiple papillomas were evident around the mouth, mostly bilateral but asymmetrical in the upper and lower limbs. Exaggerated frontal bossing (macrocephaly) and in some patients’ microcephaly with variable skeletal defects of the craniocervical junction and diverse forms of lower limb deformities of syndactyly, polydactyly, and split-hand/foot (ectrodactyly). Results: All patients manifested the constellation of abnormalities with variable intensities ranging between alopecia, papillomas, striated skin pigmentations split-hand/foot (ectrodactyly), and major bone defects. A 3D reconstruction CT scan was directed mainly to further scrutinize children with pseudo cleft lip, submucus cleft, and cleft palate. Interstingly, they manifested massive demineralization of the cranium associated with severely defective dentition. A spine 3D reconstruction CT scan in two girls showed marked cystic cavitation of the upper jaw associated with excessive cavitation of the mastoid, causing tremendous frailty of the mastoid bone. A 3D sagittal CT scan showed odontoid hypoplasia and C1-2 instability associated with the rudimentary atlas and the persistence of extensive synchondrosis of the cervico-thoracic spine. The overall clinical and radiological phenotypic characterizations were consistent with the diagnosis of focal dermal hypoplasia (Goltz syndrome). Two children manifested heterozygous mutations in the PORCN gene, chromosome Xp11. Conclusions: In this study, we believe it’s a good opportunity to share our novel scientific findings, which are intriguing and can be inspiring to readers, and to further aid the current scientific literature with exceptionally new unveiling results. This is the first comprehensive study of the cranio-skeletal malformation complex in children with GS.