RIB PENETRATION INTO THE SPINAL CANAL IN CASES OF SCOLIOSIS IN PATIENTS WITH NEUROFIBROMATOSIS TYPE-1 (case report and literature review)

Background. Rib penetration into the spinal canal in patients with scoliosis secondary to neurofibromatosis type-1 (NF-1) was described in a relatively small number of publications, though it’s common in clinical practice and not always diagnosed.Materials. The authors report on an adolescent male with NF-1 left thoracic kyphoscoliosis and rib head protrusion into spinal canal with mild initial neurological deficit.Results. A 14-year-old male patient with NF-1 and 68 degrees left thoracic scoliosis and 65 degrees kyphosis was undergoing treatment at the authors’ institution.Preoperative CT scans demonstrated protrusion of the left T11 rib head into the spinal canal on the convexity of the curve, without spinal cord compression. Surgical procedure for resection of the rib head and correction of the spinal deformity was performed which allowed to achieve good deformity correction. The authors also observed almost complete restoration of sensitive functions.Conclusion. Rib head protrusion into the spinal canal can occur in cases of spine deformities with NF-1. If present, the imaging findings should be carefully reviewed for appearance of such lesion that may be obscured by the limitations of CT in the context of a dysplastic spinal deformity

The seasonal cycle of the greenhouse gas balance of a continental tundra site in the Indigirka lowlands, NE Siberia

International audienceCarbon dioxide and methane fluxes were measured at a tundra site near Chokurdakh, in the lowlands of the Indigirka river in north-east Siberia. This site is one of the few stations on Russian tundra and it is different from most other tundra flux stations in its continentality. A suite of methods was applied to determine the fluxes of NEE, GPP, Reco and methane, including eddy covariance, chambers and leaf cuvettes. Net carbon dioxide fluxes were unusually high, compared with other tundra sites, with NEE=?92 g C m?2 yr?1, which is composed of an Reco=+141 g C m?2 yr?1 and GPP=?232 g C m?2 yr?1. This large carbon dioxide sink may be explained by the continental climate, that is reflected in low winter soil temperatures (?14°C), reducing the respiration rates, and short, relatively warm summers, stimulating high photosynthesis rates. Interannual variability in GPP was dominated by the frequency of light limitation (Rg ?2), whereas Reco depends most directly on soil temperature and time in the growing season, which serves as a proxy of the combined effects of active layer depth, leaf area index, soil moisture and substrate availability. The methane flux, in units of global warming potential, was +28 g C-CO2e m?2 yr?1, so that the greenhouse gas balance was ?64 g C-CO2e m?2 yr?1. Methane fluxes depended only slightly on soil temperature and were highly sensitive to hydrological conditions and vegetation composition

РЕЗУЛЬТАТЫ ЭТАПНОГО ХИРУРГИЧЕСКОГО ЛЕЧЕНИЯ ИНФАНТИЛЬНЫХ И ЮВЕНИЛЬНЫХ СКОЛИОЗОВ С ИСПОЛЬЗОВАНИЕМ РАЗЛИЧНЫХ МЕТОДИК

Introduction. The analysis Results of surgical treatment of growing children with infantile and juvenile scoliosis (IS) can the optimal method of treatment select. In young children with significant growth potential spinal fusion may not be the best option as it limits further longitudinal growth of the spine and may to the thoracic insufficiency syndrome result. To address this problem recently several techniques focused, their have advantages and drawbacks.Material and methods. Since 2008 year 127 patients (64 girls, 63 boys) aged (4.5 ± 2.1) years were operated on. In group I 65 patients were operated on using VEPTR (Vertical Expandable Prosthetic Titanium Rib) instrumentation, in group II 42 patients using various spinal instrumentation. 20 patients with congenital kyphosis were excluded. The average follow-up time was (5.6 ± 1.1) years.Results. In group I average value of the primary scoliotic curve before surgery was (74.7 ± 22.9), secondary curve (42.8 ± 16.0), thoracic kyphosis (46.3 ± 27.4), lumbar lordosis (54.6 ± 14). Average value of the primary scoliotic curve after surgery was reduced to (51 ± 20) (correction 31.7%), at followup to (56.5 ± 18.5), secondary curve (31.8 ± 12.8) (25.7%), at follow-up to (32.4 ± 18.4), thoracic kyphosis (36.8 ± 20.8) (20,5%), at follow-up to (41.8 ± 21.0), lumbar lordosis (45.4 ± 12.7) (16,9%), at follow-up to (48.2 ± 11.7) (p < 0.05). Space available for lung before surgery was (84.5 ± 8.7) %, after surgery was (94.8 ± 6.7)%, at follow-up increased to (98.6 ± 5.4) % (p < 0.05). Complications included 11 implant dislocations and 1 infection. In group II average value of the primary scoliotic curve before surgery was (87.6 ± 6.6), secondary curve (47.8 ± 4.6), thoracic kyphosis (61.4 ± 10.4), lumbar lordosis (61.8 ± 4.9). Average value of the primary scoliotic curve after surgery was reduced to 50.6 ± 5.3 (correction 42.3%), at follow-up to (66.1 ± 6.3), secondary curve (24.1 ± 2.9) (49.6%), at follow-up to (37 ± 5.4), thoracic kyphosis (38.8 ± 7.7) (36.8%), at follow-up to (59.4 ± 11.2), lumbar lordosis (47.5 ± 4.1) (23.2%), at follow-up to (64.5 ± 4.5) (p < 0.05). Complications included 23 implant dislocations and 1 infection. No neurological complications.Conclusion. Stage correction fusions using various instrumentation is a method of choice for controlled correction of growing children with IS.Введение. Анализ результатов хирургического лечения сколиотических деформаций позвоночника у активно растущих детей позволяет выбрать оптимальный метод лечения. Стабилизация позвоночника является быть оптимальным вариантом, так как может привести к ограничению его дальнейшего роста и развитию синдрома торакальной недостаточности. На решение этой задачи в последнее время ориентировано несколько методик, имеющих свои достоинства и недостатки.Цель исследования – проанализировать результаты хирургического лечения инфантильных и ювенильных сколиозов с использованием различного инструментария.Материал и методы. В период с 1998 по2014 г. оперировано 127 детей (64 девочки и 63 мальчика) с инфантильными и ювенильными деформациями позвоночника различной этиологии. Операции проводились по двум методикам: первая группа (65 больных) – по методике VEPTR (Vertical Expandable Prosthetic Titanium Rib, США), вторая (42 больных) – с использованием дорсального сегментарного инструментария. Средний возраст начала лечения составил (4,5 ± 2,1) года (I группа), (7,6 ± 2,4) года (II группа). Сроки послеоперационного наблюдения составили (5,6 ± 1,1) года (от 6 мес до 12 лет).Результаты. В группе I средняя величина основной сколиотической дуги перед началом лечения составляла (74,7 ± 22,9)°, противоискривления – (42,8 ± 16)°, грудного кифоза – (46,3±27,4)°, поясничного лордоза – (54,6 ± 14)°. Величина основной сколиотической дуги после операции составляла (51,0 ± 20,0)° (коррекция 31,7%), противоискривления – (31,8 ± 12,8)° (коррекция 25,7%), кифоза – (36,8 ± 20,8)° (коррекция 20,5%), лордоза – (45,4 ± 12,7)° (коррекция 16,9%) (p < 0,05). В конце срока наблюдения величина основной сколиотической дуги составила (56,5 ± 18,5)°, противоискривления – (32,4 ± 18,4)°, кифоза – (41,8 ± 21,0)°, лордоза – (48,2 ± 11,7)° (p < 0,05). При использовании инструментария VEPTR отмечено увеличение отношения пространств доступных для легких в сравнении с исходным – (84,5 ± 8,7), послеоперационным – (94,8 ± 6,7) и значением в конце срока наблюдения – (98,6 ± 5,4) (p < 0,05). У 11 пациентов отмечена нестабильность захватов инструментария, 1 случай нагноения. Во II группе средняя величина основной сколиотической дуги перед началом лечения составляла (87,6 ± 6,6)°, противоискривления – (47,8 ± 4,6)°, грудного кифоза – (61,4 ± 10,4)°, поясничного лордоза – (61,8 ± 4,9)°. Величина основной сколиотической дуги после операции составляла (50,6 ± 5,3)° (коррекция 42,3%), противоискривления – (24,1 ± 2,9)° (коррекция 49,6%), кифоза – (38,8 ± 7,7)° (коррекция 36,8%), лордоза – (47,5 ± 4,1)° (коррекция 23,2%) (p < 0,05). В конце срока наблюдения величина основной сколиотической дуги составила (66,1 ± 6,3)°, противоискривления – (37,0 ± 5,4)°, кифоза – (59,4 ± 11,2)°, лордоза – (64,5 ± 4,5)° (p < 0,05). У 23 пациентов отмечена нестабильность захватов инструментария, 1 случай нагноения. Неврологических осложнений не отмечено.Вывод. При хирургическом лечении инфантильных и ювенильных сколиозов методом выбора являются этапные коррекции с использованием различного инструментария

Структурно-функциональные особенности деформации позвоночника при нейрофиброматозе NF-1

To study pathogenetic mechanisms of the development of spinal deformity in neurofibromatosis.Structural components of the spine were presented as specimens obtained after surgical correction of spinal deformity performed in 10 children with III—IV grade scoliosis associated with neurofibromatosis.Etiologic factor of the development of spinal deformity in neurofibromatosis is a mutation of the NF-1 gene in cells of ganglious lamella. Migration of cells carrying mutant gene into one of the sclerotome zones results in oncogene activation and intensive proliferation of chondro-, osteo-, and fibroblasts in the growth plate, intervertebral disc, and vertebral body.Progressive development of the spinal deformity after surgical intervention is accounted both for proliferation of chondro- and fibroblasts in the vertebral body and intervertebral disc and for disturbance of the NF-1 and lumican genes expression.Представлено изучение патогенетических механизмов формирования деформации позвоночника при нейрофиброматозе.Исследованы структурные компоненты позвоночника, полученные в ходе коррекции деформации от 10 детей с III— IV степенью сколиоза на почве нейрофиброматоза.Этиологическим фактором формирования деформации позвоночника при нейрофиброматозе является мутация в клетках ганглиозной пластинки гена NF-1. Миграция клеток, несущих мутантный ген в одну из зон склеротома, приводит к активации онкогена и интенсивной пролиферации хондро-, остео- и фибробластов в пластинке роста, межпозвонковом диске и теле позвонка.Продолженный процесс деформации позвоночника после оперативного вмешательства объясняется пролиферацией хондро- и фибробластов в теле позвонка и межпозвонковом диске и нарушением экспрессии генов люмикана и NF-1

Final fusion in a complex of surgical treatment for early onset scoliosis

Introduction: Surgical treatment of early onset scoliosis (EOS) is one of the most challenging problems of spine surgery and includes staged distraction and final fusion at the end of skeletal maturity that remains debatable.Aim: The objective of the review is to evaluate the efficacy of final fusion following staged distraction with VEPTR instrumentation in patients with EOS.Materials and methods: Outcomes of multi-staged operative treatment of 37 patients with EOS of different etiology were reviewed. Medical records and radiographs of the patients were retrospectively analyzed. Standing postero-anterior and lateral spine radiographs were used for the spinal radiologic assessment before and after each stage of distraction-based treatment, before and after final fusion and at the last follow-up.Results: The mean age of patients at baseline was 5.2 years and the mean age at final fusion was 13.9 years. All patients demonstrated decrease in the angle of primary (from 81.5° to 51.6°) and secondary (from 59.3° to 37.8°) curves, increase of the height and normalized body balance. The mean height increased from 104.8 cm to 141.0 cm, and the mean weight increased from 15 kg to 35 kg throughout the treatment period. The height of the thoracic and lumbar vertebra (Th1-S1) increased from 245 mm to 340 mm, and that of the thoracic vertebra – from 136 mm to 193 mm. There was a mean of 2.3 complications per patient during distraction performed in a staged manner, and they were arrested during elective procedures. There were 7 (19%) complications after final fusion that required 6 (16%) unplanned revisions. Radiologic evidence of spontaneous autofusion was seen in the lumbar spine of the patients with the inferior anchor at the lumbar vertebra.Conclusions: Multi-staged pediatric surgeries performed in the first decade of life facilitate radical changes in the natural history of progressive scoliosis and ensure satisfactory functional and cosmetic results despite multiple difficulties and complications. The VEPTR instrumentation used for the thoracic curve is unlikely to result in the spinal fusion of the major arch and this is the cause for the use of third-generation instrumented final spinal fusion in the patients.&nbsp