In this review are discussed the most important questions of diagnostics, surgical treatment and complications in the context of  anaesthesia choice and performance in  children and adolescents with different variants of  vertebral scoliotic deformity. Vertebral scoliotic deformity is a multi-etiological disease and significant clinical problem due to frequency and severity of complications when disease progresses. Surgery is performed in  severe and super severe scoliotic deformity when conservative treatment is not effective. Surgical treatment of severe scoliotic deformity allows to improve physiological function of vertebral column and internal organs’ function, improve quality of life and increase life expectancy. Surgical treatment of severe scoliotic deformity is one of the most complex problem in traumatology and orthopedics which requires multidisciplinary coordination of surgeon, anesthesiologist, narrow specialists and the patient at every step of treatment. The most significant problems in surgical treatment of severe scoliotic deformity are forecasting and blood loss management during the operation and in post-operative care which could reach several circulating blood volumes. The problems of blood loss minimization are discussed: patient’s position on operating table, acute normovolemic haemodilution, managed hypotension, use of  antifibrinolythic medications, blood collection and re-infusion, blood transfusion. Blood loss forecasting is an important instrument to get a proactive information to develop personalized approach to patient’s care with assessment of intraoperative blood loss, risk of hemorrhagic shock and disseminated intravascular coagulation syndrome.

BACKGROUND: Surgeries to correct scoliotic spinal deformity (posterior corrective transpediculocorporal fusion) are classified as highly traumatic, are accompanied by significant blood loss, and require reliable venous access. Central vein catheterization is an important part of patient management and is a successful and safe procedure. AIM: To evaluate the effectiveness of ultrasound navigation during central venous catheterization in patients with severe and super-severe scoliotic spinal deformity. MATERIALS AND METHODS: A single-center prospective study included 52 patients aged 6 to 18 (median age 13.2) years undergoing surgical treatment to correct grade IV scoliotic spinal deformity. Patients underwent catheterization of the internal jugular vein under ultrasound navigation using an ultrasound scanner with a linear sensor and a frequency of 713 MHz. The procedures were performed by one operator. The following were assessed: anatomy of the neurovascular bundle, relative position of the vessels relative to each other, size of the internal jugular vein in a horizontal and Trendelenburg positions, frequency and time of the procedure, and complications during puncture and catheterization. RESULTS: In patients with severe scoliotic deformity of the spine, an atypical location of neck vessels was noted in every fifth patient (13.46%). The peculiarity of the location of the vessels was associated with congenital developmental anomalies. The most common anomaly in the location of the vessels relative to each other was the medial location of the internal jugular vein relative to the carotid artery. In one patient, the passage of the internal jugular vein at a considerable distance from the carotid artery was revealed, which made it impossible to puncture according to anatomical landmarks. The average diameter of the internal jugular vein in the horizontal position was 6.20.9 mm. In the Trendelenburg position, the diameter was 9.081.5 mm. The average duration of the procedure was 92 seconds (70). Taking into account the use of ultrasound navigation during catheterization of the internal jugular vein, no early and late complications occurred. CONCLUSION: The use of ultrasound navigation for central venous catheterization during surgical treatment of severe and super-severe scoliotic deformities of the spine is a safe and essential method. The Trendelenburg position allows for better visualization of the jugular vein and facilitates its puncture and catheterization. The use of ultrasonography during invasive vascular manipulations allows for minimizing the number of failed catheterizations and avoiding complications, which improves the efficiency of medical care and increases the level of comfort and safety for the patient.

Kniest dysplasia is a disease that is inherited in an autosomal dominant manner. It manifests itself as dwarfism, scoliotic deformity of the spine, impaired joint mobility, muscle weakness, visual impairment, and sensorineural deafness. As a result of disproportionate trunk shortening, lumbar hyperlordosis and kyphoscoliosis develop, leading to internal organs (respiratory, cardiovascular system) disorders, disability, and reduced life expectancy. A case of surgical treatment of a patient with Kniest dysplasia for severe kyphoscoliotic spinal deformity is described. Posterior corrective cross-rod transpediculocorporal screw spondylodesis T3-L5 with bone autoplasty was performed. While planning anesthesia, difficult tracheal intubation was evaluated on the LEMON scale of 7 points high-risk. While performing tracheal intubation, endoscopic techniques were used: videolaryngoscope, intubation bronchoscope, enabling success. Management of intraoperative blood loss was conducted by a complex of measures: laying the patient in the prone position with the release of the abdominal cavity, normothermia, intraoperative hemodilution of azlactone-balanced polyionic solutions to achieve the target hematocrit in the range of 24%26%, and controlled hypotension with blood pressure decreased by 30% from the original hardware blood reinfusion during surgery. Also, on the first postoperative day, fusing tranexamic acid, correcting anemia and deficiency of blood coagulation factors donor components contributed to the success. Discussion. When planning surgery and anesthesia, it is necessary to consider the risk of developing malignant hyperthermia, predicting difficult intubation, and complying with the algorithm to ensure airway patency and prevent massive intraoperative blood loss. With a comprehensive approach to patient management, it is possible to achieve rapid rehabilitation and discharge for outpatient treatment. Surgical treatment for rapidly progressing severe kyphoscoliathical spinal deformity can change the quality and duration of life in patients with Kniest syndrome.