Skeleton Pathology


May 11, 2021

This paper conducted by presents a case study of the two people, who suffered from progressive scoliosis combined with cervical-thoracic localization of severe disease in the examples Olier and myopathy Rossolimo. Patients were two little children, who had functional impairments that were dangerous for their lives. Surgery was likely to improve their state.

Progressive spinal deformations, together with the basic pathology, the so-called high risk cases, and regardless the etiology combine rare occurrence, atypical localization, the values of the arc of curvature, the rate of progression of violent, often involving problems with chest, pelvis, in violation of function of the chest and the supporting skeleton through static-dynamic imbalance. Everything mentioned above leads to a sharp decline in the quality of life and its duration, which determines the urgency of the problem. Traditionally, such cases are incurable, underscoring the need for early treatment of children with systemic spinal deformities. Traditionally, halo-femoral tractions are preferred in such cases. Children, who have preserved potency of growth, are cured with the usage of connector type systems with the ability of stage distraction. Both children had stator dynamic and functional impairment, which not only negatively affect the quality of life, but also threatened it. One of the patients had somatic problems, which made him stay in hospitals for a long time. Yet, it was clear that those patients needed to be cured, because without any help they would have died. Although the two patients were cured, this paper would contain detailed description of the treatment process of one of them.

Patient P., 11 years old, consulted a vertebrologist for the first time because of spine deformation in July. In the history of his treatment, it is written that he has been observed for a year already, because of limbs deformation. The doctor subscribed him conventional treatment: legs massage and wearing orthopedic shoes. In 2001, a geneticist verified Ollier disease; however, it has been progressing only for the last two years. Nowadays, the patient has a disproportionate figure, expressed cachexia (weight 18 kg), decompensated extended right-sided cervical-thoracic scoliosis curve with a deviation of the torso to the right and head to the left and 90  in Cobb , gibbus right, hypertrophy of the neck muscles, more on the right, clinodactyly with hypertrophy of the phalanges, funnel chest I degree. Severe stiffness strain at the traction test. Minimum exercise (walking up to 20 m) provoked increasing respiratory failure. Neurological status: nervous and mental development are appropriate to the patients age. He has symmetrical face. The patient is unstable in Romberg pose; performs coordination tests with intention. The tendon reflexes in the upper and lower extremities are lively and balanced. The strength of muscles of the upper extremities is 5 points, whereas the strength of lower extremities is of three points (for ASIA). Sensory disturbances and disorders of the pelvic organs are not found; patient has fatigue with episodes of hypoxic conditions (suspend).

At ray examination, atypical spinal deformity with the top of the arc on the main level CVI-VII vertebrae, vertebral-medullary absence of conflict and fibrotic changes in the lung tissue was revealed. Child was recommended to be hospitalized for further examination and decision on the possibility of surgical correction. However, admission to the clinic was delayed, because the patient had flue. Emergency hospitalization to a state childrens hospital was performed on 01.08., in bad conditions with complaints of hemoptysis, cough, frequent apnea while sleeping that lasted up to 1 min. More likely, that it was a respiratory virus infection that provoked a complication of respiratory failure at the main heavy restrictive lung disease. In the intensive care unit, the patient was conducted prolonged mechanical ventilation, as well as syndrome and antibacterial therapy. Reducing ventilator settings resulted in an increase of hypercapnia.

The patient was examined by the team of spine specialists on the fourth of August. Given the severity of the syndrome leading to respiratory failure with severe decompensated scoliosis, clinical history, location and amount of strain, it was decided to correct the deformity with the usage of halo-pelvic apparatus, as it is the least invasive, simple to perform, and adequate method for the stabilization of the external fixation device of the patient. Adjusting the halo-pelvic apparatus took place on the fifth of August; it was held in distraction regime in order to correct the spine deformity, increase the volume of chest cavity, and create favorable conditions for excursion of light. The surgery lasted for thirty-five minutes. The volume of blood loss up to 20 days for ml. during the next day normalization parameters to saturation was observed. However, on the sixteenth of August, due to an increasing respiratory distress tracheostomy was performed. The patients condition has stabilized 2 days later (18/08/), the patient transferred to the spontaneous breathing through a tracheostomy, and in 7 days (25.08.) he was transported to the somatic compartment.

The patient was transferred from the state childrens hospital on 27.09., to the department of neurosurgery and spine surgery in one of the federal hospitals with a diagnosis of Olliers disease with multiple deformities of the axial skeleton and limbs; combined with scoliosis of IV degree (at Chaklin) due to systemic disease; progressive muscular dystrophy; right lung fibrosis; myocardiodystrophy; cachexia of the third degree; tracheostomy. Neurophisical examination that was performed did not reveal any deviations from the patients age norm. On admission to the hospital, a child alone could stand up to ten minutes, walk with support for up to 6 minutes without respiratory imbalance arises, and was powered with external assistance, the balance of the body in the frontal plane was improved while maintaining decompensation, sagittal balance compensated. The device is marked deformity correction at the main cervico-thoracic arch to 32  (-31 ), lumbar level associated arc 10  (-2 ). When spirometry decrease vital capacity to 43%.

On the twenty ninth of September 2011, the patient underwent the surgery: correction and fixation of posterior multisupporting instrumental segments CII-ThIX combined with system of a connector type; rear local spinal fusion; dismantling the halo-pelvic system. Blood loss was 200 ml (5% BCC). After such surgeries, cosmetic and functional effects are obvious and rather satisfactory. The functional result is confirmed by the data spirometry in a month after surgery in the form of an increase in vital capacity to 56%, load tests to improve respiratory function, restoring the balance of the body when walking and self-recovery capabilities. The patient walks with support for up to 30 minutes without respiratory distress, gets up and eats on his own, the balance of the body in the frontal and sagittal planes is compensated, as confirmed by the pace of increases weight, which is now 21 kg (+3 kg), as well as by the results of computer optical topography.

The paper aimed at showing the detailed case study of one of the skeleton diseases. However, in this example the patient had skeleton disease combined with complications. The eleven-year-old patient needed to undergo a surgery as soon as possible; however, he had lung problems as well, which interfered the process of his treatment. The patient has been sick for a long time, and his disease with such complications that he had is uncured; however, the surgeons managed to make his life not so painful.





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