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Paper text:
a boy , 11 years old , was first seen for spinal deformity by a spinal surgeon in july 2011 .
the child 's medical history indicated that he had outpatient appointments with an orthopedic surgeon for limb deformities .
orthopedic status showed disproportionate stature ; evident cachexia with a weight of 18  kg ; decompensated , rigid , extended right - side cervicothoracic scoliosis with torso deviated to the right and head to the left up to 90 degrees of cobb 's angle ; gibbus on the right ; hypertrophic muscles of the neck more severe on the right ; clinodactyly and hypertrophied phalanges of the fingers ; and grade
photo of the patient at the intensive care unit of the regional children 's hospital .
coordination tests were performed with intention ; tendon reflexes of upper and lower limbs were brisk and symmetrical , and muscle strength of the upper limbs was graded as 5 and lower limbs as 3 according to american spinal injury association standard neurological classification of spinal cord injury .
emergency admission to the regional children 's hospital was required on august 1 , 2011 , for a severe condition with complaints of spitting up blood , coughing , and frequent episodes of sleep apnea lasting to 1 minute .
mechanical lung ventilation and antibacterial and syndrome - based therapy were performed at the intensive care unit .
imaging findings : ( a , b ) radiographs of the spinal column : c4 lateral nonsegmented hemivertebra on the right ( marked with arrow ) , scoliotic curve , and decrease in the volume of thoracic cage .
an external fixation device was applied for halo - pelvic traction by a team of spinal surgeons of the kurgan ilizarov center on august 5 , 2011 .
the frame was set up in the distraction mode of 6 cm to correct spinal deformity and to create favorable conditions for lung excursion ( fig .
however , tracheostomy was performed due to progressive respiratory failure on august 1 , 2011 .
the patient 's condition stabilized , and in 2 days he was transferred to autonomous breathing with tracheostomy and at 7 days he was transported to a somatic department ( on august 25 , 2011 ) . 

photo ( a ) and radiograph ( b ) of the patient following deformity correction with halo - pelvic system assembled of the ilizarov components .
the patient was referred from the regional children 's hospital to the neurosurgery department of the  russian ilizarov scientific centre restorative traumatology and orthopaedics  on september 29 , 2011 .
the diagnosis on admission indicated ollier disease with multiple deformities of axial skeleton , severe combined scoliosis with underlying systemic pathology and disturbed cervical spine formation , progressive muscular dystrophy , pneumofibrosis of the right lung , myocardiodystrophy , grade 3 cachexia , tracheostomy , and frame application for halo - pelvic traction . on admission to the center ,
the patient was able to stand independently for 10 minutes and walk with support for 6 minutes without respiratory distress or imbalance , but he needed help with his meals and getting up .
his torso balance improved in the coronal plane with persistent decompensation , but sagittal balance was compensated ( fig .
3a ) . deformity correction with instrumentation was achieved up to 32 degrees ( 31 degrees ) at the level of major cervicothoracic curve and up to 10 degrees ( 2 degrees ) at the level of accompanying lumbar arch ( fig .
operative intervention performed on september 29 , 2011 , included correction and multisupport instrumentation fixation of c2t9 segments with combined connector - type system ( summit - expedium ; depuy international ltd .
, leeds , uk ) , posterior local spondylodesis , and dismantling of the halo - pelvic traction system ( fig .
the procedure involved incision at the level of c2c10 spinous processes after cleaning the operation field .
evident torsion to the apex of the arch was observed in the superior portion of the thoracic spine .
pairwise interlaminar screws were placed in c2 ; lateral mass screws ( summit ) introduced in c4 , c5 , c6 ; and transpedicular and intracorporeal screws inserted in t6 , t7 , t8 , t9 ( on the left ) and t6 , t7 , t9 ( on the right ) .
connecting rods 3.5/5.5 were laid over the fixation points and connected at c6t6 level bilaterally with four parallel connectors .
acute correction with tensioned rods was produced with distraction provided on the left and compression on the right under protection of methylprednisolone injection .
the construct was reinforced by two transverse bars mounted to the cervical spine and one connection plate to the thoracic spine .
loss of blood volume was 200 ml ( 5% of the total body volume ) .
functional result assessed at 1  month after the surgery showed increased vital capacity of 56% according to spirometry findings and improved respiratory function with pulmonary stress test .
the patient could walk with support for 30 minutes without respiratory imbalance , and he could eat food and stand up by himself , with torso balance in the coronal and sagittal planes being compensated ( fig .
checkup radiographs showed adequate screw position ; spinal deformity correction was achieved at the level of major cervicothoracic curve to 12 degrees ( 51 degrees ) and at the level of accompanying lumbar arch to 10 degrees ( 2 degrees ; fig .
the child was discharged on november 4 , 2011 , and tracheostomy was removed on november 23 , 2011 . 

photo ( a ) and radiographs ( b , c ) of the patient prior to discharge .
the objective of the demonstration was to emphasize medical considerations on system abnormalities of patients with severe progressive spinal deformity , so called  high - risk deformities  that entail a severely reduced quality of life due to static and dynamic imbalance and progressive life - threatening functional disorders.5 
 6 traditionally , such cases are considered incurable .
it can be expected that the improved quality of peri- and postnatal care with persistent rate of worsening maternal health would inevitably lead to an increase in this patient cohort .
the clinical example presented here demonstrates the modern possibilities of surgical treatment , which dramatically improved a child 's condition and his quality of life .
a strategy of surgical treatment involved application of an external stabilizing system in the position of moderate spinal correction and additional correction and stabilization with a posterior internal multisupport construct after improvement of his general condition .
tactical application included a halo - pelvic device assembled of ilizarov components and a hybrid system with transpedicular supporting points , connecting rods , and connectors that provided staged distraction during growth.7 
 8 
 9 
  legal consideration of surgical treatment with detailed substantiation of a lifesaving procedure is required .
we report staged treatment of severe combined spinal deformity in an 11-year - old patient with ollier disease and abnormal cervical vertebra . combined scoliosis with systemic pathology and abnormal vertebrae 
 is a rare condition and features atypical deformity location and rapid progression rate and frequently involves the rib cage and pelvis , disturbing the function of chest organs and skeleton . 
 progressive deformity resulted in cachexia and acute respiratory failure . a halo - pelvic distraction device assembled of ilizarov components 
 was employed for a staged surgical treatment performed for lifesaving indications . 
 after vital functions stabilized , the scoliosis curve of the cervical spine was corrected and fixed with a hybrid system of transpedicular supporting points , connecting rods , and connectors that provided staged distraction during growth . 
 the treatment showed good functional and cosmetic result .