Rehabilitation of Children Suffering from Congenital Malformations of Vertebral Column and Spinal Cord

9.02.12

Rehabilitation of Children Suffering from Congenital Malformations of Vertebral Column and Spinal Cord

N.Yu. Titarenko, M.V. Dvorovoy, V.I. Shchelchkova

 Scientific Production Center “Ogonyok”, Moscow

A common term “spinal dysraphism” (from Latin raphe – a stitch) is used to define all the variety of congenital malformations of vertebral column and spinal cord, characterized by a nonclosure of vertebral column (the so called “spina bifida”). Two main types of spinal dysraphism are known: occult and open. In occult spinal dysraphism (OCSD) the skin integrity above the vertebrae defect is not damaged, while in open spinal dysraphism (OPSD) the skin integrity above a nonclosed vertebral area is damaged and a meningeal sac protrudes, its content being cerebrospinal fluid, part of spinal cord and/or its meninges.

OCSD is most common. This form of dysraphism may not be associated with neurologic deficiciency, if nonclosures of only one or two vertebral arches are present, more frequent lumbar vertebra 5 (spina bifida L5)and/or sacral vertebra 1 (spina bifida S1). Most people get to know about such vertebrae defect occasionally, during X-ray imaging performed for some other reason. However, dysraphism can be accompanied by different malformations of spinal cord and neurocanal such as myelodysplasia, a syndrome of fixed spinal cord, diastematomyelia, spinal lipomas, or dorsal dermal sinus. In these cases orthopedic-neurologic abnormalities, sometimes significant, occur: lower paraparesis, muscle tone loss, muscle atrophy, enuresis, encopresis, sensory decrement in legs and perineum, deformities of vertebral column (kyphosis, lordosis or scoliosis) and lower extremities, hip joints instability etc.

OCSD can happen not to be evident right after a child birth, but later, neurologic and orthopedic defects progressing slowly. To make an accurate diagnosis, MR-imaging is necessary. The treatment of patients with different forms of OCSD is surgical [1]. The aims and technique of the surgery depend on the form of OCSD.

In OPSD, also known as myelocele, a hernial sac consisting in myelic meninges and containing either cerebrospinal fluid, liquor (meningocele), or liquor and the tissue of spinal nerves roots (meningoradiculocele) or, besides the above mentioned components, spinal cord tissue (myelomeningocele), protrudes through the bone defect. Myelocystocele, a local distension of the central canal with protruding posterior wall of the thinned spinal cord, is also identified. Neurologic disorder in OPSD depends on the content of hernial sac and its site (cervical, thoracic or lumbosacral level).

Myelocele presents no difficulty for diagnostics and is a target of surgical intervention. The operation removes hernial sac, yet the prognosis is unfavorable and likely to reverse the onset of neurologic disorders due to irreversible changes in the spinal cord and its roots [2].

After the operative treatment of different forms of spinal dysraphism, the children need a long restorative treatment and rehabilitation. The success of medical rehabilitation depends not only on the extent of intactness of spinal cord tissue and its roots, but also the timeliness and adequacy of treatment.

A proper orthopedic care of a sick child is an important component of rehab process and a difficult medical problem in flail legs caused by different forms of spinal dysraphism. On the one hand, an orthopedic appliance should provide a support ability of the lower extremities. On the other, this appliance should not impede making steps by a child, who retains minimum motor function of his leg muscles. Traditionally, a fixing locking device for a whole leg with a semi-brace is used for orthotics in the children with flail legs. It is usually manufactured according to individual moulds, contains hip and knee hinges providing fixation of hip and shin extension at an angle of 180° in hip and knee joint, respectively. However, a locking device firstly hampers complete walking, and secondly fails to provide improvement in the position of a whirlbone in the acetabulum in the paralytic instability of hip joints.

Unfortunately, the instability of hip joints of different extent, right up to the onset of paralytic hip dislocation, occurs in most patients suffering from flail legs due to spinal dysraphism. Paralytic instability of hip joints is treated surgically, but this treatment as a rule needs long-term postoperative immobilization, in many cases resulting in a partial loss of motor skill of a sick child.

Recently, a biomechanical rotation-correcting apparatus for a lower extremity® (a dynamic orthotics technique), an innovative product of Scientific Production Center “Ogonyok”, has been applied with success for orthotics in the children of 2 to 3 years old suffering from flail legs. This orthopedic device not only improves a support ability of the lower extremities without hindering step movements, but also provides a controlled extent of hip abduction aimed at improvement of whirlbones position in the respective acetabulums (Fig. 1).

Fig. 1 Biomechanical apparatus

 

Different extent of abduction of each hip, left and right, can be achieved as needed by the apparatus, which is very important in the unilateral paralytic hip instability. Moreover, the biomechanical apparatus step-by-step corrects different pathologic deformities of shins and feet such as shin valgus/varus, torsion, and recurvation, pes valgus/varus, equinus, foot adduction/abduction (Fig. 2 a, b, c).

                                   

а                                                                 b                                                                                 c

Fig. 2 Correction of pathologic deformities of lower extremities by the biomechanical apparatus.

a – Initial pathologic deformities; b – Correction by the biomechanical apparatus, in the standing position;   c – In the sitting position

 

The apparatus has a module construction and is assembled individually for each patient out of serial components. Depending on orthopedic-neurologic disorders in a child, the apparatus can be assembled for one or both lower extremities.

Improvement of support ability of the lower extremities in flail legs is achieved, on the one hand, due to fixation of hip, shin, and foot of each extremity in the corresponding construction elements of the apparatus (sockets), articulated with load-leveling, and on the other, due to specific hinge construction with positions of the pivot pins. Individual fitting of the apparatus, corresponding to a position and size of each segment (hip, shin, and foot) of the lower extremities of a child as well as stabilization of major joints of the legs and the scope and direction of their movements is provided by the adjustment of hinges (assemblages) of the biomechanical apparatus.

Removal of pathologic deformities of the segments of the lower extremities is accomplished step-by-step with a long-term use of the biomechanical apparatus on the assumption of permanent wearing in wakefulness. Each stage of treatment takes 6 to 8 weeks at the most, since clinical control and adjustment of the apparatus should be performed at least bimonthly. A special complex of medical physical training, which should be carried out with the apparatus on, is prescribed at each stage to improve the adaptation to the corrected position of the lower extremitiesб taking into account the initial motor abilities of a child.

The susceptibility to trophic skin disturbances in the sites of excessive pressure of the constructive elements of the apparatus on the soft tissues during the removal of pathologic deformities of shin and foot, presents the most difficult issue for orthotics in patients with spinal dysraphism. In the first place, this issue necessitates a step-by-step approach to orthopedic correction and in the second place it needs the most possible softening of the contact between the details of the apparatus and the soft tissues of a child, especially, in the area of bone shelves. In the third place, the higher a pressure on the tissues is, the larger a contact area should be. That is why prior to prescription of biomechanical apparatus, it is important to adapt a child to wearing the night-time correcting tutors for ankle and knee joints.

At the first stage of dynamic orthotics, a child in wakefulness (while sitting and creeping) should permanently wear the apparatus for hip abduction and fixation in a predetermined position® (hereinafter abduction apparatus; Fig. 3, 4), which is a basic module of the biomechanical apparatus. Meanwhile, a full thrust load on the lower extremities should be excluded. This stage can take a rather long time, at an average, at least a year and can be considered finished, in case a relative stability of hip joints is formed. Evaluation of hip joints state prior to and during orthotics is both clinical and roentgenological; anterioposterior hip joint X-ray is performed at least once a year. The duration of all the next stages is individual (6 to 8 weeks, at an average) and is affected by a lot of factors such as individual adaptation abilities of a child, the evidence of neurologic deficiency, trophic skin disturbances, pathologic shin and foot deformities.

                

Fig. 3. Abduction apparatus                          Fig. 4. A child in the abduction apparatus

 

An important advantage of dynamic orthotics technique for paralytic hip joints instability is a possibility of simultaneous application of kinesitherapeutic methods including the application to a child of a thrust compression load aimed at the development of active motor skill. The application of a thrust load without dynamic hip orthotics could provoke worsening of a femoral head position in acetabulum, leading to aggravation of paralytic hip joint instability.

On the contrary, a permanent abduction position of each (left and right) hip provided by the apparatus, allows inhibiting an unfavorable effect of a thrust load on a femoral head position in acetabulum. Thus, incorporating the biomechanical apparatus in a construction of a reflex-load device “Gravistat”/”Graviton” ® enables medical rehabilitation of children suffering from different forms of spinal dysraphism by means of dynamic proprioceptive correction (DPC) (Fig. 5 a, b).

                                                     

a                                                                                        b

Fig. 5. Incorporation of a basic module of the biomechanical apparatus in a construction of a reflex-load device “Gravistat”/”Graviton” ®; a – Frontal view; b – Lateral view

 

In addition to dynamic orthotics and DPC technique, medical rehabilitation of children with orthopedic-neurological signs of spinal dysraphism at the Scientific Production Center “Ogonyok” includes (by the indications): kinesiology taping, artificial motor correction (AMC), oscillatory therapy, exterior pneumocompression, transcranial magnetic stimulation (TMS),  postural mechanostimulation, underwater vacuum massage as well as the other techniques and methods of modern physiotherapy.

The case below illustrates the efficiency of medical rehabilitation of the child with flail legs due to OCSD.

Kolya Sh., 6 years old (b. 2005). Diagnosis: OCSD, myelodysplasia. A state after removal of intradural lipoma of vertebral canal of lumbosacral spine with excision of archs L2-L5 (Feb., 2006). Dissociative mainly distal flail legs. Pelvic organs dysfunction of peripheral type (enuresis, encopresis). Kyphotic posture. Dysontogenetic hip joints formation, paralytic left hip subluxation. Pronator deformity of the lower extremities (more left). Ligamentous instability of both knee joints. Adduction left pes equinovarus, abduction right pes equinovarus.

The child underwent the first examination by the experts of “Ogonyok” in September, 2009. At the examination: the child can seat without assistance with kyphotic posture being able to correct his posture in the sitting position. He cannot stand or walk on his own. When supported at the high level, he can make step movements. The muscle strength in the lower extremities is decreased unevenly: in hips up to 4 points on the right and 2 to 2.5 points on the left; in shins up to 3 points on the left and 2 points on the right. Active feet movements are absent. Disorders of pain and temperature sensitivity in perineum and the lower extremities, significant fixed equinovarus with left forefoot adduction, equinovarus with right foot abduction (passive correction is possible). Anterioposterior hip joint X-ray film (2009): acetabulums are flat, more on the left, with uneven borders and areas of subchondral sclerosis. Femoral heads are reduced in size, more on the left, their form being irregular and the structure being heterogeneous. The heads coat is incomplete and significantly worse on the left (angle of Wiberg on the right 18°, on the left 2° (normal is +20° or bigger)). Femoral necks valgus, more on the left; antetorsion. Shenton’s arch is broken on the left (Fig. 6).

 

Fig. 6.  Anterioposterior hip joint X-ray film of Kolya Sh.  (Sep., 2009)

 

At the first stage the child was prescribed the non-load ankle tutors and knee tutors for sleeping. Despite of prevention measures, the child’s adaptation to wearing the tutors was difficult; a contact of the tutor in the area of lateral malleolus of the left shin and external surface of the left midfoot resulted in the emergence of trophic ulcers, the treatment of which increased the duration of a preliminary stage. As early as at this stage, the apparatus for adduction and fixation of hips in a predetermined position® (Fig. 4) was prescribed for the patient and his adaptation to it was quick enough. The first course of medical rehabilitation by DPC technique with incorporation of the hip adduction apparatus in a construction of a reflex-load device “Gravistat”/”Graviton” ® was performed in November, 2009. The course conducted by a skilled methodologist, included 20 daily individual studies of the child in “Gravistat”/”Graviton” ® device and the hip adduction apparatus. Different physiotherapeutic techniques, massage, kinesiology taping, stabilometry exercises, and underwater vacuum massage were applied in addition. As a result of the course, the child learned to make short walks without assistance. His gait (in the hip adduction and ankle joint apparatuses) was unsteady. He fell down frequently, could not stand on his own, and could stop with assistance only. The evident frontal body-rocking and the internal rotation of the lower extremities were marked. The gait disorders were characterized by pace decrease of the left extremity and shortening of single limb support phase of the right extremity.

In 2010 the patient underwent 2 courses of medical rehabilitation with the application of the reflex-load device “Gravistat”/”Graviton” ® including the hip adduction apparatus. In spite of application of a thrust load, a control X-ray film of hip joints in 2010 demonstrated some positive effect: the extent of femoral head coat increased (angle of Wiberg 20°), the form of the femoral heads improved (Fig. 7). The acetabulums remained flat. After the recurring treatment courses conducted in 2010, the child’s gait improved significantly: frontal body-rocking decreased, gait steadiness increased, the pace including that of the left leg, increased. The patient learned to stop on his own, stand, and rise to his feet. He fell down more seldom.

Fig. 7. Anterioposterior hip joint X-ray film of Kolya Sh.  (Oct., 2010)

 

In October, 2010 a biomechanical rotation-correcting apparatus for the lower extremity with a left knee assembly (Fig. 8) for permanent wearing in wakefulness was manufactured for the child. Along with it, the patient continued wearing the ankle apparatus with a hinge for the  right leg. The step-by-step correction of pathologic shin and feet deformities of the child was performed by means of the biomechanical apparatus. In 2011 the child underwent 3 courses of medical rehabilitation with the biomechanical apparatus incorporated in “Gravistat”/”Graviton” ® device. As a result of the recurring treatment courses, the musculature of a trunk and buttocks got stronger. Minimum active movements in the toes and minimum active dorsiflexion in the right foot appeared. Varus deformity and left forefoot abduction decreased significantly without any recurrence of trophic skin disturbances. The patient learned to run in the biomechanical apparatus. His locomotive stereotype improved essentially: the duration of single limb support phase of the right and the pace of the left extremity increased, his gait became steadier. In the last 2 months the child began feeling vesical tenesmus and the urge to defecate. He could restrain

urine and faeces for 20-30 sec.

Fig. 8. Kolya Sh. in the biomechanical apparatus

 

A slight positive effect is demonstrated on the hip joint X-ray: angle of Wiberg on the right 20°, on the left within 3-4°, the form of the femoral heads improved, their structure grew more uniform. Acetabulums remain flat (Fig. 9). Medical rehabilitation of the child continues. At least, we succeeded in delaying operative treatment of the child (before the application of the apparatus detorsion-varus hip osteotomy had been planned, which would have required inevitable long immobilization). Postoperative immobilization prevents a child from complete participation in the rehab process aimed at the development of voluntary movements. That is the reason why a benefit/risk ratio of operative treatment is doubtful in many patients with flail legs caused by spinal dysraphism.

Fig. 9. Anterioposterior hip joint X-ray film of Kolya Sh.  (Sep., 2011)

 

At present, the construction of the rotation-correction biomechanical apparatus for a lower extremity and its modules, which are used for different forms of spinal dysraphism and different shin and foot pathologic deformities, is being improved.

The biomechanical apparatus has been registered in accordance with the established procedure, which allows the disabled children getting it through budgetary financing.

 

 

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