The successful equalization of large discrepancies in limb length with use of the Ilizarov device has been reported2,3,10, but the number of associated complications has been substantial. Our management of the children in the present study was very different from that provided in the former Soviet Union. In Siberia, children who have correction of complex deformities spend many months in the hospital and receive psychological support and physical therapy. In North America, children spend only a few days in the hospital and the psychological environment is different.
Despite adequate correction of complex deformities of the lower extremities, the complications associated with any type of limb-lengthening can result in permanent psychological and functional disturbances. Multiple operations may lead to disturbance in the child's life during an active and important period of development5,7. Problems, including depression secondary to persistent pain, poor function, an unsatisfactory cosmetic appearance, and behavioral disturbances, can develop. Relatively little attention has been directed toward the functional and psychological outcomes and the satisfaction of patients and their families after correction of complex limb deformities with use of the Ilizarov method18. The Ilizarov device permits full weight-bearing or partial weight-bearing with crutches, which may create the optimum mechanical and biological conditions for osteogenesis and prompt functional recovery1. However, the Ilizarov device also is associated with a high rate of complications, including subluxation of joints, restriction in motion of joints, and prolonged pain11,19.
We reviewed the psychological and functional results after treatment of complex musculoskeletal deformities with the Ilizarov method.
*No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. Funds were received in total or partial support of the research or clinical study presented in this article. The funding sources were a grant from The Hospital for Sick Children Trainee Research Support Fund, Toronto, Ontario, Canada; a Medical Research Council Scholarship (J. G. W.); and a scholarship provided by the University of Manoufia, Egypt (H. F. G.).
†Division of Orthopaedic Surgery, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada. Please address requests for reprints to Dr. Wright.
The study included forty-five children (fifty-two extremities) in whom complex deformities of the lower extremities had been treated with the Ilizarov technique at The Hospital for Sick Children in Toronto between 1988 and 1992. The term complex deformity was used when there was more than one deformity in the same extremity, such as a limb-length discrepancy with an angular rotational or translational deformity, or a deformity of the foot that necessitated multiplanar and multidirectional correction. We excluded patients who had been managed with correction in one plane or with simple lengthening of the limb.
We collated the demographic data (the age and gender of the patient, the symptoms, the side of the deformity, and the diagnosis), the findings at the preoperative clinical examination (gait, the type of deformity, the range of motion of the joints, and the neurovascular status), the radiographic findings (the lengths and alignment of the bones as well as the bone age), and the details of treatment (the date of the operation, the level of the osteotomy, the type of frame construct, and any associated procedures such as lengthening of the Achilles tendon). The postoperative data included the delay before distraction, the rate and rhythm of distraction, the duration of hospitalization, the duration of distraction, the degree of correction of the deformity, the length that was gained, the duration that the fixator was in place, the lengthening index (the number of months in the fixator to gain one centimeter of lengthening), any complications, and any subsequent procedures.
At the latest follow-up visit, clinical, radiographic, psychological, and functional evaluations were performed as well as an assessment of the patient's over-all satisfaction with the procedure. In addition, any symptoms, limb-length discrepancy, or deformity were recorded as were the range of motion of the involved joints and the neurovascular function. The degree of correction of the foot was judged by assessment of the gait (as better, unchanged, or worse), whether the foot was plantigrade, and whether there was any pain. A satisfactory result indicated that the foot was plantigrade, the gait was improved (if it had been abnormal previously), and the patient was pain-free13. Radiographs were used to measure residual limb-length discrepancy, deformity, and associated changes in the joints.
The functional score was determined with use of the physical function subscale of the Children Health Information Service Rand Scale developed by Eisen et al. The questionnaire, which has been shown previously4 to be reliable and valid, consists of thirteen questions, with each yes answer given a score of 1 point and each no answer given a score of 0 points, so that 0 points is the best score and 13 points, the worst possible score. The thirteen questions are divided into four categories: mobility, physical activity, role activity, and self-care activities (Table I).
Two questionnaires, the Post-Hospitalization Behavior Questionnaire and the Children's Depression Inventory, were used to assess the psychological status of the child. The Post-Hospitalization Behavior Questionnaire, developed by Vernon et al., measures behavioral disturbances of children after hospitalization. It has been shown to be reliable and valid. The reliability was based on a Cronbach alpha of 0.82, determined from a sample of 387 children, and a test-retest correlation of 0.95 (p = 0.001), based on the responses of a psychologist and a psychiatrist after viewing videotapes. The validity was confirmed by ratings that showed a correlation of 0.47 with ratings based on an independent interview carried out by a child psychiatrist. Factor analysis and a priori hypotheses confirmed the validity of the construct.
With the Post-Hospitalization Behavior Questionnaire, parents rate twenty-seven behavioral patterns of their child; they employ a scale ranging from 1 point (much better) to 5 points (much worse). All of the behaviors included in the Post-Hospitalization Behavior Questionnaire were mentioned in at least two of six previously described questionnaires17. The total possible score ranges from 27 to 135 points; a total score of 81 points or less suggests no worsening in behavior.
The Children's Depression Inventory, developed by Kovacs, requires the child and his or her parents to complete twenty-seven items describing the child's feelings and ideas. The total score ranges from 0 points (best) to 54 points (worst); a score of 9 points or less is considered normal (Table II).
Children and their parents separately rated their satisfaction with the appearance and function of the extremity and the over-all outcome according to five categories: extremely satisfied (1 point), moderately satisfied (2 points), no opinion (3 points), moderately unsatisfied (4 points), and extremely dissatisfied (5 points).
The results were analyzed with the Student t test, regression analyses, and chi-square tests. We studied the effect on the outcome of the age and gender of the patient, the etiology of the underlying disease (congenital or acquired), the bone affected, the level of the osteotomy (metaphyseal or diaphyseal), the percentage of bone-lengthening, the duration that the fixator was in place, and the technique used to correct the foot (distraction through soft tissue or bone).
There were nineteen girls and twenty-six boys in the study. The deformities were unilateral in thirty-eight children and bilateral in seven children, for a total of fifty-two extremities. The average age of the patients at the time of the operation was twelve years (range, three to eighteen years). Two patients were three to five years old, twenty-three were six to twelve years old, and twenty were thirteen to eighteen years old. The average duration of follow-up was thirty-six months (range, twenty-four to seventy-two months). Thirty patients (thirty-seven extremities) had a congenital disorder, and fifteen (fifteen extremities) had an acquired disorder. The etiology of the complex deformities were a longitudinal deficiency in thirteen patients, growth-arrest secondary to infection in nine, clubfoot deformity in eight, a syndrome or dysplasia in eight, metabolic bone disease in three, growth arrest secondary to trauma in two, and paralytic disease in two.
The tibia was involved in twenty-nine extremities; the femur, in twenty-three; and the foot, in twelve. A total of sixty-four Ilizarov procedures were performed. The correction of the deformity in eight feet was not associated with another correction in the lower limb, and the correction in four feet was performed with correction of the tibia. Tibial and femoral corrections were performed simultaneously in seven patients. All of the children had a limp and deformities that were too complex to be treated with a simple osteotomy and an epiphyseodesis of the contralateral, normal extremity. Many of the children could not have been managed without the Ilizarov procedure.
The average limb-length discrepancy preoperatively was 7.6 centimeters (range, 2.5 to thirteen centimeters). The average gain in length postoperatively was 6.5 centimeters (range, two to thirteen centimeters) or 25 per cent (range, 6 to 59 per cent); the average lengthening index was one month per centimeter (range, 0.3 to 2.2 months per centimeter), and the average angular correction was 18 degrees (range, 7 to 40 degrees).
The thirty-seven children who had had lengthening had a discrepancy of two centimeters or less at the latest follow-up evaluation. The lengths of the limbs were equalized in seventeen patients. The residual discrepancy of three patients, who had a longer affected limb and open growth plates, was expected to correct with growth. For twelve of the remaining seventeen patients in whom the affected limb was shorter than the unaffected limb, the limb-length discrepancy was less than one centimeter.
The average lengthening indices for the tibia and the femora were the same (1.1 months per centimeter; p = 0.9), the average percentages of lengthening were similar (27 and 22 per cent; p = 0.44), and the average numbers of complications were also similar (1.4 and 1.7; p = 0.55).
The rate of complications increased with the amount of lengthening (p = 0.04). The average duration of hospitalization was eleven days (range, five to twenty-five days). The average duration of distraction was eleven weeks (range, two to twenty-nine weeks), and the average duration that the fixator was in place was twenty-three weeks (range, eight to sixty-eight weeks).
Pain during the immediate postoperative period was treated with a patient-controlled morphine pump. During the predistraction phase, after discharge from the hospital, the patients took acetaminophen with codeine every six hours. During the consolidation phase, the patients took analgesics only rarely. At the latest follow-up examination, none of the children had pain that interfered with activities of daily living. The ranges of motion of the hip, knee, and ankle were preserved in all but one child, who had a restricted range of motion of the knee secondary to a persistent unreduced posterior subluxation.
Of the twelve feet that had an Ilizarov procedure to correct a deformity, six also had an osteotomy and six had soft-tissue lengthening. The result was good for the six feet that had an osteotomy; the result was unsatisfactory for four of the six feet that had soft-tissue lengthening because of a recurrent equinus deformity (p = 0.01, chi-square test).
There was an average of 1.7 complications (range, zero to five complications). Eight patients did not have any complications. There were twenty-one major and fifty-five minor complications. A complication was considered major if it had adversely affected the outcome or had led to additional procedures. A minor complication was one that had resolved spontaneously or with non-operative treatment and had not affected the outcome10. The major complications included recurrence of the deformity or residual deformity in six extremities, subluxation of a joint in five, fracture after removal of the fixator in four, bending of the lengthened bone after removal of the fixator in three, premature consolidation in two, and osteomyelitis in one. The minor complications included a pin-track infection in thirty-two extremities, stiffness of a joint in eleven, delayed consolidation in nine, excessive scarring in two, and a broken pin in one. Sixteen children (36 per cent) needed an additional operation, such as removal and replacement of a pin, lengthening of the Achilles tendon, soft-tissue release to treat subluxation, immobilization of a fracture, extension of the frame across the knee because of subluxation, or corrective osteotomy.
The results for the children who were six to twelve years old were similar to those for the children who were thirteen to eighteen years old, with an average gain in length of 7.6 and 6.0 centimeters (p = 0.05); an average lengthening index of 1.0 and 1.3 months per centimeter (p = 0.06); and an average of 1.4 and 1.7 complications, respectively (p = 0.4). A comparison of the results for the patients who had an acquired deformity with those for the ones who had a congenital deformity showed that the average lengthening index was the same (1.1 months per centimeter; p = 0.7) and the average number of complications was similar (1.3 and 1.6, respectively; p = 0.9). The satisfaction and functional scores for the children who had an acquired deformity were similar to those for the children who had a congenital deformity.
Functional and Psychological Results
According to the physical function subscale of the Children Health Information Service Rand Scale, the average functional score for the entire study population was 0.7 point (range, 0 to 5 points), with forty-two children (93 per cent) having no limitations in daily activities. Only three children (7 per cent) had some limitation in activities. The patient who had a score of 5 points had residual posterior subluxation of the knee.
The average score on the Post-Hospitalization Behavior Questionnaire was 65 points (range, 60 to 81 points), and the average score on the Children's Depression Inventory was 6 points (range, 0 to 8 points). All of the scores were within normal limits. Forty children (89 per cent) were satisfied with their over-all level of function, thirty-seven (82 per cent) were satisfied with the over-all result, and thirty-four (76 per cent) were satisfied with their appearance. Fifteen of the forty-five children stated that they would not have the treatment again if it was indicated: eight, because the duration of the treatment was too long; two, because the duration of the treatment was too long and because of the appearance of the scars; two, because of complications; two, because of the duration in the fixator and because of complications; and one, because of the appearance of the scars.
Again, the results for the children who were six to twelve years old were similar to those for the children who were thirteen to eighteen years old. The average score on the Post-Hospitalization Behavior Questionnaire was 61 points for both (p = 0.3), the average rating for over-all satisfaction was 1.4 points for both (p = 0.8), and the average rating for over-all appearance was 1.0 and 1.1 points, respectively (p = 0.8). The scores on the Children's Depression Inventory and the Post-Hospitalization Behavior Questionnaire for the boys were similar to those for the girls. Nineteen (83 per cent) of the twenty-three children who were six to twelve years old were willing to be managed with the Ilizarov method a second time, if it were indicated, compared with nine (45 per cent) of the twenty children who were thirteen to eighteen years old (p = 0.01). The number of patients was too small for us to determine a specific reason for this difference.
The clinical and radiographic results of this study are similar to those of many recent reports3,12,14,16 on the treatment of complex deformities of the lower extremity in terms of the average duration of hospitalization (eleven days), average duration in the fixator (twenty-three weeks), average gain in length (6.5 centimeters), average percentage of lengthening (25 per cent), average lengthening index (one month per centimeter), and average residual limb-length discrepancy (1.1 centimeters). Other authors have reported an average of approximately six months in the fixator, an average gain in length of approximately five centimeters, an average lengthening of approximately 15 per cent, and an average lengthening index of approximately one month per centimeter3,12,14,16.
Little has been written about the physical and psychological function of children after limb-lengthening procedures18. Lavini et al., using non-standardized methods, found that five (14 per cent) of thirty-five children who had achondroplasia had difficulty pursuing normal activities after limb-lengthening. In the present study, we found that forty-two children (93 per cent) were functioning well and only three (7 per cent) had some limitation in daily activities at the latest follow-up examination.
Seven of the twenty-two children reported on by Hrutkay and Eilert were depressed and anxious after a lengthening procedure, and approximately one in four was non-compliant; regressed; or became dependent, relinquishing self-care to parents or nurses. We attempted to counter this problem by preparing the children psychologically before the lengthening procedure. The goals of the treatment, the nature of the procedure, the likely duration of the treatment, and potential complications were discussed with the patients and their families. Whenever possible, arrangements were made for the patients and their families to meet with other patients who had had the same treatment and to watch instructional videos2,9,11,15. The goal was a supportive environment. Treatment of the considerable pain that children have during lengthening with the Ilizarov device was a priority because such pain may increase psychological vulnerability19. We administered acetaminophen with codeine as long as the patients needed it, and we did not allow a fear of possible addiction to the codeine to induce us to reduce the dosage. None of our patients became addicted.
Psychological changes can be extreme, varying from anxiety and depression to suicidal thoughts6. Four of our forty-five patients were referred to a psychiatrist during the period of lengthening for emotional or behavioral difficulties. The results of our study obtained with use of standardized questionnaires suggest that our methods of preparation permitted correction of complex deformities without permanent functional or psychological disturbances in most of our patients. We found that the psychological problems were transient. The patients in the study by Hrutkay and Eilert were used for comparison, but the results of that study could not be used as matched controls because Hrutkay and Eilert evaluated problems during the process of lengthening whereas we evaluated problems that occurred after the lengthening.
Few investigators have considered the satisfaction of the patient as an outcome when assessing the success of the procedure. In the present study, forty children (89 per cent) were satisfied with their level of function, thirty-seven (82 per cent) were satisfied with the over-all result, and thirty-four (76 per cent) were satisfied with their appearance at the latest follow-up examination. The lower rate of satisfaction with appearance is mostly due to scars resulting from the entrance and exit wounds for the wires. Lavini et al. reported that only two (6 per cent) of thirty-five children were dissatisfied with function and cosmetic appearance. In our study, thirty children (67 per cent) thought that the duration of the treatment was reasonable and said that they would be willing to have the procedure a second time if it were indicated.
The Ilizarov method is a reliable way to treat extremely complex deformities of the lower extremity although it is associated with a high rate of complications. The Ilizarov method did not cause long-term psychological or functional disturbances in our patients. We recommend that lengthening procedures be initiated between the ages of six and twelve years, as the younger children in our study appeared to tolerate the procedures better than the older children did, as evidenced by their willingness to have the treatment again if indicated. Psychological preparation and a support system for the child and his or her family must be an integral part of the lengthening procedure. Better methods of scoring the behavior of a child and the functional outcome after lengthening procedures are also needed.
NOTE: The authors thank D. F. Bell, M.D., F.R.C.S.(C), and P. Armstrong, M.D., F.R.C.S.(C), for allowing us to include their patients in the study, and Nancy Young, B.Sc.P.T., M.Sc., for assistance in the performance of the study.