CORR Insights®: Does Kyphectomy Improve the Quality of Life of Patients With Myelomeningocele?

Where Are We Now? Kyphosis in patients with myelomeningocele can cause skin ulceration, pain, and substantial sitting imbalance. Bracing may stabilize the deformity or delay surgical intervention until skeletal maturity, but it is not always effective. Surgical indications include breakdown of the soft-tissue envelope overlying the gibbus, sagittal imbalance, and progressive deformity. This condition is difficult to treat surgically because complications, such as loss of correction, loss of fixation, revision surgery, and even death after surgery are disconcertingly common [1-3, 5, 6, 8-11]. Wound healing and infection are common reasons for reoperation in this population and are likely a result of operating through the tenuous soft-tissue scar of patients with myelomeningocele. By avoiding incisions through the soft-tissue scar, Hyndman’s Halifax kyphectomy and Torode’s technique may decrease the risk of wound-healing problems [3, 12], but they do not eliminate it. Loss of fixation may be caused by anatomic deficiency of the posterior elements and poor bone quality, though pedicle screw fixation has demonstrated some promise in mitigating these issues [1, 5]. In the current study, Petersen and colleagues [9] evaluated the risk of complications and reoperation as well as the impact of surgery on health-related quality of life (HRQoL) in children with myelomeningocele undergoing surgical correction of their lumbar kyphosis. Their technique involves posterior fixation using "S-shaped" rods inserted through the foramina of S1 and pedicle screws in the thoracic spine. The authors found that complications (including infection requiring débridement and wound breakdown requiring plastic surgery with advancement flap for coverage) were common and 68% of the patients underwent reoperation [9]. Their study supports several others that have documented a high risk of complications and reoperations in this patient population [2, 8, 10]. The results in the current study are important because the authors evaluated HRQoL in patients treated with kypectomy [9]. While previous studies have focused on surgical outcomes alone, the current study focuses on outcomes from the patient’s perspective. Because they reported improvement in HRQoL, despite a high complication rate, their findings allow clinicians to more confidently counsel patients about the potential impacts of the surgery [9]. Patients will expect that there may be future unplanned reoperations but that their quality of life should still be improved. Where Do We Need To Go? Although the current study advances our knowledge on kyphosis in patients with myelomeningocele, we still need to determine which surgical technique yields the lowest risk of re-operation, and whether kyphectomy positively influences HRQoL as compared to the natural history of untreated kyphosis. In addition to the treatment goals of spinal deformity correction and maintenance of growth, it also would be desirable to measure the effects of patient HRQoL and reduction in caregiver burden [4, 7] since patients with neuromuscular disease often require a considerable amount of care from their families. Peterson and colleagues [9] were the first to specifically study HRQoL in this population and should be commended for this endeavor. Still, it is difficult to compare their results to the untreated natural history of this disease. One can assume that HRQoL will deteriorate if the kyphosis is not treated. A positive outlook of their results is that the operations, even with the associated complications, appeared to maintain patients’ baseline HRQoL. Some might see this as a win for this medically frail patient population. How Do We Get There? We may be able to reduce the risk of re-operation by combining the best of the previous surgical techniques. Hyndman’s kyphectomy technique, which avoids operating though the posterior scar and avoids fixation in the deficient posterior elements, shows promise as a means of minimizing wound healing and infection risks [3]. One study suggests that it may be beneficial to use a more-rigid pedicle screw fixation in patients who are closer to skeletal maturity [5]. In patients who have not yet reached skeletal maturity, this type of distal exposure and fixation may be improved by using a more growth-friendly system as described by Howard’s group [1]. Currently, patients with kyphosis and myelomenigocele are not commonly encountered; therefore, it would be useful to study these patients through prospectively collected, multi-center registries. As scoliosis study groups such as the Pediatric Spine Study Group and Harms Study Group continue to develop, we expect more representation from centers from around the world. Over the span of time that Peterson’s group evaluated their patients [9], a disease-specific HRQoL tool for this population, such as the Early Onset Scoliosis Questionnaire (EOSQ-24), had not yet been developed [7]. By combining a general HRQoL tool such as the Pediatric Quality of Life Inventory with a disease-specific tool such as the EOSQ-24, differences in HRQoL may be more easily identifiable in future studies. Once more international centers become a part of scoliosis registries, these registries will have the patient population available to utilize the power of the study group infrastructure, and potentially better evaluate these techniques. This may allow thorough evaluation of outcomes, including HRQoL spine growth, and pulmonary function. Such a study should not only focus on radiographic correction of deformity, but also evaluate measures of growth, development, pulmonary status, HRQoL, and risk of unplanned re-operations.

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