The Journal of Bone & Joint Surgery

The Journal of Bone & Joint Surgery, Volume 91, Issue Supplement 6

Scientific Exhibits | November 01, 2009

Management of Orthopaedic Sequelae of Congenital Spinal Disorders

Michael Akbar, MD¹; Bjoern Bresch, MD¹; Thorsten M. Seyler, MD²; Wolfram Wenz, MD¹; Thomas Bruckner, PhD³; Rainer Abel, MD¹; Claus Carstens, MD¹

¹ Division of Spinal Cord Injury and Spine Center, Department of Orthopaedic Surgery and Rehabilitation Medicine, Ruprecht-Karls-University Heidelberg, Schlierbacher Landstraße 200A, 69118 Heidelberg, Germany. E-mail address for M. Akbar: michael.akbar@ok.uni-heidelberg.de
² Department of Orthopaedic Surgery, Wake Forest University School of Medicine, Medical Center Boulevard, Winston-Salem, NC 27157-1070
³ Department of Medical Biometry and Informatics, Ruprecht-Karls-University Heidelberg, Neuenheimer Feld 305, 69120 Heidelberg, Germany

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Disclosure: The authors did not receive any outside funding or grants in support of their research for or preparation of this work. Neither they nor a member of their immediate families received payments or other benefits or a commitment or agreement to provide such benefits from a commercial entity.

The Journal of Bone and Joint Surgery, Inc.

J Bone Joint Surg Am, 2009 Nov 01;91(Supplement 6):87-100. doi: 10.2106/JBJS.I.00613

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Myelomeningocele (myelodysplasia or spina bifida) refers to a wide range of neural tube defects affecting the spine and spinal cord. These defects result from maldevelopment of the neuropore and the adjacent mesodermal and ectodermal structures during embryogenesis. Myelomeningocele can be classified as either open or closed according to the presence or absence of exposed neural tissue. Myelomeningocele occurs early during embryogenesis, resulting in a certain number of fetuses being spontaneously aborted1. In addition, advances in prenatal screening have led to an increase in the number of therapeutic abortions1-3. Because of the unknown numbers of spontaneous and therapeutic abortions, establishing the true incidence of myelomeningocele is very difficult. Epidemiological studies have revealed that the prevalence of myelomeningocele varies, depending on such factors as ethnicity, race, geography, and temporal trends1. It is estimated that over 4500 pregnancies per year are affected by this malformation in the European Union4, and approximately 3000 pregnancies per year are affected in the United States5. In 2005, the rate of myelomeningocele was 18.0 per 100,000 births in the United States6.

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Introduction

Myelomeningocele (myelodysplasia or spina bifida) refers to a wide range of neural tube defects affecting the spine and spinal cord. These defects result from maldevelopment of the neuropore and the adjacent mesodermal and ectodermal structures during embryogenesis. Myelomeningocele can be classified as either open or closed according to the presence or absence of exposed neural tissue. Myelomeningocele occurs early during embryogenesis, resulting in a certain number of fetuses being spontaneously aborted¹. In addition, advances in prenatal screening have led to an increase in the number of therapeutic abortions^1-3. Because of the unknown numbers of spontaneous and therapeutic abortions, establishing the true incidence of myelomeningocele is very difficult. Epidemiological studies have revealed that the prevalence of myelomeningocele varies, depending on such factors as ethnicity, race, geography, and temporal trends¹. It is estimated that over 4500 pregnancies per year are affected by this malformation in the European Union⁴, and approximately 3000 pregnancies per year are affected in the United States⁵. In 2005, the rate of myelomeningocele was 18.0 per 100,000 births in the United States⁶.

Over the last three to four decades, there has been an overall decline in the prevalence of myelomeningocele in most industrialized countries^7,8. This is in part due to advances in the refined resolution of ultrasonography, the introduction of serum alpha-fetoprotein measurements as a diagnostic modality, elective termination of affected pregnancies, and folic acid supplementation¹. The identification of environmental risk factors, including diabetes mellitus, obesity, smoking, gene polymorphisms, and certain medications, has also contributed to this considerable decline.

The orthopaedic surgeon plays an important role in the multidisciplinary team involved in the care of children with congenital spinal disorders such as myelomeningocele. Myelomeningocele is a complex deformity that can lead to multiple musculoskeletal problems, such as clubfoot, paralytic pes calcaneus, spinal deformities, hip dislocations, fractures, and pressure ulcers. Prior to the 1950s, these problems in patients with myelomeningocele were rare because most patients died before the age of two years^9-12. With advances in medical management, the life expectancy and quality of life for these patients have increased dramatically. This paper outlines our more than twenty-year experience in treating the musculoskeletal sequelae of congenital spinal disorders in children.

Materials and Methods

Between March 1988 and June 2005, 862 patients with congenital spinal disorders were managed on an inpatient basis in the myelodysplasia program at our hospital. There were 471 men and 391 women. For the purpose of this paper, we report our experience with and discuss the challenges of treating the following musculoskeletal sequelae associated with myelomeningocele: scoliosis, kyphosis, hip deformity, paralytic pes calcaneus, clubfoot, fractures of the lower extremity, and pressure ulcers.

Source of Funding

There was no external funding source for this study.

Demographic Data

The prevalence of scoliosis is currently estimated to be between 62% and 90%^13-15. A kyphotic deformity of the lumbar spine is present in 8% to 20% of patients with myelomeningocele^16-18. Most curves have very rigid components, often exceeding 80° at the time of birth, and they progress by as much as 12° each year^16,19-27.

Nearly one-third to one-half of children with myelomeningocele will present at some time during their growth with either a hip dislocation or subluxation²⁸.

The clubfoot is the most common foot deformity in patients with myelomeningocele and is diagnosed in well over 50% of patients²⁹. Calcaneal foot deformity is present in 30% of patients with myelomeningocele³⁰. In the literature, the overall prevalence of pathological fractures is reported to be 20%, with a much higher prevalence in children who have a myelomeningocele at the thoracic level (69%)³¹.

Pressure sores are a major topic in the treatment of these children; pressure ulcers develop in more than 60% of patients who have myelomeningocele³⁰.

Spinal Deformities

Vertebral Anomalies

Children with myelomeningocele have a high prevalence of scoliosis, kyphosis, and lordosis³². Raycroft and Curtis reported that the prevalence of spinal deformity in patients with myelomeningocele was 62%^33,34. These spinal deformities are usually progressive and affect the prognosis of children with myelomeningocele. Vertebral anomalies will eventually lead to skeletal deformities that increase the complexity of the clinical picture and are classified into segmentation, formation, or mixed problems that can occur at any part of the vertebral ring. The most obvious and consistent congenital abnormality is the incomplete posterior arch of the lumbosacral spine. This abnormality affects many aspects of scoliosis and kyphosis treatment. Other congenital malformations include hemivertebrae, butterfly vertebrae, diastematomyelia and unsegmented bars³⁵. Acquired deformities are idiopathic-like scoliosis, pelvic-obliquity-related scoliosis, and neuromuscular curves secondary to asymmetric spinal muscle weakness. Deformities may occur in patients with any level of paralysis and without regard to the ability of the child to walk. The spinal curvature in patients with myelomeningocele often appears at a younger age as compared with that in patients with other developmental abnormalities³⁵. It may be present at two to three years of age and may be severe by seven years of age^15,29,32. Due to the early onset of the deformity, treatment strategies that anticipate the growth of the spine are needed. The development of such strategies represents a major challenge to the surgeon. In addition, in comparison with normal children, children with myelomeningocele have disturbed growth and early skeletal maturity secondary to growth-hormone deficiency³⁵.

Pelvic obliquity and hip deformity affect spinal balance (see Appendix) and are two important factors that contribute to the complexity and difficulty of correcting spinal deformity in this patient population. For example, asymmetric hip contractures may cause lumbar scoliosis, pelvic obliquity, and abnormal lordosis in the standing or sitting position. Similarly, correction of the spine in the treatment of scoliosis may position the lower limbs in a way that prevents functional sitting or standing. When a patient with myelomeningocele has flail or nearly flail extremities but is able to perform independent transfers, the orthopaedic surgeon must observe these transfers preoperatively to determine whether fixation of the pelvis will enable these movements to continue postoperatively.

The goals in the treatment of spinal curvatures in children with myelomeningocele are the prevention of further deformity and the creation of a stable, balanced spine. These children require more precise correction of the spine than children with other types of spinal deformity do, as residual deformity may prevent them from sitting, standing, or walking.

Scoliosis

Carstens³⁶ showed that the prevalence of scoliosis among patients with myelomeningocele is approximately 50%. The prevalence of scoliosis in patients with myelomeningocele has been reported to be as high as 80% to 90% in some studies^13-15. Trivedi et al.³³ provided useful definitions and information regarding the incidence and prevalence of scoliosis in children with myelomeningocele. Scoliosis in this population has been defined as curves with a Cobb angle of >20°³³. Most curves develop early in life, but approximately 40% develop after nine years of age, with some not appearing until fifteen years of age³³. Children with a lesion at the thoracic level or in whom the last intact laminar arch is at the thoracic level have a 90% prevalence of scoliosis³³ (Figs. 1 and 2). Eighty-five percent of these curves are >45°. A lower level of paralysis is associated with a correspondingly lower incidence of scoliosis³⁵. At the L4 level of neurological impairment, the prevalence of scoliosis decreases to about 60%, with only 40% of curves requiring surgical intervention. For children with a level of neurological impairment below L4, the prevalence is closer to 10%³³. Carstens et al. reported that patient age and the neurological level of the lesion are the most important factors in the development of the paralytic spine³⁷. Glard et al.³⁸ suggested that the level of neurological impairment is a predictor of spine deformity.

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Fig. 1: A nine-year-old child with myelomeningocele and severe thoracolumbar scoliosis.

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Fig. 2: Anteroposterior radiograph showing the neurogenic thoracolumbar scoliosis of the child in Figure 1.

Müller and Nordwall found that brace treatment was moderately effective with relatively few complications¹³. However, in our experience with patients with myelomeningocele, the conservative treatment of scoliosis with a brace (a Wilmington corset) is not effective. Hence, we recommend that a corset should only be worn to stabilize the trunk while the patient is sitting (Fig. 3). In addition, paralytic curves are notoriously resistant to brace therapy, and children with a myelomeningocele can experience skin problems, including pressure ulcers, which are challenging to heal. The use of a spinal brace is only a temporary measure that can permit the delay of surgery until the child is eight or nine years old^29,32,39.

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Fig. 3: A five-year-old patient with a myelomeningocele at the thoracic level. The spinal brace is used to stabilize the trunk. The use of a brace is only a temporary conservative treatment option that can permit the delay of surgery until the child is eight or nine years old.

If scoliosis continues to progress after neurological problems (e.g., syringomyelia and tethered cord) have been corrected, orthopaedic treatment is indicated. Curves with a Cobb angle of =30° can generally be observed, but progressive curves require surgical intervention³⁵. For unbalanced curves of >30°, the center of gravity falls outside the pelvic base of support, the spine becomes unstable, and progression of the deformity is almost assured³⁵ (Fig. 4).

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Fig. 4: Radiographs showing progression of deformity of a c-shaped thoracolumbar scoliosis during conservative treatment in a spinal brace.

We carried out a retrospective analysis of eighty-four patients who had paralytic scoliosis due to myelomeningocele and who underwent surgical treatment sometime within the years of 1988 to 2005. The aim of this study was to compare different surgical techniques and to identify clinical parameters that influence long-term results. The patient cohort was stratified by the surgical techniques that were applied: the patients in Group I underwent posterior fusion with instrumentation; the patients in Group II underwent anterior fusion without instrumentation and combined with posterior fusion with instrumentation; and the patients in Group III underwent anterior and posterior fusion with instrumentation. The average patient age at surgery was 10.7 years. A preoperative mean scoliosis angle of 97° (range, 73° to 112°) was primarily reduced to 37° (range, 30° to 50°). At the time of the final follow-up (mean, 15.3 years), mean correction deteriorated to 50° (range, 36° to 71°). The procedure used in Group III resulted in a better long-term correction of scoliosis compared with that used in Group I (p = 0.01). Extension of the anterior fusion correlated with primary and midterm correction of scoliosis (p < 0.02). This finding can be attributed mostly to the patients in Group I (p = 0.001). Hardware complications occurred in twenty-six patients (30%), with hardware failure being responsible for excessive loss of correction. Relative loss of correction among these patients was high (p = 0.001). As a result of this review, we recommend anterior and posterior fusion, each with instrumentation, for the treatment of paralytic scoliosis in patients with myelomeningocele. In patients with paralysis as a result of a lesion at the thoracic level, the two-stage procedure is mandatory to reduce the risk of hardware complications and subsequent major loss of correction.

The indications for extending the fusion mass to the sacrum (S1) are not well established. The lack of a posterior vertebral arch makes lumbosacral arthrodesis very difficult to obtain in children with myelomeningocele. In addition, pseudarthroses and instrumentation failures are frequently observed. If a successful fusion to the sacrum is obtained, it may deprive ambulatory patients of the ability to walk. Conversely, if the lumbosacral joint is not fused, the scoliosis tends to increase, unless the lumbar curve can be corrected to <20° and the pelvic obliquity can be corrected to <15°^29,40-42. Therefore, it is important to treat the scoliosis while the curve is small.

Unfortunately, many patients present with an advanced stage of scoliosis, which makes treatment very challenging. The level of surgical fusion depends on the age of the child, the location of the curve, the level of paralysis, and the ambulatory status. It is well documented that fusing short sequences is a mistake. In general, patients with a thoracic or upper lumbar level of neurological impairment should undergo fusion to the sacrum. In children with paraplegia resulting from a lesion at the lower-lumbar or sacral level, the lumbosacral joint should be spared if the patient is able to walk and the spine can be aligned satisfactorily³⁵. The sagittal deformity also must be evaluated because increased lumbar lordosis is a common deformity. Assessment of sitting, supine, and standing posture must be made before correcting the lumbar lordosis. These children often require a greater degree of lordosis than normal. Restoring the lumbar lordosis to the normal range may uncover a hip flexion contracture and prevent the patient from standing and walking. The degree of lordosis left in the spine after fusion needs to be determined on a patient-by-patient basis. It is best to treat the hip contracture before correcting the spine.

Lumbar Kyphosis

Kyphotic deformity of the lumbar spine is present in 8% to 20% of patients with myelomeningocele^16-18. Because the neural tube failed to close during development in these patients, structures are missing in the dorsal spinal column, causing the deformity. In addition to the detrimental effects on the neuronal structures in the spinal canal, the extensor muscles are displaced anteriorly. In this position, they no longer antagonize the iliopsoas muscle to ensure erection of the spine; rather, they force the lumbar spine into kyphosis¹⁷. It is widely agreed that, in most patients, deformity increases during childhood and that the increase in kyphosis is expected even after the completion of growth^24,27,43-50. The deformity is more marked in patients with high neurological lesions^17,50. Carstens et al. found apparent lumbar kyphosis on the lateral radiographs of 20% of approximately 700 patients with myelomeningocele¹⁷. Kyphosis deformity was particularly common in patients with paraplegia resulting from a thoracic or upper lumbar-level lesion. The kyphosis deformity has been described as paralytic and congenital. The paralytic type of kyphosis is seen much more commonly and can be divided into two types: collapsing kyphosis, and rigid sharp-angled kyphosis. Most curves have very rigid components, often exceed 80° at birth (Fig. 5), and progress by 6° to 12° per year^16,19-27.

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Fig. 5: Photograph showing a lumbar kyphosis with rigid components. The kyphosis exceeded 80° at the time of the child's birth.

In our experience, patients with lumbar kyphosis usually show deformities of the posterior arch that start in the lower thoracic spine and extend mostly to the sacrum. Preservation of neurological function below the level of T10 to T12 is rare, and compensatory hyperlordosis of the adjacent thoracic spine and the sacrolumbar junction may also be present (Fig. 6). Untreated, this change in the sagittal profile may cause respiratory difficulties because the respiratory muscles cannot function efficiently and the intestinal organs are crowded. The increased flexion of the trunk also may interfere with drainage of urine through the ureters, whereas the aorta crosses the kyphotic arc like a bow string. Finally, the lower rib cage comes to rest against the anterior aspect of the thighs (Fig. 7). In addition to limiting the functional capacity of the patient, the condition imposes severe postural problems, including the inability to lie supine. A special problem is the development of pressure sores over the apex of the kyphosis, which carries the risk of causing intraspinal infection and meningitis and has the potential to cause deep infections after surgical correction. The options for conservative management are limited. Bracing is extremely difficult, rarely effective, and, in advanced stages, impossible¹⁶.

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Fig. 6: Three-dimensional computed tomography reconstruction of a lumbar kyphosis in an eleven-year-old child with myelomeningocele. The kyphosis is associated with compensatory hyperlordosis of the adjacent thoracic spine and the sacrolumbar junction.

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Fig. 7: Photograph showing a child with myelomeningocele and severe rigid lumbar kyphosis. The lower portion of the rib cage has come to rest against the anterior aspect of the thighs.

Various surgical techniques with use of Luque rods, including anterior strut-grafting and spinal osteotomy with vertebral body excision, have been described to correct and stabilize lumbar kyphosis^18,27,51. Sharrard⁴⁴ and Lindseth and Stelzer¹⁸ first described resection of the vertebral bodies for the treatment of kyphosis. These techniques employed short stabilization and required prolonged postoperative immobilization. Improved results were published in 1987 by Heydemann and Gillespie²², who described long instrumentation with thoracic Luque rod wire fixation and distal anchoring in the sacrum and pelvis. Carstens et al.⁵² reported on thirty-three kyphectomies in patients with myelomeningocele. Stratification by kyphosis type revealed that the best results were obtained in the group that had the congenital rigid type of kyphosis. A marked postoperative deterioration was observed in patients who had the flexible paralytic type of kyphosis, probably due to the short fusion⁵². In the same year, Warner and Fackler reported a modified sacral anchoring technique⁵¹. Their initial series of twelve patients in whom the distal end of a pre-bent rod had been passed through the first sacral foramina to achieve better stabilization showed promising results. On the basis of this report, we implemented this technique at our institution as a standard procedure for treating children with lumbar kyphosis due to myelomeningocele. Three of us and others at our institution reported on twenty-four patients who underwent kyphectomy with use of this technique¹⁶. There was almost no loss of correction postoperatively, and, with use of a geometric force model, it was pointed out that a good correction of the lumbar kyphosis is necessary to avoid implant failure¹⁶.

A more recent retrospective review of our database revealed twenty-eight pediatric patients with myelomeningocele and lumbar kyphosis who underwent kyphectomy with use of the Warner and Fackler technique⁵¹ (Figs. 8-A, 8-B, and 8-C). The mean length of surgery was 3.7 hours (range, 1.5 to 7.6 hours), and the mean blood loss was 1160 mL (range, 300 to 3000 mL). Despite the use of a cell saver in order to retrieve some of the lost blood, all patients required blood substitution during or immediately after surgery. The mean extent of the lumbar kyphosis could be corrected from 131° (range, 90° to 170°) before surgery to 45° (range, 15° to 90°) after surgery. This represents an average correction of 86° (range, 40° to 130°). On the average, 2.5 (range, 1 to 4) vertebrae were resected. At the time of final follow-up, the average extent of kyphosis was 45° (range, 15° to 100°), with a mean loss of correction of 5° (range, 8° to 55°). Growth of the spine was observed in those parts of the thoracic spine where the Luque wire instrumentation had been performed. The crankshaft^53,54 phenomenon occurred in one patient and was attributable to the thoracic stabilization. Functional outcome was assessed very favorably by patients as well as caregivers. All patients were able to sit without manual support or braces.

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Fig. 8-A: Fig. 8-A Photograph showing lumbar kyphosis with a pressure sore at the apex. Fig. 8-B Preparation of the lumbar kyphosis before resection. Fig. 8-C Resection of the apex vertebra and stabilization with use of the Warner and Fackler technique.

Long-term complications consisted of implant failure in nine patients and the need for revision surgery in five patients. In four of these patients, the rods had penetrated through the sacrum (Fig. 9) at twenty-four, twenty-six, thirty-two, and thirty-three months, respectively, after correction. In two patients, the rods needed to be explanted, with a loss of correction of as much as 55°. In an additional five patients, rod breakage (Fig. 10) was detected at twelve, fifteen, eighteen, twenty, and twenty-two months, respectively, after correction. In three patients, the broken rods were exchanged. Implant failure was not related to age or the initial degree of kyphosis. Implant retrieval and subsequent biomechanical analysis showed a correlation between implant failure and the remaining deformity after surgery. The force that loads the anchoring rods depends on the residual distance between the apex of the kyphosis and the plumb line, and both correlate significantly with implant failure¹⁶. No correlation was found between implant failure and estimated weight of the trunk, length of the rods, or the parts thereof above or below the apex. In addition, no correlation to the length of the spine, representing body height, was seen. On the basis of these results, corrective surgery should be performed with the intention to reestablish the sagittal profile as much as possible to reduce the risk of implant failure.

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Fig. 9: Implant failure. Rod penetration through the sacrum.

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Fig. 10: Implant failure. Rod breakage and dislocation.

Hip Deformities

Hip joint contractures, subluxation, and dislocation are very common in patients with myelomeningocele. The presence of hip deformity is related to the neurological level of the disorder but not to the amount of muscle imbalance⁵⁵. Treatment of a hip deformity depends on the neurological level of the disorder, the specific deformity, and the functional level of the individual child.

The child with a thoracic or upper-lumbar (L1-L2) lesion does not have sensation in or muscle control of the lower extremities. There is little, if any, meaningful strength across the hip joint in this group of patients, and deformity is usually secondary to positioning and muscle spasm. Although many of these children are able to walk (therapeutic walking) early in life with extensive bracing and physical therapy, they can rarely do so after the age of ten. Without sensation and motor control, reduction of an unstable hip is not necessary for the purpose of sitting and walking with use of orthotic aids. However, these children need a functional range of motion of the hip without contracture⁵⁶. The goal of surgical procedures should be to provide a range of motion such that the patient can sit satisfactorily in a wheelchair, lie comfortably in bed, and use an orthosis for standing and walking. Soft-tissue releases may be indicated for contractures of >20° to maintain the ability to wear a brace and facilitate walking. An anterior hip release usually involves the iliopsoas, sartorius, rectus femoris, tensor fasciae latae, and hip capsule. Treating hip contractures in children with myelomeningocele can be frustrating because many contractures return promptly, often to a worse degree than before the surgical release³⁰. It is important that prolonged bracing and physical therapy be used to maintain whatever motion has been obtained at surgery. This can be accompanied by botulinum toxin injections. Osteotomies, although rarely indicated, can be useful in treating severe flexion deformities (>60°) or pressure sores resulting from the hip deformity.

The patient with a middle or lower-lumbar (L3-L5) lesion has sensation distal to the knee. Muscle function includes hip flexion and adduction, knee extension, and weak knee flexion. These patients have the potential for control of the hip and knee, and therefore the ability for independent walking, but the hip is a major source of deformity and disability. The natural history is for the hip to undergo progressive dysplasia and dislocation in association with the development of a hip flexion contracture, which becomes evident when the child begins to walk. Attempts to reduce the hip and correct the progressive deformity are rarely successful unless there is correction of the forces about the hip³². Regardless of the cause, hip subluxation or dislocation occurs in 50% to 70% of children with myelomeningocele and a middle or lower-lumbar level of paraplegia³⁰. It is recommended to reserve surgical treatment for ambulatory children who have unilateral hip dislocation, strong quadriceps, and a stable neurological level^57,58. Unilateral dislocations are of greater concern because they interfere with ambulation by creating pelvic obliquity and limb-length discrepancy. Unilateral hip instability is also associated with scoliosis, but, interestingly, there is no correlation between the side of instability and the direction of the curve³³. It has been shown that bilateral dislocation does not have a negative effect on function⁵⁹; therefore, these children do not require surgical treatment unless the dislocations are associated with a fixed flexion contracture.

Treating a hip problem that developed in the first year of life is particularly challenging in children with myelomeningocele. Traditional bracing and surgical treatment have been reported to result in a failure rate of 50%⁶⁰. Many authors prefer to wait until the child is one year of age before carrying out hip reduction to avoid interfering with motor development²⁹. If the child is between one year and six years of age, a balanced stable hip can be produced with open reduction and capsular plication along with anterior release. If there is deformity of the femoral neck, a varus femoral shortening osteotomy is indicated. Pelvic osteotomy is indicated for severe acetabular dysplasia⁶¹.

Foot Deformities

Paralytic Pes Calcaneus

Approximately 30% of children with myelomeningocele have a calcaneal deformity³⁰. Paralytic pes calcaneus is extremely difficult to treat, either surgically or conservatively with use of an orthosis, or even with use of a combination of the two. The calcaneal deformity often develops when the neural deficit is at the fourth and fifth lumbar nerve root, and it may develop as a result of weak plantar flexion and stronger dorsiflexion. This causes loss of normal toe-off and results in a crouching gait. Heel pads may develop (Fig. 11) as well as pressure sores (neurotrophic heel ulcer) on the heel pad and secondary osteomyelitis. These further deforming changes reduce the length of the lever arm of the triceps surae and aggravate the loss of strength.

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Fig. 11: Photograph showing calcaneal deformity of the foot with development of heel pads. The patient walks on his heels.

With classic treatment options, the aim is to increase the strength of the triceps surae muscles and/or stabilize the calcaneus⁶². One drawback of this approach is that the tendons that can be transferred to augment the triceps surae muscle typically lack sufficient strength, as the combined power of all the ankle muscles together is less than the power of the triceps surae muscle⁶². The other challenge is that the transferred tendons may be stretched so far that the muscle strength is completely lost⁶². On the basis of our previous experience with unsatisfactory results of surgical treatment of paralytic pes calcaneus solely by tendon transfer, we developed a new technique for treating this condition, with the following aims: (1) to reduce passive dorsiflexion in the ankle joint by not more than 10°, (2) to restore passive plantar flexion in the ankle joint by as much as 20° and more, (3) to restore the muscle strength of the triceps surae, (4) to improve the stabilization of the forefoot, (5) and, if necessary in a second session, to augment the dorsiflexors. This new surgical procedure combines an active (tendon transfer) with a passive (ventral bone block) technique⁶². The inverse Lambrinudi procedure is a triple arthrodesis (Fig. 12) of the talus, calcaneus, navicular, and cuboid combined with the insertion of a bone wedge between the talus and the calcaneus (Fig. 13). By inserting the bone wedge between the talus and the calcaneus, a ventral bone block (dorsiflexed talus) is produced, which inhibits dorsiflexion at the ankle joint. Transferring six tendons (tibialis anterior and posterior, peroneus brevis and longus, and extensor digitorum and hallucis longus) to the Achilles tendon creates an augmentation. The surgical exposure and procedure for this new technique have been reported recently by Wenz et al.⁶² (Fig. 14). In their small series of nine consecutive patients with myelomeningocele (six men and three women; a total of fourteen feet [eight left and six right]), the average age of the patients was 17.5 years (range, 9.0 to 47.0 years) at the time of corrective surgery. All nine patients had difficulty in walking because of a calcaneal deformity that caused a stilted gait. Excellent results were achieved in eight of the nine patients. All patients were satisfied with the outcome of surgery and the functional improvement. All patients except one could walk independently without assistive devices. One patient still required an orthosis but to a lesser degree than before the surgery. According to the grading scale of the British Medical Research Council (in which a grade of 5 indicates normal strength)⁶³, the strength of the triceps surae muscle increased from an average of 1 preoperatively to 4 postoperatively. According to the American Orthopaedic Foot and Ankle Society hindfoot scale⁶⁴, in which the maximum possible score is 100 points (50 possible points for pain; 40, for function; and 10, for alignment), pain and function improved significantly (p < 0.004) from an average of 42 to 72 points. Radiographic analysis revealed that the talocalcaneal angle had improved from a mean of 55.4° (range, 40° to 66°) preoperatively to 14.8° and that the calcaneal pitch advanced from a mean of 25.6° preoperatively to 6.2°. The lateral first talometatarsal angle improved from a mean of -11.8° preoperatively to +28.8°. All of these improvements were significant (p < 0.004). In two feet, an additional Hiroshima procedure⁶⁵ (augmentation surgery with the flexor digitorum longus and flexor hallucis) was performed at twelve months after the index surgery due to a disabling footdrop.

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Fig. 12: Left: Radiograph showing the indication for the Lambrinudi osteotomy to increase dorsiflexion in a foot with an equinus deformity and talotibial impingement (arrow). Right: Radiograph showing the indication for inverse Lambrinudi osteotomy to limit dorsiflexion (arrow) in a foot with a paralytic calcaneus deformity.

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Fig. 13: A and B: The clinical (A) and radiographic (B) appearance of a calcaneal deformity of the foot in a patient with myelomeningocele. C and D: Implantation of a bone wedge between the talus and the calcaneus limits dorsiflexion.

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Fig. 14: Postoperative result after performance of an inverse Lambrinudi osteotomy and tendon transfer to the Achilles tendon in the patient depicted in Figure 11.

Clubfoot (Equinovarus) Deformity

Clubfoot (Fig. 15) is the most common foot deformity in patients with myelomeningocele; it occurs in more than 50% of these patients²⁹. It is present in patients with paraplegia at any level, but the treatment differs according to level as a result of the muscle function that may be present. The incidence of this foot deformity is about 90% in patients with paraplegia caused by a lesion at the thoracic or lumbar level and about 50% in patients with paraplegia caused by a lesion at the sacral level. The typical clubfoot deformity in these children is often present at birth, is quite rigid, and is difficult to treat. It is frequently refractory to conservative treatment. Clubfoot can not only impair walking but also create sitting problems when the feet cannot be positioned properly on footrests. This deformity can also lead to pressure sores, especially on the dorsolateral aspect of the foot.

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Fig. 15: Neurogenic clubfoot in a patient with myelomeningocele.

Most often, conservative treatment will not prevent the patient from requiring surgical correction; thus, some surgeons defer all treatment options until the time of surgical release²⁹. We would not suggest cast treatment for clubfoot in newborns with myelomeningocele because of the high risk of the development of pressure sores. Corrective surgery should be delayed until the patient has developed to the point of being ready for brace-fitting and walking or standing. One of the advantages of performing surgery at the time that the child is ready to stand is that weight-bearing can be used as an adjunct to the orthosis in maintaining the correction. The surgical correction of the clubfoot is rarely accomplished with limited surgery; often, an extensive subtalar circumferential release is necessary^66,67.

We reviewed 167 equinovarus deformities in 123 patients with myelomeningocele who underwent surgery at our hospital. The goal of surgical treatment was to achieve a plantigrade position of the foot to make it possible for the child to use an orthotic device. This goal was achieved in 83% of the patients, with 17% requiring a second surgical procedure. The surgical approach had been carried out with use of a circumferential incision (more commonly referred to as the Cincinnati incision)⁶⁸, and patients were treated postoperatively with casts for at least six weeks, followed by the use of special shoes and night splints. Surgical correction led to plantigrade feet in 95% of the patients in Group I (the level of the lesion in this group ranged from the thoracic level to L2) and 83% of the patients in Group III (the level of the lesion in this group ranged from L5 to the sacral level). Surgical outcomes for the patients in Group II (the level of the lesion in this group ranged from L4 to L5) were satisfactory, with 64% having plantigrade feet. Within all types of paralysis, there was a better result after performing a tenotomy of the muscle tendons underlying the pathology. In comparison with the reported data of Sharrard and Grosfield⁶⁹, the low revision rate in our cohort is probably associated with the extensive Cincinnati exposure, tenotomy of the muscle tendons underlying the pathology, and extensive circumferential subtalar release. Recurrent clubfoot deformity after primary correctional surgery is unfortunately not uncommon (20% to 50%), probably as a result of incomplete correction or an unrecognized muscle imbalance. With a recurrent clubfoot deformity, peritalar release or talectomy (astragalectomy) should be performed. With these surgical procedures, good results can be achieved.

Fractures

Bone forms in response to stress and, conversely, bone is resorbed when there is little or no stress. Therefore, it is not unexpected that the bones of the lower limb of paralyzed patients rapidly become thin and osteopenic when they are not loaded. The combination of sensory loss with osteopenic or outright osteoporotic bone is a setup for fracture in children with myelomeningocele. Barnett and Menelaus³¹ reported an overall prevalence of pathological fracture of about 20%, with a much higher prevalence (69%) in children with thoracic lesions (Fig. 16). Such fractures seldom occur in ambulatory patients with low-level sacral lesions. Quan et al.⁷⁰ assessed the bone mineral density of the distal part of the radius in an unselected group of patients with myelomeningocele. They found that all patients with myelomeningocele had a lower bone mineral density when compared with a normal population. In addition, patients who had a history of fracture had densities that were lower than those of patients who did not have a history of fracture. Recently, Apkon et al. reported that the femoral neck z-score in children with myelomeningocele differed significantly according to ambulatory status⁷¹. The lowest z-scores were observed in children who were wheelchair-dependent. In addition, the mean z-score at the femoral neck decreased as the level of the lesion rose. Surprisingly, the authors could not establish a relationship between fracture and reduced bone mineral density⁷¹. The relationship between fracture and reduced bone mineral density in patients with myelomeningocele is a source of controversy, and it needs clarification in future studies.

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Fig. 16: Pathological fracture of the distal part of the femur in a child with a myelomeningocele at the thoracic level. Note the exuberant callus formation.

A major obstacle in the management of patients with myelomeningocele is the increased prevalence of fracture in the paralyzed lower extremities. Fractures restrict the mobility and independence of these patients and often necessitate long and costly periods of hospitalization. Some of these fractures can occur during the night as the patient simply turns in bed. Clinically, these fractures are frequently misdiagnosed because the symptoms are similar to those of an infection. A fracture in patients with myelomeningocele most often causes a painless swelling, hyperthermia, and hyperemia in the affected limb. Furthermore, fever and leukocytosis can develop and the erythrocyte sedimentation rate can increase, which complicates the diagnosis by suggesting infection. Hence, radiographic evaluation is the key to the correct diagnosis, as the fracture patterns are unique and different from those seen in children who are neurologically intact. Diaphyseal or metaphyseal fractures are easily recognized. Epiphyseal fractures may be confusing, however, as they often present with only a widened physis, subperiosteal new-bone formation, or a widened, irregular metaphysis.

In some studies, the prevalence of fractures has been reported to be between 9% and 20%. Epiphyseal fractures occur frequently in patients with myelomeningocele, at rates that range from 4% to 9%, and these fractures can affect further bone growth. In contrast to other kinds of fractures, epiphyseal injuries mainly seem to affect the children with myelomeningocele who can walk relatively well. Some authors have suggested that the level of paralysis may be an important factor in the development of fractures.

Treatment consists of brief immobilization with a cast or fracture brace because these types of fractures usually heal quickly with exuberant callus. However, complications such as malunion, delayed union, and physeal growth disturbance have all been reported^72,73. Physeal fractures may be slow to heal, and they require periodic reevaluation to detect subsequent growth disturbance⁷⁴. Refracture following postoperative cast immobilization is common, and several studies have reported prevalences ranging from 18% to 45%²⁹. Prevention should include early weight-bearing and keeping the time of plaster immobilization to a minimum. Failure to follow these principles can lead to increased osteopenia and refractures.

Pressure Ulcers

Despite medical improvements in managing this unique patient population, pressure ulcers remain a substantial challenge for both patient and physician. Pressure sores are an important issue in any population with sensory deficits, and patients with myelomeningocele can have major skin problems. Pressure sores develop in as much as 60% of these patients, typically on the feet and over the buttock, the sacrum, the ischial tuberosities, and the greater trochanter³⁰. These skin lesions can be very difficult to manage. Aside from the obvious neurological problems, patients with myelomeningocele have other issues that contribute to the development and perpetuation of pressure ulcers. The postural and positional problems that develop, including kyphosis, hip dislocation, and joint contractures, all lead to abnormally prominent bones.

There are numerous factors that affect our decision-making about the use of a muscle, myocutaneous, or axial flap to treat chronic pressure ulcers. These considerations must be weighed in aggregate because no single factor is usually overriding in its importance. In the past, we have been guided by the so-called reconstructive ladder^75,76 (simple closure, skin grafts, local flaps, regional flaps, pedicled flaps, and, finally, free flaps) as the primary determinant in choosing a logical surgical approach. Between 1988 and 2005, there were 415 admissions solely for the treatment of pressure ulcers at our institution. Of the 415 patients, 137 patients underwent surgical intervention. The most common ulcer locations were the ischial tuberosities (32%), the feet (16%), the sacrum (15%), and the heel (13%). The mean number of primary ulcers per patient was 1.4, and the mean number of recurrent ulcers was 1.7. An analysis of ulcer severity⁷⁷ revealed that 2.5% of the ulcers were at grade I; 29.5%, at grade II; 24.9%, at grade III; 14.8%, at grade IV; and 28.3%, at grade V. The mean size of the primary ulcer was 18.4 cm², with a mean depth of 2.6 cm. When seeking the underlying cause of these pressure ulcers, we found that 51% were related to the use of medical devices; 22%, to cast treatment; 13%, to injury of the skin; 7%, to a wheelchair cushion; and 7%, to a mattress. Surgical treatment of pressure ulcers was predominantly performed with use of myocutaneous flaps in 60%, followed by a mesh graft in 7% and fasciocutaneous flaps (Fig. 17) in 5%.

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Fig. 17: Photographs showing treatment of a small sacral pressure sore with a fasciocutaneous flap.

Conclusion

In the last three decades, great advances have been made in the medical management of children with congenital spinal disorders, and these advances have led to a longer life span for these patients. Because of the complexity of the disease, orthopaedic surgeons frequently face challenging sequelae. However, properly selected surgical procedures can often improve the patient's quality of life.

Appendix

Videos demonstrating the development of pelvic obliquity after hip dislocation and the progression of scoliosis in a patient with myelomeningocele are available with the electronic versions of this article, on our web site at (go to the article citation and click on "Supplementary Material") and on our quarterly CD/DVD (call our subscription department, at 781-449-9780, to order the CD or DVD). Image Not Available

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Topics

fracture ; calcaneus ; decubitus ulcer ; meningomyelocele ; spinal diseases ; braces ; congenital clubfoot ; congenital kyphosis ; scoliosis ; spine

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Michael Akbar, Bjoern Bresch, Thorsten M. Seyler, Wolfram Wenz, Thomas Bruckner, Rainer Abel, Claus Carstens; Management of Orthopaedic Sequelae of Congenital Spinal Disorders. The Journal of Bone & Joint Surgery. 2009 Nov;91(Supplement_6):87-100.

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