Open access peer-reviewed chapter

Orthopedic Approach to Spina Bifida

Written By

Roselle C. Okubo, Claudio Silveri and Ana C. Belzarena

Submitted: July 7th, 2020 Reviewed: November 4th, 2020 Published: November 28th, 2020

DOI: 10.5772/intechopen.94901

Chapter metrics overview

459 Chapter Downloads

View Full Metrics


Spina bifida is a common nervous system malformation and it encompasses a wide array of presentations with diverse orthopedic challenges. Manifestations of this disease can include dislocates hips, joint contractures, spine deformity such as scoliosis or kyphosis, clubfeet and limb rotational deformities. Additionally, many of these patients are non-ambulatory and prone to osteoporosis induced pathological fractures. The care of spina bifida patients is a challenging one, requiring many health care professionals from different areas to be working in conjunction. Nowadays, spina bifida patients live longer due to advances in health care and improving the quality of life of these patients is paramount.


  • spina bifida
  • myelomeningocele
  • orthopedic surgery

1. Introduction

Spina bifida is the most common nervous system malformation. This complex disease can be considered as a group of congenital defects caused by a failure in the closure of the neural tube at the fourth week of the embryonic phase [1]. The true incidence may vary from country to country but overall is at 0.5 per 1000 births [2]. Additionally, gender prevalence is more in girls than in boys, but again it varies geographically [3]. There are mainly two categories of spina bifida, open and closed ones. The open types which include meningocele and meningomyelocele have neural tissue exposed and are more severe in terms of symptoms and prognosis [4]. Closed spina bifida or occulta, has no neural tissue exposed and includes from lipomeningocele to just a sinus tract [5]. Majority of these neural tube defects are located at lower levels of the spine, mostly in the lumbar and sacral levels [6]. These defects can be diagnosed prenatally with ultrasound imaging or maternal alpha-feto-protein levels measured on the mother’s serum. Patients with spina bifida can often present with neurological deficits, motor or sensory and orthopedic conditions such as joint contractures, spine deformity, clubfeet and hip dislocations among others. The degree of the deficit and the orthopedic presentation are related to the spine level where the defect is present [4].


2. Non-orthopedic health conditions

Besides the orthopedic associated conditions, these patients can present with several other health problems. The mortality of these patients has decreased throughout the years with enhanced medical care, thus now more attention is driven at improving these patients’ quality of life [7]. Intellectual disability is present only in approximately 20% of the patients and is usually the consequence of hydrocephalus [8]. Patients usually present with bladder and/or bowel incontinence, renal failure, propensity to infections and skin ulcers due to skin insensitivity, hydrocephalus, tethered cord and Arnold Chiari II type of malformation [9]. One in three of these patients will be allergic to latex, some having anaphylactic reactions. This is thought to be the consequence of repeated surgical and medical procedures, thus the importance of avoiding latex material since the beginning of care [9].


3. Pathologic fractures

Due to the lack of ambulation, physical exercise and axial bone load spina bifida patients can present with osteoporosis and osteoporosis induced fractures [10]. The fractures usually occur below the neurological level of the defect and the incidence ranges from 11 to 30% [11]. The fracture mechanism is usually pathologic, these fractures usually being caused by minor trauma or even spontaneously [12]. Since many of these patients may have a fractured bone without an obvious trauma mechanism it can be difficult to diagnose these fractures. Patients usually present with a swollen, warm extremity with associated redness, and this should prompt obtaining a radiographic imaging study [13]. The caring orthopedist should be aware not to confuse these symptoms with an infection. The fractures are common the higher the level of the neural defect, in the distal femur or around the hip in patients from 3 to 7 years old (Figure 1) [14]. Treatment is usually non-surgical and involves immobilization in a cast. Prolonged immobilization in the cast should be minimized since this also will make osteopenia worse [15]. Patients should be assessed for bone density with dual-energy X-ray absorptiometry (DEXA scan) exams and calcium and vitamin D levels should be assessed and replaced if necessary, by the pediatrician. Weight bearing and physical exercise should be encouraged as appropriate [16].

Figure 1.

Right distal femur fracture in a myelomeningocele patient without an obvious traumatic mechanism (A) and radiographic images of post-reduction and casting (B).


4. Spine care

Besides the posterior element defect in the spine, spina bifida patients also present with severe congenital deformity and contractures of the spine. These deformities can pose a restriction to everyday activities as well as pulmonary function [17]. A third of the patients will have scoliosis, which is usually of an early onset and has a tendency to progress and cause pelvic obliquity [18]. Scoliosis has different causes in these patients such as muscle imbalance or primary malformations like hemivertebra and vertebral fusions. Kyphosis may also be present in approximately 15% of the patients (Figure 2). Is usually progressive and mostly located in the lumbar region [19]. The deformity can be so severe to cause skin breakdown at the level of the deformity (Figure 3). Surgery is necessary to correct the deformity and is not free of complications in these patients. Usually there is no role for bracing spine deformity in these patients and the skin insensitivity can predispose to skin ulcers and infection. Surgical correction is indicated in patients with progressing curves who are good candidates for surgery. Posterior fixation is the most common procedure performed but other options such as an anterior fusion or combined ones are used as well when appropriate. In patients with pelvic obliquity the fixation should be extended until the pelvis level, this is particularly important in non-ambulatory patients (Figure 4) [20]. Surgery can be associated with higher risks of infection, anesthesia complications, bleeding, non-union, hardware failure, loss of correction, pressure sores, subsequent operations and even death (Figure 5) [21]. Some studies have suggested a higher rate of union when using a combined anterior and posterior approach [22].

Figure 2.

Myelomeningocele patient with marked lumbar kyphosis.

Figure 3.

Myelomeningocele patient with marked kyphosis with skin breakdown at the level of the deformity (A) and accompanying radiographic images of the deformity (B).

Figure 4.

Myelomeningocele patient radiographic image depicting scoliotic curve (A) and postoperative radiographic study depicting spinopelvic fusion (B).

Another spine problem spina bifida patients may present with is tethered cord syndrome. This occurs when the spinal cord is stretched because it remains attached distally, usually to scar tissue from prior surgical procedures. Most patients have some degree of cord tethering but only 30% manifest clinically. Patients who have symptoms present with progressive scoliosis, new gait abnormalities or changes, weakness, spasticity or back pain [23]. Neurosurgeons are the specialists who treat this problem surgically by untethering the cord.

Figure 5.

Postoperative skin breakdown and infection in a myelomeningocele patient.


5. Hip

Thirty percent of the spina bifida patients present with hip dislocations either at birth or during their childhood (Figure 6) [24]. The number can go up to 50% if we include hip subluxations. Dislocation occurs more commonly when the spinal cord defect is at the L3 level and the patient has a muscle imbalance with unopposed hip flexion and adduction. The ability of a patient to walk does not seem to be affected by dislocation of the hips and surgical relocation does not necessarily translate in a functional improvement [25]. Additionally, this problem does not seem to cause pain to the patients. For all these reasons many orthopedic surgeons advocate against putting the patients through complex osseous and soft tissue procedures and surgical intervention can even be considered controversial in such scenario where a benefit will not necessarily be obtained and such interventions are not exempt from surgical complications [26, 27].

Figure 6.

Bilateral hip dislocation and osteopenia in a 14-years-old patient with spina bifida.


6. Knee deformities

The most common knee problems spina bifida patients present with are knee flexion contracture and knee extension contracture [13]. Less commonly valgus deformity and instability [27]. There are many causes for those deformities such as muscle imbalance, fibrosis of the surrounding tissues and eventually a fracture malunion. A flexion contracture can usually be present at birth, different form the flexed knee found in healthy newborns, in myelomeningocele patients this deformity is fixed and more difficult to treat. The higher the level of the spinal cord defect the more severe is the knee contracture [28]. Patient positioning and muscle imbalance are thought to be involved in the genesis of this deformity. If the patient is non-ambulatory the fixed knee flexion contracture does not cause any functional impairment, but in ambulatory patients it should be addressed. Surgical treatment is indicated when the flexion contracture is >20 degrees [29]. Treatment usually involves the releasing of the surrounding soft tissues such as hamstrings, gastrocnemius and posterior capsule. In more severe cases and usually in older patients an extension osteotomy may be indicated as well [30].

Knee extension is also usually present at birth, usually bilateral and much less common than the flexion contracture (Figure 7). The treating orthopedic surgeon should be aware of other associated deformities such as ipsilateral hip dislocation, external hip contracture and equinovarus foot [31]. If the patient presents with hip dislocation and knee extension deformity simultaneously at birth, the knee deformity should be addressed first, so that the newborn can afterwards, once the knee deformity has been corrected, wear a Pavlik harness to treat the hip dislocation (Figure 8) [32]. The treatment for the knee extension deformity consists in serial casting until a 90 degree flexion is achieved (Figure 9). The treating orthopedic surgeon should be aware of not utilizing much force to flex the knee since the distal femur can be bent and even fractured in extreme cases. Casting should be followed by physical therapy. In resistant cases where casting is not successful surgical intervention is indicated. The surgical procedure usually consists of V-Y quadriceps lengthening and anterior capsulotomy [33].

Figure 7.

Newborn with congenital knee dislocation in extension (A) and front and lateral radiographic image depicting the knee extension deformity (B).

Figure 8.

Newborn wearing a Pavlik harness, the harness requires the knee to be bendable in order to fit appropriately.

Figure 9.

Extension deformity being treated by serial casting aiming a achieving a 90 degrees knee flexion.


7. Foot deformities

Foot and ankle deformity are very prevalent in spina bifida patients, with an incidence ranging from 60 to 90%. They can be present at birth or developed later on in life in close relationship with the spinal defect level [34]. In addition to the muscle imbalance and deformity the patients present with insensate feet which places a risk for skin breakdown and infections. The most common foot deformities are calcaneus, equinus, Varus, valgus, clubfeet and vertical talus and they can present as a single deformity or in combination [35]. Treatment of foot and ankle deformities is aimed at achieving a braceable plantigrade foot. In general treatment may start with casting or bracing and potentially a soft tissue surgical intervention to avoid fixed bone deformities. Once those are present osteotomies are needed to correct the foot. The patient needs to be examined regularly by a specialized pediatric orthopedist to detect tightness and incipient deformities can be early addressed (Figure 10).

Figure 10.

Patient with a bilateral cavovarus deformity being examined in clinic with the help of a podoscope.

7.1 Clubfoot

Spina bifida patients present with a rigid clubfoot deformity that is in general resistant to casting. This type of deformity can occur in up to 30–50% of the patients and the frequency increases with higher levels of the spine defect [36]. Casting with the Ponseti technique should be attempted and even though most of the patients achieve correction by this method almost 70% will relapse [37]. Additionally, if serial casting is being implement is paramount to assess skin integrity at every cast change in these patients due to their insensate feet. After correction is achieved by casting the treatment is followed by an Achilles tendon tenotomy, usually open in these patients [38]. If a wider soft tissue release is needed later on due to a recurrence a radical posteromedial release is recommended. In this procedure the subtalar, talonavicular and calcaneocuboid joints are completely released. After surgery casting followed by ankle foot orthosis (AFO) is required to maintain the correction. If a recurrence is then again noted, which may occur in 20–50% of the patients, a talectomy is indicated to achieve a plantigrade braceable foot [39].

7.2 Equinus

This deformity is also associated with higher levels of spina bifida. If the deformity is flexible an AFO may be attempted to prevent further progressing to a rigid equinus (Figure 11). With increasing severity of the deformity an Achilles tendon excision is recommended and even a radical posterior release if a plantigrade foot is not achieved after the Achilles resection [40]. Once the foot is an acceptable position a K wire is used in the talocalcaneal joint to maintain the alignment while to foot remains in a cast for at least 6 weeks (Figure 12).

Figure 11.

Four-year-old patient with bilateral equinus.

Figure 12.

K wires used after posterior release in a 12 months old patient with rigid bilateral clubfeet.

7.3 Cavovarus

Cavovarus foot deformity is more prevalent in patients with a sacral level spina bifida and it is present in up to 17% of the patients [41]. The deformity is the cause of foot muscle imbalance (Figure 13). The treatment is dependent on how flexible the hindfoot is. This must be assessed by the orthopedic surgeon with the Coleman block test. If the hindfoot is flexible, only the forefoot will need to be addressed surgically. Meanwhile on the case of a rigid hindfoot several osteotomies may be needed to achieve correction. The current recommendations with high percent of success are for a first metatarsal closing wedge, an opening plantar wedge osteotomy of the medial cuneiform, a closing wedge cuboid osteotomy and sliding calcaneus osteotomy [42, 43].

Figure 13.

Myelomeningocele patient with bilateral cavovarus feet and accompanying radiographic images depicting the high medial arch and the varus deformity.


8. Conclusions

Spina Bifida comprehends a complex subset of congenital malformation with a wide array of clinical presentation and truly diverse challenges to the patients affected by it. It is paramount that a team of multiple health care professionals from several areas of specialty work together to help improve the outcomes and life quality of these patients. The orthopedic surgeon is usually involved shortly after birth and continues to follow spina bifida patients for long terms into adulthood.


Conflict of interest

The authors state no conflict of interest related to the writing of this chapter.


  1. 1. Feeley BT, Ip TC, Otsuka NY. Skeletal maturity in myelomeningocele. Journal of Pediatric Orthopedics. 2003;23(6):718-721
  2. 2. Parker SE, Mai CT, Canfield MA, et al. Updated national birth prevalence estimates for selected birth defects in the United States, 2004-2006. Birth Defects Research Part A: Clinical and Molecular Teratology. 2010;88(12):1008-1016
  3. 3. Mitchell LE, Adzick NS, Melchionne J, Pasquariello PS, Sutton LN, Whitehead AS. Spina bifida. Lancet. 2004;364(9448):1885-1895
  4. 4. Jobe AH. Fetal surgery for myelomeningocele. The New England Journal of Medicine. 2002;347(4):230-231
  5. 5. Iskandar BJ, Fulmer BB, Hadley MN, Oakes WJ. Congenital tethered spinal cord syndrome in adults. Journal of Neurosurgery. 1998;88(6):958-961
  6. 6. Sutton LN. Fetal surgery for neural tube defects. Best Practice and Research Clinical Obstetrics and Gynaecology. 2008;22(1):175-188
  7. 7. Bowman RM, McLone DG, Grant JA, Tomita T, Ito JA. Spina bifida outcome: A 25-year prospective. Pediatric Neurosurgery. 2001;34(3):114-120
  8. 8. Fletcher JM et al. Spinal lesion level in spina bifida: A source of neural and cognitive heterogeneity. Journal of Neurosurgery. 2005;268-279(PubMed: 15881750):102
  9. 9. Verhoef M, Barf HA, Post MWM, van Asbeck FWA, Gooskens RHJM, Prevo AJH. Functional independence among young adults with spina bifida, in relation to hydrocephalus and level of lesion. Developmental Medicine and Child Neurology. 2006;48(2):114-119
  10. 10. Anschuetz RH, Freehafer AA, Shaffer JW, Dixon Jr. MS. Severe fracture complications in myelodysplasia. Journal of Pediatric Orthopedics 1984; 4(1):22-24
  11. 11. Marreiros H, Monteiro L, Loff C, Calado E. Fractures in children and adolescents with spina bifida – Experience of a portuguese tertiary care hospital. Developmental Medicine and Child Neurology. 2010;52(8):754-759
  12. 12. Okurowska-Zawada B, Konstantynowicz J, Kulak W, Kaczmarski M, Piotrowska-Jastrzebska J, Sienkiewicz D, et al. Assessment of risks factors for osteoporosis and fractures in children with meningomyelocele. Advances in Medical Sciences. 2009;54(2):247-252
  13. 13. Westcott, M. A., Dynes, M. C., Remer, E. M., Donaldson, J. S., & Dias, L. S. (1992). Congenital and acquired orthopedic abnormalities in patients with myelomeningocele. Radiographics, 12(6), 1155-1173. doi:10.1148/radiographics.12.6.1439018
  14. 14. Kumar, S. J., Cowell, H. R., & Townsend, P. (1984). Physeal, metaphyseal, and Diaphyseal injuries of the lower extremities in children with myelomeningocele. Journal of Pediatric Orthopaedics, 4(1), 25-27. doi:10.1097/01241398-198401000-00006
  15. 15. Korhonen BJ. Fractures in myelodysplasia. Clinical Orthopaedics and Related Research. 1971;79:145-155
  16. 16. Shaw NJ. Management of osteoporosis in children. European Journal of Endocrinology. 2008;159:S33-S39
  17. 17. Banta JV, Park SM. Improvement in pulmonary function in patients having combined anterior and posterior spine fusion for myelomeningocele scoliosis. Spine (Phila Pa 1976). 1983;8:765-770
  18. 18. Drennan JC. The role of muscles in the development of human lumbar kyphosis. Developmental Medicine & Child Neurology. 2008;12:33-38. DOI: 10.1111/j.1469-8749.1970.tb03000.x
  19. 19. Asher M, Olson J. Factors affecting the ambulatory status of patients with spina bifida cystica. The Journal of Bone and Joint Surgery. American Volume. 1983;65:350-356
  20. 20. Bulman, W. A., Dormans, J. P., Ecker, M. L., & Drummond, D. S. (1996). Posterior spinal fusion for scoliosis in patients with cerebral palsy: A comparison of Luque rod and unit rod instrumentation. Journal of Pediatric Orthopaedics, 314-323. doi:10.1097/00004694-199605000-00005
  21. 21. Banit DM, Iwinski HJ Jr, Talwalkar V, Johnson M. Posterior spinal fusion in paralytic scoliosis and myelomeningocele. Journal of Pediatric Orthopedics. 2001;21:117-125
  22. 22. Osebold WR, Mayfield JK, Winter RB, Moe JH. Surgical treatment of paralytic scoliosis associated with myelomeningocele. The Journal of Bone and Joint Surgery. American Volume. 1982;64:841-856
  23. 23. Sarwark JF, Weber DT, Gabrieli AP, Mclone DG, Dias L. Tethered cord syndrome in low motor level children with myelomeningocele. In:Spina Bifida. 1999. pp. 128-130. DOI: 10.1007/978-4-431-68373-5_27
  24. 24. Broughton NS, Menelaus MB, Cole WG, Shurtleff DB. The natural history of hip deformity in myelomeningocele. Journal of Bone and Joint Surgery. British Volume (London). 1993;75:760-763
  25. 25. Canale G, Scarsi M, Mastragostino S. Hip deformity and dislocation in spina bifida. Italian Journal of Orthopaedics and Traumatology. 1992;18(2):155-165
  26. 26. Sherk HH, Uppal GS, Lane G, Melchionni J. Treatment versus non-treatment of hip dislocations in ambulatory patients with myelomeningocele. Developmental Medicine and Child Neurology. 1991;33(6):491-494
  27. 27. Swaroop VT, Dias LS. Strategies of hip management in myelomeningocele: To do or not to do. Hip International. 2009;19(Suppl 6):S53-S55
  28. 28. Dias LS. Myelomeningocele and intraspinal lipoma. In: Sponseller PD, editor. Orthopaedic Knowledge Update: Pediatrics 2nd Edn. American Academy of Orthopaedic Surgeons. 2002. pp. 249-259
  29. 29. Dias LS. Surgical management of knee contractures in myelomeningocele. Journal of Pediatric Orthopedics. 1982 Jun;2(2):127-131
  30. 30. Moen T, Gryfakis N, Dias L, Lemke L. Crouched gait in myelomeningocele: A comparison between the degree of knee flexion contracture in the clinical examination and during gait. Journal of Pediatric Orthopedics. 2005;25(5):657-660. DOI: 10.1097/01.mph.0000165136.76238.23
  31. 31. Herring J. Neuromuscular disorders. In: Tachdjian’s pediatric orthopaedics. Saunders Elsevier (2008), Philadelphia, pp 1405-1453
  32. 32. Tosi LL, Buck BD, Nason SS, McKay DW. Dislocation of hip in myelomeningocele. The McKay hip stabilization. The Journal of Bone and Joint Surgery. American Volume. 1996;78(5):664-673. DOI: 10.2106/00004623-199605000-00005
  33. 33. Curtis BH, Fisher RL. Congenital hyperextension with anterior subluxation of the knee. Surgical treatment and long-term observations. The Journal of Bone and Joint Surgery. American Volume. 1969;51(2):255-269
  34. 34. Noonan KJ, Didelot WP, Lindseth RE. Care of the pediatric foot in myelodysplasia. Foot and Ankle Clinics. 2000;5(2):281-304
  35. 35. Maynard MJ, Weiner LS, Burke SW. Neuropathic foot ulceration in patients with myelodysplasia. Journal of Pediatric Orthopedics. 1992;12:786-788
  36. 36. Flynn JM, Herrera-Soto JA, Ramirez NF, Fernandez-Feliberti R, Vilella F, Guzman J. Clubfoot release in myelodysplasia. Journal of Pediatric Orthopaedics. Part B. 2004;13:259-262
  37. 37. Gerlach DJ, Gurnett CA, Limpaphayom N, Alaee F, Zhang Z, Porter K, et al. Early results of the Ponseti method for the treatment of clubfoot associated with myelomeningocele. The Journal of Bone and Joint Surgery. American Volume. 2009;91(6):1350-1359
  38. 38. Swaroop VT, Dias L. Orthopaedic management of spina bifida—Part II: Foot and ankle deformities. Journal of Children's Orthopaedics. 2011;5(6):403-414. DOI: 10.1007/s11832-011-0368-9
  39. 39. Dias LS, Stern LS. Talectomy in the treatment of resistant talipes equinovarus deformity in myelomeningocele and arthrogryposis. Journal of Pediatric Orthopedics. 1987;7:39-41
  40. 40. Sharrard WJ, Grosfield I. The management of deformity and paralysis of the foot in myelomeningocele. Journal of Bone and Joint Surgery. British Volume (London). 1968;50:456-465
  41. 41. Frawley PA, Broughton NS, Menelaus MB. Incidence and type of hindfoot deformities in patients with low-level spina bifida. Journal of Pediatric Orthopedics. 1998;18:312-313
  42. 42. Mubarak SJ, Van Valin SE. Osteotomies of the foot for cavus deformities in children. Journal of Pediatric Orthopedics. 2009;29(3):294-299
  43. 43. Schwend RM, Drennan JC. Cavus foot deformity in children. The Journal of the American Academy of Orthopaedic Surgeons. 2003;11:201-211

Written By

Roselle C. Okubo, Claudio Silveri and Ana C. Belzarena

Submitted: July 7th, 2020 Reviewed: November 4th, 2020 Published: November 28th, 2020