**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

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**Figure 7.**

*the knee extension deformity (B).*

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

*Newborn with congenital knee dislocation in extension (A) and front and lateral radiographic image depicting* 

*Orthopedic Approach to Spina Bifida*

*DOI: http://dx.doi.org/10.5772/intechopen.94901*

**Figure 6.** *Bilateral hip dislocation and osteopenia in a 14-years-old patient with spina bifida.*

*Orthopedic Approach to Spina Bifida DOI: http://dx.doi.org/10.5772/intechopen.94901*

*Spina Bifida and Craniosynostosis - New Perspectives and Clinical Applications*

and posterior approach [22].

surgical complications [26, 27].

**6. Knee deformities**

**5. Hip**

this problem surgically by untethering the cord.

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

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

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

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

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

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**Figure 6.**

#### **Figure 7.**

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

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

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**7.1 Clubfoot**

*Orthopedic Approach to Spina Bifida*

*DOI: http://dx.doi.org/10.5772/intechopen.94901*

lengthening and anterior capsulotomy [33].

**7. Foot deformities**

**Figure 9.**

be early addressed (**Figure 10**).

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

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

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

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].

*Spina Bifida and Craniosynostosis - New Perspectives and Clinical Applications*

extension osteotomy may be indicated as well [30].

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

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

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**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.*

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].
