Orthopedics - Oleksa A.P. 2006

Congenital Spinal Deformities
Spina bifida

It is extremely rare to encounter clefts of the vertebral bodies in the sagittal plane, meaning when a vertebra is divided into two halves and its body appears as "butterfly wings" on an anteroposterior radiograph (Fig. 250). Such a cleft vertebra is typically widened and flattened (brachyplatyspondly). The intervertebral discs are also thinner.

These clefts occur in one or more thoracic or lumbar vertebral bodies, and if one part of the vertebral body is smaller than the other, it leads to The Development of dysplastic kyphoscoliosis.

Only an asymmetrical anomaly of spinal development causes spinal curvature. In other cases, a cleft in the vertebra may be clinically silent and discovered incidentally during an examination, much like spina bifida, sacralization, or lumbarization.

Congenital vertebral clefts vary in presentation, as shown in Fig. 251. Anterior clefting of the vertebral body (spina bifida anterior) is extremely rare, whereas posterior clefting (spina bifida posterior) is frequently detected on radiographs (Fig. 252).

Anterior clefts most commonly involve several cervical and upper thoracic vertebral bodies, though they can also occur in the lumbar spine. They may be accompanied by other congenital spinal malformations. While median clefts of the vertebral body are easier to identify on anteroposterior radiographs, oblique and lateral clefts are obscure and often go undetected.

Simultaneous failure of fusion of the vertebral body and arch (rachischisis) is more frequently observed in the lumbar spine.

If the vertebral cleft occurs in the sagittal plane directly through the middle of the vertebra, spinal deformity is absent or minimal. However, if the vertebra is cleft obliquely relative to the sagittal line and this is combined with another anomaly (such as a wedge vertebra or an articular process anomaly), it results in spinal curvature.

Posterior spinal clefting (spina bifida posterior) is frequently detected on radiographs during examinations of the lumbosacral spine (Fig. 252). In spina bifida lumbalis or lumbosacralis, radiographs reveal an absence of the spinous process and vertebral arch shadows, which normally project over the shadows of the vertebral bodies and intervertebral spaces. The horizontal contours of the vertebral bodies remain completely unobstructed.

A failure of fusion of the paired embryonic arches in several lower lumbar vertebrae, combined with a defect originating from the Base of the articular processes, results in a lack of bony coverage of the spinal canal. This leads to The formation of a meningeal hernia (spina bifida aperta).

If the dura mater remains intact and the incomplete cleft of the arches is occult (spina bifida occulta), the hernia may be clinically asymptomatic.

There are Various Forms of spinal hernia (rachischisis, meningocele, myelocele) which are typically accompanied by neurological disorders and complications (Fig. 253).

We observed a case where an infant at birth presented with a Skin defect in the lower lumbar and upper sacral regions covered by a translucent membrane through which elements of the Spinal Cord were visible. This membrane tends to dry out and, depending on the child's supine position, can undergo necrosis and rupture, resulting in CEREBROSPINAL FLUID leakage and ascending infection, which leads to a mortality rate of 50% among infants. Such patients should be operated on immediately or within 2-3 weeks of birth. The surgery consists of closing the skin defect solely by mobilizing adjacent skin flaps. If necessary, a secondary Procedure is performed when the child is three to five years old.

In occult forms of hernia associated with cleft vertebral arches, patients occasionally experience rapid fatigue, sacral pain, and sensory disturbances in the lower extremities. In such cases, conservative Treatment is prescribed.

In some cases, occult spina bifida is accompanied by fibrous adhesions and maldevelopment of the spinal cord elements. This leads not only to neurological disorders but also to Muscle imbalance and FOOT deformities. An abnormally high longitudinal arch (pes excavatus) and Hammer Toes develop. Patients complain of rapid fatigue, discomfort while walking, and Foot pain following excessive load. This symptom complex is referred to as myelodysplasia (Friedreich's foot).

Upon examination of the patient, alongside classic foot deformities with calf muscle atrophy and restricted ankle dorsiflexion, clinicians may find lumbosacral hypertrichosis, skin lesions, or even a dermoid cyst. If this deformity develops in early childhood, growth progression leads to muscle imbalance and static deformities: Genu Valgum, genu recurvatum, pes cavus, and hammer toes.

Class="center">

Fig. 252. Posterior cleft of the lumbosacral vertebrae.

Fig. 253. Congenital fusion of the cervical vertebrae.

Treatment for patients with foot deformities involves sequential manual redression and plaster boot immobilization to maintain the achieved correction, alongside muscle massage, physiotherapy, balneotherapy, and orthotic footwear. However, conservative treatment provides only temporary relief and is therefore used merely as a necessary phase of preoperative preparation.

Surgeries are performed under general anesthesia at 10–12 years of age. Typically, plantar aponeurotomy and tendon lengthening are performed to correct the hammer toe deformity. The foot is corrected sequentially, followed by the application of a plaster boot.

Once skeletal growth is complete, residual foot deformity is corrected via wedge or crescentic resection, or osteotomy of individual Tarsal Bones, similarly to Clubfoot surgery.