Congenital Scoliosis

Congenital scoliosis is an anomaly due to defects in the development of the spine in the uterus. Generally, the formation and segmentation stages of spine development are completed in the first 6 weeks of pregnancy. It is seen as a result of defects in these stages. Congenital vertebral deformities cause an imbalance in the development of the spine. The shape and progressive potential of the deformity determine the amount of this imbalance. With current imaging technologies, these deformities can be detected in the second trimester of pregnancy (3-6 months). Early acquisition of knowledge provides preparation for the search of other comorbid anomalies and for the treatment that will be performed.

These pathologies present during childbirth may appear in childhood. It usually shows symptoms with a cosmetic defect, functional limitation, and neck-back-low back pain. Although it is not certain, it is thought to be seen in one per thousand people. As they can be seen as isolated, congenital vertebral deformities can also be seen with spinal, cardiac, kidney malformations or chromosomal aberration and syndrome.

Congenital scoliosis is a difficult deformity with a malignant prognosis in which early diagnosis is much more important than idiopathic scoliosis. The important point in the treatment is to provide the most appropriate treatment at the most appropriate time in order to maximize the child’s whole body functions and growth potential. In summary, the general principle of treatment is early diagnosis and surgical correction.

Diagnosis

Vertebral anomalies are present at birth but usually may not become apparent until the end of childhood. For complete and detailed preoperative evaluation, spinal cord and soft tissues are examined with Magnetic Resonance Imaging (MRI) after direct scoliosis radiographs taken at various positions. In this way, especially the spinal cord and its canal are investigated whether there is an anomaly. Computed tomography (CT) is required in almost every patient. In this way, a detailed and three-dimensional examination of anomalous bones is performed. Heart, digestive system, urinary tract, lung, and respiratory functions should also be evaluated in these patients.

Congenital scoliosis should not be confused with Infantile Idiopathic Scoliosis (IIS). Although IIS becomes evident at an early age, there is no vertebral anomaly in radiological evaluation and corset may be considered in its treatment. Because the primary deformity in IIS is not in the vertebra, but in soft tissues.

Natural course

The type and shape of the deformity determine the rate of progression of the disease. The age of the patient at the time of consultation may also indicate the status of progress. Statistically, 25% never progresses, 25% progresses slightly, while 50% progresses and its degree increases rapidly.

As a general rule, the fastest progress occurs in two periods of life. The first is the first 3 years of life and adolescence period. One of the most important criteria showing the progression of the deformity in congenital scoliosis is the form of deformity. While some types are expected to progress 1 degree per year, some types can progress very fast up to 14 degrees per year. In addition, the region of the deformity, the age of consultation of the patient and the balance of the spine are also among the criteria showing the progression of the deformity.

In general, the more the anomaly causes deterioration of the spine balance, the faster the progression, the worse the cosmetic appearance. Even if the curvatures of the spine are at multiple levels, if they balance each other, the progression is either not seen or slow and the cosmetic appearance is satisfactory.

Treatment

When deciding the treatment, examination and detailed radiological examinations should reveal the anomaly and accompanying problems in detail. Surgery should not be delayed if there is a curvature that is thought to progress rapidly. Curvatures that can progress slowly should be followed at appropriate time intervals.

Conservative treatment: Only the curvature progression of the patient is followed-up. In non-segmented bar deformity, there is no need for follow-up, but multiple-balanced anomalies may be monitored. Monitoring may be performed from four to six months intervals, even during periods of growth can be monitored more frequently if necessary. Corset treatment is almost never used. Sometimes it can be used for long, flexible curvatures and compensatory curvatures. It should not be used especially for short and hard curvatures. The use of corsets can also adversely affect the development of the rib cage in young children. No response was reported from exercise, manipulation, or electrical stimulators.

Surgical treatment: Since progressive congenital scoliosis often causes very severe deformities, it is difficult to correct them surgically. Therefore, the best way to achieve the best results is the early recognition of curvature progression and early surgical treatment. If the deformity is not located and the growth is not completed, the growth plaques of the vertebrae can be removed in surgical treatment, and the spine can be continued to grow with extensible-instrumentation. After deformity settles, correction operations are performed. The fused vertebrae are opened, the sharp-angled vertebrae are removed and the fusion is performed to the patients by aligning the spine.