Congenital scoliosis is an anomaly due to defects in the development of the spine in the mother’s womb. 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 that occur 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 extent of this imbalance. With current imaging technologies, these deformities can be detected in the second trimester of pregnancy (3-6 months). Gaining early knowledge will enable the investigation of comorbid anomalies and allow for preparation prior to treatment.
These pathologies that are present during childbirth may emerge in childhood. It usually shows symptoms with cosmetic defects, functional limitations, and neck-back-lower back pains. Although this is not certain, it is believed to occur in one of every thousand people. They may occur as isolated, congenital vertebral deformities and also in conjunction with spinal, cardiac, kidney malformations or chromosomal anomalies and syndromes.
Congenital scoliosis is a deformity that is difficult to treat, with a malignant prognosis in which early diagnosis is much more important than in idiopathic scoliosis. The important point in the treatment is to provide the most appropriate treatment at the most appropriate time in order to maximize and maintain the child’s bodily functions and growth potential. In short, the general principle of treatment is early diagnosis and surgical correction.
Vertebral anomalies are present at birth but usually may not become apparent until the end of childhood. For a complete and detailed preoperative evaluation, spinal cord and soft tissues are examined with Magnetic Resonance Imaging (MRI) after direct scoliosis radiographs are taken at various positions. In this way, especially the spinal cord and its canal are investigated for any anomalies. Computed Tomography (CT) is required in almost all patients, thus a detailed and three-dimensional examination of anomalous bones is performed. The heart, digestive system, urinary tract, lungs, and respiratory functions should also be evaluated in these patients.
Congenital scoliosis should not be confused with Infantile Idiopathic Scoliosis (IIS). Although IIS becomes prominent at an early age, there is no vertebral anomaly in radiological evaluation and the use of corsets may be considered in its treatment. Because the primary deformity in IIS is not in the vertebrae, but in soft tissues.
The type and shape of the deformity determine the progression rate of the disease. The age of the patient at the time of consultation may also provide a prediction as to its progress. Statistically, 25% of cases do not progress at all, whereas 25% progresses slightly, and 50% progresses with a rapidly increasing degree.
As a general rule, the fastest progress occurs in two periods of life. The first period is the first 3 years of life and the 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 quickly up to 14 degrees per year. In addition, the region of the deformity, the age of the patient at the time of consultation and the balance of the spine are also among the criteria that indicate the progression of the deformity.
Typically, the more the anomaly causes deterioration of the spine balance, the faster the progression, and the worse the cosmetic appearance will be. Even in case the curvatures of the spine are at multiple levels, if they are balancing each other out, the progression is either not seen at all or seen as slow and the cosmetic appearance is still satisfactory.
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 considered as progressing rapidly. Curvatures that can progress slowly should be monitored in appropriate time intervals.
Only the curvature progression of the patient is monitored. In non-segmented bar deformity, there is no need for follow-up, but multiple-balanced anomalies may be monitored. Monitoring may be performed in four to six months intervals, and growth spurt periods can be monitored more frequently where necessary. Corset treatment is almost never preferred. Sometimes it can be used for long, flexible curvatures and compensatory curvatures. It should not be used especially for short and severe curvatures. The use of corsets can also adversely affect the development of the rib cage in young children. It was reported that exercise, manipulation or electrical stimulators yielded no response.
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 in may be ensured that the spine continues to grow with extensible instruments. After the deformity settles, corrective operations are performed. The fused vertebrae are opened, the sharp-angled vertebrae are removed and the fusion is performed on the patients by aligning the spine.
