Study Design: Retrospective study with clinical and radiological evaluation of 15 patients with congenital kyphosis or kyphoscoliosis who underwent anterior instrumented spinal fusion for posterolateral or posterior hemivertebae (HV).

Objective: To evaluate the safety and efficacy of early surgical anterior instrumented fusion with partial preservation of the HV in the treatment of progressive congenital kyphosis in children below the age of 3. We discuss the management of patients presenting with neurological compromise.

Summary of background data: A variety of treatments have been described in the literature for the treatment of congenital kyphosis due to HV. We report the results of our technique.

Materials and Methods: Between 1997 and 2005 we have treated 15 consecutive patients with progressive congenital kyphosis with anterior instrumented fusion and strut grafting. 13 patients had a single posterolateral HV and 2 patients a single posterior HV. Of the 15 patients in the study, 5 were girls and 10 boys. Mean age at surgery was 22 months (range 8–33). Mean follow-up period was 6.8 years. 13 HV were located in the thoracolumbar junction (T10-L2) and 2 in the thoracic spine.

Results: The average operating time was procedure was 150 minutes (range, 130 to 210 minutes). The average blood loss 180 mL (range, 100 to 330 mL), equivalent to a mean external blood volume loss of 15% (range, 11 to 24%).

Preoperative segmental Cobb angle averaging 34 º at last follow up. Compensatory coronal cranial and caudal curves corrected by 50%. The angle of segmental kyphosis averaged 39º (range, 20º to 80º) before surgery and 21 º (range, 11º to 40º) at last follow up. This represents a 43% of improvement of the segmental kyphosis, and a 64% of improvement of the segmental scoliosis at last followup.

One case with initial kyphosis of 80 º continued to progress and required revision anterior and posterior surgery. There were no neurologic complications.

Key points:

In progressive congenital kyphoisis, early diagnosis and aggressive surgical treatment are mandatory for a successful result.

Study Design: Retrospective study with clinical and radiological evaluation of 29 patients with congenital scoliosis who underwent 31 short segment anterior instrumented fusions of lateral hemivertebrae.

Objective: To evaluate the safety and efficacy of early surgical anterior instrumented fusion with partial preservation of the HV in the treatment of progressive congenital scoliosis in children below the age of 6.

Summary of background data: A variety of treatments have been described in the literature for the treatment of HV. We report the results of a novel technique.

Materials and Methods: Between 1996 and 2005, 29 consecutive patients with 31 lateral HV and progressive scoliosis underwent short segment anterior instrumentation and fusion with preservation of the HV. Mean age at surgery was 2.9 years. Mean follow-up period was 6.3 years.

Results: Preoperative segmental Cobb angle averaging 39°, was corrected to 150 after surgery, being 15º at the last follow up (60% of improvement). Compensatory cranial and caudal curves corrected by approximately 50% and did not change significantly on follow up. The angle of segmental kyphosis averaged 13º before surgery, 12º after surgery, and 12° at follow up. There was 2 wound infection requiring surgical debridment, 1 intraoperative fracture of the vertebral body and 1 case lost correction due to implant failure. All went on to stable bony union. There were no neurologic complications.

Conclusions: Early diagnosis and early and aggressive surgical treatment are mandatory for a successful treatment of congenital scoliosis and to prevent the development of secondary compensatory deformities. Anterior instrumentation is a safe and effective technique capable of transmitting a high amount of convex compression allowing short segment fusion which is of great importance in the growing spine

Background: Congenital anomalies of the chest wall and Sprengel’s shoulder are often associated with congenital deformities of the spine. It has been suggested that extensive rib fusions on the concavity of a congenital scoliosis may adversely affect progression of the spine deformity, thoracic function and growth of the lungs, which can lead to a thoracic insufficiency syndrome. Methods: This is a retrospective study of the medical records and spine radiographs of 620 consecutive patients with congenital spine deformities. The rib anomalies were classified into simple and complex and the presence of a Sprengel’s shoulder was recorded. Subjects: There were 497 patients (80%) with scoliosis, 88 patients (14%) with kyphoscoliosis, and 35 patients with kyphosis (6%). The mean rate of scoliosis deterioration without treatment, age and curve size at surgery was compared for the different types of vertebral abnormalities in patients with and without rib anomalies. Results: Rib anomalies occurred in 124 patients. The rib anomalies were simple in 97 patients (78%) and complex in 27 (22%). The most common simple rib anomaly (70 patients; 72%) was a fusion of two or three ribs. The most common complex rib anomaly (20 patients; 74%) was a fusion of multiple ribs associated with a large chest wall defect. Rib anomalies were most commonly associated with a congenital scoliosis (116 patients; 93.6%), and much less frequently with a congenital kyphoscoliosis or kyphosis (8 patients). In those patients with a scoliosis, the rib anomalies were simple in 91 patients (78.5%) and complex in 25 patients (21.5%). Eighty-eight of the 124 patients (76%) with rib abnormalities had a scoliosis due to a unilateral unsegmented bar with or without contra-lateral hemivertebrae at the same level, and 8 patients had mixed unclassifiable vertebral anomalies. In contrast, only 17 patients with a scoliosis and rib anomalies had hemivertebrae alone. The rib anomalies were most frequently associated with a thoracic or thoracolumbar scoliosis (107 patients; 92.2%) and occurred on the concavity in 81 patients (70%), convexity in 27 patients (23%), and were bilateral in 8 patients. Sprengel’s deformity occurred in 45 patients; 43 patients with congenital scoliosis (8.6%), and 2 with kyphoscoliosis or kyphosis. A Sprengel’s shoulder occurred most frequently in association with a thoracic scoliosis due to a unilateral failure of vertebral segmentation (28 patients). The elevated shoulder was on the concavity of scoliosis in 26 patients and on the convexity in 16 patients. We compared the mean rate of curve progression without treatment, the age and curve size at spine surgery for the different types of spine deformities in patients with and without rib anomalies either simple or complex and found no significant difference (p> 0.05); the only statistical difference was that the mean age at surgery was higher for patients with a unilateral unsegmented bar without rib anomalies (p=0.005). Conclusion: Congenital rib anomalies and Sprengel’s shoulder occur most commonly on the concavity of a thoracic or thoracolumbar congenital scoliosis due to a unilateral failure of vertebral segmentation. However, these anomalies do not have an adverse effect on curve size or rate of progression

A combined anterior and posterior surgical approach is generally recommended in the treatment of severe congenital kyphosis, despite the fact that the anterior vascular supply of the spine and viscera are at risk during exposure. The aim of this study was to determine whether the surgical treatment of severe congenital thoracolumbar kyphosis through a single posterior approach is feasible, safe and effective.

We reviewed the records of ten patients with a mean age of 11.1 years (5.4 to 14.1) who underwent surgery either by pedicle subtraction osteotomy or by vertebral column resection with instrumented fusion through a single posterior approach.

The mean kyphotic deformity improved from 59.9° (45° to 110°) pre-operatively to 17.5° (3° to 40°) at a mean follow-up of 47.0 months (29 to 85). Spinal cord monitoring was used in all patients and there were no complications during surgery. These promising results indicate the possible advantages of the described technique over the established procedures. We believe that surgery should be performed in case of documented progression and before structural secondary curves develop. Our current strategy after documented progression is to recommend surgery at the age of five years and when 90% of the diameter of the spinal canal has already developed.

Cite this article: Bone Joint J 2013;95-B:1527–32.