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Kyphectomy in Children With Severe Myelomeningocele-Related Kyphosis
1Department of Orthopaedic Surgery, University of Cape Town, Groote Schuur Hospital and Red Cross Children's Hospital, Cape Town, Western Cape, South Africa.
Insights
Kyphectomy surgery effectively corrects severe kyphosis in myelomeningocele patients, improving sitting ability and function. While generally safe, some patients with prior pressure ulcers experienced recurrence, requiring further treatment.
Area of Science:
- Spine surgery
- Pediatric orthopedics
- Neuromuscular disorders
Background:
- Myelomeningocele often leads to progressive, severe kyphosis due to posterior element abnormalities.
- This spinal deformity causes significant functional impairments, including difficulties with sitting, respiratory issues, and skin breakdown.
Purpose of the Study:
- To evaluate the safety and efficacy of kyphectomy surgery in myelomeningocele patients with severe kyphosis.
- To assess clinical and radiographic outcomes, as well as complication rates, following this surgical intervention.
Main Methods:
- Retrospective review of a prospectively maintained database of seven myelomeningocele patients who underwent kyphectomy and posterior spinal fusion.
- Patients had a minimum 24-month follow-up; surgical techniques included pedicle screw and sublaminar wire constructs.
- Three patients had surgery despite open pressure ulcers, which were closed primarily.
Main Results:
- Significant kyphosis correction from a mean of 142 degrees to 15 degrees (92% correction).
- All patients reported improved sitting ability.
- Two patients with preoperative pressure ulcers experienced recurrence of sepsis and wound breakdown at 13 months post-surgery.
Conclusions:
- Kyphectomy with posterior instrumented spinal fusion is a safe and effective procedure for severe kyphosis in myelomeningocele patients.
- The surgery provides excellent kyphotic correction, leading to improved functionality and sitting ability, even in patients with open sores.
- Primary closure of apical pressure sores is feasible due to spinal shortening and extension, avoiding the need for plastic surgery.
Study Design:
Retrospective review of prospectively maintained database.
Objectives:
To review myelomeningocele patients with severe kyphosis undergoing kyphectomy surgery in terms of complications and clinical and radiographic outcomes.
Summary Of Background Data:
Because of posterior element abnormality in myelomeningocele, the extensor muscles act as perverted flexors, driving progressive kyphosis that resulted in sitting, respiratory, and skin breakdown problems.
Methods:
Clinical case notes and x-rays of seven myelomeningocele patients undergoing kyphectomy surgery were reviewed with a minimum follow-up of 24 months. They consisted of four males and three females with an average age of 9.5 years at surgery. Surgery was performed in three despite open pressure ulcers that failed to heal. These wounds were all closed primarily at initial operation, and no flaps were required. Pedicle screw and sublaminar wire constructs were utilized with iliac screws for distal control.
Results:
The median surgical time was 245 minutes (165-285), with an estimated blood loss of 700 mL (500-2,550). The preoperative kyphosis of 142 degrees (90-180) was corrected to 15 degrees (5-45) representing a 92% correction. All experienced improved sitting. There were no early complications but 2 patients with preoperative pressure ulcers returned at 13 months with recurrent sepsis and wound breakdown. Their osteotomy had fused, and the infection settled after instrumentation removal and antibiotic administration.
Conclusion:
Although an infrequent presentation today, severe kyphosis in myelomeningocele patients causes not only a major functional impairment but threat to their life with apical pressure sores. Kyphectomy and posterior instrumented spinal fusion can be performed safely, even in the face of an open sore with excellent kyphotic correction and resultant improved functionality and ability to sit. These open sores can be closed primarily without the requirement of plastic surgery as a result of the shortening and extension of the spine.
