Our Summary
This paper discusses a rare birth disorder called Oculo-auriculo-vertebral spectrum (OAVS) which affects the development of certain facial structures and can lead to issues with the heart, kidneys, and spine. The paper focuses on the complications related to the spine, which have only recently been recognized as a serious part of this condition. The authors also share a case study of a patient with OAVS who had severe issues with their spine and required multiple surgeries. This patient’s case was unique because they also had a hidden condition called tethered cord syndrome, where the spinal cord is abnormally attached within the spine. This was successfully treated with a surgery to release the tethered spinal cord. The authors also discuss recent research on spinal abnormalities.
FAQs
- What is Oculo-auriculo-vertebral spectrum (OAVS)?
- How is the occult tethered cord syndrome related to OAVS treated?
- Why has the timing and strategy of spinal surgery become increasingly important for patients with OAVS?
Doctor’s Tip
A doctor may tell a patient undergoing detethering surgery for OAVS to follow post-operative care instructions carefully, including avoiding heavy lifting or strenuous activities, keeping the surgical site clean and dry, and attending follow-up appointments to monitor healing and prevent complications. It is important for the patient to communicate any new or worsening symptoms to their healthcare provider promptly.
Suitable For
Patients with Oculo-auriculo-vertebral spectrum (OAVS) who have characteristic vertebral cervical and thoracic involvement, as well as symptoms of tethered cord syndrome, may be recommended for detethering surgery. These patients may have a wide range of symptoms related to spinal abnormalities, and surgical intervention may be necessary to improve their quality of life and prevent further complications. Timing and strategy of spine surgery in patients with OAVS is becoming increasingly important, and a multidisciplinary approach involving neurosurgery, orthopedic surgery, and other specialists may be necessary to provide optimal care for these patients.
Timeline
Before detethering surgery, a patient may experience symptoms related to the tethered cord syndrome, such as back pain, leg weakness, numbness or tingling in the lower extremities, and difficulty walking. The patient may also have a history of spinal abnormalities and other congenital defects associated with OAVS.
After detethering surgery, the patient may experience relief from the symptoms of tethered cord syndrome, as well as improved mobility and function. The patient may require a period of rehabilitation and physical therapy to regain strength and coordination in the lower extremities. Long-term follow-up may be necessary to monitor for any recurrence of symptoms or complications related to the spinal abnormalities associated with OAVS.
What to Ask Your Doctor
What is detethering surgery and how does it help in treating tethered cord syndrome?
What are the risks and potential complications associated with detethering surgery?
What is the success rate of detethering surgery in patients with OAVS and tethered cord syndrome?
What is the recovery process like after detethering surgery?
Are there any long-term effects or implications of detethering surgery that I should be aware of?
Will I need any additional follow-up appointments or treatments after detethering surgery?
How will detethering surgery affect my overall quality of life and mobility?
Are there any alternative treatments or therapies that I should consider before opting for detethering surgery?
What can I expect in terms of pain management and rehabilitation post-surgery?
Are there any specific precautions or lifestyle changes I should make after detethering surgery to prevent future complications?
Reference
Authors: Hansen-Algenstaedt N, Liem M, Khalifah S, Giese A, Gutenberg A. Journal: J Am Acad Orthop Surg Glob Res Rev. 2019 Jul 30;3(7):e805. doi: 10.5435/JAAOSGlobal-D-17-00085. eCollection 2019 Jul. PMID: 31579884