<!-- PERSPECTIVE --> A physician recently asked me to consult on a patient who has Ehlers-Danlos syndrome (EDS), an inherited condition that results in hypermobile joints and often leads to dislocations. The patient has high posterior neck pain, early features of brachial radiculopathy, and two midcervical fusions. His neck pain is relieved by sitting with his hands cupped under his jaw or by lying down. The patient is currently using an Aspen VistaR adjustable cervical orthosis. The physician was seeking a recommendation for a device that could translate the weight of the patient's head from the cervical spine to the orthosis without putting excessive pressure on the temporomandibular joints (TMJs), which are prone to dislocation in people with EDS. Due to the potential for upper-rib subluxation, another necessity was avoiding excessive pressure directed inferiorly on the shoulder ridge. The treatment goal is to manually ease the pain while the patient is supine, then continue the pain relief while the patient is upright and wearing an orthosis. The challenge is to anchor the orthosis in a way that reduces pressure on the shoulders and the TMJs while also providing adequate distraction of the cervical spine. I explored the options available within our department, including a noninvasive halo that would provide the distraction of the cervical spine without putting undue pressure on the TMJs. Our concern with this design is that compliance may be low due to the bulkiness of the device, and the potential for excessive pressure being applied to the shoulders. For additional input, I sought assistance on the OANDP-L listserv. I received many useful recommendations to help me advise the physician about the treatment options. One recommendation was the Trulife Pneu-Trac Traction Collar, which uses pneumatic control to distract the occiput; the patient adjusts the air pressure to his or her preferred level. Another practitioner recommended using a custom Trulife Minerva cervical thoracic orthosis (CTO), which has a large surface area over the chest, shoulder, and back to prevent pressure on the shoulders, or a cervical-thoracic-lumbar-sacral orthosis that anchors at the hips. Yet another suggestion was to use a backward-donned Performance Health Headmaster Collar. The Headmaster orthosis is a soft cervical collar, and donning it backward puts the taller section at the anterior of the neck. The patient's current cervical orthosis, the Aspen Vista, was also recommended. The results of the discussions I have had suggest that a less rigid device is more likely to have a higher success rate because patients with EDS do not respond well to excessive pressure. However, they do seem to have a positive response to compression. After I sent my findings to the physician, he presented the options to the patient. I will continue to follow up to see which option will suffice for the patient's long-term use. I am encouraged by the abundance of support from O&P professionals, and it is reassuring to know that there are practitioners willing to provide their successes and failures when faced with a difficult patient presentation. If there are other options I haven't considered, I welcome further input. <em>Jason Pantages, MS, is an orthotic resident at Atlantic Prosthetics & Orthotics, Chapel Hill, North Carolina. </em>
<!-- PERSPECTIVE --> A physician recently asked me to consult on a patient who has Ehlers-Danlos syndrome (EDS), an inherited condition that results in hypermobile joints and often leads to dislocations. The patient has high posterior neck pain, early features of brachial radiculopathy, and two midcervical fusions. His neck pain is relieved by sitting with his hands cupped under his jaw or by lying down. The patient is currently using an Aspen VistaR adjustable cervical orthosis. The physician was seeking a recommendation for a device that could translate the weight of the patient's head from the cervical spine to the orthosis without putting excessive pressure on the temporomandibular joints (TMJs), which are prone to dislocation in people with EDS. Due to the potential for upper-rib subluxation, another necessity was avoiding excessive pressure directed inferiorly on the shoulder ridge. The treatment goal is to manually ease the pain while the patient is supine, then continue the pain relief while the patient is upright and wearing an orthosis. The challenge is to anchor the orthosis in a way that reduces pressure on the shoulders and the TMJs while also providing adequate distraction of the cervical spine. I explored the options available within our department, including a noninvasive halo that would provide the distraction of the cervical spine without putting undue pressure on the TMJs. Our concern with this design is that compliance may be low due to the bulkiness of the device, and the potential for excessive pressure being applied to the shoulders. For additional input, I sought assistance on the OANDP-L listserv. I received many useful recommendations to help me advise the physician about the treatment options. One recommendation was the Trulife Pneu-Trac Traction Collar, which uses pneumatic control to distract the occiput; the patient adjusts the air pressure to his or her preferred level. Another practitioner recommended using a custom Trulife Minerva cervical thoracic orthosis (CTO), which has a large surface area over the chest, shoulder, and back to prevent pressure on the shoulders, or a cervical-thoracic-lumbar-sacral orthosis that anchors at the hips. Yet another suggestion was to use a backward-donned Performance Health Headmaster Collar. The Headmaster orthosis is a soft cervical collar, and donning it backward puts the taller section at the anterior of the neck. The patient's current cervical orthosis, the Aspen Vista, was also recommended. The results of the discussions I have had suggest that a less rigid device is more likely to have a higher success rate because patients with EDS do not respond well to excessive pressure. However, they do seem to have a positive response to compression. After I sent my findings to the physician, he presented the options to the patient. I will continue to follow up to see which option will suffice for the patient's long-term use. I am encouraged by the abundance of support from O&P professionals, and it is reassuring to know that there are practitioners willing to provide their successes and failures when faced with a difficult patient presentation. If there are other options I haven't considered, I welcome further input. <em>Jason Pantages, MS, is an orthotic resident at Atlantic Prosthetics & Orthotics, Chapel Hill, North Carolina. </em>