The mean operation time was 220.2109.0 minutes for untethering surgery and 399.59.6 minutes for SSO; as these numbers clearly indicate, the time was significantly longer for the SSO group (p=0.01). Bulbocavernosus reflex (BCR) monitoring is used to assess the integrity of urinary and bowel function. Adult tethered cord syndrome. WebMedian time to symptomatic improvement was least for pain (1 month), then motor (2.3 months), and then urinary symptoms (4.3 months; p = 0.04). WebHave you lie flat on your back for up to 72 hours to prevent cerebrospinal fluid from leaking around the spinal cord During your stay, the hospital staff will take steps to lower your In children, the surgery to de-tether the spinal cord is more common and can be done at many institutions; the procedure is also lower risk in children than in adults. In addition, in terms of cost-effectiveness, SSO is substantially more costly than untethering, which means that SSO can be a financially viable option mainly just in very challenging cases of adult TCS. Tethered cord syndrome is a stretch-induced functional disorder associated with the fixation (tethering) effect of inelastic tissue on the caudal spinal cord, limiting its movement. FOIA We talked about bracing but it had to be delayed because a follow up MRI showed a tethered cord that needed surgery. Changes of symptoms were associated with the course of disease; patients with relatively shorter disease course were shown to have a mild Hoffman grading, whereas patients with relatively longer disease course were indicated to have a severe Hoffman grading. Surgical treatment on adult patients with TCS can improve the neurologic deficits which are associated with the course of disease, early treatment has much better curative effect. A total of 32 consecutive adult patients underwent untethering surgery for secondary tethered cord syndrome in our department from January 2008 until December 2018. Altered sensation (numbness or paresthesia) and bladder and/or fecal dysfunction were the most common complaints among 11 patients (79%). Call Today. 10 Your child will also need a COVID-19 PCR test 48 hours (2 days) before surgery. Epub 2018 Mar 8. [16] On the whole, patients with filar TCS had the lightest symptoms, corresponding surgery was relatively easy, and prognosis in the follow-up period was relatively better after removing filum terminale. 13 general health condition is usually good, so SSO could be an appropriate procedure for adults with TCS. doi: 10.1097/BRS.0b013e3181fc2edd. Get the latest news on COVID-19, the vaccine and care at Mass General. After surgery, the lipoma was removed almost completely (B). . Congenital tethered spinal cord syndrome in adults. This abnormal fixation limits or prohibits movement of the cord within the spinal column. Although surgery in adults involves greater risk of neurological injury than in children, it is a low-risk procedure with encouraging results. Laurent D, Bardhi O, Gregory J, Yachnis A, Governale LS. Muscle weakness was present in 10 patients (71%), 8 (57%) had leg pain and sciatica, and 6 (43%) had back pain. Patients who underwent surgery for TCS secondary to posttraumatic or postinflammatory conditions were not included in this study. . Recovery National Library of Medicine In some people, these symptoms may not be noticeable until adulthood. Surgical treatment was indicated for patients with radiologically proven tethering of the spinal cord who consistently showed progressive neurologic deficits, back/lower limb pain, or sphincter dysfunction. Tethered Cord Syndrome (TCS) is a complex of neurologic symptoms that include pain, incontinence, musculoskeletal deformities, motor weakness, and sensory abnormalities resulting from abnormal stretch placed on the distal spinal cord by congenital or acquired factors. The duration of symptoms was significantly longer in the SSO group (2512.4 years) than in the untethering surgery group (8.26.3 years; p=0.01). J Neurosurg. The mean age of the patients was 46 13 years (range 23-74 years) and the mean follow-up duration was 61 62 months. [2] As for normal embryo under 20 weeks, the termination of spinal cord was located at the level of L4 to L5, and at the level of L3 under 40 weeks, when the baby was born, it was located at the level of L1 to L2. Values of p<0.05 were considered to indicate statistical significance. Tubbs RS, Bui CJ, Loukas M, Shoja MM, Oakes WJ. This is called tethered cord. Primary is typically a form of OSD while secondary usually occurs following a myelomeningocele repair or other type of spinal cord surgery History and Exam Tethered spinal cord is most commonly diagnosed in infancy by the discovery of a abnormality noticed on the skin of the back. In children surgery prevents further neurological deterioration. The treatment of tethered cord syndromes in adults is discussed regarding the natural history and surgical indications. Intraoperative feasibility of bulbocavernosus reflex monitoring during untethering surgery in infants and children. 3 If no imaging has been done, your child may need a magnetic resonance imaging (MRI) test of their spine before the appointment. Neurosurg Focus. However, untethering carries risks of spinal cord injury and re-tethering. Hoffman HJ, Hendrick EB, Humphreys RP. A tethered spinal cord occurs when the inelastic tissue on the caudal spinal cord is abnormally attached to a structure instead of free floating. 1. Highlight selected keywords in the article text. The care team will discuss the type of tethered cord your child has when they review the imaging of their spine with you. Federal government websites often end in .gov or .mil. A tethered cord does not move. As an alternative to untethering surgery, Kokubun et al have performed SSO since 1995 in patients with TCS caused by a lipomyelomeningocele because osteotomy is believed to reduce the tension in the spinal cord.10 Fourteen patients (37.712.5 years) with TCS were enrolled at 6 hospitals. Tethered cord syndromea study of the short-term effects of surgical detethering on markers of neuronal injury and electrophysiologic parameters. The tethering effect was caused either by a split cord malformation, a thick filum terminale, a conus medullaris lipoma with extradural extension, or various combinations of these mechanisms. As for the postoperative complications, there were 4 cases (5%) of spinal fluid leakage, and the 2 patients were cured following vacuum aspiration and pressurized dressing; there were 6 cases (7%) showing delayed wound healing, mainly caused by spinal fluid leakage or fat liquefaction. An official website of the United States government. Independent sample t tests and Fisher exact tests were performed to compare the results between the untethering and SSO groups. Tethered cord syndrome (also called fastened cord syndrome) is a condition in which the spinal cord is not able to float freely within the spinal column because of an abnormal (unusual) attachment to tissue surrounding it. 8 Surgery for a Tethered Spinal Cord. Reduction of caudal traction force using dural sac opening rather than spinal cord detethering for tethered cord syndrome caused by lipomyelomeningocele: a case report. Asian J Neurosurg. Revision surgery in patients with complex dysraphic lesions should be performed in exceptional cases only. We would like to thank our colleagues from the Department of Neurosurgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, and the Department of Anesthesia, Critical Care and Pain Medicine, Massachusetts General Hospital, Harvard Medical School, Harvard University. 15. stretching. Tremors or spasms in the leg muscles. Medicine. The preoperative pathology was lipomeningocele in all SSO group and lipoma or tight terminal filum in the untethering group. The severity of the condition and the associated signs and symptoms vary from person to person. World J Clin Cases. During the follow-up period, 2 patients in the untethering surgery group complained of new back pain, and 2 other patients (neither of whom was the previously discussed revision-surgery patient) experienced new leg numbness. 9 1. Careers, Unable to load your collection due to an error. All 6 patients had tethered spinal cords, and 1 patient in each group had diastematomyelia. Tokumi Kanemura, none 5 The mean age at onset of symptoms and diagnosis was 30 years and 37 years, respectively. After identification of the terminal filum, we confirmed electromyography activity on bipolar stimulation before clip ligation and definitive sectioning. [3,4] Adult onset cases are rare compared to that in children. In syringomyelia, the watery liquid known as . Epub 2015 Nov 26. Throughout her time in high school, she had frequent . The photograph shows thick filum terminale isolated at the time of surgery before sectioning. Rev. The tethered spinal cord is developed by the following ways: A . A lumbar laminectomy for release of a tethered cord. During the first 24 hours, your child will remain flat on their back to prevent fluid leak from the incision. Keyword Highlighting Search for condition information or for a specific treatment program. 7 11 In children, the surgery to de-tether the spinal cord is more common and can be done at many institutions; the procedure is also lower risk in children than in adults. A retrospective analysis of 82 adult patients (17 male cases, 82% and 24 female cases, 59%) with TCS treated by surgery was conducted between March, 2005 and December, 2015, with an average age of 31.6 years and average disease course of 6.7 years. Each time she had a surgery to scrape away the scar tissue, there was more of it, and her doctors had to make larger incisions on her back. 11 Miyakoshi et al reported complete clinical recovery without complications in 2009, which led to the hope that SSO would be the way to reduce perioperative complications and provide better neurologic outcomes.10 Although Kokubun et al also reported good clinical results after SSO in 2011,11 there have been no reports until now of a comparative study or review of these two procedures. The surgical scheduler will work with you and family to coordinate a surgical date that fits best into your and your childs schedules. 8. The term occult spinal dysraphism (OSD) encompasses a group of abnormalities that occur during the development of a human embryo, beginning in the third week of gestation. Liu JJ, Guan Z, Gao Z, et al. A hairy patch overlying the spine in any area is almost always associated with an underlying splitting of the spinal cord by a band of fibrous tissue or bone (a diastematomyelia). Thoracolumbar surgery for degenerative spine diseases complicated with tethered cord syndrome: A case report. As an alternative to untethering, Kokubun et al introduced spine-shortening osteotomy (SSO) for patients with TCS caused by a lipomyelomeningocele.11 SSO reduces the tension in the spinal cord and minimizes the perioperative complications.10 A total of 72 cases applied positive straight incision, 10 cases of lumbosacral lipoma with longitudinal incision. Improvement in back pain and leg pain or numbness usually happens first, and bladder and bowel improvement happen last. Tethered cord syndrome treatment. Definition. Through the long-term follow-up, patients with a shorter duration, lighter TCS degree, generally the prognosis would be good, and symptoms improved significantly; on the other hand, for patients with longer course of disease, serious TCS, and higher frequency that tumor wrapped around the cauda equina, corresponding surgery effect was not so obvious; some patients even showed no improvement of symptoms, and the risk of postoperative TCS was relatively high. However, his condition subsequently deteriorated, and he could not walk by himself 1year after untethering surgery. Because the incision is lower on the back around a part of the spine that does 1Department of Orthopedic Surgery, Nagoya University Graduate School of Medicine, Nagoya, Japan, 2Department of Orthopedic Surgery, Nagoya Daini Red Cross Hospital, Nagoya, Japan, 3Department of Orthopedic Surgery, Chubu Rosai Hospital, Nagoya, Japan, 4Department of Orthopedic Surgery, Konan Kosei Hospital, Aichi, Japan, 5Department of Orthopedic Surgery, Aichi Medical University, Aichi, Japan, 6Department of Orthopedic Surgery, Hamamatsu Medical University, Shizuoka, Japan. Milano JB, Barcelos ACES, Onishi FJ, Daniel JW, Botelho RV, Dantas FR, Neto ER, de Freitas Bertolini E, Mudo ML, Brock RS, de Oliveira RS, Joaquim AF. In this study, we evaluated the feasibility of BCR monitoring during untethering surgery in infants and children to predict postoperative urinary and bowel dysfunction. Up to 6% of the normal population will be found to have fat within their filum terminale, 14 and many of these individuals will have symptoms of a tethered spinal cord ().The term filum terminale syndrome was coined in 1953 by Garceau, 15 who reported three patients with progressive spinal deformity and neurological dysfunction. Twenty-eight patients remained in stable clinical condition. In contrast, sensory deficits were less likely to improve; numbness and paresthesias remained unchanged in 55% of the patients. Conclusions: I am just your average. The population consisted of 12 men and 22 women, ranging in age from 18 to 70 years (mean 34 years). The authors prefer to limit bathing to a sponge bath until the patient is seen in clinic 1 week later. http://creativecommons.org/licenses/by/4.0, Received in revised form October 19, 2016. After surgery, the lipoma was removed almost completely (Fig. In addition, telephone interviews were obtained after a period of 8.6 years. 4. Meanwhile, patients with shorter disease courses were suggested to accompany with obvious improvement of symptoms postoperatively when compared to those patients preoperatively; besides, the course of disease was within 1 year regarding those patients showing a completely recovery of the abovementioned symptoms. 2007;23(2):E11. Chern JJ, Dauser RC, Whitehead WE, Curry DJ, Luerssen TG, Jea A. Spine (Phila Pa 1976). UNDERSTANDING TETHERED SPINAL CORD SURGERY AFTER THE SURGERY THE FIRST 24-48 HOURS CONTROLLING YOUR CHILD'S PAIN THE SURGICAL WOUND GOING HOME For the first 12-48 hours after surgery, your child must remain flat in bed. Typically, there is also a short filum and, as a result of both anomalies, a tethered cord. 10 [] This entity was first described by Garceau (1953) and For cyst wall with many serious adhesions of cauda equina nerve, partial resection of the cyst wall can be performed under electrophysiological monitoring, which will also have a good operation effect. Symptoms of Tethered Spinal Cord Syndrome in Teens and Adults. flag football tournaments 2022 tethered spinal cord surgery recovery time. Pathway Background and Objectives. In conclusion, SSO appears to provide clinical improvement at least comparable to that achievable with the untethering procedure, especially in more challenging cases where successful untethering is quite difficult to achieve, such as cases of patients with complex malformations, arachnoid adhesions, and revision surgery. Suite F4300. The site is secure. Spinal column shortening is an indirect detethering surgery which shortens and fuses the spine to relieve longitudinal pressure on the spinal cord. tethered spinal cord constipation . 2018 Apr-Jun;13(2):264-270. doi: 10.4103/1793-5482.228566. 20. Activity modification. 2001 Jan 15;10(1):e7. According to Hoffman grading system, the neurologic symptoms were improved in 22 patients (27%) and stabilized in 60 patients (73%). J Surg Case Rep. 2020 Mar 24;2020(3):rjaa041. The authors reviewed their institutional experience with the surgical management of adult TCS to assess the time course of symptomatic improvement, and to . The next day, your child sit up and the care team will check whether your child has a headache. By the time of birth the spinal cord is located between L1 and L2. This can cause the spinal cord to stretch out as the spine grows, leading to possible nerve damage, pain and other symptoms. This is common problem for people after any surgery, takes time. It may not be possible for the syrinx to be fully removed, so the goal of surgery may be . 11 Miyakoshi et al reported that all of the neurologic symptoms in the subjects of one of their studies were relieved without complications after SSO,10 and it was hoped on the basis of that study that SSO would become a preferable approach for TCS in adults, but the number of patients in their study was small (n=3). Your child may need an operation to help the spinal cord move freely. 2 The tethering effect was caused either by a split cord malformation, a thick filum terminale, a conus medullaris lipoma with extradural extension, or various combinations of these mechanisms. Epub 2019 Oct 9. FOIA Two groups were distinguished based on the absence (Group A, 43 patients) or presence (Group B, 42 patients) of an associated lipoma or dysraphic cyst (that is, dermoid, epidermoid, or neurenteric cyst). 11 Web Spinal cord tethering may be either primary or secondary. Bookshelf The mean blood loss was 575.51316.5 mL in untethering surgery cases, but significantly greater in the SSO group: 1,971.81,739.2 mL (p<0.001). The operation curative effects with curative rates for TCS with different symptoms, and signs are shown in Table 2 in detail. Because neurological deficits are generally irreversible, early surgery is recommended. As first-line treatment, patients presenting with TCS typically undergo direct spinal cord untethering, often as young children or infants. This means a shorter, Walk on their own (if appropriate for age), Diazepam (Valium) to prevent muscle spasms. Among individuals who did not undergo surgery, 17 patients refused surgery and 25 patients underwent recommended conservative treatment. Surgical management of tethered spinal cord in adults: report of 54 cases. Tethered cord results when the spinal cord cannot normally ascend with growth, which . The PubMed wordmark and PubMed logo are registered trademarks of the U.S. Department of Health and Human Services (HHS). 2. If the nerves are stretched, they may not work properly, and this can cause problems for your child. Note: Results are the number of patients with improvement/the number of patients with each symptom preoperatively. Fatty Filum Terminale. Others could end up re-tethered within months of the first surgery. In the early stage of embryo, spinal cord and vertebral canal were roughly equal, but in the later development process, bony spinal growth were indicated to be faster, which was out of synchronization with the growth of spinal cord. Yukihiro Matsuyama, none 2014; 192:221-7. . The .gov means its official. SSO is a highly invasive type of surgery, but as the average age of adults with TCS ranges from 35 to 46 years,1 The combined complication rate of this surgery is usually 1-2%. Activity modification. Management of adult tethered cord syndrome: our experience and review of literature. 714-509-7070. The variations of tethering lesions were tight terminal filum (present in 1 patient), lipoma (5 patients), and lipomyelomeningocele (8 patients). What is Adult Tethered Cord? Lumbosacral laminectomies were performed to obtain adequate exposure of the conus medullaris and cauda equina. Before your childs first visit, be sure to bring or send any imaging tests of your childs spine. Although untethering surgery has been a standard treatment in patients with adult tethered cord syndrome (TCS), spine-shortening osteotomy (SSO) has recently been performed as an alternative technique. Pediatric pathology all grown up - An interesting case of adult tethered spinal cord. 5 [11] In this paper, we suggest that it is possible to cut off the filum terminale if there will have no injury to the nerve under electrophysiological monitoring, corresponding prognostic outcome will be better than that that without disconnection of filum terminale. In adults, dethetering of the spinal cord . Klekamp J. Tethered cord syndrome in adults. The low growth ability of lipoma also leads to the problem that whether the tumor should be removed completely or not. Pathophysiology of tethered cord syndrome and similar complex disorders. A retrospective analysis of 82 adult patients (17 male cases, 82% and 24 female cases, 59%) with TCS treated by surgery was conducted between March, 2005 and December, 2015, with an average age of 31.6 years and average disease course of 6.7 years. Garceau GJ. Neurosurg Focus. 3 If they do experience a headache, your child will lay back down flat. Thus, additional prospective randomized large-scale studies are needed to confirm our results. He presented with symptoms of lower back pain and legs pain. To investigate effects of surgical treatment on adult TCS, a retrospective analysis of 82 adult patients with TCS treated by surgery was conducted between March 2005 and December 2015. Refer a Patient. 6 For patients treated conservatively, follow-up information could be obtained in 33 of 42 patients. 6. Object: Although postsurgical neurological outcomes in patients with tethered cord syndrome (TCS) are well known, the rate and development of neurological improvement after first-time tethered cord release is incompletely understood. 1994 Oct;81(4):513-9. doi: 10.3171/jns.1994.81.4.0513. Clipboard, Search History, and several other advanced features are temporarily unavailable. 5 One of the most common complications related to this surgery is wound infection, especially if the incision (cut into the skin) is made around the low back. doi: 10.1097/MD.0000000000010111. The effect of tethered cord release on coronal spinal balance in tight filum terminale. Short-term postoperative results indicated a significant improvement of pain and a stabilization of neurological symptoms. 7 Wolters Kluwer Health A tethered cord release reduces or removes the . But in Case 3, the paraesthesia and weakness of lower extremities were persistent, and there Urologic dysfunction subjectively improved in 36% of the patients with that complaint. These guidelines often differ depending on surgical procedure. After exposing the dura mater spinalis, it was cut from the normal anatomical structure to the lesion. Surgery may also restore some function or We offer diagnostic and treatment options for common and complex medical conditions. The patient with symptoms following resection of a lipomyelomeningocele: do increases in the lumbosacral angle indicate a tethered spinal cord? Bethesda, MD 20894, Web Policies This lessens the chance of any major complications caused by damage to the spinal cord. This site needs JavaScript to work properly. Spinal cord infarction caused by sacral canal epidural steroid injection: A case report. To investigate effects of surgical treatment on adult tethered cord syndrome (TCS). Surgery was recommended for patients with symptoms only. 13 On the other hand, although massive intraoperative bleeding is a problem, the percentage of cases in which complications have developed has been low with SSO (Table 5). Red flags that might lead a doctor to suspect tethered cord include any of its symptoms (although the same symptoms can be caused by a number of other spinal cord conditions); a previous diagnosis of a congenital spinal malformation; a history of cancer, infection, spine surgery; or spinal cord injury. Frequent micturition, diminished knee and ankle reflexes, and difficulty in bending were exhibited in partial patients. sharing sensitive information, make sure youre on a federal WebThe dysfunction of nervous system in Cases 1, 2, and 4 disappeared within 3 weeks. Of the 2515 patients, 85 adults with a tethered cord syndrome formed the basis of this study. Your childs urinary catheter will be removed. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript. All the patients were from China and of Asian ethnicity. The clinical records were reviewed for preoperative symptoms, duration of symptoms, complications, and neurologic improvements. The result may be nerve damage and severe pain. For more information about these cookies and the data In adults, if the only abnormality is a thickened, shortened filum, then a limited lumbosacral laminectomy may suffice, with division of the filum once identified. Some people might continue to have The site is secure. The severity of the condition and the associated signs and symptoms vary from person to person. Stretching and tension, especially in a growing child, can cause neurologic damage. Untethering surgery was performed as a first procedure at our institution, and a massive arachnoidal scar and adhesion were found intraoperatively. Because the incision is lower on the back around a part of the spine that does not bear your childs body weight, pain is limited. WebSpray Foam Equipment and Chemicals. This causes extra stress on the nerves and can cause a range of symptoms known as tethered cord syndrome. Tethered Cord Syndrome in Children and Adults. Chapman P H. Congenital intraspinal lipomas: anatomic considerations and surgical treatment. Mohd-Zin SW, Marwan AI, Abou Chaar MK, Ahmad-Annuar A, Abdul-Aziz NM. One patient showed worsening of sensory function and another patient complained of a new lower back pain in the SSO group. Some patients may be misdiagnosed as having sciatica, a more common source of lower back . Tethered cord syndrome (TCS) is basically a condition where anchorage of a part of the spinal cord, most commonly the area of the conus, by some inelastic structure, which results in functional dysfunction involving the adhered segment of the spinal cord or at times segments beyond that. [2], In 1886, Von Reeklinghausen reported autopsy results of the patients with lumbosacral hypertrichosis accompanied with spina bifida, showing that the spinal cord was adhered to fat in the lumbosacral region, conus terminalis was indicated to be tensed. Complications after spinal anesthesia in adult tethered cord syndrome. These patients included those who had either tight terminal filum or secondary lesions that restricted the movement of the caudal spinal cord. [10] Of course, if the relief of tethered parts of the cauda equina obtained a relatively satisfactory outcome during the surgery, most occupying lesions and diseased filum terminale were removed, postoperative symptoms improved at different degrees, further recovery of the nerve function could thus be observed in the long-term follow-up period. The mean estimated blood loss during surgery (300 ml in the open group vs 167 ml in the mini-open group, p = 0.313) and the mean length of stay (7 days in the open group vs 6.3 days in the mini-open group, p = 0.718) were similar between the 2 groups. Improvement in clinical features was compared in the untethering and SSO groups (Table 3). There were 10 cases of lumbosacral intraspinal canal lipoma (12%), 32 cases of (39%) dermoid cyst and epidermoid cyst, and 40 cases (49%) without occupying lesions of tethered spinal cord. Bethesda, MD 20894, Web Policies Correspondence: Yongning Li, Department of Neurosurgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, No. [5] In 1976, Hoffman et al[6] reported 31 patients combined with conus medullaris after stretching slenderization, corresponding nerve function were improved following cutting off the tensive and thicker and filum terminale; besides, syndrome that the conus medullaris was stretched was named tethered spinal cord syndrome, has been used to describe for nervous dysfunction caused by conus medullaris stretching. 9 (A) A 37-year-old male patient with a lumbar spinal lipoma at L3/L4 level. 6 Tethered spinal cord syndrome is a neurologic disorder as well as a stretch-induced functional disorder caused . These electrodes connect to a computer that lets doctors know how well the nerves in your childs head, arms and legs are working throughout the surgery. Disclosures Hiroaki Nakashima, none WebConclusions: Tethered spinal cord syndrome in adults is an uncommon entity that can become symptomatic. The operation curative effects for TCS with different symptoms. Tethering lesions due to lipomas were maximally debulked, and occasionally the Cavitron Ultrasonic Surgical Aspirator was used (Valleylab, Boulder, Colorado, United States). 6 This can cause many different symptoms called tethered cord syndrome. There is very little out there on tethered cord in adults. Nineteen (86%) of 22 employed patients returned to work after surgery. 8 Posterior Vertebral Column Subtraction Osteotomy for Recurrent Tethered Cord Syndrome: A Multicenter, Retrospective Analysis. 13 Preoperative shorter duration of symptoms is associated with favorable clinical outcome because the pathophysiology of TCS is associated with impaired oxidative metabolism in the affected spinal cord.7 Tethered cord is usually present at birth . 11/2021. 2004 Aug;62(2):127-33; discussion 133-5. doi: 10.1016/j.surneu.2003.11.025. Only 5 of the conservatively treated patients experienced clinical deterioration over time; in 4 of these individuals with deterioration, surgery had been recommended but was refused by the patient. 9 PMC Apropos of a surgically treated case. From a surgical perspective, it is only necessary to remove the bony or .