Eagle syndrome: A rare neuropathic disorder affecting head and neck.

To investigate the clinical characteristics of Eagle syndrome (ES) and evaluate psychological distress of the patients. Ten cases of ES were enrolled, clinical characteristics and management were analyzed. Psychological disorders of the patients were assessed by the test of self-rating anxiety scale (SAS) and self-rating depression scale (SDS). There were 8 females and 2 males varying from 34 to 56 years with a mean age of 44.86 ± 8.38 years. The main complaints included foreign body sensation of pharynx, odynophagia, vertigo with turning of the head-neck, pain of anterolateral neck, and toothache. Three cases were right-side affected, 6 cases were left-sided and 1 case was bilateral. Radiographic examination showed the elongated styloid process of bilateral in all cases, however, hypertrophy, improper inclination, abnormal angulation of styloid process and more complete calcification of stylohoid ligament of the complained side were observed compared to the opposite side. Eight cases suffered from anxiety and/or depression. A surgical intervention was carried out on 6 patients to resect the elongated styloid process, the symptoms and mental distress disappeared after the operation and no recurrence was found in their follow-ups. Meticulous interrogation of illness history, proper examination, and radiological studies may be valuable in diagnostic confirmation of ES. It is the hyperostosis, abnormal angulation of the styloid process rather than the simple elongation which is more likely to be attributed to the development of ES. Psychological disorders in ES patients were observed in our study and should be paid more attention in the future research.

Ischemic stroke due to stylocarotid artery syndrome: a case report and review.

Stylocarotid artery syndrome (SAS) is a rare variant of Eagle's syndrome that may lead to transient ischemic attack or stroke. The underlying pathophysiological mechanism involves compression of the internal carotid artery by an elongated styloid process (ESP), potentially resulting in vascular occlusion or dissection. An ESP exceeding 2.5 cm is deemed elongated, with a length of 3.0 cm considered clinically significant. Although the prevalence of ESP ranges from 4.0% to 7.3%, symptomatic cases are rare; symptoms are present in only approximately 4.0% of individuals with an ESP. Unlike the typical symptoms of Eagle's syndrome, SAS may not cause pharyngeal discomfort, the sensation of a foreign body in the throat, dysphagia, or facial pain. This absence of characteristic symptoms as well as the development of central nervous system symptoms often leads patients to seek care from neurologists instead of otolaryngologists, increasing the likelihood of misdiagnosis or underdiagnosis. We herein report a unique case of ischemic stroke caused by SAS and present a literature review on cases of SAS-associated ischemic stroke published in the past decade. The reporting of this study conforms to the CARE guidelines.

A rare case of extensive neurogenic heterotopic ossification: a case report.

INTRODUCTION: Neurogenic Heterotopic ossification (NHO) is a potential sequalae and a detrimental complication following neurological insult. It is characterized by formation of localized gradually progressive, peri-articular lamellar bone formation in extra-skeletal tissues. We would like to report a rare case of heterotopic ossification involving all 4 limbs, in which we tried to restore joint mobility to improve his functional status so that he could perform his daily tasks. CASE PRESENTATION: We present a case of a 33-year-old bed ridden male, diagnosed with NHO involving all 4 limbs (bilateral hip, right knee, right shoulder, left elbow). The patient had a crippled posture, significant pain and impaired range of motion hampering movement of all four limbs which prevented him from lying down supine, sitting, walking and performing activities of daily living. After three surgeries, the patient achieved wheelchair mobilization and upright posture with the assistance of calipers. CONCLUSION: The management of NHO requires a multidisciplinary approach involving orthopaedic surgeons, neurologists & rehabilitation specialists. Prognosis of NHO depends on factors such as extent of ossification, underlying neurological condition & patients overall health.

Healing an ischial tuberosity pressure ulcer in a patient with neurogenic heterotopic ossification: a case report.

Neurogenic heterotopic ossification (NHO) is widely recognised as an aberrant bone formation in soft tissue following central nervous system injury. It is most frequently associated with pain and limited movement, especially in the hip. However, it may be neglected in patients with paraplegia with a pressure ulcer (PU). We report the case of an 18-year-old male patient who presented with a hard-to-heal ischial tuberosity PU and who had undergone three operations at other hospitals during the previous six months, which had failed to repair the PU. There was a history of paraplegia as a consequence of spinal cord injury two years previously. Computed tomography and three-dimensional reconstruction showed massive heterotopic ossification (HO) in the wound bed and around the right hip. Histological findings were consistent with a diagnosis of HO. The HO around the wound was completely excised, negative pressure wound therapy was used to promote granulation, and a gluteus maximus musculocutaneous flap was used to cover the wound. We conclude that for patients with paraplegia, with a hard-to-heal PU, it should be determined whether it is associated with NHO. Surgical resection of HO surrounding the wound and improving the microcirculation are critical for repair and reconstruction of these PUs.

Safety and efficacy of one-hole split endoscope technique for surgical treatment of thoracic ossification of the ligamentum flavum.

Surgical intervention is typically recommended for thoracic ossification of the ligamentum flavum (TOLF). This study aimed to evaluate the efficacy and safety of a novel non-coaxial one-hole split endoscope (OSE) technique for treating TOLF. We performed OSE procedure on 13 patients with TOLF from June 2022 to July 2023. The mean operative time was 117.5 ± 15.4 min. VAS scores for lower limbs decreased from 6.5 ± 0.8 preoperative to 1.6 ± 0.4 at the last follow-up (P < 0.001). ODI scores improved from 62.4 ± 5.7 preoperative to 18.6 ± 2.2 at the last follow-up (P < 0.001), and mJOA scores increased from 5.1 ± 1.6 preoperative to 8.4 ± 1.5 at the latest follow-up (P < 0.001). All patients achieved ASIA scale grade D or E at the final follow-up, except for two patients remained residual limb numbness. None of the thirteen patients suffered from severe perioperative complications. The OSE technique proves to be a safe and effective procedure for treating TOLF or even with dura mater ossification, characterized by minimal surgical trauma, relatively smooth learning curve and flexible operation.

3D-Printed Model in Preoperative Planning of Sciatic Nerve Decompression Because of Heterotopic Ossification: A Case Report.

CASE: A 31-year-old patient presented with an encapsulated sciatic nerve secondary to extensive hip heterotopic ossification (HO), which prevented visualization of a safe osteotomy site to avoid nerve damage. The 3D-printed model demonstrated an easily identifiable osseous reference point along the inferior aspect of the heterotopic mass, allowing for a vertical osteotomy to be safely performed. CONCLUSION: HO is associated with loss of normal anatomic topography. The current case report illustrates the use of a 3D-printed model to identify pertinent anatomic landmarks required for safe decompression of an encapsulated sciatic nerve within the anatomic region of the hip.

Eagle Syndrome: An Unusual Cause of Axial Neck Pain: A Case Report.

CASE: A 42-year-old woman presented with left-sided axial neck pain with radiation behind left ear of 3 months unresponsive to conservative treatment. As clinical examination revealed tenderness anterior to the mastoid process, she was referred to an otolaryngologist. Computed tomography showed an elongated left styloid process. Diagnostic local anesthetic infiltration provided complete pain relief confirming the diagnosis of Eagle syndrome (ES) after which a transoral excision of the left styloid process was performed. She was asymptomatic till the final follow-up of 15 months. CONCLUSION: ES should be considered in differential diagnosis of axial neck pain radiating to ear, and excision of the styloid process in resistant cases provides complete pain relief.

Effectiveness of topical sodium thiosulfate for ectopic calcifications and ossifications. Results of the CATSS-O study.

INTRODUCTION: Ectopic calcifications (ECs) and heterotopic ossifications (HOs) form in non-mineralized tissues, most often in subcutaneous and muscular areas. Local and systemic complications can cause severe disability. Systemic administration of sodium thiosulfate (STS) gives promising results but is difficult to use in clinical practice. OBJECTIVE: Evaluation of the efficacy and safety of topical STS in ECs and HOs. METHODS: Retrospective analysis of the CATSS-O registry that included patients receiving topical STS 25 % prepared by the pharmacy of Limoges hospital during 2014-2020. The efficacy of STS was assessed by imaging (radiography or CT) after at least 6 months' treatment. RESULTS: Among 126 patients who received STS 25 %, 35 had complete clinical and radiographic data for analysis (28 with ECs and 7 with HOs; 18 children [mean age 8.9 years, range 1.5-16], 17 adults [mean age 52.4 years, range 24-90]). Calcifications or ossifications were due to dermatomyositis (8 children, 6 adults), systemic scleroderma (6 adults) or pseudo-hypoparathyroidism 1A (7 children). They were single (37.1 %) or multiple (62.9 %). Treated regions were in the lower limbs (31.4 %), upper limbs (37.1 %) or both (28.6 %) and the axial region (2.9 %). Topical STS was clinically effective in 9/28 (32.1 %) patients with ECs and 2/7 (28.6 %) children with HOs. Three patients experienced complete disappearance of their calcifications. Response for ECs was better in children than adults (54.5% vs 17.6 %, p = 0.035). Topical STS was well tolerated. CONCLUSION: Local STS seems effective for ossifications, particularly pediatric calcifications or ossifications. Randomized and experimental studies are needed to confirm this observation and to identify the underlying mechanisms.

The biomechanical effect of different types of ossification of the ligamentum flavum on the spinal cord during cervical dynamic activities.

Ossification of the ligamentum flavum (OLF) is thought to be an influential etiology of myelopathy, as thickened ligamentum flavum causes the stenosis of the vertebral canal, which could subsequently compress the spinal cord. Unfortunately, there was little information available on the effects of cervical OLF on spinal cord compression, such as the relationship between the progression of cervical OLF and nervous system symptoms during dynamic cervical spine activities. In this research, a finite element model of C1-C7 including the spinal cord featured by dynamic fluid-structure interaction was reconstructed and utilized to analyze how different types of cervical OLF affect principal strain and stress distribution in spinal cord during spinal activities towards six directions. For patients with cervical OLF, cervical extension induces higher stress within the spinal cord among all directions. From the perspective of biomechanics, extension leads to stress concentration in the lateral corticospinal tracts or the posterior of gray matter. Low energy damage to the spinal cord would be caused by the high and fluctuating stresses during cervical movements to the affected side for patients with unilateral OLF at lower grades.

Endometrial Osseous Metaplasia-A Rare Cause of Infertility with Unknown Etiology.

Background: Osseous tissue in the endometrium is a rare find, and it is most often discovered when the patient presents with infertility. It is frequently associated with dysmenorrhea and abnormal menstrual bleedings. Although its etiology remains unclear, in almost all described cases until now, the patient has an obstetrical history. Case report: In this report, we present a unique case of endometrial osseous metaplasia in a 27-year-old primary infertile patient. The transvaginal ultrasound revealed a 18/13/7 mm hyperechoic endometrial mass with posterior acoustic shadowing and no flow on color Doppler. A hysteroscopic examination found a polygonal calcification on the endometrial posterior face of the uterine cavity, in the corporeal isthmic region, which was extracted. The histopathological evaluation revealed microscopic elements compatible with endometrial calcification. The patient had a good postoperative course and the complex endocrinologic, immunologic and electrolytical investigation failed to prove any abnormality. Follow-up transvaginal ultrasound examinations revealed no modifications. Three years later, the patient conceived spontaneously, had an uneventful pregnancy and delivered a full-term fetus. Conclusion: We assumed that this entity can be a serious cause of infertility since the patient had a long history of (primary) infertility and its resection made the pregnancy's occurrence possible. Finally, since neither history of abortion or chronic inflammation nor any abnormal laboratory test were noticed, we concluded that the etiology of this entity remained unclear.

Giant eagle syndrome with pseudoarthrosis and peripheral facial palsy. / Síndrome de Eagle con pseudoartrosis y parálisis facial periférica.

TITLE: Síndrome de Eagle con pseudoartrosis y parálisis facial periférica.

Learning Curve and Clinical Outcomes of Ultrasonic Osteotome-based En Bloc Laminectomy for Thoracic Ossification of the Ligamentum Flavum.

OBJECTIVE: Despite rapid advances in minimally invasive surgery, en bloc laminectomy remains the most common surgical approach for treating thoracic ossification of the ligamentum flavum (TOLF). However, the learning curve of this risky operation is rarely reported. Therefore, we aimed to describe and analyze the learning curve of ultrasonic osteotome-based en bloc laminectomy for TOLF. METHODS: Among 151 consecutive patients with TOLF who underwent en bloc laminectomy performed by one surgeon between January 2012 and December 2017, we retrospectively analyzed their demographic data, surgical parameters, and neurological function. Neurological outcome was evaluated with the modified Japanese Orthopaedic Association (mJOA) scale, and the Hirabayashi method was used to calculate the neurological recovery rate. The learning curve was assessed with logarithmic curve-fitting regression analysis. Univariate analysis methods were used for statistical analysis, including t-test, rank sum test, and chi-square test. RESULTS: A total of 50% of learning milestones could be reached in approximately 14 cases, and the asymptote in 76 cases. Therefore, 76 of the 151 enrolled patients were defined as the "early group," and the remaining 75 were delimitated as the "late group" for comparison. There was a significant intergroup difference in the corrected operative time (94.80 ± 27.77 vs 65.93 ± 15.67 min, P < 0.001) and the estimated blood loss (median 240 vs 400 mL, P < 0.001). The overall follow-up was 83.1 ± 18.5 months. The mJOA significantly increased from a median of 5 (IQR: 4-5) before the surgery to 10 (IQR: 9-10) at the last follow-up (P < 0.001). The overall complication rate was 37.1%, and no significant intergroup difference was found, except for the incidence of dural tears (31.6% vs 17.3%, p = 0.042). CONCLUSION: Initially, mastering the en bloc laminectomy technique using ultrasonic osteotome for TOLF treatment can be challenging, but the surgeon's experience improves as the operative time and blood loss decrease. Improved surgical experience reduced the risk of dural tears but was not associated with the overall complication rate or long-term neurological function. Despite the relatively long learning curve, en bloc laminectomy is a secure and valid technique for TOLF treatment.

Laminectomy for the Removal of Thoracic Ossification of the Ligamentum Flavum (TOLF) Using Ultrasonic and Conventional Osteotomes.

Thoracic ossification of the ligamentum flavum (TOLF) is a common cause of progressive thoracic myelopathy. TOLF is typically treated with surgical decompression. A variety of surgical techniques, including laminoplasty, laminectomy, and lamina fenestration, are used for the effective treatment of TOLF. However, traditional methods are associated with a substantial risk of perioperative complications, including dural laceration and/or iatrogenic spinal cord injury. Therefore, it is important to develop an efficient and secure surgical technique for TOLF. Herein, we describe a method for laminectomyperformed at the thoracic spine using an ultrasonic osteotome combined with a conventional osteotome. This technique can reduce intraoperative complications. This is a relatively safe and easy-to-learn method that should be recommended for the treatment of TOLF.

Differences in the Osseous Ultrastructure in 2 Differing Etiologies of Eagle Syndrome. A Micro-CT Study.

OBJECTIVES: Eagle syndrome is a rare disease caused by an elongated styloid process (type I) or ossified stylohyoid ligament (type II) and causes a heterogeneous symptom complex, ranging from pain in the throat and neck to neurological symptoms and neurovascular entrapment. The 2 different types present differing shapes and ultrastructures and cause different symptoms. This study aimed to distinguish the 2 types by investigating the structures by micro-computed tomography. METHODS: Micro-computed tomography was performed and evaluated in n=10 resected styloid processes from patients diagnosed with Eagle syndrome. The tissues were measured for their shape, ratio of soft tissue and bone amounts, bone volume, and ultrastructure, and compared within the groups. RESULTS: The shapes of the different types were different and the ultrastructure differed between the 2 groups, with an absence of trabecular architecture in type II. The area of bone to nonbone tissues in type I samples was significantly higher compared with type II ( P =0.007). Alike these results, the bone volume and bone-to-soft tissue ratio were significantly higher in type I compared with type II ( P =0.009). CONCLUSIONS: The findings suggest that both the popular theories (hyperplasia and metaplasia) may be probable but each solely valid for 1 type of Eagle. Type I may derive from bone hyperplasia with cancellous bone formation and rather high bone density in the elongated styloid process. Type II most likely originates from ligament metaplasia into bone without a compact structure.

Indomethacin for heterotopic ossification prophylaxis following surgical treatment of elbow trauma: a randomized controlled trial.

BACKGROUND: Heterotopic ossification is a frequent complication following surgical treatment of elbow trauma. The use of indomethacin to prevent heterotopic ossification is reported in the literature; however, its effectiveness is controversial. The purpose of this randomized, double-blind, placebo-controlled study was to determine whether indomethacin is effective in reducing the incidence and severity of heterotopic ossification after surgical management of elbow trauma. METHODS: Between February 2013 and April 2018, 164 eligible patients were randomized to receive postoperative indomethacin or placebo medication. The primary outcome was the incidence of heterotopic ossification on elbow radiographs at 1-year follow-up. Secondary outcomes included the Patient Rated Elbow Evaluation score, Mayo Elbow Performance Index score, and Disabilities of the Arm, Shoulder and Hand score. Range of motion, complications, and nonunion rates were also obtained. RESULTS: At 1-year follow-up, there was no significant difference in the incidence of heterotopic ossification between the indomethacin group (49%) and the control group (55%) (relative risk, 0.89; P = .52). There were no significant differences in postoperative Patient Rated Elbow Evaluation, Mayo Elbow Performance Index, and Disabilities of the Arm, Shoulder and Hand scores or range of motion (P = .16). The complication rate was 17% in both the treatment and control groups (P > .99). There were no nonunions in either group. CONCLUSION: This Level I study demonstrated that indomethacin prophylaxis against heterotopic ossification in the setting of surgically treated elbow trauma was not significantly different from placebo.

Full-Endoscopic Decompression for Thoracic Ossification of the Ligamentum Flavum Combined with Dural Ossification.

Ossification of the ligamentum flavum (OLF) can result in spinal stenosis. Thoracic spinal cord compression due to spinal stenosis is a common cause of progressive thoracic myelopathy in Asian countries. The incidence of complications is high in open decompression surgeries for thoracic OLF. With dural ossification (DO), the risk of complications is even higher in thoracic OLF. We introduce a full-endoscopic decompression surgery for thoracic OLF combined with DO under local anesthesia. Hemilaminectomy is performed using a high-speed burr under the endoscopy first, and then decompression of the contralateral spinal canal is completed using an "over the top" technique. DO resection uses the eggshell technique; after the base of the DO is cut from the lamina, forceps or lamina rongeurs are typically used for removal. The dural defect left after resection does not need repair. Neurological function was improved, and no complications such as hematoma or neck pain occurred. On imaging, no pseudodural cyst, cerebrospinal fluid leakage, or wound complications were observed after the operation. Endoscopic surgery causes less damage to the posterior ligament complex, so no cases of persistent back pain complaints or secondary internal fixation requirements were found in this study. Full-endoscopic decompression can achieve good imaging and clinical effects in the treatment of thoracic OLF with DO.

Lumbar ossification of the ligamentum flavum reflects a strong ossification tendency of the entire spinal ligament.

Patients with ossification of the ligamentum flavum (OLF) in the lumbar spine may be at high risk of developing concomitant ossification of the entire spinal ligament, but the etiology remains unclear. We investigated the propensity for spinal ligament ossification in asymptomatic subjects with lumbar OLF using the data of 595 Japanese individuals receiving medical check-ups, including computed tomography (CT) scanning. The severity of OLF (total number of intervertebral segments with OLF) of the entire spine on CT was quantified using an OLF index. Subjects with OLF were grouped according to this index: localized OLF (n = 138), intermediate OLF (n = 70), and extensive OLF (n = 31). The proportion of subjects with lumbar OLF increased with increasing OLF index (localized 13.7%, intermediate 41.4%, and extensive 70.9%). Multiple regression analysis found that lumbar OLF index was associated with thoracic OLF index, and co-existence of ossification of the posterior longitudinal ligament (OPLL) of the thoracic and lumbar spine. This study showed that subjects with more multilevel lumbar OLF were more likely to develop multilevel thoracic OLF and to have coexisting OPLL. Patients with lumbar OLF may be a distinctive subgroup with a strong tendency to ossification of the entire spinal ligament.

Novel therapeutic strategy in the treatment of ossification of the ligamentum flavum associated with dural ossification.

PURPOSE: To investigate the imaging characteristics of thoracic ossification of ligamentum flavum (OLF) combined with dural ossification (DO) and the clinical efficacy of zoning laminectomy. METHOD: The clinical data of 48 patients with thoracic OLF combined with DO who underwent zoning laminectomy between June 2016 and May 2020 were retrospectively analyzed. The modified Japanese Orthopedic Association (mJOA) score was used to evaluate neurological function before and after surgery, and the clinical efficacy was evaluated according to the improvement rate. RESULTS: The symptoms of all patients significantly improved after the operation, and the average follow-up time was 27.8 (10-47) months. In addition, the average mJOA score had increased from 5.0 (2-8) preoperatively to 8.7 (6-11) postoperatively (t = 18.880, P < 0.05). The average improvement rate was 62.6% (25-100%), with 16 patients graded as excellent, 21 as good, and 11 as fair. Cerebrospinal fluid leakage occurred in 12 cases (25.0%), and all of them healed well after treatment. No postoperative aggravation of neurological dysfunction, wound infection or hematoma occurred. At the last follow-up, there was no recurrence of symptoms and kyphosis. CONCLUSION: The Zoning laminectomy described here is both safe and effective.

Bony Cubital Tunnel Syndrome: A Case Report of Heterotopic Ossification Causing Circumferential Ulnar Nerve Encasement at the Elbow and Review of Current Management.

BACKGROUND: Heterotopic ossification (HO) is a rare but known complication of brain and nerve trauma, orthopedic trauma, and burns. Nerve compression due to HO is extremely rare; "bony cubital tunnel syndrome," or compression of the ulnar nerve at the elbow due to HO, is an unusual presentation that requires special considerations for treatment. CASE PRESENTATION: We present a 50-year-old man who presented to our hospital after vehicular polytrauma with associated car fire and prolonged extrication. He experienced extensive trauma, with all classically described risks for HO. He developed bony cubital tunnel syndrome, with ulnar neuropathy confirmed on electrodiagnostic studies, and underwent surgical decompression. Surgical decompression revealed circumferential encasement of the ulnar nerve in heterotopic bone, all of which was removed. He demonstrates appropriate recovery of nerve function. LITERATURE REVIEW: All perineural HO should be excised early to prevent nerve injury, because excision within 4 months of development is linked to improved functional outcomes. Measures to prevent nerve compression by HO are all associated with delayed wound or bone healing and should be considered on an individual basis.