Restricted Neck Movements Research Articles

Neck Pain in a Young Girl: A Pott's Disease Case Study

Cervical spinal tuberculosis (TB) is a rare variant of extrapulmonary TB with a high complication rate. Tuberculosis of the cervical spine is reported in about 6 to 9% of all cases of spinal TB. Early diagnosis and treatment of spinal TB is essential in order to prevent neural deficit. Management strategies for spinal TB range from ambulatory chemotherapy to radical surgical debridement with fusion. We present a case of an Old Dhaka citizen, 15-year-old girl, who was admitted to the Department of Physical Medicine and Rehabilitation, Bangabandhu Sheikh Mujib Medical University, because of a severe painful restriction of neck movement with torticollis to left for 5 months, neck stiffness, and restriction of neck movement in all planes of cervical spine. On physical examination, the girl was subfebrile, mildly anemic, and tenderness present over the upper part of cervical spine. Head was deviated toward left side. Neurological examination revealed no focal motor weakness. The roentgenograms of chest, pelvis, and cranium were without pathological changes. Abdominal ultrasonography was normal. A magnetic resonance imaging scan revealed marrow contusion in body of C2 with odontoid process, adjacent anterior and left side of prevertebral soft tissue swelling with small collection at C2 level, and scoliosis with straightening of cervical spine. Histopathological examination of the removed material showed typical granulomatous inflammation with characteristic infiltrate of lymphocytes, epithelioid macrophages, and Langhans-type multinucleated giant cells.

Comparison of success of tracheal intubation using Macintosh laryngoscope-assisted Bonfils fiberscope and Truview video laryngoscope in simulated difficult airway

Background and Aims:Restriction of head and neck movements prevents the alignment of the oral, pharyngeal, and laryngeal axes and increases the incidence of difficult tracheal intubation in patients with cervical spine fractures. Video laryngoscopes have gained an important role in the management of difficult intubation, especially in situations with limited head and neck movements. This study compares the success of intubation using Macintosh laryngoscope assisted Bonfils® fiberscope (ML-BF) with TruviewPCD video laryngoscope (TV) in patients with simulated restricted head and neck movements.Material and Methods:One hundred and fifty-two patients satisfying the inclusion criteria were randomly allocated to two groups of 76 each. Patients were made to lie supine on the table without a pillow and a soft collar was used to restrict head and neck movements. After a standardized premedication-induction sequence, tracheal intubation was done either with ML-BF or TV. Success of intubation, time taken for successful intubation, hemodynamic changes, airway trauma, and postoperative oropharyngeal morbidity were noted.Results:Intubation was successful in all the 76 patients in direct laryngoscopy-Bonfils fiberscope group and 75 out of 76 patients in TV group within the specified time (90 s). The median time taken for successful intubation with TV and ML-BF were 44 (range 26–80) s and 49 (range 28–83) s, respectively. Hemodynamic changes, airway trauma, and postoperative oropharyngeal morbidity were similar in both groups.Conclusion:Both TV and ML-BF are equally effective for successful tracheal intubation in patients with simulated restricted head and neck movements. In cases of difficult laryngeal visualization with routine Macintosh laryngoscope, Bonfils can be used as an adjunct to achieve successful intubation in the same laryngoscopy attempt.

Management of a Case of Dropped Nucleus following Small Incision Cataract Surgery in a Patient with Thoracic Kyphoscoliosis and Review of Literature

ABSTRACT A curved spine accompanied by restricted neck motion poses a challenge for an ophthalmic surgeon, especially a vitreoretinal surgeon, who needs the patient’s eyes in a horizontal position to operate with the microscope. Literature is sparse with case reports of thoracic kyphoscoliosis for vitreoretinal surgery, although many reports are available for cataract surgery. We report a case of thoracic kyphoscoliosis and ankylosing spondylitis with dropped nucleus into the vitreous cavity following a complicated cataract surgery posted for pars plana vitrectomy, pars plana lensectomy, and phacofragmentation under general anesthesia and review the current literature of such case. How to cite this article Mandal B, Katoch D, Handa S. Management of a Case of Dropped Nucleus following Small Incision Cataract Surgery in a Patient with Thoracic Kyphoscoliosis and Review of Literature. Res Inno in Anesth 2017;2(1):21-23.

A proposal for a new scoring system to predict difficult ventilation through a supraglottic airway

BackgroundThe aim of this study was to propose and validate a new clinical score to predict difficult ventilation through a supraglottic airway device. MethodsThe score was proposed from our previously reported derivation data, and we prospectively validated the score in 5532 patients from November 2013 to April 2014. Predictive accuracy of the score was compared by the area under the receiver operating characteristic (ROC) curve (AUC). We assigned point values to each of the identified four risk factors: male, age >45 yr, short thyromental distance, and limited neck movement, their sum composing the score. The score ranged between 0 and 7 points. The optimal predictive level of the score was determined using ROC curve analysis. ResultsThe AUC of the score was 0.75 (95% CI 0.66 to 0.84) in the validation data set, and was similar to that in the derivation data set (0.80; 95% CI 0.75 to 0.86). In derivation and validation data sets, the incidence of low risk categories (scores 0–3) was 0.42% vs 0.32% and of high risk categories (scores 4–7) was 3% vs 1.7% respectively. A score 4 or greater is associated with a six to seven fold increased risk of difficult ventilation through a supraglottic airway device. ConclusionsThe new score for prediction of difficult ventilation through a supraglottic airway device is easy to perform and reliable, and could help anaesthetists plan for difficult airway management.

Evaluation of six videolaryngoscopes in 720 patients with a simulated difficult airway: a multicentre randomized controlled trial

BackgroundVideolaryngoscopes are aggressively marketed, but independent evaluation in difficult airways is scarce. This multicentre, prospective randomized controlled trial evaluates six videolaryngoscopes in patients with a simulated difficult airway. MethodsWith ethics committee approval and written informed consent, 12 senior anaesthetists intubated the trachea of 720 patients. A cervical collar limited mouth opening and neck movement, making intubation difficult. We evaluated three unchannelled (C-MAC™ D-blade, GlideScope™, and McGrath™) and three channelled videolaryngoscopes (Airtraq™, A.P. Advance™ difficult airway blade, and KingVision™). The primary outcome was first-attempt intubation success rate. Secondary outcomes included overall success rate, laryngeal view, intubation times, and side-effects. The primary hypothesis for every videolaryngoscope was that the 95% confidence interval of first-attempt success rate is ≥90%. ResultsMouth opening was decreased from 46 (sd 7) to 23 (3) mm with the cervical collar. First-attempt success rates were 98% (McGrath™), 95% (C-MAC™ D-blade), 87% (KingVision™), 85% (GlideScope™ and Airtraq™), and 37% (A.P. Advance™, P<0.01). The 95% confidence interval of first-attempt success rate was >90% only for the McGrath™. Overall success, laryngeal view, and intubation times differed significantly between videolaryngoscopes (all P<0.01). Side-effects were minor. ConclusionsThis trial revealed differences in the performance of six videolaryngoscopes in 720 patients with restricted neck movement and limited mouth opening. In this setting, first-attempt success rates were 85–98%, except for the A.P. Advance™ difficult airway blade. Highest success and lowest tissue trauma rates were achieved by the McGrath™ and C-MAC™ D-blade, highlighting the importance of the videolaryngoscope blade design. Clinical trial registrationClinicalTrials.gov: identifier NCT01692535.

Occipitalization of atlas - a case report

AbstractThe atlas vertebra articulates with the occipital bone of the skull by a pair of sunovial joints. Occipitalization is a congenital fusion of the atlas to the base of the occiput which reduces the foramen mangnum dimension leading to neurological complications due to compression of spinal cord. Individuals with occipitalization of the atlas may have low hairline, torticollis, restricted neck movement and abnormal short neck. During routine osteology demonstration class for undergraduate students, a total atlanto-occipital fusion was seen. The clinical implications of altanto-occipitalization may be beneficial for neurosurgeons, neurologists, orthopaedic surgeons and radiologists in day-to-day clinical practice.

Klippel-Feil syndrome with butterfly vertebrae: rare case

A 42-year-old woman presented to the neurosurgery clinic with neck pain. She exhibited short neck, low posterior hairline, and limited neck movement. She had a normal neurologic examination. Magnetic resonance imaging and computed tomography revealed multiple congenital fusions of the cervical (C5–C7) and upper thoracic (T1–T3) vertebral body (Fig. 1, Fig. 2). Multiple hemivertebrae and butterfly vertebrae were also determined by magnetic resonance imaging (Fig. 1). Klippel-Feil syndrome with butterfly vertebrae is very rare but has been reported [ [1] Rahimi-Movaghar V. Moghtaderi A. Shahriarian S. Keshmirian B. Klippel-Feil anomaly associated with thoracic hemivertebrae/butterfly vertebrae and patella hypoplasia. Clin Dysmorphol. 2004; 13: 39-41 Crossref Scopus (3) Google Scholar ]. Fig. 2Three-dimensional volume-rendering computed tomographic (CT) image shows multiple congenital fusions of the cervical and upper thoracic vertebral body. View Large Image Figure Viewer Download Hi-res image

EVALUATION AND MANAGEMENT OF DIFFICULT AIRWAY IN OBESITY: A SINGLE CENTER RETROSPECTIVE STUDY

The primary aim of this single center retrospective study was to evaluate difficult mask ventilation (DMV) and difficult laryngoscopy (DL) in a unique group of obese patients. A total of 427 adult patients with body mass index (BMI) ≥ 25 and surgically treated for endometrial cancer from 2011 to 2014 were assessed. Additional increase in BMI, comorbidities, bedside screening tests for risk factors, and the tools used to manage the patients were noted and their effects on DMV and/or DL investigated. Every escalation in the number of risk factors increased the probability of DMV 2.2-fold, DL 1.8-fold and DMV+DL 3.0-fold. Among bedside tests, limited neck movement (LNM), short neck (SN) and absence of teeth were significant for DMV (p < 0.05), LNM, SN and obstructive sleep apnea for DL (p < 0.05), and LNM and SN for DMV+DL (p < 0.05). However, a 10-point increase of BMI was not an independent risk factor when patients with BMI > 25% were considered. In conclusion, LNM and SN are independent risk factors for developing DMV and/or DL in obese endometrial cancer patients, while BMI increase over 30 was not additionally affecting difficult airway.

AIRWAY MANAGEMENT WITH DIRECT LARYNGOSCOPY IN A CHILD WITH GOLDENHAR SYNDROME

Goldenhar syndrome, also known as oculoauriculovertebral dysplasia, is a rare congenital condition characterized by facial, cranial, vertebral, ocular, auricular and cardiac abnormalities. This syndrome is associated with hemifacial microsomia due to inadequate growth of the mandible and vertebral anomaly of the cervical part of the spine. For anesthesiologists, airway management is of great interest because of facial and oral abnormalities such as mandibular hypoplasia and limitation of neck movement. Considering different conditions of Goldenhar syndrome, every patient should be preoperatively evaluated in order to make the plan for the anticipated difficult airway management. We report a case of a 2.5-year-old boy undergoing palatoplasty, who required general anesthesia and orotracheal intubation. Our decision to intubate with direct laryngoscopy and with slight external pressure on the larynx proved successful.

A rare case of klippel feil syndrome with cleft palate: A case report

Klippel-Feil syndrome (KFS) is a rare condition also known as synostosis of cervical spine, first described in the medical literature in 1912. It consists of the triad of symptoms such as short neck, limitation of neck movements, and low posterior hairline and also associated with somatic diseases and defects such as scoliosis, anomalies in urogenital system, congenital heart defects, deafness, facial asymmetry, synkinesis, or mirror movements. KFS manifests in the oral cavity with cleft palate, limitation of mouth opening, hypodontia oligodontia, and micrognathia.

Difficult Intubation Factors in Prehospital Rapid Sequence Intubation by an Australian Helicopter Emergency Medical Service

ObjectivePrehospital rapid sequence intubation (RSI) of critically ill trauma patients is a high-risk procedure that may be associated with an increased rate of severe complications such as failed intubation, failure of oxygenation, hypoxia, hypotension, or need for surgical airway. The objective of this study was to describe the factors associated with difficult intubation in prehospital RSI as defined by more than a single look at laryngoscopy to achieve tracheal intubation. MethodsThis is an observational study using prospectively collected data. ResultsFour hundred forty-three RSIs were performed. Paramedics were the initial laryngoscopist in 290 (65.5%). First-look laryngoscopy resulted in successful tracheal intubation (TI) in 372 (84.0%) (95% confidence interval, 80.3%-87.1%). Intubation was achieved on second look at laryngoscopy in 58 (13.1%). “First-pass” TI was achieved in 394 (88.9%). Overall, successful TI was achieved in 438 (98.9%) (95% confidence interval, 97.4%-99.5%). Complications occurred in 116 (26.2%), with desaturation the commonest in 77 (17.4%). ConclusionFactors associated with more than 1 look at laryngoscopy before TI included paramedic laryngoscopist and the presence of at least 1 of the following indicators: blood/vomitus in the airway, limited mouth opening, and limited neck movement. Trauma to face/neck, obese body habitus, C-spine precautions, cricoid pressure, midline stabilization, and intubation on the ground did not influence the level of difficulty encountered.

General anaesthesia for parturients with spondyloepiphyseal dysplasia: Risky but possible!

Sir, A 28-year-old primigravida, 135 cm tall and a known case of spondyloepiphyseal dysplasia (SED), with 37 weeks of gestation, was scheduled for elective caesarean section. The patient was unable to walk and was wheelchair bound. The flexion deformity of the knee joint, as well as the hip joint, made it difficult for the patient to lie down straight [Figure 1]. In addition, she had flexion deformities of the elbow and wrist joints.Figure 1: Patient with fixed flexion deformities of the knee and hip jointsThe patient was the first child of her parents who had non-consanguineous marriage. The other siblings of the patient also had similar deformities. Patient's biochemical, haematological investigations, echocardiography and pulmonary function tests were unremarkable. The patient had limited neck movements but had modified Mallampati Airway Class 3. The patient's neck extension was two-thirds restricted and her mouth opening was about 40 mm. Other airway parameters were within normal limits. Examination of the spine revealed scoliosis, and intervertebral spaces were difficult to palpate. Radiographic examination (pre-pregnancy) revealed platyspondyly, reduced disc height as well as features of premature secondary arthritis in both hip joints [Figure 2]. She was unable to position herself for regional anaesthesia. The fixed flexion deformity of the knee prevented lateral supine positioning of the patient. She could sit with two pillows behind her back and a pillow below her knee, and slept in head-up position.Figure 2: X-ray of the hip joints showing early osteoarthritis resulting in fixed flexion deformityThe patient did not consent for regional anaesthesia. Hence, it was decided to conduct caesarean section under general anaesthesia. She was positioned with a pillow under the knees along with a wedge under the abdomen. The head was also supported on a head ring with two pillows below her head. Intravenous line was secured with an 18 gauge cannula and infusion of Ringer lactate was started. Routine monitors were connected and basal readings noted. Awake nasal fibre-optic intubation was performed with small size of endotracheal tube (ETT) using cuffed polyvinyl chloride 6.5 mm ETT uneventfully. The rest of the anaesthetic and post-operative period was uneventful. SED is an extremely rare genetic entity with incidence of 1–4 per million. Regional anaesthesia is the method of choice in parturients with SED although it is technically difficult and has variable effects. Administration of general anaesthesia in such patients is not without risks. The presence of kyphoscoliosis in SED may lead to significant respiratory compromise that gets exaggerated in pregnancy due to diaphragmatic splinting caused by gravid uterus.[1] Pregnancy further exaggerates the already existing respiratory compromise and enhances aortocaval compression. The presence of atlantoaxial instability due to odontoid hypoplasia, central anterior pointing of the cervical spine along with ligamentous laxity in SED may increase the risk of severe spinal cord compression. Tetraparesis has been reported after intubation in these patients due to cervical spine instability.[2] Pregnancy also poses an added risk for aspiration.[1] Patients with SED may have associated laryngotracheal stenosis, which may pose difficulty in airway management. There are reports of intubation being guided by lightwand.[3] Intubation with video laryngoscope could be another tangible alternative.[4] However, video laryngoscope was unavailable during intubation in the management of the present patient. Thus, patients with SED with pregnancy can pose a special challenge for the anaesthesiologist. However, while administering general anaesthesia, proper assessment and monitoring for cervical spine stability and respiratory status, positioning of the patient so as to prevent any untoward neurological injury and meticulous planning of airway management and perioperative care may go a long way in successful outcome. Declaration of Patient Consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. Acknowledgement We would like to acknowledge Dr. P. N. Gupta, Associate Professor, Department of Orthopaedics, Government Medical College and Hospital, Chandigarh, for his help.

Bronchoscopic concerns in Proteus syndrome: a case report.

Proteus syndrome (PS) is a rare congenital hamartomatous disorder with multisystem involvement. PS shows highly clinical variability due to overgrowth of the affected areas, and several features can make anesthetic management challenging. Little is known about the airway problem associated with anesthesia in PS patients. An 11-year-old girl with PS was scheduled for ear surgery under general anesthesia. She had features complicating intubation including facial asymmetry and disproportion, abnormal teeth, limitation of neck movement due to torticollis, and thoracolumbar scoliosis. This study reports on a case of deformed airway of a PS patient under fiberoptic bronchoscopy.

Clinical analysis of acute limited neck motion in 420 children

Objective To study the clinical features of acute limited neck motion in children and its differen-tial diagnosis, and to improve doctors' awareness and the early diagnosis of the disease. Methods A total of 420 patients with acute limited neck motion as the chief complaint and atlantoaxial rotary subluxation as the first diagnosis between January 2005 and January 2014 in Nanjing Children's Hospital Affiliated to Nanjing Medical University were enrolled.The clinical manifestations, imaging and consultation data of the 420 patients were retrospectively analyzed. Results There were totally 248 male patients and 172 female patients.The ages ranged from 2 to 14 years old(the mean age was 7.2 years old), the course ranged from 2 hours to 2 days.Among 420 patients, 353 patients were diagnosed as atlantoaxial rotatory subluxation.Forty-two patients were diagnosed as cervical intervertebral disc calcification.Seven patients were diagnosed as Tourette syndrome.Four patients were diagnosed as intracranial tumors.Three patients were diagnosed as cervical eosinophilic granuloma.Two patients were diagnosed as cervical spinal tuberculosis.Two patients were diagnosed as viral encephalitis.Two patients were diagnosed as benign paroxysmal torticollis.Two patients were diagnosed as Kawasaki disease.One patient was diagnosed as neuroblastoma.One patient was diagnosed as hemophilia A. One patient was diagnosed as drug adverse reaction of metoclopramide.According to Fielding clinical classification, 204 patients were type Ⅰ, 122 patients were type Ⅱ, 21 patients were type Ⅲ, and 6 patients were type Ⅳ. Conclusions Based on disease distribution of acute limited neck motion in children, atlantoaxial rotatory subluxation seems to be the predominant while non-atlantoaxial rotatory subluxation is not uncommon.Differential diagnoses should be considered to avoid misdiagnosis.For atlantoaxial rotatory subluxation, conservative treatment including continuous mandible occipital belt traction and brace has been proved to be very effective methods. Key words: Atlantoaxial rotatory subluxation; Acute limited neck motion; Differential diagnosis; Child

Are C2 pars-pedicle screws alone for type II Hangman's fracture overrated?

Background/objectThe recent trend for treatment of certain cases of type II Hangman's fracture has been towards motion preserving surgery. This is claimed to be achieved with placement of pedicle screws across the fracture fragments. However, the long term outcome in clinical scenario is not yet clear, neither are the factors determining suitability of such a technique. Materials and methodsWe have retrospectively analyzed the results of 11 patients of type II Hangman's fracture, according to the extent of translation. Nine patients underwent stabilization of fracture with C2 pedicle screws and 2 were managed with halo immobilization. The conservative management failed in one and this patient underwent internal fixation using pars-pedicle screw as well. The long term clinical and radiological (CT and dynamic X-rays) outcome was analyzed. ResultsAll patients including the one with halo immobilization, showed solid fusion across the fracture fragments. With the exception of one patient none had any clinical symptoms. This lone patient complained of restricted neck movements. Three different types of radiological results were observed. Two patients with translation >8mm showed C2–3 body fusion. Three of 6 patients with minimal translational (3–4mm) showed facet fusion. Three patients with moderate translational dislocation (4.5–5.5mm) showed persisting C2–3 angular instability. ConclusionsThe C2 pedicle screw is a good technique for osteosynthesis. However, the claimed long term advantage of motion segment preservation with this technique remains doubtful. It may be suitable for those fractures with minimal translation (<4mm), where the superiority of surgery, itself, over external immobilization is questionable. C2–3 fusion is preferable for those fractures with translation >4mm as these are unstable and C2 pedicle screws alone are likely to have less desirable results.

Head and neck surgery in a tertiary centre: Predictors of difficult airway and anaesthetic management

Introduction: The management of head and neck surgical patients is associated with increased morbidity and mortality, and so anticipating the difficult airway is important. Methods: We undertook a prospective survey on consecutive adult patients scheduled on the elective operating lists of four head and neck consultant surgeons. Data were collected over a 36 month period. Data included: patient characteristics; routine predictors of difficulty in airway management (bedside tests of the airway, a history of previous surgery or radiotherapy and the presence of airway symptoms); laryngoscopy grade; method of anaesthesia and airway management; and any airway complications arising during induction of anaesthesia and extubation. Results: The ‘study’ group consisted of 818 patients. The ‘direct laryngoscopy’ group contained 674 patients, that is, patients who had direct laryngoscopy and could therefore be classified as easy or difficult intubation. The prevalence of difficult intubation was 12.6%. Factors or tests that were statistically significantly associated with difficult intubation were: history of difficult airway; previous head or neck radiotherapy treatment; presence of airway symptoms; presence of moderate or severe limited neck movement; and short interdental distance. The sensitivity, specificity and positive predictive values were: history of difficult airway 16.5%, 98.6% and 63.6%; previous radiotherapy 12.9%, 96.6% and 35.5%; airway symptoms 42.9%, 69.6% and 15.9%; moderate/severe neck limitation 16.7%, 97.2% and 46.7%; Mallampati score 3 or 4, 38.8%, 83.8% and 25.8%; and interdental distance 9.4%, 98.8% and 53.3%, respectively. The Bonfils intubation fibrescope was the most commonly used indirect laryngoscopy device (63.9% of all such cases). Twenty-six patients (3.2%) had complications during their initial airway management after induction of anaesthesia. There was one case of ‘cannot intubate, cannot oxygenate’, which required an emergency tracheostomy. Conclusion: The prevalence of difficult intubation in head and neck surgical patients was higher than in the general population, but predictive tests for difficult intubation have poor to moderate value. In our study, rates of difficult face mask ventilation, failed intubation and complications during induction and extubation were low. However, serious morbidity, although rare, can still be encountered. Head and neck surgical patients can be managed safely in a tertiary centre where there is appropriate surgical and anaesthetic expertise in managing difficult airways.

Anterior Retropharyngeal Reduction and Sequential Posterior Fusion for Atlantoaxial Rotatory Fixation With Locked C1-C2 Lateral Facet.

Retrospective case series. To introduce a new type of atlantoaxial rotatory fixation (AARF) with a locked C1-C2 lateral facet and evaluate its therapeutic strategy. AARF presenting with torticollis and limited neck motion is commonly seen in teenagers. Fielding and Hawkins' classification is generally applied to AARF. Although conservative treatment is effective for acute AARF, it is often unsuccessful for chronic AARF, which ultimately requires surgery. We introduce a new type of chronic AARF with a locked C1-C2 lateral facet that does not fit into Fielding and Hawkins' classification and describe the appropriate treatment. Eight patients who had chronic AARF with a unilaterally locked C1-C2 lateral facet were referred to our clinic. Reduction had failed after traction for 3 to 4 weeks. After open release and reduction using the anterior retropharyngeal approach, we applied posterior C1-C2 transpedicular screw fixation with an autologous iliac bone graft for stage I or II. The anterior retropharyngeal approach provided direct access to the C1-C2 locked lateral facet. The patient in an overall poor condition (stage II) underwent delayed posterior C1-C2 arthrodesis. The others (stage I) underwent immediate posterior C1-C2 arthrodesis. All patients were followed up for an average of 14.8 months (5-37 mo). Three-dimensional computed tomography revealed C1-C-2 arthrodesis bone graft fusion after an average of 3.1 months (2-4 mo). There was no recurrence of symptoms and no dislocations or internal fixation device loosening or breakage. AARF with a locked C1-C2 lateral facet is a new type of AARF that cannot be classified using Fielding and Hawkins' classification. The anterior retropharyngeal approach for the release and reduction of AARF, followed by posterior C1-C2 arthrodesis is an effective therapeutic strategy for AARF with a locked C1-C2 lateral facet. 4.

Chronic Obstructive Pulmonary Disease and Pulmonary Hypertension in a Patient With Klippel-Feil Syndrome: A Rare Association

SESSION TITLE: Airways Global Case Reports I SESSION TYPE: Global Case Report Poster PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM INTRODUCTION: Klippel-Feil syndrome (KFS) is a rare condition associated with multiple congenital anomalies. It's actual incidence is unknown. Nevertheless, it has been estimated that it occurs in approximately 1 in 40,000 to 42,000 newborns. Reported cases with pulmonary involvement includes agenesis and hypoplasia of the lung, pulmonary hypertension (PH) and bronchial asthma. To our knowledge, chronic obstructive pulmonary disease (COPD) with pulmonary hypertension in a patient with KFS has never been cited in the literature, hence we report the finding in a patient with such an association. CASE PRESENTATION: A 54-year-old obese Filipino male diagnosed with congenital heart disease (patent foramen ovale) and hypertension was admitted at the Philippine Heart Center due to progressive dyspnea and non productive cough for twelve days. He was a previous 30-pack year smoker. Physical examination revealed a short statured-individual with low hairline, short and webbing of the neck. He has evident chest wall deformity associated with thoracolumbar scoliosis. He was in cardio-respiratory distress. Auscultation of the chest showed bibasal crackles and expiratory wheezes. Arterial blood gas analysis showed an acute respiratory acidosis with adequate oxygenation, hence he required assisted mechanical ventilatory support. Chest radiograph showed mild pulmonary congestion with pneumonic infiltrates and magnified heart. Electrocardiogram showed nonspecific ST-T wave changes with right axis deviation. Troponin I was elevated. He was then started with acute coronary syndrome regimen, antibiotics and inhaled short-acting bronchodilator. Echocardiography showed dilated right ventricle with hypertrophy and dilated main pulmonary artery with mild PH (48 mmHg). In lieu with the acute renal failure, he was referred to a nephrologist. A request of kidney ultrasound showed absent right kidney. In line with the clinical features KFS a radiography of the spine showed fusion of C1 and basal output and multiple fusion of C2, C3 and C4 cervical vertebrae, hence confirming the diagnosis. Pulmonary function test showed very severe obstructive ventilatory defect with no significant response to bronchodilator, hence a diagnosis of severe COPD was made. He was successfully liberated from ventilatory support and discharged after 3 weeks of hospitalization with treatment of inhaled long-acting bronchodilator plus steroid. DISCUSSION: Klippel-Feil syndrome is a rare skeletal disorder primarily characterized by abnormal union of two or more cervical vertebrae. The triad of low hairline, short neck and limited neck movement which are present in the case remains to be the hallmark of the disorder. Moreover, involvement of visceral anomalies such as patent foramen ovale and right kidney agenesis merely defined the diagnosis. The association with COPD and PH is yet to be highlighted since no reports are available in the literature. Nevertheless, with the patient's history of chronic smoking provides a strong risk factor for the development of severe airflow obstruction. The presence of patent foramen ovale is rare in this case and poses no effect in the development of PH. The combined effects of hypoxic pulmonary vasoconstriction, inflammation and loss of pulmonary capillaries due to severe COPD could be the main insult. Furthermore, Suga et al explained the contributory effect of thoracic deformity in PH. This is due to hypoventilation and restriction of the pulmonary vascular bed causing reactive pulmonary arterial vasoconstriction. Treatment of patients with KFS is directed on associated anomalies or co-morbidities. In this case, management of acute respiratory failure due to complicating comorbidities was made. CONCLUSIONS: Klippel-Feil syndrome is a clinically well recognized disorder and commonly associated with many congenital malformations. Although rare, COPD and PH may occur with this syndrome. Clinicians should be directed towards detecting associated anomalies or co-morbidities for appropriate management. Reference #1: Klippel M, Feil A. “Un cas d'absence des vertebres cervicales. Avec cage thoracique remontant jusqu'a la base du crane (cage thoracique cervicale)”. Nouv Iconog Salpetriere 1912. 25: 223-250. Reference #2: Tracy M, Dormans JP,. Klippel-Feil syndrome: clinical features and current understanding of etiology. Clin Orthop Relat Res 2004; (424): 183-90 Reference #3: Suga K, Motoyama K, Hara A, Kume N et al. Respiratory failure and pulmonary hypertension associated with KFS. Annals of nuclear medicine 1999. 13(6): 441-446. DISCLOSURE: The following authors have nothing to disclose: Edgardo Tiglao, Teresita DeGuia, Maria Paz Mateo No Product/Research Disclosure Information

Is It Statin Induced Polymyalgia Rheumatica or Just a Coincidence?

©2015 Turkish League Against Rheumatism. All rights reserved. A 53-year-old female presented with pain in her bilateral shoulders, hips and neck, stiffness (lasting one hour), and proximal weakness for three months. She declared that her symptoms had ensued one week after the usage of daily 40 mg atorvastatin for hyperlipidemia. As such, atorvastatin was discontinued; however, her complaints did not decline. Physical examination revealed painful and restricted shoulder and neck movements, weakness in shoulder and hip muscles, and a swollen, warm right knee. Laboratory investigations including creatine kinase (CK), rheumatoid factor and anti-nuclear antibody levels were within normal values, except for the increased C-reactive protein (0.744 mg/dL, N: 0-0.5) and erythrocyte sedimentation rate (75 mm/h, N: 0-20). X-rays of the hand, knee, hip and shoulder were non-contributory. Ultrasound illustrated biceps tenosynovitis and trochanteric bursitis bilaterally, and increased suprapatellar fluid in right knee (Figure 1). Overall, the patient was diagnosed with polymyalgia rheumatica (PMR) according to the 2012 European League Against Rheumatism/American College of Rheumatology classification criteria.1 Her symptoms and clinical findings (erythrocyte sedimentation rate and C-reactive protein levels) responded to medium dose of prednisolone (20 mg/d) treatment dramatically. Although statins have been reported to be associated with some musculoskeletal side effects such as myalgia, myositis, myopathy, and elevated CK, statin induced PMR is a very rare condition which has been reported only in a few anecdotal reports.2-4 Likewise, statin treatment might trigger the development of PMR in our patient. Since statin induced PMR and myalgia have similar clinical features, the exact diagnosis can be challenging. Statin induced myalgia presents with/without increased CK levels with normal erythrocyte sedimentation rate and C-reactive protein levels. Therefore, patients with statin induced myalgia may have normal serum CK levels, which sometimes make it difficult to distinguish PMR from myopathy syndromes. In case of drug discontinuation, symptoms heal in a period of one-week to four-month in statin, myopathy and treatment of steroid is rarely required.5 PMR patients do not recover spontaneously and corticosteroid treatment is usually needed.6

A Clinical Comparative Study of Ultrasound-Normal Versus Ultrasound-Abnormal Congenital Muscular Torticollis

ObjectiveTo investigate the clinical features and outcome of outpatient-based physiotherapy (manual stretch) of congenital muscular torticollis (CMT) with passive neck motion limitation (≥10°) according to whether the finding on ultrasonography (US) is normal or abnormal. DesignCase-control study. SettingInstitutional practice. ParticipantsA total of 149 patients with CMT who met eligibility criteria were included: age at presentation ≤6 months, limitation of passive neck rotation (ΔROT) or lateral flexion (ΔLAT) ≥10°, and completion of our outpatient-based physiotherapy program. InterventionsPatients were allocated to the US-normal or US-abnormal group. Patients underwent physiotherapy and were followed-up monthly until ΔROT and ΔLAT were ≤5° or did not respond to treatment. Main Outcome MeasurementsBaseline characteristics, initial ΔROT and ΔLAT, age at presentation, treatment durations, and success rates of physiotherapy were compared between 2 groups. Treatment duration was adjusted for initial ΔROT, ΔLAT, and age at presentation using analysis of covariance. ResultsMean initial ΔROT and ΔLAT in US-abnormal (28.5°; 17.0°) were greater than in US-normal (7.9°, P < .001; 12.3°, P = .001, respectively). Mean age at presentation was older in US-normal (3.8 months) than in US-abnormal (1.8 months, P < .001). Treatment duration was shorter in the US-normal (5.1 weeks) than US-abnormal (14.9 weeks, P < .001). Adjusted treatment duration was also shorter in US-normal (9.7 weeks) than US-abnormal (13.8 weeks, P < .05). The success rates of physiotherapy were 95% in US-abnormal and 100% in US-normal. Two of 6 treatment failures in the US-abnormal group underwent surgery. ConclusionsIn CMT with passive neck motion limitation (≥10°), patients in the US-normal group demonstrated lesser passive neck motion limitation and older age at presentation than US-abnormal. It seems that US-normal showed shorter treatment duration irrespective of severity of neck motion limitation and age at presentation. Additionally, manual stretching applied before 6 months of age appears to show generally good outcome regardless of US findings.