Clinical Appropriateness Guidelines: Advanced Imaging

Total Page:16

File Type:pdf, Size:1020Kb

Clinical Appropriateness Guidelines: Advanced Imaging Clinical Appropriateness Guidelines: Advanced Imaging Imaging Program Guidelines: Pediatric Imaging Effective Date: February 20, 2017 Proprietary Guideline Last Revised Last Reviewed Administrative 07-26-2016 07-26-2016 Head and Neck 11-01-2016 11-01-2016 Chest 08-27-2015 07-26-2016 Abdomen and Pelvis 11-01-2016 11-01-2016 Spine 08-27-2015 07-26-2016 Extremity 08-27-2015 07-26-2016 ARCHIVED 8600 W Bryn Mawr Avenue South Tower – Suite 800 Chicago, IL 60631 P. 773.864.4600 Copyright © 2017. AIM Specialty Health. All Rights Reserved www.aimspecialtyhealth.com Table of Contents Description and Application of the Guidelines ........................................................................4 Administrative Guidelines ........................................................................................................5 Ordering of Multiple Studies ...................................................................................................................................5 Pre-test Requirements ...........................................................................................................................................6 Head & Neck Imaging ...............................................................................................................7 CT of the Head – Pediatrics ...................................................................................................................................7 MRI of the Head/Brain – Pediatrics ......................................................................................................................14 CTA/MRA Head: Cerebrovascular – Pediatrics ....................................................................................................21 Functional MRI (fMRI) Brain – Pediatrics .............................................................................................................24 PET Brain Imaging – Pediatrics ...........................................................................................................................25 CT Orbit, Sella Turcica, Posterior Fossa, Temporal Bone, including Mastoids – Pediatrics ................................26 CT Paranasal Sinus & Maxillofacial Area – Pediatrics .........................................................................................29 MRI Orbit, Face & Neck (Soft Tissues) – Pediatrics ............................................................................................32 MRI Temporomandibular Joint (TMJ) – Pediatrics ...............................................................................................36 CT Neck for Soft Tissue Evaluation – Pediatrics ..................................................................................................37 CTA/MRA of the Neck – Pediatrics .......................................................................................................................40 Chest Imaging .........................................................................................................................42 CT Chest – Pediatrics ..........................................................................................................................................42 CTA of the Chest (Non-Coronary) – Pediatrics ...................................................................................................47 MRI Chest – Pediatrics ........................................................................................................................................49 MRA of the Chest – Pediatrics .............................................................................................................................52 Abdominal & Pelvic Imaging ..................................................................................................54 CT Abdomen – Pediatrics .....................................................................................................................................54 MRI Abdomen – Pediatrics ...................................................................................................................................65 MR Cholangiopancreatography (MRCP) Abdomen – Pediatrics .........................................................................74 CTA and MRA of the Abdomen - Pediatrics..........................................................................................................76 CT Pelvis – Pediatrics ..........................................................................................................................................79 MRI Pelvis – PediatricsARCHIVED ........................................................................................................................................85 CTA and MRA of the Pelvis – Pediatrics ..............................................................................................................91 CT of the Abdomen & Pelvis Combination – Pediatrics .......................................................................................93 CTA of the Abdomen and Pelvis Combination – Pediatrics ................................................................................101 Table of Contents – Pediatrics | Copyright © 2017. AIM Specialty Health. All Rights Reserved. 2 Spine Imaging .......................................................................................................................103 CT Cervical Spine – Pediatrics ...........................................................................................................................103 MRI Cervical Spine – Pediatrics .........................................................................................................................106 CT Thoracic Spine – Pediatrics ..........................................................................................................................110 MRI Thoracic Spine – Pediatrics ........................................................................................................................113 CT Lumbar Spine – Pediatrics ...........................................................................................................................117 MRI Lumbar Spine – Pediatrics .........................................................................................................................120 MRA Spinal Canal – Pediatrics ..........................................................................................................................124 Extremity Imaging .................................................................................................................125 CT Upper Extremity – Pediatrics ........................................................................................................................125 MRI Upper Extremity (Any Joint) – Pediatrics ....................................................................................................128 MRI Upper Extremity (Non-Joint) – Pediatrics ...................................................................................................132 CTA and MRA Upper Extremity – Pediatrics ......................................................................................................135 CT Lower Extremity – Pediatrics ........................................................................................................................137 MRI Lower Extremity (Joint & Non-Joint) – Pediatrics .......................................................................................140 CTA and MRA Lower Extremity – Pediatrics ......................................................................................................147 ARCHIVED Table of Contents – Pediatrics | Copyright © 2017. AIM Specialty Health. All Rights Reserved. 3 Description and Application of the Guidelines AIM’s Clinical Appropriateness Guidelines (hereinafter “AIM’s Clinical Appropriateness Guidelines” or the “Guidelines”) are designed to assist providers in making the most appropriate treatment decision for a specific clinical condition for an individual. As used by AIM, the Guidelines establish objective and evidence-based, where possible, criteria for medical necessity determinations. In the process, multiple functions are accomplished: ● To establish criteria for when services are medically necessary ● To assist the practitioner as an educational tool ● To encourage standardization of medical practice patterns ● To curtail the performance of inappropriate and/or duplicate services ● To advocate for patient safety concerns ● To enhance the quality of healthcare ● To promote the most efficient and cost-effective use of services AIM’s guideline development process complies with applicable accreditation standards, including the requirement that the Guidelines be developed with involvement from appropriate providers with current clinical expertise relevant to the Guidelines under review and be based on the most up to date clinical principles and best practices. Relevant citations are included in the “References” section attached to each Guideline. AIM reviews all of its Guidelines at least annually. AIM makes its Guidelines publicly available on its website twenty-four hours a day, seven days a week. Copies of AIM’s Clinical Appropriateness Guidelines are also available upon oral or written request. Although the Guidelines are publicly-available, AIM considers the Guidelines to be important, proprietary information of AIM, which cannot be sold, assigned, leased, licensed, reproduced or distributed without the written consent
Recommended publications
  • Idiopathic Scoliosis
    Close (https://www.aetna.com/) Idiopathic Scoliosis Number: 0398 Policy History Policy I. Aetna considers surface electrical muscle stimulators Last Review 06/13/2017 (direct or alternating current, not high-voltage Effective: 05/04/2000 galvanic current) experimental and investigational for Next Review: 04/12/2018 the management of idiopathic scoliosis because there is inadequate evidence of its effectiveness and Review History safety in the peer-reviewed published medical literature. Definitions II. Aetna considers surgery (e.g., spinal fusion with instrumentation and bone grafting) for the treatment of idiopathic scoliosis medically necessary for any of Additional the following conditions: Information A. An increasing curve (greater than 40 degrees) in a Clinical Policy Bulletin growing child; or Notes B. Scoliosis related pain that is refractory to conservative treatments; or C. Severe deformity (curve greater than 50 degrees) with trunk asymmetry in children and adolescents; or D. Thoracic lordosis that can not be treated conservatively. Aetna considers idiopathic scoliosis surgery experimental and investigational when these criteria are not met. III. Aetna considers growing rods technique medically necessary in the treatment of idiopathic scoliosis for persons who meet criteria for surgery above. Please note this include the MAGEC System; but does not apply to other expandable magnetic growing rods (e.g., Phenix Growing Rod device) which are considered investigational and experimental. IV. Scoliosis braces and casts A. Aetna considers the following types of braces and casts medically necessary DME for the treatment of scoliosis: 1. Boston scoliosis brace 2. Charleston scoliosis brace 3. Milwaukee scoliosis brace 4. Providence brace 5. Rigo-Cheneau brace 6.
    [Show full text]
  • Saethre-Chotzen Syndrome
    Saethre-Chotzen syndrome Authors: Professor L. Clauser1 and Doctor M. Galié Creation Date: June 2002 Update: July 2004 Scientific Editor: Professor Raoul CM. Hennekam 1Department of craniomaxillofacial surgery, St. Anna Hospital and University, Corso Giovecca, 203, 44100 Ferrara, Italy. [email protected] Abstract Keywords Disease name and synonyms Excluded diseases Definition Prevalence Management including treatment Etiology Diagnostic methods Genetic counseling Antenatal diagnosis Unresolved questions References Abstract Saethre-Chotzen Syndrome (SCS) is an inherited craniosynostotic condition, with both premature fusion of cranial sutures (craniostenosis) and limb abnormalities. The most common clinical features, present in more than a third of patients, consist of coronal synostosis, brachycephaly, low frontal hairline, facial asymmetry, hypertelorism, broad halluces, and clinodactyly. The estimated birth incidence is 1/25,000 to 1/50,000 but because the phenotype can be very mild, the entity is likely to be underdiagnosed. SCS is inherited as an autosomal dominant trait with a high penetrance and variable expression. The TWIST gene located at chromosome 7p21-p22, is responsible for SCS and encodes a transcription factor regulating head mesenchyme cell development during cranial tube formation. Some patients with an overlapping SCS phenotype have mutations in the FGFR3 (fibroblast growth factor receptor 3) gene; especially the Pro250Arg mutation in FGFR3 (Muenke syndrome) can resemble SCS to a great extent. Significant intrafamilial
    [Show full text]
  • New Jersey Chapter American College of Physicians
    NEW JERSEY CHAPTER AMERICAN COLLEGE OF PHYSICIANS ASSOCIATES ABSTRACT COMPETITION 2015 SUBMISSIONS 2015 Resident/Fellow Abstracts 1 1. ID CATEGORY NAME ADDITIONAL PROGRAM ABSTRACT AUTHORS 2. 295 Clinical Abed, Kareem Viren Vankawala MD Atlanticare Intrapulmonary Arteriovenous Malformation causing Recurrent Cerebral Emboli Vignette FACC; Qi Sun MD Regional Medical Ischemic strokes are mainly due to cardioembolic occlusion of small vessels, as well as large vessel thromboemboli. We describe a Center case of intrapulmonary A-V shunt as the etiology of an acute ischemic event. A 63 year old male with a past history of (Dominik supraventricular tachycardia and recurrent deep vein thrombosis; who has been non-compliant on Rivaroxaban, presents with Zampino) pleuritic chest pain and was found to have a right lower lobe pulmonary embolus. The deep vein thrombosis and pulmonary embolus were not significant enough to warrant ultrasound-enhanced thrombolysis by Ekosonic EndoWave Infusion Catheter System, and the patient was subsequently restarted on Rivaroxaban and discharged. The patient presented five days later with left arm tightness and was found to have multiple areas of punctuate infarction of both cerebellar hemispheres, more confluent within the right frontal lobe. Of note he was compliant at this time with Rivaroxaban. The patient was started on unfractionated heparin drip and subsequently admitted. On admission, his vital signs showed a blood pressure of 138/93, heart rate 65 bpm, and respiratory rate 16. Cardiopulmonary examination revealed regular rate and rhythm, without murmurs, rubs or gallops and his lungs were clear to auscultation. Neurologic examination revealed intact cranial nerves, preserved strength in all extremities, mild dysmetria in the left upper extremity and an NIH score of 1.
    [Show full text]
  • Rectal Indomethacin Or I.V. Gabexate Mesylate As Prophylaxis for AP ERCP
    European Review for Medical and Pharmacological Sciences 2017; 21: 5268-5274 Rectal indomethacin or intravenous gabexate mesylate as prophylaxis for acute pancreatitis post-endoscopic retrograde cholangiopancreatography V. GUGLIELMI, M. TUTINO, V. GUERRA, P. GIORGIO National Institute of Gastroenterology, “S. de Bellis” Research Hospital Castellana Grotte, Bari, Italy Abstract. – OBJECTIVE: We aimed to evaluate mechanisms of AP are obstruction of the com- the results in our case series of AP ERCP over mon bile duct by stones, and alcohol abuse. En- the last three years. The prophylaxis for acute doscopic retrograde cholangiopancreatography pancreatitis (AP) post-endoscopic retrograde (ERCP) is the most frequent iatrogenic cause. cholangiopancreatography (ERCP) consists of rectal indomethacin, but some studies are not Post-ERCP AP is the most common and fear concordant. some adverse event for this procedure. In the PATIENTS AND METHODS: We compared 241 United States this complication costs $150 mil- ERCP performed from January 2014 to February lion annually for American Healthcare2,3 and it 2015 with intravenous gabexate mesylate (Group is a common cause of endoscopy-related lawsu- A), with the 387 ERCP performed from March its against gastroenterologists in the world. It 2015 to December 2016 with rectal indometha- has a significant morbidity and rare mortality cin (Group B) as prophylaxis for AP post-ERCP. RESULTS: There were 8 (3.31%) AP post-ERCP rate. About 10% of post-ERCP AP is moderate in Group A vs. 4 (1.03%) in Group B. or severe. Post-ERCP severe AP is associated CONCLUSIONS: Rectal indomethacin shows a with a higher mortality than non-ERCP-indu- better statistically significant performance than ced pancreatitis, but without statistical eviden- intravenous gabexate mesylate in the prophylax- ce4.
    [Show full text]
  • Oral Surgery Procedures in a Patient with Hajdu-Cheney Syndrome Treated with Denosumab—A Rare Case Report
    International Journal of Environmental Research and Public Health Article Oral Surgery Procedures in a Patient with Hajdu-Cheney Syndrome Treated with Denosumab—A Rare Case Report Magdalena Kaczoruk-Wieremczuk 1,†, Paulina Adamska 1,† , Łukasz Jan Adamski 1, Piotr Wychowa ´nski 2 , Barbara Alicja Jereczek-Fossa 3,4 and Anna Starzy ´nska 1,* 1 Department of Oral Surgery, Medical University of Gda´nsk,7 D˛ebinkiStreet, 80-211 Gda´nsk,Poland; [email protected] (M.K.-W.); [email protected] (P.A.); [email protected] (Ł.J.A.) 2 Department of Oral Surgery, Medical University of Warsaw, 6 St. Binieckiego Street, 02-097 Warsaw, Poland; [email protected] 3 Department of Oncology and Hemato-Oncology, University of Milan, 7 Festa del Perdono Street, 20-112 Milan, Italy; [email protected] 4 Division of Radiotherapy, IEO European Institute of Oncology, IRCCS, 435 Ripamonti Street, 20-141 Milan, Italy * Correspondence: [email protected] † Co-first author, these authors contributed equally to this work. Abstract: Background: Hajdu-Cheney syndrome (HCS) is a very rare autosomal-dominant congenital disease associated with mutations in the NOTCH2 gene. This disorder affects the connective tissue and is characterized by severe bone resorption. Hajdu-Cheney syndrome most frequently affects Citation: Kaczoruk-Wieremczuk, M.; the head and feet bones (acroosteolysis). Case report: We present an extremely rare case of a 34- Adamska, P.; Adamski, Ł.J.; Wychowa´nski,P.; Jereczek-Fossa, year-old male with Hajdu-Cheney syndrome. The patient was admitted to the Department of Oral B.A.; Starzy´nska,A. Oral Surgery Surgery, Medical University of Gda´nsk,in order to perform the extraction of three teeth.
    [Show full text]
  • Frey Operation for Chronic Pancreatitis Associated with Pancreas Divisum: Case Report and Review of the Literature
    Case report Frey operation for chronic pancreatitis associated with pancreas divisum: case report and review of the literature Magdalena Skórzewska1, Tomasz Romanowicz2, Jerzy Mielko1, Andrzej Kurylcio1, Jan Pertkiewicz3, Robert Zymon2, Wojciech P. Polkowski1 1Department of Surgical Oncology, Medical University of Lublin, Poland 2Department of General and Oncological Surgery, Pope John Paul II Regional Hospital, Zamosc, Poland 3Olympus Endotherapy, Warsaw, Poland Prz Gastroenterol 2014; 9 (3): 175–178 DOI: 10.5114/pg.2014.43581 Key words: pancreas divisum, chronic pancreatitis, surgery. Address for correspondence: Prof. Wojciech P. Polkowski MD, PhD, Department of Surgical Oncology, Medical University of Lublin, 11 Staszica St, 20-081 Lublin, Poland, phone: +48 81 534 43 13, fax: +48 81 532 23 95, e-mail: [email protected] Abstract Pancreas divisum (PD) is the most common congenital anomaly of the pancreas, which increases susceptibility to recurrent pancreatitis. Usually, after failure of initial endoscopic therapies, surgical treatment combining pancreatic resection or drainage is used. The Frey procedure is used for chronic pancreatitis, but it has not been reported to be applied in an adult patient with PD-associated pancreatitis. The purpose of the paper was to describe effective treatment of this rare condition by the Frey procedure after failure of interventional endoscopic treatment. A 39-year-old female patient was initially treated for recurrent acute pancreatitis. After endoscopic diagnosis of PD, the minor duodenal papilla was incised and a plastic stent was inserted into the dorsal pancreatic duct. During the following 36 months, the patient was hospitalised several times because of recur- rent episodes of pancreatitis. Thereafter, local resection of the pancreatic head combined with lateral pancreaticojejunostomy was performed with no complications.
    [Show full text]
  • A Gastric Duplication Cyst with an Accessory Pancreatic Lobe
    Turk J Gastroenterol 2014; 25 (Suppl.-1): 199-202 An unusual cause of recurrent pancreatitis: A gastric duplication cyst with an accessory pancreatic lobe xxxxxxxxxxxxxxx Aysel Türkvatan1, Ayşe Erden2, Mehmet Akif Türkoğlu3, Erdal Birol Bostancı3, Selçuk Dişibeyaz4, Erkan Parlak4 1Department of Radiology, Türkiye Yüksek İhtisas Hospital, Ankara, Turkey 2Department of Radiology, Ankara University Faculty of Medicine, Ankara, Turkey 3Department of Gastroenterological Surgery, Türkiye Yüksek İhtisas Hospital, Ankara, Turkey 4Department of Gastroenterology, Türkiye Yüksek İhtisas Hospital, Ankara, Turkey ABSTRACT Congenital anomalies of pancreas and its ductal drainage are uncommon but in general surgically correctable causes of recurrent pancreatitis. A gastric duplication cyst communicated with an accessory pancreatic lobe is an extremely rare cause of recurrent pancreatitis, but an early and accurate diagnosis of this anomaly is important because suitable surgical treatment may lead to a satisfactory outcome. Herein, we presented multidetector com- puted tomography and magnetic resonance imaging findings of a gastric duplication cyst communicating with an accessory pancreatic lobe via an aberrant duct in a 29-year-old woman with recurrent acute pancreatitis and also reviewed other similar cases reported in the literature. Keywords: Aberrant pancreatic duct, accessory pancreatic lobe, acute pancreatitis, gastric duplication cyst, multi- detector computed tomography, magnetic resonance imaging INTRODUCTION Herein, we presented multidetector CT and MRI find- Report Case Congenital causes of recurrent pancreatitis include ings of a gastric duplication cyst communicating with anomalies of the biliary or pancreatic ducts, espe- an accessory pancreatic lobe via an aberrant duct in a cially pancreas divisum. A gastric duplication cyst 29-year-old woman with recurrent acute pancreatitis communicating with an aberrant pancreatic duct is and also reviewed other similar cases reported in the an extremely rare but curable cause of recurrent pan- literature.
    [Show full text]
  • Intraductal Papillary Mucinous Carcinoma of The
    Nishi et al. BMC Gastroenterology (2015) 15:78 DOI 10.1186/s12876-015-0313-3 CASE REPORT Open Access Intraductal papillary mucinous carcinoma of the pancreas associated with pancreas divisum: a case report and review of the literature Takeshi Nishi1,2*, Yasunari Kawabata2, Noriyoshi Ishikawa3, Asuka Araki3, Seiji Yano2, Riruke Maruyama3 and Yoshitsugu Tajima2 Abstract Background: Pancreas divisum, the most common congenital anomaly of the pancreas, is caused by failure of the fusion of the ventral and dorsal pancreatic duct systems during embryological development. Although various pancreatic tumors can occur in patients with pancreas divisum, intraductal papillary mucinous neoplasm is rare. Case presentation: A 77-year-old woman was referred to our hospital because she was incidentally found to have a cystic tumor in her pancreas at a regular health checkup. Contrast-enhanced abdominal computed tomography images demonstrated a cystic tumor in the head of the pancreas measuring 40 mm in diameter with slightly enhancing mural nodules within the cyst. Endoscopic retrograde pancreatography via the major duodenal papilla revealed a cystic tumor and a slightly dilated main pancreaticductwithanabruptinterruptionattheheadofthe pancreas. The orifice of the major duodenal papilla was remarkably dilated and filled with an abundant extrusion of mucin, and the diagnosis based on pancreatic juice cytology was “highly suspicious for adenocarcinoma”. Magnetic resonance cholangiopancreatography depicted a normal, non-dilated dorsal pancreatic duct throughout the pancreas. The patient underwent a pylorus-preserving pancreaticoduodenectomy under the diagnosis of intraductal papillary mucinous neoplasm with suspicion of malignancy arising in the ventral part of the pancreas divisum. A pancreatography via the major and minor duodenal papillae on the surgical specimen revealed that the ventral and dorsal pancreatic ducts were not connected, and the tumor originated in the ventral duct, i.e., the Wirsung’s duct.
    [Show full text]
  • Albany Med Conditions and Treatments
    Albany Med Conditions Revised 3/28/2018 and Treatments - Pediatric Pediatric Allergy and Immunology Conditions Treated Services Offered Visit Web Page Allergic rhinitis Allergen immunotherapy Anaphylaxis Bee sting testing Asthma Drug allergy testing Bee/venom sensitivity Drug desensitization Chronic sinusitis Environmental allergen skin testing Contact dermatitis Exhaled nitric oxide measurement Drug allergies Food skin testing Eczema Immunoglobulin therapy management Eosinophilic esophagitis Latex skin testing Food allergies Local anesthetic skin testing Non-HIV immune deficiency disorders Nasal endoscopy Urticaria/angioedema Newborn immune screening evaluation Oral food and drug challenges Other specialty drug testing Patch testing Penicillin skin testing Pulmonary function testing Pediatric Bariatric Surgery Conditions Treated Services Offered Visit Web Page Diabetes Gastric restrictive procedures Heart disease risk Laparoscopic surgery Hypertension Malabsorptive procedures Restrictions in physical activities, such as walking Open surgery Sleep apnea Pre-assesment Pediatric Cardiothoracic Surgery Conditions Treated Services Offered Visit Web Page Aortic valve stenosis Atrial septal defect repair Atrial septal defect (ASD Cardiac catheterization Cardiomyopathies Coarctation of the aorta repair Coarctation of the aorta Congenital heart surgery Congenital obstructed vessels and valves Fetal echocardiography Fetal dysrhythmias Hypoplastic left heart repair Patent ductus arteriosus Patent ductus arteriosus ligation Pulmonary artery stenosis
    [Show full text]
  • Update on Scoliosis
    Update on Evaluation and Treatment of Scoliosis Ron El-Hawary, MD, MSc, FRCS(C)*, Chukwudi Chukwunyerenwa, MD, MCh, FRCS(C) KEYWORDS Scoliosis Bracing Surgery VEPTR KEY POINTS Scoliosis can arise from a variety of causes and is defined as a lateral curvature of the spine greater than 10. The most common cause of scoliosis is idiopathic, which accounts for up to 80% of scoli- osis in children. The Adam’s forward bending test is a clinical evaluation of axial plane rotation that is asso- ciated with scoliosis. The goal of treatment is to prevent curve progression. If a curve progresses beyond 50,it will likely continue to progress into adulthood. For children with early onset scoliosis, the goal of treatment is also to maintain spine, chest, and pulmonary development throughout childhood. INTRODUCTION Scoliosis can arise from a variety of causes and is defined as a lateral curvature of the spine greater than 10 on an anterior-posterior standing radiograph (Fig. 1). However, in reality, it is a 3-dimensional structural deformity that includes a curvature in the anterior-posterior plane, angulation in the sagittal plane, and rotation in the transverse plane. This 3-dimensional deformity differentiates scoliosis from nonstructural spine deformities, which arise as compensation for abnormalities in other regions (eg, lower limb disorders resulting in limb length discrepancy), in which case the deformity is mono-planer and resolves when the primary abnormality is treated. IDIOPATHIC SCOLIOSIS The most common cause of scoliosis is idiopathic, which accounts for up to 80% of scoliosis in children.1 The cause of idiopathic scoliosis is unknown and is a diagnosis Disclosures: Consulting Depuy-Synthes Spine, Medtronic Canada, Halifax Biomedical Inc, research/educational support Depuy-Synthes Spine, Medtronic Canada (R.
    [Show full text]
  • The Filum Disease and the Neuro-Cranio-Vertebral Syndrome
    Royo-Salvador et al. BMC Neurology (2020) 20:175 https://doi.org/10.1186/s12883-020-01743-y RESEARCH ARTICLE Open Access The Filum disease and the Neuro-Cranio- vertebral syndrome: definition, clinical picture and imaging features Miguel B. Royo-Salvador1*, Marco V. Fiallos-Rivera1, Horia C. Salca1 and Gabriel Ollé-Fortuny2 Abstract Background: We propose two new concepts, the Filum Disease (FD) and the Neuro-cranio-vertebral syndrome (NCVS), that group together conditions thus far considered idiopathic, such as Arnold-Chiari Syndrome Type I (ACSI), Idiopathic Syringomyelia (ISM), Idiopathic Scoliosis (IS), Basilar Impression (BI), Platybasia (PTB) Retroflexed Odontoid (RO) and Brainstem Kinking (BSK). Method: We describe the symptomatology, the clinical course and the neurological signs of the new nosological entities as well as the changes visible on imaging studies in a series of 373 patients. Results: Our series included 72% women with a mean age of 33.66 years; 48% of the patients had an interval from onset to diagnosis longer than 10 years and 64% had a progressive clinical course. The commonest symptoms were: headache 84%, lumbosacral pain 72%, cervical pain 72%, balance alteration 72% and paresthesias 70%. The commonest neurological signs were: altered deep tendon reflexes in upper extremities 86%, altered deep tendon reflexes in lower extremities 82%, altered plantar reflexes 73%, decreased grip strength 70%, altered sensibility to temperature 69%, altered abdominal reflexes 68%, positive Mingazzini’s test 66%, altered sensibility to touch 65% and deviation of the uvula and/or tongue 64%. The imaging features most often seen were: altered position of cerebellar tonsils 93%, low-lying Conus medullaris below the T12L1 disc 88%, idiopathic scoliosis 76%, multiple disc disease 72% and syringomyelic cavities 52%.
    [Show full text]
  • Cervical Medullary Syndrome Secondary to Craniocervical
    Neurosurgical Review https://doi.org/10.1007/s10143-018-01070-4 ORIGINAL ARTICLE Cervical medullary syndrome secondary to craniocervical instability and ventral brainstem compression in hereditary hypermobility connective tissue disorders: 5-year follow-up after craniocervical reduction, fusion, and stabilization Fraser C. Henderson Sr1,2 & C. A. Francomano1 & M. Koby1 & K. Tuchman2 & J. Adcock3 & S. Patel4 Received: 10 October 2018 /Revised: 28 November 2018 /Accepted: 10 December 2018 # The Author(s) 2019 Abstract A great deal of literature has drawn attention to the Bcomplex Chiari,^ wherein the presence of instability or ventral brainstem compression prompts consideration for addressing both concerns at the time of surgery. This report addresses the clinical and radiological features and surgical outcomes in a consecutive series of subjects with hereditary connective tissue disorders (HCTD) and Chiari malformation. In 2011 and 2012, 22 consecutive patients with cervical medullary syndrome and geneticist-confirmed hereditary connective tissue disorder (HCTD), with Chiari malformation (type 1 or 0) and kyphotic clivo-axial angle (CXA) enrolled in the IRB-approved study (IRB# 10-036-06: GBMC). Two subjects were excluded on the basis of previous cranio-spinal fusion or unrelated medical issues. Symptoms, patient satisfaction, and work status were assessed by a third-party questionnaire, pain by visual analog scale (0–10/10), neurologic exams by neurosurgeon, function by Karnofsky performance scale (KPS). Pre- and post-operative radiological measurements of clivo-axial angle (CXA), the Grabb-Mapstone- Oakes measurement, and Harris measurements were made independently by neuroradiologist, with pre- and post-operative imaging (MRI and CT), 10/20 with weight-bearing, flexion, and extension MRI.
    [Show full text]