Botulinum Toxin Injections

Jurisdiction: JE Part B. States: California, Hawaii, Nevada, American Samoa, Guam, Northern Mariana Islands. Type: Active LCD

General Indications and Limitations of Coverage Botulinum toxin dosing must be used in accordance with the United States Food and Drug Administration (FDA) approved labeling. For off-label botulinum toxin use without an FDA approved dosing indication, the qualified healthcare professional must provide robust published clinical evidence to support the dosing use. For off-label botulinum toxin use without an FDA approved serotype indication, the qualified healthcare professional must provide robust published clinical evidence to support the serotype use. For new FDA approved indications for botulinum toxin serotype or dosing recommendations after the effective date of this policy, the qualified healthcare professional must provide the updated published FDA prescribing information to support the use of the botulinum toxin serotype or dose. Botulinum toxin administration must not be given more frequently than every 12 weeks, regardless of diagnosis, unless specifically addressed in the policy. The use of botulinum toxin for all cosmetic procedures is not a covered benefit under Medicare; AND When botulinum toxin is used for an approved diagnosis, but the botulinum is also being used with cosmetic intent, the entire claim for the botulinum is not considered reasonable and necessary, AND The potency of each of the botulinum toxin serotypes are not interchangeable with other botulinum toxin serotypes and, therefore, units of biological activity of any 1 serotype cannot be compared to or converted into units of any other botulinum toxin serotype; AND Botulinum toxin injections (BTIs) are not considered reasonable and necessary for patients with a contraindication to botulinum toxin; AND BTIs are not considered reasonable and necessary for patients with existing medical conditions which could affect the neuromuscular function; AND BTIs are not considered reasonable and necessary for patients with hypersensitivity to any botulinum toxin preparation or to any of the components in the formulation of the serotype; AND BTIs are not considered reasonable and necessary for patients with severe clotting disorders; AND BTIs are not considered reasonable and necessary for patients with an infection at the injection site; AND Both conscious sedation and monitored anesthesia care (MAC) are not medically necessary for BTIs; AND When conservative treatment needs to be provided prior to the administration of botulinum toxin, a vague or nonspecific physician statement that conservative treatment measures were completed is not sufficient. The documentation must provide specific information regarding the conservative treatments which were used and an objective assessment of the intolerance or inadequacy of the response to the conservative measures. Image guidance is not considered reasonable and necessary for injection of botulinum toxin. Medicare will allow payment for 1 injection per site regardless of the number of injections made into the site. A site is defined as 1 area. Specific Indications and Limitations of Coverage by Diagnosis Achalasia Definition: Achalasia is a relatively rare primary motor esophageal disorder, characterized by incomplete relaxation of the esophageal gastric junction (EGJ) coupled with the absence of organized peristalsis along the esophageal body. Three achalasia subtypes have been defined based on the high-resolution manometry (HRM) findings in the esophageal body: type I or classic achalasia with low intraesophageal pressure, type II with pan-esophageal pressurization, and type III with high-amplitude spastic contractions. Diagnosis: The diagnostic criterion for achalasia is evaluated by HRM, which is recognized as the gold standard for diagnosis. 1,2 HRM measures the integrated relaxation pressure (IRP) at the EGJ, with an IRP greater than 15 mmHg serving as a critical diagnostic marker for achalasia. The Chicago Classification system enables the categorization of achalasia into 3 distinct subtypes based on the IRP and esophageal pressurization patterns observed during swallowing. Each subtype has specific clinical implications and guides the management strategy, with type II achalasia generally associated with the most favorable treatment outcomes. 1,2 Treatment: BTI is an FDA off-label use consisting of endoscopic injections of the toxin into 4 quadrants of the lower esophageal sphincter (LES). The treatment options in achalasia patients aim to improve symptoms by reducing the functional obstruction at the level of the gastroesophageal junction. Injection of botulinum toxin reduces LES pressure by inhibiting release of acetylcholine from nerve endings. 3-9 Indications of Coverage Initial Botulinum Toxin Injections In the management of achalasia, BTI is an FDA off-label use consisting of endoscopic injections of the toxin into 4 quadrants of the LES. 3-9 Initial BTIs for achalasia will be considered reasonable and necessary when the following requirements are met: Objective documentation of the clinical features consistent with the diagnosis of achalasia; AND Chronic achalasia measured on objective clinical scale*; AND Medically high-risk patients diagnosed with achalasia who cannot undergo other invasive treatments (peroral endoscopic myotomy (POEM), Heller myotomy, pneumatic dilation [PD]); 1,2,8,10-15 OR As a bridge for those patients diagnosed with achalasia awaiting more effective treatments such as Heller myotomy, PDs or POEM; 2 OR During work-up and treatment planning of definitive treatments for achalasia. 16,17 * The objective assessment must be performed and documented at baseline, after each diagnostic procedure, and at each follow-up assessment using the same scale during each assessment. There are several clinical scales to measure severity of achalasia, for example the Eckert Scale. Initial Dosing Guidelines The initial dose of onabotulinumtoxinA is 80 to 100 Units. Subsequent Botulinum Toxin Injections Subsequent BTIs for achalasia will be considered reasonable and necessary when the following requirements are met: Documentation of informed clinical decisions regarding repeat botulinum toxin injections; AND Reassessment of the degree of persistent moderate to severe achalasia; AND There is evidence of a significant beneficial symptomatic response to the initial dose. Subsequent Dosing Guidelines The subsequent dose of up to 100 Units onabotulinumtoxinA may be given 30 days after the initial dose. Limitations of Coverage BTIs are not considered reasonable and necessary for: The patient who has achalasia symptoms but insufficient manometric criteria to make the diagnosis. Patients with contraindications for upper endoscopy. 18 Injection of botulinum toxin in the esophageal body. 1,2,8,13 Initial or subsequent onabotulinumtoxinA doses above 100 Units. 1,2 Anal Fissure Definition: A linear tear in the anal mucosa typically extending into the internal anal sphincter. Anal fissures that persist for more than 6 weeks are classified as chronic. The internal sphincter spasm is believed to be the main etiology for the pathogenesis of chronic anal fissure. 19 Diagnosis: Anal fissures are characterized by a longitudinal linear tear primarily caused by trauma or irritation from constipation or diarrhea. 20 Fissures are commonly located in the posterior midline. Variations in location, such as anterior midline or atypical lateral positions, necessitate a thorough evaluation due to potential underlying conditions. 20 Chronic fissures exhibit additional features such as a hypertrophied anal papilla, a sentinel tag, and possibly exposed internal anal sphincter muscle, indicative of underlying issues like sphincter hypertonicity and impaired wound healing. This distinction is crucial for diagnosis and treatment, as chronic fissures may display additional physical features such as a hypertrophied anal papilla at the proximal edge of the fissure, a sentinel tag (or skin tag) at the distal edge, and exposure of the internal anal sphincter muscle at the base of the fissure, indicating a more complex condition requiring specialized management strategies. 20 Indications of Coverage Initial Botulinum Toxin Injections Initial BTIs for anal fissure will be considered reasonable and necessary when the following requirements are met: The anal fissure has been present for more than 6 weeks; AND Conservative treatment has been tried for eliminating constipation and reducing anal sphincter spasm. Initial Dosing Guidelines The initial treatment with onabotulinumtoxinA dosing for anal fissure is 20 Units (10 Units on each side of the fissure). 21,22 Subsequent Botulinum Toxin Injections Subsequent BTIs for anal fissure will be considered reasonable and necessary when the following requirements are met: Documentation for informed clinical decision-making regarding repeat botulinum toxin injections and surgical treatment; AND Reassessment of the symptoms and degree of persistent anal fissure. Subsequent Dosing Guidelines A subsequent dose of onabotulinumtoxinA for anal fissure is up to 60 Units with the clinical trials demonstrating greater efficacy with lower doses. 20 Blepharospasm Definition: A focal dystonia involving the involuntary closure of the eyelids and spasms of the orbicularis oculi muscles characterized by sustained or intermittent muscle contractions resulting in mild blinking or sustained forced closure of the eyes. Diagnosis: To diagnose a patient with blepharospasm, clinicians should observe for involuntary contraction of the orbicularis oculi and other muscles involved in eyelid closure, which can range from sporadic and mildly irritating to functionally blinding. 23 The disorder often begins with infrequent bilateral eyelid twitching and may progress to forceful and frequent spasms, including symptoms like eyelid apraxia. Sensory stimulation such as touching the eyelids or certain activities may temporarily reduce the contractions. Diagnosis is primarily clinical and considered a diagnosis of exclusion, with imaging or laboratory studies typically not indicated. The diagnostic criteria for blepharospasm have been proposed, with key features being bilateral spasms of the orbicularis oculi and nearby muscles of the upper face often with excessive blinking. 23 Clinicians may encounter patients presenting with associated symptoms such as anxiety, depression, or ocular surface diseases like blepharitis. 4 Differential diagnosis includes distinguishing from conditions like Meige syndrome, myokymia, hemifacial spasm, and others based on the symmetry, distribution, and accompanying symptoms. Photophobia and response to sensory triggers are common, and the patient's history of triggers and symptom progression is essential for accurate diagnosis. To accurately diagnose blepharospasm without inadvertently diagnosing Meige Syndrome, it's essential to focus on the primary symptom of involuntary eyelid closure, ensuring it is bilateral, synchronous, and stereotyped, without the presence of significant lower facial or neck muscle involvement. 23 Despite this focal blepharospasm being the second most common type of dystonia, a high percentage of individuals given this diagnosis had dystonia outside of the eye/upper face region 5 which makes the diagnosis inconsistent with existing guidelines for the diagnosis and classification of focal blepharospasm. These authors proposed that the diagnosis of focal blepharospasm be based on dystonic spasms of muscles in the upper face around the eyes only. The most sensitive findings to diagnose blepharospasm is a stereotypical bilateral and synchronous orbicularis oculi muscle spasms inducing eyelid narrowing closure, the presence of a sensory trick (the sensory trick was defined as any kind of maneuver performed by the patient that led to a transient reduction in spasm severity in the period of time immediately after its execution 24 ), and increased blinking. 4 Blepharospasm can be classified into idiopathic, acquired, and inherited subtypes. 25 The rating instruments in the medical literature have coalesced into several main clinical scales, including the Jankovic Rating Scale (JRS) and Blepharospasm Disability Index (BSDI). 26 Indications of Coverage Initial Botulinum Toxin Injections Initial BTIs for blepharospasm will be considered reasonable and necessary when the following requirements are met: Objective documentation of the clinical features consistent with the diagnosis of blepharospasm; AND Chronic blepharospasm of at least 30 days duration measured using an objective clinical scale*; AND BTI therapy is accepted first line treatment for patients with blepharospasm. * The objective assessment must be performed and documented at baseline, after each diagnostic procedure, and at each follow-up assessment using the same scale during each assessment. For example, there are several clinical scales to measure severity of blepharospasm, including the JRS and BSDI. Initial Dosing Guidelines The initial treatment with onabotulinumtoxinA dosing for blepharospasm associated with dystonia is 1.25 Units-2.5 Units into each of 3 sites per affected facial or ocular muscle. 27 Subsequent Botulinum Toxin Injections Subsequent BTIs for blepharospasm will be considered reasonable and necessary when the following requirements are met: Documentation of informed clinical decisions regarding repeat botulinum toxin injections; AND Reassessment of the severity and frequency of persistent blepharospasm; AND Persistence or reoccurrence of blepharospasm; AND The initial treatment is considered sufficient, and administration of same dose is recommended; OR The initial treatment is considered insufficient (defined as an effect which does not last longer than 2 months) and administration of an increased dose is recommended. Based upon the initial response, the dosage may be increased up to 5 units per site. Subsequent Dosing Guidelines The subsequent treatment with incobotulinumtoxinA dosing is based on the previous dosing with onabotulinumtoxinA. If previously treated with onabotulinumtoxinA, if not known or not previously treated with onabotulinumtoxinA, the incobotulinumtoxinA starting dose is 1.25 Units-2.5 Units per injection site. 28 For onabotulinumtoxinA in blepharospasm, subsequent doses may be increased up to two-fold if the initial response is insufficient. However, injecting more than 5 units per facial or ocular muscle site provides little additional benefit. Clinical circumstances that necessitate an exception of OnabotulinumtoxinA dose up to 10 units per site may be considered on an individual basis and the rationale for the higher dose must be clearly documented in the medical record. Blepharospasm Associated with Orofacial Dystonia Definition A rare neurological movement disorder characterized by involuntary and often forceful contractions of the muscles of the jaw and tongue (oromandibular dystonia) and involuntary muscle spasms and contractions of the muscles around the eyes (blepharospasm) is known as Meige Syndrome. 29 Overall, half of all blepharospasm subjects experience spread over a period of 5 years. 30 Diagnosis The syndrome is clinically suspected by the clinical symptoms of eyelid spasms accompanied by jaw clenching or mouth opening, grimacing, and/or tongue movement. This condition must be objectively diagnosed by a clinical scale such as the Craniocervical Dystonia Questionnaire. Indications of Coverage Initial Botulinum Toxin Injections Initial BTIs for blepharospasm associated with orofacial dystonia will be considered reasonable and necessary when the following requirements are met: Objective documentation of the clinical features consistent with the diagnosis of blepharospasm associated with orofacial dystonia; AND Moderate to severe chronic blepharospasm associated with orofacial dystonia measured on objective clinical scale*, AND OnabotulinumtoxinA injection therapy is accepted first line treatment for patients with blepharospasm associated with orofacial dystonia. * The objective assessment must be performed and documented at baseline, after each diagnostic procedure, and at each follow-up assessment using the same scale during each assessment. There are several clinical scales to measure severity of blepharospasm associated with orofacial dystonia, including the Burke-Fahn-Marsden scale (BFMS), the Global Dystonia Severity Rating scale (GDRS), Craniocervical Dystonia Questionnaire, and the JRS. Initial Dosing Guidelines There are no standard dosing recommendations of onabotulinumtoxinA for orofacial dystonia which is typically administered into masseters, temporalis, medial pterygoids, submentalis, lateral pterygoids, platysma, genioglossus, and hyoglossus. 31 Subsequent Botulinum Toxin Injections Subsequent BTIs for associated with orofacial dystonia will be considered reasonable and necessary when the following requirements are met: Documentation of informed clinical decision regarding repeat botulinum injections; AND Reassessment of persistent blepharospasm associated with orofacial dystonia. Subsequent Dosing Guidelines The number of injections sites and muscles injected is determined by the response to initial injections. When the initial treatment is considered sufficient, and administration of same dose is recommended, OR When the initial treatment or subsequent treatments are considered insufficient (defined as an effect which does not last longer than 2-months) administration of an increased dose is recommended. Cervical Dystonia (CD) Definition: Cervical dystonia (CD), also known as spasmodic torticollis, is a rare neurological disorder caused by an impairment of the central nervous system 32 with an estimated prevalence range from 5 to 30 cases per 100,000 individuals. 33,34 The disorder is characterized by upper motor neuron velocity-dependent hypertonic skeletal muscles, the inability to relax the muscles and characteristic intermittent or sustained muscle contractions causing abnormal and frequent repetitive movements and posture 32 of the cervical and/or shoulder muscles. 35 There are 2 groups of CD. The first type of CD is idiopathic or primary CD which is believed to be due to genetic or sporadic onset. The patients with primary CD have no evidence by history, physical examination or laboratory studies (except primary dystonia gene) of any secondary cause for the dystonic symptoms. CD is a part of either generalized or focal dystonic syndrome which may have a genetic basis, with an identifiable genetic association. The second type of CD is acquired or secondary CD (or sometimes described as “symptomatic” CD). Secondary CD may be caused by central or peripheral trauma, exposure to dopamine receptor antagonists (tardive CD), neurodegenerative disease, and other conditions associated with abnormal functioning of the basal ganglia. 36 There is controversy as to the development of post-traumatic CD 37 and the contribution of peripheral acute trauma to causing idiopathic dystonia is negligible. CD is not the only cause of neck rotation and torticollis may be caused by orthopedic, musculofibrotic, infectious and other neurological conditions that affect the anatomy of the neck, and structural causes need to be assessed. 36 The accuracy of the diagnosis is critical. There was a pivotal trial for the FDA approval of botulinum toxin for CD. 36 Diagnosis CD is clinically suspected but the condition is not simply a manifestation of focal muscle pain, muscle spasm, cervical movements and cervical posture. 38 This clinical diagnosis must be associated with excessive pulling of the muscles of the neck and shoulder. These dystonic movements, which may be twisting, sustained, jerking, or tremulous, are the hallmark symptoms. The abnormal postures or movements, often exacerbated by voluntary actions, can lead to significant neck pain or discomfort. The presence of such symptoms, alongside the use of specific maneuvers like gestes antagonistes, which can temporarily alleviate dystonic postures confirms the clinical diagnosis. The diagnostic criteria emphasize the importance of these involuntary movements' characteristics and their impact on the patient's quality of life. 38 This condition must be objectively measured by a clinical scale. Several scales have been recommended by medical literature. Scales that are used to evaluate CD include but are not limited to the Fahn-Marsden Scale, Unified Dystonia Rating Scale, Columbia Torticollis Rating Scale, Tsui Scale, Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS), and Cervical Dystonia Rating Scale. The TWSTRS scale is the most widely utilized rating scale for CD. 39 Some authors have indicated that the rating scales (such as the Tsui score and the TWSTRS) used at present have not been rigorously tested for responsiveness to detect significant changes in clinical status after therapeutic interventions. 39 Botulinum toxin is used to reduce the severity of the abnormal head positions and neck pain associated with CD. Indications of Coverage Initial Botulinum Toxin Injections Initial BTIs for CD will be considered reasonable and necessary when the following requirements are met: The documentation supports a diagnosis of CD; AND The etiology of the central nervous system impairment which is causing the CD is documented, AND Beneficiary has a history of recurrent clonic or tonic involuntary contractions of 1 or more of the following muscles: sternocleidomastoid, splenius, trapezius and/or posterior cervical muscles; AND There is moderate to severe CD assessed by an objective scale*; AND There are objective measurements of abnormal posturing, with limited range of motion in the neck, or sustained head tilt; AND The duration of the CD is greater than 6 months; AND The initial onabotulinumtoxinA dose is based on the patient’s head and neck position, localization of pain, muscle hypertrophy, patient response, and adverse event history; use lower initial dose in botulinum toxin naïve patients. 27 * The objective assessment must be performed and documented at baseline, after each diagnostic procedure, and at each follow-up assessment using the same scale during each assessment. Some clinical scales include Tsui score and the TWSTRS. Initial Dosing Guidelines The initial dose of abobotulinumtoxinA is up to 500 Units given intramuscularly as a divided dose among the affected cervical muscles 40 ; The initial dose of rimabotulinumtoxinB is up to a total dosage of 2,500 Units to 5,000 Units divided among affected cervical muscles. 41 The initial dose of incobotulinumtoxinA is up to 120 Units divided among affected cervical muscles per treatment session. 28 The initial dose of daxibotulinumtoxinA-lanm is 125 Units to 250 Units given intramuscularly as a divided dose among affected muscles. 330 Subsequent Botulinum Toxin Injections Subsequent BTIs for CD will be considered reasonable and necessary when the following requirements are met: Beneficiary is requesting subsequent injections; AND Response to initial treatment is documented in the medical records; AND Reassessment of clinical utility of the injection with documentation of informed clinical decision regarding repeat botulinum injections. Subsequent Dosing Guidelines The subsequent onabotulinumtoxinA dosing for CD is based on the clinical response, head and neck position, localization of pain, and muscle hypertrophy. The subsequent abobotulinumtoxinA doses are administered between 250 and 1000 Units to optimize clinical benefit and titrated in 250 Units in incremental steps according to patient’s response. 40 The subsequent dose of rimabotulinumtoxinB is up to a total dosage of 2,500 Units to 5,000 Units divided among effected cervical muscles. 41 The subsequent dose of IncobotulinumtoxinA is 120 Units per treatment session. 28 The subsequent doses of daxibotulinumtoxinA-lanm can be adjusted in 50 to 75 Unit increments according to the individual patient’s response. The total dose administered in a single treatment is between 125 Units and 250 Units. 330 Limitations of Coverage: It is not reasonable and necessary for multiple procedures (e.g., epidural injections, sympathetic blocks, trigger point injections, etc.) to be provided to a beneficiary on the same day as the BTI. BTIs are not medically necessary for generalized pain conditions (such as fibromyalgia), chronic nonspecific neck pain with or without cervical rotation, or chronic centralized pain syndromes, temporomandibular disorders with or without neck pain, severe bruxism with or without neck pain, myofascial pain syndrome, and cervical spondylosis with or without neck pain. Chronic Migraine Definition: Chronic migraine is a recurrent chronic headache disorder with 2 major types described as either episodic migraines or chronic migraines based on the frequency of the headaches. Patients presenting with headaches should be evaluated to determine whether their headache is a primary or a secondary headache disorder. Migraine headaches can present with or without aura. Diagnosis: 42,43 Migraine without aura is a clinical syndrome characterized by the following diagnostic criteria: At least 5 attacks fulfilling criteria b.-d. Headache attacks lasting 4-72 hours (untreated or unsuccessfully treated) Headache has at least 2 of the following 4 characteristics: Unilateral location Pulsating quality Moderate or severe pain intensity Aggravation by or causing avoidance of routine physical activity (e.g., walking or climbing stairs) During headache at least 1 of the following: Nausea and/or vomiting Photophobia and Phonophobia Not better accounted for by another International Classification of Headache Disorders, Third Edition (ICHD-3) diagnosis. Migraine with aura is a clinical syndrome characterized by the following diagnostic criteria: At least 2 attacks fulfilling criteria b. and c. One or more of the following fully reversible aura symptoms: Visual Sensory Speech and/or language Motor Brainstem Retinal At least 3 of the following 6 characteristics: At least 1 aura symptom spreads gradually over ≥5 minutes Two or more aura symptoms occur in succession. Each individual aura symptom lasts 5-60 minutes. At least 1 aura symptom is unilateral. At least 1 aura symptom is positive. The aura is accompanied, or followed within 60 minutes, by headache. Not better accounted for by another ICHD-3 diagnosis. Migraine Headache Prophylaxis: The botulinum toxin, onabotulinumtoxinA, is beneficial for the prophylaxis of chronic migraine headaches based upon FDA approval, a few randomized controlled clinical trials, published practice guidelines, and professional society evidence reviews. The reporting of headaches is often clinically based on the patient’s historical recall of the past headache frequency, intensity and duration. There is a potential for recall bias when patients retrospectively report the past headaches and some studies have shown the recall has been “reasonably accurate” 44 with the headache intensity appearing to be more difficult to remember and report than headache frequency and duration. Additional studies have indicated that recall questionnaires administered on a monthly basis may underestimate headache frequency compared to headache diaries. 45 Daily prospective headache diaries may reduce recall bias and increase the reliability of patient’s descriptions of migraine headache characteristics. 46 The use of the standardized questionnaires has shown moderately high test-retest reliability between the questionnaire and the headache diaries. 46 van der Meer endorsed that more research is needed to enhance the level of evidence for existing measurement instruments for multiple headaches. 47 Indications of Coverage Initial Botulinum Toxin Injections Initial BTIs for chronic migraine prophylaxis will be considered reasonable and necessary when the following requirements are met: The BTI is being used only for the indication of chronic migraine prophylaxis, AND The monthly headache days have occurred ≥15headaches days per month, AND The monthly migraine headache days have occurred ≥ 8 migraine headache days per month, AND The migraine headaches are documented to be lasting for a duration ≥4 hours on a migraine day, AND The chronic headaches have been present for a period of at least 3 months, AND The beneficiary has had a trial of and inadequate response to a 2-month trial of at least 1 agent in any 2 of the following classes or has contraindication to the following medications; AND Antidepressant class: amitriptyline, venlafaxine, nortriptyline, duloxetine; OR Beta blocker class: metoprolol, propranolol, timolol (oral), nadolol, atenolol, nebivolol; OR Calcium channel blocker class: verapamil; OR Antiepileptic class: valproate sodium, divalproex sodium, topiramate, gabapentin. The 2-month trial of the oral pharmacologic classes above for chronic migraine prophylaxis must have been at the target dose of the usual effective dose or an intolerance to the 2 agents in each class, AND If the beneficiary also currently using a calcitonin gene-related peptide (CGRP) agent for chronic migraine prophylaxis and is going to be using CGRP and botulinum toxin together the following must apply: The beneficiary had a reduction in the overall number of migraine days or reduction in number of severe migraine days per month with CGRP use, but still has chronic migraines requiring additional therapy for chronic migraine prevention; AND The headaches are causing an objective significant functional disability, AND The headaches are moderate to severe intensity with typical migraine headache characteristics; AND The botulinum toxin serotype is approved by the FDA for chronic migraine prophylaxis. The objective assessment must be performed and documented at baseline, after each diagnostic procedure, and at each follow-up assessment using the same scale during each assessment. Initial Dosing Guidelines The onabotulinumtoxinA dose ranges between 155-195 Units. The initial onabotulinumtoxinA dose is 155 Units given as 5 Units per each site divided across 7 head/neck muscles (frontalis, corrugator, procerus, occipitalis, temporalis, trapezius, and cervical paraspinal muscle group). 27 Subsequent Botulinum Toxin Injections Clinicians must be aware and address the importance of cognitive-affective processes in headache disorders and address the potential for hypervigilance to bodily sensations and anxiety regarding the symptoms of headache pain when managing chronic migraine headaches. Subsequent BTIs for migraine prophylaxis will be considered reasonable and necessary when the following requirements are met: The number of the total chronic migraine headache days have demonstrated ≥50% reduction in migraine headache days per month; AND The frequency of the total chronic migraine headache episodes has demonstrated ≥50% reduction in migraine headache episodes per month; AND There is a minimal important change (MIC) and significant reduction of headache-related disability and objective improvement in functioning; AND Biobehavioral therapy (cognitive behavioral therapy, biofeedback, relaxation therapies, mindfulness-based therapies, acceptance and commitment therapy) has been assessed and implemented as appropriate for preventive and acute headache treatment; AND If the beneficiary is using concurrently with a CGRP agent for migraine prophylaxis, the beneficiary has had further reduction in the overall number of migraine days or reduction in number of severe migraine days per month compared to monotherapy with the initial agent (either botulinum toxin or the CGRP agent); AND The botulinum toxin serotype is approved by the FDA for chronic migraine prophylaxis. Subsequent Dosing Guidelines The subsequent onabotulinumtoxinA dose ranges between 155-195 Units to allow a discretionary 40 Units to be administered using a “follow-the-pain” strategy, resulting in 195 Units onabotulinumtoxinA over 39 sites. 27 Limitations of Coverage It is not reasonable and necessary for multiple procedures (e.g., epidural injections, sympathetic blocks, trigger point injections, etc.) to be provided to a beneficiary on the same day as the BTI. BTIs are not medically necessary for generalized pain conditions (such as fibromyalgia) or chronic centralized pain syndromes, episodic migraine prophylaxis, temporomandibular disorders with or without headaches, tension headaches, severe bruxism with or without headache, chronic daily headaches, myofascial pain syndrome with or without headaches and cervical spondylosis with or without headaches. Initial or subsequent onabotulinumtoxinA doses above 195 Units. Focal Hand Dystonia(FHD) Definition: Focal hand dystonia (FHD) is a disabling task-specific movement disorder, which can generalize across tasks and be present at rest. It is also referred to as writer’s cramp, musician’s dystonia, and other occupational hand dystonias. Diagnosis: FHD is a rare primary hand dystonia. The diagnosis of FHDs includes neuropathies, myopathies, myotonias, radiculopathies, plexopathies, complex regional pain syndrome, repetitive stress injury, focal seizures, thoracic outlet syndrome, medications effects, and a psychogenic movement disorder. The patient’s history and clinical examination are both critical for its diagnosis and for identification of dystonic muscles. Since FHDs are uncommon, the use of botulinum toxin for the treatment of a FHD should be infrequent. BTI therapy dosing in FHD is highly individualized due to different activity types, levels, multiple potential target muscles with varying BTI doses and intervals. 2 Currently, no effective alternative medical or proven surgical therapies have been established for FHD. 42,43,48-52 Indications of Coverage Initial Botulinum Toxin Injections BTI therapy is an accepted FDA off-label use first line treatment for patients with FHDs. In the management of FHD, BTI is considered reasonable and necessary consisting of focal injections of the toxin into the muscles responsible for the abnormal postures, and initial BTIs for FHD will be considered reasonable and necessary when the following requirements are met: Objective documentation of the clinical features consistent with the diagnosis of FHD; AND Moderate to severe chronic FHDs measured on objective clinical scale*; AND The injections are used with guidance either by ultrasound or by electromyography (EMG) with or without electrostimulation. * The objective assessment must be performed and documented at baseline, after each diagnostic procedure, and at each follow-up assessment using the same scale during each assessment. For example, scales that could be used include the Fahn-Marsden rating scale, Unified Dystonia Rating Scale, Arm Dystonia Disability Scale (ADDS); Tubiana-Chamagne Scale; and Writer's Cramp Rating Scale. Initial Dosing Guidelines The appropriate dosing range of onabotulinumtoxinA depends on the site of muscle injection. Clinician must assess the specific pattern of dystonic postures and movements. 53 For flexor muscles (flexor digitorum superficialis: 25-50 Units, flexor pollicis longus: 5-20 Units, flexor digitorum profundus: 20-60 Units, flexor carpi radialis: 20-50 Units, flexor carpi ulnaris: 15-60 Units). For extensor muscles (extensor digitorum communis: 10-25 Units, extensor pollicis longus: 5-20 Units, extensor indicis: 5-10 Units, extensor carpi radialis longus: 5-20 Units, extensor carpi ulnaris: 5-20 Units). Subsequent Botulinum Toxin Injections Subsequent BTIs for FHD will be considered reasonable and necessary when all the following requirements are met: Documentation of informed clinical decision regarding repeat botulinum injections; AND Reassessment of the degree of persistent FHD; AND The initial treatment is considered sufficient, and administration of same dose is recommended; OR The initial treatment is considered insufficient (defined as an effect which does not last longer than 2 months) and administration of an increased dose is recommended. Subsequent Dosing Guidelines Appropriate subsequent dosage of onabotulinumtoxinA range depends on the site of muscle injection. Clinician must assess the specific pattern of dystonic postures and movements. 53 For flexor muscles (flexor digitorum superficialis: 25-50 Units, flexor pollicis longus: 5-20 Units, flexor digitorum profundus: 20-60 Units, flexor carpi radialis: 20-50 Units, flexor carpi ulnaris: 15-60 Units). For extensor muscles (extensor digitorum communis: 10-25 Units, extensor pollicis longus: 5-20 Units, extensor indicis: 5-10 Units, extensor carpi radialis longus: 5-20 Units, extensor carpi ulnaris: 5-20 Units). Hemifacial Spasm/Facial Dystonia Definition: Hemifacial spasm (HFS) is a chronic neurological disorder characterized by usually unilateral hyperkinetic movements with short or persistent, intermittent synchronous twitching of the muscles innervated by the facial nerve and spontaneous recovery is rare. The etiology of the disorder is the seventh cranial nerve. HFS is classified as primary facial nerve damage (79%) or secondary facial nerve damage (21%). 54 Most cases of hemifacial spasm are idiopathic and probably caused by vascular compression of the facial nerve, other etiologies should be considered in the differential diagnosis, particularly if there are atypical features. 55 Diagnosis: HFS is characterized by involuntary contractions of the facial muscles innervated by the seventh cranial nerve. An accurate diagnosis is important as many conditions can mimic HFS. The diagnosis of this condition is made clinically based on the detailed history coupled with a neurological and local physical examination. Electrophysiological testing may not be required to make the diagnosis, but an electromyogram can be used in the early stages of the disease when it is difficult to distinguish clinically from facial myokymia, blepharospasm, complex partial motor seizures, or motor tics. The diagnostic finding on electrophysiological testing is lateral spread and variable synkinesis on blink reflex testing. Brain magnetic resonance imaging may be needed to determine if there is a suspected facial nerve compression at the brainstem nerve root exit zone or near the cerebellopontine angle. 56 Similarly, facial dystonia is a movement disorder characterized by sustained or intermittent muscle contractions causing abnormal, often repetitive movements, postures, or both involving the facial muscle(s). Diagnosis is made clinically but will often require neuroimaging and for certain other etiologies, may require additional laboratory testing. Indications of Coverage Initial Botulinum Toxin Injections Initial BTIs for HFS will be considered reasonable and necessary when all the following requirements are met: BTI therapy is accepted as first line treatment for patients with primary or secondary HFS; AND Objective documentation of the clinical features consistent with the diagnosis of primary or secondary HFS; AND Moderate to severe primary or secondary HFS measured on objective clinical scale to measure severity, complexity and psychosocial aspects of HFS.* * The objective assessment must be performed and documented at baseline, after each diagnostic procedure, and at each follow-up assessment using the same scale during each assessment. The selection of the clinical scale is not defined but the selected scale should provide a comprehensive and sensitive rating tool with both clinical and subjective parameters for HFS to enable a standardized assessment of HFS and treatment outcome. 26,57 Initial Dosing Guidelines The administration of a total dose 25-30 Units of onabotulinumtoxinA into the orbicularis oculi, procerus, mentalis, platysma, orbicularis oris and depressor anguli oris on the side of the face affected by the HFS. 27 Subsequent Botulinum Toxin Injections Subsequent BTIs for HFS will be considered reasonable and necessary when all the following requirements are met: Documentation of informed clinical decision regarding repeat botulinum injections; AND Reassessment of the degree of persistent moderate to severe HFS. Subsequent Dosing Guidelines A gradual increase in the number of onabotulinumtoxinA Units (5-15 additional Units) after 1 year, may be required. Hyperhidrosis Definition: Primary hyperhidrosis is defined as excessive, uncontrollable sweating without any discernible cause and commonly involves the axillae, palms, and soles. Secondary hyperhidrosis is caused by an underlying medical condition or due to taking certain medications, such as pain relievers, antidepressants, diabetes medications, and hormonal medications. Severely affected patients have skin maceration and secondary microbial infections. Diagnosis: Hyperhidrosis is characterized by excessive sweating beyond thermoregulatory needs. 58-60 This condition is classified into mild, moderate and severe forms based on the quantity of sweating and its impact on patients’ lives. 58-60 Those with mild hyperhidrosis experience uncomfortable yet tolerable sweating that does not require changes of clothing. Moderate hyperhidrosis involves visible sweat droplets but no need for multiple clothing changes per day. However, patients with severe hyperhidrosis undergo profuse, uncontrollable sweating necessitating multiple clothing changes daily, causing significant disruption of professional, social and personal activities. 58-60 Accurately diagnosing hyperhidrosis requires qualitative and quantitative assessments. Qualitative methods like visual inspection and patient reporting assess localization and situational triggers of sweating through examining clothing sweat stains and patient narratives. More precise quantitative techniques like gravimetric testing directly measure sweat production but can be impractical for routine diagnosis. Minor’s starch-iodine test localizes affected areas but does not quantify sweat volumes. 58-60 Critically, quality-of-life questionnaires like the Hyperhidrosis Disease Severity Scale evaluate functional life impairment across professional, social and psychological domains. Comprehensive assessment combining patient-reported symptoms, visual observations, sweat quantification and impact evaluation is key for confirming hyperhidrosis and determining optimal, personalized treatment approaches tailored to the severity. 58-60 Indications of Coverage Initial Botulinum Toxin Injections Initial BTIs for primary axillary hyperhidrosis will be considered reasonable and necessary when the following requirements are met: Beneficiary has a diagnosis of primary or secondary axillary hyperhidrosis; AND Excessive sweating in the axilla lasting 6 months or more; AND Bilateral symmetric sweating in the axilla; AND Cessation of focal sweating while asleep; AND The failure of a 6-month trial to respond to other noninvasive conservative management for axillary hyperhidrosis (systemic anticholinergics, tranquilizers, topical dermatologics such as aluminum chloride, tannic acid, or glutaraldehyde, or non-steroid anti-inflammatory drugs); AND Severe chronic hyperhidrosis measured on objective clinical scale*; AND There is severe chronic axillary hyperhidrosis manifested by medical complications or skin maceration with secondary infection; AND There is severe chronic axillary hyperhidrosis associated with and impairment of daily activities; AND Significant functional impairment due to the hyperhidrosis * The objective assessment must be performed and documented at baseline, after each diagnostic procedure, and at each follow-up assessment using the same scale during each assessment, such as the Hyperhidrosis Disease Severity Scale (HDSS). 61,62 Initial Dosing Guidelines The initial administration of onabotulinumtoxinA is up to a total 50 Units per axilla. 27 Subsequent Botulinum Toxin Injections Subsequent BTIs for primary axillary hyperhidrosis will be considered reasonable and necessary when the following requirements are met: Documentation of informed clinical decision regarding repeat botulinum toxin injections; AND Reassessment of the degree of persistent hyperhidrosis and assessment of previous response to botulinum toxin; AND Severe chronic hyperhidrosis measured on objective clinical scale*; AND Retreatment no greater than once every 6 months. Subsequent Dosing Guidelines The subsequent dose of onabotulinumtoxinA is 50-75 Units per axilla. Limitations of Coverage The safety and effectiveness of onabotulinumtoxinA for hyperhidrosis in body areas other than axillary are not considered to be reasonable and necessary. Secondary axillary hyperhidrosis due to endocrine, neurologic, medication adverse effects, infection or malignancy is not considered to be reasonable and necessary. Previous surgical debulking of the axillary sweat glands make the administration of botulinum toxin not reasonable and necessary. Initial administration of onabotulinumtoxinA over 50 Units or subsequent doses over 75 Units. Laryngeal Dystonia (Spasmodic Dysphonia) Definition: Laryngeal spasmodic dysphonia, a type of laryngeal dystonia (LD), is a task-specific focal dystonia involving the laryngeal muscles characterized by sustained or intermittent muscle contractions and due to abnormal neuronal function at several potential sites along the neuroaxis from the motor cortex, supplementary motor areas, brainstem, putamen, globus pallidus, cerebellum, and other potential locations in the basal ganglia. Diagnosis: The diagnostic criteria for laryngeal spasmodic dysphonia include the evaluation of voice quality changes such as alterations in pitch, loudness, or vocal effort that impair communication or reduce the quality of life. 63 A thorough history and physical examination are crucial to identify underlying causes of dysphonia and to differentiate laryngeal spasmodic dysphonia from other voice disorders. Laryngoscopy is recommended when dysphonia fails to resolve or improve within 4 weeks or when a serious underlying cause is suspected, irrespective of the duration of symptoms. 63 Laryngyeal dystonia (spasmodic dysphonia) typically presents as adductor type, Adductor Spasmodic Dysphonia (ADSD), 90% versus the less common abductor type, Abductor Spasmodic Dysphonia (ABSD). Studies show onaboutlinum toxinA to be an effective treatment option for ADSD, however, there is currently inadequate evidence to support the effectiveness of botulinum toxin injections for the ABSD variant. Consequently, consistent with evidence-based guidelines, coverage has been extended for the off-label coverage for botulinum toxin for ADSD. Indications of Coverage Initial Botulinum Toxin Injections Initial BTIs for LD will be considered reasonable and necessary when the following requirements are met: Objective documentation of the clinical features consistent with the diagnosis of adductor type, ADSD; AND Objective assessment and documentation to rule out non-organic voice disorders; AND Moderate to severe chronic ADSD measured on objective clinical scale*; 64 AND BTI therapy is an accepted FDA off-label use first line treatment for patients with ADSD. * The objective assessment must be performed and documented at baseline, after each diagnostic procedure, and at each follow-up assessment using the same scale during each assessment. For example, the Voice Handicap Index (VHI) and the Vocal Performance Questionnaire (VPQ). Initial Dosing Guidelines The initial injection of onabotulinumtoxinA takes place at two separate sites of the thyroarytenoid (TA) and/or lateral cricoarytenoid (LCA) muscle on one side to a total dose between 1.5 to 3.5 Units per site. 65 Subsequent Botulinum Toxin Injections Subsequent BTIs for LD will be considered reasonable and necessary when the following requirements are met: Documentation of informed clinical decision regarding repeat botulinum toxin injections; AND Reassessment of the degree of persistent LD and assessment of previous response to botulinum toxin. Subsequent Dosing Guidelines A second injection of onabotulinumtoxinA in contralateral side at separate time (typically 2 to 4 weeks later); titrate to response for patients who require bilateral injections for ADSD. Subsequent injections are usually given 12-week intervals (a maximum of 7 Units). Limitations of Coverage: Medicare will allow payment for one injection per site regardless of the number of injections made into the site. A site is defined as one area (including all the muscles around the vocal cords). Bilateral TA and LCA injections for ADSD will not be considered reasonable and necessary when administered concurrently. Neurogenic Bladder Definition: The term neurogenic lower urinary tract dysfunction (NLUTD) is usually referred to as a neurogenic bladder which is the loss of normal bladder function caused by damage to part of the nervous system. The neurogenic bladder can be classified as an uninhibited bladder, upper motor neuron bladder, or lower motor neuron bladder. The voiding dysfunction may cause urinary incontinence (UI) which may be due to neurogenic detrusor overactivity (and possibly associated with detrusor sphincter dyssynergia for neurologic lesions below the pontine micturition center). The uninhibited bladder and upper motor neuron bladder commonly occur after a neurologic injury to the central nervous system such as spinal cord injuries (SCI), cerebral vascular accident (CVA) or multiple sclerosis (MS). Diagnosis: The diagnostic criteria for NLUTD involve a detailed assessment of the patient's neurologic condition, urinary symptoms, and urodynamic findings. 66 A NLUTD is diagnosed and risk-stratified by objective testing such as post-void residual (PVR), urinary tract imaging, and urodynamics. Clinicians should first evaluate the patient's neurological status and history of neurological diseases, such as SCI, MS, or CVAs, which are commonly associated with NLUTD. The presence of lower urinary tract symptoms (LUTS), including UI, urinary retention,