Series Editor: Arthur T. Skarin, MD, FACP, FCCP

Systemic cancer can affect the central nervous system in several different ways, including direct tumor metastasis and indirect remote effects. Intracranial metastasis can involve the skull, dura, and leptomeninges (arachnoid and pia mater), as well as the brain parenchyma. Of these, parenchymal brain metastases are the most common and have been found in as many as 24% of cancer patients in autopsy studies. It has been reported that metastatic brain tumors outnumber primary brain tumors 10 to 1.

To read the full article in PDF:

Click here

Series Editor: Arthur T. Skarin, MD, FACP, FCCP

Systemic cancer can affect the central nervous system in several different ways, including direct tumor metastasis and indirect remote effects. Intracranial metastasis can involve the skull, dura, and leptomeninges (arachnoid and pia mater), as well as the brain parenchyma. Of these, parenchymal brain metastases are the most common and have been found in as many as 24% of cancer patients in autopsy studies. It has been reported that metastatic brain tumors outnumber primary brain tumors 10 to 1.

To read the full article in PDF:

Click here

Series Editor: Arthur T. Skarin, MD, FACP, FCCP

Systemic cancer can affect the central nervous system in several different ways, including direct tumor metastasis and indirect remote effects. Intracranial metastasis can involve the skull, dura, and leptomeninges (arachnoid and pia mater), as well as the brain parenchyma. Of these, parenchymal brain metastases are the most common and have been found in as many as 24% of cancer patients in autopsy studies. It has been reported that metastatic brain tumors outnumber primary brain tumors 10 to 1.

To read the full article in PDF:

Click here

Snoring can range in significance from disturbing a bed partner to being a symptom of obstructive sleep apnea, a risk factor for cardiac disease and stroke. Snoring that is unrelated to obstructive sleep apnea may respond to a combination of nonsurgical treatments. However, if the problem persists despite conservative therapy, then surgical options may be considered.

This article explores why people snore, provides guidance for evaluating it and ruling out obstructive sleep apnea, and describes the available surgical treatments. Snoring associated with obstructive sleep apnea requires a different surgical treatment strategy that is beyond the scope of this article.

WHY PEOPLE SNORE

Humans go through four stages of sleep in each sleep cycle (and four or five cycles per night), and each stage has unique physiologic characteristics. As we progress deeper into sleep with each successive stage, the skeletal muscles of the body relax and eventually become atonic, except for the respiratory and ocular muscles. Soft tissues of the upper aerodigestive tract also lose their muscular tone.

Snoring is an undesirable vibratory sound that originates from the soft tissues of the upper respiratory tract during sleep, as airflow causes the relaxed tissues to vibrate.

The upper airway can be obstructed by the nasal septum, inferior nasal turbinates, adenoids, tonsils, uvula, soft palate, and base of the tongue—and often by more than one (Figure 1).³ In rare cases, obstruction can occur at the level of the larynx, such as from a tumor, laryngomalacia, or a laryngeal defect.

A SPECTRUM OF SLEEP-DISORDERED BREATHING

The American Academy of Sleep Medicine’s International Classification of Sleep Disorders¹ defines a number of sleep disorders. In clinical practice, the first-line diagnostic test for sleep disorders is polysomnography.

Snoring is only one sign of sleep-disordered breathing; others are excessive daytime somnolence, restless sleep, and witnessed apnea.

Considerable evidence links obstructive sleep apnea with serious medical problems including hypertension, coronary artery disease, heart failure, cardiac arrhythmia, and stroke.² Others include mood disorders, decreased libido, and cognitive impairment, with changes in attention, concentration, executive function, and fine-motor coordination.⁴ Therefore, ruling out obstructive sleep apnea is essential before pursuing interventions for primary snoring, although both disorders may warrant surgery.

PATIENT HISTORY

Most patients who present to the office because of snoring have snored for many years. Many seek medical attention at the request of a long-suffering bed partner.

Associated symptoms in primary snoring may include mouth breathing, chronic nasal congestion, and morning dry throat. Witnessed apnea, frequent awakenings during sleep, restless sleep, daytime somnolence, frequents naps, and memory impairment may be signs of more significant sleep-disordered breathing, such as obstructive sleep apnea.

The Epworth sleepiness scale may help quantify the severity of daytime somnolence.⁵ It is measured in a short questionnaire in which the patient indicates, on a scale of 0 to 3, his or her likelihood of dozing in a variety of situations.

The STOP-BANG questionnaire consists of eight yes-no questions:

• Snore: Have you been told that you snore?
• Tired: Are you often tired during the day?
• Obstruction: Do you know if you stop breathing, or has anyone witnessed you stop breathing while you are asleep?
• Pressure: Do you have high blood pressure or are you on medication to control high blood pressure?
• Body mass index: Is your body mass index higher than 35 kg/m²?
• Age: Are you age 50 or older?
• Neck: Do you have a neck circumference greater than 17 inches (men) or greater than 16 inches (women)?
• Gender: Are you male?

A score of three or higher has shown a sensitivity of 93% for detecting moderate obstructive sleep apnea and 100% for severe obstructive sleep apnea.⁶

SEARCHING FOR ANATOMIC CAUSES OF SNORING

A thorough physical examination should be done, focusing on potential anatomic causes of snoring. Nasal septal deviation or inferior turbinate hypertrophy with mucosal congestion may contribute to chronic mouth breathing secondary to nasal obstruction. Patients with a body mass index over 35 kg/m² and neck circumference over 17 inches (16 inches in women) are at higher risk of obstructive sleep apnea.

Indirect mirror examination or flexible transnasal endoscopy may reveal obstructing or persistent adenoid lymphoid tissue, particularly in young adults. Transnasal endoscopy may also reveal dynamic collapse of the palate and lateral oropharyngeal wall or fullness of the tongue base with subsequent narrowing of the oropharynx.

Examination of the oral cavity may reveal a disproportionately large tongue, a narrow opening into the oropharynx, or tonsillar hypertrophy. The Friedman classification (Figure 2), also called the modified Mallampati scale, can be used to describe the findings on physical examination of the palate and tongue in a systematic way. There are four grades of increasing severity, and the higher the grade, the less likely that surgery will succeed in patients with obstructive sleep apnea.⁷ The mouth is examined with the tongue in a relaxed position; in contrast, the original Mallampati classification, which is often used by anesthesiologists in assessing the oral airway, is assessed with the tongue protruding.

During flexible endoscopy, asking the patient to attempt to recreate the snoring can sometimes reveal the causative anatomic structure, which is usually the soft palate.

SLEEP STUDIES

A full diagnostic workup should include a sleep study if obstructive sleep apnea cannot be ruled out by the history and examination. Sleep studies include either polysomnography in a sleep laboratory or a home sleep test. They allow the clinician to further evaluate the severity of sleep-disordered breathing and to distinguish primary snoring from obstructive sleep apnea. This is particularly important if elective surgical intervention is planned. Sleep studies can also be used to evaluate for other sleep disorders.

Apnea is considered obstructive when polysomnography reveals episodes of no oral or nasal airflow with continued inspiratory effort, evidenced by abdominal or thoracic muscle activity. Hypopnea is defined as a 30% or greater reduction in airflow lasting at least 10 seconds, with an associated 4% or greater oxygen desaturation.¹ The combined number of apnea and hypopnea events per hour, or apnea-hypopnea index, is used clinically to quantify the severity of sleep-disordered breathing.

Primary snoring is diagnosed if the apnea-hypopnea index is 5 or less. Obstructive sleep apnea is considered mild when the apnea-hypopnea index is greater than 5 but less than 15, moderate from 15 to 30, and severe if over 30.

LIMITED ROLE FOR IMAGING

Cephalometric radiography (plain radiography of the airways) has limited value in the workup of primary snoring and is discouraged. Imaging is most useful in assessing craniofacial skeletal abnormalities. Lateral airway images can help in diagnosing adenoid hypertrophy in children. However, flexible nasopharyngoscopy can obtain this information by direct visualization with no radiation exposure.

Computed tomography and magnetic resonance imaging are seldom used in the workup of snoring because they do little to guide therapeutic intervention, are expensive, and, in the case of computed tomography, expose the patient to unnecessary radiation. Imaging does a have a role when planning surgical intervention of obstruction that involves the maxillofacial skeleton.

NONSURGICAL MANAGEMENT

The primary goal of therapy for snoring is to eliminate or reduce noise levels.

Although no study to date has analyzed the efficacy of nonsurgical management, several treatments are aimed at the root causes of snoring in an attempt to decrease it.

Intranasal topical steroids reduce inflammation of the nasal mucosa that occurs with allergic and nonallergic rhinitis, thereby opening up the nasal airway. They may reduce snoring in a small number of cases. These drugs must often be used in the long term to maintain their efficacy.

Devices. Other than continuous positive airway pressure (CPAP), the only currently available nonsurgical device approved by the US Food and Drug Administration for the treatment of snoring and obstructive sleep apnea is an oral dental appliance, which is customized to the patient’s dentition to relieve upper-airway obstruction by soft tissues of the oral cavity. The lower jaw is forced anteriorly, pulling the tongue and attached soft tissues forward. Custom-fitted oral appliances are an effective option for mild to moderate sleep apnea and associated snoring, and are more effective than thermoplastic “boil-and-bite” devices.⁸ These can easily be used in patients who have primary snoring.

Over-the-counter remedies such as nasal strips and head-positioning pillows have not been shown to be efficacious for snoring.⁹

Weight loss. Patients should be encouraged to join a weight-management program if overweight.

Sleep on the side, not on the back. Changing the sleep position may be useful in patients who have positional symptoms. Snoring is often worse in the supine position because gravity acting on the palate and tongue causes narrowing of the airway. “Positional therapy” employs devices to force patients to sleep in a lateral decubitus position to counter the effects of gravity.

Alcohol cessation. Alcohol has a relaxing effect on the muscles of the upper-respiratory tract, and abstaining from alcohol may therefore reduce snoring.

INDICATIONS FOR SURGERY

Surgery can decrease the noise level of snoring and thus bring relief for the patient’s bed partner.

Assessment of the upper airway may suggest the appropriate treatment, depending on whether the patient has nasal obstruction, adenoid hypertrophy, or palatal movement. A sleep study, if not previously done, should be done before surgery to rule out obstructive sleep apnea.

Many patients opt for surgery after noninvasive forms of treatment have proven ineffective or difficult to tolerate. When medical therapy for snoring has been unsuccessful, a discussion of the benefits, risks, and alternatives to surgery must take place between the patient and the surgeon.

SURGICAL PROCEDURES

Septoplasty

Septoplasty—straightening the nasal septum to improve the nasal airway—is an outpatient procedure. Although a deviated septum alone is not often the sole cause of snoring, most otolaryngologists agree that the septum should be addressed before or concomitantly with any palatal surgery for sleep-disordered breathing.

Nasal congestion often comes from a deviated bony or cartilaginous septum, enlarged turbinates, or bone spurs. Septal deviation may be developmental or the result of trauma to the nose.

Complications of septoplasty are rare but include septal perforation, scar-band formation, septal hematoma, epistaxis, and infection.

Radiofrequency ablation of the inferior turbinates

Hypertrophy of the inferior turbinate is the most common cause of nasal obstruction, followed by structural deformity of the nasal airway by septal deviation.³ Many patients report fixed or fluctuating nasal congestion and chronic mouth-breathing. The causes of turbinate congestion or enlargement include allergic rhinitis, upper-respiratory infection, and chronic rhinitis. In most cases, turbinate hypertrophy occurs at the level of the submucosa.

Radiofrequency ablation uses radiofrequency energy to generate heat at approximately 85°C (185°F) to create finely controlled coagulative lesions. The lesions are naturally resorbed in 3 to 8 weeks, inducing fibrosis, reducing excess tissue volume, and thus opening the airway. The procedure can be repeated several times to achieve optimal results. Radiofrequency ablation can also be used to reduce anatomic obstruction in other parts of the airway, such as the soft palate and the base of tongue.

Submucosal radiofrequency ablation of the inferior turbinate is a simple office-based procedure. It is often combined with septoplasty to optimize the nasal airway.

Mild to moderate edema with subsequent nasal obstruction and thick mucus formation can be expected the first week after the procedure. The risk of postoperative bleeding and infection is low. When performed with septoplasty, there is a low risk that scar tissue, or synechiae, may form between the turbinate and the septum.

Radiofrequency ablation of the palate

The soft palate is the most common anatomic source of snoring, and radiofrequency ablation can be applied to it as well. As with radiofrequency ablation in other areas, coagulative necrosis leads to fibrosis, and the soft tissue eventually contracts in volume with increased stiffness, thereby resulting in less tissue elasticity and vibration.

Carroll et al¹⁰ reported that nasal surgery combined with radiofrequency ablation of either the palate or the base of the tongue completely resolved snoring (according to the patient’s bed partner) in 42% of cases and improved it in 52%, with few complications. Also, patients who received more than one radiofrequency ablation application were more than twice as likely to have resolution of their snoring.

A systematic review of palatal radiofrequency ablation for snoring found that it is safe with minimal complication rates and reduces snoring in short-term follow-up.¹¹ The authors reviewed 30 studies: two randomized controlled trials, four clinical controlled trials, and 24 prospective uncontrolled studies. The only placebo-controlled randomized controlled trial found soft-palate radiofrequency ablation to be superior to placebo. In these studies, follow-up varied from 6 weeks to 26 months. However, the relapse rate was as high as 50% at a mean follow-up time of 13.2 months.

Thus, most of the information in this review has come from observational studies with short follow-up time. In another study, however, the authors presented a 5-year follow-up of palatal radiofrequency ablation that showed persistent and satisfying reduction of snoring.¹²

Injection snoreplasty

Alternative procedures have been used to reduce palatal flutter that leads to snoring.

Injection snoreplasty was first described by Brietzke and Mair al in 2001.¹³ Sodium tetradecyl sulfate, a sclerotherapy agent, is injected directly into the submucosal layer of the soft palate to induce scarring and reduce or eliminate snoring caused by the soft palate.

In a cohort study of 25 patients, the subjective success rate was 75% (13 patients) as far out as 19 months.¹⁴ In a separate cohort of 17 patients, home polysomnography with audio recordings was done before and after treatment in patients who underwent injection snoreplasty. Twelve (17%) of these patients had a significant reduction in the proportion of palatal snoring, loudness, and flutter frequency. Long-term success and snoring relapse rates of injection snoreplasty were reported to be similar to those of other current treatments.¹⁴

Pillar implants

The Pillar implant (Medtronic) was approved by the US Food and Drug Administration in 2002 for snoring and in 2004 for mild to moderate obstructive sleep apnea.

The implant, made of a woven polyester material, is designed to reduce vibration of the soft palate by increasing its stiffness. The implant induces a chronic inflammatory response that is thought to result in the formation of a fibrous capsule, which may also play a role in palatal stiffening. Three thin implants are inserted into the paramedian soft palate in a parallel orientation. This is an outpatient procedure done in the office.

The short-term benefits of the Pillar implant procedure have been well documented.^15,16 A meta-analysis of seven case-controlled studies that included 174 patients found the Pillar implant significantly decreased the loudness of snoring by 59%.¹⁵ The major disadvantage of Pillar implants was their high extrusion rate, which was reported to be 9.3%.¹⁵ While statistically significant improvement has been shown at up to 1 year, a recent longitudinal study suggests a clinical deterioration in snoring scale scores by 4 years after the procedure.¹⁶

Laser-assisted uvulopalatoplasty

Laser-assisted uvulopalatoplasty is a staged office-based procedure that involves removal of excess uvular mucosa and the creation of transpalatal vertical troughs to widen the retropalatal airway for the treatment of snoring and mild obstructive sleep apnea. The treatment typically requires about three sessions. It aims to mimic the palatal appearance of uvulopalatopharyngoplasty used to treat obstructive sleep apnea and has been proposed to have similar surgical outcomes in properly selected patients.

Krespi and Kaeker,¹⁷ in 1994, were among the first to describe the technique in the United States.

Kyrmizakis et al,¹⁸ in a retrospective study of 59 patients with habitual snoring who underwent laser-assisted uvulopalatoplasty, showed that a significant number of patients benefited from the procedure. During a follow-up ranging from 6 months to 5 years (mean 40 months), 91.5% of the patients with habitual snoring reported significant short-term improvement based on a posttreatment questionnaire, and 79.7% reported long-term subjective improvement.

Unfortunately, most of the studies have been small, and thus there is some controversy about the efficacy of laser-assisted uvulopalatoplasty, particularly in patients with obstructive sleep apnea. The most significant complication during healing is pain, which may deter patients from completing the full course of treatment.

Snoring can range in significance from disturbing a bed partner to being a symptom of obstructive sleep apnea, a risk factor for cardiac disease and stroke. Snoring that is unrelated to obstructive sleep apnea may respond to a combination of nonsurgical treatments. However, if the problem persists despite conservative therapy, then surgical options may be considered.

This article explores why people snore, provides guidance for evaluating it and ruling out obstructive sleep apnea, and describes the available surgical treatments. Snoring associated with obstructive sleep apnea requires a different surgical treatment strategy that is beyond the scope of this article.

WHY PEOPLE SNORE

Humans go through four stages of sleep in each sleep cycle (and four or five cycles per night), and each stage has unique physiologic characteristics. As we progress deeper into sleep with each successive stage, the skeletal muscles of the body relax and eventually become atonic, except for the respiratory and ocular muscles. Soft tissues of the upper aerodigestive tract also lose their muscular tone.

Snoring is an undesirable vibratory sound that originates from the soft tissues of the upper respiratory tract during sleep, as airflow causes the relaxed tissues to vibrate.

The upper airway can be obstructed by the nasal septum, inferior nasal turbinates, adenoids, tonsils, uvula, soft palate, and base of the tongue—and often by more than one (Figure 1).³ In rare cases, obstruction can occur at the level of the larynx, such as from a tumor, laryngomalacia, or a laryngeal defect.

A SPECTRUM OF SLEEP-DISORDERED BREATHING

The American Academy of Sleep Medicine’s International Classification of Sleep Disorders¹ defines a number of sleep disorders. In clinical practice, the first-line diagnostic test for sleep disorders is polysomnography.

Snoring is only one sign of sleep-disordered breathing; others are excessive daytime somnolence, restless sleep, and witnessed apnea.

Considerable evidence links obstructive sleep apnea with serious medical problems including hypertension, coronary artery disease, heart failure, cardiac arrhythmia, and stroke.² Others include mood disorders, decreased libido, and cognitive impairment, with changes in attention, concentration, executive function, and fine-motor coordination.⁴ Therefore, ruling out obstructive sleep apnea is essential before pursuing interventions for primary snoring, although both disorders may warrant surgery.

PATIENT HISTORY

Most patients who present to the office because of snoring have snored for many years. Many seek medical attention at the request of a long-suffering bed partner.

Associated symptoms in primary snoring may include mouth breathing, chronic nasal congestion, and morning dry throat. Witnessed apnea, frequent awakenings during sleep, restless sleep, daytime somnolence, frequents naps, and memory impairment may be signs of more significant sleep-disordered breathing, such as obstructive sleep apnea.

The Epworth sleepiness scale may help quantify the severity of daytime somnolence.⁵ It is measured in a short questionnaire in which the patient indicates, on a scale of 0 to 3, his or her likelihood of dozing in a variety of situations.

The STOP-BANG questionnaire consists of eight yes-no questions:

• Snore: Have you been told that you snore?
• Tired: Are you often tired during the day?
• Obstruction: Do you know if you stop breathing, or has anyone witnessed you stop breathing while you are asleep?
• Pressure: Do you have high blood pressure or are you on medication to control high blood pressure?
• Body mass index: Is your body mass index higher than 35 kg/m²?
• Age: Are you age 50 or older?
• Neck: Do you have a neck circumference greater than 17 inches (men) or greater than 16 inches (women)?
• Gender: Are you male?

A score of three or higher has shown a sensitivity of 93% for detecting moderate obstructive sleep apnea and 100% for severe obstructive sleep apnea.⁶

SEARCHING FOR ANATOMIC CAUSES OF SNORING

A thorough physical examination should be done, focusing on potential anatomic causes of snoring. Nasal septal deviation or inferior turbinate hypertrophy with mucosal congestion may contribute to chronic mouth breathing secondary to nasal obstruction. Patients with a body mass index over 35 kg/m² and neck circumference over 17 inches (16 inches in women) are at higher risk of obstructive sleep apnea.

Indirect mirror examination or flexible transnasal endoscopy may reveal obstructing or persistent adenoid lymphoid tissue, particularly in young adults. Transnasal endoscopy may also reveal dynamic collapse of the palate and lateral oropharyngeal wall or fullness of the tongue base with subsequent narrowing of the oropharynx.

Examination of the oral cavity may reveal a disproportionately large tongue, a narrow opening into the oropharynx, or tonsillar hypertrophy. The Friedman classification (Figure 2), also called the modified Mallampati scale, can be used to describe the findings on physical examination of the palate and tongue in a systematic way. There are four grades of increasing severity, and the higher the grade, the less likely that surgery will succeed in patients with obstructive sleep apnea.⁷ The mouth is examined with the tongue in a relaxed position; in contrast, the original Mallampati classification, which is often used by anesthesiologists in assessing the oral airway, is assessed with the tongue protruding.

During flexible endoscopy, asking the patient to attempt to recreate the snoring can sometimes reveal the causative anatomic structure, which is usually the soft palate.

SLEEP STUDIES

A full diagnostic workup should include a sleep study if obstructive sleep apnea cannot be ruled out by the history and examination. Sleep studies include either polysomnography in a sleep laboratory or a home sleep test. They allow the clinician to further evaluate the severity of sleep-disordered breathing and to distinguish primary snoring from obstructive sleep apnea. This is particularly important if elective surgical intervention is planned. Sleep studies can also be used to evaluate for other sleep disorders.

Apnea is considered obstructive when polysomnography reveals episodes of no oral or nasal airflow with continued inspiratory effort, evidenced by abdominal or thoracic muscle activity. Hypopnea is defined as a 30% or greater reduction in airflow lasting at least 10 seconds, with an associated 4% or greater oxygen desaturation.¹ The combined number of apnea and hypopnea events per hour, or apnea-hypopnea index, is used clinically to quantify the severity of sleep-disordered breathing.

Primary snoring is diagnosed if the apnea-hypopnea index is 5 or less. Obstructive sleep apnea is considered mild when the apnea-hypopnea index is greater than 5 but less than 15, moderate from 15 to 30, and severe if over 30.

LIMITED ROLE FOR IMAGING

Cephalometric radiography (plain radiography of the airways) has limited value in the workup of primary snoring and is discouraged. Imaging is most useful in assessing craniofacial skeletal abnormalities. Lateral airway images can help in diagnosing adenoid hypertrophy in children. However, flexible nasopharyngoscopy can obtain this information by direct visualization with no radiation exposure.

Computed tomography and magnetic resonance imaging are seldom used in the workup of snoring because they do little to guide therapeutic intervention, are expensive, and, in the case of computed tomography, expose the patient to unnecessary radiation. Imaging does a have a role when planning surgical intervention of obstruction that involves the maxillofacial skeleton.

NONSURGICAL MANAGEMENT

The primary goal of therapy for snoring is to eliminate or reduce noise levels.

Although no study to date has analyzed the efficacy of nonsurgical management, several treatments are aimed at the root causes of snoring in an attempt to decrease it.

Intranasal topical steroids reduce inflammation of the nasal mucosa that occurs with allergic and nonallergic rhinitis, thereby opening up the nasal airway. They may reduce snoring in a small number of cases. These drugs must often be used in the long term to maintain their efficacy.

Devices. Other than continuous positive airway pressure (CPAP), the only currently available nonsurgical device approved by the US Food and Drug Administration for the treatment of snoring and obstructive sleep apnea is an oral dental appliance, which is customized to the patient’s dentition to relieve upper-airway obstruction by soft tissues of the oral cavity. The lower jaw is forced anteriorly, pulling the tongue and attached soft tissues forward. Custom-fitted oral appliances are an effective option for mild to moderate sleep apnea and associated snoring, and are more effective than thermoplastic “boil-and-bite” devices.⁸ These can easily be used in patients who have primary snoring.

Over-the-counter remedies such as nasal strips and head-positioning pillows have not been shown to be efficacious for snoring.⁹

Weight loss. Patients should be encouraged to join a weight-management program if overweight.

Sleep on the side, not on the back. Changing the sleep position may be useful in patients who have positional symptoms. Snoring is often worse in the supine position because gravity acting on the palate and tongue causes narrowing of the airway. “Positional therapy” employs devices to force patients to sleep in a lateral decubitus position to counter the effects of gravity.

Alcohol cessation. Alcohol has a relaxing effect on the muscles of the upper-respiratory tract, and abstaining from alcohol may therefore reduce snoring.

INDICATIONS FOR SURGERY

Surgery can decrease the noise level of snoring and thus bring relief for the patient’s bed partner.

Assessment of the upper airway may suggest the appropriate treatment, depending on whether the patient has nasal obstruction, adenoid hypertrophy, or palatal movement. A sleep study, if not previously done, should be done before surgery to rule out obstructive sleep apnea.

Many patients opt for surgery after noninvasive forms of treatment have proven ineffective or difficult to tolerate. When medical therapy for snoring has been unsuccessful, a discussion of the benefits, risks, and alternatives to surgery must take place between the patient and the surgeon.

SURGICAL PROCEDURES

Septoplasty

Septoplasty—straightening the nasal septum to improve the nasal airway—is an outpatient procedure. Although a deviated septum alone is not often the sole cause of snoring, most otolaryngologists agree that the septum should be addressed before or concomitantly with any palatal surgery for sleep-disordered breathing.

Nasal congestion often comes from a deviated bony or cartilaginous septum, enlarged turbinates, or bone spurs. Septal deviation may be developmental or the result of trauma to the nose.

Complications of septoplasty are rare but include septal perforation, scar-band formation, septal hematoma, epistaxis, and infection.

Radiofrequency ablation of the inferior turbinates

Hypertrophy of the inferior turbinate is the most common cause of nasal obstruction, followed by structural deformity of the nasal airway by septal deviation.³ Many patients report fixed or fluctuating nasal congestion and chronic mouth-breathing. The causes of turbinate congestion or enlargement include allergic rhinitis, upper-respiratory infection, and chronic rhinitis. In most cases, turbinate hypertrophy occurs at the level of the submucosa.

Radiofrequency ablation uses radiofrequency energy to generate heat at approximately 85°C (185°F) to create finely controlled coagulative lesions. The lesions are naturally resorbed in 3 to 8 weeks, inducing fibrosis, reducing excess tissue volume, and thus opening the airway. The procedure can be repeated several times to achieve optimal results. Radiofrequency ablation can also be used to reduce anatomic obstruction in other parts of the airway, such as the soft palate and the base of tongue.

Submucosal radiofrequency ablation of the inferior turbinate is a simple office-based procedure. It is often combined with septoplasty to optimize the nasal airway.

Mild to moderate edema with subsequent nasal obstruction and thick mucus formation can be expected the first week after the procedure. The risk of postoperative bleeding and infection is low. When performed with septoplasty, there is a low risk that scar tissue, or synechiae, may form between the turbinate and the septum.

Radiofrequency ablation of the palate

The soft palate is the most common anatomic source of snoring, and radiofrequency ablation can be applied to it as well. As with radiofrequency ablation in other areas, coagulative necrosis leads to fibrosis, and the soft tissue eventually contracts in volume with increased stiffness, thereby resulting in less tissue elasticity and vibration.

Carroll et al¹⁰ reported that nasal surgery combined with radiofrequency ablation of either the palate or the base of the tongue completely resolved snoring (according to the patient’s bed partner) in 42% of cases and improved it in 52%, with few complications. Also, patients who received more than one radiofrequency ablation application were more than twice as likely to have resolution of their snoring.

A systematic review of palatal radiofrequency ablation for snoring found that it is safe with minimal complication rates and reduces snoring in short-term follow-up.¹¹ The authors reviewed 30 studies: two randomized controlled trials, four clinical controlled trials, and 24 prospective uncontrolled studies. The only placebo-controlled randomized controlled trial found soft-palate radiofrequency ablation to be superior to placebo. In these studies, follow-up varied from 6 weeks to 26 months. However, the relapse rate was as high as 50% at a mean follow-up time of 13.2 months.

Thus, most of the information in this review has come from observational studies with short follow-up time. In another study, however, the authors presented a 5-year follow-up of palatal radiofrequency ablation that showed persistent and satisfying reduction of snoring.¹²

Injection snoreplasty

Alternative procedures have been used to reduce palatal flutter that leads to snoring.

Injection snoreplasty was first described by Brietzke and Mair al in 2001.¹³ Sodium tetradecyl sulfate, a sclerotherapy agent, is injected directly into the submucosal layer of the soft palate to induce scarring and reduce or eliminate snoring caused by the soft palate.

In a cohort study of 25 patients, the subjective success rate was 75% (13 patients) as far out as 19 months.¹⁴ In a separate cohort of 17 patients, home polysomnography with audio recordings was done before and after treatment in patients who underwent injection snoreplasty. Twelve (17%) of these patients had a significant reduction in the proportion of palatal snoring, loudness, and flutter frequency. Long-term success and snoring relapse rates of injection snoreplasty were reported to be similar to those of other current treatments.¹⁴

Pillar implants

The Pillar implant (Medtronic) was approved by the US Food and Drug Administration in 2002 for snoring and in 2004 for mild to moderate obstructive sleep apnea.

The implant, made of a woven polyester material, is designed to reduce vibration of the soft palate by increasing its stiffness. The implant induces a chronic inflammatory response that is thought to result in the formation of a fibrous capsule, which may also play a role in palatal stiffening. Three thin implants are inserted into the paramedian soft palate in a parallel orientation. This is an outpatient procedure done in the office.

The short-term benefits of the Pillar implant procedure have been well documented.^15,16 A meta-analysis of seven case-controlled studies that included 174 patients found the Pillar implant significantly decreased the loudness of snoring by 59%.¹⁵ The major disadvantage of Pillar implants was their high extrusion rate, which was reported to be 9.3%.¹⁵ While statistically significant improvement has been shown at up to 1 year, a recent longitudinal study suggests a clinical deterioration in snoring scale scores by 4 years after the procedure.¹⁶

Laser-assisted uvulopalatoplasty

Laser-assisted uvulopalatoplasty is a staged office-based procedure that involves removal of excess uvular mucosa and the creation of transpalatal vertical troughs to widen the retropalatal airway for the treatment of snoring and mild obstructive sleep apnea. The treatment typically requires about three sessions. It aims to mimic the palatal appearance of uvulopalatopharyngoplasty used to treat obstructive sleep apnea and has been proposed to have similar surgical outcomes in properly selected patients.

Krespi and Kaeker,¹⁷ in 1994, were among the first to describe the technique in the United States.

Kyrmizakis et al,¹⁸ in a retrospective study of 59 patients with habitual snoring who underwent laser-assisted uvulopalatoplasty, showed that a significant number of patients benefited from the procedure. During a follow-up ranging from 6 months to 5 years (mean 40 months), 91.5% of the patients with habitual snoring reported significant short-term improvement based on a posttreatment questionnaire, and 79.7% reported long-term subjective improvement.

Unfortunately, most of the studies have been small, and thus there is some controversy about the efficacy of laser-assisted uvulopalatoplasty, particularly in patients with obstructive sleep apnea. The most significant complication during healing is pain, which may deter patients from completing the full course of treatment.

Snoring can range in significance from disturbing a bed partner to being a symptom of obstructive sleep apnea, a risk factor for cardiac disease and stroke. Snoring that is unrelated to obstructive sleep apnea may respond to a combination of nonsurgical treatments. However, if the problem persists despite conservative therapy, then surgical options may be considered.

This article explores why people snore, provides guidance for evaluating it and ruling out obstructive sleep apnea, and describes the available surgical treatments. Snoring associated with obstructive sleep apnea requires a different surgical treatment strategy that is beyond the scope of this article.

WHY PEOPLE SNORE

Humans go through four stages of sleep in each sleep cycle (and four or five cycles per night), and each stage has unique physiologic characteristics. As we progress deeper into sleep with each successive stage, the skeletal muscles of the body relax and eventually become atonic, except for the respiratory and ocular muscles. Soft tissues of the upper aerodigestive tract also lose their muscular tone.

Snoring is an undesirable vibratory sound that originates from the soft tissues of the upper respiratory tract during sleep, as airflow causes the relaxed tissues to vibrate.

The upper airway can be obstructed by the nasal septum, inferior nasal turbinates, adenoids, tonsils, uvula, soft palate, and base of the tongue—and often by more than one (Figure 1).³ In rare cases, obstruction can occur at the level of the larynx, such as from a tumor, laryngomalacia, or a laryngeal defect.

A SPECTRUM OF SLEEP-DISORDERED BREATHING

The American Academy of Sleep Medicine’s International Classification of Sleep Disorders¹ defines a number of sleep disorders. In clinical practice, the first-line diagnostic test for sleep disorders is polysomnography.

Snoring is only one sign of sleep-disordered breathing; others are excessive daytime somnolence, restless sleep, and witnessed apnea.

Considerable evidence links obstructive sleep apnea with serious medical problems including hypertension, coronary artery disease, heart failure, cardiac arrhythmia, and stroke.² Others include mood disorders, decreased libido, and cognitive impairment, with changes in attention, concentration, executive function, and fine-motor coordination.⁴ Therefore, ruling out obstructive sleep apnea is essential before pursuing interventions for primary snoring, although both disorders may warrant surgery.

PATIENT HISTORY

Most patients who present to the office because of snoring have snored for many years. Many seek medical attention at the request of a long-suffering bed partner.

Associated symptoms in primary snoring may include mouth breathing, chronic nasal congestion, and morning dry throat. Witnessed apnea, frequent awakenings during sleep, restless sleep, daytime somnolence, frequents naps, and memory impairment may be signs of more significant sleep-disordered breathing, such as obstructive sleep apnea.

The Epworth sleepiness scale may help quantify the severity of daytime somnolence.⁵ It is measured in a short questionnaire in which the patient indicates, on a scale of 0 to 3, his or her likelihood of dozing in a variety of situations.

The STOP-BANG questionnaire consists of eight yes-no questions:

• Snore: Have you been told that you snore?
• Tired: Are you often tired during the day?
• Obstruction: Do you know if you stop breathing, or has anyone witnessed you stop breathing while you are asleep?
• Pressure: Do you have high blood pressure or are you on medication to control high blood pressure?
• Body mass index: Is your body mass index higher than 35 kg/m²?
• Age: Are you age 50 or older?
• Neck: Do you have a neck circumference greater than 17 inches (men) or greater than 16 inches (women)?
• Gender: Are you male?

A score of three or higher has shown a sensitivity of 93% for detecting moderate obstructive sleep apnea and 100% for severe obstructive sleep apnea.⁶

SEARCHING FOR ANATOMIC CAUSES OF SNORING

A thorough physical examination should be done, focusing on potential anatomic causes of snoring. Nasal septal deviation or inferior turbinate hypertrophy with mucosal congestion may contribute to chronic mouth breathing secondary to nasal obstruction. Patients with a body mass index over 35 kg/m² and neck circumference over 17 inches (16 inches in women) are at higher risk of obstructive sleep apnea.

Indirect mirror examination or flexible transnasal endoscopy may reveal obstructing or persistent adenoid lymphoid tissue, particularly in young adults. Transnasal endoscopy may also reveal dynamic collapse of the palate and lateral oropharyngeal wall or fullness of the tongue base with subsequent narrowing of the oropharynx.

Examination of the oral cavity may reveal a disproportionately large tongue, a narrow opening into the oropharynx, or tonsillar hypertrophy. The Friedman classification (Figure 2), also called the modified Mallampati scale, can be used to describe the findings on physical examination of the palate and tongue in a systematic way. There are four grades of increasing severity, and the higher the grade, the less likely that surgery will succeed in patients with obstructive sleep apnea.⁷ The mouth is examined with the tongue in a relaxed position; in contrast, the original Mallampati classification, which is often used by anesthesiologists in assessing the oral airway, is assessed with the tongue protruding.

During flexible endoscopy, asking the patient to attempt to recreate the snoring can sometimes reveal the causative anatomic structure, which is usually the soft palate.

SLEEP STUDIES

A full diagnostic workup should include a sleep study if obstructive sleep apnea cannot be ruled out by the history and examination. Sleep studies include either polysomnography in a sleep laboratory or a home sleep test. They allow the clinician to further evaluate the severity of sleep-disordered breathing and to distinguish primary snoring from obstructive sleep apnea. This is particularly important if elective surgical intervention is planned. Sleep studies can also be used to evaluate for other sleep disorders.

Apnea is considered obstructive when polysomnography reveals episodes of no oral or nasal airflow with continued inspiratory effort, evidenced by abdominal or thoracic muscle activity. Hypopnea is defined as a 30% or greater reduction in airflow lasting at least 10 seconds, with an associated 4% or greater oxygen desaturation.¹ The combined number of apnea and hypopnea events per hour, or apnea-hypopnea index, is used clinically to quantify the severity of sleep-disordered breathing.

Primary snoring is diagnosed if the apnea-hypopnea index is 5 or less. Obstructive sleep apnea is considered mild when the apnea-hypopnea index is greater than 5 but less than 15, moderate from 15 to 30, and severe if over 30.

LIMITED ROLE FOR IMAGING

Cephalometric radiography (plain radiography of the airways) has limited value in the workup of primary snoring and is discouraged. Imaging is most useful in assessing craniofacial skeletal abnormalities. Lateral airway images can help in diagnosing adenoid hypertrophy in children. However, flexible nasopharyngoscopy can obtain this information by direct visualization with no radiation exposure.

Computed tomography and magnetic resonance imaging are seldom used in the workup of snoring because they do little to guide therapeutic intervention, are expensive, and, in the case of computed tomography, expose the patient to unnecessary radiation. Imaging does a have a role when planning surgical intervention of obstruction that involves the maxillofacial skeleton.

NONSURGICAL MANAGEMENT

The primary goal of therapy for snoring is to eliminate or reduce noise levels.

Although no study to date has analyzed the efficacy of nonsurgical management, several treatments are aimed at the root causes of snoring in an attempt to decrease it.

Intranasal topical steroids reduce inflammation of the nasal mucosa that occurs with allergic and nonallergic rhinitis, thereby opening up the nasal airway. They may reduce snoring in a small number of cases. These drugs must often be used in the long term to maintain their efficacy.

Devices. Other than continuous positive airway pressure (CPAP), the only currently available nonsurgical device approved by the US Food and Drug Administration for the treatment of snoring and obstructive sleep apnea is an oral dental appliance, which is customized to the patient’s dentition to relieve upper-airway obstruction by soft tissues of the oral cavity. The lower jaw is forced anteriorly, pulling the tongue and attached soft tissues forward. Custom-fitted oral appliances are an effective option for mild to moderate sleep apnea and associated snoring, and are more effective than thermoplastic “boil-and-bite” devices.⁸ These can easily be used in patients who have primary snoring.

Over-the-counter remedies such as nasal strips and head-positioning pillows have not been shown to be efficacious for snoring.⁹

Weight loss. Patients should be encouraged to join a weight-management program if overweight.

Sleep on the side, not on the back. Changing the sleep position may be useful in patients who have positional symptoms. Snoring is often worse in the supine position because gravity acting on the palate and tongue causes narrowing of the airway. “Positional therapy” employs devices to force patients to sleep in a lateral decubitus position to counter the effects of gravity.

Alcohol cessation. Alcohol has a relaxing effect on the muscles of the upper-respiratory tract, and abstaining from alcohol may therefore reduce snoring.

INDICATIONS FOR SURGERY

Surgery can decrease the noise level of snoring and thus bring relief for the patient’s bed partner.

Assessment of the upper airway may suggest the appropriate treatment, depending on whether the patient has nasal obstruction, adenoid hypertrophy, or palatal movement. A sleep study, if not previously done, should be done before surgery to rule out obstructive sleep apnea.

Many patients opt for surgery after noninvasive forms of treatment have proven ineffective or difficult to tolerate. When medical therapy for snoring has been unsuccessful, a discussion of the benefits, risks, and alternatives to surgery must take place between the patient and the surgeon.

SURGICAL PROCEDURES

Septoplasty

Septoplasty—straightening the nasal septum to improve the nasal airway—is an outpatient procedure. Although a deviated septum alone is not often the sole cause of snoring, most otolaryngologists agree that the septum should be addressed before or concomitantly with any palatal surgery for sleep-disordered breathing.

Nasal congestion often comes from a deviated bony or cartilaginous septum, enlarged turbinates, or bone spurs. Septal deviation may be developmental or the result of trauma to the nose.

Complications of septoplasty are rare but include septal perforation, scar-band formation, septal hematoma, epistaxis, and infection.

Radiofrequency ablation of the inferior turbinates

Hypertrophy of the inferior turbinate is the most common cause of nasal obstruction, followed by structural deformity of the nasal airway by septal deviation.³ Many patients report fixed or fluctuating nasal congestion and chronic mouth-breathing. The causes of turbinate congestion or enlargement include allergic rhinitis, upper-respiratory infection, and chronic rhinitis. In most cases, turbinate hypertrophy occurs at the level of the submucosa.

Radiofrequency ablation uses radiofrequency energy to generate heat at approximately 85°C (185°F) to create finely controlled coagulative lesions. The lesions are naturally resorbed in 3 to 8 weeks, inducing fibrosis, reducing excess tissue volume, and thus opening the airway. The procedure can be repeated several times to achieve optimal results. Radiofrequency ablation can also be used to reduce anatomic obstruction in other parts of the airway, such as the soft palate and the base of tongue.

Submucosal radiofrequency ablation of the inferior turbinate is a simple office-based procedure. It is often combined with septoplasty to optimize the nasal airway.

Mild to moderate edema with subsequent nasal obstruction and thick mucus formation can be expected the first week after the procedure. The risk of postoperative bleeding and infection is low. When performed with septoplasty, there is a low risk that scar tissue, or synechiae, may form between the turbinate and the septum.

Radiofrequency ablation of the palate

The soft palate is the most common anatomic source of snoring, and radiofrequency ablation can be applied to it as well. As with radiofrequency ablation in other areas, coagulative necrosis leads to fibrosis, and the soft tissue eventually contracts in volume with increased stiffness, thereby resulting in less tissue elasticity and vibration.

Carroll et al¹⁰ reported that nasal surgery combined with radiofrequency ablation of either the palate or the base of the tongue completely resolved snoring (according to the patient’s bed partner) in 42% of cases and improved it in 52%, with few complications. Also, patients who received more than one radiofrequency ablation application were more than twice as likely to have resolution of their snoring.

A systematic review of palatal radiofrequency ablation for snoring found that it is safe with minimal complication rates and reduces snoring in short-term follow-up.¹¹ The authors reviewed 30 studies: two randomized controlled trials, four clinical controlled trials, and 24 prospective uncontrolled studies. The only placebo-controlled randomized controlled trial found soft-palate radiofrequency ablation to be superior to placebo. In these studies, follow-up varied from 6 weeks to 26 months. However, the relapse rate was as high as 50% at a mean follow-up time of 13.2 months.

Thus, most of the information in this review has come from observational studies with short follow-up time. In another study, however, the authors presented a 5-year follow-up of palatal radiofrequency ablation that showed persistent and satisfying reduction of snoring.¹²

Injection snoreplasty

Alternative procedures have been used to reduce palatal flutter that leads to snoring.

Injection snoreplasty was first described by Brietzke and Mair al in 2001.¹³ Sodium tetradecyl sulfate, a sclerotherapy agent, is injected directly into the submucosal layer of the soft palate to induce scarring and reduce or eliminate snoring caused by the soft palate.

In a cohort study of 25 patients, the subjective success rate was 75% (13 patients) as far out as 19 months.¹⁴ In a separate cohort of 17 patients, home polysomnography with audio recordings was done before and after treatment in patients who underwent injection snoreplasty. Twelve (17%) of these patients had a significant reduction in the proportion of palatal snoring, loudness, and flutter frequency. Long-term success and snoring relapse rates of injection snoreplasty were reported to be similar to those of other current treatments.¹⁴

Pillar implants

The Pillar implant (Medtronic) was approved by the US Food and Drug Administration in 2002 for snoring and in 2004 for mild to moderate obstructive sleep apnea.

The implant, made of a woven polyester material, is designed to reduce vibration of the soft palate by increasing its stiffness. The implant induces a chronic inflammatory response that is thought to result in the formation of a fibrous capsule, which may also play a role in palatal stiffening. Three thin implants are inserted into the paramedian soft palate in a parallel orientation. This is an outpatient procedure done in the office.

The short-term benefits of the Pillar implant procedure have been well documented.^15,16 A meta-analysis of seven case-controlled studies that included 174 patients found the Pillar implant significantly decreased the loudness of snoring by 59%.¹⁵ The major disadvantage of Pillar implants was their high extrusion rate, which was reported to be 9.3%.¹⁵ While statistically significant improvement has been shown at up to 1 year, a recent longitudinal study suggests a clinical deterioration in snoring scale scores by 4 years after the procedure.¹⁶

Laser-assisted uvulopalatoplasty

Laser-assisted uvulopalatoplasty is a staged office-based procedure that involves removal of excess uvular mucosa and the creation of transpalatal vertical troughs to widen the retropalatal airway for the treatment of snoring and mild obstructive sleep apnea. The treatment typically requires about three sessions. It aims to mimic the palatal appearance of uvulopalatopharyngoplasty used to treat obstructive sleep apnea and has been proposed to have similar surgical outcomes in properly selected patients.

Krespi and Kaeker,¹⁷ in 1994, were among the first to describe the technique in the United States.

Kyrmizakis et al,¹⁸ in a retrospective study of 59 patients with habitual snoring who underwent laser-assisted uvulopalatoplasty, showed that a significant number of patients benefited from the procedure. During a follow-up ranging from 6 months to 5 years (mean 40 months), 91.5% of the patients with habitual snoring reported significant short-term improvement based on a posttreatment questionnaire, and 79.7% reported long-term subjective improvement.

Unfortunately, most of the studies have been small, and thus there is some controversy about the efficacy of laser-assisted uvulopalatoplasty, particularly in patients with obstructive sleep apnea. The most significant complication during healing is pain, which may deter patients from completing the full course of treatment.

A 58-year-old man with a history of irritable bowel syndrome and diabetes presents for an evaluation in early November. He is taking metformin and insulin glargine 10 units. He smokes 1 pack per day. He believes that his childhood immunizations were completed, but he has no records. He thinks his last “shot” was 15 years ago when he cut his hand on some wood.

Which immunizations, if any, would be most appropriate for this patient?

The explosion of new vaccines, new formulations, and new combinations made available in recent years makes managing immunizations a challenge. This article reviews common immunizations and current recommendations for their appropriate use.

Immunization recommendations (Table 1) are made predominantly by the Advisory Committee on Immunization Practices (ACIP) of the US Centers for Disease Control and Prevention (CDC). The last 15 years have seen the arrival of new vaccines (eg, varicella, hepatitis A, pneumococcal, and human papillomavirus), new formulations (eg, intranasal influenza), and new combinations.

To keep clinicians abreast of new indications, the ACIP issues immunization schedules annually for children and adults, available online and downloadable for easy reference.¹ For adults, the ACIP provides schedules based on age and medical condition. The schedule for medical conditions offers specific information regarding immunization and pregnancy, human immunodeficiency virus (HIV) infection, kidney failure, heart disease, asplenia, and other conditions. The ACIP also provides guidance on contraindications; for example, pregnant and immunocompromised patients should not receive the live-attenuated vaccines, ie, zoster, varicella, and combined measles, mumps, and rubella [MMR]).

Adult awareness of vaccines is low, as are vaccination rates: in people older than 60, the vaccination rate is about 70% for influenza, 60% for pneumococcus, 50% for tetanus, and 15% for zoster. The lack of vaccine awareness and the availability of new vaccines and indications have made it difficult to manage immunizations in the primary care setting. The electronic medical record is useful for tracking patient vaccine needs. Ideally, keeping up with immunizations should be a routine part of visits provided by a physician’s care team and does not always require direct physician coordination.

TETANUS, DIPHTHERIA, PERTUSSIS EVERY 10 YEARS

Tetanus (also called “lockjaw”) is a nervous system disorder characterized by muscle spasms. Caused by infection with Clostridium tetani, it is a rare disease in the United States thanks to widespread immunization, and it causes fewer than 50 cases annually. Worldwide, the incidence is about 1 million cases a year with 200,000 to 300,000 deaths.

Diphtheria (formerly sometimes called “throat distemper”) is caused by the gram-positive bacillus Corynebacterium diphtheriae and can occur as a respiratory illness or as a milder cutaneous form. The last outbreak in the United States was in Seattle in the 1970s, with the last reported case in 2003. The ACIP recommends booster shots for tetanus and diphtheria every 10 years following completion of the primary series.

Pertussis or whooping cough, caused by Bordetella pertussis infection, is a highly contagious disease increasingly seen in adults in the United States. It causes few deaths but high morbidity, with coughing that can persist up to 3 months. Coughing can be severe enough to cause vomiting, a characteristic sign.

In July 2012, the CDC reported that the United States was at a 50-year high for pertussis, with 18,000 cases reported and 8 deaths.² In Washington State alone, more than 2,520 cases had been seen through June 16 of that year, a 1,300% increase over the previous year. Rates were high in older children and adolescents despite previous vaccination, suggesting an early waning of immunity.

The ACIP recommends a single dose of the combination of high-dose tetanus and low-dose diphtheria and pertussis vaccines (Tdap) for all adults regardless of age and for all pregnant women with each pregnancy between 27 and 36 weeks of gestation. A dose of Tdap counts as the tetanus-diphtheria booster shot that is recommended every 10 years.

The patient described above is due for his tetanus-diphtheria booster and so should be given Tdap.

MEASLES, MUMPS, RUBELLA FOR THOSE BORN AFTER 1957

Measles remains a problem in the developing world, with an estimated average of 330 deaths daily. The number of cases fell 99% in the United States following the vaccination program that started in the early 1960s. Before the measles vaccine was available, an estimated 90% of children acquired measles by age 15.

The clinical syndrome consists of fever, conjunctivitis, cough, rash, and the characteristic Koplik spots—small white spots occurring on the inside of cheeks early in the disease course.

During the first 5 months of 2014, the CDC reported 334 cases of measles in the United States in 18 states, with most people affected being unvaccinated.³ In comparison, from 2001 to 2008, the number of cases averaged 56 annually.

Many of the recent cases were associated with infections brought from the Philippines. The increased number of measles cases underscores the need for vaccination to prevent measles and its complications.

Mumps is an acute, self-limited viral syndrome, and parotitis is the hallmark. Vaccination led to a 99% decline in cases in the United States. Although complications are rare, they can be serious and include orchitis (with risk of sterility), meningoencephalitis, and deafness.

Mumps outbreaks still occur, especially in close-contact settings such as schools, colleges, and camps. During the first half of 2014, central Ohio had more than 400 cases linked to The Ohio State University.

Rubella, also known as German measles, is a generally mild infection but is associated with congenital rubella syndrome. If a woman is infected with rubella in the first trimester of pregnancy, the risk of miscarriage is greater than 80%, as is the risk of birth defects, including hearing loss, developmental delay, growth retardation, and cardiac and eye defects.

Recommendations for MMR vaccination. People born before 1957 are considered immune to measles and usually to mumps. Health care workers should document immunity before assuming no vaccination is needed.

People born in 1957 or after should have one dose of MMR vaccine unless immunity is documented or unless there is a contraindication such as immunosuppression. A second dose is recommended for those born in or after 1957 who are considered to be at high risk: eg, health care workers, students entering college, and international travelers. The second dose should be given 4 weeks after the first.

Women of childbearing age should be screened for immunity to rubella. Susceptible women should receive MMR, although not during pregnancy and not if they may get pregnant within 4 weeks.

The patient described above was born before 1957, and so he is probably immune to measles and mumps.

HEPATITIS B FOR THOSE AT RISK

Hepatitis B vaccination is recommended for all adolescents and adults at increased risk: eg, men who have sex with men, intravenous drug users, people with multiple sexual partners, health care workers, patients with end-stage renal disease on hemodialysis, patients with chronic liver disease, and those with diabetes (age < 60).

Immunization consists of a series of three shots (at 0, 1–2, and 4–6 months). Booster doses are not recommended. Postvaccination testing for immunity is available and is recommended for health care workers, patients on hemodialysis, patients with HIV infection or who are otherwise immunocompromised, and sexual partners of people who are positive for hepatitis B surface antigen. Nonresponders should be revaccinated with the entire three-shot schedule. Hepatitis B vaccination is safe in pregnancy.

The patient described above has diabetes and so is a candidate for vaccination.

HEPATITIS A: A SLIGHTLY DIFFERENT RISK GROUP

Hepatitis A vaccination is recommended only for at-risk populations: international travelers; intravenous drug users; men who have sex with men; patients with clotting disorders, chronic liver disease, or hepatitis C infection; international adoptees; and laboratory personnel working with hepatitis A virus. The vaccination is given in two doses with a minimum interval of 6 months between doses.

A hepatitis A and hepatitis B combination vaccine (Twinrix) is also available. It is given in three doses, at 0, 1, and 6 months.

ANNUAL INFLUENZA VACCINE FOR ALL

In 2010, the ACIP recommended a policy of universal annual vaccination for everyone age 6 months and older. Some patients are at especially high risk themselves or are at high risk of exposing others and so are given higher priority during vaccine shortages—ie, patients who are immunosuppressed or have other medical risk factors, health care workers, household members of at-risk patients, and pregnant women after 13 weeks of gestation.

There are few contraindications, so almost everyone should be encouraged to receive the influenza vaccine. The flu shot does not cause the flu, but it may cause soreness at the injection site. Those with severe egg allergy should not receive the standard flu shot; a recombinant vaccine that does not use egg culture is available.

The standard flu shot is an inactivated influenza vaccine. In the past, most formulations were trivalent, but quadrivalent formulations are becoming more common. Special high-dose formulations are believed to elicit a better immune response and can be recommended for people over age 65. Intradermal and intramuscular formulations are available.

An intranasal live-attenuated influenza vaccine is also available and may be used for people ages 2 through 49. It should not be given to immunosuppressed people or to pregnant women.

Our patient should get a flu shot.

PNEUMOCOCCAL VACCINE FOR THOSE AGE 65 AND OLDER OR AT RISK

Two formulations are now available for pneumococcal immunization. The standard is a 23-valent polysaccharide vaccine (PPSV23; Pneumovax) indicated for people age 65 and older.

Patients under age 65 can receive PPSV23 if they have chronic lung disease, chronic cardiovascular disease, diabetes, chronic liver disease, or alcoholism or are a resident of a nursing home or an active smoker.

Our patient is a candidate for PPSV23 since he smokes and has diabetes.

The other formulation is a conjugate 13-valent vaccine (PCV13; Prevnar 13). Patients over age 19 at high risk should be given PCV13 plus the PPSV23 8 weeks later. Those who already received PPSV23 should be given PCV13 vaccine more than 1 year later. Candidates for PCV13 are those with immunocompromising conditions (including chronic renal failure and nephrotic syndrome), functional or anatomic asplenia, cerebrospinal fluid leaks, or cochlear implants.

The current revaccination schedule for PPSV23 is as follows:

• One-time revaccination 5 years after the first dose in patients with chronic renal failure, nephrotic syndrome, asplenia, or an immunosuppressive condition
• One-time revaccination for patients age 65 or older if they were younger than 65 when first immunized (with one or two doses of PPSV23) and 5 years have passed
• No revaccination is needed for people vaccinated with PPSV23 after age 65.

HUMAN PAPILLOMAVIRUS VACCINE

Human papillomavirus is the most common sexually transmitted infection in the United States and is strongly associated with cervical cancer. Immunization is now indicated for both sexes, generally between the ages of 9 and 26. Two vaccines are available: the quadrivalent formulation (Gardasil) for males or females and the bivalent formulation (Cervarix) for females only.

Immunization should be given in three doses: at 0, 1 to 2 months, and 6 months. It can be given to patients who are immunocompromised as a result of infection (including HIV infection), disease, or medications, or who have a history of genital warts, an abnormal Papanicolaou test, or a positive human papillomavirus DNA test.

It is hoped that immunization will lead to a significant decrease in cervical cancer rates. Eradication is unlikely because other papillomavirus strains also can lead to cancer, so cancer screening is still warranted. For men who have sex with men, it is hoped that immunization will prevent condyloma and anal cancer.

CHICKENPOX AND SHINGLES VACCINES

Varicella vaccine (Varivax) contains a live-attenuated virus to protect against chickenpox. It is recommended for all adults who have no evidence of immunity. Immunity is assumed with a history of chickenpox, being born before 1980, or having positive titers. Vaccination should be emphasized for those who come in contact with patients at high risk of severe disease (eg, health care workers, family contacts of immunocompromised patients) and in individuals with a high risk of personal exposure (eg, teachers, day care workers).

The vaccine is given in two doses, 4 to 8 weeks apart. Women who are pregnant or who may get pregnant within 4 weeks should not be vaccinated.

The shingles vaccine (Zostavax) is a larger dose of the varicella vaccine and reduces the incidence of shingles by 50% and postherpetic neuralgia by 66%.⁴ It was approved by the US Food and Drug Administration in May 2006 for people starting at age 50, but was recommended by ACIP in October 2006 for people age 60 and older; as a result, some insurance companies deny coverage for patients ages 50 through 59.

The shingles vaccine can be given to patients who have already had shingles. Pregnancy and severe immunodeficiency are contraindications.

Our patient, 58 years old, could be considered for shingles vaccine if covered by his insurance company or if he wishes to pay for it.

MENINGOCOCCUS VACCINE

Meningococcal immunization is recommended for people at high risk: college students who plan to live in dormitories, adults without a spleen or with complement deficiencies or HIV infection, or travelers to the “meningitis belt” of sub-Saharan Africa.

Two types of meningococcal vaccine are available: the conjugate quadrivalent vaccine (MCV4) for people age 55 and younger, and the polysaccharide quadrivalent vaccine (MPSV4) for people over age 56.

A 58-year-old man with a history of irritable bowel syndrome and diabetes presents for an evaluation in early November. He is taking metformin and insulin glargine 10 units. He smokes 1 pack per day. He believes that his childhood immunizations were completed, but he has no records. He thinks his last “shot” was 15 years ago when he cut his hand on some wood.

Which immunizations, if any, would be most appropriate for this patient?

The explosion of new vaccines, new formulations, and new combinations made available in recent years makes managing immunizations a challenge. This article reviews common immunizations and current recommendations for their appropriate use.

Immunization recommendations (Table 1) are made predominantly by the Advisory Committee on Immunization Practices (ACIP) of the US Centers for Disease Control and Prevention (CDC). The last 15 years have seen the arrival of new vaccines (eg, varicella, hepatitis A, pneumococcal, and human papillomavirus), new formulations (eg, intranasal influenza), and new combinations.

To keep clinicians abreast of new indications, the ACIP issues immunization schedules annually for children and adults, available online and downloadable for easy reference.¹ For adults, the ACIP provides schedules based on age and medical condition. The schedule for medical conditions offers specific information regarding immunization and pregnancy, human immunodeficiency virus (HIV) infection, kidney failure, heart disease, asplenia, and other conditions. The ACIP also provides guidance on contraindications; for example, pregnant and immunocompromised patients should not receive the live-attenuated vaccines, ie, zoster, varicella, and combined measles, mumps, and rubella [MMR]).

Adult awareness of vaccines is low, as are vaccination rates: in people older than 60, the vaccination rate is about 70% for influenza, 60% for pneumococcus, 50% for tetanus, and 15% for zoster. The lack of vaccine awareness and the availability of new vaccines and indications have made it difficult to manage immunizations in the primary care setting. The electronic medical record is useful for tracking patient vaccine needs. Ideally, keeping up with immunizations should be a routine part of visits provided by a physician’s care team and does not always require direct physician coordination.

TETANUS, DIPHTHERIA, PERTUSSIS EVERY 10 YEARS

Tetanus (also called “lockjaw”) is a nervous system disorder characterized by muscle spasms. Caused by infection with Clostridium tetani, it is a rare disease in the United States thanks to widespread immunization, and it causes fewer than 50 cases annually. Worldwide, the incidence is about 1 million cases a year with 200,000 to 300,000 deaths.

Diphtheria (formerly sometimes called “throat distemper”) is caused by the gram-positive bacillus Corynebacterium diphtheriae and can occur as a respiratory illness or as a milder cutaneous form. The last outbreak in the United States was in Seattle in the 1970s, with the last reported case in 2003. The ACIP recommends booster shots for tetanus and diphtheria every 10 years following completion of the primary series.

Pertussis or whooping cough, caused by Bordetella pertussis infection, is a highly contagious disease increasingly seen in adults in the United States. It causes few deaths but high morbidity, with coughing that can persist up to 3 months. Coughing can be severe enough to cause vomiting, a characteristic sign.

In July 2012, the CDC reported that the United States was at a 50-year high for pertussis, with 18,000 cases reported and 8 deaths.² In Washington State alone, more than 2,520 cases had been seen through June 16 of that year, a 1,300% increase over the previous year. Rates were high in older children and adolescents despite previous vaccination, suggesting an early waning of immunity.

The ACIP recommends a single dose of the combination of high-dose tetanus and low-dose diphtheria and pertussis vaccines (Tdap) for all adults regardless of age and for all pregnant women with each pregnancy between 27 and 36 weeks of gestation. A dose of Tdap counts as the tetanus-diphtheria booster shot that is recommended every 10 years.

The patient described above is due for his tetanus-diphtheria booster and so should be given Tdap.

MEASLES, MUMPS, RUBELLA FOR THOSE BORN AFTER 1957

Measles remains a problem in the developing world, with an estimated average of 330 deaths daily. The number of cases fell 99% in the United States following the vaccination program that started in the early 1960s. Before the measles vaccine was available, an estimated 90% of children acquired measles by age 15.

The clinical syndrome consists of fever, conjunctivitis, cough, rash, and the characteristic Koplik spots—small white spots occurring on the inside of cheeks early in the disease course.

During the first 5 months of 2014, the CDC reported 334 cases of measles in the United States in 18 states, with most people affected being unvaccinated.³ In comparison, from 2001 to 2008, the number of cases averaged 56 annually.

Many of the recent cases were associated with infections brought from the Philippines. The increased number of measles cases underscores the need for vaccination to prevent measles and its complications.

Mumps is an acute, self-limited viral syndrome, and parotitis is the hallmark. Vaccination led to a 99% decline in cases in the United States. Although complications are rare, they can be serious and include orchitis (with risk of sterility), meningoencephalitis, and deafness.

Mumps outbreaks still occur, especially in close-contact settings such as schools, colleges, and camps. During the first half of 2014, central Ohio had more than 400 cases linked to The Ohio State University.

Rubella, also known as German measles, is a generally mild infection but is associated with congenital rubella syndrome. If a woman is infected with rubella in the first trimester of pregnancy, the risk of miscarriage is greater than 80%, as is the risk of birth defects, including hearing loss, developmental delay, growth retardation, and cardiac and eye defects.

Recommendations for MMR vaccination. People born before 1957 are considered immune to measles and usually to mumps. Health care workers should document immunity before assuming no vaccination is needed.

People born in 1957 or after should have one dose of MMR vaccine unless immunity is documented or unless there is a contraindication such as immunosuppression. A second dose is recommended for those born in or after 1957 who are considered to be at high risk: eg, health care workers, students entering college, and international travelers. The second dose should be given 4 weeks after the first.

Women of childbearing age should be screened for immunity to rubella. Susceptible women should receive MMR, although not during pregnancy and not if they may get pregnant within 4 weeks.

The patient described above was born before 1957, and so he is probably immune to measles and mumps.

HEPATITIS B FOR THOSE AT RISK

Hepatitis B vaccination is recommended for all adolescents and adults at increased risk: eg, men who have sex with men, intravenous drug users, people with multiple sexual partners, health care workers, patients with end-stage renal disease on hemodialysis, patients with chronic liver disease, and those with diabetes (age < 60).

Immunization consists of a series of three shots (at 0, 1–2, and 4–6 months). Booster doses are not recommended. Postvaccination testing for immunity is available and is recommended for health care workers, patients on hemodialysis, patients with HIV infection or who are otherwise immunocompromised, and sexual partners of people who are positive for hepatitis B surface antigen. Nonresponders should be revaccinated with the entire three-shot schedule. Hepatitis B vaccination is safe in pregnancy.

The patient described above has diabetes and so is a candidate for vaccination.

HEPATITIS A: A SLIGHTLY DIFFERENT RISK GROUP

Hepatitis A vaccination is recommended only for at-risk populations: international travelers; intravenous drug users; men who have sex with men; patients with clotting disorders, chronic liver disease, or hepatitis C infection; international adoptees; and laboratory personnel working with hepatitis A virus. The vaccination is given in two doses with a minimum interval of 6 months between doses.

A hepatitis A and hepatitis B combination vaccine (Twinrix) is also available. It is given in three doses, at 0, 1, and 6 months.

ANNUAL INFLUENZA VACCINE FOR ALL

In 2010, the ACIP recommended a policy of universal annual vaccination for everyone age 6 months and older. Some patients are at especially high risk themselves or are at high risk of exposing others and so are given higher priority during vaccine shortages—ie, patients who are immunosuppressed or have other medical risk factors, health care workers, household members of at-risk patients, and pregnant women after 13 weeks of gestation.

There are few contraindications, so almost everyone should be encouraged to receive the influenza vaccine. The flu shot does not cause the flu, but it may cause soreness at the injection site. Those with severe egg allergy should not receive the standard flu shot; a recombinant vaccine that does not use egg culture is available.

The standard flu shot is an inactivated influenza vaccine. In the past, most formulations were trivalent, but quadrivalent formulations are becoming more common. Special high-dose formulations are believed to elicit a better immune response and can be recommended for people over age 65. Intradermal and intramuscular formulations are available.

An intranasal live-attenuated influenza vaccine is also available and may be used for people ages 2 through 49. It should not be given to immunosuppressed people or to pregnant women.

Our patient should get a flu shot.

PNEUMOCOCCAL VACCINE FOR THOSE AGE 65 AND OLDER OR AT RISK

Two formulations are now available for pneumococcal immunization. The standard is a 23-valent polysaccharide vaccine (PPSV23; Pneumovax) indicated for people age 65 and older.

Patients under age 65 can receive PPSV23 if they have chronic lung disease, chronic cardiovascular disease, diabetes, chronic liver disease, or alcoholism or are a resident of a nursing home or an active smoker.

Our patient is a candidate for PPSV23 since he smokes and has diabetes.

The other formulation is a conjugate 13-valent vaccine (PCV13; Prevnar 13). Patients over age 19 at high risk should be given PCV13 plus the PPSV23 8 weeks later. Those who already received PPSV23 should be given PCV13 vaccine more than 1 year later. Candidates for PCV13 are those with immunocompromising conditions (including chronic renal failure and nephrotic syndrome), functional or anatomic asplenia, cerebrospinal fluid leaks, or cochlear implants.

The current revaccination schedule for PPSV23 is as follows:

• One-time revaccination 5 years after the first dose in patients with chronic renal failure, nephrotic syndrome, asplenia, or an immunosuppressive condition
• One-time revaccination for patients age 65 or older if they were younger than 65 when first immunized (with one or two doses of PPSV23) and 5 years have passed
• No revaccination is needed for people vaccinated with PPSV23 after age 65.

HUMAN PAPILLOMAVIRUS VACCINE

Human papillomavirus is the most common sexually transmitted infection in the United States and is strongly associated with cervical cancer. Immunization is now indicated for both sexes, generally between the ages of 9 and 26. Two vaccines are available: the quadrivalent formulation (Gardasil) for males or females and the bivalent formulation (Cervarix) for females only.

Immunization should be given in three doses: at 0, 1 to 2 months, and 6 months. It can be given to patients who are immunocompromised as a result of infection (including HIV infection), disease, or medications, or who have a history of genital warts, an abnormal Papanicolaou test, or a positive human papillomavirus DNA test.

It is hoped that immunization will lead to a significant decrease in cervical cancer rates. Eradication is unlikely because other papillomavirus strains also can lead to cancer, so cancer screening is still warranted. For men who have sex with men, it is hoped that immunization will prevent condyloma and anal cancer.

CHICKENPOX AND SHINGLES VACCINES

Varicella vaccine (Varivax) contains a live-attenuated virus to protect against chickenpox. It is recommended for all adults who have no evidence of immunity. Immunity is assumed with a history of chickenpox, being born before 1980, or having positive titers. Vaccination should be emphasized for those who come in contact with patients at high risk of severe disease (eg, health care workers, family contacts of immunocompromised patients) and in individuals with a high risk of personal exposure (eg, teachers, day care workers).

The vaccine is given in two doses, 4 to 8 weeks apart. Women who are pregnant or who may get pregnant within 4 weeks should not be vaccinated.

The shingles vaccine (Zostavax) is a larger dose of the varicella vaccine and reduces the incidence of shingles by 50% and postherpetic neuralgia by 66%.⁴ It was approved by the US Food and Drug Administration in May 2006 for people starting at age 50, but was recommended by ACIP in October 2006 for people age 60 and older; as a result, some insurance companies deny coverage for patients ages 50 through 59.

The shingles vaccine can be given to patients who have already had shingles. Pregnancy and severe immunodeficiency are contraindications.

Our patient, 58 years old, could be considered for shingles vaccine if covered by his insurance company or if he wishes to pay for it.

MENINGOCOCCUS VACCINE

Meningococcal immunization is recommended for people at high risk: college students who plan to live in dormitories, adults without a spleen or with complement deficiencies or HIV infection, or travelers to the “meningitis belt” of sub-Saharan Africa.

Two types of meningococcal vaccine are available: the conjugate quadrivalent vaccine (MCV4) for people age 55 and younger, and the polysaccharide quadrivalent vaccine (MPSV4) for people over age 56.

A 58-year-old man with a history of irritable bowel syndrome and diabetes presents for an evaluation in early November. He is taking metformin and insulin glargine 10 units. He smokes 1 pack per day. He believes that his childhood immunizations were completed, but he has no records. He thinks his last “shot” was 15 years ago when he cut his hand on some wood.

Which immunizations, if any, would be most appropriate for this patient?

The explosion of new vaccines, new formulations, and new combinations made available in recent years makes managing immunizations a challenge. This article reviews common immunizations and current recommendations for their appropriate use.

Immunization recommendations (Table 1) are made predominantly by the Advisory Committee on Immunization Practices (ACIP) of the US Centers for Disease Control and Prevention (CDC). The last 15 years have seen the arrival of new vaccines (eg, varicella, hepatitis A, pneumococcal, and human papillomavirus), new formulations (eg, intranasal influenza), and new combinations.

To keep clinicians abreast of new indications, the ACIP issues immunization schedules annually for children and adults, available online and downloadable for easy reference.¹ For adults, the ACIP provides schedules based on age and medical condition. The schedule for medical conditions offers specific information regarding immunization and pregnancy, human immunodeficiency virus (HIV) infection, kidney failure, heart disease, asplenia, and other conditions. The ACIP also provides guidance on contraindications; for example, pregnant and immunocompromised patients should not receive the live-attenuated vaccines, ie, zoster, varicella, and combined measles, mumps, and rubella [MMR]).

Adult awareness of vaccines is low, as are vaccination rates: in people older than 60, the vaccination rate is about 70% for influenza, 60% for pneumococcus, 50% for tetanus, and 15% for zoster. The lack of vaccine awareness and the availability of new vaccines and indications have made it difficult to manage immunizations in the primary care setting. The electronic medical record is useful for tracking patient vaccine needs. Ideally, keeping up with immunizations should be a routine part of visits provided by a physician’s care team and does not always require direct physician coordination.

TETANUS, DIPHTHERIA, PERTUSSIS EVERY 10 YEARS

Tetanus (also called “lockjaw”) is a nervous system disorder characterized by muscle spasms. Caused by infection with Clostridium tetani, it is a rare disease in the United States thanks to widespread immunization, and it causes fewer than 50 cases annually. Worldwide, the incidence is about 1 million cases a year with 200,000 to 300,000 deaths.

Diphtheria (formerly sometimes called “throat distemper”) is caused by the gram-positive bacillus Corynebacterium diphtheriae and can occur as a respiratory illness or as a milder cutaneous form. The last outbreak in the United States was in Seattle in the 1970s, with the last reported case in 2003. The ACIP recommends booster shots for tetanus and diphtheria every 10 years following completion of the primary series.

Pertussis or whooping cough, caused by Bordetella pertussis infection, is a highly contagious disease increasingly seen in adults in the United States. It causes few deaths but high morbidity, with coughing that can persist up to 3 months. Coughing can be severe enough to cause vomiting, a characteristic sign.

In July 2012, the CDC reported that the United States was at a 50-year high for pertussis, with 18,000 cases reported and 8 deaths.² In Washington State alone, more than 2,520 cases had been seen through June 16 of that year, a 1,300% increase over the previous year. Rates were high in older children and adolescents despite previous vaccination, suggesting an early waning of immunity.

The ACIP recommends a single dose of the combination of high-dose tetanus and low-dose diphtheria and pertussis vaccines (Tdap) for all adults regardless of age and for all pregnant women with each pregnancy between 27 and 36 weeks of gestation. A dose of Tdap counts as the tetanus-diphtheria booster shot that is recommended every 10 years.

The patient described above is due for his tetanus-diphtheria booster and so should be given Tdap.

MEASLES, MUMPS, RUBELLA FOR THOSE BORN AFTER 1957

Measles remains a problem in the developing world, with an estimated average of 330 deaths daily. The number of cases fell 99% in the United States following the vaccination program that started in the early 1960s. Before the measles vaccine was available, an estimated 90% of children acquired measles by age 15.

The clinical syndrome consists of fever, conjunctivitis, cough, rash, and the characteristic Koplik spots—small white spots occurring on the inside of cheeks early in the disease course.

During the first 5 months of 2014, the CDC reported 334 cases of measles in the United States in 18 states, with most people affected being unvaccinated.³ In comparison, from 2001 to 2008, the number of cases averaged 56 annually.

Many of the recent cases were associated with infections brought from the Philippines. The increased number of measles cases underscores the need for vaccination to prevent measles and its complications.

Mumps is an acute, self-limited viral syndrome, and parotitis is the hallmark. Vaccination led to a 99% decline in cases in the United States. Although complications are rare, they can be serious and include orchitis (with risk of sterility), meningoencephalitis, and deafness.

Mumps outbreaks still occur, especially in close-contact settings such as schools, colleges, and camps. During the first half of 2014, central Ohio had more than 400 cases linked to The Ohio State University.

Rubella, also known as German measles, is a generally mild infection but is associated with congenital rubella syndrome. If a woman is infected with rubella in the first trimester of pregnancy, the risk of miscarriage is greater than 80%, as is the risk of birth defects, including hearing loss, developmental delay, growth retardation, and cardiac and eye defects.

Recommendations for MMR vaccination. People born before 1957 are considered immune to measles and usually to mumps. Health care workers should document immunity before assuming no vaccination is needed.

People born in 1957 or after should have one dose of MMR vaccine unless immunity is documented or unless there is a contraindication such as immunosuppression. A second dose is recommended for those born in or after 1957 who are considered to be at high risk: eg, health care workers, students entering college, and international travelers. The second dose should be given 4 weeks after the first.

Women of childbearing age should be screened for immunity to rubella. Susceptible women should receive MMR, although not during pregnancy and not if they may get pregnant within 4 weeks.

The patient described above was born before 1957, and so he is probably immune to measles and mumps.

HEPATITIS B FOR THOSE AT RISK

Hepatitis B vaccination is recommended for all adolescents and adults at increased risk: eg, men who have sex with men, intravenous drug users, people with multiple sexual partners, health care workers, patients with end-stage renal disease on hemodialysis, patients with chronic liver disease, and those with diabetes (age < 60).

Immunization consists of a series of three shots (at 0, 1–2, and 4–6 months). Booster doses are not recommended. Postvaccination testing for immunity is available and is recommended for health care workers, patients on hemodialysis, patients with HIV infection or who are otherwise immunocompromised, and sexual partners of people who are positive for hepatitis B surface antigen. Nonresponders should be revaccinated with the entire three-shot schedule. Hepatitis B vaccination is safe in pregnancy.

The patient described above has diabetes and so is a candidate for vaccination.

HEPATITIS A: A SLIGHTLY DIFFERENT RISK GROUP

Hepatitis A vaccination is recommended only for at-risk populations: international travelers; intravenous drug users; men who have sex with men; patients with clotting disorders, chronic liver disease, or hepatitis C infection; international adoptees; and laboratory personnel working with hepatitis A virus. The vaccination is given in two doses with a minimum interval of 6 months between doses.

A hepatitis A and hepatitis B combination vaccine (Twinrix) is also available. It is given in three doses, at 0, 1, and 6 months.

ANNUAL INFLUENZA VACCINE FOR ALL

In 2010, the ACIP recommended a policy of universal annual vaccination for everyone age 6 months and older. Some patients are at especially high risk themselves or are at high risk of exposing others and so are given higher priority during vaccine shortages—ie, patients who are immunosuppressed or have other medical risk factors, health care workers, household members of at-risk patients, and pregnant women after 13 weeks of gestation.

There are few contraindications, so almost everyone should be encouraged to receive the influenza vaccine. The flu shot does not cause the flu, but it may cause soreness at the injection site. Those with severe egg allergy should not receive the standard flu shot; a recombinant vaccine that does not use egg culture is available.

The standard flu shot is an inactivated influenza vaccine. In the past, most formulations were trivalent, but quadrivalent formulations are becoming more common. Special high-dose formulations are believed to elicit a better immune response and can be recommended for people over age 65. Intradermal and intramuscular formulations are available.

An intranasal live-attenuated influenza vaccine is also available and may be used for people ages 2 through 49. It should not be given to immunosuppressed people or to pregnant women.

Our patient should get a flu shot.

PNEUMOCOCCAL VACCINE FOR THOSE AGE 65 AND OLDER OR AT RISK

Two formulations are now available for pneumococcal immunization. The standard is a 23-valent polysaccharide vaccine (PPSV23; Pneumovax) indicated for people age 65 and older.

Patients under age 65 can receive PPSV23 if they have chronic lung disease, chronic cardiovascular disease, diabetes, chronic liver disease, or alcoholism or are a resident of a nursing home or an active smoker.

Our patient is a candidate for PPSV23 since he smokes and has diabetes.

The other formulation is a conjugate 13-valent vaccine (PCV13; Prevnar 13). Patients over age 19 at high risk should be given PCV13 plus the PPSV23 8 weeks later. Those who already received PPSV23 should be given PCV13 vaccine more than 1 year later. Candidates for PCV13 are those with immunocompromising conditions (including chronic renal failure and nephrotic syndrome), functional or anatomic asplenia, cerebrospinal fluid leaks, or cochlear implants.

The current revaccination schedule for PPSV23 is as follows:

• One-time revaccination 5 years after the first dose in patients with chronic renal failure, nephrotic syndrome, asplenia, or an immunosuppressive condition
• One-time revaccination for patients age 65 or older if they were younger than 65 when first immunized (with one or two doses of PPSV23) and 5 years have passed
• No revaccination is needed for people vaccinated with PPSV23 after age 65.

HUMAN PAPILLOMAVIRUS VACCINE

Human papillomavirus is the most common sexually transmitted infection in the United States and is strongly associated with cervical cancer. Immunization is now indicated for both sexes, generally between the ages of 9 and 26. Two vaccines are available: the quadrivalent formulation (Gardasil) for males or females and the bivalent formulation (Cervarix) for females only.

Immunization should be given in three doses: at 0, 1 to 2 months, and 6 months. It can be given to patients who are immunocompromised as a result of infection (including HIV infection), disease, or medications, or who have a history of genital warts, an abnormal Papanicolaou test, or a positive human papillomavirus DNA test.

It is hoped that immunization will lead to a significant decrease in cervical cancer rates. Eradication is unlikely because other papillomavirus strains also can lead to cancer, so cancer screening is still warranted. For men who have sex with men, it is hoped that immunization will prevent condyloma and anal cancer.

CHICKENPOX AND SHINGLES VACCINES

Varicella vaccine (Varivax) contains a live-attenuated virus to protect against chickenpox. It is recommended for all adults who have no evidence of immunity. Immunity is assumed with a history of chickenpox, being born before 1980, or having positive titers. Vaccination should be emphasized for those who come in contact with patients at high risk of severe disease (eg, health care workers, family contacts of immunocompromised patients) and in individuals with a high risk of personal exposure (eg, teachers, day care workers).

The vaccine is given in two doses, 4 to 8 weeks apart. Women who are pregnant or who may get pregnant within 4 weeks should not be vaccinated.

The shingles vaccine (Zostavax) is a larger dose of the varicella vaccine and reduces the incidence of shingles by 50% and postherpetic neuralgia by 66%.⁴ It was approved by the US Food and Drug Administration in May 2006 for people starting at age 50, but was recommended by ACIP in October 2006 for people age 60 and older; as a result, some insurance companies deny coverage for patients ages 50 through 59.

The shingles vaccine can be given to patients who have already had shingles. Pregnancy and severe immunodeficiency are contraindications.

Our patient, 58 years old, could be considered for shingles vaccine if covered by his insurance company or if he wishes to pay for it.

MENINGOCOCCUS VACCINE

Meningococcal immunization is recommended for people at high risk: college students who plan to live in dormitories, adults without a spleen or with complement deficiencies or HIV infection, or travelers to the “meningitis belt” of sub-Saharan Africa.

Two types of meningococcal vaccine are available: the conjugate quadrivalent vaccine (MCV4) for people age 55 and younger, and the polysaccharide quadrivalent vaccine (MPSV4) for people over age 56.

A 45-year-old man with chronic alcoholism for the past 20 years and chronic liver disease for the past 2 years was admitted to the hospital with abdominal distention, yellowish discoloration of the eyes, itching all over the body, decreased appetite, and fresh rectal bleeding.

He had the classic signs of chronic liver disease, including icterus, pallor, parotid swelling, gynecomastia, spider angiomata, sparse axillary and pubic hair, transverse stretched umbilicus, divarication of the rectus abdominis muscles, caput medusae, small testes, and bilateral pedal edema.

Examination of the fingernails revealed a distal thin pink-to-brown transverse band 0.5 to 2.0 mm in width, a white nail bed, and no lunula (Figure 1)—the characteristic findings of Terry nails.

Systemic examination revealed moderate ascites (shifting dullness present), splenomegaly, and external hemorrhoids suggestive of portal hypertension.

TERRY NAILS

In 1954, Dr. Richard Terry first reported the finding of a white nail bed with ground-glass opacity in patients who had hepatic cirrhosis.¹ The condition is bilaterally symmetrical, with a tendency to be more marked in the thumb and forefinger.¹

In 1984, Holzberg and Walker² consecutively studied 512 hospitalized patients and observed Terry nails in 25.2% of them. Based on their findings, they redefined the criteria for Terry nail as follows:

• Distal thin pink-to-brown transverse band, 0.5 to 3.0 mm in width
• Decreased venous return not obscuring the distal band
• White or light pink proximal nail
• Lunula possibly absent
• At least 4 of 10 nails with the above criteria.

Patients who do not have the findings on all fingernails commonly have involvement of the thumb and forefinger. Holzberg and Walker confirmed a statistically significant association of Terry nails with cirrhosis, chronic heart failure, and adult-onset diabetes, especially in younger patients.² Terry nails have also been observed in thyrotoxicosis, pulmonary eosinophilia, malnutrition, actinic keratosis, and advanced age.^1–4

Using the updated diagnostic criteria, Park et al⁵ studied fingernails in 444 medical inpatients with chronic systemic disease, and only 30.6% had Terry nails. There were statistically significant associations with cirrhosis (57%), congestive heart failure (51.5%), and diabetes mellitus (49%); the associations with chronic renal failure (19%) and cancer (18%) were not statistically significant.⁵ They were more common in older patients. The average number of nails affected per patient tended to be higher in frequency close to the thumb; 28.7% patients had all nails affected.⁵

Terry nails should alert the clinician to the possibility of an underlying systemic disease, especially advanced liver disease. Possible explanations for the clinical changes observed in Terry nails include abnormal steroid metabolism, abnormal estrogen-androgen ratio, alteration of nail bed-to-nail plate attachment, hypoalbuminemia, increased digital blood flow, and overgrowth of the connective tissue between nail bed and the growth plate. The pathologic study of longitudinal nail biopsy specimens shows telangiectasia in the upper dermis of the distal nail band.³

Important differential diagnoses are Lindsay (half-and-half) nails, associated with chronic renal failure, and Neapolitan nails, associated with aging.³

A 45-year-old man with chronic alcoholism for the past 20 years and chronic liver disease for the past 2 years was admitted to the hospital with abdominal distention, yellowish discoloration of the eyes, itching all over the body, decreased appetite, and fresh rectal bleeding.

He had the classic signs of chronic liver disease, including icterus, pallor, parotid swelling, gynecomastia, spider angiomata, sparse axillary and pubic hair, transverse stretched umbilicus, divarication of the rectus abdominis muscles, caput medusae, small testes, and bilateral pedal edema.

Examination of the fingernails revealed a distal thin pink-to-brown transverse band 0.5 to 2.0 mm in width, a white nail bed, and no lunula (Figure 1)—the characteristic findings of Terry nails.

Systemic examination revealed moderate ascites (shifting dullness present), splenomegaly, and external hemorrhoids suggestive of portal hypertension.

TERRY NAILS

In 1954, Dr. Richard Terry first reported the finding of a white nail bed with ground-glass opacity in patients who had hepatic cirrhosis.¹ The condition is bilaterally symmetrical, with a tendency to be more marked in the thumb and forefinger.¹

In 1984, Holzberg and Walker² consecutively studied 512 hospitalized patients and observed Terry nails in 25.2% of them. Based on their findings, they redefined the criteria for Terry nail as follows:

• Distal thin pink-to-brown transverse band, 0.5 to 3.0 mm in width
• Decreased venous return not obscuring the distal band
• White or light pink proximal nail
• Lunula possibly absent
• At least 4 of 10 nails with the above criteria.

Patients who do not have the findings on all fingernails commonly have involvement of the thumb and forefinger. Holzberg and Walker confirmed a statistically significant association of Terry nails with cirrhosis, chronic heart failure, and adult-onset diabetes, especially in younger patients.² Terry nails have also been observed in thyrotoxicosis, pulmonary eosinophilia, malnutrition, actinic keratosis, and advanced age.^1–4

Using the updated diagnostic criteria, Park et al⁵ studied fingernails in 444 medical inpatients with chronic systemic disease, and only 30.6% had Terry nails. There were statistically significant associations with cirrhosis (57%), congestive heart failure (51.5%), and diabetes mellitus (49%); the associations with chronic renal failure (19%) and cancer (18%) were not statistically significant.⁵ They were more common in older patients. The average number of nails affected per patient tended to be higher in frequency close to the thumb; 28.7% patients had all nails affected.⁵

Terry nails should alert the clinician to the possibility of an underlying systemic disease, especially advanced liver disease. Possible explanations for the clinical changes observed in Terry nails include abnormal steroid metabolism, abnormal estrogen-androgen ratio, alteration of nail bed-to-nail plate attachment, hypoalbuminemia, increased digital blood flow, and overgrowth of the connective tissue between nail bed and the growth plate. The pathologic study of longitudinal nail biopsy specimens shows telangiectasia in the upper dermis of the distal nail band.³

Important differential diagnoses are Lindsay (half-and-half) nails, associated with chronic renal failure, and Neapolitan nails, associated with aging.³

A 45-year-old man with chronic alcoholism for the past 20 years and chronic liver disease for the past 2 years was admitted to the hospital with abdominal distention, yellowish discoloration of the eyes, itching all over the body, decreased appetite, and fresh rectal bleeding.

He had the classic signs of chronic liver disease, including icterus, pallor, parotid swelling, gynecomastia, spider angiomata, sparse axillary and pubic hair, transverse stretched umbilicus, divarication of the rectus abdominis muscles, caput medusae, small testes, and bilateral pedal edema.

Examination of the fingernails revealed a distal thin pink-to-brown transverse band 0.5 to 2.0 mm in width, a white nail bed, and no lunula (Figure 1)—the characteristic findings of Terry nails.

Systemic examination revealed moderate ascites (shifting dullness present), splenomegaly, and external hemorrhoids suggestive of portal hypertension.

TERRY NAILS

In 1954, Dr. Richard Terry first reported the finding of a white nail bed with ground-glass opacity in patients who had hepatic cirrhosis.¹ The condition is bilaterally symmetrical, with a tendency to be more marked in the thumb and forefinger.¹

In 1984, Holzberg and Walker² consecutively studied 512 hospitalized patients and observed Terry nails in 25.2% of them. Based on their findings, they redefined the criteria for Terry nail as follows:

• Distal thin pink-to-brown transverse band, 0.5 to 3.0 mm in width
• Decreased venous return not obscuring the distal band
• White or light pink proximal nail
• Lunula possibly absent
• At least 4 of 10 nails with the above criteria.

Patients who do not have the findings on all fingernails commonly have involvement of the thumb and forefinger. Holzberg and Walker confirmed a statistically significant association of Terry nails with cirrhosis, chronic heart failure, and adult-onset diabetes, especially in younger patients.² Terry nails have also been observed in thyrotoxicosis, pulmonary eosinophilia, malnutrition, actinic keratosis, and advanced age.^1–4

Using the updated diagnostic criteria, Park et al⁵ studied fingernails in 444 medical inpatients with chronic systemic disease, and only 30.6% had Terry nails. There were statistically significant associations with cirrhosis (57%), congestive heart failure (51.5%), and diabetes mellitus (49%); the associations with chronic renal failure (19%) and cancer (18%) were not statistically significant.⁵ They were more common in older patients. The average number of nails affected per patient tended to be higher in frequency close to the thumb; 28.7% patients had all nails affected.⁵

Terry nails should alert the clinician to the possibility of an underlying systemic disease, especially advanced liver disease. Possible explanations for the clinical changes observed in Terry nails include abnormal steroid metabolism, abnormal estrogen-androgen ratio, alteration of nail bed-to-nail plate attachment, hypoalbuminemia, increased digital blood flow, and overgrowth of the connective tissue between nail bed and the growth plate. The pathologic study of longitudinal nail biopsy specimens shows telangiectasia in the upper dermis of the distal nail band.³

Important differential diagnoses are Lindsay (half-and-half) nails, associated with chronic renal failure, and Neapolitan nails, associated with aging.³