22.1 Head and Neck

The Head

The head is a complex anatomical region that houses the brain, sensory organs, and various structures responsible for functions such as eating, breathing, and communication. Beyond housing the brain, the head houses the sensory organs responsible for vision, hearing, smell, taste, and touch. To better understand the functional significance of the head, let us further explore the anatomy of the cranium and face.

Cranium

The cranium, commonly known as the skull, is the bony structure that encloses and protects the brain as well as various sensory organs and structures in the head. It is composed of several bones (Figure 22.2) that are connected by sutures, which are fibrous joints that allow for limited movement during childbirth and growth but eventually fuse to provide stability and protection. The main bones that make up the cranium include the following:

• Frontal bone: The frontal bone forms the forehead and the upper portion of the eye sockets (or orbits). It also contains the frontal sinuses, which are air-filled cavities that contribute to the resonance of the voice.
• Parietal bones (left and right): The parietal bones form the majority of the upper sides and the roof of the cranium. They are joined at the midline by the sagittal suture.
• Temporal bones (left and right): The temporal bones are located on the sides and base of the skull. They contain the ear structures, including the external auditory canal, middle ear cavity, and inner ear. The temporal bones also have an important role in housing the mandibular fossa, where the lower jaw articulates.
• Occipital bone: The occipital bone forms the back and base of the cranium. It contains the foramen magnum, a large opening through which the spinal cord passes to connect to the brain. The occipital bone also has attachment points for neck muscles.
• Sphenoid bone: The sphenoid bone is a complex bone that sits at the base of the skull, contributing to the sides of the skull, the base of the cranium, and the posterior portion of the orbits. It houses the pituitary gland and contains several important foramina (openings) for nerves and blood vessels.
• Ethmoid bone: The ethmoid bone is located between the eye sockets and forms part of the nasal cavity. It contains thin plates that contribute to the structure of the nasal septum and the walls of the nasal cavity.

Figure 22.2 The intricate arrangement of the cranial bones, with this figure highlighting their unique shapes and connections in the human skull, protects and supports the brain. (credit: modification of work from Anatomy and Physiology. attribution: Copyright Rice University, OpenStax, under CC BY 4.0 license)

These bones protect the brain and provide structural support for the head. Additionally, they provide attachment points for muscles that control facial expressions, mastication (chewing), and various head movements. The openings within the cranial bones allow for the passage of blood vessels, nerves, and other important structures that connect the brain with the rest of the body.

In a newborn, the pressure from vaginal delivery compresses the head and causes the bony plates to overlap at the sutures (Figure 22.3), creating a small ridge. Over the next few days after birth, the head expands, the overlapping disappears, and the edges of the bony plates meet edge to edge. This is the normal position for the remainder of the life span, and the sutures become immobile. There are several main cranial sutures:

• Coronal suture: The coronal suture runs horizontally across the skull, connecting the frontal bone to the parietal bones on each side. It is located at the anterior (front) portion of the skull.
• Sagittal suture: The sagittal suture runs vertically along the midline of the skull, connecting the two parietal bones. It divides the skull into right and left halves.
• Lambdoid suture: The lambdoid suture is located at the posterior (back) part of the skull. It separates the occipital bone from the parietal bones on each side. The suture’s shape resembles the Greek letter “lambda” (Λ).
• Squamous sutures: There are two squamous sutures on each side of the skull:
  • Temporal squamous suture: This suture separates the temporal bone from the parietal bone on each side.
  • Pterion: The pterion is a point where the frontal, parietal, sphenoid, and temporal bones meet. It is an important landmark because the middle meningeal artery lies beneath it and can be vulnerable to injury in head trauma.

Figure 22.3 Cranial sutures connect the cranial bones together. (credit: modification of work from Anatomy and Physiology. attribution: Copyright Rice University, OpenStax, under CC BY 4.0 license)

Face

In addition to the cranial bones, the cranium includes the facial bones, which contribute to the formation of the face and its features. These facial bones include the orbit, nasal bones, mandible, maxilla, and zygomatic bones. The orbit is the bony socket that houses the eyeball and the muscles that move the eyeball. Inside the nasal area of the skull, the nasal cavity is divided into halves by the nasal septum that consists of both bone and cartilage components. The mandible forms the lower jaw and is the only movable bone in the skull. The maxilla forms the upper jaw and supports the upper teeth. The zygomatic bone, also known as the cheekbone, functions as a structure that joins the bones of the face while protecting the arteries, nerves, veins, and organs below the surface. Together, the cranial and facial bones create a complex and protective framework for the structures within the head.

The facial muscles are a group of muscles that control the movements of the face, allowing us to express emotions, communicate, and perform various facial actions. There are around forty-three facial muscles that are arranged in intricate patterns to create the wide range of expressions from smiling and frowning, to raising an eyebrow or wrinkling the forehead. Several nerves innervate the facial muscles to create facial expressions. Figure 22.4 illustrates the nerves that affect the facial muscles. These nerves and muscles are tested during a cranial nerve examination. For example, when a patient is experiencing a cerebrovascular accident (e.g., stroke), it is common for facial drooping to occur. In facial drooping, an asymmetrical facial expression occurs due to damage of the nerve innervating a specific part of the face (Figure 22.5).

Figure 22.4 Nerve branches affecting the facial muscles. (credit: “Head facial nerve branches.jpg ” by Patrick J. Lynch/Wikimedia Commons, CC BY 2.5)

Figure 22.5 Facial droop, also known as ptosis, affecting the right side of the face. (credit: “Bell’s Palsy smiling” by “Shantoo”/Wikimedia Commons, CC BY 1.0)

Neck

The neck is the region of the body that connects the head to the shoulders and the rest of the torso. It contains various structures, including muscles, vertebrae, blood vessels, thyroid gland, and lymph nodes. The neck is a vital region of the body that supports critical functions, including head movement, breathing, swallowing, communication, circulation, and immune responses.

Muscles and Cervical Vertebrae

The neck muscles are a group of muscles located in the region between the head and the shoulders (Figure 22.6). The neck muscles can be broadly categorized into these groups:

• Anterior neck muscles:
  • Sternocleidomastoid: The sternocleidomastoid muscle runs diagonally from the base of the skull behind the ear to the sternum and clavicle. It allows rotation and flexion of the head and neck.
  • Scalene muscles: The scalene muscles are located on the sides of the neck and assist in various neck movements as well as in breathing.
• Posterior neck muscles:
  • Trapezius: The trapezius is a large muscle that extends from the base of the skull down to the upper back. It helps stabilize and move the shoulder blades and supports head movements.
  • Levator scapulae: Located along the side of the neck, the levator scapulae muscle helps elevate the shoulder blades.
• Lateral neck muscles:
  • Sternocleidomastoid: While primarily located on the anterior neck, the sternocleidomastoid muscle also contributes to lateral neck movements.

Figure 22.6 The neck muscles are a group of muscles located in the region between the head and the shoulders. (credit: modification of work from Anatomy and Physiology. attribution: Copyright Rice University, OpenStax, under CC BY 4.0 license)

These muscles are responsible for supporting and moving the head, facilitating breathing, and aiding in swallowing. The neck muscles contribute to maintaining good posture by supporting the head and upper body in an upright position and play a crucial role in allowing the head to move in different directions, including flexion (forward bending), extension (backward tilting), rotation (turning side to side), and lateral bending (tilting to the side). The neck muscles, especially the trapezius and levator scapulae, help stabilize the head, neck, and shoulders during various activities. Some neck muscles, particularly the scalene muscles, assist in the process of breathing by elevating the ribs during inhalation. Muscles in the neck work together to facilitate the swallowing process. They help move the larynx upward and forward to close off the airway while swallowing, preventing food or liquid from entering the trachea.

The cervical vertebrae consist of seven vertebrae that make up the upper part of the spine, forming the neck region. They are numbered C1 to C7, starting from the top, closest to the skull (Figure 22.7). Each cervical vertebra has specific anatomical features:

• Atlas (C1): The atlas is the first cervical vertebra that connects with the skull. It lacks a traditional vertebral body and instead has a ringlike structure that supports the skull’s weight and allows nodding movements.
• Axis (C2): The axis is the second cervical vertebra and connects with the atlas. It has a unique bony projection called the odontoid process (dens), which allows the head to pivot for side-to-side movements.
• C3 to C7: These cervical vertebrae have similar structure, with vertebral bodies, neural arches, and processes. They gradually increase in size from C3 to C7.

The cervical vertebrae provide structural support for the head and protect the spinal cord, which passes through the vertebral canal formed by the stacked vertebrae. The unique structure of the atlas and axis allows for a significant range of motion in the neck, enabling nodding and rotating movements. Cervical vertebrae serve as attachment points for various muscles, ligaments, and tendons involved in neck movement and stability.

Figure 22.7 The cervical vertebrae consist of seven vertebrae that make up the neck region of the upper part of the spine. (credit: modification of work “Blausen 0222 CervicalSpine” by "BruceBlaus"/Wikimedia Commons, CC BY 3.0)

Blood Vessels

The neck contains several important blood vessels that supply blood to the brain, head, and other regions (Figure 22.8). These blood vessels include arteries, which carry oxygenated blood away from the heart, and veins, which return deoxygenated blood back to the heart. The major neck blood vessels include the following:

• Carotid arteries:
  • Common carotid arteries: There are two common carotid arteries, one on each side of the neck. They branch off from the aorta (the main artery leaving the heart) and supply oxygenated blood to the head and neck. The carotid arteries can be felt on both sides of the neck as strong pulses. The carotid pulse is often used to assess heart rate and rhythm.
  • Internal carotid arteries: These arteries further divide into smaller branches within the skull to supply blood to the brain.
  • External carotid arteries: These arteries supply blood to the face, scalp, and neck muscles.
• Jugular veins:
  • Internal jugular veins: Paired internal jugular veins drain deoxygenated blood from the brain, face, and neck muscles. The internal jugular veins are positioned deeper in the neck and are not visible or palpable like the carotid arteries. They merge with the subclavian veins to form the brachiocephalic veins, which eventually lead to the superior vena cava, returning blood to the heart.
  • External jugular veins: These veins drain deoxygenated blood from the scalp and face. The external jugular veins are more superficial and can be seen as prominent veins on the sides of the neck. They typically run superficially along the sides of the neck.

Figure 22.8 Location of the blood vessels found within the neck. (credit: modification of work “Circulatory System en” by "LadyofHats"/Mariana Ruiz Villarreal/Wikimedia Commons, Public Domain)

The carotid arteries play an important role in oxygen and nutrient delivery by supplying oxygenated blood rich in nutrients to the brain, face, scalp, and neck muscles. The internal carotid arteries play a critical role in delivering oxygen and nutrients to the brain, supporting its metabolic needs. Blood flow through the neck vessels helps regulate body temperature by distributing heat away from or toward the skin’s surface. Blood vessels also play a role in carrying waste products, such as carbon dioxide and metabolic by-products, away from tissues for elimination and contribute to maintaining proper fluid balance in tissues by transporting fluid between the bloodstream and surrounding tissues. In the event of reduced blood flow through one carotid artery due to disease or blockage, the other carotid artery can provide collateral circulation to the brain.

Lymph Nodes of the Head and Neck

The lymphatic system is the system of vessels, cells, and organs that carries excess interstitial fluid to the bloodstream and filters pathogens from the blood through lymph nodes found near the neck, armpits, chest, abdomen, and groin. See Figure 22.9 and Figure 22.10 for illustrations of the lymph nodes found in the head and neck regions. When a person is fighting off an infection, the lymph nodes in that region become enlarged, indicating an active immune response to infection.

Figure 22.9 The lymphatic system maintains fluid balance, filters harmful substances, supports immune responses, and facilitates the transportation of nutrients and waste products. Its intricate network contributes to the body’s ability to fight infections, heal injuries, and maintain a balanced internal environment. (credit: modification of work from Anatomy and Physiology. attribution: Copyright Rice University, OpenStax, under CC BY 4.0 license)

Figure 22.10 Lymph nodes found within the head and neck play a crucial role in filtering lymph and facilitating immune responses. They help trap and destroy harmful substances such as bacteria, viruses, and abnormal cells, contributing to the body’s defense against infections and diseases. (credit: modification of work “Cervical lymph nodes and levels” by Mikael Häggström, M.D./Wikimedia Commons, Public Domain)

Subjective Data

Subjective data are self-reported experiences, feelings, and perceptions that cannot be directly measured. Begin the head and neck assessment by asking focused interview questions to determine whether the patient is currently experiencing any symptoms or has a previous medical history related to head and neck issues. Common interview questions to collect subjective assessment data of the head and neck can be found in Table 22.1.

Objective Data

Objective data are measurable and observable data that can be assessed through examination, tests, or observations. During a head and neck assessment, inspection involves visually examining the external structures of the head and neck for any abnormalities, asymmetry, swelling, skin changes, or signs of underlying conditions. Use any information obtained during the subjective interview to guide your physical assessment.

Observe the patient’s general appearance, noting their posture, facial expressions, and overall demeanor. Assess for signs of distress, discomfort, or unusual behavior. Examine the face for symmetry. Note any facial drooping, muscle weakness, or asymmetry that could indicate underlying neurological or muscular issues. Inspect the skin of the face, neck, and scalp for color, texture, and any lesions. Look for signs of rashes, bruising, moles, or lesions that may require further examination. Assess the eyes for any swelling, puffiness, or discoloration around the eyelids. Observe the position of the eyes and note any abnormalities in alignment. Check for any loss of hair from the eyebrows or eyelashes, which could indicate underlying conditions such as thyroid dysfunction. Assess the neck for symmetry, noting any visible masses, lumps, or swelling. Observe the trachea for proper midline alignment. Palpate gently for any enlarged or tender lymph nodes in the neck and behind the ears. Note their size, mobility, and tenderness. Examine the scalp for any lesions, lumps, or areas of tenderness. Check the hair for thickness, texture, and any signs of hair loss or bald patches. Observe the jaw’s movement as the patient opens and closes their mouth. Note any clicking, popping, or pain in the temporomandibular joint area in the jaw.

Validating and Documenting Findings

Validating and documenting assessment findings of the head and neck are essential steps in the nursing process to ensure accurate communication and informed decision-making. Subjective and objective findings should be cross-referenced to ensure the data collected are reliable and accurate. Data may be validated by repeating the assessment, asking additional questions to clarify data, and comparing the objective findings with the subjective findings to determine if any discrepancies are present. For example, additional assessment and questioning may be warranted if the patient reports not having a headache, yet they are holding their head with eyes squinted.

Documentation provides a clear record of the patient’s condition for future reference and collaboration among healthcare providers. Documentation should include both subjective and objective data. Subjective data may include findings such as: “Patient reports headache lasting five days,” “Patient reports headache rated 7/10 using the numerical pain scale,” or “Patient reports falling down the stairs and hitting their head.” When documenting subjective data, include direct quotes when possible. For example, document “Patient complains of headache that feels like someone is ‘squeezing their head’ for the past two days.” Objective data are collected through the physical assessment. Examples of documentation for objective data may include findings such as: “Trachea is midline,” “Face is symmetrical, no swelling or facial droop observed,” “Palpable lymph node behind left ear with tenderness noted,” or “Hair is thin and brittle with moderate hair loss noted.”

Headache

A headache is a common and uncomfortable sensation of pain or discomfort in the head or upper neck area. Headaches range greatly in severity of pain and frequency of occurrence. For example, some patients experience mild headaches once or twice a year, whereas others experience disabling migraine headaches on a monthly basis. Severe headaches, such as migraines, may be accompanied by symptoms of nausea or increased sensitivity to noise or light. Primary headaches occur independently and are not caused by another medical condition. Migraine, cluster (intense headaches for several weeks, followed by periods of remission), and tension-type headaches (described as a constant bandlike pressure around the head) are types of primary headaches. Secondary headaches are symptoms of another health disorder that causes pain-sensitive nerve endings to be pressed on or pulled out of place. An example of a secondary headache may be a sinus headache in which inflammation and congestion of the nasal passages result in pain in the forehead and around the eyes. Headaches are caused by factors such as stress, tension, hormonal changes (common in migraines), dehydration, lack of sleep or poor sleep quality, certain foods or drinks (like caffeine or alcohol), environmental factors (loud noise, bright lights), sinus infections, allergies, dental problems, hypertension, head injury or trauma, fever, and infection. A description of the different types of headaches can be found in Table 22.2.

Type of Headache	Description	Additional Information	Location
Primary Headaches
Tension	Dull; aching sensation; tenderness or sensitivity around the neck, forehead, scalp, or shoulder muscles	Often triggered by stress	(attribution: Copyright Rice University, OpenStax, under CC BY 4.0 license)
Cluster	Severe burning and piercing pain occurring around or behind one eye or on one side of the face	A series of headaches that reoccur over days or weeks	(attribution: Copyright Rice University, OpenStax, under CC BY 4.0 license)
Migraine	Intense pulsing pain deep in the head, throbbing pain usually on one side of the head, may cause light and sound sensitivity, may cause nausea and vomiting	May be triggered by sleep disruption, dehydration, some foods, hormone fluctuations, exposure to chemicals	(attribution: Copyright Rice University, OpenStax, under CC BY 4.0 license)
Secondary Headaches
Allergy or sinus	Pain in the sinus area and frontal portion of the head	Common in those with chronic seasonal allergies or sinusitis	(attribution: Copyright Rice University, OpenStax, under CC BY 4.0 license)
Hormone	Throbbing pain that starts on one side of the head, may also involve sensitivity to light and nausea or vomiting	Headache linked to hormonal fluctuations, such as those who menstruate, use birth control, or are pregnant	(attribution: Copyright Rice University, OpenStax, under CC BY 4.0 license)
Caffeine	Pain and pressure that started behind the eyes and pushes outward from the brain	Associated with too much caffeine or abruptly quitting caffeine	(attribution: Copyright Rice University, OpenStax, under CC BY 4.0 license)
Exertion	Throbbing on both sides of the head, resolves in minutes to hours	Occur after periods of intense physical activity, such as weightlifting and running	(attribution: Copyright Rice University, OpenStax, under CC BY 4.0 license)
Hypertension	Usually occurs on both sides of the head and is typically worse with any activity, often described as pulsating	Medical emergency signaling dangerously high blood pressure (usually greater than 180/120 mm Hg)	(attribution: Copyright Rice University, OpenStax, under CC BY 4.0 license)
Spinal	Pain in the forehead, temples, upper neck, and back of the head	Results from low cerebrospinal fluid pressure following a lumbar puncture	(attribution: Copyright Rice University, OpenStax, under CC BY 4.0 license)

Table 22.2 Types of Headaches

Not all headaches require medical attention, but some types of headaches can signify a serious disorder and require prompt medical care. Symptoms of headaches that require immediate medical attention include a sudden, severe headache unlike any the patient has ever had; a sudden headache associated with a stiff neck; a headache associated with convulsions, confusion, or loss of consciousness; a headache following a blow to the head; or a persistent headache in a person who was previously headache free.

Concussion

A concussion is a type of traumatic brain injury (TBI) that occurs when the brain experiences a sudden and forceful movement within the skull. This movement can result from a blow, bump, jolt, or any impact to the head or body that causes the brain to shake inside the skull. Concussions are commonly caused by accidents, falls, sports-related injuries, or any situation where the head or body experiences a rapid deceleration or acceleration; however, they can also occur from something as simple as running into a glass door.

Symptoms of a concussion include headache or pressure in the head, confusion or feeling dazed, temporary loss of consciousness, memory problems or difficulty concentrating, dizziness or balance issues, nausea or vomiting, sensitivity to light or noise, mood changes, irritability, and blurred vision. If any of these symptoms are present after a head injury, it is important to seek medical attention right away. Diagnostics may involve assessing the individual’s symptoms, performing a neurological examination, and consulting imaging tests (such as a computed tomography [CT] scan or magnetic resonance imaging [MRI]) to rule out more serious injuries. Treatment for concussions typically involves rest (both physical and cognitive), avoiding activities that could worsen symptoms (such as reading or screen time), gradually returning to activity, and closely monitoring the patient to ensure symptoms are improving and not worsening.

Head Injury

A head injury refers to any trauma or damage that occurs to the head, scalp, skull, or brain. Head injuries can range from minor bumps and bruises to more serious conditions like concussions or traumatic brain injury (TBI) (damage to the brain caused by a sudden, external force or trauma). Strong blows to the brain case of the skull can produce fractures resulting in bleeding inside the skull. A blow to the lateral side of the head may fracture the bones of the pterion. If the underlying artery is damaged, bleeding can cause the formation of a hematoma (collection of blood) between the brain and interior of the skull. As blood accumulates, it will put pressure on the brain. Symptoms associated with a hematoma may not be apparent immediately following the injury, but if untreated, blood accumulation will continue to exert increasing pressure on the brain and can result in death within a few hours.

If a head injury occurs, check for responsiveness and breathing. If the person is unconscious or having difficulty breathing, call for emergency medical help. If there is bleeding, apply gentle pressure with a clean cloth to stop it. Avoid pressing directly on the injury site if a skull fracture is suspected. If a head or neck injury is suspected, avoid moving the person’s head or neck until medical professionals arrive. Even seemingly minor head injuries should be evaluated by a healthcare professional, as symptoms can worsen over time. Some head injuries can have delayed symptoms, so monitoring for changes in behavior, consciousness, or symptoms is crucial.

Acromegaly

A rare hormonal disorder termed acromegaly occurs when the pituitary gland, located at the base of the brain, produces an excessive amount of growth hormone (GH). Typically, the cause of the excessive GH is due to a noncancerous tumor in the pituitary gland called an adenoma. This excess GH leads to the enlargement and overgrowth of certain body tissues, primarily bones and soft tissues in the hands, feet, face, and internal organs. Acromegaly usually develops slowly over time and becomes more noticeable with advanced age. Common signs and symptoms of acromegaly include enlarged hands and feet, gradual changes in facial features (such as a prominent forehead, enlarged nose, protruding jaw, and gaps between teeth), skin changes (such as thick, oily skin; skin tags; or excessive sweating); enlarged internal organs (such as the heart, liver, and kidneys), joint pain, deepened voice, carpal tunnel syndrome, tiredness and weakness, headaches, vision problems, and sleep apnea. Acromegaly is often diagnosed through a combination of physical examination, medical history review, blood tests to measure GH and insulin-like growth factor-1 (IGF-1) levels, and imaging studies like MRI or CT scans to identify if a pituitary adenoma is present.

Early diagnosis and treatment are important to prevent the symptoms from getting worse and reduce the chance of complications. Treatment options for acromegaly typically involve addressing the underlying cause, which is usually the pituitary adenoma. Treatment methods include surgery to remove the adenoma; medications such as somatostatin analogs (e.g., octreotide, lanreotide) and GH receptor antagonists (e.g., pegvisomant) to help control GH levels and alleviate symptoms; and radiation. Regular follow-up with medical professionals, including endocrinologists and specialists in pituitary disorders, is important for long-term management.

Cushing Syndrome

Cushing syndrome, also known as hypercortisolism, is characterized by an excessive and prolonged exposure to high levels of the hormone cortisol. Cortisol is produced by the adrenal glands, which are located on top of each kidney, and plays a vital role in regulating various bodily functions such as metabolism, immune response, and blood pressure. Common signs and symptoms of Cushing syndrome include weight gain, a round face, increased fat around the base of the neck, a fatty hump between the shoulders, thin arms and legs, muscle weakness and atrophy, easy bruising, slowed wound healing, stretch marks, high blood pressure, osteoporosis, mood swings, depression, glucose intolerance, increased hair growth, and irregular menstrual cycles (Figure 22.11).

Figure 22.11 Signs and symptoms of Cushing syndrome. (credit: modification of work “Cushing’s syndrome (vector image).svg” by Mikael Häggström/Wikimedia Commons, Public Domain)

The most common cause of Cushing syndrome is long-term use of high-dose cortisol, such as glucocorticoids and corticosteroids. Other times, people develop endogenous Cushing syndrome because their bodies make too much cortisol. Additionally, several types of tumors can cause the body to make excess cortisol, such as adrenal tumors and ectopic ACTH-producing tumors.

Diagnosis of Cushing syndrome involves a combination of clinical evaluation, hormone tests to measure cortisol and ACTH levels, and imaging studies like MRI or CT scans to identify potential tumors in the adrenal glands or pituitary gland. If a tumor is identified, its removal may be considered as a treatment option. Other treatment options may include cortisol-lowering medications or gradual steroid reduction. The goal of treatment is to normalize cortisol levels and alleviate the symptoms associated with Cushing syndrome.

Scleroderma

The chronic, autoimmune disorder scleroderma (also, systemic sclerosis) causes inflammation in the skin and can also affect various internal organs such as the heart, lungs, and bowels. A hallmark sign of scleroderma is the abnormal accumulation of collagen, a protein that forms the connective tissues in the body, formed by the immune system tricking the body into thinking it is injured, which leads to inflammation and subsequent collagen formation. This excessive collagen production leads to thickening, hardening, and tightening of the skin and connective tissues, as well as potential damage to organs.

Scleroderma affects many body systems. It can be categorized as a connective tissue disease, an autoimmune disease, and a rheumatic disease. A connective tissue disease affects tissues such as skin, tendons, and cartilage. An autoimmune disease occurs when the body attacks its own tissues. A rheumatic disease leads to inflammation and/or pain in the muscles, joints, or fibrous tissue.

Scleroderma can be classified as localized or systemic. Localized scleroderma affects the skin and underlying tissues. It results in patches of scleroderma, known as morphea, or a line of scleroderma that runs down the arm, leg, forehead, or face, known as linear scleroderma (Figure 22.12).

Figure 22.12 Scleroderma may present in patches (morphea) or lines (linear), as seen here. (credit: “En coup de sabre lesion” by National Library of Medicine, CC BY 2.0)

Systemic scleroderma affects the skin, blood vessels, and major organs. There are two main types of systemic scleroderma, limited or diffuse, depending on the degree of skin involvement. Limited scleroderma comes on gradually and typically involves the skin of the face, hands, and feet. It often begins with Raynaud phenomenon (Figure 22.13), which is characterized by the fingers and toes turning white or blue in response to cold temperatures or stress. Other symptoms can include skin thickening, difficulty swallowing, and mild lung and heart issues. Calcium deposits can form under the skin, particularly at the fingertips, causing bumps that can be seen on x-rays. Some people also experience telangiectasia, a condition caused by the swelling of blood vessels, which results in small red spots on their hands and face. Diffuse scleroderma affects a larger area of the skin, expanding above the knees and elbows, and can progress more rapidly. It can also involve internal organs such as the lungs, heart, kidneys, and digestive system. Scarring of the lung and heart tissues may lead to shortness of breath, hypertension, irregular heart rate, and congestive heart failure. Sudden increases in blood pressure may cause kidney crisis, which can lead to sudden kidney failure if prompt treatment is not provided. Digestive problems may lead to heartburn, difficulty swallowing, bloating, diarrhea, constipation, and fecal incontinence. In addition, individuals with systemic scleroderma often experience joint pain, restricted movement, muscle weakness, and fatigue.

Figure 22.13 Raynaud phenomenon causes the fingers to turn white or blue due to reduced blood flow. (credit: “Raynaud’s phenomenon 1” by “knotimpressed”/Wikimedia Commons, CC BY 1.0)

Anyone can get scleroderma, but there are several genetic and environmental factors associated with the condition. Although not an inheritable condition, those with a first-degree relative with scleroderma are at a higher risk of developing the condition. Women are more likely to develop scleroderma than men, most likely due to hormonal differences. Additional factors include abnormal immune or inflammatory conditions as well as exposure to viruses and chemicals.

There is no known cure for scleroderma. Treatment of the condition focuses on managing symptoms, preventing complications, and improving quality of life. Medications, physical therapy, and lifestyle modifications can help control symptoms and slow disease progression. In severe cases where internal organs are significantly affected, organ-specific treatments may be necessary. Regular medical monitoring is crucial for individuals with scleroderma to catch and manage any potential organ complications early. A multidisciplinary approach involving rheumatologists, dermatologists, pulmonologists, and other specialists is often needed to provide comprehensive care.

Bell Palsy

Bell palsy is a neurological disorder characterized by the sudden onset of facial muscle weakness or paralysis (Figure 22.14). It is considered a form of temporary facial nerve dysfunction (also known as the seventh cranial nerve) that leads to the inability to control facial muscles. Symptoms of Bell palsy can develop rapidly, often within forty-eight to seventy-two hours and may include sudden weakness or paralysis on one side of the face, drooping of the mouth and eyelid, drooling from one side of the mouth, and difficulty closing the eyelid. Typically, Bell palsy affects one side of the face, though there are rare occasions when both sides of the face may be impacted.

Figure 22.14 Bell palsy causes paralysis on one side of the face. (credit: modification of work “Bells palsy diagram” by Patrick J. Lynch/Wikimedia Commons, CC BY 2.5)

In most cases, Bell palsy is a self-limiting condition and improves over a period of weeks to a few months. The majority of people experience near-complete recovery of facial muscle function, though some may experience permanent facial weakness. Individuals with Bell palsy may subsequently develop a loss of taste on the front two-thirds of the tongue, increased sensitivity to sound in one ear, dry eye or excessive tearing, altered speech (such as difficulty pronouncing certain words), facial pain or abnormal sensations, as well as pain around the jaw and behind the ear.

The exact cause of Bell palsy is not definitively understood; however, it is believed to be related to inflammation and swelling of the facial nerve, often triggered by a viral infection, commonly the herpes simplex virus (the same virus responsible for cold sores). Other factors that might contribute to the development of Bell palsy include pregnancy, preeclampsia, obesity, hypertension, diabetes, stress, sleep deprivation, and autoimmune disorders.

Diagnosis is made by examining the symptoms and ruling out other causes of facial weakness, such as stroke or brain tumor. While there is no lab test to diagnose Bell palsy, tests may be conducted to assess nerve damage and to determine the possible underlying cause of developing the disorder. For example, electromyography (EMG) can assess electrical activity to determine severity and extent of nerve damage. MRI or CT can assess the nerves and rule out structural causes of nerve damage. Blood tests can be used to diagnose and assess contributing factors, such as diabetes and infections.

Treatment of Bell palsy primarily focuses on supportive measures and management of symptoms. Medication therapy may include administering steroid medications within seventy-two hours of symptom onset to reduce inflammation and speed up the recovery process, antiviral agents, and over-the-counter analgesics, such as aspirin, acetaminophen, or ibuprofen. To prevent complications related to the inability to close the affected eye, artificial tears or eye patches may be used. In severe cases, taping the eye shut during sleep might be recommended. Physical therapy, facial exercises, massage, acupuncture, and electrical stimulation can help maintain muscle tone and promote better recovery. Though rare, surgical interventions may be warranted to correct facial damage.

Myxedema

The term myxedema is used to describe a severe form of hypothyroidism that occurs when the thyroid gland does not produce enough thyroid hormones. It is characterized by the accumulation of a substance called mucin in the skin and other tissues. This leads to characteristic symptoms such as swelling and puffiness in various parts of the body, particularly around the eyes, face, hands, and feet; skin that may appear thickened, swollen, dry, rough, and “waxy”; hair that may become thin, brittle, and dry; nails that may become brittle and slow-growing; as well as hoarseness in the voice (Figure 22.15). Other symptoms may include cold intolerance, fatigue and weakness, memory problems, difficulty concentrating, slowed thinking, depression, and constipation.

Figure 22.15 These photos highlight the characteristic symptoms of myxedema, a severe form of hypothyroidism. The puffiness around the eyes, (a) thick and dry skin, brittle hair, and (b) overall swelling are hallmark indicators of this condition. (credit: modification of work “Myxedema” by Herbert L. Fred, MD, and Hendrik A. van Dijk/Wikimedia Commons, CC BY 2.0)

Treatment for myxedema involves replacing the deficient thyroid hormones through medication, typically with synthetic thyroid hormones like levothyroxine. The goal is to restore thyroid hormone levels to normal and alleviate the associated symptoms. Regular monitoring and management are essential to ensure the effectiveness of treatment and to prevent complications. Myxedema is a medical emergency when it becomes severe, leading to a condition called myxedema coma. Myxedema coma is characterized by extreme lethargy, confusion, hypothermia, low blood pressure, and other life-threatening complications. It requires immediate medical attention and intensive care.

Simple Goiter

A simple goiter (also, endemic goiter), is a noncancerous enlargement of the thyroid gland that results in visible swelling of the neck (Figure 22.16). It occurs when the thyroid gland becomes larger than normal, leading to a noticeable lump or protrusion in the front of the neck. This condition is usually caused by an insufficient intake of iodine, a trace mineral that is essential for the production of thyroid hormones. If a person’s diet lacks sufficient iodine, the thyroid gland may not be able to produce enough thyroid hormones, leading to an increase in thyroid-stimulating hormone production. Thyroid-stimulating hormone stimulates the thyroid gland to grow in an attempt to produce more thyroid hormones, resulting in the enlargement of the thyroid gland and the development of a goiter.

Figure 22.16 A simple goiter is an abnormality that causes visible swelling in the neck. (credit: “Goiter” by Jerry Kirkhart/Flickr, CC BY 2.0)

While simple goiters are generally benign and not associated with cancer, they can cause discomfort or aesthetic concerns due to the visible swelling in the neck. Additionally, if left untreated, they may lead to complications such as difficulty breathing or swallowing, or in some cases, changes in thyroid hormone levels that affect overall health. Treatment for a simple goiter typically involves addressing the underlying iodine deficiency. In many cases, the enlargement of the thyroid gland can be reversed with adequate intake of iodine through iodized salt or other dietary sources rich in iodine.