Managing Pectus Excavatum

Managing Pectus Excavatum: A Helpful Guide

What is Pectus Excavatum?

Pectus excavatum, often referred to as “funnel chest,” is a condition where the breastbone (sternum) sinks inward, creating a depression in the chest. This happens because the cartilage that connects the ribs to the sternum grows abnormally. The severity of pectus excavatum can vary greatly from a mild, barely noticeable indentation to a deep hollow. While many individuals with pectus excavatum have no health problems associated with it, some may experience physical symptoms or be concerned about their appearance.

Classify Pectus Excavatum

Pectus excavatum is classified as a congenital condition and a chest wall deformity. It is present at birth, although it might not become noticeable until later in childhood or adolescence. It involves an abnormality in the development of the chest wall structure.

Pectus Excavatum Statistics:

Pectus excavatum is estimated to occur in about 1 out of every 300 to 400 live births, making it the most common congenital chest wall deformity. It is more common in males than females, with a ratio of approximately 3:1. While it can occur in people of all races and ethnicities, specific statistics for different populations may vary.

Types of Pectus Excavatum

While the general characteristic of pectus excavatum is a sunken chest, there can be variations in its presentation:

• Symmetrical Pectus Excavatum: The indentation is centered in the chest.
• Asymmetrical Pectus Excavatum: The indentation is more pronounced on one side of the chest than the other. This is quite common, with the left side often being more affected.
• Broad Pectus Excavatum: The indentation is wide and shallow.
• Deep Pectus Excavatum: The indentation is narrow and deep.

These variations can influence the appearance and potentially the impact on the heart and lungs.

Health Signs and Symptoms

Detecting pectus excavatum early can help in timely management. While the sunken chest is the most obvious sign, here are some early indicators and common symptoms:

Early Signs:

• A subtle indentation in the center of the chest that becomes more noticeable as the child grows.
• Parents or caregivers might notice the chest shape during bathing or dressing.

Common Signs and Symptoms:

• Visible Indentation: The most prominent sign is a noticeable inward sinking of the breastbone.
• Shortness of Breath: Especially during physical activity or exercise. This can be due to the chest wall restricting lung expansion.
• Chest Pain: Some individuals may experience discomfort or pain in the chest area.
• Fatigue: Feeling unusually tired, particularly after exertion.
• Rapid Heartbeat or Palpitations: An awareness of the heart beating quickly or irregularly.
• Reduced Exercise Tolerance: Difficulty keeping up with peers during physical activities.
• Postural Issues: Some individuals may develop a rounded shoulder posture to try and conceal the chest deformity.
• Psychological Impact: Concerns about body image and self-esteem can be significant.

Anatomy and Physiology

Pectus excavatum primarily affects the following body parts:

• Sternum (Breastbone): This is the long, flat bone located in the central part of the chest. In pectus excavatum, the lower portion of the sternum is typically displaced inward.
• Ribs: The bones that form the rib cage. The cartilage connecting the ribs to the sternum grows abnormally, pulling the sternum inward.
• Costal Cartilage: This is the flexible tissue that connects the ribs to the sternum. In pectus excavatum, there is often an overgrowth of this cartilage, leading to the deformity.
• Heart and Lungs: In more severe cases, the inward displacement of the sternum can put pressure on the heart and lungs, potentially affecting their function. The heart might be shifted to the left, and lung capacity could be reduced.

Causes

The exact cause of pectus excavatum is not fully understood, but it is believed to be multifactorial. Here’s a brief description followed by common contributing factors:

Pectus excavatum is likely the result of a combination of genetic and developmental factors that lead to the abnormal growth of the costal cartilage.

Common Causes:

• Genetics: There is a strong indication that genetics play a significant role. Approximately 40% of individuals with pectus excavatum have a family history of the condition or other chest wall deformities. Specific genes involved are still being researched.
• Connective Tissue Disorders: Pectus excavatum is more common in individuals with certain connective tissue disorders such as Marfan syndrome, Ehlers-Danlos syndrome, and Noonan syndrome. These disorders affect the body’s connective tissues, which include cartilage.
• Skeletal Abnormalities: In some cases, pectus excavatum may be associated with other skeletal abnormalities, such as scoliosis (curvature of the spine).
• Idiopathic: In many cases, the cause of pectus excavatum remains unknown (idiopathic).

Common Triggers of Pectus Excavatum

Pectus excavatum is a congenital condition, meaning it is present at birth. Therefore, there are no specific triggers that cause it to develop. The condition becomes more noticeable as the child grows and the chest wall develops. However, certain factors might make the symptoms more apparent or worsen over time:

• Growth Spurts: During periods of rapid growth, particularly in adolescence, the deformity might become more pronounced.
• Physical Activity: While not a trigger for the condition itself, increased physical activity might make symptoms like shortness of breath or chest pain more noticeable in individuals with significant pectus excavatum.

Complications:

If not treated or when left untreated, symptoms may worsen and affect your day-to-day life. Possible complications include:

• Cardiopulmonary Issues:
  • Reduced Exercise Tolerance: Difficulty participating in physical activities due to shortness of breath and fatigue.
  • Compression of the Heart: In severe cases, the sternum can press on the heart, potentially affecting its function. This can lead to reduced cardiac output.
  • Lung Restriction: The inward sinking of the chest can restrict lung expansion, leading to decreased lung capacity and potential breathing difficulties.
  • Mitral Valve Prolapse: A condition where the mitral valve in the heart doesn’t close properly, which has been observed more frequently in individuals with pectus excavatum.
• Psychological Impact:
  • Body Image Issues: The visible chest deformity can lead to significant concerns about appearance, affecting self-esteem, confidence, and social interactions.
  • Anxiety and Depression: Some individuals may experience anxiety or depression related to their condition and body image.
• Musculoskeletal Issues:
  • Poor Posture: Individuals might adopt poor posture to try and hide the deformity, which can lead to back and neck pain.

Risk factors:

Certain factors can increase the likelihood of developing pectus excavatum:

• Family History: Having a family member with pectus excavatum significantly increases the risk.
• Connective Tissue Disorders: Individuals with conditions like Marfan syndrome, Ehlers-Danlos syndrome, and Noonan syndrome have a higher incidence of pectus excavatum.
• Genetic Syndromes: Some genetic syndromes are associated with an increased risk of pectus excavatum.

Pectus Excavatum Stages

Pectus excavatum doesn’t typically progress through distinct “stages” in the way some other illnesses do. Instead, it is usually classified by its severity, which can range from mild to severe. The severity is often determined by the depth of the indentation and its impact on the heart and lungs. Here’s a general way to think about the progression of its visibility and potential impact:

• Beginner (Mild): The indentation is shallow and may only be noticeable upon close inspection. Individuals may not experience any physical symptoms.
• Medium (Moderate): The indentation is more pronounced and easily visible. Some individuals may start to experience mild symptoms like shortness of breath during strenuous exercise or occasional chest discomfort.
• Advanced (Severe): The indentation is deep and very noticeable. Individuals are more likely to experience significant symptoms such as shortness of breath even with mild exertion, chest pain, fatigue, and potentially cardiac or respiratory issues. The psychological impact can also be more significant at this stage.

It’s important to note that the severity can increase as a child grows, particularly during growth spurts in adolescence.

Prevention

There is currently no known way to prevent pectus excavatum, as it is often a congenital condition with a strong genetic component. If there is a family history of pectus excavatum or related connective tissue disorders, genetic counseling might be considered for families planning to have children. However, this is more about understanding the risk rather than preventing the condition.

Pectus Excavatum Diagnosis

Diagnosing pectus excavatum typically involves a combination of a physical examination and imaging tests.

• Physical Examination: A doctor will visually inspect the chest and feel the indentation. They will also ask about the individual’s medical history and any symptoms they are experiencing. The Haller index, which is a ratio calculated from a CT scan, is often used to quantify the severity of the pectus excavatum.
• Medical History: The doctor will inquire about family history of chest wall deformities or connective tissue disorders.

Tests & Examinations

To further evaluate the pectus excavatum and its potential impact, the following tests and examinations may be conducted:

• Chest X-ray: This can help visualize the shape of the chest and the position of the heart and lungs.
• Computed Tomography (CT) Scan: A CT scan provides detailed cross-sectional images of the chest, allowing for precise measurement of the depth and width of the indentation. The Haller index is calculated from measurements taken on a CT scan.
• Electrocardiogram (ECG or EKG): This test measures the electrical activity of the heart and can help detect any cardiac abnormalities.
• Echocardiogram: An ultrasound of the heart that can assess its structure and function and check for conditions like mitral valve prolapse.
• Pulmonary Function Tests (PFTs): These tests measure how much air the lungs can hold and how quickly air can be inhaled and exhaled, helping to assess lung capacity and function.
• Exercise Testing: Also known as cardiopulmonary exercise testing, this can evaluate how the heart and lungs function during physical activity and can help determine the impact of pectus excavatum on exercise tolerance.
• Genetic Testing: If a connective tissue disorder is suspected, genetic testing may be recommended.

Health Professionals:

Several types of health professionals may be involved in the diagnosis and treatment of pectus excavatum:

• Pediatrician: Often the first point of contact for children, they can identify the condition and refer to specialists.
• General Practitioner (GP): For adults, the GP can make the initial assessment and provide referrals.
• Thoracic Surgeon: This is a surgeon who specializes in the chest cavity, including the lungs, heart, and esophagus. They are typically the specialists who perform surgical corrections for pectus excavatum.
• Cardiologist: A heart specialist who may be consulted if there are concerns about the impact of pectus excavatum on the heart.
• Pulmonologist: A lung specialist who may be involved if there are breathing problems or concerns about lung function.
• Geneticist: If a connective tissue disorder is suspected, a geneticist can provide diagnosis and counseling.
• Physical Therapist: Can help with posture correction, strengthening chest muscles, and improving breathing mechanics, especially in non-surgical management or post-surgery.
• Psychologist or Counselor: Can provide support for body image issues and the emotional impact of the condition.

Reasons to see a Professional:

You should consider seeing a healthcare professional if you or your child:

• Notice a visible indentation in the chest.
• Experience symptoms such as shortness of breath, chest pain, or fatigue, especially during physical activity.
• Have concerns about the appearance of the chest and its impact on self-esteem.
• Have a family history of pectus excavatum or connective tissue disorders.
• Are considering treatment options for pectus excavatum.

Process to find the right professional:

Finding the right healthcare professional is crucial for effective management of pectus excavatum:

1. Start with your Primary Care Physician (Pediatrician or GP): They can provide an initial assessment and refer you to specialists.
2. Ask for Referrals: Seek recommendations from your primary doctor or other trusted healthcare providers for thoracic surgeons or other specialists experienced in treating pectus excavatum.
3. Check Credentials and Experience: Research the specialists’ qualifications, board certifications, and experience in treating pectus excavatum, particularly the chosen treatment method (e.g., Nuss procedure or Ravitch procedure).
4. Consider Hospital or Clinic Reputation: Look into the reputation of the hospital or clinic where the specialist practices, especially their experience with chest wall deformities.
5. Read Reviews and Testimonials: If available, read reviews from other patients to get an idea of the doctor’s communication style and the overall patient experience.
6. Schedule a Consultation: Once you have identified potential specialists, schedule a consultation to discuss your concerns, ask questions, and understand their approach to diagnosis and treatment.
7. Trust Your Gut: Choose a healthcare professional with whom you feel comfortable and who you believe understands your concerns and goals.

Visit Preparation

Preparing for your consultation can help you make the most of your appointment:

• Document Symptoms: Keep a record of any symptoms you or your child have been experiencing, including when they started, how often they occur, and what seems to trigger them.
• Gather Medical History: Compile a list of past medical conditions, allergies, medications, and any previous treatments. Include family history of pectus excavatum or related conditions.
• Prepare Questions: Write down a list of questions you want to ask the doctor (see the “Questions to ask?” section below).
• Bring Imaging Reports (if available): If you have had any previous X-rays or scans, bring the reports or the images themselves.
• Bring a Support Person: Consider bringing a family member or friend for support and to help take notes.
• Be Ready to Describe the Condition: Be prepared to describe when you first noticed the chest shape and how it has changed over time.

Questions to ask?

Here are some important questions to ask a healthcare professional about pectus excavatum:

• What is the severity of my/my child’s pectus excavatum?
• What are the potential health implications of this condition?
• What tests do you recommend? Why?
• What are the treatment options available?
• Am I/Is my child a candidate for non-surgical treatment (e.g., vacuum bell)?
• Am I/Is my child a candidate for surgical treatment (e.g., Nuss procedure, Ravitch procedure)?
• What are the risks and benefits of each treatment option?
• What is the typical recovery timeline for each treatment?
• What are the long-term outcomes of treatment?
• Will the condition worsen over time if left untreated?
• Are there any lifestyle modifications or exercises that might be helpful?
• What is your experience in treating pectus excavatum?
• How often will I/my child need follow-up appointments?
• Are there any support groups or resources available for individuals with pectus excavatum?

Diagnosis –

The diagnosis of pectus excavatum is typically confirmed through a physical examination where the characteristic inward sinking of the breastbone is observed. Imaging tests like chest X-rays and CT scans are used to assess the severity of the deformity, measure the Haller index, and evaluate the impact on the heart and lungs. Other tests like ECG, echocardiogram, and pulmonary function tests may be conducted to assess cardiac and respiratory function.

Procedures –

The following procedures may be involved in the diagnosis and treatment of pectus excavatum:

• Physical Examination: Visual inspection and palpation of the chest.
• Chest X-ray: Imaging test to visualize the chest structure.
• CT Scan: Detailed imaging to measure the severity of the deformity (Haller index).
• ECG (Electrocardiogram): Measures the electrical activity of the heart.
• Echocardiogram: Ultrasound of the heart to assess its structure and function.
• Pulmonary Function Tests (PFTs): Measures lung capacity and airflow.
• Exercise Testing: Evaluates heart and lung function during physical activity.
• Vacuum Bell Therapy: A non-surgical treatment using a suction device to lift the sternum.
• Nuss Procedure: A minimally invasive surgical procedure where a curved metal bar is inserted under the sternum to push it outward.
• Ravitch Procedure: An open surgical procedure that involves reshaping the cartilage and repositioning the sternum.

Treatments –

Treatment for pectus excavatum depends on the severity of the condition and whether it is causing symptoms or significant psychological distress. Options include:

• Observation: For mild cases with no symptoms, the doctor may recommend regular check-ups to monitor the condition.
• Vacuum Bell Therapy: A non-surgical option for children and adolescents with mild to moderate pectus excavatum. A suction cup is placed on the chest and creates a vacuum that gently lifts the sternum over time.
• Surgical Correction: Surgical options are considered for more severe cases or when symptoms are present. The two main surgical procedures are:
  • Nuss Procedure: A minimally invasive procedure where one or more curved metal bars are inserted through small incisions on the sides of the chest and placed under the sternum to push it outward. The bar(s) are typically left in place for 2-3 years and then removed.
  • Ravitch Procedure: An open surgical procedure that involves making a larger incision in the chest to reshape the costal cartilage and reposition the sternum. Sometimes, a support bar is used temporarily.

Comparison of Treatment Options.

Here’s a comparison of the main treatment options for pectus excavatum:

• Treatment: Observation
  • Pros: Non-invasive, no risks associated with procedures.
  • Cons: Does not correct the deformity, may not be suitable for symptomatic or severe cases.
  • When To Use: Mild cases with no or minimal symptoms, when the individual is not bothered by the appearance.
• Treatment: Vacuum Bell Therapy
  • Pros: Non-surgical, can be done at home, may improve mild to moderate deformities, especially in younger individuals.
  • Cons: Requires consistent daily use for a prolonged period, may cause temporary skin irritation, effectiveness can vary.
  • When To Use: Mild to moderate pectus excavatum in children and adolescents, as a first-line treatment or when surgery is not preferred.
• Treatment: Nuss Procedure
  • Pros: Minimally invasive, smaller scars, shorter hospital stay compared to open surgery.
  • Cons: Requires surgery and anesthesia, potential for bar displacement, pain after surgery, requires a second surgery for bar removal.
  • When To Use: Moderate to severe pectus excavatum, especially in adolescents and young adults.
• Treatment: Ravitch Procedure
  • Pros: Can correct more complex or severe deformities, may have a lower risk of recurrence in some cases.
  • Cons: More invasive surgery with a larger incision, longer hospital stay, potentially more post-operative pain and a longer recovery period.
  • When To Use: Severe or complex cases, in individuals where the Nuss procedure is not suitable, or in revision surgeries.

Recovery Timeline & Expectations.

The recovery timeline and expectations vary depending on the chosen treatment method:

Observation: No specific recovery period. Regular check-ups with a healthcare professional are recommended.

Vacuum Bell Therapy: This is an ongoing therapy. Improvement is gradual and requires consistent daily use over months or even years. Expect to use the device for a certain number of hours each day as recommended by your doctor. There might be mild skin irritation initially.

Nuss Procedure:

• Week 1: Expect significant pain, managed with pain medication. Limited mobility and activity. Hospital stay typically lasts a few days.
• Month 1: Gradual increase in activity level. Avoid heavy lifting and strenuous activities. Regular follow-up appointments. Pain and discomfort will gradually decrease.
• Month 3: Most daily activities can be resumed. Continue to avoid contact sports or activities that could impact the chest. Physical therapy is often recommended to improve posture and range of motion.
• 6 Months – 1 Year: Continued improvement in strength and endurance. Gradual return to more vigorous activities as advised by the surgeon.
• 2-3 Years: The metal bar(s) will be surgically removed in a separate procedure, which will also have its own recovery period (shorter than the initial surgery).

Common Setbacks and How to Handle Them:

• Pain: Manage with prescribed pain medication. Communicate with your doctor if pain is not well-controlled.
• Bar Displacement (Nuss Procedure): If the bar shifts, it may require surgical repositioning. Follow post-operative instructions carefully to minimize this risk.
• Infection: Watch for signs of infection at the incision sites (redness, swelling, pus) and contact your doctor immediately.
• Breathing Difficulties: If you experience new or worsening shortness of breath, seek medical attention.

When to return to normal activity/exercise safely:

Return to normal activities and exercise should be gradual and guided by your surgeon’s recommendations and your body’s response. Generally:

• Light walking and non-strenuous activities can usually be resumed within a few weeks after surgery.
• More vigorous activities and sports are typically allowed after several months, but only with the surgeon’s approval. Contact sports are usually restricted until after the bar removal in the Nuss procedure.

Ravitch Procedure: The recovery timeline is generally longer than the Nuss procedure, with similar phases but potentially extended durations for each stage. Follow your surgeon’s specific instructions.

Daily Self-Care Routine for Pectus Excavatum.

For individuals with pectus excavatum, a general healthy lifestyle is important. There isn’t a specific daily self-care routine directly targeting the condition itself, but focusing on overall well-being can be beneficial:

Morning Routine:

• Gentle Stretching: Start the day with light stretches to improve posture and flexibility. Focus on shoulders and chest.
• Deep Breathing Exercises: Practice deep, diaphragmatic breathing to help maximize lung capacity.
• Hydration: Drink water to stay hydrated.

Throughout the Day:

• Maintain Good Posture: Be mindful of your posture while sitting and standing. Avoid slouching.
• Regular Gentle Exercise: Engage in light to moderate physical activity as tolerated, such as walking or swimming.
• Stay Hydrated: Continue to drink water throughout the day.
• Mindfulness and Stress Management: Practice relaxation techniques to manage any anxiety or body image concerns.

Evening Recovery:

• Relaxing Activities: Engage in calming activities before bed, such as reading or taking a warm bath.
• Continue Gentle Stretching: Repeat some of the morning stretches if comfortable.
• Ensure Adequate Sleep: Aim for sufficient sleep to support overall health and recovery, especially after any treatment.

Health Monitoring –

Health professionals monitor pectus excavatum symptoms and treatment progress through:

• Regular Physical Examinations: To assess the appearance of the chest and check for any new symptoms.
• Imaging Tests: Periodic chest X-rays or CT scans may be ordered to monitor the severity of the deformity and the position of any surgical bars.
• Pulmonary Function Tests: To track changes in lung capacity and function over time.
• Exercise Testing: To evaluate improvements in exercise tolerance.

Home Devices or Tests:

There are no specific home devices or tests to directly monitor pectus excavatum. However, individuals can:

• Keep a Symptom Diary: Note any changes in symptoms like shortness of breath, chest pain, or fatigue.
• Monitor Exercise Tolerance: Pay attention to how physical activity affects their breathing and energy levels.
• Observe the Appearance of the Chest: Note any changes in the shape or depth of the indentation.

Regular communication with your healthcare team about any changes or concerns is crucial.

How to manage Pectus Excavatum

Living with pectus excavatum involves a combination of medical management and self-care strategies:

• Follow Medical Advice: Adhere to the treatment plan recommended by your healthcare team, including attending follow-up appointments and adhering to any restrictions after treatment.
• Maintain a Healthy Lifestyle: Eat a balanced diet, engage in regular physical activity as tolerated, and get enough sleep.
• Exercise Regularly: Participate in activities that improve cardiovascular health and strengthen chest and back muscles, as recommended by your doctor or physical therapist. Swimming is often well-tolerated.
• Seek Psychological Support: If you are struggling with body image issues or anxiety related to your pectus excavatum, consider talking to a therapist or counselor.
• Join Support Groups: Connecting with other individuals who have pectus excavatum can provide emotional support and valuable insights.
• Educate Yourself: Continue to learn about the condition and treatment options to make informed decisions about your care.
• Be Patient: Treatment, especially non-surgical options, can take time to show results. Be patient and consistent with your therapy.
• Protect Your Chest: If you have undergone surgical correction, follow your surgeon’s instructions on protecting your chest from trauma, especially during activities and sports.

Nutrition Dos and Dont’s

There are no specific dietary recommendations directly related to pectus excavatum. However, maintaining a healthy and balanced diet is important for overall health and can support recovery after any treatment:

Dos:

• Eat a Balanced Diet: Include plenty of fruits, vegetables, whole grains, lean protein, and healthy fats.
• Stay Hydrated: Drink an adequate amount of water throughout the day.
• Consume Calcium and Vitamin D: These are important for bone health, especially during growth and recovery.
• Include Foods Rich in Antioxidants: These can help support overall health and reduce inflammation.

Dont’s:

• Avoid Processed Foods: These can be high in unhealthy fats, sugar, and sodium.
• Limit Sugary Drinks: These can contribute to weight gain and provide empty calories.
• Avoid Crash Diets: Focus on sustainable healthy eating habits rather than restrictive diets.

Lifestyle Dos and dont’s

Adopting a healthy lifestyle can help manage pectus excavatum and improve overall well-being:

Dos:

• Engage in Regular Exercise: Choose activities you enjoy and that are appropriate for your condition and any treatment you have received. Focus on cardiovascular fitness and strengthening core and back muscles.
• Maintain Good Posture: Be conscious of your posture while sitting, standing, and walking.
• Get Enough Sleep: Aim for 7-9 hours of quality sleep per night to support recovery and overall health.
• Manage Stress: Practice relaxation techniques like deep breathing, meditation, or yoga.
• Seek Support: Connect with friends, family, or support groups for emotional well-being.

Dont’s:

• Avoid Smoking: Smoking can negatively impact lung function and overall health.
• Limit Alcohol Consumption: Excessive alcohol intake can have negative health effects.
• Avoid Heavy Lifting or Strenuous Activities (if restricted by your doctor): Especially after surgery, follow your doctor’s recommendations regarding physical activity.
• Ignore Symptoms: If you experience new or worsening symptoms, consult your healthcare provider.

Emergency Situations

While pectus excavatum itself is not usually a cause for emergency, certain situations might require immediate medical attention:

• Sudden Severe Chest Pain: Especially if accompanied by shortness of breath, dizziness, or sweating. This could indicate a cardiac issue.
• Severe Shortness of Breath: If you experience a sudden and significant difficulty breathing that does not improve with rest.
• Signs of Infection After Surgery: Such as high fever, increased redness, swelling, or pus at the incision sites.
• Sudden Injury to the Chest: If you experience a direct blow to the chest, especially if you have had surgical correction.

In any of these situations, seek immediate medical help by calling emergency services or going to the nearest emergency room.