Pectus Carinatum: Symptoms, Types, Causes and Treatment

Pectus carinatum, often called "pigeon chest," is a chest wall deformity that affects both appearance and, in some cases, physical well-being. While less common than its counterpart, pectus excavatum, this condition can have a significant impact on self-esteem, body image, and, for some, quality of life. Understanding its symptoms, types, causes, and treatment options is essential for patients, families, and healthcare professionals seeking the best care strategies.

Symptoms of Pectus Carinatum

Pectus carinatum manifests primarily as a visible outward protrusion of the chest wall, but its impact extends beyond appearance. Recognizing the range of symptoms—physical and psychological—is key to early intervention and effective management.

Symptom	Description	Prevalence/Notes	Source(s)
Protruding Chest	Sternum projects outward (pigeon chest)	Most common	7 8
Chest Pain	Discomfort or pain in the chest area	~28% of pediatric patients	2
Palpitations	Sensation of abnormal heartbeats	~8% of pediatric patients	2
Breathlessness	Shortness of breath, especially on exertion	Variable, less common	7
Body Image Distress	Dissatisfaction with appearance	Highly prevalent	1 7
Reduced Mental QoL	Lowered mental quality of life	Noted in many patients	1

Table 1: Key Symptoms

Visible Chest Protrusion

The hallmark feature of pectus carinatum is the abnormal outward projection of the sternum and adjacent costal cartilages, giving the chest a "pigeon-like" appearance. This characteristic is often the first sign noticed by patients or their caregivers, typically becoming more pronounced during childhood and adolescence, especially around puberty 7 8.

Physical Symptoms

• Chest Pain and Palpitations: While many individuals are asymptomatic, a significant minority—especially children and adolescents—report chest pain and palpitations. However, these symptoms do not always correlate with underlying heart abnormalities, as most patients with such complaints have normal cardiac function on echocardiogram 2.
• Breathlessness: Some patients may experience mild breathlessness or exercise intolerance, but severe cardiopulmonary compromise is uncommon 7.

Psychological and Quality of Life Impact

• Body Image Concerns: The impact on self-esteem and body image can be profound. Many patients express dissatisfaction with their appearance, and this may be even more pronounced compared to those with pectus excavatum 1.
• Reduced Mental Quality of Life: Studies indicate that patients with pectus carinatum often experience lower mental quality of life, closely linked to body image distress and reduced self-esteem 1.
• Social Withdrawal: Due to cosmetic concerns, some patients may avoid activities such as swimming or sports, further affecting social interactions and overall well-being 1.

Rare and Associated Symptoms

• Poland Syndrome: In rare cases, pectus carinatum may be part of broader syndromes (e.g., Poland syndrome), which can include asymmetry of the chest, absence of pectoral muscles, and other systemic anomalies 3 8.

Types of Pectus Carinatum

Pectus carinatum is not a uniform condition. Its clinical presentation varies depending on which part of the chest is affected, the symmetry of the protrusion, and any associated syndromic features. Distinguishing between these types aids in tailoring treatment and understanding prognosis.

Type/Subtype	Description	Distinguishing Features	Source(s)
Chondrogladiolar	Most common, involves sternal body	Symmetrical, central protrusion	7 8
Chondromanubrial	Involves upper sternum (manubrium)	"Pouter pigeon" or "horseshoe" chest, often more rigid	5 7
Asymmetric/Lateral	One side of chest more affected	May result in rotation or lateral protrusion	6 9 10
Syndromic/Poland	Part of broader syndrome (e.g., Poland)	Includes muscle/rib defects, hand anomalies	3 8

Table 2: Clinical Types of Pectus Carinatum

Chondrogladiolar Type

• Prevalence: The most common form, affecting the central or lower part of the sternum (gladiolus) 7.
• Appearance: Produces a prominent, symmetrical protrusion along the midline of the chest.
• Flexibility: Often more flexible and amenable to non-surgical treatment such as bracing 7 16.

Chondromanubrial Type

• Description: Involves the upper portion of the sternum (manubrium) and adjacent costal cartilages 7.
• Other Names: Also referred to as "pouter pigeon breast" or "horseshoe chest" due to its characteristic appearance 7.
• Features: Tends to be more rigid, less responsive to bracing, and may require surgical correction 5.

Asymmetric or Lateral Pectus Carinatum

• Presentation: One side of the chest protrudes more than the other, sometimes with associated sternal rotation 6 9 10.
• Complexity: May be more challenging to treat, particularly if the thoracic wall is stiff or there is significant asymmetry 5 6.

Syndromic and Associated Types

• Poland Syndrome: Characterized by absence or underdevelopment of chest muscles, rib anomalies, and sometimes limb defects. Pectus carinatum in this context requires tailored management, as functional deficits are often minimal, but cosmetic and psychological impact can be significant 3 8.
• Genetic Syndromes: Pectus carinatum can be a feature of connective tissue disorders such as Marfan or Noonan syndrome 7 8.

Causes of Pectus Carinatum

Although the outward appearance of pectus carinatum is unmistakable, its underlying causes are varied and still not fully understood. Both genetic and developmental factors play a role, with emerging research challenging previous assumptions.

Factor	Description	Evidence/Notes	Source(s)
Costal Cartilage Overgrowth	Abnormal elongation of costal cartilages	Traditional theory; not sole cause	8 9 10 11
Rib Undergrowth	Shorter ribs relative to cartilage	Recent imaging studies	9 10
Genetic Factors	Familial occurrence; syndromic associations	~25% with family history; Marfan, Noonan	7 8
Developmental Dysfunction	Growth plate/cartilage-ossification defects	May explain sternal/rib changes	10
Secondary/Iatrogenic	Post-surgical changes (e.g., after cardiac surgery)	Sternal wires, trauma	11
Syndromic Associations	Part of broader genetic syndromes	Poland, Marfan, Noonan	3 7 8

Table 3: Contributing Factors to Pectus Carinatum

Costal Cartilage Overgrowth and Rib Undergrowth

• Traditional View: Historically, pectus carinatum was believed to result from overgrowth of the costal cartilages, pushing the sternum outward 8 9.
• Recent Insights: Imaging studies show that, in addition to cartilage elongation, there may be undergrowth or shortening of the ribs. The overall length of cartilage plus rib is often similar to controls, suggesting a complex interplay rather than simple overgrowth 9 10.
• Cartilage Morphology: Some research points to abnormal shape or distortion of cartilage, rather than just length, as a key factor in the deformity 10.

Genetic and Developmental Factors

• Familial Patterns: About a quarter of cases have a family history of chest wall deformities, and several genetic syndromes (e.g., Marfan and Noonan) explicitly include pectus carinatum as part of their diagnostic criteria 7 8.
• Connective Tissue Disorders: Mutations affecting collagen and other matrix proteins in cartilage may contribute to abnormal growth patterns 8.

Growth Plate Dysfunction

• Abnormal Ossification: In the chondromanubrial subtype, defective ossification and developmental dysfunction at the costochondral junction are suspected causes 7 10.
• Sternal Curvature: Variations in sternal curvature patterns have been observed in different types of pectus carinatum, suggesting a spectrum of underlying developmental disturbances 6.

Syndromic and Secondary Causes

• Poland Syndrome: This rare congenital disorder involves unilateral absence of chest muscles and may include pectus carinatum among its manifestations 3 8.
• Iatrogenic Causes: Rarely, pectus carinatum can develop after cardiac surgery due to improper healing or placement of sternal wires 11.

Treatment of Pectus Carinatum

Advances in the understanding of pectus carinatum have revolutionized its management. Today, a range of non-surgical and surgical options are available, with treatment tailored to the patient's age, chest wall flexibility, type of deformity, and personal goals.

Treatment	Description	Indications/Notes	Source(s)
Bracing	External compression brace; non-surgical	First-line for flexible chests, especially in youth	12 13 14 16
Minimally Invasive Surgery	Substernal bar placement (e.g., Abramson technique)	For rigid or bracing-resistant cases	14 15
Open Surgery	Ravitch procedure; cartilage resection	For complex, stiff, or recurrent cases	4 5 15
Staged Approach	Bracing first, surgery if needed	Combines non-surgical and surgical	14
Psychological Support	Counseling, support groups	For body image or mental health distress	1

Table 4: Treatment Modalities

Bracing Therapy

• Dynamic Compression Bracing: The primary and most effective treatment for flexible pectus carinatum in children and adolescents. The brace applies external pressure to gradually remodel the chest wall 12 13 16.
  • Success Rates: High success, especially when initiated before skeletal maturity and with good patient compliance.
  • Protocols: The "Calgary Protocol" involves continuous bracing (23 hours/day) during the correction phase, followed by nighttime bracing until skeletal maturity (maintenance phase) 12 13.
  • Duration: Correction phase may last from 4 to 14 months depending on age and flexibility; maintenance varies 13 16.
  • Adverse Effects: Minor and infrequent (skin irritation, vasovagal episodes) 16.
  • Challenges: Patient motivation and compliance are critical; recurrence is rare but possible if maintenance is discontinued early 13 14.

Minimally Invasive Surgery

• Abramson Procedure: Involves placement of a curved steel bar anterior to the sternum, compressing the protrusion. Suitable for patients with rigid chest walls not amenable to bracing 14 15.
  • Recovery: Short hospital stay, quick return to normal activity, and minimal blood loss 15.
  • Bar Removal: Performed after 2 years or more, with most patients achieving good or excellent cosmetic results 15.
  • Complications: Low rates; may include pneumothorax, skin adherence, or seroma 15.

Open Surgical Correction

• Ravitch Procedure: Traditional open surgery with cartilage resection and sternal osteotomy, reserved for severe, complex, or recurrent cases, or when less invasive methods fail 4 5.
  • Technique: Removal of abnormal cartilages, repositioning, and stabilization of the sternum 4 5.
  • Indications: Stiff, asymmetric, or syndromic cases; failure of bracing and minimally invasive options 5 14.

Staged Management and Patient Selection

• Sequential Approach: Many centers now use a staged approach—beginning with bracing and reserving surgery for those who fail to respond or have rigid deformities 14.
  • Patient Assessment: Flexibility of the chest wall is measured (e.g., pressure of initial correction) to determine suitability for bracing versus surgery 16.

Psychological and Supportive Care

• Body Image and Mental Health: Given the strong psychological impact, counseling and support are vital components of holistic care. Addressing body image concerns can improve mental quality of life and satisfaction with treatment outcomes 1.

Conclusion

Pectus carinatum is a multifaceted condition with a spectrum of symptoms, types, and causes. Advances in non-surgical and surgical treatments have made correction more accessible and effective, especially when care is individualized and holistic.

Key Points:

• Symptoms range from visible chest protrusion and occasional physical discomfort to significant psychological distress and reduced quality of life.
• Types include chondrogladiolar (most common), chondromanubrial, asymmetric, and syndromic forms.
• Causes are multifactorial, involving abnormal cartilage and rib growth, genetic predisposition, developmental dysfunction, and rare secondary factors.
• Treatment prioritizes dynamic compression bracing for flexible cases, with minimally invasive or open surgery reserved for rigid or complex deformities. Psychological support is essential for optimal outcomes.

Early recognition, individualized assessment, and a combination of physical and psychological care offer the best path to improved health and confidence for those living with pectus carinatum.