Chapter X.13. Pectus Excavatum and Pectus Carinatum
Collin G.Z. Liang
Devin P. Puapong, MD
November 2022

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Case: A 14 year-old boy presents to his primary care pediatrician because of a recent inability to keep up with his peers on his high school basketball team. His parents wanted him to try out for the team because he is particularly tall for his age with an impressive arm span. He states that he gets short of breath, dizzy, and begins to wheeze when running drills. His medical history is significant for childhood asthma. Upon further questioning, he reveals that he avoids showering in his campus locker room because he gets anxious thinking about his teammates making fun of the indentation on his chest.

Exam: VS T 37.0, P 69, RR 14, BP 110/76. His heart has a normal rate and rhythm. No S3 or S4. No sternal rubs, gallops, or murmurs are heard. His lung fields are clear bilaterally without wheezes or crackles. The chest appears sunken in with 2 cm depression of the sternum.


The etiologies of pectus excavatum and pectus carinatum remain unknown, and the majority of cases occur in isolation; however, a family history of chest wall deformity is suggested in up to one-third to two-thirds of patients. Defective elongated growth of the costal cartilage is a suspected hypothesis, which may explain why 10% of cases are associated with genetic musculoskeletal disorders such as Marfan or Ehlers-Danlos syndrome. Other conditions that are associated with pectus deformities include scoliosis, mitral valve prolapse, homocystinuria, Morquio syndrome (mucopolysaccharidosis type IVA), Noonan syndrome, and osteogenesis imperfecta (1).

Pectus excavatum is an anterior depression of the chest wall that can be easily identified shortly after birth. Pectus deformities make up approximately 90% of all congenital chest wall anomalies, with pectus excavatum being the most common (2). Pectus excavatum has an incidence of 1 in 350 live births and affects males more than females (3).

Symptomatology varies widely from minor, cosmetic issues to disabling cardiopulmonary symptoms. Commonly reported symptoms include exercise-induced dyspnea and wheezing, chest pain, fatigue, palpitations, tachycardia, postprandial satiety, fainting or dizziness, and psychological distress regarding body image. Interestingly, although the depth of sternal depression varies widely with the extreme being the sternum lying adjacent to the spine, depth alone does not accurately correlate with the degree of cardiopulmonary effects (2).

The diagnosis of pectus deformities is made clinically by visual inspection. Pectus excavatum can be detected at birth or early childhood but becomes increasingly pronounced in early adolescence when there is a pubertal growth spurt. On general appearance, patients are typically round-shouldered, stooped forward, and have a relatively prominent abdomen and costal margins (3). The third, fourth, and fifth costal cartilages are frequently involved either symmetrically or asymmetrically with varying degrees of sternal rotation towards the depressed side (4). Although females are less affected compared to males, females may be underdiagnosed due to breast tissue disguising the defect during or after adolescence.

Formal evaluation of pectus excavatum includes pulmonary functions tests, cardiology evaluation (such as an echocardiogram), and calculation of the Haller index. The Haller index uses transverse computed tomography (CT) to calculate the ratio of the transverse diameter (width) of the chest wall by the greatest anteroposterior diameter (posterior surface of the sternum to the anterior surface of the spine) (4). The Haller index is used to discern and categorize pectus excavatum by severity as either mild (2 to 3.2), moderate (3.2 to 3.5), or severe (>3.5). Moderate or severe deformities are considered for corrective surgery.

When surgical correction is considered for pectus excavatum, the Ravitch technique is a historical open repair performed by resecting the abnormal cartilages and fracturing the sternum to fix it in a corrected position. The Nuss procedure, a minimally invasive technique, has since replaced the Ravitch technique. It involves advancing a curved strut upside down across the mediastinum via two incisions in the mid-axillary line. The strut is then flipped into position with securement via metal plates to the ribs bilaterally, causing mechanical lifting of the sternum and chest wall into a corrected position. This is left in place for 2 to 3 years with removal typically by an ambulatory procedure (3). Postoperative complications include pneumothorax, hemothorax, bar displacement, bar allergy, pleural effusion, pericardial effusion, and wound infection. Severe postoperative pain is another complication that may be prophylactically treated via cryoanalgesia of the intercostal nerves to reduce pain and shorten hospital length of stay and postoperative narcotic use (5). Vacuum bell therapy is a noninvasive, conservative management option for patients who desire non-surgical correction. The apparatus, invented by Eckart Klobe, consists of a silicon ring that fits to the chest with a transparent polycarbonate window and pump used to create suction on the patient’s chest. Currently, the precise clinical indications and assessment criteria are not well defined. An optimal regimen is still being studied, but basic protocols include, at the minimum, two 30-minute application periods daily (with no more than eight hours daily) for at least a year. Lower initial BMI and young age (better chest wall flexibility <12 years old) demonstrated significant positive results with respect to improved Haller Index after one-year of treatment (6). Complications are largely temporary and include bruising, erythema, skin irritation, flaccid skin, and discomfort of the chest and/or back.

Although exertional dyspnea and exercise tolerance may subjectively be improved following repair, cardiopulmonary function and pulmonary function testing have not objectively and consistently improved in postoperative studies (7). There is no definitive treatment and the decision to repair the defect is based primarily on cosmetic considerations with studies showing consistent significant improvement in psychological outcomes postoperatively (8).

Pectus carinatum is an anterior sternal protrusion that more commonly involves the middle and lower sternum (chondrogladiolar prominence; known as chicken breast) rather than the upper sternum (chondromanubrial prominence; known as pigeon breast). Pectus carinatum has an incidence of roughly 1 in 1000 and affects males more frequently with a ratio of 4:1 (1). Like pectus excavatum, pectus carinatum mostly occurs as an isolated defect, and shares the risk factor of a family history of chest wall deformity. Pectus carinatum is typically considered a purely aesthetic deformity that does not usually result in significant cardiopulmonary symptomatology (9). The most common symptom is tenderness of the protrusion with certain activities where a person is prone for long periods of time, such as surfing. Rarely, some individuals can experience decreased pulmonary endurance with physical activity, which symptoms are more commonly associated with the chondromanubrial variation (1).

Contrary to pectus excavatum, pectus carinatum is commonly identified during puberty when growth spurts accelerate bone growth. Pectus carinatum deformity tends to be unilateral, with angling of the sternum towards the right (1) but may also occur bilaterally. The diagnosis can be made with visual inspection only, but radiographic imaging with lateral chest x-ray or CT can provide more specific diagnostic details (utilizing the Haller index), especially when considering surgical management options.

Historically, the Ravitch technique was the only option for treatment in moderate to severe cosmetic appearances. Surgery has since been replaced as the first-line treatment by external, orthotic bracing. Bracing is most effective before a patient’s growth spurt and is less effective after age 19 due to changes in the flexibility of the chest wall. There currently is no defined regimen. The duration of correction ranges between months to years. Each brace is custom-made for a child and can be worn either over or under clothing. For patients that may find the lengthy treatment time of nonsurgical bracing unfavorable, the Abramson technique which uses a modified Nuss bar has been in use since 2006 but is rarely performed. The bar remains in the pre-sternal, extra-thoracic space for about two years and is removed. It is worth noting that the Abramson technique is used primarily for the chondrogladiolar variation because the chondromanubrial variation has less flexibility of the chest wall (10). Nonmedical treatment options include bodybuilding to minimize the appearance of the deformity, but also to improve self-esteem and confidence. The American Academy of Pediatrics does not support strenuous levels of bodybuilding in children because of incomplete bone maturation. Female patients may consider breast augmentation to alter the physical appearance of the chest (1). Even without treatment, the prognosis of pectus carinatum is excellent.


Questions
1. The most common risk factor for pectus excavatum is?
   a. Intrauterine growth restriction
   b. Family history of chest wall deformities
   c. Congenital diaphragmatic hernia
   d. Pulmonary hypoplasia

2. At what age range does pectus carinatum commonly present?
   a. Infancy
   b. Adolescence
   c. Adulthood
   d. Elderly

3. What is the age range where orthotic bracing produces the best outcomes of pectus carinatum treatment?
   a. 5 to 10 years old (before growth spurts)
   b. 11 to 18 years old (during growth spurts)
   c. 19 to 20 years old (after growth spurts)
   d. There is no idea time for orthotic bracing

4. What is the most common reason/complaint for which patients elect surgical repair of pectus chest deformities?
   a. Cosmetic appearance
   b. Chronic chest pain
   c. Cardiac dysfunction
   d. Pulmonary dysfunction

5. Pectus chest wall deformities can be associated with all the following systemic diseases except:
   a. Marfan syndrome
   b. Mucopolysaccharidosis
   c. Sarcoidosis
   d. Homocystinuria

6. True or False: Pectus carinatum, although mostly asymptomatic, occurs more commonly than pectus excavatum.


References
1. McHam B, Winkler L. Pectus Carinatum. In: StatPearls. StatPearls Publishing, Treasure Island, FL, 2022. Accessed April 3, 2022. http://www.ncbi.nlm.nih.gov/books/NBK541121/
2. Sharma G, Carter YM. Pectus Excavatum. In: StatPearls. StatPearls Publishing, Treasure Island, FL, 2022. Accessed April 3, 2022. https://www.ncbi.nlm.nih.gov/books/NBK430918/
3. Hills-Dunlap JL, Rangel SJ. Chapter 45. Pediatric Surgery. In: Doherty GM (ed). Current Diagnosis & Treatment: Surgery, 15th edition, 2020. McGraw Hill, New York. pp: 1168-1227. https://accessmedicine.mhmedical.com/content.aspx?aid=1171286475
4. Abid I, Ewais MM, Marranca J, Jaroszewski DE. Pectus Excavatum: A Review of Diagnosis and Current Treatment Options. J Am Osteopath Assoc. 2017;117(2):106-113. doi:10.7556/jaoa.2017.021
5. Morikawa N, Laferriere N, Koo S, Johnson S, Woo R, Puapong D. Cryoanalgesia in Patients Undergoing Nuss Repair of Pectus Excavatum: Technique Modification and Early Results. J Laparoendosc Adv Surg Tech A. 2018;28(9):1148-1151. doi:10.1089/lap.2017.0665
6. Yi E, Lee K, Jung Y, et al. Finding suitable candidates for vacuum bell therapy in pectus excavatum patients. Sci Rep. 2021;11(1):22787. doi:10.1038/s41598-021-02250-x
7. Obermeyer RJ, Cohen NS, Jaroszewski DE. The physiologic impact of pectus excavatum repair. Semin Pediatr Surg. 2018;27(3):127-132. doi:10.1053/j.sempedsurg.2018.05.005
8. Houin P, Stillwell P, DeBoer EM, Hoppe J. Chapter 19. Respiratory Tract & Mediastinum. In: Bunik M, Hay WW, Levin MJ, Abzug MJ (eds). Current Diagnosis & Treatment: Pediatrics, 26th edition, 2022. McGraw-Hill, New York. pp: 492-540.
9. Emil S. Current Options for the Treatment of Pectus Carinatum: When to Brace and When to Operate? Eur J Pediatr Surg. 2018;28(4):347-354. doi:10.1055/s-0038-1667297
10. Sakamoto Y, Yokoyama Y, Nagasao T, et al. Outcomes of the Nuss procedure for pectus excavatum in adults. J Plast Reconstr Aesthet Surg. 2021;74(9):2279-2282. doi:10.1016/j.bjps.2020.12.068


Answers to questions
1.b, 2.b, 3.a, 4.a, 5.c, 6.False


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