Figure 101-4. Locations of thyroglossal duct cysts.

Thyroglossal Duct Cysts

TGDCs are congenital lesions that develop from the thyroglossal duct tract that occurs during the

descent of the thyroid. The thyroid descends from the foramen cecum at the sixth week of gestation and

tracts along the anterior neck in close association with the developing hyoid bone. This tract usually

involutes, but if it persists the tract can present as a TGDC at any point from the foramen cecum to the

thyroid (Fig. 101-4). TGDCs are the most common neck mass besides benign lymphadenopathy in the

pediatric population.17 Physical presentation reveals a midline neck mass that may move with

protrusion of the tongue (Fig. 101-5). US is useful to look at the characteristics of the cyst, but is most

important to determine if there is a normal thyroid gland present. In rare cases, the TGDC could be the

sole functioning thyroid tissue in the patient.

Figure 101-5. Thyroglossal duct cyst.

Treatment is surgical excision and removing the entire tract with a core of tissue and the central

portion of the hyoid bone can minimize recurrence. Rarely, papillary thyroid carcinoma can be found in

the specimen.18 The procedure is performed under general anesthesia with the neck extended and

placement of a shoulder roll. A horizontal skin incision is made over the cyst, with no need to excise

skin unless a fistulous tract or infection has involved the dermis. Careful dissection around the cyst then

allows continued dissection superiorly toward the hyoid bone. Muscular attachments to the central

hyoid bone are removed with cautery and the hyoid is removed en bloc with the cyst and tract. A core

of tissue is then removed above the hyoid bone toward the base of tongue. The tract is then ligated with

absorbable suture. Placing a gloved finger at the base of tongue can sometimes facilitate this maneuver.

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The wound should then be irrigated copiously and closed in a layered fashion. A drain is often used.

Complete excision is considered curative and TGDCs have a recurrence rate of less than 10% when using

this method.17

Other midline lesions to keep in the differential include dermoid and epidermoid cysts, lymph nodes,

thymic cysts, and bronchogenic cysts.

Dermoid Cysts

Dermoid cysts should be considered in the differential of any midline lesion, although they can be off

midline as well. On examination, dermoid cysts present as firm masses that are soft, mobile, nontender,

and grow slowly. Dermoid cysts contain tissue from the three germinal layers, which can include sweat

glands and sebaceous glands. Treatment is total surgical excision. If a nasal dermoid occurs, it can

penetrate the bone and a CT or MRI should be performed to rule out intracranial extension. Midline

dermoid cysts are likely entrapped epithelium at the time of embryonic fusion.19

Thymic Cysts

Lesions of the thymus usually occur in the lower neck, but can present anywhere from the piriform

sinus to the chest. The differential includes thymic cysts, thymic hyperplasia, or thymoma.

Infectious

The most common cause of a neck mass in the pediatric population is benign cervical adenopathy.

Cervical lymph nodes are palpable in 40% of infants and 55% of pediatric patients on examination.20,21

Lymph nodes that are less than 1 cm in size and asymptomatic are considered normal in children less

than 12 years of age.14 Lymphadenitis is most commonly seen in the submandibular region and cervical

nodes, and the source of infection can be viral, bacterial, fungal, or neoplastic. Bacterial and viral

infections causing upper respiratory infections are the most common cause of cervical

lymphadenopathy. Other possible sources of cervical LAD include fungal (in immunocompromised

patients), cat scratch disease, mononucleosis, atypical mycobacterium, and tuberculosis. As mentioned

previously, lymphomas or metastatic disease can also present with nodal enlargement in the neck.

Although rare, one should also consider Kawasaki disease, sarcoidosis, Rosai–Dorfman syndrome, and

histiocytosis X.

Acute suppurative lymphadenitis is most common in the 6-month to 3-year range, with lymph node

enlargement associated with an upper respiratory tract infection. The most common etiologies include

Staphylococcus aureus and group A beta-hemolytic streptococci. There is commonly overlying skin

changes with erythema and cellulitis, with an associated leukocytosis and fever. The lymph node

enlarges and eventually outgrows the blood supply, resulting in central necrosis. Management can

sometimes include needle aspiration of purulence, but often requires an incision and drainage with

placement of a drain or packing under anesthesia. An incision is made in the area of fluctuance and

blunt dissection should break up all loculations. It is important to place the incision in a place that

minimizes risk to vital structures, such as the facial nerve, and allows for the best cosmesis. Antibiotics

are geared toward Staphylococcus and Streptococcus but cultures should be taken with a change in

antibiotic therapy if necessary. The induration from the abscess can take several weeks to resolve, but

recurrence is rare. If the abscess recurs, it should prompt investigation of a possible underlying

anomaly. Viral infections rarely cause suppurative adenitis.

Lymphadenitis can also be chronic or subacute with enlargement of lymph nodes not associated with

an acute infection, although sometimes can occur in a patient with recurrent URIs, tonsillitis, otitis

media, or allergic rhinitis. Often the lymph node is solitary, nontender, and mobile. Excisional biopsies

are recommended generally when the lymph node is larger than 2 cm and has been present for longer

than 8 weeks, or has concerning characteristics such as immobility or rapid growth. Once excised, it is

important to obtain cultures, histopathology, and flow cytometry.

Mycobacterial infections, usually caused by the atypical mycobacterium Mycobacterium aviumintracellulare-scrofulaceum (MAIS) complex, can be very challenging to manage. The pathogens are

usually acquired through the mucous membranes during the eruption of teeth, not through person-toperson transmission. These lesions often cause discoloration of the skin and can fistulize over time (Fig.

101-6). Antibiotic therapy is not always curative. It most commonly occurs in children 1 to 5 years of

age and often has associated asymptomatic lymphadenopathy but no fever or leukocytosis. The response

to antibiotic therapy can be disheartening with little improvement and sometimes surgery is required to

excise the infection. Adjuvant treatment with clarithromycin or rifabutin is often indicated, as is a

consultation with the infectious disease team. In contrast, children with M. tuberculosis usually have

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fever and respiratory symptoms along with positive findings on chest x-ray and the purified protein

derivative skin test.

Cat scratch disease, caused by Bartonella henselae, can cause tender cervical lymph node enlargement

in 25% of cases. Usually there is a history of contact with a cat within 2 weeks of the development of

tender lymphadenopathy. The site of inoculation is usually an extremity. The diagnosis can be made

using B. henselae-based serologic indirect fluorescent antibody test or enzyme immunoassays. The

disease is usually self-limited with resolution in 2 to 8 weeks and no antibiotic therapy is necessary;

however, rarely patients can suffer disseminated disease.22

Figure 101-6. Atypical mycobacterium infection.

Vascular Malformations

Infantile Hemangioma

Infantile hemangiomas (IH) are the most common tumor of infancy. IH are endothelial tumors

characterized by an increased proliferation and turnover of endothelial cells, which differentiates this

from other vascular malformations. IH grow rapidly during the first 9 months (proliferative phase) and

then slowly regress (involuting phase). Regression is usually complete by 4 to 5 years of age.23 They

can occur anywhere in the body, with frequent involvement of the tongue and parotid gland. Usually

hemangiomas occur as a solitary lesion, but 20% of patients can have multiple affected sites. On

examination, hemangiomas are a well-demarcated, raised, red mass that blanches with pressure (Fig.

101-7). Known risk factors include female sex, Caucasian race, preterm birth, and low birth weight.

Most hemangiomas are not visible at birth but become apparent in the first few months of life and 90%

are noticeable by 6 months of age.24 Hemangiomas can occur in the subglottic region and present with

biphasic stridor, presenting between 1 and 3 months of age. Similarly, parotid hemangiomas present as

an enlarging mass within the parotid gland at age 1 to 3 months. Evaluation by Doppler US showing

fast-flow is diagnostic.

Treatment for most IH is conservative, as 90% are small, localized, and not problematic. IH can

ulcerate, bleed, and cause deformities and in these cases local wound care can help and sometimes

intralesional steroid injections may be necessary.25 Injection with steroids can stabilize the growth of IH

in 95% of patients and decrease the size in 75% of patients.26 Systemic corticosteroid treatment can be

used for large lesions that become symptomatic and are not amenable to injection. Propanolol has also

been used in the treatment of IH with varying results. Patients must be monitored closely while on

propranolol therapy due to possible serious side effects.

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Figure 101-7. Hemangioma.

Figure 101-8. Picture of a patient with lymphatic malformation.

Lymphatic Malformation

Lymphatic malformations (LMs) are slow-flow vascular anomalies and 75% of LMs present in the head

and neck. The incidence ranges from 1 in 2,000 to 4,000 births and usually occurs in the first decade of

life. The lymphatic system develops around week 5 of gestation and although the mechanism for the

development of an LM is not completely understood, it is generally thought to occur due to parts of the

lymphatic system that fail to establish connections with the main lymphatic system or venous channels

(Fig. 101-8). Histopathology of LMs shows progressive dilation of abnormal vessels and LMs are

composed of vascular spaces filled with eosinophilic and protein-rich fluid. LMs are present at birth, but

sometimes are not apparent until enlargement due to infection or trauma with hemorrhage. Some

patients are diagnosed with LM when found on prenatal US, and can potentially obstruct the airway and

may require an ex utero intrapartum treatment procedure (EXIT procedure).

LMs are divided in microcystic, macrocystic, and mixed lesions. Microcystic lesions are usually above

2903

the mylohyoid and can involve the floor of mouth, tongue, parotid, and lip. These lesions have poorly

defined borders and infiltrate nearby muscle and fatty planes. Macrocystic lesions are usually below the

mylohyoid and have multiple large, loculated cysts in varying size and shape. LMs can enlarge with

infection or trauma, which can lead to airway obstruction, functional issues (swallowing, speech), or

deformity. Imaging can be helpful to determine the extent of the lesion as well as involvement of

surrounding structures.

Treatment for these lesions is challenging and the main options include observation, sclerotherapy, or

surgery. LMs are benign lesions and this must be kept in mind when planning treatment. Macrocystic

lesions are more amenable to both sclerotherapy and surgical excision, whereas complete removal of

microcystic lesions would often result in unacceptable morbidity due to their infiltrative nature.

Subtotal resection leads to a high recurrence rate and surgery can be difficult due to previous

inflammation and scarring from sclerotherapy. Radiofrequency ablation can be useful in relief of

ulceration tongue lesions or oral mucosa.27 The CO2

laser can help with symptomatic lesions of the

airway or oral cavity, which can reduce bleeding and ulceration. Recently, rapamycin or “sirolimus” has

been investigated as a possible oral medication for LMs with encouraging preliminary results.28

Airway

Airway obstruction can occur from congenital or acquired lesions at any level from the nose to distal

airway. Children can present with tachypnea, dyspnea, and chest-wall retractions and it is imperative to

establish an airway while investigating the potential causes with history and physical, chest radiograph,

and arterial blood gas if appropriate. Laryngoscopy and bronchoscopy are often warranted.

Infants are obligate nasal breathers and nasal obstruction, especially bilateral obstruction, can cause

acute respiratory distress at birth. Choanal atresia or stenosis, piriform aperture stenosis, or nasal septal

deviation can cause significant obstruction. Piriform aperture stenosis is a narrowing of the anterior

nasal passageway that can also cause similar symptoms to choanal atresia. Nasal obstruction can also

result from lesions such as a nasolacrimal duct cyst, encephalocele, or nasal glioma. Nasal examination

and sometimes a CT scan can help to determine the diagnosis.

Macroglossia or retrognathia/micrognathia may also cause obstruction of the oral cavity in the infant.

In the oropharynx, a TGDC, a vallecular cyst, or lingual thyroid can cause airway obstruction.

In the supraglottic region, laryngomalacia can affect the larynx in newborns. Laryngomalacia results

from immature cartilage in the larynx and discoordination of the arytenoids and epiglottis. The

diagnosis is made using a flexible laryngoscope and viewing the larynx while the patient is awake.

High-pitched inspiratory stridor that is worsened with exertion, crying, or excitement is the classic

description. Most children do not require any intervention, but a supraglottoplasty can be performed

when laryngomalacia causes breathing or feeding difficulties. There are many different variations of the

supraglottoplasty, but the basic concept is to remove excess or floppy supraglottic tissues to improve

the airway.

At the level of the glottis, obstruction can occur for several reasons, including vocal cord paralysis

and glottis webs. Bilateral vocal cord paralysis can present with a normal cry but biphasic stridor,

whereas unilateral vocal cord paralysis usually presents with a weakened cry and no respiratory

distress. A mediastinal lesion should be ruled out in patients with vocal cord paralysis and patients with

bilateral vocal cord paralysis warrant a neurologic consultation for possible Arnold–Chiari malformation

or hydrocephalus. A tracheotomy may be necessary for patients with bilateral vocal cord paralysis if

they are in respiratory distress.

Glottic webs usually affect the anterior portion of the vocal cords and can range from a thin

membrane to a thick web that can extend inferiorly to the subglottis. Patients present with dysphonia

and sometimes respiratory distress. Treatment is dependent on the extent of the glottis web, which can

be divided endoscopically in the majority of cases.29

The subglottis is the narrowest part of the airway in an infant due to the cricoid cartilage. Stenosis

can occur congenitally or can be acquired after long-term intubation. Subglottic hemangiomas are

another source of airway obstruction that enlarge after birth and are associated with cutaneous

hemangiomas. The subglottic hemangiomas can occur as an isolated lesion or as part of the PHACES

syndrome (posterior fossa malformation, arteriovenous malformations, cardiac/aortic defects, eye

anomalies, and sternal defect). Treatment can be systemic or intralesional corticosteroids, laser therapy

via endoscopic approach, or open excision.

In patients with a prior intubation, one or multiple subglottic cysts may form and cause airway

obstruction. Treatment entails endoscopic evaluation with marsupialization or removal with a laser or

struction. Treatment entails endoscopic evaluation with marsupialization or removal with a laser or

2904

laryngeal microdebrider.

Tracheomalacia can occur in a primary or secondary form. Primary tracheomalacia is a rare

congenital disease in which the tracheal cartilages collapse on expiration. Most cases of primary

tracheomalacia are mild and no intervention is required, although if severe a tracheostomy may be

required. Secondary tracheomalacia occurs when an extrinsic compression causes collapse of the trachea

on expiration. Treatment is then tailored to the source of compression, whether a vascular anomaly or

mass.

Tracheal stenosis can occur due to congenital complete tracheal rings and can involve a segment or

the entire trachea. Depending on the extent of the stenosis, a tracheal resection or a slide tracheoplasty

may be performed.

Acquired obstruction of the airway includes infectious sources, such as epiglottitis, and foreign-body

aspiration. Although rare, epiglottitis is a life-threatening disease caused by Haemophilus influenza type

B (90% of cases) that occurs most commonly in children ages 2 to 4 years. The H. influenza vaccination

has reduced the number of cases, but pneumococci and B-hemolytic streptococci can also cause

epiglottitis. The disease presents rapidly, with stridor, airway obstruction, drooling, odynophagia, fever,

tachypnea, and tachycardia. As the disease progresses, the child will often assume the “tripod” position

by leaning forward, drooling, and suffering from air hunger. It is important not to agitate the patient

and obtain an airway in the operating room with a tracheostomy set available. Depending on the

stability of the patient, a lateral neck film can confirm the diagnosis with the “thumbprint” sign

demonstrating an edematous epiglottis. The patient should be started on antibiotic therapy and

laryngoscopies performed until the patient’s epiglottis/airway edema allows for a safe extubation.

Airway foreign bodies can be life threatening and the history is of utmost importance. Combining the

convincing history of a coughing/choking episode with an abnormal chest radiograph warrants a

bronchoscopy. Sometimes a bronchoscopy is warranted on the history alone, as less than half of the

patients with an airway foreign body have an abnormal chest radiograph.30 If it was a witnessed

aspiration, obtaining a detailed description of the object and practicing with the bronchoscopy foreignbody forceps on a similar object can be helpful if the patient is stable.

References

1. Torsiglieri AJ Jr, Tom LW, Ross AJ 3rd. Pediatric neck masses: guidelines for evaluation. Int J

Pediatr Otorhinolaryngol 1988;16(3):199–210.

2. Fefferman NR, Milla S. Ultrasound imaging of the neck in children. Ultrasound Clin 2009;4(4):553–

569.

3. Robson CD. Imaging of head and neck neoplasms in children. Pediatr Radiol 2010;40(4):499–509.

4. Lauer MS. Elements of danger – the case of medical imaging. N Engl J Med 2009;361(9):841–843.

5. Amis ES Jr, Butler PF; American College of Radiology. ACR white paper on radiation dose in

medicine: three years later. J Am Coll Radiol 2010;7(11):865–870.

6. Amis ES Jr, Butler PF, Applegate KE, et al. American college of radiology white paper on radiation

dose in medicine. J Am Coll Radiol 2007;4(5):272–284.

7. Patel PM, Alibazoglu H, Ali A, et al. Normal thymic uptake of FDG on PET imaging. Clin Nucl Med

1996;21(10):772–775.

8. Weinblatt ME, Zanzi I, Belakhlef A, et al. False-positive FDG-PET imaging of the thymus of a child

with Hodgkin’s disease. J Nucl Med 1997;38(6):888–890.

9. Rood SR, Johnson JT, Lipman SP, et al. Diagnosis and management of congenital head and neck

masses. American Academy of Otolaryngology – Head and Neck Surgery SIPAC 76481. Washington,

DC: US Government Printing Office; 1981:12–24.

10. Cunningham MJ. The management of congenital neck masses. Am J Otolaryngol 1992;13(2):78–92.

11. Lambert PR, Dodson EE. Congenital malformations of the external auditory canal. Otolaryngol Clin

North Am 1996;29(5):741–760.

12. Ikarashi F, Nakano Y, Nonomura N, et al.. Clinical features of first branchial cleft anomalies. Am J

Otolaryngol 1996;17(4)233–239.

13. Choi SS, Zalzal GH. Branchial anomalies: a review of 52 cases. Laryngoscope 1995;105:909–913.

14. Wiatrak BJ. Clinical Evaluation of the neck. In: Wetmore RF, Muntz HR, McGill T, eds. Pediatric

Otolaryngology: Principles and Practice Pathways. New York, NY: Thieme; 2012:841–856.

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15. Goff CJ, Allred C, Glade RS. Current management of congenital branchial cleft cysts, sinuses, and

fistulae. Curr Opin Otolaryngol Head Neck Surg 2012;20(6):533–539.

16. Leopardi G, Chiarella G, Conti S, et al. Surgical treatment of recurring preauricular sinus: supraauricular approach. Acta Otorhinolaryngol Ital 2008;28(6):302–305.

17. Roback SA. Telander RL. Thyroglossal duct cysts and branchial cleft anomalies. Semin Pediatr Surg

1994;3(3):142–146.

18. Wigley TL, Chonkich GD, Wat BY. Papillary carcinoma arising in a thyroglossal duct cyst.

Otolaryngol Head Neck Surg 1997;116(3):386–388.

19. Grimmer JF. Congenital masses in the neck. In: Wetmore RF, Muntz HR, McGill T, eds. Pediatric

Otolaryngology: Principles and Practice Pathways. New York, NY: Thieme;2012:857–868.

20. Bamji, M, Stone RK, Kaul A, et al. Palpable lymph nodes in healthy newborns and infants. Pediatrics

1986;78(4):573–575.

21. Park YW. Evaluation of neck masses in children. Am Fam Physician 1995;51(8):1904–1912.

22. Rolain JM, Brouqui P, Koehler JE, et al. Recommendations for treatment of human infections

caused by bartonella species. Antimicrob Agents Chemother 2004;48(6):1921–1933.

23. Couto RA, AK Greene. Hemangiomas. In: Rahbar R, Rodriguez-Galindo C, Meara JG, et al, eds.

Pediatric Head and Neck Tumors: A-Z Guide to Presentation and Multimodality Treatment. New York,

NY: Springer;2014:183–192.

24. Oldham KT, Aiken JJ. Pediatric Head and Neck. In: Mulholland MW, Lillemoe KD, Doherty GM, et

al., eds. Greenfield Surgery: Scientific Principles and Practice. 5th ed. Philadelphia, PA: Lippincott,

Williams and Wilkins;2011.

25. Greene AK. Management of hemangiomas and other vascular tumors. Clin Plast Surg 2011;38(1):45–

63.

26. Sloan GM, Renisch JF, Nichter LS, et al. Intralesional corticosteroid therapy for infantile

hemangiomas. Plast Reconstr Surg 1989;83:459–467.

27. Grimmer F, Mulliken JB, Burrows PE, et al. Radiofrequency ablation of microcystic lymphatic

malformation in the oral cavity. Arch Otolaryngol Head Neck Surg 2006;132:1251–1256.

28. Hammill AM, Wentzel M, Gupta A, et al. Sirolimus for the treatment of complicated vascular

anomalies in children. Pediatr Blood Cancer 2011;57(6):1018–1024.

29. Amir M, Youssef T. Congenital glottic web: management and anatomical observation. Clin Respir J

2010;4(4):202–207.

30. Zerella JT, Dimler M, McGill LC, et al. Foreign body aspiration in children: value of radiography

and complications of bronchoscopy. J Pediatr Surg 1998;33(11):1651–1654.

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Chapter 102

The Pediatric Chest

Mary C. Santos and Robert E. Cilley

Key Points

1 Pectus excavatum repair should occur in late childhood or adolescence and is now most frequently

performed with a rigid transversely oriented pectus excavatum bar that pushes the deformity back

into place.

2 Pectus carinatum occurs less frequently than pectus excavatum and does not cause physiologic

impairment; bracing has become the method of choice for treatment.

3 Pulmonary sequestrations (PSs) are abnormal lung tissue with anomalous systemic blood supply that

can be either intralobar or extralobar lesions based on their relationship with the investing visceral

pleura and adjacent normal lung tissue. They do not communicate normally with the trachea or a

bronchus.

4 Congenital cystic adenomatoid malformations (CCAMs) have a normal vascular supply and

communicate with the normal tracheobronchial tree. They result from excessive proliferation of

bronchial structures without the development of the corresponding alveoli. Management of

asymptomatic CCAMs remains controversial.

5 Benign cysts of the mediastinum are relatively common and include thymic cysts, enterogenous

cysts, dermoid cysts, lymphatic malformations (cystic hygromas), and pericardial cysts. Resection is

usually recommended because of the possibility of progressive enlargement with compression,

hemorrhage, or infection.

6 Primary lung tumors in children are rare and include bronchial adenoma, pulmonary blastoma,

inflammatory pseudotumor (composed of inflammatory cells), hamartoma and rarely

bronchoalveolar carcinoma. Resection is generally the appropriate initial therapy.

7 Metastasectomy for many solid embryonal neoplasms can improve survival if the primary disease is

controlled by resection.

8 Tracheoesophageal fistula can occur as esophageal atresia with proximal pouch and distal

tracheoesophageal fistula (most common, at 85% to 95%), esophageal atresia without fistula (5% to

7%), tracheoesophageal fistula without esophageal atresia H type (2% to 6%), and rarer forms of

this anomaly including esophageal atresia with proximal tracheoesophageal fistula and esophageal

atresia with both proximal and distal tracheoesophageal fistula.

9 Approximately 75 children die from foreign-body aspiration or ingestion each year. Bronchoscopic

evaluation and foreign-body removal are performed in the operating room under general anesthesia,

where the rigid bronchoscope with optical forceps can be used to remove most aspirated foreign

bodies safely.

10 Congenital diaphragmatic hernia (CDH) is a physiologic emergency and not a surgical emergency.

The newborn with CDH should be stabilized by nonsurgical means and delayed, well-planned

surgical repair undertaken subsequently.

CHEST WALL DEFORMITIES

Deformities of the chest wall may be obvious at birth but often become more noticeable at the time of

preadolescent and adolescent growth. The physical appearance may vary from barely detectable to

grotesquely deforming. Although there may be some physiologic benefit from surgical correction of

these deformities, restoration of a more normal appearance is equally important in the decision to

operate. The impact of these deformities on normal psychosocial development, as well as the less

certain evidence of cardiorespiratory impairment, constitutes adequate justification in the affected child.

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Embryology, Development, and Etiology of Chest Wall Deformities

In the embryo, the ribs are derived from individual somites as mesoderm differentiates into cartilage

and advances ventrally toward the developing sternum. The rib cartilage eventually approaches the

sternum. The sternum itself is derived independently from two parallel bands of mesoderm that develop

away from the midline. The two sternal bands fuse in a cranial-to-caudal direction and become

progressively chondrified. Transverse divisions of the cartilaginous sternum differentiate into segments

opposite each end of the rib pairs. At birth, small ossification centers are present in the sternum and the

ribs have largely ossified. Final ossification is usually complete by mid adolescence. The process is

sufficiently predictable that sternal ossification is a reliable method of determining bone age. There is a

sharp demarcation in each rib between the ossified portion and the cartilaginous portion, with the latter

becoming ossified only much later in life. The etiology of chest wall deformities is poorly understood.1

Explanations include abnormal intrauterine pressure applied to the chest, abnormalities of

diaphragmatic development, connective tissue abnormalities, and genetic predisposition. Abnormal,

excessive, or asymmetric growth of the costal cartilages associated with the 3rd to 10th ribs is most

implicated.2 Sternal clefts are understood as a failure of fusion of some portion of the sternal bands.

Pectus Excavatum

Pectus excavatum from the Latin “hollowed chest” is the most common deformity of the anterior chest

wall occurring in 1 in 400 to 1 in 1,000 children and accounting for more than 87% of chest wall

deformities. A family history is present in 45% of patients.2,3 Pectus excavatum results from abnormal

regulation of the growth of the costal cartilages. There is a corresponding posterior curve in the body of

the sternum beginning at the manubrium and extending to the xiphoid. The deformity is rarely precisely

symmetric, with one side (usually the right) slightly more curved in than the other. Asymmetry may be

pronounced with the sternum rotated nearly to the sagittal plane. The deformity may be apparent at

birth but may become more apparent during growth and development, particularly during adolescence.

Pectus excavatum occurs more frequently in males than females. Scoliosis is present at increased

incidence in this population although rarely requires surgical correction. Patients often have an asthenic

build and stoop-shouldered posture. Marfan syndrome predisposes to pectus excavatum. The diagnosis

of Marfan syndrome should be considered when patients are referred for evaluation of a chest wall

deformity.

At the time of surgical consultation, concerns about the cardiopulmonary implications of the chest

wall deformity are often paramount in the mind of the referring physician as well as the family. Many

patients will have complaints of chest pain and/or shortness of breath. Most patients are disturbed by

the appearance of the deformity, and some may be profoundly depressed and dysfunctional. The

physiologic consequences of the deformity are less certain. Invasive and echocardiographic assessment

of cardiovascular performance has demonstrated improvement in cardiac function and resolution of

mitral valve prolapse in some patients following surgical correction of pectus excavatum. Studies have

also demonstrated improved body image and subjective physical ability.2,5–9 The cardiorespiratory

benefits of the repair remain controversial with more recent studies demonstrating improvement after

bar removal especially with dynamic testing.9 Multiple studies of patient and parent satisfaction have

demonstrated very well to excellent results.2,5,8–12 Clinical assessment of the severity of the deformity

has included chest radiographs, computerized tomography (CT), caliper measurements, and contrast

volume measurements of the cavity. The “pectus index” is defined as the ratio of the maximum internal

transverse diameter of the thorax to the minimum sternovertebral distance.7 If cardiac disease is

suspected on the basis of the history and physical examination, an echocardiogram may be indicated.

Static pulmonary function testing is rarely helpful but exercise testing may be helpful.

Surgical Correction of the Pectus Excavatum

1 Although pectus excavatum is diagnosed in infants and young children, repair should be deferred to

teenage years. This leads to improvement in cardiopulmonary status while avoiding recurrence and

acquired thoracic dystrophy of early repair.

Traditional Operations

Traditional surgical correction is performed through an inframammary incision which is kept within the

nipple lines (Fig. 102-1). The operation requires the exposure of costal cartilage by elevation of the

pectoral muscles from the chest wall. The involved cartilages usually from 3 to 7 and parts of 8 to 10

2908