Chest Wall




(1)
Groningen, The Netherlands

 




Chest Wall Deformity






  • A312070_1_En_1_Figa_HTML.gif Complaint: there is a deformity of the chest wall.


  • A312070_1_En_1_Figb_HTML.gif Assessment: deformity of the chest wall.


  • A312070_1_En_1_Figc_HTML.gif Differential diagnosis:



    • pectus carinatum (pigeon breast)


    • pectus excavatum (funnel chest)


    • fusion disorder of the sternum


    • absent ribs


    • Poland syndrome


  • A312070_1_En_1_Figd_HTML.gif Explanatory note: pectus carinatum. The sternum and the adjoining cartilage and bone are anteriorly prominent (Fig. 1.1). The abnormality is most noticeable in the first year of life, but can also develop during puberty (Fig. 1.2). As a rule, pigeon breast is only a cosmetic issue. Respiratory difficulties may occur when the sternum shifts strongly forward, causing the thorax to be in a continuous inspiratory position.

    A312070_1_En_1_Fig1_HTML.jpg


    Fig. 1.1
    (a) Boy, about 5 years old, with a pectus carinatum (pigeon breast). (b) After treatment with a trunk orthosis with a pressure pad


    A312070_1_En_1_Fig2_HTML.jpg


    Fig. 1.2
    A pectus carinatum (pigeon breast) can also develop during puberty


Pectus excavatum

There is an indentation on the front side of the chest. The deepest area lies at the level of the distal part of sternum and the xiphoid process (Fig. 1.3). The abnormality is usually present at birth, but can also develop later. A funnel chest seldom causes physical complaints, the issue tends to be only cosmetic. The abnormality is common in Marfan syndrome.

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Fig. 1.3
Pectus excavatum (funnel chest)


Fusion disorder of the sternum

Partial fusion disorders of the cranial area are the most common. In the distal area they are very rare. It is also possible that the sternum is split along the entire length (total sternal fissure). This abnormality is accompanied by cardiac anomalies and/or defects in the diaphragm.


Absent ribs

In this abnormality several ribs are usually absent on one side of the chest wall. There may also be sternal and vertebral abnormalities such as hemivertebrae and block vertebrae. As a result there is a flail thorax, which can lead to a shortness of breath.


Poland syndrome

In Poland syndrome1 there is a unilateral absence of the pectoralis minor muscle and the sternal part of the pectoralis major muscle (Fig. 1.4). Males are affected in 70 % of cases. It is generally combined with abnormalities of the hand on the same side, such as small hands (hypoplasia) with absent fingers, webbed fingers (syndactyly) and/or shortened fingers (brachydactyly). The combination of hypoplasia of the hand, syndactyly and brachydactyly is sometimes called symbrachydactyly. There may also be absence of the forearm flexor muscles and the entire arm may be under-developed. Associated anomalies may be the Klippel-Feil syndrome1(short neck), Möbius syndrome1 (paralysis of the facial muscles), a Sprengel deformity (elevated shoulder blade) and pectus excavatum.

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Fig. 1.4
Poland syndrome. On the right side there is a unilateral absence of the pectoralis minor muscle and the sternal portion of the pectoralis major muscle



Jun 26, 2017 | Posted by in PEDIATRICS | Comments Off on Chest Wall

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