Congenital Chest Wall Deformities Pectus Excavatum, Funnel Chest |
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Incidence | |
Pectus excavatum is the most frequent type of congenital chest wall deformities with one in 1000 children. | |
Clinical significance | Illustrations |
1. A pectus excavatum is an anomaly already recognizable in the first year of life. Later on, the deformity remains the same, or, less frequently, increases during growth. 2. In the second decade psychological embarrassment is possible in teens, but severe disturbances of circulation or respiration are unusual. 3. Important combined malformations, such as Marfan syndrome, should not be missed. |
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Etiology | |
Unknown. | |
Pathology, anatomical types | Illustrations |
The depression of the sternum and of the adjacent pairs of ribs is wide and dell-like or narrow and deep, and asymmetrical or symmetrical, constituting 4 anatomical variations. | show details |
Pathophysiology | |
Mostly in young adults cardiovascular and respiratory dysfunctions may be observed which hinder physical activities. Due to a shifting of the heart, the large vessels and lungs, a decreased cardiac output and a restrictive pulmonary dysfunction result. | |
Clinical presentation (history) | Illustrations |
In infants and toddlers one may observe inappetence, dysphagia, failure to thrive, inspiratory stridor, and akinesia. | show details |
Clinical presentation (local findings) | Illustrations |
The clinical presentation is according to the already mentioned anatomical types and variations. A general clinical examination is very important: muscular or asthenic posture, the latter with hanging shoulders, bowing of the upper part of the body, and protruding belly. In addition, scoliosis and other deformities of the spine, and combined malformations can be present. | show details |
Clinical presentation (clinical skills) | Illustrations |
The outer sagittal diameter of the thorax serves as an objective and reproducible measure (the depth of the sternum is one of the various elements of a depression deformity); it can be determined with a pelvimeter and includes the distance between the deepest point of the sternum and the opposite spinous process. In body lengths of 55, 105 and 155 cm, the values of 8, 12 and 14 cm or lower, respectively, are abnormal. Beside the body length and weight and their percentiles, deviations of the spine at rest and on defined movements must be determined. Also, a photographic documentation of the deformity is necessary with shots taken from the front and from both sides with an oblique view. | show details |
Natural history | |
See Pathophysiology and clinical presentation. The following points are important for therapeutic and prognostic reasons: | |
Differential diagnosis | Illustrations |
With the knowledge of the clinical features of a pectus excavatum and a precise clinical examination, there is no differential diagnosis except for: | show details |
Work-up examinations | Illustrations |
The additional examinations serve as an objective definition of the type and the degree of funnel chest or another chest wall deformity. | show details |
Therapy | Illustrations |
In contrast to the pectus carinatum, there is no other therapy in funnel chest than surgery, and the indications are: | show details |
Prognosis | |
With open surgery, the deformity remains relieved in more than 80 %, and physical activity is restored distinctly. Nevertheless, at least in open surgery there is a decreased elasticity of the chest wall. For the Nuss-procedure which was initiated in 1998, long-term data over many years are not yet available, and beyond puberty the procedure is more difficult to perform. The early results are excellent or good in more than 90 % while asymmetric variants of funnel chest are less suitable for this type of surgery (Hosie S et al and Nuss D et al: Eur J Pediatr Surg 12:235 and 230, 2002). In general, a more advanced age at the time of surgery, and an asthenic posture are unfavorable conditions for surgery. Recurrences occur with both procedures. In Nuss-procedure the implants are removed 2 to 4 years later.
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