pectus excavatum (pe). there is a large group of congenital abnormalities of the thoracic cage that...

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Pectus excavatum (PE)

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Pectus excavatum (PE)

There is a large group of congenital abnormalities of the thoracic cage that manifest as deformities of the anterior chest wall.

Question: How many groups of the deformities of the

anterior chest wall ?

AnswerThis diverse group includes :1. pectus excavatum2. pectus carinatum3. Pouter pigeon breast4. poland syndrome5. cleft sternum

Pectus excavatum (PE) is the most common chest wall malformation

and one of the most frequent major congenital anomalies.

It approximately occurs in 1 in every 400 births, with a 4:1 male predominance.

In patients with PE, the sternum and adjacent chest wall are displaced posteriorly toward the spine, creating

a depression of the anterior chest wall.

A preoperative computed tomography (CT) chest scan is currently recommended by some authors to evaluate the extent of the deformity and to plan repair.

Non-enhanced CT scanning.

Pathogenesis The currently accepted etiologic theory is

that excessive growth of the lower costalb cartilages (gristle)occurs that forces the sternum into a concave position.

The reason for this overgrowth of costal cartilages, which frequently increases the severity of the deformity in the preschool-age child and again during the pubertal years, is currently unknown.

Symptoms Symptoms are infrequent during

early childhood, apart from a shy awareness of the abnormality and a typical unwillingness to expose the chest while swimming or taking part in other social or athletic activities.

It is particularly important that this deformity be corrected, if possible, before the child starts school,because it may cause severe psychologica ( mental ) and emotional damage.

During early adolescence Easy fatigability Shortness of breath with mild exercise Decreased stamina Tachycardia ( bradycardia ) ,and/or

palpitations are common Pectus excavatum patients commonly

try hard to keep up with their peers physically

Who? PE ? pectorales

Treatment

Resect a major portion of the deformed costal cartilages 。

The sternal osteotomy and resection of short segments of cartilages from the deformed ribs.

A new minimally invasive ( invade ) technique for repair of PE (MIRPE) was reported by Nuss et al. in 1998 for young children (mean age under 5 years)

Donald Nuss

The Nuss procedure Minimally invasive repair us

ing a convex steel bar guided across the chest through a substernal tunnel and then twisted with a wrench to force the convex side anteriorly, which elevates the depressed sternum and deformed costal cartilages.

Pre-operation

During-operation

post-operation X-ray

After-operation

Recent modifications of the MIRPE adopted by many surgeons include the use of thoracoscopy to visualize passage of the clamp behind the sternum

References1. Feng J, Hu T, Liu W, Zhang S, Tang Y, Chen R, Jiang X, Wei F:The biomecha

nical, morphologic, and histochemical properties of the costal cartilages in children with pectus excavatum.J Pediatr Surg 2001,36(12):1770-1776

2. Fokin AA, Steuerwald NM, Ahrens WA, Allen KE:Anatomical, histologic,and genetic characteristics of congenital chest wall deformities.Semin Thorac Cardiovasc Surg2009,21(1):44-57.

3. Hebra A. Minimally invasive pectus surgery. Chest Surg. Clin. N. Am.2000;10:329–339

4. Molik KA, Engum SA, Rescorla FJ, et al. Pectus excavatum repair:experience with standard and minimal invasive techniques. J. Pediatr.Surg. 2001;36:324–328

5. Nuss D, Kelly RE Jr, Croitoru DP, et al. A ten-year review of a mini-mally invasive technique for the correction of pectus excavatum. J. Pe-diatr. Surg. 1998;33:545–552

6. Eric W. Fonkalsrud, M.D. Current Management of Pectus Excavatum. World J. Surg. 27, 502–508, 2003

7. Martinez D, Juame J, Stein T, et al. The effect of costal cartilage resection on chest wall development. Pediatr. Surg. Int. 1990;5:170–173