Kelainan Konginetal pada Dinding

Dada
 Osseus abnormalities
• Pectus excavatum
• Pectus carinatum
• Tilting of the sternum
• Sternal fusion, rib, and scapula abnormalities
 Soft tissue abnormalities
• Poland syndrome
• Lymphatic and venous malformation
 Pectus excavatum
• Pectus excavatum, also known as funnel chest, is a congenital chest
wall deformity in which several ribs and the sternum grow
abnormally, producing a concave, or caved-in, appearance in the
anterior chest wall.
• Pectus excavatum is the most common type of congenital chest wall
abnormality (90%), followed by pectus carinatum (5-7%)
• Pectus excavatum occurs in an estimated 1 in 300-400 births, with
male predominance (male-to-female ratio of 3:1)
• Usually sporadic, but increased familial incidence
 Pectus Carinatum
• Pectus carinatum is defined as a continuum of anterior chest wall
deformities noted by the protrusion of the sternum and associated convex
deformity of the adjacent costal cartilages
• Types:
– Chondrogladiolar prominence
• Middle and lower portions of sternum prominent and arch forward
• Most common
– Chondromanubrial prominence
• Upper portion of sternum anteriorly prominent, body of sternum depressed posteriorly,
and a final anterior deflection od distal sternum
• Z-shape in lateral view
• Less common
• incidence: 1 in 1500 live births, male 4:1 female
 Tilting of the Sternum
• Deviation of the typical horizontal positioning of the
sternum in the transverse axis of the body
• Imaging :
– Usually not appearent on radiographs
– Secondary lateral displacement of medial heads of the adjacent
clavicles may assist detection
• Association
– Anterior subluxation of the adjacent clavicular head
– Abnormal convexity of the adjacent rib resulting in palpable
chest wall pump
 Sternal Fusion Abnormalities
• Example : axial noncontract CT of 1 month old
boy with a bifid sternum marked separation of
the clavicular heads and depression of the soft
tissues in the location of the expected upper
sternum
• May be an isolated abnormality and ca require
surgical correction to prevent cardiopulmonary
compromise
 Rib Abnomarmalities
• Types
– Agenesis, hypoplasia, and bifid configuration
• Development anatomic variations can present as
asymptomatic palpable chest wall masses, ex:
– Prominent convexity of anterior rib or costal cartilage
– Prominance of costochondral junction
– Small parachondral nodules of unknown origin
 Scapula Abnormalities
Sprengel’s Deformity
• Failure of descent of the scapula
• Most notabble scapula deformity
• Sometimes the scapula is tethered to the spine
by an osteocartilaginous connection called the
omophyoid bone
• Can cause neck stiffness and restrict abduction of
the arm
Soft Tissues Abnormalities
 Polands Syndrome
• Rare conginetal malformation of chest wall with
hypoplasia or aplasia of the pectoralis major msucle
and adjacent cartilaginous, osseous, and soft tissue
structures.
• Clinical asymetry of the chest
• Pathophysiology : unknown, hypothesizzed to occur
as a result of ipsilateral subclavian artery disruption
• Incidence:
– 1/30000 live births
– Usually unilateral
– Males > females
– Right > left
 Lymphatic Malformations
• Increased number of dilated lymphatic channels lined
by endothelium
• Microcystic, macrocystic, or combined
• Most common in : axilla, chest, cervicofacial region
• In the chest :
– Focal or diffuse masses confined to the subcutanous
tissues or
– Involve the spine mediastinum
 Venous Malformations
• Isolated or multiple dilated, tortuous, thin-walled
(lack of smoooth muscle) venous structures
• Grow in proportion to child growth
• Focal abnormalities through to diffuse
involvement of the deeper soft tissues and bone
• Affects chest wall less than lymphatic
abnormalities