Upload
ali-ahmad
View
457
Download
0
Embed Size (px)
Citation preview
Congenital Anterior Chest Wall Deformities
Dr. Ali M AhmadMBBCh, MS, MD, MRCS-Ed, EBPS
Associate Consultant Pediatric Surgery; KAAUH_ PNU
Congenital Anterior Chest Wall Deformities
1. Pectus excavatum2. Pectus carinatum3. Poland syndrome4. Jeune syndrome5. Sternal defects
Most common anterior chest wall deformity
Male : Female, 3:1
Deep depression of the sternum, usually involving the lower half
Usually the sternum is asymmetric & mostly the depression is more on the right side
1- Pectus Excavatum
The next most common disorder of the chest wall (7%).
Boys : girls (4:1)
2- Pectus Carinatum
Spectrum of disorders involving hypoplasia of the chest wall
This may involve, alone or in combination, the pectoralis major, pectoralis minor, serratus anterior, ribs, and soft tissue.
Incidence:1:32,000 live births
Boys: Girls 3:1
Right side in 75% of patients
3- Poland Syndrome
Autosomal recessive Failure of chest wall growth in utero Narrow chest and pulmonary hypoplasia
Patients typically die at birth Though many cases are being recognized
with prolonged survival
No adequate treatment exists
4- Jeune Syndrome{Asphyxiating Thoracic Dystrophy}
Categorized Into 4 Types
A. Thoracic ectopia cordisB. Cervical ectopia cordisC. Thoracoabdominal ectopia cordisD. Cleft sternum
5- Sternal Defects
Naked heart
Failure of somatic structures to form over the heart
Leaving it completely exposed
Sternal anomalies run a spectrum from being completely split to being almost completely intact with a central defect
A- Thoracic Ectopia Cordis
Differs from thoracic ectopia cordis by the amount of superior displacement of the heart.
Craniofacial abnormalities are often present and extremely severe; they can include fusion of the apex of the heart to the mouth.
No survivors or successful repairs have yet been documented
B- Cervical Ectopia Cordis
Inferiorly displaced heart Inferiorly cleft sternum Heart is covered by
membrane or thin skin.
Abdominal wall defects are also common {Cardiac, pericardial, diaphragmatic, abdominal wall, and sternal defects is also referred to as Cantrell Pentalogy}
C- Thoraco-abdominal Ectopia
Cordis
Least severe o Partially or completely cleft
sternumo Heart is covered and is in a normal
position
Several associations are seen with cleft sternum, but cardiac defects are rare.
A band like scar often extends from the sternum to the umbilicus or superiorly to the neck is seen
Hemangioma of the head and neck can also be found
Surgical Repair is performed through a midline incision, and the 2 halves of the sternum are approximated with non absorbable suture
D- Cleft Sternum
Pectus Excavatum
Pectus Excavatum
Deep depression of the sternum, usually involving the lower half
Lower 4-6 costal cartilages, dip backward abnormally to increase the deformity and push the sternum backward toward the spine
Sternum is asymmetric & depression is more on the right side
90% of chest wall deformity
Incidence: 1 in 300 live births
More frequent in males 3:1
It does occur in families
It progresses to its maximum after the growth period in
adolescence.
Pectus Excavatum
20% Scoliosis
2% cardiac anomalies
Ehlers-danlos or Poland’s syndrome
2% Marfan's syndrome (Mostly the sever form)o Need genetic evaluationo Ophthalmologic screening for subluxation of the lenso Echocardiography to evaluate for dilatation of the aortic
root and mitral valve prolapse
Associated Abnormalities
Cause is unclear May be due to overgrowth of costal cartilage
Coexistence with other musculoskeletal disorders, such as Marfan syndrome and scoliosis suggests that some abnormality of connective tissue may be involved
37% have a family history of PE further supports the theory of genetic predisposition
Etiology
PE can range from mild shallow defects to defects in which the sternum almost touches the vertebral bodies
Due to 2 factors: o The degree of posterior
angulation of the sternumo The posterior angulation of the
costal cartilages.
Assessment
Psychological : o Shy & Not participate where their chest is exposed such
as in swimming or athletic events
Chest pain
Tenderness and pain in the area of overgrown cartilage
Breathing difficulty upon exertion or exercise
Clinical Presentation
In severe defects, Heart is displaced to the left of the sternum
ECG may demonstrate strain on the right side of the heart
The diaphragm make larger movements to provide enough oxygen and carbon dioxide exchange to meet the demand of the body under exercise conditions.
Clinical Presentation
Plain chest x-ray
Computed tomography
Pulmonary function tests
Cardiac investigations
Investigation
1. Lateral dimension of cardiac silhouette
2. The distance between the most prominent and the recessed point of sternum and the anterior edge of vertebral body
3. The lateral transverse dimension of chest
Chest X-Ray {Lateral View is More Valuable Than PA X-ray}
Ratio of the transverse distance to the antero-posterior distance
Score of 3.25 or higher is associated with a severe defect requiring surgery
CT
PFT & cardiac output are normal with the patient at rest
In upright intense exerciseo Cardiac output is usually decreased when compared to
normal individuals of the same age
o PFT is reduced, and depending on the severity of the defect, this reduction can be from 10-30%.
Pulmonary function test & cardiac output evaluation
Psychological support
Asymptomatic patients are given an exercise program to correct their posture and are reevaluated every six months to follow their progress
Non-surgical Managements
External bracing technique PECTUS CARINATUMNon Surgical Rx
Ravitch procedure Leonard procedure Silicone implants Nuss procedure
Surgical Managements
1. Excision of all deformed cartilage from the perichondrium2. Division of the xiphoid from the sternum3. Transverse sternal osteotomy4. The sternum displaced anteriorly and held into position by using wires.
Ravitch procedure for PE
Ravitch For Pectus Carinatum
Bilateral curvilinear incision Resection of lower 4-5 cartilages Wedge sternotomy Wires through sternum and brace for 6-12 weeks During that period of time, the cartilages reform in the new
position and the defect, thus, is corrected
Leonard procedure
Silicone implants in PE
Rt
Silicone implant in POLAND SYNDROME
In 1998, Donald Nuss et al presented their 10-year results of a new and minimally invasive approach to repairing PE.
Their repair was based on the observation that even the chest wall of adults can be remodeled, as seen in adults with barrel chest due to emphysema, without the need for resecting ribs or cartilage.
The other key observation was based on the management of orthopedic conditions, such as scoliosis and club foot. These conditions can be corrected conservatively by placing splints and leaving them in place for long periods.
MIS {NUSS PROCEDURE}
MIS with pectus bar implant to remodel the chest wall over a 2 to 3 year The operating time is 1-2 hours Immediate visual fix of the deformity No cutting / removal of cartilage & Minimal blood loss
Congenital Anterior Chest Wall Deformities
Dr. Ali M AhmadMBBCh, MS, MD, MRCS-Ed, EBPS
Associate Consultant Pediatric Surgery; KAAUH_ PNU
Thank you