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