Photos of Pectus Excavatum (Funnel chest)

Definition :

Congenital posterior displacement of lower aspect of sternum.This gives the chest a somewhat "hollowed-out" appearance.
Pectus Excavatum is one of the most common congenital deformity of the chest wall "The condition is more common than Down syndrome".in Pectus Excavatum several ribs and the sternum grow abnormally, producing a concave, or caved-in, appearance in the anterior chest wall and a displaced heart which is often palpable on the left mid-axillary line slightly below the armpit.

-Pectus Excavatum is sometimes also called sunken chest or funnel chest.
-Pectus excavatum occurs more often in males than females (3:1) ,some studies say that this ratio reached 6:1 ,and accounts for 90% of congenital chest wall deformities.

Pathogenesis :

pathogenesis of pectus excavatum still unclear, and, to date, no known genetic defect is directly responsible for the development of pectus excavatum despite of familial occurrence of pectus deformity that reported in 35% of cases, investigators have hypothesized that the deformity results from unbalanced overgrowth in the costochondral regions.in other words; an abnormality of connective tissue (cartilage) sometimes associated with Marfan and Ehlers Danlos syndromes.

The "Nuss" procedure or pectum bar operation, This operation involves the placement of a customized, long, curved metal bar under the sternum. Two small incisions are made on each side of the chest to allow insertion of the bar. Another small incision is made to insert a thoracoscope, which allows us to watch the bar as it goes under the sternum. The bar is secured to the ribs on the side of the chest and pushes the sternum forward. Neither the rib cartilages nor sternum are cut. The bar usually remains in place for two years and is then removed with a second, smaller operation. The principle of the pectus bar operation is that the rib cartilage remodel while the bar is in place--similar to the remodeling of the mouth with orthodonture.

Repair of Tetralogy of Fallot with Atrioventricular Canal

Redmond Burke MD, Chief of Pediatric Cardiovascular Surgery at Miami Children's Hospital demonstrates the repair of TOF with AVC, Tetralogy of Fallot with Atrioventricular Canal. The video is graphic, showing the operative repair and the postoperative recovery. The program website is www.pediatricheartsurgery.com

Lymphoma; a common cause of Intussusception above 6 years

An 8 year old is seen in the emergency room secondary to abdominal pain. Further evaluation confirms the presence of intussusception. The most likely precipitating cause is

• A) colon polyp
• B) Meckel's diverticulum
• C) lymphoma
• D) parasite infection
• E) foreign body

The answer is ( C ).
Intussusception is the most common cause of intestinal obstruction in the first 2 years of life. It is more common in males than in females. In most cases (85%) the cause is not apparent. Associated conditions that can result in intussusception include polyps, Meckel's diverticulum, Henoch–Schönlein purpura, lymphoma, lipoma, parasites, foreign bodies, and viral enteritis with hypertrophy of Peyer patches.

Intussusception of the small intestine occurs in patients with celiac disease and cystic fibrosis—related to the bulk of stool in the terminal ileum. Henoch–Schönlein purpura may also cause isolated small-bowel intussusception. In children older than 6 years, lymphoma is the most common cause. Intermittent small-bowel intussusception is a rare cause of recurrent abdominal pain.

Pediatric Bone Marrow Aspiration

Osteogenesis imperfecta and it`s subtypes

Krish 5 Year old boy brought to the hospital by his mother because she has noticed that her child did not have appropriate height for his age and having Mild limb deformity.This child has a previous Hx of Right tibial fracture when the child was 3years old.

Osteogenesis imperfecta

Osteogenesis imperfecta also known as Brittle Bone Disease or Lobstein syndrome
It is a group of diseases where there is an increased fragility of bone with frequent fractures.
They are all associated with type I collagen mutations.It is a genetic disorder with autosomal dominant inheritance

Mainly there are four subtypes of Osteogenesis imperfecta
Type I mild disease(Collagen is of normal quality but is produced in insufficient quantities)

1. Blue sclerae
2. Slight protrusion of the eyes
3. Mild prepubertal bone fragility
4. Little or no deformity or Slight spinal curvature
5. Loose joints
6. Mild short stature
7. Poor muscle tone
8. Hearing loss in about 50% of adults
9. Bruises easily
10. Dentinogenesis imperfecta may be absentin 1A and present in 1B


Type II perinatal lethal(Collagen is not of a sufficient quality or quantity)

1. Most cases die within the first year of life due to respiratory failure or intracerebral hemorrhage
2. blue sclerae
3. In utero fractures
4. Micromelia
5. Marked long bone deformity
6. Platyspondyly
7. Lethal in the perinatal period
8. Broad but poorly mineralized skull


Type III progressive deforming(Collagen quantity is sufficient but is not of a high enough quality)

1. Blue then white sclerae
2. Progressing deforming of the long bones and spine secondary to fractures and gravity
3. Relative macrocephaly and triangular faces
4. Extremely short stature and barrel-shaped rib cage
5. Respiratory problems
6. Poor muscle tone and Loose joints


IV moderately severe disease(Collagen quantity is sufficient but is not of a high enough quality)

1. White sclerae
2. Moderate fragility and mild to moderate bowing of long bones especially before puberty
3. Variable short stature
4. Dentinogenesis imperfecta absent in IVA and present in IVB