CASE CONFERENCE

AUGUST 3, 2016Jayanth H Keshavamurthy. M.D.

CHECK FEEDING TUBE

CXR FINDING

•atracheal air cyst

Apical lung hernias can be seen on chest radiography as lucent regions that are continuous with the lung parenchyma and displace the trachea.

Apical lung hernias account for 20-30% of all lung hernias.

Surgery is only required when apical lung hernias result in severe symptoms such as cough, hoarseness, or dyspnea.

An apical lung hernia is defined as a protrusion of the lung parenchyma and its pleural coverings through Sibson fascia covering the superior thoracic aperture.

 Apical lung hernias account for 20-30% of lung hernias and are less common than thoracic lung hernias, which protrude between the ribs or around the lateral margin of the thorax.

DEXTROCARDIA WITH SITUS INVERSUS TOTALIS

Situs inversus totalis is the mirror image of normal anatomic position with the right-sided cardiac atria in the left thorax and the right abdominal organs located in the left abdomen and vice versa for the left-sided cardiac atria and abdominal organs.

The incidence and complexity of congenital heart diseases increase with the amount of discordance between the cardiac atria, cardiac ventricle, and position of the abdominal organs.

Situs inversus with levocardia and situs ambiguous with asplenia have close to a 100% rate of associated complex congenital heart disease.

Situs inversus has a 3–5% risk of congenital heart disease, and

situs solitus has a < 0.8% risk of congenital heart disease.

RIGHT AORTIC ARCH

Right Aortic Arch

Types At least five different types Only two of importance

Mirror Image Type — Type I Aberrant left subclavian — Type II

Associated with cardiac defects 5-10% of the timeTetralogy of Fallot most often (71%)ASD or VSD next most often  (21%)Coarctation of aorta rarely (7%)

SHORTNESS OF BREATH

Bochdalek hernia is the result of a diaphragmatic defect arising from incomplete pleuroperitoneal membrane closure in utero.

Large Bochdalek hernias present in the neonatal period; are most frequently left-sided and are associated with pulmonary hypoplasia.  Small hernias are most likely to be discovered incidentally in adulthood.

Bochdalek hernias may contain intraperitoneal or retroperitoneal fat as well as abdominal viscera.

Congenital diaphragmatic hernia of the Bochdalek type is much more common than a Morgagni or a congenital hiatal hernia.

In distinction to the Bochdalek hernia, the Morgagni hernia classically occurs at the right cardiophrenic angle.

•Differential diagnosis•Pericardial fat pad•Pericardial cyst•Mediastinal lipoma•Lymphadenopathy•Cystic thymoma•Bochdalek hernia Differential Diagnosis

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A Morgagni hernia is a rare type of hernia accounting for 2% of congenital diaphragmatic hernias.Herniation through the foramen of Morgagni results from maldevelopment of the septum transversum and fusion failure of the costal and sternal fibrotendinous elements of the diaphragm. 

This herniation usually occurs on the right side.This type of hernia often can be asymptomatic and discovered incidentally through imaging for unrelated problems.

The incidence of congenital diaphragmatic hernias is estimated to be 1 in 2000-5000 live births, with Morgagni hernias accounting for approximately 2% of all cases. The other type of congenital diaphragmatic hernia, known as Bochdalek hernia, is primarily posterolateral and left-sided, and it accounts for more than 95% of all cases.

Approximately 90% of Morgagni hernias are right-sided; 8% are bilateral, and 2% are left-sided. It is a direct hernia because it always contains the peritoneal sac.

POST OP

Congenital diaphragmatic hernias are seen in 1 of every 2000-4000 live births. 84% are left-sided, 13% are right-sided and 2% bilateral .

Associations While a CDH can occur as an isolated condition, associated anomalies

are relatively common and include: pulmonary hypoplasia: also a complication bronchopulmonary sequestration aneuploidy: can be present in up to 50% of cases ref

trisomy 13 trisomy 18 trisomy 21 Turner syndrome: monosomy X Pallister-Killian syndrome: tetrasomy 12p

Fryns syndrome Cornelia de Lange syndrome congenital cardiac anomalies neural tube defects

anencephaly spina bifida

NF 1

The cardio-thoracic ratio (CTR) as assesed on a chest x-ray can appear smaller with a number of entities. They include:

adrenal insufficiency: e.g. Addison disease heart transplant cachexic state 

senility malnutrition bulimia nervosa

asthmatic paroxysm with emphysema constrictive pericarditis physiological factors: e.g. Valsalva manoeuver