Situs Inversus

 

Situs Inversus.

P.K.Ghatak, M.D.

Situs Inversus is an abnormal position of internal organs. When all the thoracic and abdominal organs are on the revered side of the body, the condition is called Situs Inversus Totalis. Situs Inversus Totalis is rare, happens in 1 out of 10, 000 births, but it does not interfere with a normal life. This condition may be missed by a doctor when examining a child, acting more like a TV doctor, pretends to be listening with a stethoscope but actually thinking something else.

Less complete reversed position of viscera in the body are known by these terms:-

1. Dextrocardia. The heart is on the Right Side of the chest cavity and other organs are in the normal side.

2. Situs inversus with Levocardia. Only the heart is on the correct side (Left) and other organs on the wrong side of the body cavities.

3. Situs Inversus Ambiguus. The organs are placed in the body at random fashion, some are on the correct side, others are on reveres side. The Heart and the Spleen are often placed on the right side.

In this category, Reduplication of viscera and absence of viscera are also included.

4. Malrotation of bowel. Mal- rotation and Non-rotation of bowel are sub varieties of situs Inversus ambiguous.

In fetal life, the intestinal tract undergoes 3 stages of rotations. Normal rotation is 270 degrees. Rotation may be only to 180 degrees or 90 degrees and called incomplete or non-rotation respectively.

In incomplete rotation the upper part of the mid-gut (Duodenum) is on the right side of the Superior Mesenteric Artery(SMA) and the lower part of the mid-gut (from distal part of the duodenum to 2 /3 of proximal colon) are in front and to the left of the superior mesenteric artery. The cecum, appendix and right half of colon is unattached. In non-rotation, the gut does not rotate more than 90 degrees. The upper mid-gut is anterior and right of the superior mesenteric artery and the rest of the gut is anterior and let of (SMA).

[From the proximal midgut the small intestine develops and from the distal midgut the cecum and colon develop.]

(On the left, a normal rotation on the right malrotated bowel).

Malrotation of gut is a serious health problem. Most patients experience intermittent incomplete bowel obstruction. Others, develop volvulus and gangrene of bowel. As soon as a diagnosis is made, surgical correction is needed in a short order, whether the patient has any symptoms or not.

Situs inversus is inherited as an autosomal recessive pattern, at times, it is linked with X chromosome. Over 100 genes are implicated in this inherited disorder but the NODAL gene mutation is best known for its clarity. In the very early development, the respiratory tract develops cilia. These cilia beat in unison in one direction - to the left. NODAL gene produces proteins which are components of ciliary axial structures. The directional movement of cilia creates a left to right orientation of the body. It directs rotation of gut and placement of viscera. Mutation of genes DNI 1 and DAH 5 result in Primary Ciliary Dyskenesia. Primary ciliary dyskinesia is associated with situs inversus a well known condition is called Katagener's syndrome. Here patients develop chronic sinusitis, otitis and deafness, bronchiectasis, male infertility due to absence of ciliary movements in vas difference, and later, develop pulmonary insufficiency.

History of Situs Inversus.

It is reported that Aristotle noted the reversed position of viscera in animals. The first human case was reported by Fabricum in 1600, in a man with the liver on the left and the spleen on the right side of the abdomen. In 1663 Marco Severio reported dextrocardia and 1652 Riolan reported more cases of situs inversus.

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