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Harvard Commentaries
Harvard Commentaries
Reviewed by the Faculty of Harvard Medical School

What Your Doctor Is Saying What Your Doctor Is Saying

Twisted Medicine

September 12, 2012

By Robert H. Shmerling M.D.

Beth Israel Deaconess Medical Center

Has your stomach ever felt like it's all in knots? Are your nerves jangled? Got a twisted sense of humor? These, of course, are figures of speech.

In the human body, a certain amount of twisting and turning is normal but too much can be terrible. But there are conditions that result when things in the body get twisted or even knotted. Some are serious. Here are some of the most common and important conditions.

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The Twisted Ovary

Although suspended by ligaments from the pelvic wall, the ovary has a fair amount of mobility within the pelvis. Occasionally, the ovary twists on its ligaments. This condition is called ovarian torsion. It usually occurs when there is a cyst or other benign tumor. It can occur in any female, even newborns, but it's most common in young adult women.

Torsion of the ovary is an emergency. As blood continues to flow into the ovary, the twisting of the veins blocks the blood's exit. This leads to enlargement, increased pressure, and death of the ovary; bleeding and abdominal infection may complicate this condition. Treatment requires surgical "detorsion" (untwisting) of the ovary. Depending on the duration of symptoms and appearance of the ovary, the surgeon may be able to save the ovary or may need to remove it.

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The Twisted Testicle

Testicular torsion can develop when the testis is not adequately attached to its covering (called the tunica vaginalis) within the scrotum. Risk factors include an attachment of the testicle that is higher up in the scrotum (closer to the pelvis), strenuous physical activity and testicular trauma.

With testicular torsion, the twisted blood vessels cause severe pain and swelling. It can affect males of any age but the condition is most common among newborns and after puberty. Immediate surgery is required to untwist the testicle. Often, it's necessary to remove the testicle, especially if the condition has been present for more than 12 hours.

If the testicle suffers significant tissue death, proteins from within the testicle may enter the blood stream. The immune system may make anti-sperm antibodies that can lower fertility. Prompt diagnosis and treatment may be important to maintain normal fertility.

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The Twisted Intestine

The intestinal tract is particularly mobile. Although it is fixed to the abdominal wall in several key spots, twisting of the bowel (the medical term is volvulus) is not a rare problem. Children in particular are prone to volvulus. As with ovarian and testicular torsion, volvulus can cause tissue death by cutting off the circulation. It can be life threatening if it isn't diagnosed and corrected quickly with surgery. One risk factor is abnormal fetal development of the intestinal tract. But, it can also occur in kids who are perfectly healthy.

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Twisted Bones -- Spiral Fractures and Scoliosis

When you think of a bone fracture, you may think of it as a twig snapping in half. But the way a bone breaks depends on the forces that cause it. For example, if the leg is twisting or spinning when the injury occurs, a "spiral fracture" can occur. It causes the bone to break into several pieces with a diagonal (rather than horizontal) fracture and the bone fragments rotate. Although a spiral fracture can occur in toddlers who are unsteady with walking — in fact, spiral fractures of the lower leg are often called "toddler's fractures" — they are also a common injury in cases of child abuse.

Abnormal spine curvature is called scoliosis (from the Greek, skolíosis, which means "a bending"). The spine might appear C- or S-shaped rather than straight when viewed from the back. From the side, excessive forward curvature is called lordosis while excessive backward curvature is called kyphosis. While scoiliosis affects an estimated 2% of people, mild cases may cause no symptoms. More significant scoliosis that gets worse over time may cause back pain, deformity and breathing problems.

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Even the Heart Can Twist

Torsades de pointes, meaning "twisting of the points," is an unstable heart rhythm that can cause fainting or sudden death. (See the EKGs below.) Risk factors include certain medications, inherited conditions and low potassium levels in the blood. Fortunately, it can be treated by stopping the offending medication or by treatment with medications that stabilize the heart rhythm, a pacemaker, electrical shocks or a combination of these.

Normal EKG jpgNormalEKG

Torsades de pointes EKG jpgTorsadesEKG

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Umbilical Cord Knots

The umbilical cord would seem to be a prime candidate for knotting. It's long and the baby is moving around — key factors in twisting or kinking. An estimated 1% of pregnancies are complicated by a knot in the umbilical cord. They are most common with identical twins, early in pregnancy, with older mothers who have had previous children, and with long umbilical cords. Knots can restrict blood flow through the umbilical vessels. This increases the chances of miscarriage or fetal death by four times. It can be detected by ultrasound or a doctor may suspect it when there are signs of fetal distress. A caesarean section is necessary.

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Fetal Development -- Normal Twisting Gone Awry

There's actually a lot of twisting going on during fetal development. For example, the intestinal tract starts out as a straight tube. As it lengthens during development, it makes a number of twists and turns. As a result, the stomach winds up on the left, the small intestine moves toward the center of the abdomen, and the large intestine crosses under the ribs to the left, then descends to the rectum on the left. Some strategically placed "anchors" prevent excessive movement of these structures.

Perhaps the most amazing thing is how often all this twisting and turning goes well! But, it can go terribly wrong at times. For example, in a condition called "situs inversus," the contents of the chest and abdomen twist the wrong way and land in the opposite location from what is normal. The heart, stomach and spleen are on the right, while the liver, gall bladder and appendix are on the left. This may cause no health problems (other than confusing doctors when X-rays or EKGs or other tests are taken). However, situs inversus can be associated with other genetic disorders, such as Kartagener's syndrome, in which sinus and lung infections are more common.

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Knotty Treatments

As an example of Murphy's law, a catheter (tube) or wire placed in the body as a treatment or test may, on occasion, get tangled. Examples include catheters inserted into the bladder, pacemaker wires placed in the heart, or heart catheters placed within vessels near the heart or within its chambers. As you might imagine, it's important to uncoil these. Even experienced doctors run into these problems at times. Experience (or the help of surgical colleagues) help them resolve these problems.

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The Bottom Line

What all of these conditions have in common is the mobility of the structure and some risk factor (such as an ovarian cyst or long umbilical cord). However, in many cases, there's no clear cause for abnormal twisting, turning or knotting. If you've never heard of these disorders, it's likely you've never had one of them. Be glad about that! These conditions are painful, dangerous or both. Fortunately, they are relatively rare.

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Robert H. Shmerling, M.D. is associate physician at Beth Israel Deaconess Medical Center and associate professor at Harvard Medical School. He has been a practicing rheumatologist for over 20 years at Beth Israel Deaconess Medical Center. He is an active teacher in the Internal Medicine Residency Program, serving as the Robinson Firm Chief. He is also a teacher in the Rheumatology Fellowship Program.


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