Wednesday, April 7, 2010

Top 10 Bizarre Medical Anomalies

[WARNING: some images and content may disturb and are not work safe.] Gone are the days of sideshows displaying freaks of nature, but the diseases that caused these so-called “freaks” are still with us. Political correctness has made it impolite to display the sufferers of these illnesses, so we are left with the Internet – the last resort of those with a fascinating for the bizarre. This list looks at some of the most unusual (and sometimes horrifying) anomalies of medical science. Viewer Discretion is advised

Diprospus
(sometimes called Craniofacial duplication) is a rare disorder in which the face is duplicated on the head (as in the picture above). This is not to be confused with fetus in fetu (item 9) which is a joining of two separate fetuses; diprosopus is caused by a protein called (believe it or not) “sonic hedgehog homolog”. The odd name is due to a controversial tradition in molecular biology to use unusual names for genes. The protein determines the makeup of the face, and when there is too much of it, you get a second face in a mirror image. If you do not have enough of the protein, you can end up with underdeveloped facial features. Children with this defect are normally stillborn, but a young girl, Lali Singh, born in 2008 survived for 2 full months before dying of a heart attack.


Fetus in Fetu
The man pictured above is Sanju Bhagat aged 36 from India. He is fully pregnant with his own twin. Because Sanju lacked a placenta, the fetus inside him attached directly to his blood supply. Doctors delivered the twin which was severely malformed and did not survive. Fetus in fetu is an extremely rare disorder in which a twin somehow becomes connected (internally or partly externally) to its twin while still in the womb. In some cases the fetus in fetu will remain inside the host twin unknown until it begins to cause problems. In more common cases, the signs are visible from the outset and are often initially confused with cysts or cancers. In a recent case a 7 year old boy was discovered to be carrying his twin when his parents noticed that something was moving in his stomach


Proteus Syndrome
The Elephant Man (Joseph Merrick) is probably the most famous case of Proteus Syndrome. The disease causes excessive bone growth, excessive skin growth, and frequently comes with tumors. Only 200 cases have been confirmed worldwide since the disease was officially discovered in 1979. It is possible to have a minor form of this disease which can go undiagnosed. The case of the Elephant Man has been the sole reason that this disease is so widely known. Sufferers have normal brain function and intelligence.


Möbius Syndrome 
A rare disorder in which the facial muscles are paralyzed. In most cases the eyes are also unable to move from side to side. The disease prevents a sufferer from having any facial expressions, which can make them appear to be uninterested or “dull” – sometimes leading to people thinking they are rude. Sufferers have completely normal mental development. The causes are not fully understood and there is no treatment aside from addressing the symptoms (such as an inability to feed as a baby).


Hutchinson-Gilford Progeria syndrome (progeria)
will be familiar to people old enough to remember the television program That’s Incredible from the ’80s in which a young sufferer of the disorder appeared. The disease causes premature aging – so rapidly that a young child can look like a very old man (or, if I may be so callous as to point out the obvious, an alien – as in the photograph above). The disease is especially interesting for scientists as it may lend clues to the natural aging process in man. The disease is caused by a genetic mutation, and does not pass from parent to child. There is no known cure, and most children with the disease do not live beyond the age of thirteen – usually dying of stroke or heart attack (diseases usually associated with old age).


Cutaneous porphyria 
A disorder that causes blisters, excess hair, swelling, and necrosis of the skin. It can cause red colored teeth and fingernails, and after exposure to sun, urine can turn purple, pink, brown, or black. The disease is thought to be connected to the many werewolf and vampire legends of the past, where a sufferer (who would have lived apart from society) might have been confused for a monster. The disease is part of the more general group of disorders called porphyrias which cover a range of mental and physical disorders due to the overproduction of certain enzymes in the body. The disease gets its name from the Greek word “porphura” which means “purple pigment”.


Elephantiasis

First off, note the spelling – it is Elephant-iasis not Elephant-itis as many people wrongly think. Elephantiasis is a thickening of the skin (as opposed to proteus syndrome which is a thickening of the bones as well as the skin). Unfortunately, this is a disease that any one of us can get as it is caused by parasitic worms passed on through mosquito bites. It is, consequently, not uncommon in tropical regions and Africa. A slightly different form of the disease is caused through contact with certain types of soil. In some parts of Ethiopa, up to 6% of the population suffers from the disorder. It is one of the most common disabilities in the world. Efforts to eradicate the disease are well underway and it is hoped that it will be successfully relegated to the annals of history by 2020.


Fibrodysplasia Ossificans Progressiva

Fibrodysplasia Ossificans Progressiva (FOP for short) is a very rare disease that causes parts of the body (muscles, tendons, and ligaments) to turn to bone when they are damaged. This can often cause damaged joints to fuse together, preventing movement. Unfortunately surgical removal of the bone growths is ineffective as the body “heals” itself by recreating the removed bone. To make matters worse, the disease is so rare that it is often misdiagnosed as cancer, leading doctors to perform biopsies which can spark off worse growth of these bone-like lumps. The most famous case is Harry Eastlack whose body was so ossified by his death that he could only move his lips. His skeleton is now on display at the Mütter Museum. There is no cure.


Epidermodysplasia verruciformis

Lewandowsky-Lutz Dysplasia (also known as Epidermodysplasia verruciformis) is an extremely rare inheritable disorder in which warts form on the skin. It normally affects the hands and feet and while it can start in middle ages, it normally begins between the ages of one and twenty. There is no known effective treatment for the disease though surgery can be used to remove the warts. Unfortunately, after surgery the warts begin to return and it is estimated that a sufferer would need at least two surgeries per year to remove them each time they grow back. In 2007 a sufferer had surgery for the disease and thirteen pounds (5.8 kilos) of warts were removed. 95% of the warts were removed.


Diphallia

Diphallia (also known as Penile Duplication) is a condition in which a male is born with two penises. It is a rare disorder with only 1,000 cases recorded. Sufferers are also at a higher risk of spina bifida than men with one penis. A person with diphallia can urinate from one or both of his penises. In most cases, both penises are side by side and the same size, but occasionally one smaller penis will sit atop another larger one. One in 5.5 million men in the United States have two penises.

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Top 10 Bizarre Medical Treatments

Sweat Therapy
Sweat therapy is the combination of group counseling/psychotherapy with group sweating. Group sweating is social interaction while experiencing psychophysiological responses to heat exposure. Group sweating has strong cultural validity as it has existed throughout the world for thousands of years to promote well-being. Examples include the Finnish Sauna, the Russian Banya (sauna), the American Indian Sweat lodge Ceremony, the Islamic Hammam, the Japanese Mushi-Buro or Sentō, and the African Sifutu. Group sweating has been used for various physical and mental purposes for thousands of years. It has been asserted that the potential health benefits of regular participation in Native American sweat lodges are numerous, but that there is a scarcity of research about the practice.


Mud
We are all familiar with the use of clay in health resorts where people bathe in it to improve skin conditions, but what many people don’t know is that clay (or mud) is also used in internal medicines. It is sometimes used as a coating on pills but it is also consumed in larger doses for the treatment of bowel disorders. Even NASA uses clay treatments: “The effects of weightlessness on human body were studied by NASA back in the 1960s. Experiments demonstrated that weightlessness leads to a rapid bone depletion, so various remedies were sought to counter that. A number of pharmaceutical companies were asked to develop calcium supplements, but apparently none of them were as effective as clay. The special clay that was used in this case was Terramin, a reddish clay found in California. Dr. Benjamin Ershoff of the California Polytechnic Institute demonstrated that the consumption of clay counters the effects of weightlessness.”

Electrocution
Electroconvulsive therapy (ECT), also known as electroshock, is a well-established, albeit controversial, psychiatric treatment in which seizures are electrically induced in anesthetized patients for therapeutic effect. Today, ECT is most often used as a treatment for severe major depression which has not responded to other treatment, and is also used in the treatment of mania (often in bipolar disorder), catatonia and schizophrenia. It was first introduced in the 1930s and gained widespread use as a form of treatment in the 1940s and 1950s; today, an estimated 1 million people worldwide receive ECT every year, usually in a course of 6–12 treatments administered 2 or 3 times a week. Most, but not all, published reviews of the literature have concluded that ECT is effective in the treatment of depression.


Dousing
Dousing is the practice of making something or someone wet by throwing liquid over them, e.g., by pouring water, generally cold, over oneself. Cold water dousing is used to “shock” the body into a kind of fever. The body’s reaction is similar to the mammalian diving reflex or possibly temperature biofeedback. Several meditative and awareness techniques seem to share similar effects with elevated temperature, such as Tummo. Compare cold water dousing with ice swimming. The effects of dousing are usually more intense and longer lasting than just a cold shower. Ending a shower with cold water is an old naturopathic tradition. There are those who believe that this fever is helpful in killing harmful bacteria and leaving the hardier beneficial bacteria in the body.


Urine
The term urine therapy (also urotherapy, urinotherapy or uropathy) refers to various applications of human urine for medicinal or cosmetic purposes, including drinking of one’s own urine and massaging one’s skin with one’s own urine. A practitioner of urine therapy is sometimes called a psychopath. Just kidding, they are actually called uropaths. There is no scientific evidence of a therapeutic use for urine. Urinating on jellyfish stings is a common folk remedy, but has no beneficial effect and may be counterproductive, as it can activate nematocysts remaining at the site of the sting. Urine does contain substances that are beneficial, such as Vitamin C; however, these substances have been excreted because they could not be used or because they were present in excess, so re-taking them will just result in re-excretion. The most obvious physiological effect of drinking urine, at least when it is taken on an empty stomach, is bowel movement (sometimes in the form of diarrhea) due to the laxative action of hypertonic solution of urea.


Bloodletting
Bloodletting is the withdrawal of often considerable quantities of blood from a patient to cure or prevent illness and disease. It was the most common medical practice performed by doctors from antiquity up to the late 19th century, a time span of almost 2,000 years. In the overwhelming majority of cases, the historical use of bloodletting was harmful to patients. But, bloodletting has not died a death – it is still one of the most effective treatments of excess iron in the bloodstream and for treatment of excess red blood cells which can occur in diseases such as porphyria. In the old method, the patient was cut and a suction cup was placed over the wound to draw out blood. In modern times syringes are used.


Leech
Medicinal leeches are now making a comeback in microsurgery. They provide an effective means to reduce blood coagulation, relieve venous pressure from pooling blood, and in reconstructive surgery to stimulate circulation in reattachment operations for organs with critical blood flow, such as eye lids, fingers, and ears. The therapeutic effect is not from the blood taken in the meal, but from the continued and steady bleeding from the wound left after the leech has detached. The most common complication from leech treatment is prolonged bleeding, which can easily be treated, although allergic reactions and bacterial infections may also occur. Devices called “mechanical leeches” have been developed which dispense heparin and perform the same function as medicinal leeches, but they are not yet commercially available.


Helminths
Helminthic therapy, a type of Immunotherapy, is the treatment of autoimmune diseases and immune disorders by means of deliberate infestation with parasitic worms (helminths) or their eggs. This is such a cure-all that it is also occasionally used in the treatment of hay fever and asthma. Depending on the particular autoimmune disease in question, infection with helminths can result in remission of symptoms in as high as approximately 70% of patients. The worms are administered via oral doses which are taken repeatedly over a course of weeks and can result in some fairly severe side-effects. Some patients can receive up to eight doses of 2500 worm eggs over the course of their treatment.

Fecal bacteriotherapy
Used in the treatment of certain inflammatory bowel disorders such as ulcerative colitis. The treatment comes in form of a series of enemas given to the patient over a five day period. In order to create the liquid used in the enema, a “poop donor” is needed. In other words, a sample of poop is taken from a healthy person (usually a relative of the patient) and turned into a liquid for anal insertion. The idea is that the healthy bacteria from the poop provider will grow in the sick person and heal them. What is perhaps even more revolting than an enema of someone else’s poop, is the fact that the liquid can also be delivered via a tube in the nose.

Smoking?

For centuries doctors prescribed smoking for a variety of ills and while this does still happen (though the doctor’s generally don’t want it publicized) the numbers of doctors who do this has become extremely small. Research with regard to neurological diseases, evidence suggests that the risk of developing Parkinson’s disease or Alzheimer’s disease might be 50% lower in smokers, compared to non-smokers. Nicotine has also been found to improve ADHD symptoms and appears to have effects in the brain that are similar to those of stimulants. Although such findings should certainly not encourage anyone to smoke, some studies are focusing on benefits of nicotine therapy in adults with ADHD. Recent studies suggest that smokers require less frequent repeated revascularization after percutaneous coronary intervention (PCI). Risk of ulcerative colitis has been frequently shown to be reduced by smokers on a dose-dependent basis; the effect is eliminated if the individual stops smoking.

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Top 10 Bizarre Surgical Procedures

WARNING: This list contains images of surgery. This list does not include surgical procedures that are now obsolete or obsolescent. The list also excludes cosmetic surgical procedures. Viewer discretion is advised

1. Hemispherectomy
Believe it or not, this surgical procedure involves removing or disabling an entire half of the brain. This procedure is used to treat a variety of seizure disorders where the source of the epilepsy is localized to a broad area of a single hemisphere of the brain. It is reserved for cases which can’t be managed with medication alone. The first time this surgery was performed on a human was in 1923 by Walter Dandy. All of the patients who receive this treatment suffer from partial or full paralysis on the side of the body opposite to the side of the brain removed. Most patients who have undergone this procedure will have neurons from the remaining hemisphere take over the tasks from the lost hemisphere by making new neural connections.


2. Hemicorporectomy
Also known as translumbar amputation, this surgery removes the lower half of the body from above the pelvis. The result is that the sexual organs, anus, rectum, legs, pelvis bones, and urinary system are removed. It is a severely mutilating procedure recommended only as a last resort for patients with severe and potentially fatal illnesses such as osteomyelitis, tumors, severe traumas and intractable decubiti in, or around, the pelvis. This surgery has only been performed in a small number of cases. The surgical procedure is often done in two stages; however it is possible to conduct the surgery in one stage. The first stage is the discontinuation of the waste functions in colostomy (rectum) and ileal conduit (bladder). The second stage will be the actual amputation.


3. Bilateral Cingulotomy
This is a brain surgery used in pain treatment for severe cases of cancer. It involves disabling the cingulate gyrus, a small section of brain that connects the limbic region of the brain with the frontal lobes. It has also found use in psychosurgery (surgery for mental disorders) where it is very controversial. As a psychosurgical treatment it has almost totally replaced lobotomy as a procedure. Its use for alleviation of pain in cancer patients is reasonably well-documented and well-supported, but its use in treating people with depression is not.


4. Endoscopic Thoracic Sympathectomy
ETS is a surgery in which oprtions of the sympathetic nerve trunk are dissected. By cutting these nerve sections, the surgeon is able to treat severe cases of hyperhydrosis (excess sweating). What makes this bizarre is that a side-effect of this treatment is that the person no longer blushes. For that reason it has also become a cosmetic procedure for people who blush excessively. If only one side of the body is treated, a person who has undergone this surgery will have half of their body blushing while the other half remains in its natural state. For this reason the treatment is always performed on both sides of the body.


5. Vaginectomy
In a Vaginectomy, part or all of the vagina is surgically removed. This is normally used as a treatment for various forms of cancer but it also occurs in some sexual reassignment surgeries. It is normal for a surgeon to reconstruct the vagina after this surgery by using other parts of the patient’s body.



6. Lobotomy
Lobotomy is a very controversial medical treatment in which the frontal lobes of the brain are destroyed. It consists of cutting the connections to and from, or simply destroying, the prefrontal cortex. While it is seldom performed nowadays, it does still occur. Lobotomies have been used in the past to treat a wide range of mental illnesses including schizophrenia. This procedure often results in major personality changes or even mental retardation.




7. Penectomy
A Penectomy is the total removal of all, or parts, of the penis. It is normally used as a treatment for cancer, but it has sometimes had to be performed after a botched circumcision. Some men have undergone penectomies as a voluntary body modification, but professional opinion is divided as to whether or not the desire for penile amputation is a pathology, thus including it as part of a body dysmorphic disorder.




8. Circumcision
Circumcision is a very common procedure performed for religious reasons (amongst Jews and Muslims) and by many doctors who claim health benefits. Much controversy surrounds this procedure (in which the foreskin of the penis is surgically removed) as it is a non-essential surgery normally performed on a child that has no say in whether it is performed or not. Some adult men develop psychological issues as a result of circumcision, which leads us to the next bizarre surgery: foreskin restoration.

9. Foreskin Restoration
Foreskin restoration is the process of expanding the residual skin on the penis, via surgical or non-surgical methods, to create the appearance of a natural foreskin (prepuce) covering the glans penis. Foreskin restoration techniques are most commonly undertaken by men who have been circumcised or who have sustained an injury, but are also used by uncircumcised men who desire a longer foreskin and by men who have phimosis. Some men cite a desire to regain a sense of control over their sexual organs and regaining lost self esteem. European Jews, along with men circumcised for medical reasons, sought out underground foreskin restoration operations during World War II as a method to escape Nazi persecution.


10. Lindbergh Operation
The Lindbergh Operation is included here as a special mention. It was the first surgery ever performed entirely with robots being guided by doctors through telecommunications. The surgery occurred in France and was controlled by French doctors in New York. The operation was performed successfully on September 7th, 2001 by Professor Jacques Marescaux and his team from the IRCAD (Institute for Research into Cancer of the Digestive System). This was the first time in medical history that a technical solution proved capable of reducing the time delay inherent to long distance transmissions sufficiently to make this type of procedure possible. The 45-minute procedure consisted in removing the gallbladder of a patient in surgical ward A in Strasbourg Civil Hospital, in Eastern France. From New York, the surgeon controlled the arms of the ZEUS™ Robotic Surgical System, designed by Computer Motion, to operate on the patient. The link between the robotic system and the surgeon was provided by a high-speed fiberoptic service deployed thanks to the combined efforts of several France Telecom group entities.

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