Pages

Monday, July 28, 2014

Cutaneous horns


Imagine a hard, yellowish or brownish conical protrusion, uncannily similar to the horn of an animal, slowly developing on your face, arm or leg, and gradually increasing in size over a period of months or years. How bizarre is that?! This odd condition is known as cutaneous horn (cornu cutaneum in Latin). It occurs most commonly in patients 50 years or older. Certain medieval texts describe the presence of a cutaneous horn as the mark of a witch. The earliest documented case was that of an elderly Welsh woman in London who was displayed commercially as an anomaly of nature in 1588. 

The horns are growths composed of keratin, the same tough structural protein that is found in the outermost layer of the epidermis, as well as in your hair and fingernails. They can be conical, pointed or corrugated, straight or curving as a ram's horn. In the majority of cases, the lesion at the bottom of the keratin mound is benign, but in certain cases it may be a malignant tumor, usually a squamous cell carcinoma, or a premalignant condition such as actinic keratosis - thickened, rough, scaly patches of sun-damaged skin. 

The most frequent localization of cutaneous horns is on the face or hands (areas with a large degree of sun exposure). Approximately 30% reported cases are located on the upper face and scalp. Madame Dimanche, an elderly French widow living in Paris in the early 19th century, walked around with a horn on her forehead for six years. It reached a whopping length of 24.9 cm (9.8") before it was successfully removed by a surgeon. However, cutaneous horns may also develop in unexpected locations, for example on the penis!

Today, the treatment of choice for cutaneous horns is pretty much the same as it was in the 19th century - surgical excision. Afterwards, histopathologic evaluation of the specimen should be performed to rule out underlying malignancy and to guide potential further therapy.

Below is a photo of a cutaneous horn 5 cm in length which had been growing on the lower eyelid of a 78-year-old farmer for three years. After surgeons removed the growth, the lesion at its base turned out to be a well-differentiated squamous cell carcinoma. The patient had to undergo two additional operations to make sure the entire tumor had been excised, but this particular story ended well - he had no metastases and suffered no relapse.




(Image from: Sadeghi et al. 2012)


And here is another example of a cutaneous horn. This little bastard was growing on the temple of a 76-year-old Korean woman. It was surrounded by a rather large patch of reddened skin and had a a base diameter of 0.7 cm and a height of 2.7 cm. The lesion at its base turned out to be a keratoacanthoma, a relatively common epithelial tumor. It was totally excised and did not recur.





(Image from: Yang et al. 2011)


Literature:

Fernandes NF, Sinha S, Lambert WC, Schwartz RA. (2009) Cutaneous horn: a potentially malignant entity. Acta Dermatovenerol Alp Pannonica Adriat. 18(4):189–93.

Sadeghi E, Johari HG, Deylami HN. (2012) Eyelid cutaneous horn. J Cutan Aesthet Surg. 2012 Apr-Jun; 5(2):153–154.  

Yang JH, Kim DH, Lee JS, Cho MK, Lee SH, Lee SY, Hyun DC. (2011) A Case of Cutaneous Horn Originating from Keratoacanthoma. Ann Dermatol. 23(1):89–91.



Wednesday, July 9, 2014

Ever heard about lactobezoars?


A bezoar is a conglomeration of partially digested or undigested foreign material in the digestive tract. These masses most often form in the stomach, but are occasionally found in the small intestine and the colon, or sometimes even in the rectum. Depending on their location, bezoars may prove dangerous or not. They occasionally cause no symptoms at all, but their presence can also lead to severe complications, such as intestinal obstruction or even perforation. 

Bezoars are classified into several main types, named according to the materials from which they are composed. Phytobezoars consist of plant material, whereas trichobezoars (I mentioned them a while ago in the post on trichotillomania) are masses of ingested hair. But would you believe that curdled milk can also turn into a life-threatening bezoar?

A lactobezoar is a pathological conglomeration of half-digested milk and mucus - or, to put it simply, a large clot of milk curds - which forms in the stomach or small intestine of milk-fed infants, often causing obstruction. It's a fairly rare disorder that usually strikes prematurely born infants with a low birthweight, who are being given concentrated formula feeds containing lots of calories and protein. Basically, the immature digestive tract doesn't always handle these calorie-dense and high-protein feeds well. However, the incidence of lactobezoars isn't limited to preterm infants, and may be more frequent than reports suggest.

Clinically, the symptoms of a lactobezoar can include acute abdomen (sudden, severe abdominal pain), vomiting, diarrhea and/or a palpable mass in the abdomen. This condition normally occurs during the first few weeks of life; the peak incidence is between 4 and 10 days of age. Treatment involves ceasing oral feedings, administration of intravenous fluids and gastric lavage. Surgery may be necessary.

The following photo shows a lactobezoar that caused an obstruction of the terminal ileum (the final section of the small intestine) in a male infant with an extremely low birth weight, born at 26+2 weeks of gestation and weighing only 750 g at birth. The child had been receiving fortified human milk, preterm formula and supplements, and developed a lactobezoar at 12 weeks of life, with worrying abdominal symptoms that necessitated an exploratory laparotomy.




Lactobezoar blocking the ileum in a prematurely born infant. Image from: Jain et al. 2009


Surgery was performed to resolve the blockage and remove a section of necrotic bowel. The infant survived the operation and had no postoperative complications. However, he died on day 142 of life due to respiratory failure from severe chronic lung disease.


Literature:

Heinz-Erian P, Gassner I, Klein-Franke A, Jud V, Trawoeger R, Niederwanger C, Mueller T, Meister B, Scholl-Buergi S. (2012) Gastric lactobezoar - a rare disorder? Orphanet J Rare Dis. 7: 3.


Jain A, Godambe SV, Clarke S, Chow PC (2009) Unusually late presentation of lactobezoar leading to necrotising enterocolitis in an extremely low birthweight infant. BMJ Case Rep. 2009: bcr03.2009.1708.



Thursday, June 26, 2014

Some facts about gallstones


Gallstone disease, or cholelithiasis, is often thought to be a major affliction in modern society. However, gallstones must have been known to humans for many years, since they have been found in the gallbladders of Egyptian mummies dating back to 1000 BC! Today, this disease is a worldwide medical problem, although its prevalence varies depending on the geographical region.

How do gallstones form, and why? The liver secretes bile - a greenish-brown, bitter fluid that is discharged into the digestive tract (into the duodenum, to be precise) and helps emulsify the fats in food, so that they can be digested more easily. Bile is composed of water (92%), bile salts (6%), the pigment bilirubin, tiny amounts of cholesterol and mineral salts. When an imbalance in the chemical constituents of bile causes one or more of the components to precipitate, hardened crystalline deposits form. They can be as small as a grain of sand or as large as a golf ball. (Ouch!)

Gallstones are composed mainly of cholesterol, bilirubin, and calcium salts, with smaller amounts of protein and other materials. There are three types of gallstones:
(1) Pure cholesterol stones, which contain at least 90% cholesterol.
(2) Pigment stones, either brown or black, which contain at least 90% bilirubin.
(3) Mixed composition stones, which contain varying proportions of cholesterol, bilirubin and other substances such as calcium carbonate, calcium phosphate and calcium palmitate.

In Western societies and in Pakistan more than 70% of gallstones are composed primarily of cholesterol, either pure or mixed with pigment. Cholesterol gallstones form when the cholesterol concentration in bile exceeds the ability of bile to hold it in solution, so that crystals form and grow as stones.

Below, you can admire some beautiful specimens of gallstones taken out from the gallbladders of various unfortunate people.



Specimen A) shows a group of gallstones retrieved from a single patient. They all have a similar size and look pretty much the same. B) A cross section of gallstone (GS 1) and C) a cross section of gallstone (GS 4) reveal a heterogeneous appearance throughout a single gallstone. D) 4 gallstones from 4 different patients have different sizes, shapes and colors. (Image from: Marshall et al. 2014)


Gallstones are becoming increasingly common. They are seen in all age groups, but the incidence increases with age; about a quarter of women over 60 years will develop them. The good news is that in most cases gallstones do not cause symptoms. Only 10% and 20% will eventually become symptomatic within 5 years and 20 years of diagnosis. However, the bad news is that cholelithiasis can have severe consequences: acute cholecystitis (inflammation of the gallbladder) or obstruction of the bile ducts, which can lead to ascending cholangitis (infection of the bile duct) or acute pancreatitis, both of which are life-threatening medical conditions.

And how can gallstones be treated?

Cholesterol gallstones can sometimes be dissolved by oral ursodeoxycholic acid, but it may be necessary for the patient to take this medication for up to two years. Gallstones may recur, however, once the drug is stopped. Obstruction of the common bile duct with gallstones can sometimes be relieved by an endoscopic surgical intervention. Gallstones can also be broken up using a procedure called extracorporeal shock wave lithotripsy (often simply called "lithotripsy"): briefly, ultrasonic shock waves are directed onto the stones to break them into tiny pieces. These bits are then passed safely in the feces. However, this form of treatment is suitable only when there is a small number of gallstones. 

Surgical removal of the gallbladder, known as a cholecystectomy, has a 99% chance of eliminating the recurrence of cholelithiasis. Surgery is only recommended in symptomatic patients. There are two surgical options for cholecystectomy: open surgery via an abdominal incision below the lower right ribs or a laparoscopic cholecystectomy, performed via small puncture holes for a camera and instruments. The laparoscopic procedure is much less invasive - patients are often released home on the same day, or after a one-night hospital stay, and can resume normal activity after a week or so, whereas the open procedure typically requires 3-5 days of hospitalization and a much longer recovery period afterwards.

Had enough? I'll serve you one more juicy fact about gallstones: recent studies show that in chronic carriers of the disease-causing bacterium Salmonella typhi, which causes typhoid fever, the microorganism grows in biofilms on the surface of - yes, you've guessed in - gallstones in the gallbladder.
 


Literature:

Marshall JM, Flechtner AD, La Perle KM, Gunn JS. (2014) Visualization of Extracellular Matrix Components within Sectioned Salmonella Biofilms on the Surface of Human Gallstones. PLoS One. 9(2): e89243. Published online 2014 February 14.

Njeze GE. (2013) Gallstones. Niger J Surg. 19(2): 49–55.

Wikipedia: Gallstone

Tuesday, June 24, 2014

Rampant caries - a really disgusting mouth


We all know that sugar destroys teeth, right? Well, not actually sugar itself, but the acids produced from sugar by bacteria that live in our mouths. Frequent exposure to an acidic environment causes the mineral components of teeth to break down, so that cavities form. This condition is known as dental caries - or, colloquially, as tooth decay. But how bad can tooth decay actually get, when it's really, REALLY bad? 


Rampant caries is a condition where a combination of three factors: high sugar consumption (especially in the form of fizzy drinks or chewy sweets), bad oral hygiene and scant saliva production, popularly known as "dry mouth", allows acid-producing bacteria to proliferate wildly in the mouth and destroy teeth in record time. The result - severe decay on multiple tooth surfaces, often culminating in a mouthful of blackened, eroded stumps. Rampant caries is especially prevalent in folks addicted to metamphetamine, in whom it is known as "meth mouth". 


Oral hygiene in drug addicts is, as a rule, very low. One study, recently performed in Spain, showed that a whopping 64.1% (n=41) of the surveyed drug users NEVER brushed their teeth; 17.2% (n=11) brushed once a day and 18.8% (n=12) twice or more.The respondents in this study were mostly addicted to heroin (96.8%) and cocaine (90.6%); the overwhelming majority also smoked tobacco. 81.3% of those surveyed suffered from periodontitis, and a full half of the respondents had rampant caries.

Below is a yucky example of rampant caries in a drug addict. Most of this person's teeth are completely gone, leaving bare gums. The few that still survive should probably be extracted as soon as possible. Ewww.




A drug addict's nearly toothless mouth. (Image from: Mateos-Moreno et al. 2013)


The mouth contains a wide variety of oral bacteria, but only a few specific species of bacteria are believed to cause dental caries: Streptococcus mutans and Lactobacilli among them. These organisms can produce high levels of lactic acid following fermentation of dietary sugars, and are resistant to the adverse effects of low pH. The frequency of tooth exposure to acid affects the speed at which cavities develop; thus, frequently sipping sweet drinks is an especially pernicious habit. "Baby bottle caries," "baby bottle tooth decay," or "bottle rot" is a pattern of decay found in young children with their baby teeth. This type of decay is usually a result of allowing children to fall asleep with sweetened liquids in their bottles or feeding children sweetened liquids multiple times during the day.




Rampant caries in a child's baby teeth. HIV infection was probably a predisposing factor in this patient's case. (Image from: Ponnam et al. 2012)


The bottom line? It's better to eat sweets only once or twice a day, if you have to eat them at all, and brush teeth directly afterwards. As for fizzy drinks and other sugar-sweetened beverages, your teeth will definitely be thankful if you stop drinking them.



Literature:

Mateos-Moreno MV, del-Río-Highsmith J, Riobóo-García R, Solá- Ruiz MF, Celemín-Viñuela A. (2013) Dental profile of a community of recovering drug addicts: Biomedical aspects. Retrospective cohort study. Med Oral Patol Oral Cir Bucal. 18(4): e671–e679. 


Wikipedia: Dental caries

Thursday, June 19, 2014

Trichotillomania


Some people have a penchant for nervously or distractedly playing with their hair, constantly touching it or twisting it in their fingers. And some folks take this habit a step further, to the point where it becomes a real problem.

Trichotillomania is a medical condition where sufferers compulsively pluck out their hair. The name comes from the Greek words: trich (hair), till (en) (to pull) and mania (an abnormal love for a specific object, place, or action). This behavior often leads to hair thinning and even bald patches. Frequently, repetitive rituals are performed on the hairs after they have been pulled out. These rituals can include biting on the hair, twisting it, chewing it, playing with it or even swallowing it. Plucking hair out and then eating it is known as trichophagia; over time, this condition can lead to the formation of large trichobezoars (dark stinking masses composed of matted hair and food residue) in the stomach and small intestine. If untreated, such a bezoar can block the digestive tract, requiring emergency surgery.

While far more women and adolescent girls seek treatment for their trichotillomania, survey studies suggest chronic hair pulling also occurs in males. Diagnosis may be complicated by patient and family denial or ignorance of the hair pulling. Accurate scalp examination and scalp biopsies may be needed to obtain a definite diagnosis. Treatment is difficult: the tricyclic antidepressant clomipramine is the most promising agent, but many patients find it difficult to tolerate at adequate dosages, and treatment response may not be maintained over the long term. More benign medications have not demonstrated efficacy in controlled studies. Specialized psychotherapy, known as habit reversal training, is highly recommended; however, skilled therapists can prove difficult to find.



 Hair loss caused by trichotillomania in a 24-year-old woman. (Image from: Varyani et al. 2012)



An article published in the International Journal of Trichology in 2013 describes the case of a 9-year-old Hindu girl from a middle-class family who compulsively pulled out her hair and swallowed it. This behavior persisted for two years. She was treated by a pediatrician and dermatologist for alopecia areata because the mother did not wish to disclose the true nature of the problem, fearing the stigma attached to mental illness. Finally, the girl developed severe abdominal pain and a large trichobezoar had to be surgically removed from her digestive tract. After recovering, she was referred to a psychiatric department and diagnosed with trichotillomania. A treatment regime consisting of medications and psychotherapy was initiated, and the condition gradually resolved. 1 year later, the child was still symptom-free.




Hair loss caused by trichotillomania in a 9-year-old Hindu girl. (Image from: Sachdeva 2013)


Weirdly enough, a similar condition to trichotillomania also occurs in domestic cats. Cats groom themselves by licking their fur, and when stressed or bored (e.g. forced to stay alone in a small apartment all day), they can obsessively over-groom to the point where a part of their body - often the forepaws or belly - becomes completely bald. Also, cats develop trichobezoars - colloquially known as "hairballs". Hairballs, which look rather like baby dreadlocks, can cause periodic vomiting, which is quite a nuisance for the cat's owner. Some cat foods are specially formulated to help the cat naturally pass the ingested hair with stool; malt paste and giving the cat access to fresh grass are good preventive measures too. (Yes, you've guessed it, I own a cat!)

Literature:

Walsh KH, McDougle CJ. (2001) Trichotillomania. Presentation, etiology, diagnosis and therapy. Am J Clin Dermatol. 2(5):327-33.

Varyani N, Garg S, Gupta G, Singh S, Tripathi K. (2012) Trichotillomania and dermatitis artefacta: A rare coexistence. Case Rep Psychiatry. 2012: 674136.Published online Feb 9, 2012. 

Sachdeva A. (2013) Managing a case of trichotillomania with trichobezoar. Int J Trichology. 5(4): 228–230.

Saturday, June 14, 2014

Blast injury due to a pencil battery (Warning: GRAPHIC PHOTOS!)


Okay... do you know what can happen if you short-circuit a pencil battery while holding it in your mouth?

A 10-year-old boy from India found this out the hard way, as described in an article by Kumar et al. (2013)... The battery can EXPLODE and pretty much destroy your mouth and face.

This unfortunate kid tried to short-circuit three low-voltage batteries with a wire while holding them in his mouth. He was twisting the wire with his teeth when one of the batteries exploded. The gory result can be seen below. Yes, I know this is a drastic photo. Pretty much the entire lower half of the face is gone, leaving a gaping bloody ruin.





The explanation for this accident is that low-voltage batteries contain a high amount of energy. The low internal resistance of such a battery, when short-circuited, permits a heavy flow of current. The short circuit instantaneously vaporizes a miniscule portion of the wire at approximately 2000 K, resulting in a blast in a closed cavity causing sudden rise of intraoral pressure which can lead to extensive tissue damage in and around the mouth.

Even drastic facial injuries are frequently not life-threatening unless associated with other injuries of the skull and/or airway, causing brain damage or breathing trouble. This particular patient was immediately rushed to the hospital, suffering from shock, severe bleeding and breathlessness. At assessment, he had a pulse of 120/min, respiratory rate of 36/min, and blood pressure of 86/60 mm Hg. Because lack of support to the tongue was causing respiratory distress, an emergency tracheostomy was done under local anesthesia, after which the wounds were cleaned thoroughly and dressed. A feeding tube was also inserted. After stabilization and all routine blood investigations, a 3D CT scan of the head was performed, showing the full extent of the damage:




The patient had lost the arch of the upper jaw along with all the upper teeth. His lower jaw was fractured and the lower segment, carrying all incisors and canines, was also lost. Soft tissue injuries included loss of the upper and lower lips, skin of the chin region, partial loss of both alae of the nose, lacerations all around the mouth and abrasions on the chest and right arm and forearm. Debridement of nonviable tissues and daily dressing was done for a week, during which the boy was given three blood transfusions.

After stabilization, the patient was taken up for surgery under general anesthesia. A cap splint with zygomatic suspension was done for the upper jaw, the residual mandibular segments were wired together and covered with a right-sided deltopectoral flap. A split skin graft was used to cover the donor site. Here is an early postoperative photo:





While the flap coverage was successful, more surgeries will be needed to debulk the flap, reconstruct the upper lip and enable the patient to eat normally instead of being fed through a tube. In the following postoperative picture, the flap has been detached but the tracheostomy is still there. Obviously, the child has been disfigured for life.



All images from: Kumar et al. (2013)

 
So... please, folks, don't fool around with batteries, recharge them, toss them in a fire or let your kids play with them. The familiar warning that an improperly handled battery "may explode or leak" is not to be taken lightly!



Source:

Kumar V, Singh AK, Kumar P, Shenoy YR, Verma AK, Borole AJ, Prasad V. (2013) Blast injury face: An exemplified review of management. Natl J Maxillofac Surg. 4(1): 33–39.



Monday, June 2, 2014

How hemorrhoids were treated in historical times



 A pretty disgusting case of grade 4 hemorrhoids. Ugh. (Image from: Wikipedia)


Hemorrhoids are vascular structures in the anal canal which help maintain stool continence. As a person gets older, they may become swollen, inflamed and bleed or protrude from the anus. It is estimated that about half the population has hemorrhoid-related problems by the age of 50 years; in the USA, around 58% of people aged over 40 years suffer from this condition. There are two types of hemorrhoids: external and internal.
 
Hemorrhoids can occur at any age, and they affect both men and women. They can either be asymptomatic or cause itching, pain, bleeding (fresh red blood visible on the surface of stool and/or on toilet paper after defecation) and fecal soiling. Inflamed, painful hemorrhoids, also known as piles, can be treated conservatively (fluids and a fiber-rich diet to avoid constipation, medicated creams and suppositories to relieve pain, swelling and itching). Patients are also advised to avoid straining during defecation and reading on the toilet. If these dietary and lifestyle modifications have no effect, the patient must consider a more radical method of treatment, e.g. phenol oil injection or a procedure called rubber band ligation (which involves, basically, tying a rubber band around a hemorrhoid until it becomes necrotic and sloughs off). Both procedures are available on an outpatient basis. Studies show that band ligation is the most effective outpatient procedure for hemorrhoids, providing a cure in 79% of patients with first to third degree hemorrhoids. If this method fails, however, surgery is advised.

This unpleasant condition has affected humanity since ancient times. The first recorded treatment for hemorrhoids was described in an Egyptian papyrus dated 1700 BC: “… Thou shouldest give a recipe, an ointment of great protection; acacia leaves, ground, triturated and cooked together. Smear a strip of fine linen therewith and place in the anus, that he recovers immediately."

In 460 BC, Hippocrates described a method for treating hemorrhoids which sounds similar to today's rubber band ligation procedure: “And hemorrhoids in like manner you may treat by transfixing them with a needle and tying them with very thick and woolen thread, for application [...] and always leave one behind; and when the patient recovers, let him be put on a course of Hellebore.” Hellebore, a poisonous plant, was used in ancient Greece as a purgative.

A Roman physician named Celcus (25 BC - AD 14) described the ligation and excision surgeries, as well as possible complications. The famous Greek physician Galen (AD 129 - ca. AD 200) also promoted severing the connection of the arteries to veins in order to reduce pain and avoid spreading gangrene.

The Indian Susruta Samhita, an ancient Sanskrit text dated between the fourth and fifth century AD, described treatment procedures comparable to those in the Hippocratic treatise, but with advancement in surgical procedures and emphasis on wound cleanliness.

In the Middle Ages in Europe, a popular treatment for hemorrhoids was cauterization with a hot iron and/or making an incision with a sharp knife. Sufferers could also pray to St. Fiacre, the patron saint of this unpleasant condition, hoping for a cure.



A 23th-century illustration depicting a surgeon operating an unfortunate patient with hemorrhoids. The procedure must have been excruciatingly painful. Note the blood running down into a dish.


During the 19th century, hemorrhoids were often treated by anal stretching. In the USA, carbolic acid was first used for injecting hemorrhoids in 1871. In 1888, Fredrick Salmon, the founder of St. Marks’ Hospital, expanded the surgical procedure for hemorrhoids into a combination of excision and ligation. The diathermy hemorrhoidectomy by Alexander Williams, rubber band ligation by Barron, and the stapled hemorrhoidectomy by Longo were three additional developments in the late 20th century.

 If you want to avoid hemorrhoids, try not to strain while attempting to defecate, avoid sitting on the toilet for long periods, and maintain regular bowel movements through eating a high-fiber diet, drinking plenty of fluid and getting sufficient exercise.



Literature:
Agbo SP. (2011) Surgical Management of Hemorrhoids. J Surg Tech Case Rep. 3(2): 68–75.

Nisar PJ, Scholefield JH. (2003) Managing haemorrhoids. BMJ. 327(7419): 847–851. 

Wikipedia: Hemorrhoid