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Thursday, August 21, 2014

Scleroma - a nasty infection of the respiratory tract


Nasal inflammation, thickened mucosa, ulcers and polyp-like masses that form within the nose and throat, ultimately leading to airway obstruction, scarring and deformity - chances are you've never heard about this ugly malady before.

Scleroma is a chronic disease that affects the upper respiratory tract. It used to be endemic in eastern Europe; now it occurs mostly in tropical regions, such as North Africa, India, South America and Central America. The causative organism is a Gram-negative bacterium, Klebsiella rhinoscleromatis.


The bacteria that cause this highly unpleasant condition can be transmitted by directly inhaling infected droplets or by contact with contaminated material. Undernourishment and a lack of hygiene make it easier for the infection to take hold. It usually begins at the nose and may spread to the pharynx, larynx, trachea or - rarely - even the bronchi. The sinuses and soft palate may be involved, too. The nose is affected in 95-100% of cases (scleroma which affects the nose and no other parts of the respiratory tract is called rhinoscleroma). For some reason, nasal and pharyngeal scleroma is more common in males (male-to-female ratio, 2:1), whereas laryngotracheal scleroma is more common in females (female-to-male ratio, 4:1). 

Scleroma tends to progress slowly over many years, and is characterised by periods of remission and relapse. Initially, patients show symptoms of rhinitis, which may include an odorous, purulent discharge, crusting and various degrees of nasal obstruction. This early stage of the disease is known as the catarrhal stage. As the infection progresses to the granulomatous stage, well-defined masses of granulation tissue - rubbery polyps - typically develop in the affected regions. These masses can actually be mistaken for a nasal tumor. In this stage of the disease, epistaxis (bleeding from the nose) is common, and patients often lose their sense of smell (anosmia). Destruction of the nasal cartilage may occur. Frequently, the nose becomes significantly deformed; in some cases, the nasal septum is completely destroyed. Advanced scleroma causes scarring and fibrosis of the affected tissues. Altogether, the symptoms of this disease bear some similarity to leprosy, although the causative organism is completely different.

Scleroma is difficult to cure and prone to recur. Currently, the recommended treatment consists of a combination of surgical debridement and long-term antibiotic therapy. However, in tropical regions where this disease is endemic, medical care often isn't easily accessible or affordable for the sufferers. In non-endemic regions such as the United States, scleroma used to be rare, but cases occur more frequently now, due to increased migration, and aren't always diagnosed swiftly enough.

Not nice, is it? And here are some pictures:



Pharyngeal scleroma. A CT scan of the oropharynx shows narrowing of the oropharyngeal airway with abscesses in both tonsillar regions and V-shaped calcification along the soft palate. (Image from: Razek 2012)




A 45-year-old lady with septal rhinoscleroma, presenting as a large swelling on one side of the nose. (Image from: Shoeib 2010)


An article by Shoeib (2010) describes the case of a female patient aged 45 years who presented with a large swelling on her nose (see picture above) which had been present since 1 year. There was past history of nasal trauma. The nose was swollen and obviously asymmetric, with unilateral obstruction, but no other symptoms such as bleeding or crusting. Internal nasal examination showed a septal swelling protruding to the right nasal cavity, with mucosal hypertrophy. The mass was surgically excised, and the resulting defect was reconstructed with cartilage grafts. Histopathological examination of the specimen revealed the cause - rhinoscleroma. Postoperatively, the patient received antibiotic treatment in the form of ciprofloxacin. The functional and aesthetic outcome was good, with no recurrence after 6 months.

Gupta and Agrawal (1998) have described an unusual case where scleroma manifested as a solitary nodule on the cheek of a 66-year-old man from an urban slum in India. It was treated by surgical excision and antibiotics.


Literature:
Chan TV, Spiegel JH. (2007) Klebsiella rhinoscleromatis of the membranous nasal septum. J Laryngol Otol. 121(10): 9981002. Epub 2007 Mar 15.

Gupta A, Agrawal SR (1998) An unusual case of solitary scleroma of cheek. Indian J Otolaryngol Head Neck Surg. 50(3): 296297.

Razek A. (2012) Imaging of scleroma in the head and neck. Br J Radiol. 85(1020): 1551–1555.

Shoeib MA. Septal rhinoscleroma. Indian J Plast Surg. 43(2): 219–221. 



Sunday, August 3, 2014

Premonition of death (a case from 1958)


Rumor has it that some patients suffering from chronic diseases are able to predict the time of their deaths. The following slightly spooky letter was published by the British Medical Journal in 1958.

"SIR, -

On paying my usual routine visit one Tuesday afternoon to a male patient of mine dying of cancer of the lung, the patient took hold of my hands, thanked me for all my attention, and then proceeded to tell me that I would not need to call and see him after Thursday, as he was going to die at 2.30 that afternoon. Needless to say, I comforted him the best way I could, told him to exclude such thoughts from his mind, and promised to look in again.

On late Thursday afternoon I received a message from a member of his family that their father had passed away peacefully. A few days later two daughters of the deceased came to consult me, and in the course of our conversation told me that the day before their father died he had summoned all the members of the family together and repeated to them what he had told me - namely, that he would die the following day at 2.30.  On the predicted day, all the members of his family were with him in his bedroom except his wife. As 2.30 was approaching he urgently called for his wife to come in, saying "Hurry, hurry, before it is too late." She came running into the room, and on the stroke of 2.30 according to the two daughters, "He sighed, raised both hands behind his head, smiled, and passed away." I wonder if any of my colleagues have had a similar experience."

Arnold B. Cowan

(Br Med J. Sep 27, 1958; 2(5099): 797)

I'm rather sceptical as to this story - I've never really believed that such accounts are credible (crucial details such as the time of day tend to get embellished for effect). But what do you think? Has anyone heard of a similar thing happening in their family, perhaps?




Image courtesy of: dan / Free Digital Photos (www.freedigitalphotos.net)




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.