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Tuesday, September 16, 2014

7 cm pencil stuck in eye orbit


Every once in a while, children tend to get a bit wild while playing. Unfortunately, sometimes just one moment of carelessness can have severe consequences. Even something as banal as tripping and falling can result in a serious injury, as this case from Iran shows. 

In 2011, a 6-year-old boy with orbital trauma was brought to the the Ophthalmology Department of Imam Khomeini Hospital in Kermanshah. According to his parents, he suddenly fell when playing and landed on a pencil, which penetrated the upper part of his right orbit and split, so that half of it remained lodged above the eye. Because the pencil had broken at the entry point, it could not be removed manually. The situation was quite grim, since the penetration of objects into the orbit can lead to blindness and even to the death of the patient as a result of brain damage and/or infection.



 A broken piece of pencil sticking out from the upper cavity of the right orbit. Ouch!


A physical examination with a slit lamp biomicroscope showed no damage to the boy’s cornea. The conjunctiva was lacerated, with sub-conjunctival hemorrhage, and the eyeball itself was severely hypertonic and tense. Since the child was in great pain and severely agitated, doctors were unable to perform a CT scan. However, an X-ray showed a straight object, clearly looking like a pencil, stuck in the orbital space.



An X-ray shows a long object in the orbital space, behind the eye. You can see that the object's outer shell is less dense (dark) as compared to the core. X-rays pass easily through wood, but not through carbon.


The child was rushed to the operation room to try to save his eye. After general anesthesia, the pencil was carefully removed from the orbit. It turned out to be a whopping 7 centimeters long! By comparing the two broken sections of the pencil, the surgeon could confirm that nothing was left behind, but to be absolutely sure, a CT scan was performed. It showed the complete removal of the foreign object from the orbit and no damage to the patient's eye or brain. (The boy's parents must have breathed a huge sigh of relief at this point.)



The bloodstained piece of pencil - 7 centimeters long - after removal.
(All images from: Rezae et al. 2014)
 

The boy was supervised for two months after surgery and showed no adverse effects from the trauma. Notably, his eyesight did not suffer as a result of this accident - his visual acuity was still 10/10. He's one lucky child, since he could have very easily lost his eye.


Source:

Rezae L, Naiafi F, Moradinazar M, Ahmadijouvbari T. (2014) Case report on the successful removal of an organic penetrating object into the orbit. J Inj Violence Res. 6(1): 50–52.

 

Friday, September 12, 2014

Elephantiasis nostras verrucosa: two cases


Chronic lymphedema (tissue swelling caused by impaired lymphatic flow) can be an extremely disfiguring and disabling condition. In the past, I've posted descriptions and photos of the disfigurement caused by lymphatic filarial disease, with cases from Haiti and from India. I've also described a case of scrotal lymphedema caused by chronic infections of the urinary tract. This post is about an associated dermatological condition - elephantiasis nostras verrucosa. Be warned - it's not pretty.

Elephantiasis nostras verrucosa is a rare, disfiguring complication of longstanding chronic lymphedema. Recurrent soft tissue infections worsen the condition. Disruption of lymphatic vessels due to trauma or surgery, venous stasis (sluggish blood flow in the veins), radiation, obstruction due to tumors, portal hypertension, obesity and congestive heart failure can all be the precipitating factors of this disease. The affected extremity swells, sometimes to huge proportions; the skin thickens, becomes lichenified and can become covered with knobbly growths. Treatment options include use of elastic bandages, pneumatic stockings, mechanical massage, oral retinoids and surgery. The goal is to restore function and to reduce physical disability. For refractory cases, microsurgical lymphovenous anastomosis can be performed. Amputation should be the last option.

The photo below shows the legs of a 63-year-old obese patient, with chronic lymphedema, tissue inflammation (note the reddened skin) and pronounced elephantiasis nostras verrucosa of the left lower extremity, where the swelling is much greater. 8 years earlier he had received treatment for colon cancer, consisting of surgery and radiotherapy to the lower abdomen. Chronic lymphedema developed after radiotherapy in both legs.




And this is a close-up shot of the left leg. Note the size of the swelling and the thickened, rough skin.




Berngard and Narayanan have described a rather more striking case - that of a 54-year-old man with morbid obesity and congestive heart failure, who sought medical attention, complaining of difficulty breathing at night, swollen legs and weight gain. Physical examination revealed a large abdominal pannus (a thick sheet of skin and fat tissue hanging down from the belly) with lichenified skin containing cobblestone-like nodules and papules with hyperpigmentation. This large, heavy "apron" completely encircled the patient’s genitals and left a fibrotic tract from which he urinated. Ick!



Huge pannus hanging down between an obese man's legs. 



A close-up of the knobbly, hyperpigmented growths on the abdomen.


The patient was treated for decompensated heart failure and was discharged home with outpatient follow-up to be evaluated for surgical removal of the pannus.


Literature:

Akturk HK, Gbadamosi-Akindele M. (2014) Elephantiasis nostras verrucosa. BMJ Case Rep.

Berngard SC, Narayanan V. (2011) Elephantiasis nostras verrucosa of the pannus. J Gen Intern Med 26(7): 810




Thursday, September 4, 2014

Wound myiasis (Warning: GRAPHIC PHOTOS!)


As you may know - myiasis is the infestation of a living animal's tissues by fly larvae, or maggots. These infestations occur all over the world, but are most common in tropical climates. The larvae can feed on the host's living or dead tissue, bodily fluids, or even ingested food. In animal husbandry, myiasis leads to great economic losses, since it reduces milk production and hide quality. In humans, maggots are known for causing highly unpleasant - and sometimes dangerous - wound infestations. Poverty, bad living conditions and a lack of hygiene are predisposing factors. Inadequate medical and nursery care of the elderly, psychiatric patients, alcoholics, and other helpless patients, especially those with the inability to discourage flies from depositing eggs or larvae, also makes humans prone to wound infestation (on this blog, I've described the cases of an alcoholic from Brazil with a huge maggot-ridden cancer wound and a boy with cerebral palsy whose tracheostomy became infested with fly larvae, probably as a result of neglect). Poor eyesight may make it harder to detect myiasis. Human natural disasters may be another predisposing factor for wound infestation by fly larvae.

Wound myiasis is most often initiated when flies lay egges in necrotic, hemorrhaging, or pus-filled lesions. Wounds with alkaline discharges (pH 7.1 to 7.5) have been reported to be especially attractive to blow flies. The presence of necrosis is also an important factor. In human cases, there is usually only one offending species in the lesion, although mixed infestation can occur, reaching rates of 3% in one series.

Cochliomyia hominivorax, the New World screw-worm fly, or screw-worm for short, is a singularly unpleasant species of parasitic fly, well known for the way in which its larvae eat the living tissue of warm-blooded animals. While the maggots of many fly species eat dead flesh, and may occasionally infest an old and putrid wound, screw-worm maggots are unusual because they attack healthy tissue. C. hominivorax is found in the New World tropics. There are five species of Cochliomyia but only one species of screw-worm fly in the genus; there is also a single Old World species in a different genus (Chrysomya bezziana). 
 
Both New World and Old World screw-worms cause multiple infestations, with 100 to 500 eggs. The females often lay eggs on or near a wound. After hatching, the larvae immediately begin feeding, causing an extensive destruction of tissue and a foul-smelling bloody discharge. Tissues around the lesion become swollen, and pockets may be eaten out beneath the skin. C. hominivorax and C. bezziana myiases are typically very painful. Tissue invasion and local destruction caused by the larvae when leaving the necrotic tissue - more mature larvae are often more invasive - lead to significant local pain and secondary bacterial infection. Other symptoms include fever, chills, bleeding, and fistula formation. Cavernous lesions are formed, so it is difficult to extract the larvae in a single session, and this delay makes the situation more dangerous. An infested person may actually die from tissue destruction.

Scary, right? And here are some photos. Please don't lose your lunch.


Myiasis due to C. hominivorax in a B lymphoma patient. The photo shows a huge ulcer filled with larvae.



 And here is a close-up shot of the ulcer, along with the maggots in all their wriggly glory. Ugh.


 (Images from: Francesconi and Lupi 2012)

C. hominivorax myiasis in a basal cell carcinoma case. Note how large the larvae are.

Lying weak and bedridden, with stinking fluid seeping from your wounds and maggots slowly eating you alive, sounds like something from a Stephen King novel - but sadly, it does happen in real life.


Literature:

Francesconi F, Lupi O. (2012) Myiasis. Clin Microbiol Rev. 25(1): 79-105.


 

Monday, September 1, 2014

Diogenes syndrome in an elderly woman


Some time ago, I wrote a post about a computer programmer from Kolkata, India, who suffered from schizophrenia and associated Diogenes syndrome. As you might know, Diogenes syndrome is a behavioral disorder that manifests itself in self-neglect, extremely poor personal hygiene, domestic filth, a tendency to hoard rubbish and a lack of shame regarding one's living conditions. Approximately 0.05% of persons over the age of 60 suffer from this condition. Interestingly, sufferers are usually of average or above-average intelligence. Diogenes syndrome is often associated with other mental illnesses, such as schizophrenia, mania and frontotemporal dementia. It may also appear in conjunction with obsessive-compulsive disorder. The affected persons are typically in complete denial, and very reluctant to accept help. This can make treating them very difficult.

Irvine and Nwachukwu have described the case of an elderly lady suffering from this condition, with all the typical symptoms. A 61-year-old obese Caucasian lady with a previous history of bipolar 1 disorder presented for an out-patient psychiatric follow-up review. She was found to have pressured speech, an elated mood, increased energy, and very poor personal hygiene. She was disheveled, unkempt and smelly, wearing dirty clothes. She was also very agitated, and was verbally and physically abusive to staff. She had no insight, and refused any form of treatment. The diagnosis - a manic relapse as a result of not taking the prescribed medication. The woman was involuntarily admitted to the in-patient psychiatric ward. The usual laboratory tests were performed (complete blood count and so on) and all the results came back normal. She was re-started on her previous psychiatric medication.

The following day she was adamant about having to go feed her cats and dogs, and eventually gave permission for a Community Mental Health Nurse to enter her house and attend to her pets. It turned out that the home was in complete disarray. Every room was crammed with filthy clothes, garbage, dirty dishes, and rotting food. There was no kitchen sink in sight, and it looked as if some dishes were being cleaned in the toilet. Any clear space of floor was strewn with cat and dog feces. An unbearable stench emanated from the entire two-story house. When the patient was questioned regarding the state of her home and personal hygiene, she had no insight into any problems. This inability to assess the reality of one's condition is a typical and baffling feature of Diogenes syndrome.




This is what the bathroom in the patient’s house looked like. The patient had been using the toilet both for its designated use and for periodically washing her dishes. Eeek! 




The patient’s living room looked even worse. It was filled with dirty clothing, old newspaper, and animal feces. It makes me sad to think about the cats and dogs living in that filthy home, probably underfed and without any veterinary care.

The woman's mood became normalized after some time in the hospital, although she remained guarded, with little insight into her condition. Apparently, she required persistent and gentle pressure in order to even start thinking about decluttering her home and improving her personal hygiene. She was eventually persuaded to allow a company to help her clean her house, at a cost of $8,073. Even the sink was eventually found under a large pile of debris. The patient is now living at home, and receiving close psychiatric follow-up. It remains to be seen whether the interventions will make any difference to her lifestyle in the long run.

Sadly, cases like this one are fairly common. Almost all of us know at least one hoarder, although the behavior might not be as extreme. It's also quite difficult to provide help when the affected person doesn't recognize the situation as a problem, and is unwilling to seek or accept help.


Literature:

Irvine JD, Nwachukwu K. (2014) Recognizing Diogenes syndrome: a case report. BMC Res Notes. 7: 276.



Thursday, August 28, 2014

Forgotten vaginal pessaries cause fistula formation


A pessary is a medical device inserted into the vagina, either as a method of delivering medication, or to provide structural support. When properly maintained (ideally, they should be removed and cleaned every 6 weeks), pessaries have been shown to be safe for long-term care of vaginal prolapse, a condition where a portion of the vaginal canal protrudes from the opening of the vagina. However, neglected pessaries can cause complications such as vaginal irritation, impaction, ulceration and even fistula formation (a fistula is an abnormal connection - an opening or canal - that forms between two organs, such as an intestine and the skin, or the vagina and the urinary bladder). Rarely, a rectovaginal fistula can form - and believe me, this is literally a BIG pain in the butt.

 

Vaginal pessaries. (Image from: Wikipedia)


An article by Powers et al. presents the case of a 70-year-old woman who was found to have an impacted pessary that could not be removed due to pain. Examination under anesthesia revealed that the device had become firmly lodged within the rectum. It was removed through the anus, leaving a large rectovaginal fistula. The patient was scheduled for reparative surgery, but she cancelled the day before.

Hanavadi et al. describe another case of a forgotten vaginal pessary that ate its way into the elderly wearer's rectum. This one is quite disgusting, actually. An 88-year woman was referred urgently to the surgical clinic with a history of vaginal discharge containing feces. Her practice nurse noticed that following an enema, it had expelled through the vagina. The lady also reported 6-8 weeks of constipation alternating with diarrhea and fecal incontinence. Earlier, her bowel habits had been normal.

On examination, she was frail, but well oriented. Abdominal examination was normal. Digital rectal examination showed fecal loading and a hard object was felt through the rectal wall. Feces were present within the vagina, along with a hard object high up, which turned out to be a pessary. However, the patient could not remember ever having been fitted with such a device. 


She was examined more closely under anesthesia and it was determined that the handle of the forgotten pessary had eroded through the vaginal wall into the lower rectum, 8 cm from the anal verge. After the pessary was removed, something had to be done about the large rectovaginal fistula (just imagine passing feces through the vagina - ugh). Because the patient was too frail for reconstructive surgery, a colostomy was performed. Recovery was uneventful.

The article contained the following photo. I'm afraid it's pretty graphic. A red rubber tube has been passed into the elderly patient's rectum, through the defect and out of the vagina, demonstrating the abnormal connection between these anatomical structures.



Literature:

Hanavadi S, Durham-Hall A, Oke T, Aston N. (2004) Forgotten vaginal pessary eroding into rectum. Ann R Coll Surg Engl. 86(6): W18–W19. 

Powers K, Grigorescu B, Lazarou G, Greston WM, Weber T. (2008) Neglected pessary causing a rectovaginal fistula: a case report. J Reprod Med. 53(3): 235-7.]

Wikipedia: Pessary


Monday, August 25, 2014

Soft drinks can cause fatty liver disease


In warm, sunny weather, do you like to quench your thirst with a refreshing draught of chilled Coca-Cola, Sprite or Fanta? (Or Pepsi, 7-Up or Mirinda, whichever is more easily available where you live?) You're not alone. Lots of people prefer the addictively sweet taste of these fizzy beverages to plain, boring water. However, indulging the appetite for this "liquid candy" comes with a high price. The more sugary soda you drink, the higher your risk of obesity, diabetes and metabolic syndrome (the combination of abdominal obesity, high blood pressure, elevated plasma glucose and high serum triglycerides). Recent studies also show that drinking large amounts of soft drinks can cause non-alcoholic fatty liver disease.


Let's face it - we're genetically hardwired to love sweet-tasting foods and drinks. Thanks to modern agriculture and industrial production methods, sugar - once a luxury food - has become an everyday commodity for all but the very poorest. In 1776 - at the time of the American Revolution - Americans consumed about 1.8 kg (4 lb) of sugar per person each year. By 1850, this had risen to 9 kg (20 lb), and by 1994, to 54.4 kg (120 lb). In the last 40 years, the per capita consumption of refined sugar in the United States has varied between 27 and 46 kilograms (60 and 101 lb). In 2008, American per capita total consumption of sugar and sweeteners, exclusive of artificial sweeteners, equalled a whopping 61.9 kilograms (136 lb) per year. 

Our collective sweet tooth is being blamed as one of the causes of the worldwide obesity epidemic. However, an increasing body of evidence suggests that the consumption of soft drinks sweetened with sugar and/or high-fructose corn syrup poses a much bigger health risk than sugar in foods. The consumption of sugar-sweetened beverages rose by a startling 38.5 gallons per person between 1950 and 2000 (10.8 gallons per person in 1950 to 49.3 gallons per person in 2000). Controlled trials have now proved unequivocally that consumption of sugary soft drinks increases body weight and body fat. Randomized controlled trials in children and adults lasting 6 months to 2 years have shown that lowering the intake of soft drinks reduces weight gain. 

Non-alcoholic fatty liver disease (NAFLD) is a condition where fat deposits slowly accumulate in the liver in persons who do not consume excessive amounts of alcohol. In the beginning, NAFLD may cause no symptoms at all, or only mild ones (fatigue, vague abdominal discomfort). Nonetheless, this condition poses a significant health problem. Over time, it can progress to non-alcoholic steatohepatitis, a fatty liver with hepatitis. This form of liver injury carries a 20%-50% risk for progressive fibrosis, 30% risk for cirrhosis, and 5% risk for liver cancer.

Meta-analyses suggest that consumption of sugar-sweetened beverages increases the risk of diabetes, the metabolic syndrome, cardiovascular disease, and also of fatty liver. Drinking just two 16-ounce sugar-sweetened soft drinks per day for 6 months induces features of the metabolic syndrome and fatty liver. Scary, huh?

One recent study found that that 80% of patients with NAFLD had excessive intake of soft drink beverages compared to 17% of healthy controls. The NAFLD group consumed five times more carbohydrates from soft drinks compared to healthy controls (40% vs. 8%). Seven percent of patients consumed one soft drink per day, 55% consumed two or three soft drinks per day, and 38% consumed more than four soft drinks per day for most days and over the 6-month period. The most common soft drinks were Coca-Cola (regular: 32%; diet: 21%) followed by fruit juices (47%). 

And while diet soft drinks have only a fraction of the calories compared to the sugar-sweetened variety, artificial sweeteners such as aspartame aren't good for the liver, either. So if you're concerned about your diet and health, cutting out all store-bought sweet beverages - whether they're sweetened with high fructose corn syrup, sugar or aspartame - is your best bet. Get used to plain water, tea (black, green, white or red - there's a huge variety of flavors out there) or fruit teas, and enjoy an occasional cup of honey-sweetened tea or a glass of homemade lemonade as a special treat.

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




Literature:

Abid A, Taha O, Nseir W, Farah R, Grosovski M, Assy N. (2009) Soft drink consumption is associated with fatty liver disease independent of metabolic syndrome. J Hepatol. 51(5): 918-24.

Bray GA, Popkin BM. (2014) Dietary sugar and body weight: have we reached a crisis in the epidemic of obesity and diabetes? Health be damned! Pour on the sugar. Diabetes Care. 37(4): 950-6.

Nseir W, Nassar F, Assy N. (2010) Soft drinks consumption and nonalcoholic fatty liver disease. World J Gastroenterol. 16(21): 2579-88.

Wikipedia: Sugar



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.