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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. 



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.