Issue: May 2020
Source/Disclosures
Disclosures: Brien reports no relevant financial disclosures.
May 21, 2020
4 min read
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Young girl hospitalized with abdominal pain

Issue: May 2020
Source/Disclosures
Disclosures: Brien reports no relevant financial disclosures.
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A previously healthy 6-year-old female is hospitalized with mild dehydration, diarrhea and abdominal pain. The pain is described as chronic and recurrent over a few years. She had previously been evaluated in the gastrointestinal clinic about 1 year earlier, where, at that time, she was found to be healthy. Her travel history was positive for spending a month in Cuba to visit relatives when she was about 2 years of age. The parents thought the abdominal pains began shortly after that trip. There has been no other travel. The family has no pets, but she is frequently exposed to neighborhood cats and dogs. The family history is unremarkable, with no sick contacts.

Examination found that she has normal vital signs but mild abdominal pain with a liver that feels somewhat enlarged by palpation. The rest of her exam was normal.

James H. Brien, DO
James H. Brien

Her lab results revealed normal liver enzymes and a white blood cell (WBC) count of 36,900, with 76% eosinophils and mild anemia. She had an immunoglobulin G level of 2,250 mg/dL and an immunoglobulin E level of 1,031 mg/dL (both elevated). A stool pathogen panel (PCR for enterotoxigenic Escherichia coli, E. coli 0157, Shiga-like toxin-producing E. coli type 1/type 2, enteroaggregative E. coli, Salmonella spp., Shigella spp., Yersinia enterocolitica, norovirus, rotavirus, adenovirus, Clostridioides difficile, Campylobacter spp., Giardia, Cryptosporidium, Vibrio spp. and Entamoeba histolytica) was negative. However, a stool ova and parasite exam revealed Blastocystis hominis.

Her abdominal ultrasound revealed multiple small hypoechoic lesions in the liver and confirmed the mild hepatomegaly. A follow-up abdominal CT scan confirmed these findings (Figure 1).

Figure 1. Enlarged liver with numerous hypoechoic lesions
Figure 1. Enlarged liver with numerous hypoechoic lesions.
Source: James H. Brien, DO

What’s your diagnosis?

A. Blastocystosis

B. Visceral larva migrans

C. Hepatosplenic cat scratch disease

D. Ancylostoma braziliense

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The answer is B, visceral larva migrans (VLM), which is usually caused by the parasite Toxocara canis (the dog roundworm). As noted by the CDC, “Humans are accidental hosts who become infected by ingesting infective eggs or undercooked meat/viscera of infected paratenic hosts. After ingestion, the eggs hatch and larvae penetrate the intestinal wall and are carried by the circulation to a variety of tissues (liver, heart, lungs, brain, muscle, eyes). While the larvae do not undergo any further development in these sites, they can cause local reactions and mechanical damage that causes clinical toxocariasis. The main clinical presentations of toxocariasis are VLM and ocular larva migrans.” Diagnosis of VLM is usually made by serology or the finding of larvae in biopsy or autopsy specimens.

Figure 2. A follow-up CT revealing resolution of the lesions in the liver.
Figure 2. A follow-up CT revealing resolution of the lesions in the liver.
Source: James H. Brien, DO

VLM was suspected in this case by the clinical findings of an enlarged liver, combined with the CT findings of discrete liver lesions, along with a history of foreign travel and chronic abdominal pain. With the supporting lab results of a high WBC count, anemia and marked eosinophilia, the IgG and IgE levels were ordered, along with Toxocara IgG antibodies sent to the CDC, all of which were found to be elevated. She was then treated with albendazole at 400 mg twice a day for 5 days with good results (Figures 2 and 3). However, it should be noted that all patients should have a dilated eye exam by an ophthalmologist as part of the evaluation prior to initiating treatment, as the antiparasitic therapy may aggravate the inflammatory response within the eye, which is best managed by the ophthalmologist.

Toxocariasis can occur without international travel, as VLM can be seen in the Southern United States, but it is more common in tropical countries. According to CDC data, about 14% of the U.S. population, mostly in Southern states, have antibodies to this roundworm. For this reason, it requires more than just a positive Toxocara IgG test to make the diagnosis. Likewise, whether this patient’s disease was acquired in Cuba or not can never be proven.

A follow-up CT revealing resolution of the lesions in the liver
Figure 3. A follow-up CT revealing resolution of the lesions in the liver.
Source: James H. Brien, DO
Blastocystis hominis can be found in about 25% of the U.S. population. About half of those cases are asymptomatic, while others may have various abdominal symptoms, from mild diarrhea to symptoms mimicking inflammatory bowel disease. If treatment is needed, nitazoxanide is usually recommended. This organism does not migrate out of the gut and would not result in any abnormal tests (other than the stool ova and parasite screen) or abnormal imaging.

Figure 3. Cutaneous larva migrans.
Figure 4. Cutaneous larva migrans.
Source: Brent Steadman, MD

Hepatosplenic cat scratch disease (HSCSD) may also present with chronic abdominal pain. When the evaluation leads to imaging, discrete hypoechogenic lesions are typically seen in the liver and/or spleen. The diagnosis is usually confirmed by elevated Bartonella henselae immunoglobulin M and IgG titers. Various medications have been used for HSCSD. Refer to the AAP’s Red Book for current treatment recommendations.

cutaneous larva migrans
Figure 5. Resolution of the cutaneous larva migrans after treatment in the same patient shown in Figure 4. 
Source: Brent Steadman, MD

Ancylostoma braziliense is the cause of cutaneous larva migrans. The hookworm can be found in the Southeastern U.S., and it is common in tropical environments. The foot is the most common site of involvement (Figures 4 and 5). Treatment is also with albendazole, for 3 days.

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Figure 6. Hepatosplenic cat scratch disease.
Source: James H. Brien, DO

Teaching points:

A. With a markedly elevated eosinophil count with liver and/or lung symptoms, or other organ system dysfunction (vision), a migrating parasite should be high on the list.

B. Multiple liver lesions on imaging in this setting is usually where the action is.

C. VLM usually does not cause splenic lesions.

D. All patients should have an eye exam before starting treatment.

E. As this patient demonstrates, evaluating vague, chronic and recurrent abdominal pain in a child can be a diagnostic challenge, requiring a more detailed history and physical exam, and eventually expanded lab/imaging testing.

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Figures 7. Hepatosplenic cat scratch disease.
Source: James H. Brien, DO
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Columnist comments: About 1 year ago, I prescribed albendazole for an adolescent patient with suspected echinococcosis of the spleen based on MRI imaging, suggestive of multiple cystic lesions within a larger cyst. The albendazole was to be used as presurgical drainage treatment with continuation therapy, as suggested by the CDC. The prescription was written for a 2-week supply, the date for surgery (puncture-aspiration-injection-reaspiration, or PAIR, therapy) was set, and an ID clinic follow-up arranged. At the end of the day, I was contacted by one of our social workers, telling me that the albendazole was going to cost the family over $10,000 (they were uninsured), with no cheaper options available. Since the diagnosis had not been confirmed serologically, and the patient was stable, we put off the treatment and surgery pending antibody results from CDC, which returned negative, and the patient subsequently went on to have a benign, noninfectious cyst removed.

The diagnosis in this patient is not the point, but rather the incredible increase in the price of this medication. I do not prescribe albendazole often, so I never knew of any problem regarding its cost. I looked into it. The dose took a sharp rise in 2011. NPR reported in December 2017 that two, 200 mg albendazole tablets in Tanzania for hookworm disease cost 2 cents, but in the U.S. cost $400. This seems to be a much bigger problem in the U.S. than in other developed countries, where price controls are in place. This is obviously not unique to albendazole. We all remember the outrageous story from 2015 when the price of pyrimethamine rose from $13.50 per pill to $750 per pill. Some in the industry have been very slick with this practice, making deals with insurance companies to pay higher prices, resulting in higher premiums and, in the case of Medicare and Medicaid, on the backs of taxpayers.

I recommend taking any opportunity you have to speak up against this ridiculous practice for the benefit of our patients (and ourselves).

I’ll stop here. Please email me with your comments or questions about this or anything else on your mind.

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Disclosure: Brien reports no relevant financial disclosures.