Infant in Iraq presents with upper respiratory infection
What’s your diagnosis?
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During a military deployment to Iraq, a 14-month-old male in a refugee camp presented to sick call (Figure 1) for evaluation of a minor upper respiratory tract infection.
On exam, he had the typical findings of nasal congestion with mild rhonchi on auscultation of the chest. However, he was incidentally noted to have a machinelike heart murmur that one might hear with a patent ductus arteriosus. He was also noted to have an unusual appearance of his left eye (Figure 2). Other important findings included being small for his age, microcephaly, developmental delay and mild spastic quadriparesis.
The father is missing in the war, and the mother does not know what immunizations are; therefore, the patient is assumed to be unimmunized.
What’s your diagnosis?
A. Congenital cytomegalovirus
B. Congenital rubella
C. Congenital toxoplasmosis
D. Congenital syphilis
Answer and discussion:
The answer is B, congenital rubella. Rubella, a vaccine-preventable infection, has been considered eliminated from the United States since 2004. However, there are still small numbers of cases imported every year. The numbers in the U.S. may rise again with decreasing use of immunizations here and abroad. To adults and children, rubella is usually a fairly mild, febrile, rash-producing disease, but the effects on the developing fetus in a mother with rubella infection can be life-threatening. Babies affected by congenital rubella syndrome have some or all of the features in this vignette, including congenital cataracts. Before rubella immunizations, congenital rubella was a leading cause of congenital cataracts. Additionally, they may have a “blueberry muffin” rash (Figure 3) caused by extramedullary hematopoiesis around the time of birth, which is commonly seen in congenital cytomegalovirus (CMV) as well.
CMV is the most common congenital infection today and the most common cause of nongenetic neurosensory hearing loss — one of the reasons that babies have a hearing screen in the newborn period. If there is any suspicion of congenital CMV in the symptomatic baby, testing is done, and if positive, the patient may be treated with oral valganciclovir or IV ganciclovir, per Red Book guidelines.
Congenital toxoplasmosis (caused by Toxoplasma gondii, an obligate intracellular protozoan) is challenging because the baby may look normal unless it is severe, with early gestation infection. Patients with congenital toxoplasmosis and CMV may develop severe vison loss or blindness due to chorioretinitis and have neurologic problems, which are often accompanied by intracranial calcifications. The pattern of calcifications are not diagnostic, but can be predictable, with calcifications due to congenital CMV being in a periventricular pattern (Figure 4) and that of congenital toxoplasmosis being intraparenchymal (Figure 5).
Patients with congenital toxoplasmosis should almost always be referred to an infectious disease consultant for proper diagnosis and therapy, which is fairly complex. Additionally, evaluations by an ophthalmologist and neurologist are usually needed.
Congenital syphilis also has several distinctive features, such as marked desquamation, which may be accompanied by copper-colored plaquelike lesions on the palms and/or soles (Figure 6), a variety of other skin lesions, enlarged liver and spleen, and the patient may also be small for gestational age. Ocular abnormalities like cataracts can occur but are not as common as with congenital rubella.
Distinct bone abnormalities may include periostitis of the long bones and demineralization about the metaphases, such as that seen in Wimberger sign (Figure 7), where the proximal, medial aspects of the tibiae are demineralized, which is reversible with treatment of the infection (Figure 8).
All cases of congenital syphilis are a result of receiving inadequate or no prenatal care, where no prenatal screening is done. If detected in the pregnant mother, appropriate treatment is given to the fetus and the mother, and it will prevent congenital syphilis. According to the CDC, there has been a sharp rise in cases of syphilis and congenital syphilis. This is invariably tied to inadequate funding of our public health services, which does the tedious contact investigations. This is an updated and expanded version of the case shown in this column 23 years ago in April.
Columnist comments
In December, I drew attention to vaccine hesitancy with a comment to avoid making parents who might be undecided more resistant by taking on a harsh, judgmental tone when visiting with these parents, as it is a sure way to push them over the fence. Having said that, I would now like to point to the fact that measles has been rising over the last 3 years. According to CDC data, in 2019, there were 1,274 cases reported, then the number dropped to only 13 cases in 2020, followed by 49 cases in 2021. Last year, 121 cases were reported, again demonstrating a rising trend.
A case of paralytic polio in an unimmunized adult was diagnosed in New York State in July, 2022. Through investigation, the virus was determined to be an oral polio vaccine strain. Since the Sabin polio vaccine has not been made or used in this country since 2000, further investigation was undertaken with subsequent genetic sequencing of several wastewater samples, which found the live polio vaccine viruses had originated in Jerusalem and London. As an enterovirus, live polio vaccine virus is excreted in the stools for up to 6 weeks of those recently immunized with the oral polio vaccine (Sabin), and therefore can be found in waste water samples. How it got into this patient is probably the same way all enteroviruses get into us: through the fecal-oral route. This is not likely the first time this scenario has occurred, just the first time it has been documented. Healthy, exposed people would not likely get polio disease, and therefore would not be tested or diagnosed. Plus, most children and adults in this country are immunized with the killed, equally effective Salk vaccine. As time goes by and vaccine virus continues to circulate, genetic changes may occur, with the virus regaining virulence. Couple that with increasing refusal to immunize, and it may result in turning the clock back to pre-1950s when Polio was the dreaded fear of all parents. However, that fear is what it may take to get vaccine-hesitant and vaccine-resistant parents and other adults to “wake up.”
I hope your 2023 is off to a healthy and happy start. Good health is the best medicine for happiness. With a healthy mind and body, most troubles can be overcome.
References:
CDC. Congenital syphilis fact sheet. https://www.cdc.gov/std/syphilis/stdfact-congenital-syphilis.htm. Accessed Feb. 6, 2023.
CDC. Number of measles cases reported by year. https://www.cdc.gov/measles/cases-outbreaks.html. Accessed Feb. 6, 2023.
CDC. United States confirmed as country with circulating vaccine-derived poliovirus. https://www.cdc.gov/media/releases/2022/s0913-polio.html. Accessed Feb. 6, 2023.
For more information:
Brien is a member of the Healio Pediatrics Peer Perspective Board and an adjunct professor of pediatric infectious diseases at McLane Children's Hospital, Baylor Scott & White Health, in Temple, Texas. He can be reached at jhbrien@aol.com.