Neonate presents with dramatic desquamation following home delivery, no prenatal care


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James H. Brien

A 3-week-old neonate is admitted to the hospital in acute respiratory distress due to respiratory syncytial virus bronchiolitis.

The history reveals that the baby’s mother had no prenatal care and had a spontaneous home delivery with the help of a relative who is a retired nurse midwife. The midwife advised that the mother and baby go to the local ED for evaluation, but the mother declined. Therefore, there is no newborn screen or immunizations.

Examination revealed no fever, but it did show tachypnea with an oxygen requirement of 2 L per minute via nasal canula. He was also found to be small for gestational age (SGA), with hepatosplenomegaly, dramatic generalized desquamation (Figure 1) and a reddish-copper-colored plaquelike macular lesion on the sole of the left foot (Figure 2).

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Figure 1. Neonate with widespread desquamation. Source: James H. Brien, DO.

Blood tests revealed mildly elevated liver enzymes, leukocytosis, hemolytic anemia and thrombocytopenia.

Summary:

  • a 3-week-old neonate with RSV bronchiolitis;
  • no prenatal care or newborn testing;
  • SGA with hepatosplenomegaly;
  • extensive, generalized desquamation with macular lesion on sole of left foot; and
  • Elevated liver enzymes, anemia, leukocytosis and thrombocytopenia.

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Figure 2. A reddish-copper-colored plaquelike macular lesion on the sole of the infant’s left foot. Source: James H. Brien, DO.

What’s your diagnosis? (What radiographic finding is pathognomonic for the diagnosis?)

A. Parenchymal brain calcifications

B. Periventricular calcifications

C. Situs inversus

D. Wimberger sign

Answer and discussion:

The correct answer is D, Wimberger sign, which is pathognomonic for congenital syphilis. It shows the radiolucent appearance of the medial aspect of the proximal tibia (Figure 3) due to syphilitic injury to the metaphysis, resulting in periostitis and demineralization. This was first described by Han Wimberger (1887-1954), an Austrian pediatrician (not a radiologist). With proper treatment, the process reverses.

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Figure 3. Wimberger sign showing lucency in the proximal medial aspects of the tibia. Source: James H. Brien, DO.

Congenital syphilis is caused by the intrauterine infection with the Treponema pallidum subspecies pallidum. Depending on the duration of infection before treatment, the baby may be SGA, with hepatosplenomegaly, prominent desquamation, a maculopapular rash that may also show reddish-copper-colored lesions that are more prominent on the palms and soles, lymphadenopathy and copious nasal discharge (snuffles). ). Over time, damage to bone, cartilage, teeth, brain, eyes and heart may also be seen.

Congenital cytomegalovirus (CMV) shares some features of congenital syphilis (SGA, hepatosplenomegaly, thrombocytopenia, multiorgan involvement, but no bone findings. Rather, CMV of the central nervous system results inion of calcifications, classically along the periventricular areas (Figure 4)).

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Figure 4. Congenital cytomegalovirus infection with periventricular calcifications. Source: James H. Brien, DO.

Congenital toxoplasmosis may also result in intracranial calcifications, but classical calcium deposits are found within the parenchyma of the brain (Figure 5). In both cases, significant brain and eye injury can occur if not treated early.

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Figure 5. Congenital toxoplasmosis infection with intraparenchymal calcifications. Source: James H. Brien, DO.

Situs inversus universalis refers to the combination of dextrocardia (right-sided heart), a left-sided liver and right-sided splen (Figure 6). While this anomaly is not associated with congenital infection, it is associated with ciliary dyskinesia, which may result in recurrent sinopulmonary infections. If bronchiectasis develops, the patient may fit diagnostic criteria for Kartagener syndrome (an autosomal recessive genetic disorder consisting of situs inversus, chronic sinusitis and pneumonia with bronchiectasis).

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Figure 6. Situs inversus universalis associated with ciliary dyskinesia. Source: James H. Brien, DO.

Lastly, according to Raul A. Romaguera, DMD, MPH, acting director of the CDC’s Division of STD Prevention, the United States was close to eliminating syphilis 20 years ago. Since then, there has been a sharp increase in syphilis and congenital syphilis, which has quadrupled since 2015, with over 1,800 cases in 2019. As one might guess, there appears to be a correlation with cuts in public health funding. According to the Pan American Health Organization, several small Caribbean territories have eliminated HIV and syphilis transmission from mothers to babies, showing that it is possible with proper commitment.

Columnist comments:

Please join me for my 30th and last appearance at the Infectious Diseases in Children Symposium in New York this November. I plan to show some of the best cases I have encountered over the years, and I hope to see you there.

For more information:

Brien is a member of the Healio Pediatrics and Infectious Disease News Editorial Boards, 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.

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