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| Journal of the New Zealand Medical Association,
02-March-2007, Vol 120 No 1250
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Beware of infants with respiratory distress, rash,
and hepatomegaly at birth: a case of congenital syphilis
Malcolm Battin, Lesley Voss
Pregnant women with syphilis may be asymptomatic, hence
identification is dependent on serological screening. Despite New Zealand (NZ)
having a longstanding policy of such screening at pregnancy booking, congenital
syphilis still occurs.
Case reportAn 1800-gram infant was born to a 21-year-old NZ European
woman, with an unrecognised pregnancy who had no antenatal care. This was her
second pregnancy; the first resulted in a live spontaneous vaginal birth at
term. Spontaneous onset of labour occurred at 32 weeks’ gestation, based
on approximate last menstrual period (LMP), with rapid delivery shortly
thereafter.
A live male infant was born in the ambulance, with an
initial cry and some respiratory effort. He deteriorated quickly despite
receiving bag-mask positive-pressure ventilation and chest compressions in the
ambulance. On arrival at the hospital he was cold and limp with a heart rate of
40 bpm—but responded to intubation, positive-pressure ventilation, and
cardiac massage. Once stabilised, he was transferred to the neonatal unit.
On admission examination, there were multiple, punched out,
pale, blistered lesions mainly on peripheries but also on ears and bridge of
nose and associated desquamation of palms and plantar surfaces of the feet
(Figure).
 Additionally, there was meconium staining of the skin and
marked hepatomegaly. The initial chest radiograph was consistent with a
congenital pneumonia and the diagnosis of congenital sepsis with pneumonia was
made. Antibiotics were commenced with amoxycillin and gentamicin in standard
neonatal doses. Blood cultures were negative as were viral cultures of both
stool and nasopharyngeal aspirate. Swabs from the ear, lesions, gastric contents
and placenta did not reveal any pathogen.
However, the diagnosis of congenital syphilis was confirmed
with positive venereal disease research laboratory (VDRL) screen and reactive
rapid plasma regain (RPR) and Treponema pallidum particle agglutination
assay (TPPA) tests. Maternal serology was also positive, with RPR and TPPA
reactive. Examination of the infant’s cerebrospinal fluid (CSF) revealed
VDRL was reactive, red cells 244×106/L,
white cells 17×106/L (polymorphs 5%
lymphocytes 58% monocytes 37%), elevated protein 1.47 g/L (0.15–0.45 g/L),
glucose: 3.1 mmol/L (2.8–4.4 mmol/L), with no growth on culture.
Subsequent cerebral ultrasound scans were within normal limits. He completed 12
days of parenteral penicillin G.
The neonatal course included pulmonary hypertension treated
with positive pressure ventilation, two doses of surfactant, and nitric oxide
for 3 days— the infant recovered well. The family were referred to the
adult infectious diseases and sexual health teams, and the infant to the
paediatric infectious diseases (ID) team for follow-up.
DiscussionA resurgence in syphilis has been documented in a number of
developed countries in recent years.1–3
In NZ, a recent paper suggests an increase in the number of people being
diagnosed with syphilis at sexual health
services.4 An apparent increase was seen in
heterosexual cases including two women found to be positive on antenatal
screening.
Antenatal screening for syphilis is an effective method of
identifying women for treatment to prevent the birth of an infected child.
However, it does have some limitations. Firstly, it will not identify women who
acquire syphilis during later stages of pregnancy after the antenatal screening
has been performed or those incubating disease at initial
testing.5 Secondly, as in our case, when the
woman does not have antenatal bloods performed, the potential for treatment in
pregnancy is lost. Thirdly, the clinician may have difficulties in interpreting
abnormal syphilis serology resulting in either lack of follow up or suboptimal
treatment.6–8
Although the VDRL/RPR is used as a screening test for
syphilis, it is a nonspecific test and biological false positives do occur in a
number of other conditions, including pregnancy. In the past, yaws has also
caused confusion and difficulty interpreting syphilis serology, particularly in
women from the Pacific. Therefore for any women with a positive syphilis screen
in pregnancy, a specific syphilis test—TPPA/FTA-ABS (fluorescent
treponemal antibody absorption test)—should be performed and appropriate
treatment given if syphilis confirmed. If concerns remain at the time of
delivery, then the neonate should be investigated.
Congenital syphilis can present with protean manifestations
and lead to delayed diagnosis. Characteristic features include, rash
(maculopapular or vesicular), mucousal lesions, nasal discharge, hepatomegaly,
bony tenderness, or eye lesions. The Hutchinson’s triad, first described
in 1858, describes the late findings of congenital syphilis of notched incisor
teeth, interstitial keratitis, and eighth cranial nerve deafness. However
children can be born with no obvious clinical manifestations initially, and the
disease can present much later.
In the current era of rising rates of sexually transmitted
diseases in NZ, it is important to check maternal serology and consider this
disease in any child with suspicious clinical findings, particularly if
antenatal screening has not occurred, but even if the initial pregnancy
screening on the mother has been negative.
Author information: Malcolm Battin, Senior
Lecturer (Neonatology), University of Auckland and National Women’s
Health, Auckland; Lesley Voss, Paediatrician, Infectious Diseases Services,
Starship Children’s Hospital, Auckland
Correspondence: Dr Malcolm Battin, Newborn
Service, 9th Floor Support Building, Auckland City Hospital, Private Bag 92189,
Auckland. Email: malcolmb@adhb.govt.nz
References:
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