A case of congenital syphilis: the alarming and inequitable rise in congenital syphilis in Aotearoa and a call to action
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clinical correspondence
A case of congenital
syphilis: the alarming
and inequitable rise in
congenital syphilis in
Aotearoa and a call to action
Amanda Hall, Danny de Lore, Massimo Giola,
Tess Edmonds, Emma Best, Rachel Webb
A
2,400g male infant was born to a unit (PICU) at Starship Children’s Hospital.
multiparous New Zealand Māori The infant required further fluid resus-
woman in the upper North Island citation, and an adrenaline infusion and
following spontaneous onset of labour at non-invasive ventilation were commenced.
approximately 36 weeks gestation. The preg- Blood cultures flagged positive after several
nancy was not recognised until 24 weeks hours with a gram-negative bacillus, later
gestation, resulting in incomplete antenatal confirmed as Escherichia coli resistant to
screening. The infant was born in good amoxicillin. In PICU, respiratory support
condition with Apgar scores of 9 and 10 and and inotropes were discontinued within
was admitted to a Level 2 Special Care Baby several hours, but the infant developed
Unit (SCBU) to establish feeding. He was worsening conjugated hyperbilirubinaemia,
discharged following an uncomplicated ten- significant lactic acidosis and severe
day stay. hyponatraemia. Abdominal ultrasound
At 20 days of age, the infant was read- demonstrated unilateral hydronephrosis
mitted to hospital with a 24-hour history of with pelvico-ureteric junction (PUJ)
decreased feeding, irritability, abdominal obstruction, hepatosplenomegaly and a
distension and jaundice. On admission, small volume of ascites. The gallbladder
the infant appeared acutely unwell, irri- appeared normal. Urine showed nephrot-
table and deeply jaundiced with marked ic-range proteinuria. Extensive viral testing
hepatomegaly. There were no signs of was negative, including for herpes simplex
cardiorespiratory instability or seizure virus by polymerase chain reaction (PCR).
activity. The infant was assessed by the metabolic,
Initial investigations showed conjugated surgical, gastroenterology and infectious
hyperbilirubinaemia (bilirubin 166umol/L), disease teams. During an infectious diseases
anaemia (haemoglobin 66g/L), thrombocyto- consultation, a subtle desquamating rash on
penia (platelets 60x109/L) and coagulopathy. the soles of the feet was noted and urgent
C-reactive protein was elevated at 127mg/L. treponemal serology requested. On day
A blood gas demonstrated lactic acidosis five in PICU, positive reactive rapid plasma
(4mmol/L) and hypoglycaemia (blood reagin (RPR) titre of 1:32 and Treponema
glucose 2.7mmol/L). Anti-infectives were pallidum particle agglutination assay
commenced for suspected late-onset (TTPA) results became available. On further
neonatal sepsis (amoxicillin, cefotaxime and questioning, the infant’s mother revealed
aciclovir). a history of syphilis diagnosed several
years prior with incomplete treatment.
Several hours after presentation the
She was then lost to follow-up by her local
infant’s condition deteriorated with tachy-
sexual health service. Additionally, ante-
cardia and hypotension and he was urgently
natal syphilis serology was not documented
transferred to the paediatric intensive care
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NZMJ 22 October 2021, Vol 134 No 1544
ISSN 1175-8716 © NZMA
www.nzma.org.nz/journalclinical correspondence
during her current pregnancy. Further described in neonates. In a case series from
infant investigations included abnormal Bejing, 11 infants with congenital syphilis
long bone x-rays showing periostitis and who developed the Jarisch-Herxheimer
extensive bony sclerosis involving the lower reaction all had multi-organ involvement
and upper limbs, scapulae, clavicles and with high spirochete load.4
ribs. A lumbar puncture was grossly blood- New Zealand has seen a concerning
stained but showed a mild cerebrospinal increase in congenital syphilis in recent
fluid (CSF) pleocytosis (WBC 200x106/L, 74% years. Between April 2018 and June 2020,
polymorphs, RCC 31,000 x106/L, VDRL not prospective surveillance by the New Zealand
performed due to bloodstaining, culture Paediatric Surveillance Unit (NZPSU) iden-
negative). Cranial ultrasound, ophthal- tified 32 exposed infants and 12 probable/
mology and audiology evaluations were confirmed cases; seven of these infants’
normal. mothers were not diagnosed with syphilis
Congenital syphilis was diagnosed on the in pregnancy. The annual incidence was 9.4
basis of positive serology and the constel- per 100,000 live births.5 In contrast, there
lation of consistent clinical features. The were no notifications of congenital syphilis
infant was treated with a ten-day course between 2011 and 2016.6 Marked ethnic
of IV benzylpenicillin, in addition to inequities are evident among congenital
completing seven days of cefotaxime for syphilis cases in New Zealand. This infant
E. coli bacteraemia. He made an excellent was Māori, as were 43% of cases notified to
clinical recovery with jaundice, proteinuria the NZPSU.5
and oedema all resolving by the time of The increase in congenital syphilis mirrors
discharge. His parents were referred to the a dramatic rise in syphilis in adults. There
local sexual health service. were 727 cases in 2019, compared to only
82 in 2013.6 Cases are concentrated in the
Discussion Auckland region7 and upper North Island.
The recognition of congenital syphilis Although the majority of new cases are
remains challenging for clinicians.1–3 Like in men who have sex with men (MSM),
the majority of infants at risk of congenital numbers are rising among women of child-
syphilis, this infant was asymptomatic at bearing age, a particular concern given the
birth. However, he went on to develop potential for mother-to-child transmission.6
life-threatening manifestations in the first This resurgence in syphilis is occurring
weeks of life. Clinicians need to maintain around the globe,8 including other high-
a low index of suspicion for congenital income countries such as Australia, where
syphilis, especially where there is an there was a 146% increase in infectious
absence of documented antenatal serology syphilis cases between 2014 and 2018,9 and
and where a unifying explanation for multi- the United Kingdom.10
organ dysfunction is not apparent. Congenital syphilis is preventable by ante-
This infant’s presentation was initially natal screening and maternal treatment in
thought to be caused by E. coli bacteraemia early pregnancy. The steady rise in national
with a predisposing renal tract abnormality. notifications of congenital syphilis in recent
However, the severity of his multi-organ years highlights critical deficiencies in
involvement with conjugated hyperbili- maternity and sexual health services, partic-
rubinaemia, nephrotic-range proteinuria ularly impacting wahine hapū.
and haematologic abnormalities was not This case highlights the importance of
typical of E. coli infection, particularly as he reviewing maternal serology results as part
required only a brief period of non-invasive of routine newborn management. Urgent
ventilation and inotropic support. The rapid consideration should also be given to the
deterioration with haemodynamic insta- introduction of universal third-trimester
bility and lactic acidosis occurring several syphilis testing, which aligns with recom-
hours after initiation of IV antibiotics may mendations in the recently published New
have been due to the Jarisch-Herxheimer Zealand Sexual Health Society national
reaction, a transient immunologic response guidelines11 and antenatal screening prac-
following initiation of antibiotic treatment tices in other high-incidence populations.
for syphilis that has occasionally been
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NZMJ 22 October 2021, Vol 134 No 1544
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Given the current situation in New Zealand, For women in the childbearing years who
we strongly favour a universal rather than are diagnosed with syphilis, follow-up care
risk-based approach to third-trimester should include explicit guidance regarding
testing. Testing of the mother at delivery the need for antenatal care and testing in
should be universally performed in the future pregnancies, along with identification
absence of antenatal testing, in particular and treatment of sexual partners.
for syphilis, HIV and hepatitis B, where The recently announced health system
immediate clinical management may reforms and Māori Health Authority present
prevent mother-to-child transmission. a timely opportunity to re-prioritise and
Infant testing should be performed at any re-design sexual health and maternity
time upon clinical suspicion of syphilis, services for wahine Māori, in a culturally
as maternal infection may have been responsive manner with barriers to access
acquired after the time of maternal serologic removed.
screening.
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NZMJ 22 October 2021, Vol 134 No 1544
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www.nzma.org.nz/journalclinical correspondence
Figure 1.
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NZMJ 22 October 2021, Vol 134 No 1544
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Competing interests:
Nil.
Acknowledgements:
We thank this infant’s whānau, who consented for the details of the
case to be shared, and the clinicians involved with this baby’s care.
Author information:
Amanda Hall: Trainee Intern, Auckland, University of Auckland.
Danny de Lore: Paediatrician, Department of Paediatrics,
Rotorua Hospital, Lakes District Health Board.
Massimo Giola: Sexual Health and Infectious Diseases Physician,
Rotorua Hospital, Lakes District Health Board.
Tess Edmonds Senior Paediatric Registrar, Department of Paediatrics,
Rotorua Hospital, Lakes District Health Board.
Emma Best: Senior Lecturer in Paediatrics and Paediatric Infectious Diseases Physician,
Department of Paediatrics: Child and Youth Health, University of Auckland,
Starship Children’s Hospital, Auckland District Health Board.
Rachel Webb: Senior Lecturer in Paediatrics and Paediatric Infectious Diseases Physician,
Department of Paediatrics: Child and Youth Health, University of Auckland,
Kidz First Children’s Hospital, Counties Manukau District Health Board,
Starship Children’s Hospital, Auckland District Health Board.
Corresponding author:
Dr Rachel Webb, Senior Lecturer in Paediatrics and Paediatric Infectious Diseases Physician,
Department of Paediatrics: Child and Youth Health, University of Auckland,
Kidz First Children’s Hospital, Counties Manukau District Health Board,
Starship Children’s Hospital, Auckland District Health Board, 0211542226
rwebb@adhb.govt.nz, Rachel.webb@middlemore.co.nz
URL:
www.nzma.org.nz/journal-articles/a-case-of-congenital-syphilis-the-alarming-and-
inequitable-rise-in-congenital-syphilis-in-aotearoa-and-a-call-to-action
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