Congenital cutaneous candidiasis and candidemia in a preterm infant: A case report

Abstract

We report a case of congenital cutaneous candidiasis (CCC) progressing to candidemia in an extremely low birth weight infant. A high level of suspicion concerning candida sepsis as well as timely diagnostic work-up and treatment can be lifesaving when a preterm infant presents with lesions suggestive of congential cutaneous candidiasis.

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CASE REPORT
Congenital cutaneous candidiasis and candidemia in a
preterm infant: A case report
Sarah Oberhauser∗1, Sebastian Böhm1, Anita Niederer2, Bjarte Rogdo1
1Pediatric and Neonatal intensive care unit, Children’s Hospital of Eastern Switzerland, St. Gallen, Switzerland
2Department of Infectiology, Children’s Hospital of Eastern Switzerland, St. Gallen, Switzerland
Received: October 30, 2016 Accepted: December 11, 2016 Online Published: January 19, 2017
DOI: 10.5430/crcp.v4n1p68 URL: http://dx.doi.org/10.5430/crcp.v4n1p68
ABS TR ACT
We report a case of congenital cutaneous candidiasis (CCC) progressing to candidemia in an extremely low birth weight infant. A
high level of suspicion concerning candida sepsis as well as timely diagnostic work-up and treatment can be lifesaving when a
preterm infant presents with lesions suggestive of congential cutaneous candidiasis.
Key Words: Candidemia, Congenital cutaneous candidiasis, Preterm infant, Fluconazole, Leukemoid reaction
1. INTRODUCTION
Congenital cutaneous candidiasis (CCC) is a rare condition
in the neonate associated with chorioamnionitis due to Can-
dida spp.
[1]
Patients typically present on the first day of life
(DOL) with skin lesions of various morphologies. A progres-
sion to systemic candida infection occurs in up to 50% of
CCC cases in extremely low birth weight (ELBW) infants.
[2]
We report a case of candidemia in an ELBW infant with con-
gential cutaneous candidiasis. It is pointed out why a high
level of suspicion concerning candida sepsis can be lifesav-
ing when a preterm infant presents with lesions suggestive of
CCC at birth and how antifungal treatment should be done.
2. CASE PRESENTATION
A 17-year-old primigravida with an otherwise uncomplicated
pregnancy presented at 24+
6
weeks of gestation with signs of
preterm labour. She was given tocolytic therapy and antenatal
steroids as well as antibiotic treatment with cefuroxime. Due
to suspected chorioamnionitis and signs of foetal distress,
caesarean section was performed at 25+
5
weeks of gestation.
Amniotic fluid was green and foul smelling. The male infant
had a birth weight of 920 g. Apgar scores were 5, 5 and 7 at
1, 5 and 10 minutes respectively. Due to respiratory distress
and high oxygen requirements, he was immediately intubated
and treated with surfactant. Empiric antibiotic treatment with
amoxicillin and gentamicin was started after birth because
of suspected neonatal infection. In the following hours, he
developed tachycardia and fever. On physical examination
white papules were noticed on the face, the sole of feet as
well as in the axillary and inguinal regions (see Figures 1-2).
The initial white blood count revealed leucocytosis (41 G/L)
and mild thrombocytopenia (100 G/L). C-reactive protein
was 14 mg/L. A leukemoid reaction with a maximum leuco-
cyte count of 103 G/L developed within the first days of life.
Blood glucose levels were elevated with a maximal value of
25 mmol/L, so that insulin therapy was required (see Figure
3).
∗Correspondence: Sarah Oberhauser, M.D.; Email: sarah.oberhauser@kispisg.ch; Address: Claudiusstrasse 6, 9000 St. Gallen, Switzerland.
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Figure 1.
White papules on the face, upper trunk and axillae
Figure 2. White papules on sole of the feet
Figure 3. Leucocyte count and blood glucose levels day
1-31, and Insulin therapy
Due to persisting signs of infection and growth of C. albicans
in amniotic fluid and maternal vaginal swabs, fluconazole
was added to the antimicrobial therapy on the 2
nd
DOL. The
rash was fading and disappeared shortly after. Ultrasound
screening for systemic candidiasis the following day was neg-
ative for renal or cerebral abscess and endophthalmitis was
excluded by ophthalmologic examination. Urine cultures
from the 3
rd
DOL showed growth of C. albicans 3 days
later and blood culture from the 6
th
DOL grew C. albicans
after 24 hours. Culture and polymerase chain reaction (PCR)
taken from cerebrospinal fluid (CSF) on the 11
th
DOL were
both negative for C. albicans. Despite systemic antimycotic
treatment, the patient deteriorated with respiratory failure, re-
quiring high frequency oscillation ventilation on the 7
th
DOL
and blood and urine cultures still grew C. albicans, without
bacterial growth. Susceptibility testing of C. albicans to
fluconazole revealed a high minimal inhibitory concentra-
tion (3
µ
g/ml),
[3]
and treatment was changed to liposomal
amphotericin B. Because of persisting candidemia 48 hours
later therapy was again changed to micafungin on the 9
th
DOL. Subsequently leucocyte count and blood glucose levels
normalized over the next days (see Figure 3). Blood cultures
remained negative from the 12
th
DOL and clinical condition
improved markedly. Cerebral ultrasound and ophthalmologic
follow up exams remained normal. The antifungal treatment
with micafungin (10 mg/kg/day) was stopped after 3 weeks.
Placenta histology showed signs of acute chorioamnionitis
with omphalovasculitis and funisitis with ubiquitous pres-
ence of fungal elements (see Figure 4).
Figure 4. Acute fungal funisitis with evidence of hyphea
and spores of candida species (PAS-staining, 20×
augmentation)
The neonatal course was further complicated by a cen-
tral catheter associated gram-negative sepsis with Serratia
marcescens at 3 weeks of age, as well as bronchopulmonary
dysplasia.
Despite this complicated neonatal course, neurodevelopmen-
tal examination at 3 months of corrected age revealed normal
motor and mental development. Later, at the corrected age
of 23 months the patient showed a delay of mental develop-
ment appropriate to a child of 16 months and the linguistic
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competences were appropriate to a child of 13 to 15 months.
Motor skills were within normal ranges for corrected age.
3. DISCUSSION
C. albicans is a common vaginal pathogen found in up to 30%
of pregnant women, however Candida is recovered by culture
from less than 1% of placentas.
[2, 4, 5]
Known risk factors for
CCC are intrauterine devices (IUD) during early pregnancy
and cervical cerclage later in pregnancy.
[2]
Ascending infec-
tion may occur either with rupture of membranes or through
intact membranes resulting in whitish plaques on membranes
and umbilical cord along with skin lesions.
[6]
Due to im-
maturity of the immune system and of the mucocutaneous
barrier, ELBW infants are at substantially higher risk for sys-
temic involvement than term infants. Some authors propose
to consider CCC in preterm infants as an invasive Candida
infection and to start systemic treatment as soon as diagnosis
is suspected.[7]
In our case, placenta histology showed acute fungal
chorioamnionitis, confirming the suspected diagnosis. There
was no history of IUD, cervical cerclage or premature rupture
of membranes. The infant had multiple skin lesions at birth
as well as clinical signs and changes in white blood count
consistent with systemic infection. Therefore, antifungal
treatment was started on 2
nd
DOL, when cultures of amni-
otic fluid grew C. albicans. Routine antifungal prophylaxis
is not given in our centre, owing to very low rates of neonatal
fungal infection.
Neonates with clinical signs of systemic fungal disease and
cutaneous lesions at birth should undergo a complete workup
for systemic fungal disease, including renal, ophthalmologic
and central nervous system evaluation.
[1]
In our patient C.
albicans grew in blood and urine, but no other organ system
was involved. Observed hyperglycaemia can be interpreted
as a marker for invasive fungal infection.[8]
Retrospectively our patient could possibly have benefited
from a higher dosing regimen of fluconazole, especially in
view of the high MIC of C. albicans for fluconazole. Cur-
rently recommended dosing regimens for invasive candida
infection are considerably higher than previously used regi-
mens (6-12 mg/kg every 72 hours).
[9]
Wade et al. show that a
therapeutic concentration of fluconazole in premature infants
with invasive candidiasis requires substantially higher dosing
of 12 mg/kg every 24 hours,
[10]
and loading dose of 25 mg/kg
is recommended to achieve therapeutic levels faster.[9, 10]
Invasive candida infections in ELBW infants are associated
with high mortality rates and increased risk for poor neu-
rodevelopmental outcome.
[11]
In our patient CCC rapidly
progressed to candida sepsis. After changing antifungal ther-
apy to micafungin, the clinical situation improved rapidly.
At 23 months of corrected age, the patient showed moderate
delays in cognitive development, with motor skills within
normal range for age. Although the extreme prematurity
must be considered the main risk factor for development
delays, the two episodes of sepsis contributed to a higher risk
of neurodevelopmental delays in our patient.
ACKNOWLEDGEMENTS
Regulo Rodriguez, senior consultant, Institute of Pathol-
ogy, Kantonsspital St. Gallen, for the appropriation of the
histopathological pictures. Christian Kahlert, senior con-
sultant, Department of Infectiology, Children’s Hospital St.
Gallen, for his participation at this article. Michael von
Rhein, senior consultant, Department of Pediatric Develop-
ment, Kantonsspital Winterthur, for the disclosure of the
follow-up examination.
CON FLI CT S OF INTEREST DISCLOSURE
The authors declare no conflicts of interest.
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