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Congenital Cytomegalovirus Infection
Michael S. Schimmel
Drora Fisher
Yechiel Schlesinger
CASE PRESENTATION
A term baby was born to a gravida 3 para 2 mother who did
not receive any prenatal care. The mother did not recall any
illness during pregnancy. At birth, meconium-stained amniotic
fluid was noticed. Apgar scores were 4, 7, and 9 at 1, 5, and
10 minutes, respectively. Physical findings included a birth
weight of 2510 g (3% percentile), head circumference of 28.5
cm ( <3% percentile), hepatosplenomegaly of 5 cm below
costal margin, and petechia over the entire thorax. The baby
was severely hypotonic. Pertinent laboratory findings included a
platelet count of 80,000 on the first day of life, which decreased
to 26,000 on the following day. Due to meconium aspiration
syndrome the baby required mechanical ventilation for 24 hours
followed by oxygen supplement through nasal canula for an
additional 2 weeks. Imaging studies included transfontanel brain
sonography and brain computerized tomography (CT). Para-
sagittal transfontanel sonography at the level of the body of the
right lateral ventricle shows severe dilatation and extensive
hyperechogenicity of the ventricular wall (Figure 1 panel A).
Coronal transfontanel sonogram at the level of the third
ventricle shows the same findings, with faint calcifications in
the thalami (Figure 1 panel B). Axial CT of the brain at the
level of the third ventricle shows calcifications lining the wall of
the lateral ventricle. Calcifications are also seen in the basal
ganglia. Axial CT at the level of the bodies of the lateral
ventricles shows extensive dilatation and calcification surround-
ing the ventricles (Figure 2). Brain stem±evoked responses
audiometry and fundoscopy were both normal.
The baby was discharged home at the age of 3 weeks. On physical
examination at discharge he was still hypotonic and microcephalic;
the rest of his examination was within normal limits.
DENOUEMENT AND DISCUSSION
Congenital Cytomegalovirus Infection (CMV)
On the first day of life total IgM was 56 mg/dl and CMV IgM was
positive. Urine culture and polymerase chain reaction (PCR) were
strongly positive for CMV.
We report a neonate with congenital CMV who presented with
strikingly severe brain abnormalities. Congenital CMV infection is the
Department of Neonatology ( M. S. S. ) , Shaare Zedek Medical Center, Jerusalem, Israel;
Department of Infectious Diseases ( Y. S. ) , Shaare Zedek Medical Center, Jerusalem, Israel;
Department of Radiology ( D. F. ) , Shaare Zedek Medical Center, Jerusalem, Israel.
Address correspondence and reprint requests to Michael S. Schimmel, Department of
Neonatology, Shaare Zedek Medical Center, Jerusalem 91031, Israel.
Figure 1. Panel A: Para-sagittal transfontanel sonography at the level of
the body of the right lateral ventricle shows severe dilatation and extensive
hyperechogenicity of the ventricular wall. Panel B: Axial CT of the brain at
the level of the third ventricle shows calcifications lining the wall of the
lateral ventricle. Calcifications are also seen in the basal ganglia.
Congenital Cytomegalovirus (CMV) infection is the most common
intrauterine infection in the developed world. We present a neonate with
severe brain imaging abnormalities due to congenital CMV and a brief
review of the pertinent literature.
Journal of Perinatology 2001; 21:209± 210.
Clinical Perinatal/Neonatal
Casebook
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209
Journal of Perinatology 2001; 21:209 ± 210
# 2001 Nature Publishing Group All rights reserved. 0743-8346/01 $17
www.nature.com/jp
most common intrauterine infection in the developed world. About
40% of babies born to mothers with primary CMV during pregnancy
will be infected at birth, approximately 10% of whom will be
symptomatic. In the symptomatic CMV-infected newborn the most
common clinical manifestations are petechia (76%), hepatosple-
nomegaly (60%), sensorineural hearing loss (58%), microcephaly
(53%), ventriculomegaly and intracranial calcification (30% to
50%).
1
Of the symptomatic babies, 80% will develop severe
neurologic sequelae whereas 10% to 15% of the asymptomatic babies
will have an impaired outcome,
2
most notably hearing loss.
In this baby, the striking brain-imaging manifestations were due
to a CMV infection.
Severe neurocalcifications at birth may follow various etiologies
including vascular anomalies, severe birth asphyxia,
3
and most
commonly, intrauterine infections. The leading infections causing
brain calcifications are CMV, toxoplasmosis, and less frequently,
rubella.
4,5
Imaging findings in CMV include ventricular dilatation and brain
calcifications, predominantly in the periventricular region. In
toxoplasmosis there is also ventricular dilatation, but the calcification
are scattered throughout the brain and are more prominent in the
basal ganglia than in the periventricular region. Congenital rubella is
rare and its calcifications are frequent in the basal ganglia and in the
cortex but usually not in the periventricular region.
4,5
Treatment of symptomatic congenital CMV infection with
gancyclovir have anecdotally been reported by many authors,
5
and is
now being evaluated through an ongoing placebo-controlled study
performed by the NIAID.
7
Initial results are encouraging,
6,7
but firm
recommendations regarding this experimental treatment cannot be
made at this point. In the present case, the parents chose not to treat
the infant.
References
1. Brown HL, Abernathy MP. Cytomegalovirus infection. Semin Perinatol
1998;22:260± 266.
2. Stagno S. Cytomegalovirus. In: Remington JS, Klein JO, editors. Infectious
Diseases of the Fetus and Newborn Infant. 4th ed. Philadelphia, PA: WB
Saunders; 1995. p. 320.
3. Boppana S, Pass RF, Britt WS, et al. Symptomatic congenital cytomegalovirus
infection: neonatal morbidity and mortality. Pediatr Infect Dis J 1992;11:93 ±99.
4. Govaert P, de Vries L. An atlas of neonatal brain sonography. Clin Dev Med
1997;319± 321.
5. Barkovich JA. Infections of the nervous system, congenital infections. In:
Pediatric Neuroimaging. 3rd ed. Philadelphia, PA: Lippincott Williams &
Wilkins; 2000. pp. 715± 724.
6. Nigro G, Scholtz, Bartmann U. Ganciclovir therapy for symptomatic congenital
cytomegalovirus infection in infants: a two -regimen experience. J Pediatr
1994;124:318± 322.
7. Whitley RJ, Cloud G, Gruber W, et al. Ganciclovir treatment of symptomatic
congenital Cytomegalovirus infection: results of a phase II study, National
Institute of Allergy and Infectious Diseases Collaborative Antiviral Study. J
Infect Dis 1997;175:1080± 6.
Figure 2. Panel A: Coronal transfontanel sonogram at the level of the
third ventricle shows the same findings, with faint calcifications in the
thalmi. Panel B: Axial CT at the level of the bodies of the lateral ventricles
shows extensive dilatation and calcification surrounding the ventricles.
Schimmel et al. Congenital Cytomegalovirus Infection
210
Journal of Perinatology 2001; 21:209 ± 210