Gestational Lyme Disease as a Rare Cause of Congenital Hydrocephalus
Gülzade ÖNK1, Ceyda ACUN1, Murat KALAYCI2, Ferda ÇAĞAVİ2, Bektaş AÇIKGÖZ2, H. Alper TANRIVERDİ3
1Department of Pediatrics, Faculty of Medicine, Zonguldak Karaelmas University, Zonguldak, Turkey
2Department of Neurosurgery, Faculty of Medicine, Zonguldak Karaelmas University, Zonguldak, Turkey
3Department of Obstetrics and Gynecology, Faculty of Medicine, Zonguldak Karaelmas University, Zonguldak, Turkey
Received 19 October 2004; received in revised form 20 December 2004; accepted 21 January 2005
Lyme disease is an inflammatory disorder caused by infection with Borrelia burgdorferi. In this report a rare case of a girl surviving intrauterine Lyme disease, who subsequently developed triventricular hydrocephalus and aquaductus cerebri stenosis was presented. In earlier cases modern imaging techniques have not been used. In our patient, intrauterine magnetic resonance imaging technique has been helpful in the diagnosis and management of the therapy.
Despite vaccines, new antimicrobials, and improved hygienic
practices, congenital infections remain an important cause of
death and long-term neurologic morbidity among infants
worldwide. Important agents include Toxoplasma gondii,
cytomegalovirus, treponema pallidum, herpes simplex virus
types 1 and 2, and rubella virus. In addition, several other
agents, such as the varicella zoster virus, human parvovirus
B19, and Borrelia burgdorferican potentially infect the fetus
and cause adverse fetal outcomes (1).
The clinical note presents the prenatal sonographic diagnosis
(LD), confirmed by in utero magnetic resonance imagin
(MRI), in an apparently uneventful pregnancy.
An 18-year-old nullgravida at 34 weeks of gestation was
admitted to our hospitals pregnancy department with an
uneventful pregnancy. Prenatal sonography at the time
showed enlargement of the lateral ventricles that meaned
congenital hydrocephalus. A part from head circumference
the fetal maturity was appropriate for gestational age in the
ultrasonography. In utero magnetic resonance imaging
(MRI) scans at 35 weeks of gestation demonstrated triven-
tricular hydrocephalus (Figures 1, 2). Borrelia burgdorferi
IgM antibodies in maternal blood was positive, 27.5 RU
(0-20) and no other positivity was found in the tests which
made to explore the cause of congenital hydrocephalus. The
mother had neither a clinical infection nor a history of tick bite
during pregnancy, so that she did not recieve any antibiotics.
Elective cesarean delivery was performed due to the associated
craniomegaly at 36 weeks of gestation. The infant girl had
a born weigth of 2920 gr (10-25 percentiles) and a head
circumference of 37 cm (>97 percentiles). Preoperative
MRI revealed triventricular hydrocephalus and aquaductus
cerebri stenosis, transepandymal cerebrospinal-fluid leakage.
A ventriculoperitoneal shunt was placed on day 2 of her life. She
was discharged on day 5 with her mother. After the delivery
in Western-blot analysis high-specific antibodies were found
in maternal blood and Borrelia burgdorferi non-specific
antibodies were found in neonatal blood. Species-specific and
highly spesific antigen with the molecular weigth of 31 kDa
was present in maternal IgG Western-blot analysis. A genus-
specific antigen with the molecular weigth of 41 kDa and
non-specific antigen with the molecular weigth of 75 kDa
were present in infant IgM Western-blot analysis. Up to date
she and her mother had an uneventful follow-up.
Lyme disease is a multisystem inflammatory syndrome
caused by infection with the tick-borne spirochete Borrelia
burgdorferi. Although this syndrome has important implica-
tions for human pregnancy, little is known about gestational
infection with Borrelia burgdorferi (2).
It is known that transplacental transmission of the spirochete
from mother to fetus is possible. Many studies have associated
gestational LD with fetal death, hydrocephalus, cardiovascular
anomalies, neonatal respiratory distress, hyperbilirubinemia,
intrauterine growth retardation, cortical blindness, sudden
infant death syndrome, and maternal toxemia of pregnancy (3).
The serologic evidence of Borrelia burgdorferi non-specific
antibodies in a neonate with a MRI image of congenital
hydrocephalus and maternal infection during pregnancy
proved by Borrelia burgdorferi high-specific antibodies
were crucial to diagnosing LD consisting of congenital