PEDIATRICS Vol. 103 No. 1
January 1999,
p. e4
ELECTRONIC ARTICLE:
Preschoolers With Syphilis
Cindy W. Christian*,
Jane Lavelle
, and
Louis M. Bell, §
From the Divisions of * General Pediatrics, Emergency
Medicine, and § Immunologic and Infectious Diseases, Children's
Hospital of Philadelphia, University of Pennsylvania School of
Medicine, Philadelphia, Pennsylvania.
 |
ABSTRACT |
Syphilis in preschoolers is rarely described in
current medical literature, despite the rise in syphilis in both the
adult and the pediatric populations during the past decade. Since that time, 3 children between 3 and 4 years of age have been diagnosed with
syphilis at the Children's Hospital of Philadelphia. The presentations
and clinical manifestations of syphilis in these 3 children are
described, and the difficulty in identifying the source of infection is
discussed. The presentations of these children included nephrosis and
secondary syphilis, the corymbiform and palmar rash of syphilis, and
subtle signs of late congenital infection in an otherwise asymptomatic
child. One child had documented congenital infection, 1 had probable
congenital infection that went untreated, and 1 did not have
appropriate neonatal testing documented. None of the children gave a
verbal history of sexual abuse, although it is likely that all three
cases resulted from sexual abuse. The evaluation of preschool children
with syphilis is confounded by the interpretation of acquired infection
in consideration of a history of possible or documented congenital
disease. The assessment is complicated further by problems with
recognition of clinical disease, the inability of young children to
provide a history, prenatal and neonatal testing methods used, changes
in treatment recommendations made during the past decade, and
inadequate follow-up to document cure of congenitally infected infants.
With the increase in syphilis seen in recent years, physicians are more
likely to encounter preschoolers with syphilis. Our ability to document acquired infection, however, is hampered by the difficulties
encountered in following recommended guidelines for evaluation and
follow-up and by limitations in interviewing young victims of sexual
abuse, which may impair our ability to protect children from additional harm. Understanding the pathophysiology and progression of this disease
remains challenging even in this modern era.
Key words:
syphilis,
child abuse.
The incidence of both acquired and congenital syphilis has
risen significantly during the last decade.1,2 Since that
time, 3 children between 3 and 4 years of age have been diagnosed with
syphilis at the Children's Hospital of Philadelphia. Determining the
etiology of their infection has proven challenging. Because of the
social and legal implications of syphilis in a child, identifying the
mode of infection is of utmost importance. These cases illustrate the
manifestations of syphilis in preschool children, which have been
described infrequently in current medical literature. In addition, the
dilemmas encountered in evaluating young children with syphilis are
presented.
| |
CASE 1 |
A 3-year-old girl was referred to our hospital for evaluation of
nephrosis. She was born at 25 weeks' gestation to a 24-year-old woman
who received no prenatal care. Maternal and infant rapid plasma reagin
(RPR) testing was not obtained at the time of delivery. Five
months before referral, the child developed a red and white papular
lesion on the labia majora. The child was evaluated by her primary care
physician, and the lesion resolved after 1 week. Four months later this
lesion returned. Subsequently, the child developed progressive edema,
diarrhea, cough, and fever. Urinalysis performed by her primary care
physician was consistent with nephrosis. The child lived with her
mother, grandmother, grandmother's boyfriend, older sister, and a few
family friends. Because the mother was drug-addicted, the child's
primary caregiver was her grandmother. There were no behavioral changes
or concerns identified by the family.
Physical examination at the time of referral revealed a mildly
ill-appearing child. She had generalized edema, cervical adenopathy, bilateral rales, and ascites. She had no hepatosplenomegaly. Her genital examination revealed a 1 × 1.5-cm painless, slightly
erythematous papule of the right posterior labia majora with
surrounding hyperpigmentation (Fig 1). There
was no vaginal discharge. The hymen was annular, without transections
or notching. Two small synechiae were present from the hymen to
vestibule at the 2 and 4 o'clock positions. The anal rugae were
slightly edematous, but no injuries were identified. Urinalysis
revealed hematuria and proteinuria and a 12-hour urine collection
demonstrated 1.4 g/M2/d of protein. Dark-field examination
of the labial lesion was negative for Treponema pallidum.
RPR results were positive at 1:32 and microhemagglutination assay for
antibodies to T pallidum (MHA-TP) was reactive. The
cerebrospinal fluid (CSF) profile was normal, and the CSF venereal
disease research laboratory (VDRL) test result was negative. Cultures
for Neisseria gonorrhoeae and Chlamydia trachomatis from the vagina and rectum were negative.
Results of human immunodeficiency virus (HIV) serology studies were
negative at the time of admission and 4 months later. After
consideration of the clinical findings and laboratory data, acquired
secondary syphilis with associated glomerulonephritis was diagnosed.
Treatment with 3 weekly doses of benzathine penicillin, 50 000 U/kg,
was completed, and her nephrosis resolved. The mother and child were interviewed by the physician and social worker on the hospital's child
protection team. The mother reported a history of gonorrhea and
syphilis, both of which were treated previously. The mother's RPR
finding at the time of her daughter's infection was not documented. The child gave no history of sexual abuse, in part because her verbal
skills were not well-developed. A report of suspected child abuse was
made to the county child welfare agency. Because the identity of the
perpetrator was not known at the time of diagnosis, the child was
discharged into foster care. Additional interviews were conducted by
county social workers, none of which revealed the identity of a
perpetrator. The child was returned to her mother's care within weeks
of discharge from the hospital. The identity of the perpetrator was
never known.
| |
CASE 2 |
A 31/2-year-old boy was referred to our emergency
department by his primary care physician for additional evaluation of a
rash. The child had been well until 1 week before, when he developed a
mildly pruritic rash on his feet that spread to his extremities and
trunk. He was otherwise well. His physical examination was notable for
red-brown papules, 3 to 10 mm in diameter, which were scattered on the
trunk and concentrated on the palms, soles, lower legs, and groin (Fig
2). There also was a 2.5-cm oval, slightly
erythematous plaque on the child's back. There was no hepatosplenomegaly or adenopathy. His genital examination revealed the
rash on his penis and scrotum, but no signs of injury. The RPR finding
was positive at 1:256 and fluorescent treponemal antibody absorption
(FTA-Ab) was reactive, and the family was asked to return for
treatment. On return, both mother and child denied a history of abuse
or a previous genital rash. However, the mother revealed that she had
been treated for syphilis at the time of this child's birth. The child
was referred to the hospital's child protection team for additional
evaluation and treatment. The child lived with his mother, her
boyfriend, the maternal grandmother, and his siblings. There were no
behavioral concerns noted by the mother. The child was interviewed
alone by the protection team's social worker. He did not reveal a
history of sexual abuse. A review of the mother's medical records
revealed that she was RPR-negative in 1985 and 1989, but at the time of
the patient's birth in 1990, she had a positive RPR titer of
1:2 with a reactive FTA-Ab. This infant had a negative RPR result by
cord blood study at the time of his birth and received no treatment.
Documentation of the mother's outpatient treatment was not found.
Interestingly, the mother's RPR titer again was positive at 1:16 1 year preceding this child's presentation, at which time she was
treated with three doses of benzathine penicillin. The mother's RPR
titer was not documented at the time of the child's diagnosis.
A search for late manifestations of congenital syphilis, including long
bone films; CSF analysis; and ophthalmologic, dental, and audiologic
evaluations, was conducted. The lumbar puncture was normal, and the CSF
VDRL was negative. Long bone radiographs and a panorex of the mouth
were normal. Cultures for N gonorrhoeae and C
trachomatis were negative. HIV serology was negative. Treatment with three weekly doses of 50 000 U/kg of intramuscular benzathine penicillin was completed. A report of suspected sexual abuse was made
to the county's child welfare agency. The child was interviewed again
by the county social workers and, again, did not reveal a history of
abuse. The investigation failed to identify additional evidence of
sexual abuse and was determined unfounded. The etiology of the
infection was never understood clearly. Although the maternal history
of syphilis at the time of the child's birth makes latent congenital
syphilis an (unlikely) possibility, it was felt that this case most
probably represented secondary acquired syphilis and sexual abuse.
| |
CASE 3 |
A 4-year-old was referred to our hospital for evaluation and
treatment of syphilis. She had been born at term to a 26-year-old woman
with no prenatal care and a history of cocaine and alcohol abuse. The
mother was treated for syphilis 1 year before the birth of this child.
At birth, both the mother and the infant had a negative RPR titers. At
this time, the mother was treated for fever with 10 days of intravenous
antibiotics. At 5 weeks of age, the infant developed nasal congestion,
tachypnea, and a protuberant abdomen. Her physical examination was
significant for cervical and axillary adenopathy, hepatosplenomegaly,
and palmar rash. An RPR titer was positive at 1:252. CSF profile was
normal, with a positive VDRL finding. The child was diagnosed with
congenital neurosyphilis and was treated with a 10-day course of
50 000 U/kg/d of intravenous penicillin G. The child was discharged
from the hospital after treatment and was lost to follow-up.
Four years later, an RPR was obtained during a well-child examination,
after a caregiver revealed a possible history of congenital syphilis.
The RPR titer was positive at 1:512, and the child was admitted for
treatment. The physical examination was notable for mild frontal
bossing and mild hepatomegaly. The genital examination was normal. CSF
profile was normal, and the result of CSF VDRL tests was negative. The
hospital's child protection team was consulted. There was no known
history of sexual abuse, and the child failed to reveal a history of
abuse during hospital interviews. Genital cultures for N
gonorrhoeae and C trachomatis, hepatitis B, and HIV
serologies were negative. Results of eye and hearing examinations and
long-bone films were normal. A panorex revealed mild tapering of the
incisors, suggestive of Hutchinson teeth. Because of her history of
syphilis and her previous loss to follow-up, the child was treated with
a 10-day course of intravenous penicillin G at 300 000/U/kg/d. The
diagnosis of suspected sexual abuse resulted in a report to the county
child welfare agency. The child never revealed a source of infection,
and the diagnosis of sexual abuse was never confirmed. On follow-up,
the child remained well with declining RPR titers.
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DISCUSSION |
Syphilis in preschoolers is rarely described in modern medical
literature. These three cases represent the difficulty in identifying the source of infection in young children whose verbal skills are
immature and whose medical history includes or may include congenital
infection.
The diagnosis of acquired syphilis and sexual abuse in the first case
was based on the clinical history, results of physical examination, and
natural history of syphilitic nephropathy. The child was seen with a
syphilitic chancre and again with resolving condyloma lata that
initially went undiagnosed. Because syphilis is an uncommon infection
in prepubertal children, physicians may fail to consider the diagnosis
in a young child even when the manifestations are classic for the
disease.3 Glomerulonephritis is an uncommon but
well-described manifestation of both congenital and acquired
syphilis,4,5 and results from the deposition of treponemal
antigen-antitreponemal antibody complexes in the
glomerulus.6 In congenital syphilis, nephrosis develops as
a manifestation of early disease, usually presenting in the first few
months of life. Conversely, nephropathy is a manifestation of secondary
syphilis in older patients with acquired syphilis. Although rare in
prepubertal children, syphilitic nephropathy has been reported in a
10-year-old child with acquired syphilis.7 Our patient had
a history of a recurrent genital lesion that was consistent both in
appearance and in timing with a primary syphilitic chancre followed by
condyloma lata. The nephrotic syndrome was related temporally to the
recurrence of the genital rash, consistent with acquired secondary
syphilis.
Syphilis remains an uncommon complication of child
abuse.8,9 Ingram et al8 tested 1263 prepubertal children being evaluated for sexual abuse for syphilis and
found disease in 1 patient. This was an asymptomatic 1-year-old child whose parents both tested positive for syphilis. Horowitz and Chadwick9 reported secondary syphilis in only two
5-year-old children of >6000 children evaluated for sexual abuse. In
these reported cases and in our preschool children, a history of sexual abuse was not obtained. The diagnosis of child sexual abuse usually relies on a clear history of sexual contact provided by the child. This
can prove difficult when the victim is quite young. Although the
finding of genital trauma or a sexually transmitted disease at times
can be diagnostic of sexual abuse, identifying the source of the abuse
and protecting the child from additional molestation can prove
difficult without a history of sexual contact.9 The
American Professional Society on the Abuse of Children has published
practice guidelines for the psychosocial evaluation of suspected sexual
abuse in young children.10 These guidelines recommend that
a series of forensic interviews be conducted by experienced evaluators
who have advanced training in child development and child sexual abuse.
Although additional forensic interviewing may have revealed a history
of abuse, lower rates of disclosure have been reported for children
younger than 5 years of age.11,12
The source of infection in case 2 was never understood clearly. This
child was 31/2 years old when he presented with the
papulosquamous rash of syphilis. This rash is typically seen in early
congenital syphilis or in acquired secondary syphilis. The year before
this child's birth, the mother's RPR finding was negative. At the
time of delivery, the mother had a reactive RPR and FTA-Ab and was in
either primary or secondary syphilis. Vertical transmission rates are
extremely high (70%-100%) in the first 4 years after maternal
acquisition of disease.13 Furthermore, for mothers with
untreated secondary syphilis, virtually all newborns are
infected.13 Cord blood was used to screen this infant for
disease. This method is associated with false-negative
results14 and is not recommended by the American Academy
of Pediatrics.15 However, regardless of the infant's
serologic findings, the child had met criteria for a diagnosis of
presumed congenital syphilis in that he was born to a mother who had
evidence of syphilis and who had not received adequate treatment. Thus,
it is probable that congenital infection went undiagnosed and
untreated.
A search for late manifestations of congenital syphilis was negative.
Late manifestations of congenital infection, however, are found in only
40% of untreated patients,16 and some, such as keratitis
and joint disease, typically appear in later childhood.15
The child was never treated for presumed congenital infection. Although
untreated congenital syphilis offers some protection against subsequent
infection, the protection is not complete and also may moderate the
symptoms of subsequent exposure.17 If the child's illness
had progressed to latent congenital infection before diagnosis, the
prolonged asymptomatic period before onset of dermal findings would be
difficult to explain. It has been postulated by others that antibiotic
treatment for common childhood illnesses may result in inadvertent
partial treatment of congenital syphilis, greatly altering the
manifestations of late disease.18 The possibility of
acquired secondary syphilis was strongly considered. Again, however,
the child failed to disclose a source of infection, both during the
medical evaluation and during subsequent interviews with child welfare
social workers. Despite efforts to understand this child's
presentation, the case remains an enigma.
The third case highlights the difficulty in identifying the source of
infection when response to treatment of congenital disease is
documented inadequately. Although recommendations for follow-up are
widely available,15 little is written about follow-up rates and the natural course of disease in the pediatric population. The infant originally presented at 5 weeks of age with the classic stigmata of early congenital syphilis. She received the standard antibiotic therapy for congenital neurosyphilis at that
time.19 That year, the Centers for Disease Control amended
the set of treatment guidelines for congenital syphilis, recommending
the current higher dose of 100 000 to 150 000 U/kg/d with aqueous crystalline penicillin G.20 The central nervous system may
harbor the organism and serve as a source of relapse because of
inadequate CSF penicillin levels achieved when lower-dose penicillin is
used.21 The child in this case had a positive CSF VDRL
result as a neonate and was treated with lower-dose penicillin. Although it is possible that her treatment was incomplete, the fact
that the central nervous system had been cleared of infection supports
adequate neonatal therapy. Although the findings of frontal bossing and
possible Hutchinson teeth support a diagnosis of late congenital
disease, treatment of congenital infection does not protect against the
development of Hutchinson teeth completely.22 Once again,
efforts to understand the etiology of the child's infection failed.
The possibility of acquired disease and sexual abuse was considered,
but interviews by the child protection physician and child welfare
agency staff did not reveal a history of sexual abuse.
In conclusion, the evaluation of the preschool child with syphilis is
confounded by the interpretation of acquired infection in consideration
of a previous history of possible or documented congenital disease. The
evaluation is complicated further by problems with recognition of
clinical disease, the inability of young children to provide a history,
prenatal and neonatal testing methods used, changes in treatment
recommendations, and inadequate follow-up to document cure of
congenitally infected infants. With the increase in syphilis seen in
recent years, physicians are more likely to encounter preschoolers with
syphilis. It is likely that these three cases resulted from sexual
abuse. Our ability to document acquired infection, however, is hampered
by the difficulties encountered in following recommended guidelines for
evaluation and follow-up and by limitations in interviewing young
victims of sexual abuse, which may impair our ability to protect
children from additional harm. Understanding the pathophysiology and
progression of this disease remains challenging even in this modern
era.
| |
FOOTNOTES |
Received for publication Aug 25, 1997; accepted Jul 29, 1998.
Address correspondence to Cindy W. Christian, MD, Division of
General Pediatrics, Children's Hospital of Philadelphia, Rm 2416, 34th
St at Civic Center Blvd, Philadelphia, PA 19104.
| |
ABBREVIATIONS |
RPR, rapid plasma reagin;
CSF, cerebrospinal fluid;
VDRL, venereal disease research laboratory (test);
HIV, human
immunodeficiency virus;
FTA, fluorescent treponemal antibody
absorption;
MHA-TP, microassay for antibodies to Treponema
pallidum..
| |
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Pediatrics (ISSN 0031 4005). Copyright ©1999 by the American Academy of Pediatrics
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Abstract
Discussion
