Congenital Syphilis and the Prozone Phenomenon: A Case Study.

BACKGROUND: There is a global rise in maternal syphilis and infants born with congenital syphilis. CLINICAL FINDINGS: This case report presents a 32-week gestation premature male infant with initial clinical findings significant for respiratory failure as well as blueberry muffin spot rash and hepatosplenomegaly, which are consistent with congenital syphilis. PRIMARY DIAGNOSIS: This case study is unique in that the diagnosis of congenital neurosyphilis was demonstrated despite negative maternal serology because of the prozone phenomenon. This phenomenon refers to an overwhelming acute infection that interferes with testing by antibody flocculation or clumping of antibodies, causing a false-negative result and allows an infection such as syphilis to go undetected during pregnancy when Venereal Disease Research Laboratory test is performed. INTERVENTIONS: The infant received a 10-day course of aqueous penicillin G 150,000 U/kg every 8 hours per Centers for Disease Control and Prevention and Redbook recommendations for congenital neurosyphilis. OUTCOMES: The infant was discharged home on day of life 38 and was followed as an outpatient with serial rapid plasma reagin titers confirming adequate treatment of congenital neurosyphilis. He was also referred to the high-risk infant clinic to monitor for developmental milestones secondary to prematurity and central nervous system infection. PRACTICE RECOMMENDATIONS: Providers within the neonatal setting should consider early congenital syphilis testing on infants with similar clinical presentation regardless of negative maternal testing.

A Brief Resolved Unexplained Event and Congenital Neurosyphilis.

Brief resolved unexplained event (BRUE) is a common pediatric problem that presents to ambulatory and emergency settings. Infants presenting with a BRUE can be separated into low- and high-risk groups per recent guidelines. Most low-risk infants who present with a BRUE can be discharged home with anticipatory guidance and education provided to the caregivers; however, high-risk infants should undergo further testing and observation to determine the cause of their event. Congenital neurosyphilis can be a rare cause of a BRUE. Therefore, high-risk infants with a BRUE should have evaluations focused on potential diagnosis supported by the patient's history and physical examination. [Pediatr Ann. 2017;46(2):e61-e64.].

Characterization and six-month follow-up on a cohort of newborns with congenital syphilis.

INTRODUCTION: Congenital syphilis is a preventable disease. However, the incidence in Colombia has increased and reached the figure of 3.28 cases per 1,000 live births in 2011.  OBJECTIVE: To characterize newborns with congenital syphilis and describe follow-up over 6 months following their diagnosis and treatment.  MATERIALS AND METHODS: Between August, 2011, and February, 2012, in Hospital La Victoria, sede Instituto Materno Infantil, newborns with congenital syphilis were identified and treated. Clinical and laboratory follow-up was carried out for six months until it was determined that the disease was cured. The analyzed variables included patients' clinical and laboratory characteristics.  RESULTS: In this period, we identified 29 cases that met the criteria of congenital syphilis, with a frequency in the institution of one case per 69 births. There was one stillbirth and one neonatal death, four were premature, and five had growth restriction. Of the 28 live births, 15 were asymptomatic. There were two cases with neurosyphilis, representing 15% of symptomatic newborns. Follow-up was done on 18 out of 27 children (66.6%), including most symptomatic cases and the two cases of neurosyphilis. All cases showed the expected decrease in VDRL titers without clinical sequelae, meeting the criteria of being cured.  CONCLUSIONS: A high incidence of the disease, failure to prevent it, varied expression in its presentation, and the effectiveness of treatment in the neonatal period were evidenced in this study.

Caracterización y seguimiento durante seis meses de una cohorte de recién nacidos con sífilis congénita / Characterization and six-month follow-up on a cohort of newborns with congenital syphilis

Introducción. La sífilis congénita es una enfermedad prevenible cuya incidencia en Colombia ha aumentado, alcanzando la cifra de 3,28 casos por 1.000 nacidos vivos en el 2011. Objetivo. Caracterizar a los recién nacidos con sífilis congénita y describir su diagnóstico y tratamiento durante el seguimiento de seis meses. Materiales y métodos. En el periodo comprendido entre agosto de 2011 y febrero de 2012 en el Hospital La Victoria, sede Instituto Materno Infantil, se identificaron y trataron los recién nacidos con sífilis congénita, y se hizo seguimiento clínico y de laboratorio hasta la curación de la enfermedad. Las variables analizadas incluyeron las características clínicas y de laboratorio. Resultados. En este periodo se diagnosticaron 29 pacientes que cumplían con los criterios de caso de sífilis congénita, cuya frecuencia en la institución era de un caso por cada 69 nacimientos. Hubo un mortinato y una muerte neonatal, cuatro recién nacidos fueron prematuros y cinco presentaron restricción en el crecimiento. De los 28 recién nacidos vivos, 15 fueron asintomáticos. Se presentaron dos casos con neurosífilis, es decir, 15 % de los sintomáticos. Se hizo seguimiento a 18 de los 27 niños (66,6 %), incluidos la mayoría de los sintomáticos y los dos casos con neurosífilis; la disminución esperada de los títulos en la prueba del Venereal Disease Research Laboratory , VDRL, se presentó en todos los pacientes, y no hubo secuelas clínicas, con lo que se cumplieron los criterios de curación. Conclusiones. Se evidenció una gran incidencia de la enfermedad, fallas en su prevención, una variada expresión en su presentación y la efectividad del tratamiento en el periodo neonatal.

Introduction: Congenital syphilis is a preventable disease. However, the incidence in Colombia has increased and reached the figure of 3.28 cases per 1,000 live births in 2011. Objective: To characterize newborns with congenital syphilis and describe follow-up over 6 months following their diagnosis and treatment. Materials and methods: Between August, 2011, and February, 2012, in Hospital La Victoria, sede Instituto Materno Infantil , newborns with congenital syphilis were identified and treated. Clinical and laboratory follow-up was carried out for six months until it was determined that the disease was cured. The analyzed variables included patients´ clinical and laboratory characteristics. Results: In this period, we identified 29 cases that met the criteria of congenital syphilis, with a frequency in the institution of one case per 69 births. There was one stillbirth and one neonatal death, four were premature, and five had growth restriction. Of the 28 live births, 15 were asymptomatic. There were two cases with neurosyphilis, representing 15% of symptomatic newborns. Follow-up was done on 18 out of 27 children (66.6%), including most symptomatic cases and the two cases of neurosyphilis . All cases showed the expected decrease in VDRL titers without clinical sequelae, meeting the criteria of being cured. Conclusions: A high incidence of the disease, failure to prevent it, varied expression in its presentation, and the effectiveness of treatment in the neonatal period were evidenced in this study.

Neonates at risk for congenital syphilis: radiographic and cerebrospinal fluid evaluations.

OBJECTIVE: To review the infants at risk for congenital syphilis (CS) and determine the optimal use of evaluations such as cerebrospinal fluid (CSF), the venereal disease research laboratory (VDRL) test, and long bone radiography studies. METHODS: A retrospective chart review of all of the infants at risk for CS from January 1997 to December 2002 at the Regional Medical Center at Memphis was conducted. Subjects were identified from a database of prenatal maternal records. Infant charts showing a diagnosis of presumptive CS were reviewed and data were collected. RESULTS: Of the 24,245 deliveries, maternal serology (rapid plasma reagin and microhemagglutination for treponemal antibody) was reactive in 250 women during pregnancy. Of 92 infants with a presumptive diagnosis of syphilis, only 2 (2.1%) were symptomatic. CSF examination for VDRL was feasible in 74 (80%) of the 92 infants. Only 1 (1.35%) of the 74 infants had a positive CSF-VDRL. Three infants had radiographic changes that were consistent with CS. CONCLUSIONS: The burden of syphilis in pregnancy remains high. Proper evaluation of neonates is important in preventing long-term consequences. The frequency of positive CSF and long bone radiography studies remains low. These evaluations should be made based on the symptoms and plan of treatment for individual neonates.

Lues congénita y efecto prozona / Congenital lues and prozone effect

La sífilis congénita es una patología que va actualmente en aumento. Se produce por el traspaso trasplacentario del Treponema pallidum durante el embarazo, determinando una mayor tasa de abortos, mortinatos, malformaciones y partos prematuros. En Chile la detección se realiza de rutina en el embarazo por medio de pruebas no treponémicas (VDRL, RPR). Existen algunas situaciones que pueden dificultar el diagnóstico de sífilis materna, como es el caso del efecto prozona, en que debido a un exceso de anticuerpos no se produce reacción y por lo tanto el resultado es falsamente negativo. Se presenta el caso de un lactante que debutó con lesiones perianales y neurosífilis, con screening negativo de la madre durante el embarazo

Central nervous system infection in congenital syphilis.

BACKGROUND: Identification of infants with Treponema pallidum infection of the central nervous system remains challenging. METHODS: We used rabbit-infectivity testing of the cerebrospinal fluid to detect T. pallidum infection of the central nervous system in infants born to mothers with syphilis. The results were compared with those of clinical, radiographic, and conventional laboratory evaluations; IgM immunoblotting of serum and cerebrospinal fluid; polymerase-chain-reaction (PCR) assay testing of serum or blood and cerebrospinal fluid; and rabbit-infectivity testing of serum or blood. RESULTS: Spirochetes were detected in the cerebrospinal fluid of 19 of 148 infants by rabbit-infectivity testing. Exposure of the infant to antibiotics before cerebrospinal fluid was obtained for rabbit-infectivity testing was associated with a negative test result (P=0.001). Spirochetes were detected in the cerebrospinal fluid in 17 of 76 infants (22 percent) who had no prior antibiotic exposure. These 17 infants included 41 percent (16 of 39) of those with some abnormality on clinical, laboratory, or radiographic evaluation; 60 percent (15 of 25) of those with abnormal findings on physical examination that were consistent with congenital syphilis; and 41 percent (17 of 41) of those with a positive result on IgM immunoblotting or PCR testing of serum, blood, or cerebrospinal fluid, or a positive result on rabbit-infectivity testing of serum or blood. Only one infant who had normal findings on clinical evaluation had a positive cerebrospinal fluid rabbit-infectivity test. Overall, central nervous system infection was best predicted by IgM immunoblotting of serum or PCR assay of serum or blood. CONCLUSIONS: Most infants with T. pallidum infection of the central nervous system can be identified by physical examination, conventional laboratory tests, and radiographic studies. However, the identification of all such infants requires the use of additional tests, including IgM immunoblotting and PCR assay.

Neurosyphilis in an eight-year-old child: usefulness of the SPECT study.

An 8-year-old boy with symptomatic late-onset congenital syphilis is reported. The child had been undertreated when he was 78 days of age, but his clinical and serologic follow-up did not occur until he was 3 years of age. At that time, he was asymptomatic, and cerebrospinal fluid disclosed not only hypercellularity but also a reactive Venereal Disease Research Laboratories test. Although he was then retreated at 4 years of age, he developed seizures. Cranial computed tomography and magnetic resonance imaging were normal, but two single photon emission computed tomography scans performed when he was 5 years of age and then 7 years of age demonstrated areas of hypoperfusion that closely agreed with the alterations on electroencephalograph. Brain single photon emission computed tomography was able to detect cerebral nervous system abnormalities in this young patient with neurosyphilis, whereas other image studies did not.

Dementia paralytica in a fifteen-year-old boy.

A 15-year-old boy, whose history revealed an unremarkable pregnancy, birth and neonatal period and who had shown a normal motor and mental development, presented at the hospital with deterioration of cognitive functions since the age of 7. He was bedridden with manifest ataxia involving all limbs, anisocoria and a sluggish to absent pupil reaction to light. Syphilis serology was positive with a Venereal Disease Research Laboratory (VDRL) titer of 1:256 and a Treponema pallidum Haemagglutination Assay (TPHA) titer of 1:163840. Cerebrospinal fluid (CSF) protein concentration was 55 mg/dl and CSF-leucocyte count was 14/mm3 (85% mononuclear cells). CSF-VDRL-titer was 1:16. A diagnosis of congenitally acquired dementia paralytica was made, since the boy's parents' clinical exam and serology results were suggestive for latent syphilis. Although cognition was still very much deteriorated five months following penicillin treatment, clinical examination revealed partial recuperation. Screening for syphilis should be part of routine testing in every subject presenting with cognitive deterioration, regardless of age.

Congenital neurosyphilis revisited.

The clinical symptoms, serological results and computed tomographic findings of two Zimbabwean children, one with the early meningeal and one with the late parenchymatous form of congenital neurosyphilis are reported. The paediatrician's suspicion of the existence of this condition should remain high as long as syphilis continues to flourish worldwide.

Serological tests in cerebrospinal fluid for congenital syphilis in central Africa.

Serological tests were performed in the cerebrospinal fluid (CSF) of 13 children with active congenital syphilis (presence of specific IgM FTA-ABS antibodies) and of seven seropositive children with no active syphilis (FTA-ABS IgM-negative) born to syphilitic treated mothers in Libreville, Gabon. Antibodies against treponema were measured by the Venereal Disease Research Laboratory test (VDRL), the Treponema pallidum haemagglutination assay (TPHA) and the fluorescent treponema antibody absorption tests (FTA-ABS IgG and IgM). Of the 13 children with active syphilis, seven had a positive FTA-ABS IgG in the CSF. The result of this test was not correlated with the severity of clinical features, CSF protein levels or number of CSF white blood cells. The CSF-TPHA test was positive in four out of 12 children, and the CSF-VDRL test was negative in all the children with active congenital syphilis. One of the seven newborns with mother-transmitted antibodies had a positive FTA-ABS and TPHA in the CSF. These data show that the VDRL is not sensitive enough to diagnose congenital neurosyphilis, and that FTA-ABS or, at least, TPHA are convenient, sometimes with false-positive results, when a sophisticated method of detecting specific IgM in CSF is not available.

[Congenital syphilitic meningomyelitis manifested in the adult]. / Méningo-myélite syphilitique congénitale révélée à l'âge adulte.

We report a case of neurosyphilis of the spinal cord in a 38-year-old-man presenting with urinary incontinence and Argyll-Robertson sign. Anamnesis revealed the congenital origin of the disease. Urodynamics findings were consistent with a conus medullaris involvement and CSF study revealed a specific lymphocytic meningitis. A specific spinal arteritis was found by angiography. A quarterly 15 to 20 days course of intravenous penicillin was then instituted during 2 years. A specific treatment of urinary symptoms resulted in complete disappearance of sphincter dyssynergia. Antibiotic therapy was followed by clinical improvement and regression of most of the CSF abnormalities.

Juvenile general paresis of insane.

A twenty five year old lady with general paresis of the insane presenting the classical features along with fixed dilated pupils is reported. The diagnosis was confirmed by positive serology and cerebrospinal fluid tests. The presence of a few stigmata and the early age suggest that the infection was acquired antenatally.