Abstract | November 18, 2023
A Case Report: Congenital Syphilis with Hydrops Fetalis, Severe Intrahepatic Cholestasis and Liver Failure
Learning Objectives
- Recognize that hydrops fetalis and hepatic failure are rare, but documented presentations of congenital syphilis and should be included in the differential as it is a potentially treatable condition if antibiotics are initiated early. Untreated, congenital syphilis has a 40% mortality
- Remember that Syphilis has increased 291% in the USA over the past 5 years, with a 28.6% increase in the past year (2021) Congenital syphilis shows a similar increase with rates increasing from 9.2 (2013) to 77.9 (2021)
- Ensure that repeat prenatal screening between 28-32 weeks and at time of delivery for high risk individuals is completed. False positive are frequent early in infection and syphilis can be acquired through out pregnancy
We present an unusual case of a premature female born with prenatally undiagnosed hydrops fetalis. This patient was born via emergency C-section at 32 weeks gestation due to premature rupture of membranes and non-reassuring fetal heart tracings to a 21 yo GP31001 mother. Pregnancy was complicated by limited prenatal care and multiple sexually transmitted infections. Mother was treated for Chlamydia, Trichomonas and primary herpes outbreaks. At the time of delivery, infant was found to have hydrops fetalis and required intubation in the delivery room for respiratory failure. APGARS were 2, 4, 7 at 1, 5 and 10 minutes. Additional findings of desquamating rash on admission exam. On admission to NICU, infant presented with multisystem organ failure and disseminated intravascular coagulation (DIC). Sepsis evaluation was initiated on admission, blood culture and HSV testing were sent. Infant was started on empiric antibiotics and Acyclovir. Lumbar puncture was deferred due to labile clinical status.
Further investigation into prenatal history revealed, mother had been treated for presumed primary HSV genital outbreak during the second trimester. Mother’s HSV cultures subsequently resulted negative, however, these results were not reported to her. Maternal HSV IgG positive and HSV IgM negative. Third trimester labs were not done. Based on concern regarding hydrops and desquamating rash, RPR was sent on infant and request was made for RPR to be sent on mother. Infant was started on IV Penicillin while awaiting results. Both mother and infant’s RPR later resulted positive and the confirmatory treponemal antibody was also positive.
Infant was treated for congenital syphilis, liver failure and severe cholestasis. Admission direct bilirubin was 1.5 mg/dl which continued to rise to 42 mg/dl by day of life 30. Abdominal ultrasound showed biliary sludge but was otherwise normal. Cholestasis was treated by cycling of TPN and D10W and use of Omegaven for the first month of life. Once on full enteral feeds, infant was started on Actigall. By 3 months of age, infant had a direct bilirubin of 0.8 mg/dl and Actigall was discontinued prior to hospital discharge.
References and Resources
- Screening for syphilis infection in pregnancy: Reaffirmation recommendation statement. American Family Physician. (2010, January 15). Retrieved September 25, 2022, from https://www.aafp.org/pubs/afp/issues/2010/0115/od1.html
- US Preventive Services Task Force. Screening for Syphilis Infection in Pregnant Women: US Preventive Services Task Force Reaffirmation Recommendation Statement. JAMA. 2018;320(9):911–917. doi:10.1001/jama.2018.11785
- Tennessee STD Epidemiological Profile 2021
- Bin S. Congenital pemphigus syphiliticus: a characteristic feature of a forgotten disease. BMJ Case Reports CP 2021;14:e246310.
- Molly Crimmins Easterlin, Rangasamy Ramanathan, Theodore De Beritto; Maternal-to-Fetal Transmission of Syphilis and Congenital Syphilis. Neoreviews September 2021; 22 (9): e585–e599. https://doi.org/10.1542/neo.22-9-e585
