Congenital Syphilis in an Infant at a Nigerian Hospital

Abstract

A 2-month-old male infant was diagnosed as having congenital syphilis at the Outpatient children’s Clinic at LAUTECH, Teaching Hospital, Ogbomoso. He was delivered at a primary health center with an abnormally shaped nose and oval flesh-like swelling adjacent to the lateral wall of the left nostril. Over the first 2 months of life, he developed a slowly progressive swelling around the wrist. Despite several courses of antibiotics prescribed by several doctors at both tertiary and private hospitals, for an unknown illness, he did not get better. The mother of the infant referred her infant close to the postnatal age of 3 months. The case and the challenges encountered in making a diagnosis and management are discussed.

INTRODUCTION

Syphilis is a disease, which can be transmitted horizontally and vertically.[1,2]

Sexual transmission of the infective agent, Treponema pallidum, usually occurs in adults, while transmission is commonly vertical in congenital infections.

The bacteria can infect the baby transplacentally, or the infection can be acquired from the maternal tract as the baby travels down the birth canal at delivery.[1,2,3] Congenital infections can lead to abortions or premature or mature delivery, as well as manifest as sepsis in the neonatal period or much later.[2,3,4]

Congenital syphilis is a very rare infection in this present age. It has been virtually eliminated in the developed countries while some levels of elimination has been achieved by low income countries.[2] This might be attributed to the effectiveness of the syndromic management of sexually transmitted diseases (STDs) by the World Health Organization and better access to antenatal care,[5] where STDs can be screened and treated. Unrestricted access to antibiotics by the public, in the developing world, gives abundant access to medications for the treatment of STDs. Reduced congenital syphilis infections are a logical expected consequence of reduced maternal disease. Thus, congenital cases of syphilis are rarely encountered in practice in modern day practice. Rarely encountered diseases are likely to be missed, misdiagnosed, and consequently wrongly managed. Knowledge of a disease is a prerequisite for correct and prompt diagnosis.

We report the case of a 2-month-old infant with physical and radiologic features of congenital syphilis. The infant had undergone several consultations with several doctors who had managed with different courses of antibiotics without making a diagnosis. The infant was referred verbally for a maxillofacial specialist review for an abnormally shaped face and nose by one of the attending physicians. The case and the lessons learned are discussed.

CASE REPORT

A 2 month old infant was seen at the pediatric outpatient unit of the Ladoke Akintola University of Technology, Ogbomoso, with a swelling located on the left half of the bridge of the nose. The swelling was pin-sized at birth but progressively got bigger over 2 months, thereby disfiguring the nose, with partial loss of skin and intermittent discharge of pus from the skin-denuded area. The mother did not remember the child having a nasal discharge or noisy nasal breathing. However, over the first 2 months of life, there had been a progressive swelling of the wrists of both the hands. There was no history suggestive of abuse or trauma. There was no history of fever or rash at present or in the past in this infant, nasal discharge, or snuffles. The baby had been exclusively breast fed from birth.

Pregnancy was desired and booking for antenatal care was around 3 months of pregnancy at a maternity centre in Ogbomoso and it was not adversely eventful. The mother did not have a venereal disease research laboratory test. Delivery occurred spontaneously at term and was attended by a community health extension worker. The weight of the baby was judged to be average, with the baby crying well at delivery and not needing resuscitation. Subsequently baby was discharged home in good condition.

There was no history to suggest a sexually transmitted infection in the mother or father prenatally or during pregnancy. The father was a 40-year-old bricklayer with the completion of the senior secondary school as the highest educational attainment. The father was married to two wives with the mother of the patient being the second wife. She was aged 30 year old and was a hairdresser by profession with senior secondary training as her highest formal education status. The mother had three children, all alive and well, while the second wife had three children, all alive and well.

The infant has obtained several courses of antibiotics from birth, for the paranasal swelling which has being growing bigger with intermittent discharging pus, from the skin denuded base and bridge to the nose. The antibiotics were prescribed by different physicians at the government and private hospitals consulted. The infant was verbally referred to a maxillofacial specialist for the removal of the paranasal swelling and possible nose reconstruction by one of the attending doctors.

General examination revealed a well fed male baby with a weight of 3.6 kg and a full length of 52 cm. He was not pale, cyanosed, or icteric. There was an oval sized mass with a diameter of about 1 cm adjacent to the lateral wall of the left nostril, with partial loss of the skin at the junction of the root with the nasal bridge and adjacent nostril skin. The baby also had snuffles. The baby had a normal sized lymph nodes and a normal axillary temperature of 36.5°C.

All the systemic examinations were normal except for the limbs, which showed bilateral swelling of the wrists. A picture of the infant showing the swollen wrists, the abnormal or saddle shaped nose, and the paranasal selling are shown in Figure 1.

Figure 1.

Figure of the infant with a swollen wrist, saddle nose, and paranasal swelling

A tentative diagnosis of congenital syphilis was made. Full blood count, cerebrospinal fluid (CSF) analysis, urinalysis, and a qualitative treponemal immunoglobulin (Ig) test were done because of the age of the infant and financial constraints on the part of the mother. The retrovirus status test for human immunodeficiency virus was reported negative, while the retrovirus status of the father was unknown. The managing physicians bore part of the cost of infant care. All the tests were reported normal.

Radiographs of the upper and lower limbs, however, showed widespread lytic (lucent) lesions involving the metaphysis of long bones, more prominent on the femoral and tibia bones, with destruction of the overlying cortical bone. All the long bones also showed a lamellated periosteal reaction. The medial cortex of the right radial metaphysis is irregular, indicating a fracture. Wimberger’s line (demineralization of the proximal tibia metaphysis), with multiple sites of osteochondritis of the wrists were also seen. Radiographs of the upper and lower limbs are shown in Figures 2 and 3, respectively.

Figure 2.

Right radial bone with arrow pointing to rarefaction and breaks on the cortex in the metaphysis, indicating fracture

Figure 3.

The upper arrow points to the laminated periostitis on the femoral bone, while the lower arrow points to the localized destruction of the medial part of the proximal tibia metaphysis (Wimberger’s sign)

Treatment for congenital syphilis with an abridged version of intravenous crystalline penicillin, followed up with procaine penicillin, was commenced because of financial constraints, with the mother not willing for the infant to be admitted. The infant is for follow up at the outpatient clinic.

DISCUSSION

The diagnosis of congenital syphilis was both clinical and radiological, as the serological status of the mother was unknown during pregnancy. The WHO surveillance case definition for congenital syphilis includes children born and aged below 2 years to mothers with a positive serology status for syphilis or unknown serostatus for syphilis with either clinical or laboratory or radiologic features of syphilis or all features.[6]

Although the Ig assay tests for IgM and IgG in the infant’s blood were reported as normal, the Ig assay result cannot be considered valid for many reasons, as a quantitative measurement of the Ig is the standard for diagnosing infection.[5] Second, the qualitative tests were performed in a laboratory where we could not be sure of quality assurance. Third, the patient had been subjected to many antibiotics, and the blood investigation reports were from blood samples analyzed when the infant was close to 3 months of postnatal age. This might explain the normal urinalysis and CSF results, with the exception of the bony radiographs.

The Wimberger’s sign and the generalized osteitis as well as the involvement of the wrists are characteristic of syphilis.[5] In addition, the saddle nose is characteristic of syphilis. Nodular swellings are not uncommonly associated with syphilis.[7] Financial constraints, however, discouraged requesting additional investigations to determine the etiology of paranasal swelling. Other treponomes can affect the organs affected by T. pallidum; however, other treponomes do not present with classical congenital syphilitic features like those observed in the presented case.

The risk for acquiring congenital syphilis in this child is not apparent as the mother did not admit having an STD and was not screened or treated for this, as delivery was at a maternity.

However, sometimes, STDs can be asymptomatic. The child diagnosed with congenital syphilis was also delivered into a polygamous home. Human immunodeficiency infection is a factor that has been documented to predispose and increase the prevalence of horizontal and vertical syphilis acquisition.[8,9] The retrovirus status of the mother was reported as negative. However, the retrovirus status of the father could not be ascertained.

Failure to consider a diagnosis of congenital syphilis by any of the several physicians who attended to this infant is a concern. It shows the level of ignorance among doctors regarding information on congenital syphilis, thus highlighting the importance of this case presentation. Knowledge of syphilis among health workers is very important, considering the fact that syphilis has several stages that can negatively impact the life of the infected if not promptly managed. Furthermore, managing an undetected case of syphilis puts the health worker and other patients close contacts at risk of contacting syphilis as it can be transmitted through infected biologic material.[10]

In conclusion, congenital syphilis is a disease that can be acquired congenitally. Knowledge of its mode of infection, diagnosis, and management is important to prevent misses in the diagnosis and poor management. It is quite important that prompt diagnosis and correct management are instituted for a good outcome.. Furthermore, best practices are predicated on sound knowledge, which is expected to impact the prevention of disease and spread to potential contacts and close associates.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed.

Conflicts of interest

There are no conflicts of interest.

Funding Statement

Nil.