Congenital Herpes Simplex

What is Congenital Herpes Simplex?

Congenital herpes simplex virus (HSV) is a rare but serious infection that can occur in newborns, causing significant health problems and could even be life-threatening, depending on how severe it is. Newborns tend to show nonspecific symptoms, making it crucial to diagnose the infection accurately and as soon as possible. HSV infection in newborns often appears as a disease that impacts multiple organs, most often the liver and lungs (in 25% of patients), or a local disease affecting the central nervous system, sometimes with skin symptoms (in 30% of patients). In the remaining 45% of patients, the disease only affects the skin, eyes, and mouth (SEM disease). Most newborns – over 80% – who are with SEM disease show skin blisters. Those without blisters have an eye infection alone or eyes and mouth infection.

However, diagnosing the newborn’s HSV infection could be difficult when there are no skin blisters. A widespread infection might be considered when newborns show serious illness that resembles infection caused by bacteria, have negative bacteria culture results, severe liver dysfunction, or blood clotting disorders within the first month after birth. HSV could be the cause if a newborn has a fever, a rash with blisters, or irregular cerebrospinal fluid findings. These signs become more likely if the baby also has a history of seizures or if there are no reports of enteroviruses in the newborn’s local community. While older children can often have asymptomatic HSV infection, it rarely happens in newborns. The first signs of HSV infection can show up anytime from birth to around 6 weeks of age, although almost all infected infants show symptoms within the first month of life. Infants with widespread disease and SEM disease often show symptoms between the first and second weeks of life. For infants with a disease affecting their central nervous systems, symptoms usually appear between the second and third weeks of life.

Confirmation of diagnosis can be done through methods like fluorescent antibody staining, enzyme immunoassays, and monolayer culture with typing. If the culture results show the presence of the virus 12 to 24 hours after birth, this indicates that the virus has reproduced, indicating the infant is infected, not just exposed during childbirth. The most common treatment for newborn HSV infection is a medicine called acyclovir, which should be given to all newborns with HSV disease, no matter the specific symptoms and clinical findings.

What Causes Congenital Herpes Simplex?

HSV-1 and HSV-2 are types of viruses known as Herpes Simplex Viruses (HSVs). They are wrapped in an outer layer and contain two strands of DNA. HSV-1 usually infects the face and upper body, but it’s becoming increasingly responsible for genital herpes as well. HSV-2 more often infects the genital area and lower body in sexually active teenagers and adults. However, both types of viruses can occur in both areas, and both can cause herpes disease in newborns.

Like all human herpes viruses, after an initial infection, the HSV-1 and HSV-2 viruses remain dormant in the body to reactivate from time to time. This reactivation can trigger either recurrent symptoms or silent viral shedding where the virus is present but without symptoms.

Several factors can increase the risk of a pregnant mother passing HSV to her child. These include the type of HSV infection she has (whether it’s a first-time infection or a recurring one), her immunity to the virus, which type of HSV she has, if she has an active HSV infection at the time of delivery, the method of delivery (vaginal or cesarean), if her water has broken for a long time, and if a fetal scalp electrode is used during labor.

Risk Factors and Frequency for Congenital Herpes Simplex

Congenital HSV, a type of herpes, affects between 1 in 3000 to 1 in 20,000 newborns. Its most common way of transmission to a newborn is during birth if the mother has an infected genital tract. However, it can also occur in the womb if the water bag (amniotic membranes) is ruptured. A less common way for a newborn to get the disease is from a parent or caregiver after birth, usually from a lesion somewhere other than the genitals, like the mouth or hands.

• If the mother gets a primary genital infection close to the time of delivery, the chances that the newborn will also get the virus are between 25% and 60%.
• If the mother has an older, recurring infection, the chance for the newborn to get the virus is less than 2%.

Telling the difference between a first-time (primary) and a recurring herpes infection in women can be challenging, as both may either have no symptoms, or display non-specific symptoms, such as vaginal discharge, genital pain, or shallow ulcers. Furthermore, knowing whether a mother had genital HSV doesn’t help in diagnosing a newborn, because most babies who do get herpes are born to women who didn’t know they had herpes during or before pregnancy – either because they had no symptoms, or they weren’t recognizable as being due to herpes.

Signs and Symptoms of Congenital Herpes Simplex

Congenital Herpes Simplex Virus (HSV) infection can be hard to recognize because its early symptoms are usually subtle and not specific. The skin might show signs like scarring, active lesions, uneven skin color, absence of skin, and spotted rashes. Eye examinations may detect smaller than normal eyes, abnormal retinal growth, optic nerve damage, and inflammation in the retina. There could also be neurologic symptoms like microcephaly (small head), softening of the brain tissue, water-filled sac replacing part of the brain, and calcifications within the skull. It’s important to note that Congenital HSV can imitate other conditions, like bacterial and viral infections. The disease can present itself in various forms, like in the skin, eyes, mouth, central nervous system (brain and spinal cord), or several other locations within the body.

Clinical manifestations in the skin, eyes, and mouth typically start to appear in the second or third week after birth. The most common sign is vesicular lesions or blisters on a red base. Infection in eyes can lead to inflammation, and without treatment, it can result in corneal blindness. This type of disease has low mortality but recurs in almost 90% of patients.

Infants with central nervous system involvement may initially show symptoms like fever, poor feeding or sudden seizure episodes. Around 60 to 70% of babies with central nervous system disease also have skin symptoms at some point during their illness.

Disseminated or widespread disease in infants often presents symptoms similar to sepsis within the initial three weeks of life. This can affect multiple organs, causing yellow skin or eyes, abnormal liver function, low blood sugar, and pneumonia, among other complications. Disseminated disease, 75% of the time is associated with inflammation of the brain leading to seizures.

Therefore, it’s crucial to consider congenital HSV infection in newborns that present with skin lesions, central nervous system abnormalities, or sepsis-like symptoms. In some cases, infants with HSV infection may have continuous fever with no microbial growth in cultures.

Congenital HSV infection should be suspected in newborns and infants (up to six weeks old) with any of the following symptoms:

• Blisters in the skin, eyes or mouth
• Sepsis-like symptoms (for example, fever or low body temperature, irritability, lethargy, difficulty breathing, pauses in breathing, swollen belly, enlarged liver, or fluid accumulation in the belly)
• Inflammation in the brain and spinal cord
• Seizures
• Focal neurologic signs (signs of neurologic dysfunction)
• Abnormal findings on brain imaging
• Difficulty in breathing, pauses in breathing or progressive pneumonia
• Low platelet count
• Elevated liver enzymes, viral hepatitis, or sudden liver failure
• Inflamed eyes, excessive tears, or painful eye symptoms

Testing for Congenital Herpes Simplex

Diagnosing HSV, a virus that grows easily in laboratory conditions, can be done by taking cultures from sores and other affected areas in babies. This involves collecting samples from these sites and transporting them to labs for further testing. Usually, signs of HSV infection are visible within 1 to 3 days after the sample is introduced to the lab. The diagnosis can then be confirmed through various methods such as fluorescent antibody staining, enzyme tests, and monolayer culture with typing.

If the cultures don’t show any HSV activity after 5 days, they’re likely to stay that way. A positive result from any surface test taken more than 12 to 24 hours after the baby’s birth suggests actual infection and not just exposure during delivery.

All babies with HSV should be given an eye test and a review of their brain structure. While an MRI is the best method for brain imaging, it may sometimes require sedation. In such cases, a CT scan or ultrasound can be used as alternatives.

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A test called a Polymerase Chain Reaction (PCR) assay can be used to detect HSV DNA. This is the preferred test for HSV involvement in the Central Nervous System (CNS). PCR can also be used for diagnosis from skin lesions. However, early-stage Herpes Simplex Encephalitis (HSE) may not show up in a PCR test of the Cerebrospinal Fluid (CSF) that surrounds the brain and spinal cord.

For difficult cases with negative PCR results, a biopsy of the brain tissue can confirm the diagnosis of HSE. The presence of antibodies against HSV can also help with diagnosis. Usually, cultures of CSF do not show HSV.

When diagnosing HSV in babies, samples are taken from several areas including the mouth, nasopharynx, eyes, and anus for HSV culture and PCR testing if needed. Also, samples from skin sores, and blood samples can be tested. In addition to these, a blood sample can be taken to measure the level of a liver enzyme called alanine aminotransferase (ALT).

Babies presenting seizures should be checked with an electroencephalogram (EEG), a test that checks for abnormal brain activity.

Treatment Options for Congenital Herpes Simplex

A standard treatment for babies born with a Herpes Simplex Virus (HSV) infection is an injected medicine called acyclovir. All newborns with HSV should get this treatment, regardless of the symptoms or clinical findings they have. Nurses will usually give acyclovir to babies in a three-part daily dosage.

How long the medicine is given depends on the type of HSV being treated. If babies have skin, eye or mouth (SEM) disease, they should get an intravenous injection (IV) of acyclovir for 14 days. If they have brain or organ disease, they should get the IV for at least 21 days.

Towards the end of this therapy, a medical professional should do a spinal tap test to make sure the fluid around the spinal cord is free of HSV DNA. If the test comes back positive, they will give the baby another week of acyclovir and repeat the test. If it remains positive, they give the baby another week of therapy. For such cases, it would be best to involve a specialist in infectious diseases.

Once acute treatment is complete, babies surviving HSV infections should continue to take oral acyclovir to prevent more outbreaks and improve their neurological development. The dosage should be adjusted each month to account for their growth, and blood tests should be done regularly to monitor their immune system response. Higher doses or longer therapy times aren’t shown to further improve their neurological development.

A drug called Valacyclovir has not been adequately studied in infants, so it is not recommended for regular use. If babies have eye problems linked to HSV, they should get an eye drop medicine as well as the injected antiviral therapy. An eye specialist should be involved in treating these types of HSV infections in newborns.

What else can Congenital Herpes Simplex be?

When determining if a newborn has HSV (herpes simplex virus), doctors need to consider a range of possible diagnoses based on the symptoms present. These alternative diagnoses can include the following conditions:

Skin Lesions:

• Erythema toxicum neonatorum (ETN): This produces red spots or small bumps on a baby’s skin. These spots can turn into small blisters, mostly found on the trunk and upper limbs and usually appear a day or two after birth. It clears up on its own within a week.
• Transient neonatal pustular melanosis (TNPM): This mainly affects full-term Black babies and presents as small skin blisters filled with yellowish fluid. It would not yield any organisms if cultured for disease.
• Miliaria: This is also known as heat rash and is common in babies living in hot climates. It occurs when sweat gets trapped under the skin, causing small blisters or bumps.
• Infantile acropustulosis (IA): This is a condition that causes small itchy blisters on a baby’s hands and feet. The cause is unknown, but it might be related to a reaction to scabies.
• Congenital sucking blisters: These are noninflammatory blisters filled with clear fluid found on a baby’s hands, wrists, or fingers. These are typically seen on babies who suck excessively at these areas.

Eye Disease:

• Possibility of viral or bacterial conjunctivitis.

CNS (Central Nervous System) Disease:

• Doctors should also rule out bacterial meningitis and other types of viral meningoencephalitis, besides HSV.
• Also, inherited metabolic disorders need to be considered.

Disseminated Disease:

• Babies can also have sepsis due to bacterial causes or viral infections like the adenovirus, enterovirus, and parechovirus.
• Additionally, viral hepatitis other than HSV, drug-induced hepatitis, or other causes of neonatal liver disease could be causing the symptoms.
• Lastly, doctors should also think about other neonatal infections such as cytomegalovirus, toxoplasmosis, syphilis, and rubella.

Remember, these are potential diagnoses that doctors would consider. They help to make sure that the newborn’s symptoms are not due to a different condition causing similar symptoms to HSV.

What to expect with Congenital Herpes Simplex

The future health of babies with congenital HSV (an infection caused by Herpes Simplex Virus) can depend on many factors. These can include when the mother got the infection, how quickly the baby is diagnosed, and how severely the baby’s organs are affected. Babies with mild or no symptoms may have a better outlook.

The best survival rates and health condition are seen in babies with SEM disease, which is a type of HSV infection. Around half of babies who make it through an HSV infection will get skin recurrences of the condition. This often happens within 1 to 2 weeks after stopping a medication used to treat HSV, called acyclovir. However, babies with severe cases, like brain involvement, have higher long-term risks of brain damage and developmental difficulties.

Starting an antiviral medication like acyclovir as soon as possible can improve the health of the baby. Early treatment usually leads to a better overall health outlook. However, even with treatment, the health issues and deaths in severe cases remain high. Some of the possible long-term problems can include eye issues and neurodevelopmental deficits. This highlights the need for these kids to receive long-term care from a team of health professionals.

It’s also important to try to prevent as many new HSV infections as possible, including in pregnant women. Pregnant women with a history of genital herpes can take antiviral medications late in pregnancy and during labor to help protect their babies. This strategy can lower the number of babies born with HSV, making both diagnosis and treatment easier.

Possible Complications When Diagnosed with Congenital Herpes Simplex

Congenital HSV, an infection caused by the herpes simplex virus, can lead to a range of complications. It has a particular tendency to affect the nervous system and can spread to different organs of the body.

Let’s take a closer look at these complications.

• Central nervous system problems: Serious complications can occur if HSV affects the brain. This can lead to encephalitis, which can in turn result in neurological impairment, developmental delays, and long-term issues with cognitive function.
• Eye problems: HSV can affect the eyes, leading to conditions like chorioretinitis, which can result in either impaired vision or complete loss of sight.
• Skin problems: HSV can cause skin lesions, which can leave behind scars or lead to the infection spreading.
• Organ damage: HSV infection can also affect other, internal organs like the liver, lungs, and adrenal glands, which can impair their function.
• Complications due to premature birth: Babies with congenital HSV may be born prematurely because the infection can induce labor.

Despite advancements in antiviral treatments, the risk of death and long-term health issues remain substantially high, highlighting the need for early intervention, thorough management strategies, and constant post-treatment care to manage the numerous complications arising from congenital HSV infection.

Preventing Congenital Herpes Simplex

Preventing and educating patients is key to reducing the chances of newborns getting HSV (herpes simplex virus). Doctors and healthcare providers have an important role in informing pregnant women about the risk of HSV during pregnancy. They also talk about ways to keep the unborn child from getting the virus. For example, they might suggest not having sexual intercourse or using protection like condoms, particularly if one person has had genital herpes before.

Additionally, doctors provide information on how to recognize the signs and symptoms of HSV during pregnancy and what could happen to the newborn. This can help pregnant women get medical help quickly if they need it. The team taking care of pregnant women must follow the guidelines provided by the American College of Obstetricians and Gynecologists (ACOG) for treating and delivering such patients effectively.

By learning about newborn HSV and how to prevent it, patients can make knowledgeable decisions and take measures to protect their health and their baby’s health.