Congenital syphilis in the context of Intrauterine growth restriction


Congenital syphilis in the context of Intrauterine growth restriction

⭐ Core Definition: Congenital syphilis

Congenital syphilis is syphilis that occurs when a mother with untreated syphilis passes the infection to her baby during pregnancy or at birth. It may present in the fetus, infant, or later. Clinical features vary and differ between early onset, that is presentation before 2-years of age, and late onset, presentation after age 2-years. Infection in the unborn baby may present as poor growth, non-immune hydrops leading to premature birth or loss of the baby, or no signs. Affected newborns mostly initially have no clinical signs. They may be small and irritable. Characteristic features include a rash, fever, large liver and spleen, a runny and congested nose, and inflammation around bone or cartilage. There may be jaundice, large glands, pneumonia (pneumonia alba), meningitis, warty bumps on genitals, deafness or blindness. Untreated babies that survive the early phase may develop skeletal deformities including deformity of the nose, lower legs, forehead, collar bone, jaw, and cheek bone. There may be a perforated or high arched palate, and recurrent joint disease. Other late signs include linear perioral tears, intellectual disability, hydrocephalus, and juvenile general paresis. Seizures and cranial nerve palsies may first occur in both early and late phases. Eighth nerve palsy, interstitial keratitis and small notched teeth may appear individually or together; known as Hutchinson's triad.

It is caused by the bacterium Treponema pallidum subspecies pallidum when it infects the baby after crossing the placenta or from contact with a syphilitic sore at birth. It is not transmitted during breastfeeding unless there is an open sore on the mother's breast. The unborn baby can become infected at any time during the pregnancy. Most cases occur due to inadequate antenatal screening and treatment during pregnancy. The baby is highly infectious if the rash and snuffles are present. The disease may be suspected from tests on the mother; blood tests and ultrasound. Tests on the baby may include blood tests, CSF analysis and medical imaging. Findings may reveal anemia and low platelets. Other findings may include low sugars, proteinuria and hypopituitarism. The placenta may appear large and pale. Other investigations include testing for HIV.

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Congenital syphilis in the context of Syphilis

Syphilis (/ˈsɪfəlɪs, ˈsɪfɪlɪs/) is a sexually transmitted infection caused by the bacterium Treponema pallidum subspecies pallidum. The signs and symptoms depend on the stage it presents: primary, secondary, latent or tertiary. The primary stage classically presents with a single chancre (a firm, painless, non-itchy skin ulceration usually between 1 cm and 2 cm in diameter), though there may be multiple sores. In secondary syphilis, a diffuse rash occurs, which frequently involves the palms of the hands and soles of the feet. There may also be sores in the mouth or vagina. Latent syphilis has no symptoms and can last years. In tertiary syphilis, there are gummas (soft, non-cancerous growths), neurological problems, or heart symptoms. Syphilis has been known as "the great imitator", because it may cause symptoms similar to many other diseases.

Syphilis is most commonly spread through sexual activity. It may also be transmitted from mother to baby during pregnancy or at birth, resulting in congenital syphilis. Other diseases caused by Treponema bacteria include yaws (T. pallidum subspecies pertenue), pinta (T. carateum), and nonvenereal endemic syphilis (T. pallidum subspecies endemicum). These three diseases are not typically sexually transmitted. Diagnosis is usually made by using blood tests; the bacteria can also be detected using dark field microscopy. The Centers for Disease Control and Prevention (U.S.) recommends for all pregnant women to be tested.

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Congenital syphilis in the context of Inheritance (Munch)

Inheritance (Norwegian: Arv; 1897–1899) is an oil painting on canvas created by the Norwegian painter Edvard Munch (1863–1944). It portrays a syphilitic mother holding her child, born with congenital syphilis. Munch completed the work after visiting the Hôpital Saint-Louis in Paris, where he saw a woman crying for her child with the disease.

The baby in the painting is lifeless, pale and covered in spots. The mother, whose hands and patterned skirt are especially prominent, has a tearful red face and sits on a bench in front of a green background.

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Congenital syphilis in the context of Pneumonia alba

Pneumonia alba (white pneumonia) is often seen in neonates with congenital syphilis. The lung may be firm and pale, owing to the presence of inflammatory cells and fibrosis in the alveolar septa. Spirochetes are readily demonstrable in tissue sections.

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Congenital syphilis in the context of Saddle nose deformity

Saddle nose is a condition associated with nasal trauma, congenital syphilis, relapsing polychondritis, granulomatosis with polyangiitis, cocaine abuse, and leprosy, among other conditions. The most common cause is nasal trauma. It is characterized by a loss of height of the nose, because of the collapse of the nasal bridge. The depressed nasal dorsum may involve bony, cartilaginous, or both bony and cartilaginous components of the nasal dorsum.

It can usually be corrected with augmentation rhinoplasty by filling the dorsum of the nose with cartilage, bone or synthetic implant. If the depression is only cartilaginous, cartilage is taken from the nasal septum or auricle and laid in single or multiple layers. If deformity involves both cartilage and bone, cancellous bone from iliac crest is the best replacement. Autografts are preferred over allografts. Saddle deformity can also be corrected by synthetic implants of teflon or silicon, but they are likely to be extruded.

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Congenital syphilis in the context of Higouménakis' sign

Higouménakis' sign is a unilateral enlargement of the sternoclavicular portion of the right clavicle, seen in congenital syphilis.

The sign was named for George Higoumenakis, who first described it in 1927 in the Greek periodical Proceedings of the Medical Society of Athens (Πρακτικά Ιατρικής Εταιρείας Αθηνών). He subsequently published the description of the sign in a German article, making it more known among dermatologists.

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Congenital syphilis in the context of Rhagades

Rhagades are fissures, cracks, or linear scars in the skin, especially at the angles of the mouth and nose. They tend to form at areas of motion. They can be a result from bacterial infection of skin lesions. They are one of the late-stage manifestations of congenital syphilis; others are saber shins, Hutchinson teeth, saddle nose, and Clutton's joints (usually knee synovitis).

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Congenital syphilis in the context of Hutchinson teeth

Hutchinson's teeth is a sign of congenital syphilis. Affected people have teeth that are smaller and more widely spaced than normal and which have notches on their biting surfaces.

It is named for Sir Jonathan Hutchinson, a British surgeon and pathologist, who first described it.

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Congenital syphilis in the context of Hutchinson's triad

Hutchinson triad is a triad of signs that may be seen in late congenital syphilis, including: interstitial keratitis, malformed teeth (Hutchinson incisors and mulberry molars), and eighth nerve deafness.

Late congenital syphilis typically manifests after 2 years of age. It is a rare condition due to the high mortality rate of mother-to-child transmission (MTCT) of syphilis. Nearly 40% of MTCT of syphilis result in pregnancy loss or neonatal fatality. Additionally, only 15% of liveborn infants are symptomatic.

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