Here, we report a case of late congenital syphilis presenting as palatal perforation and saddle nose deformity, also fulfilling the Kassowitz law. [ijstd.org]

Abstract A patient who presented with recurrent bilateral knee effusions was found to have positive serum syphilis serology. [ncbi.nlm.nih.gov]

Presents outstanding photographs for virtually every common skin disorder. [books.google.com]

• Splenomegaly

  Common features: stillbirth prematurity, low birth weight, IUGR hepatomegaly, splenomegaly skeletal abnormalities skin lesions pneumonia hyperbilirubinemia anemia snuffles (nasal discharge) CSF abnormalities. [johnsarm.net]

  Common manifestations are skin rashes, mucous patches, lymphadenitis and hepato- splenomegaly. Late congenital syphilis refers to the individuals with syphilis from the age of 2 to 30 years. [ijstd.org]

  Less common presentations include patchy alopecia, anterior uveitis, meningitis, cranial nerve palsies, hepatitis, splenomegaly, periostitis and glomerulonephritis. [patient.info]

  Features of syphilis in HIV include: generalized lymphadenopathy; splenomegaly; hepatitis; skin rashes, alopecia or both; oral manifestations; cognitive impairment; meningitis; cranial nerve palsies; myopathies; and uveitis. [academic.oup.com]

• Hutchinson's Triad

  Skin -rhagades(cracks, fissures atmucocutaneousjunctions) #1, 2, & 3 are part of Hutchinson’s triad Extended discussion Epidemiology (Treponema Pallidum): Transmitted at any time during pregnancy or at birth Transmitted at any disease stage Almost 100% [johnsarm.net]

  triad ), such as Hutchinson teeth and perforation of the hard palate interstitial keratitis (part of Hutchinson triad ) neurosyphilis including sensorineural deafness (part of Hutchinson triad ) musculoskeletal anomalies paroxysmal cold hemoglobinuria [radiopaedia.org]

  […] part of nose) hard palate defect swollen knees saber shins short maxillae protruding mandible A frequently-found group of symptoms is Hutchinson's triad, which consists of Hutchinson's teeth (notched incisors), keratitis and deafness and occurs in 63% [en.wikipedia.org]

  Hutchinson’s triad (Hutchinson’s teeth, interstitial keratitis, and VIIIth nerve deafness) is considered pathognomonic of late congenital syphilis. [dartmouth.edu]

  The constellation of interstitial keratitis, cranial nerve VIII deafness, and Hutchinson teeth is called the Hutchinson triad. [aao.org]

• Saddle Nose

  The saddle nose deformity was due to the failure of development of nasal bones. [ijstd.org]

  Clinical examination showed the "saddle-nose" deformity, Hutchinson's teeth, high-arched palate, Dubois little-finger sign, and small maxillae resulting in shallow depression of the central part of the face. [ncbi.nlm.nih.gov]

  Similar to Secondary Syphilis in adults Triad Hutchinson Teeth (1 st lower molors) Deafness (8 th Nerve) Interstitial Keratitis Lymphadenopahy Autoimmune Haemolytic anemia Osteochondritis and Metaphysitis Glaucoma Pseudoparalysis of parrot (Painful limbs) Saddle [mediconotebook.com]

  Common late manifestations (appear > 2 yrs of age): Teeth -Hutchinson teeth (notched, pegged), mulberry molars Eye -interstitial keratitis Cranial nerves -8th nerve deafness Skeletal -frontal bossing, high arched palate, saddle nose (markedlydepressed [johnsarm.net]

  The first is a depressed deformity of the bridge of the nose (saddle nose), and the second is anterior bowing of the tibias (saber shins). Infection of the enamel causes notched incisors (Hutchinson’s teeth). [histopathology-india.net]

• Failure to Thrive

  Rhinitis with highly infectious nasal discharge, nontender generalized adenopathy, alopecia, iritis, and failure to thrive occur less frequently. [dartmouth.edu]

  Congenital Syphilis Complications The following complications may develop in patients suffering from CS: Acidosis Deafness Blindness Pancreatitis Liver failure Hypoglycemia Severe anemia Failure to thrive Respiratory failure Thrombocytopenia Leukemoid [hxbenefit.com]

  Signs and symptoms include irritability, fever, failure to thrive, saddle nose, cutaneous rash, and pneumonia. [fpnotebook.com]

  Patient also had complaints of high grade fever since ten days and history of failure to thrive. On general examination there was pallor and hepatosplenomegaly but no jaundice, lymphadenopathy or maculopapular rash. [ijri.org]

• Hepatomegaly

  Congenital SyphilisHydropsfetalis Nasal discharge Petechial rash Necrotizing funisitis within the matrix of the umbilical cord Hepatomegaly Rash Ostitis, Metaphysitis, P eriostitis Wimberger sign 12. [slideshare.net]

  Common features: stillbirth prematurity, low birth weight, IUGR hepatomegaly, splenomegaly skeletal abnormalities skin lesions pneumonia hyperbilirubinemia anemia snuffles (nasal discharge) CSF abnormalities. [johnsarm.net]

  References: [3] [4] [5] [2] [6] Congenital syphilis Onset Characteristic features Early congenital syphilis (onset prior to 2 years of age) Hepatomegaly and jaundice Rhinorrhea with white or bloody nasal discharge (“ snuffles ”) Maculopapular rash on [amboss.com]

  […] manifestation Occurs in approximately 40% of cases Rash Onset in first eight weeks Occurs in approximately 50% of cases – usually diffuse maculopapular rash but can also have desquamation alone, vesicular, bullous, papulosquamous or mucosal lesions Hepatomegaly [cps.ca]

  Presence of hydrops, hepatomegaly, ascites, or thickened placenta among other signs suggest a higher risk of fetal treatment failure. Evidence to recommend specific therapy in these cases is insufficient. Congenital syphilis can cause fetal demise. [clinicaladvisor.com]

• Hutchinson's Teeth

  Clinical examination showed the "saddle-nose" deformity, Hutchinson's teeth, high-arched palate, Dubois little-finger sign, and small maxillae resulting in shallow depression of the central part of the face. [ncbi.nlm.nih.gov]

  General wasting, enlarged liver, gumma of the frontal bone, Hutchinson’s teeth, gummatous ulcer of palate Stigmata of late congenital syphilis in a boy at age 15 years, year 1943. [pathologylearningcentre.uct.ac.za]

  Hutchinson’s triad (Hutchinson’s teeth, interstitial keratitis, and VIIIth nerve deafness) is considered pathognomonic of late congenital syphilis. [dartmouth.edu]

  Occurs in the untreated infant regardless of early sx’s; results from chronic inflammation of the bone, teeth, and CNS HEENT: Saddle nose frontal bossing (“olympian brow”) keratitis CN VIII deafness Teeth/bone (6th year): Hutchinson teeth mulberry molars [johnsarm.net]

  The constellation of interstitial keratitis, cranial nerve VIII deafness, and Hutchinson teeth is called the Hutchinson triad. [aao.org]

• Mulberry Molar

  Common late manifestations (appear > 2 yrs of age): Teeth -Hutchinson teeth (notched, pegged), mulberry molars Eye -interstitial keratitis Cranial nerves -8th nerve deafness Skeletal -frontal bossing, high arched palate, saddle nose (markedlydepressed [johnsarm.net]

  The present cases were diagnosed based only on the dental finding such as Hutchison's teeth and mulberry molars during school dental check-up. [jisppd.com]

  The most important signs are frontal bossae (bony prominences of the forehead) (87%), saddle nose (74%), short maxilla (83%), high arched palate (76%), mulberry molars (more than four small cusps on a narrow first lower molar of the second dentition), [dartmouth.edu]

  It can even cause mulberry molars in fetus. Syphilis can result in miscarriages, stillbirths, premature births, or even death of the newborn babies. [hxbenefit.com]

  Late manifestations (age 3 or older) result from scarring during early systemic disease and include Hutchinson teeth, Mulberry molars, abnormal facies, cranial nerve VIII deafness, bony changes such as saber shins and perforations of the hard palate, [aao.org]

• High Arched Palate

  Clinical examination showed the "saddle-nose" deformity, Hutchinson's teeth, high-arched palate, Dubois little-finger sign, and small maxillae resulting in shallow depression of the central part of the face. [ncbi.nlm.nih.gov]

  The most important signs are frontal bossae (bony prominences of the forehead) (87%), saddle nose (74%), short maxilla (83%), high arched palate (76%), mulberry molars (more than four small cusps on a narrow first lower molar of the second dentition), [dartmouth.edu]

  Common late manifestations (appear > 2 yrs of age): Teeth -Hutchinson teeth (notched, pegged), mulberry molars Eye -interstitial keratitis Cranial nerves -8th nerve deafness Skeletal -frontal bossing, high arched palate, saddle nose (markedlydepressed [johnsarm.net]

  arched palate, short upper jawbone, nerve deafness and fissuring around the mouth and anus. [rarediseases.org]

  High arched palate 6. “Bull dog jaw” (prominent mandible) 7. “ Sabre tibia” 8. Scaphoid shape of the scapula 9. [slideshare.net]

• Notched Incisors

  Infection of the enamel causes notched incisors (Hutchinson’s teeth). In the liver large areas of parenchymal cells are separated by loose fibrous connective tissue (hepar lobatum). [histopathology-india.net]

  […] part of nose) hard palate defect swollen knees saber shins short maxillae protruding mandible A frequently-found group of symptoms is Hutchinson's triad, which consists of Hutchinson's teeth (notched incisors), keratitis and deafness and occurs in 63% [en.wikipedia.org]

  Syphilitic vasculitis can damage developing tooth buds, leading to peg-shaped, notched incisors (Hutchinson teeth) and multicusped first molars (mulberry molars). Deciduous teeth are not affected. [clinicaladvisor.com]

• Petechiae

  […] imaging in 70% of symptomatic infants (e.g., periventricular calcifications ) Hydrocephalus, intraventricular hemorrhage Microcephaly Sensorineural hearing loss ( ∼ 30% ) Chorioretinitis ( ∼ 10% ) Nonspecific findings (similar to other TORCH infections): petechiae [amboss.com]

  Du Bois sign, narrowing of the little finger poorly developed maxillae. enlarged liver. enlarged spleen. petechiae. other skin rash. sabre shins. anemia. lymph node enlargement. jaundice. pseudoparalysis. snuffles, aka "syphilitic rhinitis", which appears [en.wikipedia.org]

  These include: Fever Anemia Jaundice Deafness Irritability Petechiae Skin rashes Sabre shins Joint swelling Enlarged liver Frontal bossing Enlarged spleen Pseudoparalysis Abnormal x-rays Lymph node enlargement Poorly developed maxillae Failure to gain [hxbenefit.com]

  Petechiae may be present with severe thrombocytopenia. Most instances of neurosyphilis in neonates are asymptomatic, with diagnosis based on abnormal cerebrospinal fluid (CSF) results (see below). [clinicaladvisor.com]

• Saber Shin

  The first is a depressed deformity of the bridge of the nose (saddle nose), and the second is anterior bowing of the tibias (saber shins). Infection of the enamel causes notched incisors (Hutchinson’s teeth). [histopathology-india.net]

  shin Bilateral forward bowing of tibia is conclusive evidence for acquired, tertiary syphilis although similar changes can occur with congenital form Acquired syphilis affects skull (mixed sclerosis and lysis) Clavicle and tibia [learningradiology.com]

  Late manifestations (age 3 or older) result from scarring during early systemic disease and include Hutchinson teeth, Mulberry molars, abnormal facies, cranial nerve VIII deafness, bony changes such as saber shins and perforations of the hard palate, [aao.org]

  Bony changes include sclerosing lesions, saber shin, frontal bossing and gummatous or destructive lesions within long bones. [hon.ch]

• Corneal Opacity

  Eyes revealed dense corneal opacities with complete loss of vision in right eye. Left eye was normal. Patient was edentulous. VDRL test was reactive in dilution and TPHA was positive. Ultra sound showed hepatosplenomegaly with left renal cyst. [ijdvl.com]

  Stigmata due to late congenital syphilis are perforation of palate, corneal opacities, optic atrophy and periosteal changes of tibia. [6] The transmission of infection to the fetus can occur at any time, but in most of the cases it occurs in utero. [ijstd.org]

• Visual Impairment

  Other affected organs may include liver, kidneys, central nervous system ( CNS ), joints and eyes (resulting in visual impairment). Secondary syphilis See more images of syphilis. [dermnetnz.org]

• Frontal Bossing

  Secondary Syphilis in adults Triad Hutchinson Teeth (1 st lower molors) Deafness (8 th Nerve) Interstitial Keratitis Lymphadenopahy Autoimmune Haemolytic anemia Osteochondritis and Metaphysitis Glaucoma Pseudoparalysis of parrot (Painful limbs) Saddle Nose Frontal [mediconotebook.com]

  Common late manifestations (appear > 2 yrs of age): Teeth -Hutchinson teeth (notched, pegged), mulberry molars Eye -interstitial keratitis Cranial nerves -8th nerve deafness Skeletal -frontal bossing, high arched palate, saddle nose (markedlydepressed [johnsarm.net]

  Mulberry molars (permanent first molars with multiple poorly developed cusps). [6] frontal bossing. [en.wikipedia.org]

  These include: Fever Anemia Jaundice Deafness Irritability Petechiae Skin rashes Sabre shins Joint swelling Enlarged liver Frontal bossing Enlarged spleen Pseudoparalysis Abnormal x-rays Lymph node enlargement Poorly developed maxillae Failure to gain [hxbenefit.com]

  Bony changes include sclerosing lesions, saber shin, frontal bossing and gummatous or destructive lesions within long bones. [hon.ch]

• Genital Lesions

  The mother gave a history of taking some treatment for a genital lesion two years prior to the birth of the patient, The records of the above-mentioned condition or any treatment taken were not available. [jmedicalcasereports.biomedcentral.com]

  The majority of infants with congenital syphilis are infected in utero after the fourth month of gestation [2], but infection can occur as early as nine weeks’ gestation [6] or via contact with an active genital lesion at the time of delivery. [cps.ca]

She was asymptomatic; there was no evidence of active disease on either clinical examination or serology and no treatment was required. However, it is important to differentiate this condition from others which may require treatment. [ncbi.nlm.nih.gov]

To what extent treatment or time alone contribute to improvements cannot yet be stated. [pediatrics.aappublications.org]

King, Ambrose, “Discussion on the Treatment of Congenital Syphilis”, Brit. Journ. of Ven. Dis ., 1934, x, p. 25. [bjp.rcpsych.org]

Variations in Treatment Timeline Within the literature, there are notable variations from the aforementioned CDC/WHO treatment guidelines. [medicalreview.columbia.edu]

Babies who get infected while passing through birth canal generally have a better prognosis. Congenital Syphilis Prevention Safer sexual practices can help to prevent the development of this disease. [hxbenefit.com]

Prognosis is generally good unless there are established non-reversible changes associated with tertiary syphilis 1,2. Treatment of congenital syphilis is also with bezathine penicillin G 19. [radiopaedia.org]

The prognosis is very much better in those presenting in the postneonatal period. [4] In a fetus, the neonate, and the very young infant, bone abnormalities include osteochondritis, diaphyseal osteomyelitis (osteitis), and periostitis. [2] Metaphysitis [ijri.org]

Prognosis Congenital infection can lead to spontaneous abortion or fetal demise or stillbirth. These pregnancy complications are preventable through appropriate prenatal care. [clinicaladvisor.com]

Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]

Manifestation code identifies conditions for which it is important to record both the etiology and the symptom of the disease. [books.google.com]

(Etiology) Syphilis is caused by the bacterium Treponema pallidum. Congenital transmission of syphilitic infection that takes place inside the uterus, from a mother to the infant child, causes Congenital Syphilis. [dovemed.com]

Etiology The causative organism Treponema pertenue is identical morphologically with T. palladium of syphilis. Flies of the genus Hippelates transmit infections to human beings. [drmhijazy.com]

Ongoing controversies regarding etiology, diagnosis, treatment Syphilis is uncommon among international adoptees, but congenital syphilis can go undiagnosed or may be inadequately treated in some countries. [clinicaladvisor.com]

Late congenital syphilis was diagnosed in a 22-year-old woman on the basis of clinical, serologic and epidemiologic features. [ncbi.nlm.nih.gov]

References: [7] [8] [9] [10] [11] [12] [13] Congenital and newborn listeriosis Epidemiology : Incidence in US: 3/100,000 births Pathogen : Listeria monocytogenes Transmission Clinical features Intrauterine transmission to the fetus ↑ Risk of premature [amboss.com]

Skin -rhagades(cracks, fissures atmucocutaneousjunctions) #1, 2, & 3 are part of Hutchinson’s triad Extended discussion Epidemiology (Treponema Pallidum): Transmitted at any time during pregnancy or at birth Transmitted at any disease stage Almost 100% [johnsarm.net]

"Syphilis: Review with Emphasis on Clinical, Epidemiologic, and Some Biologic Features". Clinical Microbiology Reviews. 12 (2): 187–209. PMC 88914. [en.wikipedia.org]

Epidemiology There are an estimated 10.6 million incident cases of syphilis worldwide each year [ 2 ]. There were 5,288 new diagnoses of syphilis in England in 2015, a 19.9% increase from 4,412 in 2014. [patient.info]

Maternal syphilis: pathophysiology and treatment. Bull World Health Organ. 2004;82:433-38. Saloojee H, Velaphi S, Goga Y, et al. The prevention and management pof congenitalsyphilis: an overview and recommendations. [rarediseases.org]

While the gestational outcomes of syphilis have been well described, the specific pathophysiologic changes occurring in utero have not been clearly elucidated. [medicalreview.columbia.edu]

Special attention is given to the prevention and treatment of these diseases found in developing countries as well as the latest findings about new antimicrobial agents, gram-negative infections and their management, and recommendations for immunization [books.google.com]

Congenital syphilis is not always preventable. In cases when congenital syphilis cannot be prevented, the impact on the health of the baby can be mitigated with proper testing, treatment, and care before and shortly after delivery. [astho.org]

We are reporting here a case of late congenital syphilis in order to emphasize that congenital syphilis still exists in 21 st century and global antenatal screening is mandatory to prevent this serious, yet largely preventable disease. [jmedicalcasereports.biomedcentral.com]

By this early diagnosis and systemic phase of treatment helped us in preventing a child from further complications, which could have been fatal. [jisppd.com]

Primary prevention includes vaccination for varicella and rubella (prior to pregnancy ), screening for syphilis, and hygiene measures (washing hands, and avoiding certain foods) during pregnancy. [amboss.com]