CLINICAL MANIFESTATIONS OF CONGENITAL SYPHILIS
Dr Ira Shah
Organism: Treponema pallidum belonging to spirochetes

Transmission: Sexual contact (adults), Transplacental passage Contact with an infectious lesion during passage from birth canal.

High risk groups:

=> Unmarried mother Teenage mother Inadequate prenatal care Drug use in mother / sexual partner Sexual promiscuity Sexual contact with a known case of Sexually Transmitted Disease

=> History of sexually transmitted disease

Pathology & Pathogenesis:

Infection through placenta leads to hematogenous spread to all organs. On Histology, lesions show perivascular infiltration of lymphocytes, plasma cells & histiocytes with obliterative endarteritis & extensive fibrosis.

Appearance of placenta:
It is paler, thicker & larger than normal. Histology shows focal inflammation of the villus with endovascular & perivascular proliferation & relative immaturity of the villi. Villi become enlarged & have bullous projections. There are larger villi in relation to blood and hence appear pale.

Stillborn syphilitic infant:

Macerated Collapsed skull Protuberant abdomen Skin shows vesicular / bullous lesions fluid rich in treponeme Hepatosplenomegaly
X-ray periostitis / Osteochondritis.

Organs involved :

Liver - Inflammation in interstitial stroma & perivascular network. Spleen - enlarged because of extensive nonspecific inflammatory reaction as well as extramedullary hematopoiesis. Lung - "Pneumonia alba". There is marked increase in the amount of connective tissue in the inter alveolar septa & interstitium with collapse & loss of alveolar spaces. Stomach & intestine: Submucosal inflammation & fibrosis (More in the small intestine) Pancreas - Pancreatitis Kidneys: Immune complex deposition CNS: Discoloration & thickening of basilar meninges. Endarteritis. When infection resolves it leads to fibrosis, adhesions leading to obstructive hydrocephalus and cranial nerve palsies. Pituitary: Interstitial inflammation & fibrosis of anterior lobe of pituitary. Bone: Osteochondritis, Periostitis, Osteomyelitis especially of long bones & ribs.
Blood / Blood forming tissues: Initially, severe anemia followed by chronic progressive anemia due to marrow hypoplasia.

Stigma of syphilis in a pregnant mother :

Genital chancres (300 weeks after contact) Extragenital lesions - lips, tongue, nipple, finger, anus. Skin rash - in adults usually macular, papular, follicular, papulosquamous, pustular. Condyloma lata Mucus patches Lymphadenopathy Constitutional symptoms.
Less common - hepatitis, Osteitis, iritis, meningitis

EARLY CONGENITAL SYPHILIS

Hepatosplenomegaly: (33% have jaundice). Seen in nearly all infants. Hepatomegaly may occur in the absence of splenomegaly. Generalized lymphadenopathy (50%): Non-tender, ulcer especially epitrochlear Hematological: Anemia, leukopenia, leucocytosis, thrombocytopenia (Coombs negative hemolytic anemia) in new born period Mucocutaneous (15-60%): Snuffles (after 1st week up to 3 month of life). If ulceration of nasal mucosa deep it leads to "Saddle nose deformity" Skin: maculopapular oval rash, pink/red later becomes coppery-brown with very fine superficial desquamation and scaling on palms and soles. Lesion more posteriorly than anteriorly. Jaundice Petechiae Generalized Edema Bone: Pseudoparalysis of parrot. Renal: Usually at 2-3 month of age. Edema. CNS: Acute syphilitic leptomeningitis (3-6 months of age). CSF has aseptic meningitis picture with up to 200 mononuclear cells, increased proteins (50-200mg/dl) and normal glucose. CSF VDRL is positive. Chronic meningovascular syphilis is seen after several years and presents as progressive hydrocephalus, cranial palsies and vascular lesions. Ocular: Rare in early syphilis. Presents as chorioretinitis, salt & pepper fundus, glaucoma, uveitis, cataract and eyelid chancre. IUGR Other findings: Failure to thrive (marasmus)
Pneumonia alba

LATE CONGENITAL SYPHILIS

They are malformation or stigmata that represent scars induced by initial lesions of early congenital syphilis or reaction to persistent / ongoing inflammation. Hutchinson's triad (Hutchinson's teeth, Interstitial keratitis, eight nerve deafness) is common.

Dentition: due to vasculitis at the time of birth causing damage to the developing tooth bud. Leads to Hutchinson's teeth (permanent upper peg-shaped central incisors) and Mulberry molars (many small cusps). Early treatment prevents dental changes. Eyes: Interstitial keratitis at 5-20 yrs age. Healed chorioretinitis. Secondary glaucoma (uveitis). Corneal scarring.

Ear: 8th nerve deafness (only 3%). Osteochondritis affecting the otic capsule may lead to cochlear degeneration & fibrous adhesions.

Nose & Face: Failure of maxilla to grow fully leads to concave configuration of middle section of face + relative protuberance of mandible & associated high palatal arch. Saddle-nose is seen.

Skin: "Rhagades" early linear scans around body orifices. CNS: Mental Retardation, arrested hydrocephalus, convulsion, cranial nerve abnormality.

Bones & joints: Because of periosteal reaction there is frontal bossing (Olympian brow), saber shin (tibia), Higoumenaki's sign (sternoclavicular portion of clavicles) and Clutton's joints.

X-ray Findings:

Osteomyelitis (Osteitis like dystrophy) Osteochondritis (metaphyseal dystrophy) Periostitis (periosteal dystrophy). "Wimberger's sign" - lesion on the upper medial aspect of tibia (occurs bilaterally) D / D of Wimberger's sign: Osteomyelitis, Hyperparathyroidism, Infantile generalized fibromatosis. "Celery stick" appearance: Radiolucent areas in cortex appearing as columns. D / D of celery stick: Rubella, CMV. The inflammatory reaction in diaphysis stimulates periosteum to lay down new bone.
How to Cite URL :
Shah I D. CLINICAL MANIFESTATIONS OF CONGENITAL SYPHILIS. Pediatric Oncall [serial online] 2006[cited 2006 January 1];3. Art #12. Available From : http://www.pediatriconcall.com/Journal/Article/FullText.aspx?artid=766&type=J&tid=&imgid=&reportid=359&tbltype=
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