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)
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.
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=|