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Year : 2005  |  Volume : 15  |  Issue : 1  |  Page : 53-57
Different radiological presentations of congenital syphilis : four cases.


Dept. Of Radiodiagnosis & Imaging, B.J.Medical College, Civil Hospital, Asarwa, Ahmedabad-380016., India

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How to cite this article:
Sharma M, Solanki R N, Gupta A, Shah A K. Different radiological presentations of congenital syphilis : four cases. Indian J Radiol Imaging 2005;15:53-7

How to cite this URL:
Sharma M, Solanki R N, Gupta A, Shah A K. Different radiological presentations of congenital syphilis : four cases. Indian J Radiol Imaging [serial online] 2005 [cited 2019 Jul 16];15:53-7. Available from: http://www.ijri.org/text.asp?2005/15/1/53/28745

   Introduction Top


We wish to report, "Different Radiological Presentations of Congenital Syphilis - Four cases" diagnosed by Plain Radiography and confirmed by immunoassay (VDRL).

The incidence of syphilis is on a decline in Western Europe and North America while figures for developing countries though not very reliable also suggest steady decline. The rate of transmission from an infected partner is 30%. [1]

Congenital syphilis presents as Early congenital or Late congenital. Early congenital form occurs in pregnant women with untreated early or latent syphilis. The risk of transmission is highest with primary and secondary syphilis during pregnancy, and diminishes as the duration of latent syphilis increases.


   Case reports Top


CASE NO. 1:

A five days old preterm male child with history of vaginal delivery presented with complaints of abdominal distension, low-grade fever and excessive crying. On general examination there was no pallor, jaundice, lymphadenopathy, maculopapular rash or hepatosplenomegaly.

'Radiograph of chest with both upper & lower limbs' [Figure - 1][Figure - 2] was done which shows bilaterally symmetrical multiple areas of erosion and destruction of the metaphyses of proximal and distal humerus, femur and tibia suggestive of changes of bilaterally symmetrical OSTEOCHONDRITIS.

CASE NO. 2:

A two months old male child presented with complaints of decreased urine output and excessive crying. At birth and during perinatal period patient had history of septicemia, which responded to the penicillin group of antibiotics, given supposedly for bacterial peritonitis and septicemia. On general examination there was no pallor, jaundice, lymphadenopathy, maculopapular rash or hepatosplenomegaly.

'Radiograph of chest and both upper & lower limbs' [Figure - 3][Figure - 4] showed asymmetrical areas of erosion and destruction involving the proximal and distal metaphysic of right humerus, distal metaphysis of left femur suggestive of changes of bilaterally asymmetrical OSTEOCHONDRITIS with Wimberger's sign.

CASE NO. 3:

A two and half years old male child presented with pain and tenderness in right upper limb following some insignificant trauma in form of sudden jerk while playing. 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.

'Radiograph of chest and both upper & lower limbs' [Figure - 5][Figure - 6] showed permeative pattern of bone destruction in the diaphyseal region radius & ulna, distal humerus, proximal tibia & fibula on both sides. There is evidence of periosteal reaction noted along the diaphysis of femur, tibia, humerus, radius and ulna on both sides. There is evidence of pathological fracture noted through proximal

end of left tibia. The radiological bone age of the patient appears to be less than the chronological age i.e. between six months and one year (appearance of epiphysis of two carpal bones and non-appearance of epiphysis for distal end of radius). Findings suggested changes of OSTEOCHONDRITIS, PERIOSTITIS, PATHOLOGICAL FRACTURE and RETARDED BONE AGE.

CASE NO. 4:

A twenty-five days old male child presented with complaints of pain and swelling in both lower limbs. There is exaggeration of complaints while movements especially while extension. On general examination there was no pallor, jaundice, lymphadenopathy, maculopapular rash or hepatosplenomegaly.

'Radiograph of chest and both upper & lower limbs' [Figure - 7][Figure - 8] showed metaphyseal lucency with erosion along the distal end of right femur, proximal end of left tibia and large area of erosion and destruction at the distal metaphysis of left radius suggestive of changes of OSTEOCHONDRITIS and OSTEITIS.


   Investigations Top


Laboratory investigations showed normal hemoglobin, WBC, platelet counts and fundus examination revealed no abnormality but VDRL test for the patient and the mother were reactive.

IMPRESSION:

SUGGESTIVE OF CONGENITAL SYPHILIS



   Discussion Top


Etiopathogenesis:

Syphilis is caused by T. pallidum, one of a small group of treponemas pathogenic to man. It is a spiral organism 6- 15 m in length and 0.15 m in width, visible by light microscopy only under conditions of dark-field illumination, and cannot be grown on artificial media. [1]

T. pallidum has not been shown to produce either endotoxins or exotoxins. An initial polymorphonuclear response is soon replaced by an infiltrate of T and B lymphocytes. Both circulating T. pallidum specific T cells and specific antibody can be found in majority of the cases

of primary syphilis. Much of the pathology of secondary syphilis is immune complex mediated. Eventually cellmediated immune responses to T. pallidum are restored as the lesions are brought under control, leading to latent stage. [1]

In case of congenital syphilis the disorder originates from transplacental migration of the treponemas and invasion of the perichondrium, periosteum, cartilage, bone marrow, and sites of active endochondral ossification. The spirochetes inhibit osteogenesis. [1]

Clinical Features:

The fetus that is infected with spirochetes may be aborted or die shortly after birth. Other fetuses may survive developing the stigmata of the congenital syphilis. Studies conducted in the pre-antibiotic era found that untreated early syphilis in the mother lead to stillbirth in 25% of cases, neonatal death in some 15% of cases and a syphilitic infant in about 40% of cases. [1]

Signs of congenital syphilis in a neonate include a bullous rash, anemia, jaundice and hepatosplenomegaly. The infant is often small for dates and may have feeding difficulties. More commonly the syphilitic infant appears normal at birth, and presents in the first three months of life with: failure to thrive, a rash resembling that of secondary syphilis, with desquamation involving palms and soles, persistent nasal discharge; and anemia or hepatosplenomegaly. [1]

Late congenital syphilis in a child or an adolescent corresponds to tertiary syphilis in an adult. Manifestations include hutchinsonian triad, consisting of Hutchinson's teeth, interstitial keratitis, and nerve deafness may appear. Additional manifestations include fissuring about mouth and anus, anterior bowing of the lower leg, saddle nose, and perforation of palate. [2]

Radiological Findings:

Congenital syphilis has been divided on the basis of the presentation of the child clinically as well as radiological manifestation as EARLY & LATE congenital syphilis. LATE congenital syphilis in as child or adolescent corresponds to tertiary syphilis in an adult and radiological changes resemble the changes observed in acquired syphilis. [1]

Bone disease, although usually asymptomatic, is the most common early manifestation, having been reported in 97 percent of autopsied infants younger than 6 months of age. In many infants, healing occurs regardless of treatment. The long bones are predominantly affected. [3] Periostitis of the long bones, with or without metaphyseal abnormalities, is radiologically evident in more than 90% of cases, and may present clinically as pseudoparalysis of one or more limbs. 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 and periostitis are the commonest radiological findings. Metaphysitis is seen in 76.3%. Its degree varies from clear-cut zones of increased density, with or without a zone of rarefaction, to complete disorganization of the metaphysis. Periostitis is seen in 69.1% of cases. Osteitis is noted only in 6.0% cases. [5] We are presenting here the cases, which showed presence of radiological presentations varying from metaphysitis and periostitis (common) and osteitis (rare).

(1) Syphilitic osteochondritis (metaphysitis) usually results in symmetric involvement of sites of endochondral ossification. The epiphysealmetaphyseal junction of tubular bones, the costochondral regions, and in severe cases, the flat bones is affected. In the growing metaphysis of the long bones, widening of the provisional calcification zone, serrations, and adjacent osseous irregularity are seen. Erosive lesions develop along the contour of the bone at the metaphyseal-growth plate junction. [2] The most common osseous lesion, osteochondritis, is diagnosed by its characteristic radiographic "sawtooth" appearance in the metaphysis. In the medial aspect of the proximal tibial metaphysis, this defect occurs in one-fifth of early cases and has been called the Wimberger's or catbite sign. [3]

(2) Diaphyseal osteomyelitis (osteitis) relates to the extension of granulation tissue. Osteolytic lesions with surrounding bony eburnation and overlying periostitis are seen. [2]

(3) Periostitis can relate to infiltration by syphilitic granulation tissue. The long bones and, less commonly, the flat bones are affected. Reparative periostitis is a second variety of periosteal response that is noted about healing foci of osteochondritis or after epiphyseal slipping. [2]

(4) Other uncommon radiological features that may be seen are Syphilitic dactylitis (rare) and Skull lesions that may be purely sclerotic or may present as a combination of sclerosis and osteolysis. In purely sclerotic lesions, new bone may be laid down in the frontal and parietal regions, so producing the 'hot cross bun' skull. [6]


   Conclusion Top


As per the cases mentioned above, there are varied radiological presentations of congenital syphilis. No two cases mentioned above had the same clinical or radiological findings. The Above-mentioned patients presented with radiological features of OSTEOCHONDRITIS- bilaterally symmetrical or asymmetrical with/without PERIOSTITIS and/or OSTEITIS.

 
   References Top

1.Mabey D, Richens J. Sexually Transmitted Diseases (Excluding HIV). In: Cook G. Manson's Tropical Diseases, 20th ed. W.B. Saunders Company, 1996: 336-340.  Back to cited text no. 1    
2.Resnick D. Osteomyelitis, Septic Arthritis, and Soft Tissue Infection: Organisms. In: Resnick D, Bone and Joint Imaging, 2nd ed. W.B. Saunders Company, 1996, 1989: 697-699.  Back to cited text no. 2    
3.Azimi P, Syphilis. In: Behrman RE, Kliegman RM, Jenson HB, Nelson Textbook of Pediatrics, 17th ed. Saunders, 2004: 978-982  Back to cited text no. 3    
4.Hira SK, Bhat GJ, Patel JB et al. Early Congenital Syphilis: Clinicoradiologic features in 202 patients. Sex Transm Dis 1985; 12: 177-183.  Back to cited text no. 4    
5.Sachdev M, Bery K, Chawla S: Osseous manifestations in congenital syphilis: A study of 55 cases. Clin Radiol 1982; 33: 319.  Back to cited text no. 5    
6.Renton P. Periosteal Reaction; Bone and Joint infection; Sarcoid. In: Sutton D, Textbook of Radiology and Imaging, 6th ed. Churchill Livingstone, 1998:56-57.  Back to cited text no. 6    

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Correspondence Address:
M Sharma
C-6, Sudarshan Towers, Near New Nikita Park Society, Sun-n-Step Club Road, Thaltej, Ahmedabad-380054
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-3026.28745

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    Figures

[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7], [Figure - 8]



 

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    Introduction
    Case reports
    Investigations
    Discussion
    Conclusion
    References
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