Pediatric Radiology
Pediat. Radiol. 7, 232-234 (1978)
© by Springer-Verlag1978
Unusual Bone Involvement in Congenital Syphilis Mimicking the Battered Child Syndrome C. H o r o d n i c e a n u , M. G r t i n e b a u m , B. V o l o v i t z , a n d M. N i t z a n Pediatric Radiology Unit and Department of Pediatrics, Beilinson Medical Center, Petah Tiqva, and Tel-Aviv University Medical School, Israel
Abstract. C o n g e n i t a l syphilis is r e - e m e r g i n g in m o d e r n society. T h e p e d i a t r i c r a d i o l o g i s t m a y c o n t r i b u t e to its early diagnosis. A case o f u n u s u a l bone involvement with fracture of the olecranon a n d facial b o n e l e s i o n s is d e s c r i b e d ; t h e c o n d i t i o n h a d to be d i f f e r e n t i a t e d f r o m t h e " b a t t e r e d c h i l d s y n d r o m e " . T h e p r o b l e m o f c o n g e n i t a l syphilis as a n i n f e c t i o u s d i s e a s e v e r s u s b o n e d y s t r o p h y is also d i s c u s s e d .
Key words: Syphilis - B o n e s - F r a c t u r e - Syphilitic bone dystrophy - Battered child
I n the last two d e c a d e s t h e r e h a s b e e n a d e c r e a s e in t h e a w a r e n e s s o f syphilis. H o w e v e r , this d i s e a s e is taking o n a r e n e w e d i m p o r t a n c e [4, 10, 11] u n d e r m o d e r n c o n d i t i o n s o f life a n d the " m o r a l f r e e d o m " o f society. A s c o n g e n i t a l syphilis has m a n y w a y s o f p r e s e n t a t i o n , the pediatric r a d i o l o g i s t m a y c o n t r i b u t e to its e a r l y d e t e c t i o n . T h e p u r p o s e "~f this p r e s e n t a t i o n is to d e s c r i b e u n u s u a l b o n e i n v o l v e m e n t in a n i n f a n t w h o pres e n t e d as a " b a t t e r e d c h i l d " a n d to d i s c u s s t h e controversy of bone dystrophy versus bone infect i o n in this entity.
Case Presentation A six-week-old infant was admitted to the emergency room because of signs of pain in his extremities, particularly of his right forearm which was also swollen. The pregnancy of the mother was uneventful, and normal labor occurred after 40 weeks of gestation. The newborn infant was discharged from the maternity hospital without any remarks, weighing 3250 grams. On physical examination at six weeks of age there was limitation of movements of the extremities, especially of the
0301-0449/78/0007/0232/S01.00
right forearm where swelling was identified. No skin lesions or nasal discharge were apparent. The heart and lungs were normal. The liver was not palpable and the spleen was slightly enlarged. No lymphadenopathy was present. The various laboratory tests included hemoglobin 8.6 gm %, leukocytes 12,800, a reticuloeytosis of 3.7 % was present, and the platelet count was 546,000. Immunoelectrophorosis of the plasma proteins was: Igm - 130 rag%; IgA - 75 rag%; IgG - 520 rag%. A spinal tap was negative. Although no pertinent history of trauma was obtained, the parents' behavior and the clinical signs of the infant raised the suspicion of the "battered child syndrome". Because of the pain and swelling of the right forearm, an X-ray of this region was obtained and immediately followed by a skeletal survey. A fracture with periosteal-callus formation was noted at the proximal part of the ulna, beneath the coronoid process, in good alignment. Another fracture, without callus-formation was seen in the distal metaphyseal region of the ulna (Fig. 1). Multiple destructive lesions were identified throughout all metaphyses of the long bones. These osteolytic lesions were symmetrically distributed and located beneath the preserved provisional zone of calcification. No periosteal reaction was evident along the cortex of the diaphyses. Skull X-rays revealed an ill-defined osteolytic lesion at the medial aspect of the left supra-orbital margin and the superciliary ridge of the same side (Fig. 2). The nasal bone was absent and at its root an irregular erosive defect was seen (Fig. 3). The radiographic diagnosis was summarized as being consistent with congenital syphilitic bone involvement. At this point serological examinations were performed which included the Treponema pallidum immobilization test, the Wasserman and the VDRL tests, all of which were positive. The same serological tests were also performed in the parents, and were positive. It is worthwhile mentioning that the mother underwent serological examinations during the first and second trimesters of her pregnancy, these being negative. Penicillin treatment was started in the infant and his parents. After four months of treatment, the serological tests in the parents became negative. Despite the antibacterial treatment, since his first month of life, the serological examinations of the infant remained positive at the age of 15 months. Repeated skeletal radiograms of the patient, at the age of ten months, showed that all the metaphyseal lesions had
C. Horodniceanu et al.: Bone Involvement in Syphilis
233
Fig. 1. Six weeks of age. Anteroposterior view of the right elbow and forearm. Periosteal callus formation at the fracture site in the ulna. Fracture without callus formation at the distal metaphysis of the radius and ulna. No periosteal new-bone formation Pig. 2. Six weeks of age. Anteroposterior view of skull. Osteolytic lesion at the medial aspect of the left supraorbital margin and the supraciliary ridge 1.
3.
2.
4°
Fig. 3. A Six weeks of age. Lateral view of the facial bones. Absence of the nasal bone. B Ten months of age. Lateral view of the facial bones. Well formed nasal bone Pig. 4. Ten months of age. Antero-posterior view of the right hand and wrist. Disturbance of modelling and trabeculation of the distal metaphyses of the radius and ulna
234 disappeared with a normal trabecular pattern in the affected regions. The fractured area at the proximal end of the right ulna recovered without any deformity, but in the distal radius and ulna on the right side, disturbance in modelling and trabeculation persisted. The distal epiphyses of the radius and ulna appeared as if "buried" within the metaphyseal area (Fig. 4). The orbital lesions healed completely, and the nasal bone was well identified (Fig. 3 b). The roentgen report was concluded as showing no further evidence of a bone lesion to indicate active syphilis.
Comment The case presented is of interest from several points of view. Although demonstrating widespread symmetrical skeletal lesions, there were no clinical signs, whatsoever, indicating congenital syphilis. The pediatric radiologist may have elucidated the correct diagnosis [1, 3, 4, 6]. The infant was admitted with suspected diagnosis of the "battered child syndrome" and when considering this syndrome in an infant, one should include in the differential di~tgnosis the possibility of congenital syphilis complicated by fractures [5]. The symmetrical distribution of the metaphyseal involvement is in favor of the last diagnosis. It seems worthwhile performing serological tests, even during the third trimester of pregnancy in order to detect or prevent early disease in the newborn [5, 7, 8]. The radiographic changes we have described correspond well with the "osteitis like" dystrophy group II, type B and C of Cremin and Fisher's classification [4]. These changes are characterized by the typical Wimberger sign in the metaphyses as well as fractures through the "eroded" areas. Of interest are also the facial lesion in the orbital region and the absence of ossification in the nasal bone. Orbital lesions have been described as a rarity by several authors [1, 4]; however, a lesion of the nasal bone at this early age has not been mentioned so far. Normally, the nasal bone is already ossified at birth and is seen on the lateral facial or skull roentgenogram [2]. In this case, the nasal bone was still not ossified at the age of six weeks (Fig. 3a), and it could only be clearly seen at the age of ten months (Fig. 3 b). It is assumed that the delay in appearance was the result of the destructive-erosive process.
C. Horodniceanu et al.: Bone Involvement in Syphilis
The equal and symmetric distribution of the lesions with the well preseIved provisional zone of calcification favors the notion of bone changes in congenital syphilis as a bone-dystrophy disease rather than as a bone-inflammatory disease [1, 4]. In favor of bone-dystrophy changes, the following facts should be considered: no periosteal new bone formation along the eroded areas in the metaphyseal region; no porosis of the provisional zone of calcification; and no soft tissue swelling adjacent to the osteolytic lesions. The healing appearance of the right distal ulna ressembles a healed dystrophic lesion. All of the above mentioned criteria raise the question of whether a revision of congenital syphilis in its pathological classification is not necessary.
References 1. Caffey, J.: Pediatric X-ray diagnosis, 6th Ed. New York: Yearbook Medical Publishers 1973 2. Chasler, C. N.: Atlas of roentgen anatomy of the newborn and infant skull. St. Louis: W. H. Green 1972 3. Chipps, B. E., Swischuk, L. E., Woelter, W. W.: Single bone involvement in congenital syphilis. Pediatr. Radiol. 5, 50 (1976) 4. Cremin, B. J., Fisher, R. M.: The lesions of congenital syphilis. Br. J. Radiol. 43, 333 (1970) 5. Finmara, M. J.: Syphilis in newborn children. Clin. Obstet. Gynecol. 18, 1 (1975) 6. Fisher, R. H., Kaplan, J., Holder, J. C.: Congenital syphilis mimicking the battered child syndrome. Clin. Pediatr. (Phila.) 11,305 (1972) 7. Hoffman, E. D., Herwey, J. C.: States of serologic testing for congenital syphilis. J. Pediatr. 71, 686 (1967) 8. Rein, C. R., Reyn, A.: Serology of treponematoses, recent developments. Bull. WHO 14, 193 (1956) 9. Rosen, E. U., Solomon, A.: Bone lesions in early congenital syphilis. S. Afr. Med. J. 50, 135 (1976) 10. Solomon, A., Rosen, E.: The aspect of trauma in the bone changes of congenital lues. Pediatr. Radiol. 3, 176 (1975) 11. Ton, K. L.: The re-emergence of early congenital syphilis. Acta Paediatr. Scan& 62, 6 (1973)
Date offinal acceptance: January 12, 1978 C. Horodniceanu Pediatric Radiology Unit Beilinson Medical Center Petah Tiqva Israel
Pediat. Radiol. 7, 232-234 (1978)
© by Springer-Verlag1978
Unusual Bone Involvement in Congenital Syphilis Mimicking the Battered Child Syndrome C. H o r o d n i c e a n u , M. G r t i n e b a u m , B. V o l o v i t z , a n d M. N i t z a n Pediatric Radiology Unit and Department of Pediatrics, Beilinson Medical Center, Petah Tiqva, and Tel-Aviv University Medical School, Israel
Abstract. C o n g e n i t a l syphilis is r e - e m e r g i n g in m o d e r n society. T h e p e d i a t r i c r a d i o l o g i s t m a y c o n t r i b u t e to its early diagnosis. A case o f u n u s u a l bone involvement with fracture of the olecranon a n d facial b o n e l e s i o n s is d e s c r i b e d ; t h e c o n d i t i o n h a d to be d i f f e r e n t i a t e d f r o m t h e " b a t t e r e d c h i l d s y n d r o m e " . T h e p r o b l e m o f c o n g e n i t a l syphilis as a n i n f e c t i o u s d i s e a s e v e r s u s b o n e d y s t r o p h y is also d i s c u s s e d .
Key words: Syphilis - B o n e s - F r a c t u r e - Syphilitic bone dystrophy - Battered child
I n the last two d e c a d e s t h e r e h a s b e e n a d e c r e a s e in t h e a w a r e n e s s o f syphilis. H o w e v e r , this d i s e a s e is taking o n a r e n e w e d i m p o r t a n c e [4, 10, 11] u n d e r m o d e r n c o n d i t i o n s o f life a n d the " m o r a l f r e e d o m " o f society. A s c o n g e n i t a l syphilis has m a n y w a y s o f p r e s e n t a t i o n , the pediatric r a d i o l o g i s t m a y c o n t r i b u t e to its e a r l y d e t e c t i o n . T h e p u r p o s e "~f this p r e s e n t a t i o n is to d e s c r i b e u n u s u a l b o n e i n v o l v e m e n t in a n i n f a n t w h o pres e n t e d as a " b a t t e r e d c h i l d " a n d to d i s c u s s t h e controversy of bone dystrophy versus bone infect i o n in this entity.
Case Presentation A six-week-old infant was admitted to the emergency room because of signs of pain in his extremities, particularly of his right forearm which was also swollen. The pregnancy of the mother was uneventful, and normal labor occurred after 40 weeks of gestation. The newborn infant was discharged from the maternity hospital without any remarks, weighing 3250 grams. On physical examination at six weeks of age there was limitation of movements of the extremities, especially of the
0301-0449/78/0007/0232/S01.00
right forearm where swelling was identified. No skin lesions or nasal discharge were apparent. The heart and lungs were normal. The liver was not palpable and the spleen was slightly enlarged. No lymphadenopathy was present. The various laboratory tests included hemoglobin 8.6 gm %, leukocytes 12,800, a reticuloeytosis of 3.7 % was present, and the platelet count was 546,000. Immunoelectrophorosis of the plasma proteins was: Igm - 130 rag%; IgA - 75 rag%; IgG - 520 rag%. A spinal tap was negative. Although no pertinent history of trauma was obtained, the parents' behavior and the clinical signs of the infant raised the suspicion of the "battered child syndrome". Because of the pain and swelling of the right forearm, an X-ray of this region was obtained and immediately followed by a skeletal survey. A fracture with periosteal-callus formation was noted at the proximal part of the ulna, beneath the coronoid process, in good alignment. Another fracture, without callus-formation was seen in the distal metaphyseal region of the ulna (Fig. 1). Multiple destructive lesions were identified throughout all metaphyses of the long bones. These osteolytic lesions were symmetrically distributed and located beneath the preserved provisional zone of calcification. No periosteal reaction was evident along the cortex of the diaphyses. Skull X-rays revealed an ill-defined osteolytic lesion at the medial aspect of the left supra-orbital margin and the superciliary ridge of the same side (Fig. 2). The nasal bone was absent and at its root an irregular erosive defect was seen (Fig. 3). The radiographic diagnosis was summarized as being consistent with congenital syphilitic bone involvement. At this point serological examinations were performed which included the Treponema pallidum immobilization test, the Wasserman and the VDRL tests, all of which were positive. The same serological tests were also performed in the parents, and were positive. It is worthwhile mentioning that the mother underwent serological examinations during the first and second trimesters of her pregnancy, these being negative. Penicillin treatment was started in the infant and his parents. After four months of treatment, the serological tests in the parents became negative. Despite the antibacterial treatment, since his first month of life, the serological examinations of the infant remained positive at the age of 15 months. Repeated skeletal radiograms of the patient, at the age of ten months, showed that all the metaphyseal lesions had
C. Horodniceanu et al.: Bone Involvement in Syphilis
233
Fig. 1. Six weeks of age. Anteroposterior view of the right elbow and forearm. Periosteal callus formation at the fracture site in the ulna. Fracture without callus formation at the distal metaphysis of the radius and ulna. No periosteal new-bone formation Pig. 2. Six weeks of age. Anteroposterior view of skull. Osteolytic lesion at the medial aspect of the left supraorbital margin and the supraciliary ridge 1.
3.
2.
4°
Fig. 3. A Six weeks of age. Lateral view of the facial bones. Absence of the nasal bone. B Ten months of age. Lateral view of the facial bones. Well formed nasal bone Pig. 4. Ten months of age. Antero-posterior view of the right hand and wrist. Disturbance of modelling and trabeculation of the distal metaphyses of the radius and ulna
234 disappeared with a normal trabecular pattern in the affected regions. The fractured area at the proximal end of the right ulna recovered without any deformity, but in the distal radius and ulna on the right side, disturbance in modelling and trabeculation persisted. The distal epiphyses of the radius and ulna appeared as if "buried" within the metaphyseal area (Fig. 4). The orbital lesions healed completely, and the nasal bone was well identified (Fig. 3 b). The roentgen report was concluded as showing no further evidence of a bone lesion to indicate active syphilis.
Comment The case presented is of interest from several points of view. Although demonstrating widespread symmetrical skeletal lesions, there were no clinical signs, whatsoever, indicating congenital syphilis. The pediatric radiologist may have elucidated the correct diagnosis [1, 3, 4, 6]. The infant was admitted with suspected diagnosis of the "battered child syndrome" and when considering this syndrome in an infant, one should include in the differential di~tgnosis the possibility of congenital syphilis complicated by fractures [5]. The symmetrical distribution of the metaphyseal involvement is in favor of the last diagnosis. It seems worthwhile performing serological tests, even during the third trimester of pregnancy in order to detect or prevent early disease in the newborn [5, 7, 8]. The radiographic changes we have described correspond well with the "osteitis like" dystrophy group II, type B and C of Cremin and Fisher's classification [4]. These changes are characterized by the typical Wimberger sign in the metaphyses as well as fractures through the "eroded" areas. Of interest are also the facial lesion in the orbital region and the absence of ossification in the nasal bone. Orbital lesions have been described as a rarity by several authors [1, 4]; however, a lesion of the nasal bone at this early age has not been mentioned so far. Normally, the nasal bone is already ossified at birth and is seen on the lateral facial or skull roentgenogram [2]. In this case, the nasal bone was still not ossified at the age of six weeks (Fig. 3a), and it could only be clearly seen at the age of ten months (Fig. 3 b). It is assumed that the delay in appearance was the result of the destructive-erosive process.
C. Horodniceanu et al.: Bone Involvement in Syphilis
The equal and symmetric distribution of the lesions with the well preseIved provisional zone of calcification favors the notion of bone changes in congenital syphilis as a bone-dystrophy disease rather than as a bone-inflammatory disease [1, 4]. In favor of bone-dystrophy changes, the following facts should be considered: no periosteal new bone formation along the eroded areas in the metaphyseal region; no porosis of the provisional zone of calcification; and no soft tissue swelling adjacent to the osteolytic lesions. The healing appearance of the right distal ulna ressembles a healed dystrophic lesion. All of the above mentioned criteria raise the question of whether a revision of congenital syphilis in its pathological classification is not necessary.
References 1. Caffey, J.: Pediatric X-ray diagnosis, 6th Ed. New York: Yearbook Medical Publishers 1973 2. Chasler, C. N.: Atlas of roentgen anatomy of the newborn and infant skull. St. Louis: W. H. Green 1972 3. Chipps, B. E., Swischuk, L. E., Woelter, W. W.: Single bone involvement in congenital syphilis. Pediatr. Radiol. 5, 50 (1976) 4. Cremin, B. J., Fisher, R. M.: The lesions of congenital syphilis. Br. J. Radiol. 43, 333 (1970) 5. Finmara, M. J.: Syphilis in newborn children. Clin. Obstet. Gynecol. 18, 1 (1975) 6. Fisher, R. H., Kaplan, J., Holder, J. C.: Congenital syphilis mimicking the battered child syndrome. Clin. Pediatr. (Phila.) 11,305 (1972) 7. Hoffman, E. D., Herwey, J. C.: States of serologic testing for congenital syphilis. J. Pediatr. 71, 686 (1967) 8. Rein, C. R., Reyn, A.: Serology of treponematoses, recent developments. Bull. WHO 14, 193 (1956) 9. Rosen, E. U., Solomon, A.: Bone lesions in early congenital syphilis. S. Afr. Med. J. 50, 135 (1976) 10. Solomon, A., Rosen, E.: The aspect of trauma in the bone changes of congenital lues. Pediatr. Radiol. 3, 176 (1975) 11. Ton, K. L.: The re-emergence of early congenital syphilis. Acta Paediatr. Scan& 62, 6 (1973)
Date offinal acceptance: January 12, 1978 C. Horodniceanu Pediatric Radiology Unit Beilinson Medical Center Petah Tiqva Israel
