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Special Feature |

Radiological Case of the Month FREE

[+] Author Affiliations

Section Editor: Beverly P. Wood, MD

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Arch Pediatr Adolesc Med. 2001;155(5):613-614. doi:10.1001/archpedi.155.5.613.
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DENOUEMENT AND DISCUSSION: CONGENITAL SYPHILIS PRESENTING AS OSTEOMYELITIS WITH NORMAL RADIOISOTOPE BONE SCAN

Figure 1. Axial computed tomogram of the left humerus, performed on the 10th hospital day, shows extensive areas of osteolysis and cortical destruction of the proximal metaphysis. There is soft tissue swelling.

Figure 2. Frontal radiographs of the shoulders on the 10th hospital day show osteolytic metaphyseal lesions of both proximal humeri.

Figure 3. Radiographs of the long bones of the lower extremities (10th hospital day) show bilaterally symmetrical diaphyseal periostitis and metaphysitis of both distal femora and proximal tibiae, a pathognomonic sign of congenital syphilis.

On the second hospital day, the infant's serum VDRL test was 1:16. It rose 16-fold and reached a dilution level of 1:256 by the 10th hospital day. This result was 4 times higher than her mother's serum VDRL test result (1:64). Findings from a serum microagglutination test for cerebrospinal fluid were normal, as were results of a cerebrospinal fluid VDRL test. The diagnosis of congenital syphilis was established. The infant was treated with intravenous cefuroxime sodium, 150 mg/kg daily, because hematogenous osteomyelitis was initially suspected. After the positive serum VDRL test result was reported, cefuroxime was replaced by intravenous aqueous crystalline penicillin G, 300 000 µm/kg daily, which was given for 14 days. The infant recovered completely.

The incidence of early congenital syphilis remains relatively high.1,2 Early diagnosis and timely treatment are important to prevent serious complications. Congenital syphilis usually manifests during the first 3 months after birth with signs of fever, prolonged rhinitis, erythematous maculopapular rash of the palms and soles, hepatosplenomegaly, pseudoparalysis, Coomb-negative hemolytic anemia, leukocytosis, and monocytosis.35 The definitive diagnosis is based on the results of a serum quantative nontreponemal test (VDRL or rapid plasma reagin) considered conclusive when the infant's titer is at least 4 times higher than that of the mother. The infant's antitreponemal immunoglobulin antibody test is also used for diagnosis.1 Bone involvement with bilaterally symmetrical periostitis and metaphysitis occurs in 95% of symptomatic infants.6,7 Asymmetric focal osteolytic metaphyseal lesions with sequestra or pathological fractures are also reported.8

To our knowledge, this is the first reported demonstration of diagnostic computed tomography of the skeletal lesions of early congenital syphilis. Computed tomography shows osteolysis and cortical destruction of the proximal left humerus and soft tissue swelling. These changes account for acute pain, which leads to pseudoparalysis in congenital syphilis.

Despite the clinical, laboratory, and later imaging signs, the 3-phase technetium 99m methylene diphosphonate bone scan in this patient repeatedly had negative findings. Increased accumulation radioactive tracer at the site of inflammation is characteristic of osteomyelitis.9 Lim et al10 reported a false-negative result from bone scan in a case of pseudoparalysis from congenital syphilis. The occurrence reflects the specific character of metaphyseal pathology. Histopathological analysis of the metaphysis in syphilis reveals an obliterative endarteritis secondary to binding of spirochetes to endothelial cells with inflammatory plasma cell–rich perivascular infiltration.1113 Spirochetes are identified in bone tissue by specific Dieterle stains.1,14 Obliterative vasculitis leads to decreased blood flow, atrophy, focal necrosis, and diffuse interstitial fibrosis.12,13,15 These changes may explain why the radioactive tracer does not accumulate at the site of syphilitic osseous lesions.

Accepted for publication June 27, 1999.

Reprints: Daniel Landau, MD, Department of Pediatrics B, Soroka Medical Center, PO Box 151, Beer Sheva 84101, Israel.

American Academy of Pediatrics, Syphilis. Peter  GHall  CBHalsey  NAMarcy  SMPickering  LKeds.1997 Red Book: Report of the Committee on Infectious Diseases 24th ed. Elk Grove Village, Ill American Academy of Pediatrics1997;504- 514
Kosher  MSCaniza  M Parrot pseudoparalysis of the upper extremities. J Bone Joint Surg Am. 1996;78284- 287
Rasool  MNGovender  S The skeletal manifestations of congenital syphilis: a review of 197 cases. J Bone Joint Surg Br. 1989;71752- 755
Sachdev  MBery  KChawla  S Osseus manifestations in congenital syphilis: a study of 55 cases. Clin Radiol. 1982;33319- 323
Link to Article
Dorfman  DHGlaser  JH Congenital syphilis presenting in infants after the newborn period. N Engl J Med. 1990;3231299- 1302
Link to Article
Giacola  GPWood  BP Congenital syphilis. AJDC. 1991;1451045- 1046
Brion  LPManuli  MDRai  BKresh  MJPavlov  HGlaser  J Long-bone radiographic abnormalities as a sign of active congenital syphilis in asymptomatic newborns. Pediatrics. 1991;881037- 1040
Solomon  ARosen  E Focal osseous lesions in congenital lues. Pediatr Radiol. 1978;736- 39
Link to Article
Mandell  GA Imaging in the diagnosis of musculoskeletal infections in children. Curr Prob Pediatr. 1996;26218- 237
Lim  HKSmith  WLSato  YChoi  J Congenital syphilis mimicking child abuse. Pediatr Radiol. 1995;25560- 561
Link to Article
Samuelson  JVan Lichtenberg  F Syphilis. Cotran  RSKumar  VRobbins  SLSchoen  FJeds.Robbins Pathologic Basis of Disease 5th ed. WB Saunders Co1994;343- 346
O'Shea  Not Available Congenital syphilis in a twin gestation. Pediatr Pathol. 1992;12865- 870
Link to Article
Froberg  MKFitzgerald  TJHamilton  TRHamilton  BZarabi  M Pathology of congenital syphilis in rabbits. Infect Immun. 1993;614743- 4749
Dzebolo  NN Congenital syphilis: an unusual presentation. Radiology. 1980;136372
Levine  ZSherer  DMJacobs  ARotenberg  O Nonimmune hydrops fetalis due to congenital syphilis associated with negative intrapartum maternal serology screening. Am J Perinatol. 1998;15233- 236
Link to Article

Tables

References

American Academy of Pediatrics, Syphilis. Peter  GHall  CBHalsey  NAMarcy  SMPickering  LKeds.1997 Red Book: Report of the Committee on Infectious Diseases 24th ed. Elk Grove Village, Ill American Academy of Pediatrics1997;504- 514
Kosher  MSCaniza  M Parrot pseudoparalysis of the upper extremities. J Bone Joint Surg Am. 1996;78284- 287
Rasool  MNGovender  S The skeletal manifestations of congenital syphilis: a review of 197 cases. J Bone Joint Surg Br. 1989;71752- 755
Sachdev  MBery  KChawla  S Osseus manifestations in congenital syphilis: a study of 55 cases. Clin Radiol. 1982;33319- 323
Link to Article
Dorfman  DHGlaser  JH Congenital syphilis presenting in infants after the newborn period. N Engl J Med. 1990;3231299- 1302
Link to Article
Giacola  GPWood  BP Congenital syphilis. AJDC. 1991;1451045- 1046
Brion  LPManuli  MDRai  BKresh  MJPavlov  HGlaser  J Long-bone radiographic abnormalities as a sign of active congenital syphilis in asymptomatic newborns. Pediatrics. 1991;881037- 1040
Solomon  ARosen  E Focal osseous lesions in congenital lues. Pediatr Radiol. 1978;736- 39
Link to Article
Mandell  GA Imaging in the diagnosis of musculoskeletal infections in children. Curr Prob Pediatr. 1996;26218- 237
Lim  HKSmith  WLSato  YChoi  J Congenital syphilis mimicking child abuse. Pediatr Radiol. 1995;25560- 561
Link to Article
Samuelson  JVan Lichtenberg  F Syphilis. Cotran  RSKumar  VRobbins  SLSchoen  FJeds.Robbins Pathologic Basis of Disease 5th ed. WB Saunders Co1994;343- 346
O'Shea  Not Available Congenital syphilis in a twin gestation. Pediatr Pathol. 1992;12865- 870
Link to Article
Froberg  MKFitzgerald  TJHamilton  TRHamilton  BZarabi  M Pathology of congenital syphilis in rabbits. Infect Immun. 1993;614743- 4749
Dzebolo  NN Congenital syphilis: an unusual presentation. Radiology. 1980;136372
Levine  ZSherer  DMJacobs  ARotenberg  O Nonimmune hydrops fetalis due to congenital syphilis associated with negative intrapartum maternal serology screening. Am J Perinatol. 1998;15233- 236
Link to Article

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