Volume 6, Case 4
Jennifer M. Ragsdale, MD
Shawn N. Gentry, MD
Martin I. Herman, MD
LeBonheur Children's Medical Center
University of Tennessee School of Medicine
This is a 2-1/2 month old female whose mother brought her to the ED with a chief complaint of refusing to use her right arm since she had awoken that morning. Also, the child had been noted to be warm to touch for one day, but her temperature was not checked. Mother had also observed that the child's right wrist appeared slightly swollen and red for one day. The patient had spent the previous day in the care of her aunt. No history of trauma was given. Four days prior, she had a one day episode of fever occurring approximately 24 hours after receiving her first set of immunizations. Her oral intake was good. Urinary output was normal. No vomiting or diarrhea. She had a 2-3 day history of clear nasal drainage.
PMH - She was a full term vaginal delivery without complications. Birth weight 3.5 kg. Prenatal history was significant for maternal syphilis for which she was treated.
Exam showed a healthy appearing female infant. The exam was normal except for a seemingly tender right wrist (cries with palpitation). No deformity was noted. No tenderness in right upper arm. No skin abnormalities. Radiographs of the wrist were obtained.
View wrist radiographs.
Question: This radiograph shows? (1) lytic lesions, (2) artifacts, (3) surgically induced lesions from internal fixation devices, (4) pathologic fracture, (5) normal bone
Question: What do you think is the cause of these findings? (1) fibrous dysplasia, (2) enchondroma, (3) giant cell tumor, (4) lymphoma, (5) congenital syphilis, (6) osteomyelitis
A skeletal survey is obtained.
View shoulder radiographs.
View elbow radiographs
View lower extremity radiographs
There is soft tissue swelling of the right wrist with destructive lytic changes of the metaphyses of the right radius and ulna. There is periosteal reaction of the distal ulna and possibly the distal radius.
The shoulder radiographs are probably normal.
The elbow radiographs show some mild periosteal reaction of the humerus which is difficult to appreciate.
The lower extremities show periosteal reaction of the femurs and tibiae. There are destructive metaphyseal abnormalities of the medial aspects of the tibiae. These findings are compatible with the Wimberger sign of congenital syphilis. There is scalloping and destruction of the upper medial tibial metaphyses bilaterally with diaphyseal periosteal new bone deposition. There is also some destruction of the left fibula proximally with periosteal reaction.
Radiographs of the skull (not shown here) showed no intracranial calcifications. The sella turcica was normal. Radiographs of the spine, ribs, chest and abdomen were normal.
Discussion: One must consider several etiologies of cystic/lytic lesions on pediatric radiographs; several which predispose to pathologic fractures. The differential diagnosis includes: simple bone cyst (lucent lesion, sharply demarcated, presents as pathologic fracture), fibrous dysplasia (radiolucent area in long bones, beginning in early childhood), giant cell tumor (eccentric lucent lesion in metaphysis of long bones, not involving the joint, 20% malignant), enchondroma (benign, lucent, well-demarcated near epiphysis in children and adults), chondroblastoma (rare, benign eccentric oval lucency in epiphysis), lymphoma, infection (fungal infection, cystic osteomyelitis), angiomatous lesion (lucent metaphyseal lesion, rare congenital malformation) and syphilis of the bone.
Syphilis of the bone is rare. There are two forms. The infantile form involves the metaphyseal regions of multiple bones. The juvenile form is diffuse or localized with subperiosteal thickening. This case most likely represents a syphilitic lesion because of the location of the lucent area and the mother's history of syphilis during pregnancy.
The etiology of syphilis is the spirochete Treponema pallidum. Syphilis is described in three stages. Stage one is the manifestation of the painless chancre usually found on the genitalia. If untreated, within a few months, syphilis develops into stage two in which the patient develops a maculopapular rash which is generalized and often involving the palms and soles. During stage two the patient often has flu-like symptoms including malaise, headache, lymphadenopathy, fever, and arthralgias. These symptoms of stage two may wax and wane over a period of many years. The tertiary stage manifests years later as neurosyphilis, aortitis, and gummatous changes of the skin, bone, and viscera. Transmission of the spirochete is via sexual contact or transplacentally. The risk of transplacental transmission is nearly 100% during the second stage of syphilis and slowly decreases thereafter. Fetuses infected with syphilis may develop hydrops fetalis and may be premature or stillborn. Congenital syphilis may present in a variety of ways. Common features include rhinitis, the saddle bridge nose, and Hutchison teeth. Other clinical findings include hepatosplenomegaly, lymphadenopathy, neurocutaneous lesions, osteochondritis, hemolytic anemia, and thrombocytopenia. Treatment for congenital syphilis is 10-14 days of IV penicillin G. If more than one day of therapy is missed the entire course must be restarted.
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