Higoumenakis’ sign in the diagnosis of congenital syphilis in anthropological specimens

Abstract

Higoumenakis' sign (HS) is a diagnostic criterion referring to the enlargement of the sternal end of the (right) clavicle, frequently observed in patients with late congenital syphilis. Although indexed for several years in clinical medicine textbooks, it has not been extensively applied for the diagnosis of congenital syphilis among anthropological specimens. This is highly significant, since the other major palaeopathology findings refer almost exclusively to the skull and diagnosis thus becomes difficult if only peripheral skeletal remains are available for evaluation. The potential effectiveness of the proposed use of HS as a marker of syphilis in anthropology appears adequate, since descriptions very similar to that of HS have been reported for certain findings, although no attempt has been made to correlate them with the presence of the disease. Higoumenakis himself originally observed this sign in 86% of his patients with congenital syphilis, and this report was subsequently verified by other independent researchers. His attempt to explain the pathophysiology of the sign and its localization, on the basis of anatomical, biological, and mechanical reasons, however, has been questioned. On the other hand, the application of the remaining markers of congenital syphilis is also problematic, due to sensitivity and/or specificity limitations, and other signs may not be detected due to inability to retain soft tissue samples in anthropological populations and a lack of reliable techniques for treponematous DNA amplification in such old samples. Thus, the fact that the onset of any of the signs of syphilis is not a constant finding justifies the authors' suggestion that HS should be checked for in any available anthropological specimen, because it is highly indicative of possible infection by Treponema pallidum.

Congenital Anatomical Variant of the Clavicle

“…Moreover, periosteal reactions can result in destructive lesions in the medial third of the clavicle in combination with the extensive formation of new bone (Rasool and Govender, ; Freyschmidt, ; Kim et al, ). Other signs of congenital syphilis include what is known as Higoumenakis' sign, which refers to the enlargement of the sternal end of the clavicle (Frangos et al, ).…”

“…Abnormalities and pathological conditions. The clavicle is subject to pronounced morphological abnormalities and pathological conditions (e.g., Kim et al, 2007;Curraino and Herring, 2009;Frangos et al, 2011;Figueiredo et al, 2012;van der Meijden et al, 2012;Shukla et al, 2014), and generally, there should be little difficulty in distinguishing these diseases and anomalies from normal variants of the clavicle (Freyschmidt, 2003).…”

“…what is known as Higoumenakis' sign, which refers to the enlargement of the sternal end of the clavicle (Frangos et al, 2011).…”

Advancing the understanding of treponemal disease in the past and present

“…Mulberry, or Fournier's, molars occur in CS, but they are not diagnostic because they also develop in other conditions (Hillson et al, ; Iouannou, Sassani, Henneberg, & Henneberg, ; Iouannou, Henneberg, & Henneberg, ). Beyond these dental alterations, macroscopically observable skeletal lesions in both early (onset under 2 years of age) and late (age 3–30 years) stages of CS are indistinguishable from those observed in other forms of treponemal infection (see, for example, Crissey & Denenholz, ; Rasool & Govendor, ), with the possible exception of Higoumenakis' sign of the clavicle (Frangos et al, ). Extensive clinical studies show that the radiologically detectable lesions associated with CS (including Wegner's sign and Wimberger's sign) are age‐related and ephemeral (Brion et al, ; Crissey & Denenholz, :156–157; McLean, ; Rasool & Govender, ).…”

“…Because many of the lesions characteristic of treponemal disease are also found in other conditions, they have low specificity (i.e., the lesion occurs in multiple diseases). Lesions also differ in sensitivity, or the proportion of affected individuals who exhibit the lesion (Frangos, Lavranos, & Frangos, ; Hackett, :102–103; Zuckerman et al, ). Periosteal reaction, for example, has low specificity but high sensitivity and, by itself, is not diagnostic of any specific condition (see Roberts, :107; Weston, , , ).…”