American Journal of Medical Genetics 121A:90 –91 (2003)
Correspondence Supernumerary Digital Flexion Creases To the Editor: Kamath et al. [2002] have observed supernumerary digital creases in one-third of children with Alagille syndrome and speculate on the molecular development of digital crease patterns. They also state that the prevalence of supernumerary digital creases is less than 1% in the general population. Recently, we examined by means of simple visual inspection the palms and fingers of a total of 396 inmates (219 boys and 177 girls) of two Hungarian homes for children with severe mental retardation. Their age varied from 2 to 21 years with a mean of 10.4 years, their I.Q. was less than 35 in each case. Unanimous determination of ethnic origin of the patients was not possible, but the majority were white Hungarian children, about one fifth of them belonged to the local Gypsy minority. Genetically determined diseases were identified in 46 children, perinatal brain damage was suspected in nearly half of the cases, while no cause could be detected in the rest. No signs of Alagille syndrome occurred in any of the patients. Out of the 46 subjects with malformation syndromes or inborn errors of metabolism, two had supernumerary digital flexion creases (1 Ellis van Creveld syndrome and 1 18p-deletion), and one had no distal flexion creases on fingers 3–4 (campomelic dysplasia). Genetically determined diseases were excluded from the evaluation, thus the findings in the remaining 350 children were as follows. Absence of the distal flexion creases, especially on the 3rd and 4th fingers, were registered in three children (0.8%) with no additional dysmorphology. Supernumerary flexion creases of the fingers were seen in 11 children (3.1%). In four patients an extra flexion crease, similar to the usual ones, occurred on all fingers. One child had supernumerary creases of the fingers I–IV, but not of the V (Fig. 1). In six cases such creases occurred on the volar surface of the 3rd and 4th fingers over the middle phalanges between the second
Grant sponsor: Hungarian Ministry of Health; Grant numbers: ETT 349/2000, 342/2000. *Correspondence to: Prof. Dr. Gyo¨rgy Kosztola´nyi, Jo´zsef Attila u. 7, H-7623 Pe´cs, Hungary. E-mail:
[email protected] Received 18 November 2002; Accepted 30 January 2003 DOI 10.1002/ajmg.a.20118
ß 2003 Wiley-Liss, Inc.
and third ‘‘normal’’ flexion creases. Out of them, three subjects had additional thin transverse creases on the finger-tips of these fingers and on the thumbs as well. In four children similar transverse creases of various expression were found in irregular distribution. Joint anatomy and movements seemed to be normal in all of these 11 subjects. No sex or ethnic differences in the prevalence and patterns of digital creases were observed. We had no special control group in this study. However, when investigating the prevalence of mild errors of morphogenesis in childhood malignancies in earlier studies [Me´hes et al., 1985, 1998], no supernumerary digital creases were found in 204 children with leukemia or soft tissue tumors, in their 408 firstdegree relatives, and in 204 healthy control subjects. This is why, we believe that, irrespective of ethnic origin [Aue-Hauser, 1980], absence of flexion creases and supernumerary digital creases are probably very rare in the general population. They are practically
Fig. 1.
Supernumerary flexion creases of all fingers except the fifth one.
Supernumerary Digital Flexion Creases
always associated with some kind of prenatal pathology, and should be sought for in the physical investigation of children. This association may be characteristic of some conditions, but is certainly not specific for any particular condition. REFERENCES Aue-Hauser G. 1980. Die Beugefurchen der menschlichen Finger. Anthrop Anz 38:85–116. Kamath MB, Loomes KM, Oakey RJ, Krantz ID. 2002. Supernumerary digital flexion creases. An additional clinical manifestation of Alagille syndrome. Am J Med Genet 112:171–175.
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Me´hes K, Signer E, Plu¨ss HJ, Mu¨ller HJ, Stalder G. 1985. Increased prevalence of minor anomalies in childhood malignancy. Eur J Pediatr 144:243–249. Me´hes K, Kajta´r P, Sa´ndor G, Scheel-Walter M, Niethammer D. 1998. Excess of mild errors of morphogenesis in childhood lymphoblastic leukemia. Am J Med Genet 75:22–27.
Gyo¨rgy Kosztola´nyi* Ka´roly Me´hes MTA-PTE Research Group of Clinical Genetics Department of Medical Genetics and Child Development University of Pe´cs, Pe´cs, Hungary
