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Autosomal Recessive Inheritance in the Setleis Bitemporal 'Forceps Marks' Syndrome

Robert W. Marion, MD; David Chitayat, MD; R. Gordon Hutcheon, MD; Rosalie Goldberg, MS; Robert J. Shprintzen, PhD; M. Michael Cohen Jr, DMD, PhD
Am J Dis Child. 1987;141(8):895-897. doi:10.1001/archpedi.1987.04460080081033.
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• We present the clinical findings in two children with the Setleis bitemporal "forceps marks" syndrome. The striking features include the following: (1) bitemporal scarring, an anomaly that resembles forceps marks; (2) periorbital puffiness with wrinkling of the skin; (3) abnormalities of the eyebrows; (4) anomalles of the eyelashes; (5) flattening of the nasal bridge with a bulbous nasal tip; (6) increased mobility of the skin, associated with severely redundant facial soft tissue; and (7) normal growth and development. The evidence that suggests that this unusual syndrome is inherited in an autosomal recessive fashion includes the following: (1) seven of the patients have come from the relatively isolated towns of San Sebastian and Aguadilla in Puerto Rico; (2) two sets of affected siblings have been described, and, in both cases, the siblings' Parents were normal; and (3) one of the children described herein is the product of a consanguineous mating. Although the pathogenetic mechanism Is unknown, Setleis syndrome is clearly inherited as an autosomal recessive trait.

(AJDC 1987;141:895-897)


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