The Clinical Features of Ulnar Polydactyly in a Middle Eastern Population

Abstract

Two clinical forms of ulnar polydactyly are recognised in the literature, viz the African and the Caucasian forms. The current study investigated the clinical and radiological features of ulnar polydactyly in 94 Saudi patients. The incidence of ulnar polydactyly was one in 1000 live births. There were 41 males and 53 females. Positive family history, syndromal cases, associated hand anomalies, polydactyly of the little toe and systemic abnormalities were seen in 11%, 6%, 5%, 29% and 23% of cases, respectively. There were 50 unilateral (53%) and 44 bilateral cases (47%). In unilateral cases, the left hand was more commonly affected. Using a modified Rayan-Frey classification, the majority of cases were classified as Type II (pedunculated polydactyly, 52 (55%) cases) and Type III (a functioning and articulating extra digit without complete duplication of the metacarpal, 29 (31%) cases). It was concluded that the Saudi clinical presentation of ulnar polydactyly is somewhat different epidemiologically and lies between the African and Caucasian forms.

Isolated postaxial polydactyly: Epidemiologic characteristics from a multicenter birth defects study

“…Probably our prevalence is lower than that observed in some other ethnically related populations because in the present study we only included live births isolated PAP or cases with PAP as the unique defect. Some of the prevalences observed from other studies included stillbirths and multiple malformed babies in their reporting, and some others including together postaxial and preaxial polydactyly as a whole (Al‐Qattan et al, ; Castilla et al, ; International Clearinghouse for Birth Defects Surveillance (ICBDSR), http://www.icbdsr.org and Research). Under‐reporting may also, significantly affect prevalence differences due in part to be I‐PAP considered a minor morphological defect.…”

“…PAP affects hands and the left limbs more frequently (Castilla et al, ). In people of non‐African descent, the ulnar polydactylies are usually sporadic, 20% are bilateral, and males are more commonly affected than females (Al‐Qattan et al, ). Cases with both, UL and LL PAP subphenotypes are more frequently associated with other congenital malformations (23%), contrasting with the observed 6% for UL and 15% for LL PAP (Castilla et al, ).…”

“…Although a frequent malformation, prevalence data is scarce, and epidemiological data limited to certain selected populations (Al‐Qattan, Al‐Shanawani, Al‐Thunayan, & Al‐Namla, ; Bingle & Niswander, ; Boeing et al, ; Castilla et al, ; Castilla et al, ; Holmes, Nasri, Hunt, Toufaily, & Westgate, ; Orioli, ). The Mexican program, of Registry and Epidemiological Surveillance of Congenital Malformations (RYVEMCE) (Castilla & Mastroiacovo, ; Mutchinick, Lisker, & Babinsky, ); and http://www.icbdsr.org; documents all types of major and minor CM, including isolated PAP (I‐PAP) independently of the anatomical and functional characteristics of the defect.…”

“…PAP is usually grouped in Types A and B, with Type A defined as a fully developed, functional extra articulated digit, and Type B as an incompletely developed, unarticulated, nonfunctional extra digit (Temtamy & McKusick, ). However, there are other classifications, most of them based on its morphology alone, upholding the controversy in this respect (Al‐Qattan et al, ; Blauth & Olason, ; Tonkin, ).…”

Complications of Suture Ligation Ablation for Ulnar Polydactyly: A Report of Two Cases

“…Its prevalence is one in 531 live births but varies from one in 143 black infants to one in 1,339 white infants making it roughly ten times more common among black infants [20]. While ulnar duplication may be associated with an underlying syndrome, it is most often an isolated finding, particularly in African Americans where it typically assumes an autosomal dominant mode of inheritance and is bilateral in 70% of cases [1,20].…”