AMNIOTIC BAND SYNDROME - THE ASSOCIATION BETWEEN RARE FACIAL CLEFTS AND LIMB RING CONSTRICTIONS

The pathologic cause of both rare craniofacial clefts and congenital l imb ring constrictions is the subject of some debate. Uncommon though these two conditions are, they have often been anecdotally reported in association. This study sought to determine the frequency of congenit al limb anomalies in general and limb ring constrictions in particular amongst a population of patients with rare craniofacial clefts. Eight y-five cases of nonsyndromal, rare, craniofacial cleft were identified from a population of 280 patients assessed at the Women's and Childre n's Hospital, North Adelaide, with a diagnosis of rare craniofacial cl eft classifiable by the Tessier system. Twenty-two patients (25.9 perc ent) displayed congenital limb anomalies. Eleven of these (12.9 percen t) showed evidence of limb ring constrictions, a frequency much greate r than in the general population. The group with limb ring constrictio ns demonstrated a significantly greater complexity of involvement with craniofacial clefting than the non-limb ring constriction group (4.27 clefts/patient versus 2.3 clefts/patient, p < 0.01). The distribution of craniofacial cleft locations in patients with evidence of Limb rin g constrictions was found to differ significantly from those with othe r or no limb anomalies (p < 0.01). The clefts in cases in which limb r ing constrictions coexist are largely restricted to the paramedian 2-1 2, 3-11, 4-12 axes. This study confirms an association between rare cr aniofacial clefts and limb ring constrictions. The two conditions may therefore possess common etiology.