Abstract 422

Introduction: Poland sequence, first described in 1841, is a unilateral defect of the pectoralis major muscle with syndactyly of the ipsilateral hand. Several etiologies have been proposed including vascular disruption. Bavinck (1986) relates Poland sequence to subclavian artery disruption in early uterine life. While evidence most strongly suggests that cocaine exposure in utero causes genitourinary abnormalities in the exposed fetus (Buehler), its role in other vascular disruption defects, and especially limb reduction anomalies, has not been established. Kanikirawata (1993) reported a case of in utero cocaine exposure in association with Mobius syndrome. Hoyme (1990) reported a series of ten patients with prenatal cocaine exposure associated with vascular disruption defects, seven of whom had limb reduction anomalies. Viscarello (1992) reported two cases of limb-body wall complex associated with cocaine abuse by pregnant women. A study of prenatal ultrasound diagnosis of fetal anomalies by Hume (1994) found a three-fold relative risk of vascular disruption defects in the fetuses of cocaine abusing women and reported one case of unilateral limb reduction. Hannig (1991) reported a case of bilateral upper extremity limb reduction. Despite these associations and pharmacologic evidence for a causal role of cocaine in vascular disruption defects, a large case-control study failed to prove an association, although another 21 cases of transverse limb reduction defects were reported in cocaine-exposed fetuses (Hume, 1997). We report a patient with Poland sequence born to a cocaine-abusing mother.

Case Report: This 11-year old male was born to a 23 year old G2P2 HIV-positive mother. Throughout the pregnancy, the mother smoked "crack" cocaine, adulterated with unknown chemicals. She had no significant ethanol exposure and abused no other drugs. She does not recall any infections during the pregnancy and was on no HIV medications. Newborn examination revealed syndactyly of the second and third digits of the right hand and a deformity of the right pectoralis muscle with a hypoplastic right nipple. At age 6 1/2 years, the cutaneous syndactyly was corrected. The patient has gross motor and language delays. The patient has had intermittent complaints of profuse sweating from the right axilla and hand treated with topical preparations with little success. He tested HIV-negative at age 10. Presently, the patient has a shortened right arm, hypoplastic right nipple with absent right pectoralis major muscle, and well-healed surgical scars on the right hand.

Discussion: This is the first well-described patient with Poland sequence associated with in utero cocaine exposure. A previous report of Poland sequence associated with anhidrotic axilla has been published (Holmstrom). This is also the first report of hyperhidrosis in Poland sequence.