Original Investigations

European Journal of Pediatrics

, Volume 128, Issue 1, pp 17-26

First online:

Vascular origin of Poland syndrome?

A comparative rheographic study of the vascularisation of the arms in eight patients
  • Jean-Pierre BouvetAffiliated withRecipient of the Fonds d'Etude du Corps Médical Hospitalier de Paris. Service de Rhumatologie, Hôpital Ambroise Pare
  • , Denis LevequeAffiliated withLaboratoire d'Hémodynamique, Hôpital Lariboisiere
  • , Francine BernetieresAffiliated withLaboratoire d'Hémodynamique, Hôpital Lariboisiere
  • , Jean-Jacques GrosAffiliated withLaboratoire d'Hémodynamique, Hôpital Lariboisiere

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Vascularization of the arms has been studied by impedance plethysmography (rheography) in eight children with Poland syndrome, a common malformation characterized by unilateral hand anomaly and ipsilateral aplasia of the inferior head of the pectoralis major muscle. A marked decrease of the velocity of the systolic increase in the arterial volume (Vel m) was shown in the affected arms, and the difference between the two arms was significantly higher in the patients than in a control group (P<0.02). These results, which are also observed in stenotic atherosclerosis, support the hypothesis of hypoplasia of the ipsilateral subclavian artery as the origin of the malformation, although a local anomaly in arterial-wall viscosity cannot be ruled out. The opacification of the aortic arch in another patient confirmed this hypothesis. Aortography in further patients will be necessary to substantiate our results. However, this technique is not without danger and should only be used during the first months of life when a vascular operation can be envisaged.

Key words

Poland syndrome Malformation of hand Impedance plethysmography Vascular malformation