Vascular origin of Poland syndrome? by J.-P. Bouvet et al.

✍ Scribed by T. J. David; J.-P. Bouvet

• Publisher: Springer
• Year: 1979
• Tongue: English
• Weight: 124 KB
• Volume: 130
• Category: Article
• ISSN: 0340-6997
• DOI: 10.1007/bf00441367

✦ Synopsis

Dear Sir, Dr. Bouvet and his colleagues are to be congratulated on a fascinating study. May I add a further interpretation of their findings? They say that their results "support the hypothesis of hypoplasia of the subclavian artery in Poland syndrome". However, whether the arterial hypoplasia so elegantly displayed causes the malformation, as opposed to it being a predictable consequence associated with a slightly hypoplastic upper and lower arm is another matter. I respectfully suggest that their evidence in no way supports a causal relationship.

Before a vascular hypothesis for the aetiology of the Poland anomaly can be accepted, or indeed before any aetiological hypothesis can be accepted, the hypothesis will have to explain why the condition is so consistently unilateral (8/8 cases in Bouvet's series, 78/78 in mine).

There are two other minor points. Just because a child has a hand deformity does not mean that one cannot tell whether he is naturally left or right handed. A clear history will usually indicate which hand is preferred in even the most severe cases, unless the parents have been grossly overprotective. Further information can usually be obtained by watching which foot is used to kick a ball and so on. Also, I would question the description of a malformation with an incidence of i in 30,000 as "common". (Incidentally this estimate of incidence was based on only two cases.) Many paediatricians have never seen a case or heard of the condition, and it is generally accepted that in the field of malformations a defect is regarded as "common" if it has an incidence of 1 per 1000 births or higher (Carter, 1969). However, if the authors are trying to say that the condition is more frequent than generally realised then I would certainly agree with them.