In the past quarter century, I have been fortunate in having a number of parents of children with BWS who have kept me in touch with the growth, development, and lives of their children, supplemented by annual photographs. This series presents an extremely optimistic outlook for the infant or young child who is diagnosed as having BWS. The adolescent or adult who had this diagnosis in childhood can rarely be recognized as such in maturity, even when it was very apparent in early life. The cases in my files have grown up to be normal in appearance. Their adult height is variable, but neither gigantic nor uncommonly short stature has been seen, and intelligence is generally in the normal range, with more exceptions on the high than the low side. The incidence of diabetes, coronary artery disease, and neoplasm in adult life remain uncertain, as does the outcome of their pregnancies. However, to date I am aware of nothing that would modify the very optimistic long-term prognosis I give to these families. There are not many birth defect syndromes that get better with time, but this seems to be one of those. Parents who are deeply concerned that their baby looks "different" can be reassured that when their child is grown up, nobody would know they had been a "BWS baby"!

 The main points I emphasize to parents of newly diagnosed cases are

1. the usual complications of BWS can usually be anticipated, and can almost always be managed successfully;
2. the older they become the more normal they become, and
3. most affected offspring are likely to have the disorder in such a subtle form that it will not alter their quality of life.

I do advise ultrasound exam of the abdomen every 3 months for detection of tumors at a stage when they are still easily curable. This should continue until age 8. The tongue, if still protuberant and interfering with speech and dental development, should be considered for reduction surgery by about age 4 years, in order to give time for improved speech and appearance before the child enters that sociological jungle known as school.

There has long been a need for information written for parents of children with BWS, and for a parent support group. ... I hope that among the readers of this letter some will come forth to volunteer their time, energy, and talents to ensure the success of this effort.

Sincerely,
Dr. Beckwith

(this letter is from the mid 1990's)

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The following is a more recent note (dated September 10th, 2003) from Dr Beckwith, in response to a parent's request for his opinion as to when is best to perform a tongue reduction surgery:

I used to recommend waiting until about age 4 or 5 for tongue surgery in all but the most severe cases, but that recommendation has been changed in recent years, due in large part to the experience of Dr. Marsh. The reasons we used to recommend waiting were that some children "grew into" their tongues early in childhood. We advised age 4 to 5 because that gave them time to recuperate prior to the school years, where any deformity is likely to generate unpleasant behavior on the part of other children. However, when it is performed at that age the experience is much worse, and certainly much more clearly remembered, than with earlier surgery. I have been very much impressed with talking to parents whose baby was operated in the first year or two, about how glad they were it was all over with so early, usually with a very satisfactory result.

But I am not the person to advise you on this. I am a retired pediatric pathologist, and despite having a real concern for and interest in "my" children with BWS, I am not a real clinician, and certainly not a surgeon. I have great respect for Jeff Marsh, and have been impressed by the parents I have talked with who have had surgery done by him. His support team is also excellent, which is a real advantage.

If you wait for documented evidence in the literature on matters like this, you might wait forever. Numbers are nice, but each case is so individual in degree, exact anatomy, social and family setting, etc. Dr. Marsh has more experience than anyone in the world, but of course that does not guarantee he will be right 100% of the time. But if it were my grandchild, I would have them evaluated by him, and would abide by his recommendation. What I can tell you is that almost every child who waited until 4 or 5 remembers the surgery as one of the worst things in their life experience. Those who had it very early on have no memory of the surgery.

Bruce Beckwith