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MEDICINE | THE UNREAL WORLD

A delicate procedure for family

September 25, 2006|Marc Siegel | Special to The Times

"Nip/Tuck," FX, Episode 2, Monday, Sept. 12.

The Premise:

PLASTIC surgeon Sean McNamara, played by Dylan Walsh, has learned that his unborn son (to be named Connor) has ectrodactyly (malformed hands or feet with absence of fingers or toes) and a cleft palate. McNamara plans to operate on Connor soon after his birth to try to correct these congenital malformations.

The medical questions

HOW common are these malformations? How successful would corrective surgery be if performed early? Is it possible to operate in utero (while the fetus is still in the womb)? What are the ethics or advisability of a father operating on his child?

The reality

ECTRODACTYLY, often known as lobster claw syndrome or split hand/foot malformation, is fairly common, with six cases per 10,000 human births. All forms are associated with at least one genetic mutation, one of the most frequent (Type 1) caused by a mutation on chromosome 7. Ectrodactyly often occurs in common with other congenital anomalies -- such as a cleft lip and palate, and ectodermal dysplasia (hair, skin and nail deformities). In "Nip/Tuck," McNamara views a photograph of a hand with ectrodactyly and contemplates Connor's surgery.

This surgery should be performed between the ages of 1 and 2, before hand dominance is defined and psychological trauma accelerates, according to Dr. James P. Bradley, chief of pediatric plastic surgery at UCLA. The surgery involves separating out a thumb, leaving the patient with a three-fingered hand, which "allows the patient to eat and care for himself. He will not be able to perform fine motor functions like writing, but the deformity will not be as easily observed." More fingers can't be created because of the preexisting bones and joints.

In terms of the cleft lip and palate, Bradley believes that it should also be corrected between 1 and 2 years "to avoid long-lasting speech problems."

Although surgery for these malformations can be done in utero, providing "scarless healing," and overall better results, this is generally not done because the benefit is not worth the risk of premature labor. But if a more life-threatening abnormality is being corrected in utero, then the hand and facial surgery can be done at the same time, Bradley says.

As to whether McNamara should operate on his own child, Bradley bucks conventional wisdom. Most doctors say that the lack of emotional distance interferes with medical judgment and surgical precision. But Bradley disagrees, asserting that such a decision isn't unwise as long as the doctor is qualified. "A good surgeon can learn to suspend the emotional component," Bradley says. "If you're the best-trained surgeon for the procedure, then you're the one to do it."

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Dr. Marc Siegel is an internist and an associate professor of medicine at New York University's School of Medicine. He is also the author of "False Alarm: The Truth About the Epidemic of Fear." In The Unreal World, he explains the medical facts behind the media fiction. He can be reached at marc@doctorsiegel.com.

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