Innovative surgical technique creates a nose for patients with 'extremely rare' genetic syndrome

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Innovative surgical technique creates a nose for patients with 'extremely rare' genetic syndrome
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Congenital arhinia (meaning patients born without a nose) is a rare condition associated with high mortality if not identified. As most babies when born are obligate nose breathers, the condition requires immediate attention. The clinical condition is a very rare genetic disorder that, in severe cases, causes congenital absence of the nose with life threatening conditions.

, an expert team of plastic and cranio-maxillofacial surgeons describes an innovative new approach to constructing a nose in two

Eric C. Liao, MD, Ph.D., of Massachusetts General Hospital/Harvard Medical School and colleagues report on twofrom a family affected by congenital arhinia, and the surgical technique designed to create a cosmetically acceptable nose. The experience"highlights key principles of a staged approach to nasal construction in arhinia and discusses nuances and improvements learned between both patients," the researchers write.

Not only was the external nose absent, but the skeletal foundation of the midface was severely underdeveloped. The two patients had no facial air passages, so the newly created nose was nonfunctional; the patients were able to breathe through the mouth.To meet the complex challenges posed by this severe defect, Dr. Liao and colleagues designed a staged approach to augment the underlying bones of the face and to construct an external nose.

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