Half-nose; a very rare congenital anomaly

Abstract

Aplasia of the nose is a very rare, congenital anomaly in our field. It emerges as a complete absence of the external nose and all nasal cavities on both sides, or an absence of one half of the nose and its associated nasal cavity. The latter case is called; “halfnose.”Etiologically, it is akin to tubular proboscis, anomaly with a snout-like nasal appendage on, or by the mid-line of the face. Tubular proboscis is caused by a failure of the embryonal frontonasal process to migrate downward, and aplasia occurs when the frontonasal process and nasal sac fails to develop due to the same and more severe cause.Hitherto, only several clinical cases of aplasia have been reported in the literatures.Here a case of half-nose was reported. The patient was a five-year-old girl born with congenital absence of the left external nose and nazis. Moreover, her appearance was featured by epiphora only from the left eye. Left choanal atresia was found by posterior rhinoscopy. X-ray examination revealed complete absence of the nasal cavity, accessory sinuses and nasolacrymal duct on the same side. Systolic heartmurmur by auscultation suggested some congenital heart anomaly, but chest X-ray examination and electrocardiography presented no pathological findings. She had no other serious diseases since the birth. She was one of three offsprings of healthy parents without consanguineous marriage. The two siblings were normal. In the family-tree no other patient of congenital malformation could be seen.Concerning surgical procedures, intervention to the bony tissues of such a young patient should be avoided not to disturb a further growth of the facial bone. Then, plastic surgery was carried out only on the external nose in April, 1966. Under general anesthesia, incision was made on the left cheek, and tongueshaped pedicle skin flap was raised up. The nasal septum was exposed, on the left side there could be seen no nasal cavity but bony tissues. The left nasal ala was made of the pedicle flap by V-Y suture method. The new nais was lined with free Thiersch graft taken from the thigh. The wound healed up with satisfying result, and the patient left the hospital in June, 1966.