ABSENT
NOSE
By H. K. DAS GUPTA,M.S., M.Ch.(Urology), VIDHYAGUPTA,M.B., B.S., and MUSKESHGUPTA,M.D.(Paed.) Departments of Surgery and Paediatrics, Sarda Pate1 Medical College, Bikaner (Rajasthan), India
AGENESISof the nose is very rare. Only 6 cases have so far been reported, 5 in living babies (Berger and Martin, 1969; Gifford et al., 1972; Walker, 1961) and I in a stillborn foetus (Wahby, 1903-04). The complex facial anomaly with absence of the nose reported here is described for the first time. The boy (Figs. I and 2) was brought to us when 8 months old. He was the first born and his mother had no complications during pregnancy nor had taken any medication. The nose and nasal airways were entirely missing and this had caused great difficulty in breast feeding since the child could not breathe and suck simultaneously. The eyes were set widely apart with interorbital and interpupillary distances of 60 mm Just medial to each inner canthus were blind oval pits 2.5 cm and 80 mm respectively. by I ‘5 cm in size. They were lined with mucosa and discharged mucus. In the floor of each a turbinate-like process was present. The lacrymal puncta led into short canals which appeared to end blindly since fluid injected by syringe flowed out at the puncta. The mouth was constantly kept open for breathing and deliberate closure evoked restlessness with impending cyanosis.
FIG. I. FIG. 2.
Complete
‘The child aged 8 months.
absence of the nose, hypertelorism, abortive attempt to form nasal placodes and open mouth. 85
86
BRITISH
JOURNAL
OF PLASTIC
SURGERY
There was a very high arched palate (Fig. 3) almost occupying the space where the nasopharynx should have been and a left partial alveolar cleft. The nasi-occiput distance was 43.75 cm and there were no skull abnormalities. There were constriction bands on both lower legs and bilateral talipes varus (Fig. 4). DISCUSSION
A possible explanation of this facial deformity is that there was a delayed stimulus for the develpoment of the nose and nasal airways and an abortive late attempt which produced the mucosa lined pits when the rest of the face had formed. Nasal reconstructive surgery has been deferred until adulthood because of the absence of the choanal space. REFERENCES
BERGER, M. and MARTIN, C. (1969). L’Arhinogenesis totale (absence congenitale d’une fosses nasales). A propos dune observation exceptionelle. Revue de Laryngologie, Otologie, Rhinologie, 90, 300. Congenital absence of GIFFORD, G. H. JR., SWANSON,L. and MACCOLLUMN, D. W. (1972). the nose and anterior nasopharynx. Report of two cases. Plastic and Reconstructive Surgery, 50, 5. Congenital absence of nose and premaxilla. Journal of Anatomy, WAHBY, B. (1903-04). 3% 49. Congenital absence of nose and “Malformation of the Face.” WALKER, D. G. (1961). premaxilla, p. 32. Edinburgh and London: E. and S. Livingstone.
NOSE
By H. K. DAS GUPTA,M.S., M.Ch.(Urology), VIDHYAGUPTA,M.B., B.S., and MUSKESHGUPTA,M.D.(Paed.) Departments of Surgery and Paediatrics, Sarda Pate1 Medical College, Bikaner (Rajasthan), India
AGENESISof the nose is very rare. Only 6 cases have so far been reported, 5 in living babies (Berger and Martin, 1969; Gifford et al., 1972; Walker, 1961) and I in a stillborn foetus (Wahby, 1903-04). The complex facial anomaly with absence of the nose reported here is described for the first time. The boy (Figs. I and 2) was brought to us when 8 months old. He was the first born and his mother had no complications during pregnancy nor had taken any medication. The nose and nasal airways were entirely missing and this had caused great difficulty in breast feeding since the child could not breathe and suck simultaneously. The eyes were set widely apart with interorbital and interpupillary distances of 60 mm Just medial to each inner canthus were blind oval pits 2.5 cm and 80 mm respectively. by I ‘5 cm in size. They were lined with mucosa and discharged mucus. In the floor of each a turbinate-like process was present. The lacrymal puncta led into short canals which appeared to end blindly since fluid injected by syringe flowed out at the puncta. The mouth was constantly kept open for breathing and deliberate closure evoked restlessness with impending cyanosis.
FIG. I. FIG. 2.
Complete
‘The child aged 8 months.
absence of the nose, hypertelorism, abortive attempt to form nasal placodes and open mouth. 85
86
BRITISH
JOURNAL
OF PLASTIC
SURGERY
There was a very high arched palate (Fig. 3) almost occupying the space where the nasopharynx should have been and a left partial alveolar cleft. The nasi-occiput distance was 43.75 cm and there were no skull abnormalities. There were constriction bands on both lower legs and bilateral talipes varus (Fig. 4). DISCUSSION
A possible explanation of this facial deformity is that there was a delayed stimulus for the develpoment of the nose and nasal airways and an abortive late attempt which produced the mucosa lined pits when the rest of the face had formed. Nasal reconstructive surgery has been deferred until adulthood because of the absence of the choanal space. REFERENCES
BERGER, M. and MARTIN, C. (1969). L’Arhinogenesis totale (absence congenitale d’une fosses nasales). A propos dune observation exceptionelle. Revue de Laryngologie, Otologie, Rhinologie, 90, 300. Congenital absence of GIFFORD, G. H. JR., SWANSON,L. and MACCOLLUMN, D. W. (1972). the nose and anterior nasopharynx. Report of two cases. Plastic and Reconstructive Surgery, 50, 5. Congenital absence of nose and premaxilla. Journal of Anatomy, WAHBY, B. (1903-04). 3% 49. Congenital absence of nose and “Malformation of the Face.” WALKER, D. G. (1961). premaxilla, p. 32. Edinburgh and London: E. and S. Livingstone.
