Clinical spectrum of arhinia

(A) Two-year-old child with arhinia and left-sided iris coloboma.
(B) Ten-year-old child with arhinia and left-sided microphthalmia and bilateral nasolacrimal duct stenosis.
(C) Newborn with arhinia and normal eye anatomy and vision.
(D) Sixteen-year-old with arhinia and right-sided microphthalmia.
(E) One-year-old child with arhinia, bilateral colobomatous microphthalmia, cataracts, and nasolacrimal duct atresia.

(F-J) Craniofacial radiographic images from the two-year-old child pictured in panel A:

• (F) Surface rendering reconstruction from a MRI 3D T1 weighted sequence showing complete absence of the nose (large arrowhead).
• (G) 3D volume rendering technique reconstruction from spiral CT showing complete absence of nasal bones (thick arrow).
• (H) Coronal reconstruction from CT showing absence of nasal septal structures; the maxilla articulates with the nasal process of the frontal bone (arrowhead).
• (I) Coronal MRI T2 weighted sequence showing absence of the olfactory bulb and olfactory sulcus (dashed arrow).
• (J) Midline MRI sagittal T1 weighted sequence (j) showing a high-arched palate (cleft not visible on this image) and decreased distance between the oral cavity and the anterior cranial fossa (arrow). The rudimentary nasopharynx (*) is blind and air filled. The pituitary gland (short arrow) appears normal.