Classification (Losee prs feb 2004) Type I anomalies: Hypoplasia and Atrophy



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Classification of Congenital Nasal Deformities

See Embryology

Classification (Losee PRS Feb 2004)
Type I Anomalies: Hypoplasia and Atrophy

  • represent paucity, atrophy, or underdevelopment of skin, subcutaneous tissue, muscle, cartilage, and/or bone.

  • entire range of hypoplasia, from underdevelopment or partial absence of parts to complete arhinia, is included.

  1. Arhinia

  2. Heminose

  3. Hypoplasia/absence of parts

  4. Craniofacial syndromes

    • Binder syndrome, or nasomaxillary hypoplasia

      1. midface retrusion

      2. hypoplasia of the anterior nasal spine

      3. a short columella

      4. an obtuse nasofrontal angle

    • Fraser syndrome,

      1. rare autosomal recessive disorder

      2. presents with cryptophthalmos and nasal anomalies, including a broad nose with midline groove, a depressed nasal bridge, hypoplastic nares with colobomas, choanal stenosis, and a beak-like appearance

  5. Nasal cavity atresia

    • nasal cavity is divided into three regions. Obstruction may occur at these points:

      1. anterior bony inlet (pyriform aperture stenosis)

        • believed to be due to overgrowth of the maxillary nasal process and hypoplasia of the anterior hard palate and inferior pyriform aperture

      2. stenosis of the cavity

        • very rare

      3. posterior outlet obstruction (choanal atresia)

        • 1 in 5-8000

        • majority of outlet obstructions are believed to be both bony and membranous, consisting of a foreshortened and narrowed nasal cavity, lateral bony thickening, and obstruction by a thickened, deviated vomer.

        • thought to result from persistence of the bucconasal membrane or an insufficient excavation of the nasal pits

    • Babies are obligate nasal breathers

    • Any level of nasal cavity obstruction may present with apneic episodes and cyclical cyanosis, which are worsened by feeding and relieved with crying


Type II Anomalies: Hyperplasia and Duplications

  1. Duplication of parts

    • Rare

  1. Proboscis lateralis.

    • 1 in 100,000

    • rectangular, tubular, fleshy appendage that is 1 to 3 cm long and 1 cm wide and connected to the inner canthus by a broad sessile attachment

    • proximal canal is blind without communicating with the nasal cavity.


Type III Anomalies: Atypical Clefts

  • Facial clefts involving the nose (Tessier 0, 1, 2, and 3) and their cranial extensions (Tessier 11, 12, 13, and 14)

  • 0/14 clefts of frontonasal dysplasia most common


Type IV Anomalies: Neoplasms and Vascular Anomalies

  • differential diagnosis includes encephaloceles, meningoceles, gliomas, dermoids, vascular malformations, and malignant and benign neoplasms

  • rarer lesions include cysts, polyps, fibromas, fibromyxomas, granulomas, and mucoceles



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