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Nager syndrome symptoms
- AKA preaxial acrofacial dysostosis
- zygomatic hypoplasia
- downward slantting palpebral fissures from hypoplastic orbitomalar
- cleft palate
- preaxial limb deformities.
Nager common features
- Zygomatic hypoplasia +
- Downslanting palpeblar ﬁssures +
- Lower eyelid colobomas
- Mandibular hypoplasia +
- External ear malformation +
- Thumb aplasia or hypoplasia
- Radial defects +
- Genitourinary abnormalities +
- Reduced stature +
Nager development defect origins
- branchial arch 1,2
- limb buds
failure of fusion of the lateral palatine processes, the nasal septum, and/or the median palatine processes (formation of the secondary palate).
failure of fusion of the maxillary and medial nasal processes (formation of the primary palate).
Cleft lip surgery
- rule of 10s
- 10 weeks of age; weighs at least 10 pounds, and has at least 10g hemoglobin
The rotation-advancement procedure for cleft lip repair, also known as the Millard Repair, is designed to create a softer, more natural-looking lip. Surgery performed prior to the Millard procedure involved pulling both sides of the cleft lip together resulting in a tightly closed upper lip. The Millard Procedure rotates the tissue and creates a "Z" shaped scar instead. The "Z" shape gives the tissue more elasticity, resulting in greater flexibility and restoration of the Cupid's bow.
Cleft palate surgery,
- between 6 and 12 months.
- Approximately 20–25% only require one palatal surgery to achieve a competent velopharyngeal valve capable of producing normal, non-hypernasal speech.
surgically inserted by use of pins during the child's 4th or 5th month. After it is in place, the doctor, or parents, turn a screw daily to bring the cleft together to assist with future lip and/or palate repair.
surgery for speech correction done at 3-4 yrs old
alveolar bone graft (cleft palate)
- done 5-9 yrs old if necessary
- may need palatal expansion