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In-Service Exam
Cleft Lip/Palate - 2001






Patients with unilateral cleft lip and associated nasal deformities have each of the following findings EXCEPT

(A) attenuation and inferior positioning of the lower lateral cartilage on the side of the cleft
(B) elongation of the philtrum
(C) insertion of the orbicularis oris muscle into the cleft margin and alar wing
(D) outward rotation and projection of the premaxilla
(E) unilateral shortening of the columella

Reproduced with permission of McCarthy JG, ed. Plastic Surgery. Philadelphia, Pa: WB Saunders Co; 1990;4:2799.

The correct response is Option B.

In patients who have unilateral cleft lip and associated nasal deformities, the premaxilla is rotated and projected outward, and the lateral maxillary element is collapsed and retropositioned. The inferior edge of the septum lies outside of the vomer groove, while the nasal spine is located in the floor of the normal nostril. The affected columella is 25% to 50% shorter than the unaffected side. The lower lateral cartilage is attenuated, and the nasal dome lies separate, below the opposite cartilage. The alar base is flared and rotated outward, and the vestibular lining is deficient on the side of the cleft.

With regard to the lip deformities, the philtrum termination of the orbicularis oris muscle in the lateral lip is shortened at the margin of the cleft; at this point, the muscle inserts into the alar wing. The muscles between the philtral midline and the cleft are hypoplastic. Two thirds of the cupid's bow is preserved, as well as one philtral column and a dimple hollow.


References
1. Burt JD, Byrd HS. Cleft lip: unilateral primary deformities. Plast Reconstr Surg. 2000;105:1043.
2. Fara M. Anatomy and arteriography of cleft lips in stillborn children. Plast Reconstr Surg. 1968;42:29.
3. Millard DR Jr. Unilateral cleft lip deformities. In: McCarthy JG, ed. Plastic Surgery. Philadelphia, Pa: WB Saunders Co; 1990;4:2627.
4. Mulliken JB, Pensler JM, Kozakewich HP, et al. The anatomy of cupid's bow in normal and cleft lip. Plast Reconstr Surg. 1993;92:395.


A 1-year-old child with Pierre Robin sequence has normal mandibular growth. His jaw deformity is best described as

(A) brachygnathia
(B) hypoplasia
(C) microgenia
(D) micrognathia
(E) retrognathia


The correct response is Option E.

Retrognathia, which is defined as posterior displacement of the chin with normal mandibular dimensions, is best used to describe the findings seen in this patient with Pierre Robin sequence. Other terms such as brachygnathia, micrognathia, congenital mandibular atresia, mandibular hypoplasia, and mandibular hypotrophy have been used in the description of this condition. However, because all of these terms denote abnormalities in mandibular growth, they are frequently used incorrectly; instead, normal mandibular growth is a classic finding of Pierre Robin sequence. In addition, the surgeon should be aware that the growth potential in patients with retrognathia and posterior displacement of the chin on external manipulation is excellent. Pierre Robin sequence is also characterized by respiratory obstruction and glossoptosis.

Microgenia is defined as abnormal development in the region of the mental symphysis. However, this term can be differentiated from micrognathia because all of the mandibular components do not have to be involved; instead, some patients have an isolated small chin deformity with normally-sized jaw components. Microgenia can also occur in conjunction with mandibular hypoplasia or mandibular prognathism.


References
1. McCarthy JG, Kawamoto H, Grayson BH, et al. Surgery of the jaws. In: McCarthy JG, ed. Plastic Surgery. Philadelphia, Pa: WB Saunders Co; 1990;2:1188-1474.
2. McCarthy JG, Kay PP, Randall P, et al. Craniofacial syndromes. In: McCarthy JG, ed. Plastic Surgery. Philadelphia, Pa: WB Saunders Co; 1990;4:3101-3160.


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