A 10-year-old boy who underwent bilateral cleft lip repair during infancy has tightness of the upper lip. A photograph is shown above. On physical examination, the philtrum is excessively wide and hypoplastic with absence of muscle competence. Redundancy of the lower lip is also noted. Which of the following is the most appropriate method of flap reconstruction?

(A) Abbe flap
(B) Banked fork flaps
(C) Gilles fan flap
(D) Karapandzic flap
(E) Nasolabial flap

The correct response is Option A.

The most appropriate management is reconstruction with an Abbe flap. This lip-switch flap has been designed specifically to create a functional philtrum in patients who have tightness of the upper lip following cleft lip repair. After the abnormal philtrum is excised, redundant tissue is harvested from the lower lip to replace the resultant defect as an aesthetic subunit. The Abbe flap is pedicled on the submucosal labial artery of the lower lip at the superior edge of the inner free border of the lip. The pedicle is subsequently divided during a delayed second procedure, which is performed a minimum of 10 days to four weeks after flap transfer.

In adults, the reconstructed philtrum should be no wider than 10 mm and no longer than 15 mm; however some surgeons advocate using a smaller flap that has a width of 7 to 9 mm at the cupid’s bow and 4 to 5 mm at the labial-columellar junction.

Banked fork flaps were used previously for bilateral lip reconstruction. At the time of primary lip repair, extra tissue on either side of the new philtrum from the prolabium was “banked” within the creases of the alar base to be used as forked flaps for columellar reconstruction at a later date. However, it was subsequently determined that the columella in fact lies within the nasal region and that primary nasoplasty should be performed instead at the time of lip repair to create the columella. In addition to primary nasoplasty, adequate preoperative nasoalveolar molding has been determined to be more appropriate, and banked fork flaps are no longer used.

The Gilles fan and Karapandzic flaps are oral circumference advancement flaps that were originally designed for lower lip reconstruction, but are also used for upper lip defects. These flaps rotate around the commissure and narrow the oral aperture. They are not designed for reconstruction of the philtrum as an aesthetic subunit.

The nasolabial flap is a transposition flap that is derived from the nasolabial crease. It is most often used for reconstruction of the nasal alar subunit and has limited indications for lip reconstruction. It is not typically used for aesthetic subunit reconstruction of the philtrum.

References
1. Garza JR, Pessa JE, Futrell JW. Secondary deformities of the cleft lip and nose. In: Bentz ML, ed. Pediatric Plastic Surgery. Stamford, Conn: Appleton & Lange; 1998:81.
2. Stal S, Hollier L. Correction of secondary cleft lip deformities. Plast Reconst Surg. 2002;109:1672-1681.

A 5-year-old child who underwent cleft palate repair in infancy has velopharyngeal insufficiency. Nasoendoscopy shows closure of the central velopharyngeal mechanism with residual openings on the left and right. The patient is scheduled to undergo sphincter pharyngoplasty for reconstruction of the pharynx.

This procedure involves transposition of which of the following muscles?

(A) Buccinator
(B) Levator veli palatini
(C) Palatoglossus
(D) Palatopharyngeus
(E) Tensor veli palatini

The correct response is Option D.

In this child who is scheduled to undergo sphincter pharyngoplasty, the palatopharyngeus muscle is incorporated within the flap. Two techniques are used primarily for reconstruction of the pharynx in patients with velopharyngeal insufficiency. The sphincter pharyngoplasty elevates and insets the posterior tonsillar pillars, which contain the palatopharyngeus muscles, into the posterior pharyngeal wall to create a sphincter. In contrast, during pharyngeal flap reconstruction, a flap of tissue from the posterior pharyngeal wall is elevated and attached to the palate. This procedure is typically reserved for correction of deficits of the central palate.

The levator veli palatini muscle, which is found in the midline of the soft palate, is used only for pharyngeal flap reconstruction, not for sphincter pharyngoplasty. The buccinator muscle lies anterior to the pterygomandibular raphe, the palatoglossus muscles are contained within the anterior tonsillar pillars, and the tensor veli palatini is located anterior to the levator veli palatini. None of these muscles are used in sphincter pharyngoplasty.

References
1. Clemente CD, ed. Gray's Anatomy of the Human Body. 30th ed. Philadelphia, Pa: Lea & Febiger; 1985:1440-41.
2. Moore KL, Dalley AF, eds. Clinically Oriented Anatomy. 4th ed. Philadelphia, Pa: Lippincott, Williams & Wilkins; 1999:937-939.

Which of the following muscles courses around the pterygoid hamulus?

(A) Levator veli palatini
(B) Palatoglossus
(C) Palatopharyngeus
(D) Tensor veli palatini
(E) Uvula

The correct response is Option D.

The tensor veli palatini muscle descends from the base of the skull at a point adjacent to the eustachian tube, courses around the hamulus of the pterygoid, and then forms a broad aponeurosis with the contralateral muscle within the anterior soft palate.

The levator veli palatini passes posterior to the pterygoid hamulus and creates a muscular sling that is critical to palatal function. The palatoglossus and palatopharyngeus muscles are components of the palate but do not pass around the hamulus. The musculus uvula is confined within the soft palate and acts to alter the shape of the uvula.

References
1. Clemente CD, ed. Gray's Anatomy of the Human Body. 30th ed. Philadelphia, Pa: Lea & Febiger; 1985:1440-41.
2. Moore KL, Dalley AF, eds. Clinically Oriented Anatomy. 4th ed. Philadelphia, Pa: Lippincott, Williams & Wilkins; 1999:938-939.

(A) Lateral, inferior, and anterior
(B) Lateral, inferior, and posterior
(C) Medial, inferior, and posterior
(D) Medial, superior, and anterior
(E) Medial, superior, and posterior

The correct response is Option B.

In patients who have nasal deformities associated with complete unilateral cleft lip, the ala is displaced laterally, inferiorly, and posteriorly. The orbicularis oris inserts onto the alar base abnormally, on the lateral cleft side, and pulls the ala laterally and inferiorly. The medial orbicularis inserts on the nasal spine and caudal septum and pulls these structures to the contralateral side. The piriform rim, which normally supports the ala, is also deficient, allowing the ala to be displaced posteriorly on the side of the cleft.

References
1. McComb H. Primary correction of unilateral cleft lip nasal deformity: a 10-year review. Plast Reconstr Surg. 1985;75:791-799.
2. Salyer KE. Primary correction of the unilateral cleft lip nose: a 15-year experience. Plast Reconstr Surg. 1986;77:558-568.

A 6-year-old boy who underwent repair of a right-sided unilateral cleft lip and palate in infancy has an unrepaired alveolar cleft. Which of the following best describes the malocclusion on the cleft side in this patient?

(A) Angle class II, division I
(B) Angle class II, division II
(C) Deep overbite
(D) Excessive overjet
(E) Posterior crossbite

The correct response is Option E.

A child with an unrepaired alveolar cleft will exhibit collapse of the maxillary arch. The arch is deficient in all dimensions: anteroposterior, transverse, and vertical. Therefore, this child will have posterior crossbite of the maxillary dentition with respect to the mandibular dentition. In patients with crossbite, the lower dentition is positioned labial to the upper dentition; this is the opposite of normal occlusion. The arch form should be corrected orthodontically and dental compensation should be eliminated prior to bone grafting of the alveolar cleft.

The Angle classification of occlusion is based on the relationship of the mesiobuccal cusp of the maxillary first molar to the buccal groove of the mandibular first molar. In patients with class I (normal) occlusion, the mesiobuccal cusp of the maxillary first molar lies in the buccal groove of the mandibular first molar. Angle class II malocclusion is defined as the mesiobuccal cusp of the maxillary first molar located mesial (anterior) to the buccal groove of the mandibular first molar. This classification of malocclusion has two divisions; in class II, division 1, the lateral incisors are flared labially, and in class II, division 2, the incisors are lingually inclined. In Angle class III malocclusion, the mesiobuccal cusp of the maxillary first molar lies distal (posterior) to the buccal groove of the mandibular first molar and is located instead in the buccal groove of the mandibular second molar.

Overbite describes the distance between the maxillary and mandibular incisors in the vertical plane with the jaws in centric occlusion, and overjet describes the distance between the maxillary and mandibular incisors in the horizontal plane with the jaws in centric occlusion.

References
1. Ferraro JW. Oral anatomy. In: Ferraro JW, ed. Fundamentals in Maxillofacial Surgery. New York, NY: Springer-Verlag; 1997:127-157.
2. Simmons KE. Orthodontic role in clefts. In: Booth PW, Schendel SA, Hausamen JE, eds. Maxillofacial Surgery. London, England: Churchill Livingstone, Inc; 1999;2:1101-1111.
3. Vlachos CC. Orthodontic treatment for the cleft palate patient. Semin Orthod. 1996;2:197-204.

During embryologic development, which of the following structures arises from the lateral nasal processes?

(A) Columella
(B) Nasal bridge
(C) Nasal tip
(D) Nasal ala
(E) Nasal septum

The correct response is Option D.

The nasal structures form during the sixth week of gestation as the medial nasal processes enlarge and coalesce in the midline. Any abnormalities that occur during formation of the nasal structures may result in deformity, such as the cleft nasal deformity.

The nasal alae arise from the lateral nasal processes. The medial nasal processes give rise to the columella, nasal tip, philtrum, and premaxilla. The bridge and root of the nose arise from the frontonasal processes.

References
1. Gosain AK, Moore OF. Embryology of the head and neck. In: Aston SJ, Beasley RW, Thorne CH, eds. Grabb & Smith’s Plastic Surgery. 5th ed. Philadelphia, Pa: Lippincott-Raven; 1997:223-236.
2. Johnston MC, Bronsky PT, Millicovsky G. Embryogenesis of cleft lip and palate. In: McCarthy JG, ed. Plastic Surgery. Philadelphia, Pa: WB Saunders Co; 1990;4:2525.

A neonate is undergoing evaluation because of airway obstruction. Physical examination shows retrogenia and glossoptosis. Which of the following is the most appropriate initial management of the airway obstruction?

(A) Prone positioning
(B) Orotracheal intubation
(C) Lip-tongue adhesion
(D) Tracheostomy
(E) Mandibular distraction osteogenesis

The correct response is Option A.

This neonate has the triad of symptoms associated with Pierre Robin sequence: retrogenia, glossoptosis, and respiratory distress. Approximately 50% of neonates with Pierre Robin sequence have a high-arched cleft in the midline of the soft palate; clefting of the hard palate also occurs in some patients. Glossoptosis is associated with airway obstruction, increased expenditure of energy, and impaired feeding resulting from decreased caloric intake. These feeding difficulties and failure to thrive can be fatal if not managed properly.

The most appropriate initial management of airway obstruction is to place the neonate in the prone position, which relieves the glossoptosis and opens the airway. In some infants, this position must be maintained constantly, even during feeding, bathing, and diaper changing. Intubation may be necessary if prone positioning does not resolve the respiratory distress.

Lip-tongue adhesion, tracheostomy, and mandibular distraction osteogenesis are secondary procedures that should only be considered if prone positioning is unsuccessful.

References
1. Denny AD, Talisman R, Hanson PR, et al. Mandibular distraction osteogenesis in very young patients to correct airway obstruction. Plast Reconstr Surg. 2001;108:302-311.
2. Pasyayan HM, Lewis MB. Clinical experience with the Robin sequence. Cleft Palate J. 1984;21:270-276.