Cleft / Craniofacial

Question 1

Cleft Lip / Palate Incidence

Answer 1

Cleft lip (with / without cleft palate) = 1/700 - 1/940
Cleft palate (without cleft lip) = 1/1574
Native Americans and Asian populations have the highest incidence (1/500)
African descent has lowest incided (1/2500)
Cleft lip more likely in males, left-sided, 15% syndromic association
Isolated cleft palate higher in females (3:2), and 50% associated with sequence/syndrome

Question 2

Development

Answer 2

6 weeks gestation: Median nasal process fuses with maxillary process to form upper lip, philtrum, base of nose, primary palate. Failure produces cleft lip / alveolous
8-12 weeks gestation: Palatine shelves of the maxillary processes merge in the midline to fuse with nasal septum / vomer to form posterior palate (anterior to posterior). Clefting is dependent on timing of disruption.
Factors: Family history (4, 9, and 16 percent for parent or sibling, two siblings, one sibling and parent), zinc or folate deficiency, smoking/alcohol exposure, anticonvulsants

Question 3

Millard’s modified Kernahan / Stark classification

Answer 3

AP classification of cleft lip / palate. Does not describe completeness or VPI

Question 4

Sequence

Answer 4

Team approach
Ultrasound 13-20 weeks, cleft palate harder to detect. Chromosomal testing and echo for isolated cleft palate patients.
1) Lactation / speech therapy consultation at birth (pigeon bottle, Haberman with one-way valve, Dr. Brown’s cleft palate bottle). Feed early and often, sitting baby upright.
2) Birth - 3 months: Preoperative orthopedics - taping, DynaCleft, molding appliance, Latham appliance - improves tension free closure, goal to get segments within 5mm
3) 9-12 weeks: cheilorrhaphy - 10 weeks of age, 10 pounds, 10mg/dL of hemoglobin. Millard
4) 9 -12 months: palate repair
5) Speech therapy
6) 4+ years: VPI surgery
7) 6-9 years: orthodontic treatment, alveolar cleft grafting
8) 16 - 18 years: orthognathic surgery, rhinoplasty as needed

Question 5

Cheilorhinoplasty

Answer 5

• Reapproximate nasal sill, vermillion border, and cupid’s bow
• Mucosa, orbicularis oris, and skin
• Millard approach (backcut along the nasal columella and rotation of the flap to the contralateral side)
• Complications: wound dehiscence, partial necrosis of the philtrum, cicatricial scarring, hypertrophic scarring, vermilion alignment, whistle deformity

Question 6

Primary Palatoplasty

Answer 6

• Closure of oro-antral communication, anatomic/functional re-alignment of the soft palate musculature, restore velopharyngeal valve
• Bardach layered closure with anterior releasing incision
• Complication: Bleeding, fistula, VPI, maxillary hypoplasia

Question 7

VPI

Answer 7

Inability to close the connection between the nasopharynx and the oropharynx due to anatomical dysfunction in the soft palate, lateral pharyngeal wall, or posterior pharyngeal wall

• Diagnoses via speech evaluation, nasoendoscopy, videofluoroscopy
• Superior based pharyngeal flap with proper lateral wall motion (creates two lateral tunnels)
• Sphincter pharyngoplasty for those with inadequate lateral wall movement (palatopharyngeus muscle)

Question 8

Orthodontic intervention

Answer 8

Maxillary expansion

• improve buccal crossbite
• Uncover occult nasal fistula
• Facilitate nasal closure
• Requires eruption of first molars

Question 9

Alveolar Bone Grafting

Answer 9

Allow eruption of dentition, provide periodontal / bone support, stabilization of maxillary segments, closure of oro-nasal fistula, improve speech/language development, reconstruct nasal floor and lift the alar base, allow for greater lip support