Unilateral Cleft Lip

10.1055/b-0034-97701

Unilateral Cleft Lip

Albert S. Woo

Full-term newborn male presents to the clinic with the displayed congenital anomaly.

Description

Complete unilateral cleft lip deformity
- Cleft nasal deformity: Nostril is widened and slumped (alar cartilage is inferiorly, posteriorly, and laterally displaced), but not hypoplastic. The nasal tip is bulbous and shifted toward the cleft.
- Septal deformity: The septum is shifted away from the cleft.
Alveolar cleft.
Complete unilateral cleft palate.

Work-up

History

Family history of orofacial clefting.
Feeding difficulties, appropriate weight gain.
Additional medical problems and associated syndromes.

Physical examination

Evaluate involved structures (lip, alveolus, palate, unilateral, bilateral).
Evaluate for associated birth anomalies consistent with a syndromic presentation.

Diagnostic studies

Only if concern for other systemic illness or syndrome

Consultations

Best managed by a multidisciplinary team: Plastic surgery, pediatric otolaryngology, speech pathology, child psychology, audiology, genetics, pediatric dentistry, orthodontics, maxillofacial surgery, social work, and nursing.
Genetic evaluation if any concern exists.

Treatment

Management via a multidisciplinary team.

Schedule of treatment

Multiple procedures anticipated (see Table 11.1 for cleft management timeline).

Timeline for the management of a child with cleft lip and palate deformity
Age	Treatment
Newborn	Feeding assessment, initial clinical evaluation, possible genetics referral
0–3 mo	Molding therapy, possible cleft lip adhesion
3 mo (or after molding)	Definitive cleft lip repair
1 y	Cleft palate repair
3–4 y	Assessment of velopharyngeal competence
7–10 y	Alveolar bone grafting following presurgical orthodontics (during period of mixed dentition)
Skeletal maturity	Septorhinoplasty, final revisions as necessary; orthognathic surgery if evidence of midfacial growth disturbance

Feeding: critical aspect of cleft care.
- Specialized nipples/bottles: Haberman feeder (with a squeezable tip), or Pigeon nipple (with cross-cut opening for faster flow), or Dr. Brown′s Level 2 nipple with Pigeon valve.
Molding: Narrows cleft and aligns alveolar arch to optimize repair.
- Not employing any molding technique is also a reasonable option.
- Lip taping: With Steri-Strips or commercially available devices (e.g., DynaCleft; Canica Design, Almonte, Ontario, Canada).
- Nasoalveolar molding (NAM)
  - Passive molding appliance rapidly becoming the gold standard for optimizing nasal shape.
  - Alveolar molding alone takes place until alveolar ridges are 5 mm apart, then nasal prongs are attached to improve the shape of the nose.
- Latham appliance
  - Active molding appliance expands palate and retracts premaxilla.
  - Less commonly used because of concerns regarding maxillary growth.
Lip adhesion (not mandatory)
- Performed surgically, in place of molding techniques.
- Preliminary repair of skin ± muscle between 6 weeks and 3 months of age.
- Goal: Minimize tension during the definitive cleft repair performed around 3 to 6 months of age.
Cleft lip repair: At approximately 3 months of age
- Rule of 10s: 10 lb of weight, 10 g of hemoglobin, 10 weeks of age.
- May be delayed secondary to molding (NAM) or earlier lip adhesion.
Cleft palate repair: At approximately 1 year of age
- Earlier repairs favor speech but potentially compromise maxillary growth and vice versa.
Alveolar bone grafting
- Performed during period of mixed dentition (roughly 7 to 10 years of age) after appropriate orthodontics.
Cleft nasal/septal reconstruction
- Optimally performed once the patient has reached skeletal maturity. Can be combined with “touch-up” procedures to optimize appearance.
- Septoplasty is frequently deferred until this time.