CLEFT LIP AND PALATE

INTRODUCTION

• Cleft lip with or without palate (CL/P) is the most common

craniofacial birth defect with an estimated quarter of a million
affected babies born each year in the world
• Birth defects are emerging as a cause of neonatal mortality in
countries that have made progress in controlling infectious
diseases and malnutrition
Strategies to reduce the global impact
of birth defects
1. effective family planning, genetic counseling, and prenatal diagnosis
2. education for couples to decrease maternal exposure to avoidable environmental risk
factors such as tobacco, alcohol, and teratogenic medications
3. Improving periconception maternal intake of micronutrients such as folic acid (400 μg)
4. improving the availability of medical and surgical care locally for the affected infants
 Epidemiology of oral clefts

 Cleft lip and palate 46%, Cleft palate only 33%, Cleft lip only 21%
Cleft lip with or without palate
 Average birth prevalence 1 : 700
 More common in males
 Unilateral > bilateral and Left side > right side
 Association with other anomalies 10%
Cleft palate only
 Average birth prevalence 1 : 2000
 More common in females
 Association with other anomalies 50–60%
 ETIOLOGI
Interaction gen to gen
 Mutations in IRF6 lead to Van
der Woude and popliteal
pterygium syndromes
 Mutations in other
genes, TBX22, FGFR1, and P63,
also contribute to
syndromic cleft
 Aberrant transforming growth
factor beta-3 (TGF- β3)
Interaction gen - environment
 Cigarette smoking, folic acid
defiiency during the
periconceptional period, and
maternal exposure to alcohol
and teratogenic medications
such as retinoids, corticosteroids
& anticonvulsants (phenytoin
and valproic acid)
 Co-sanguinous marriages,
maternal diabetes, and obesity
 maternal viral infections such as
rubella and varicell
 Embryology

• begins at 4 weeks after conception from the neural crest

ectomesenchyme that forms fie prominences; the frontonasal
process, and paired maxillary and mandibular processes
surrounding a central depression
• During the fith and sixth weeks of embryonic development
bilateral maxillary processes derived from fist brachial arch fuse
with the medial nasal process to form the upper lip, alveolus, and
the primary palate
• The lateral nasal process forms the alar structures of the nose. The
lower lip and jaw are formed
by the mandibular processes
 Classification

Veau
1. cleft of the soft palate;
2. cleft of the hard &soft palate
up to incisive foramen;
3. complete unilateral cleft lip
and palate;
4. complete bilateral cleft lip
and palate
Berkowitz
1. Clefts of lip & alveolus.
2. Clefts of primary (include lip)
& secondary palate.
3. Clefts of secondary palate
only
4. Submucous cleft
 Timing of treatment I
• Prenatal  Diagnosis and parental counseling
• 0–6 months
General assessment for associated anomalies ENT evaluation –
breathing, feeding, swallowing, and hearing; Presurgical
orthopedics (0–3 months); Primary lip repair (3–4 months)
• 6 months–2 years
Speech and oral sensory motor assessment; Grommets/ear
tubes (as needed); Primary palate repair (9–12 months)
• Preschool: 3–5 years
Dental care; Speech assessment & therapy (continue as needed);
Assess need for lip revision
 Timing of treatment II

• Childhood: 6–12 years

Correction of velo-pharyngeal dysfunction (as needed);
Orthodontic treatment – phase I; Alveolar cleft repair (8–
11 years)
• Adolescence: 13–18 years
Orthodontic treatment – phase II; Orthognathic surgery
(if needed) – 14–16 years (female),16–18 years (male)
Revision chielorhinoplasty
Replacement of missing teeth (as needed)
Feeding & Nutrition
• squeezable bottles may be easier to use than rigid bottles for
children with CL/P
• A feeding tube

Ear, nose, and throat evaluation

 If there are signs of airway obstruction, a pediatric
otolaryngologist should be consulted to perform an
endoscopic evaluation of upper and lower airway to
look for possible cause of obstruction
 An audiology assessment is recommended soon
after birth to check for hearing abnormalities
 Early speech & language stimulation & an initial
speech evaluation no later than 6 months after birth
are recommended for children with clefts of palate.
 Presurgical Orthopedics
Benefit
better alignment of the
alveolar segments &
premaxilla, tension-free
approximation of the cleft lip
edges, improvement of nostril
symmetry & shape
Example
 palatal acrylic plate bring the
collapsed maxillary alveolar
segments into proper alignment
prior to lip surgery
 Active expansion device  align
the collapsed lateral maxillary
segments & retract the premaxilla
in complete bilateral clefts & to
achieve symmetry of the alveolar
arch in complete unilateral clefts
 Cleft Lip Repair

• The goal  reconstruct a functional lip with minimal scarring and normal appearance
• The timing  between 3 and 6 months after birth, the “rule of 10s” (at least 10 weeks
of age, weigh at least 10 lb, and have a hemoglobin level of at least 10 g/100 mL
• A surgical lip adhesion may be preferred as an initial surgical procedure within 6–8
weeks after birth
Advantage  to align the maxillary alveolar segments and achieve a tension-free
defiitive lip repair at a later date, allows the surgeon to perform a
gingivoperiosteoplasty at the time of defiitive cheiloplasty
Disadvantage  the need for an extra operation and the
possibility of excising more tissue at the time of defiitive lip repair.
 Unilateral Cleft Lip

Is an asymmetric deformity that presents

with a multitude of inherent anatomic
variations such as abnormal position of the
orbicularis oris muscle
 Technique Unilateral Cleft Lip Repair

1. Millard (rotation–advancement concept)  the medial flp is rotated

downward to achieve length, while the lateral flp is advanced
• The advantage the suture line lies on the recreated philtral column &
incision allows easy access for primary rhinoplasty to reposition the nasal
septum,lower lateral cartilage, and alar base
• Disadvantage  the inexperienced surgeon requires good surgical
judgment during the operation as it is not based on exact measurements
2. The triangular flap technique (Tennison and Randall) based on exact
measurements, can be reproduced well, and used more easily in wide clefts of
the lip.