Cleft Lip
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oris which is inserted into the cleft margin and alar wing. In addition,
the children will have a predictable pattern of nasal deformities including a
caudally dislocated nasal septum separated from a displaced anterior nasal
spine of the maxilla, a shortened columella, attenuated flattened lower lateral
nasal cartilage on the cleft side with the flared alar base, and an inferiorly
rotated upper later nasal cartilage. Also, patients with cleft lips inherently will
have some degree of the alveolar cleft with potential for collapse of the
maxillary arch and class III malocclusion (the maxillary teeth sit posterior to
the mandibular teeth). These hard and soft tissue anatomic changes translate
to the various changes in appearance, speech, and swallowing/feeding seen in
cleft lip patients. [7]
Cleft lip usually develops at the junction between the lateral and central
segments of the upper lip. The cleft usually affects the upper lip and may
extend into the maxilla and palate.
History and Physical
The first evaluations of patients with cleft lip occur at an early age as the
physical appearance is readily noted on examination after birth. In early cleft
evaluation, one must note concomitant cleft palate as this will have several
implications on feeding, swallowing, and speech. One should note the width
of the cleft, whether the cleft is unilateral or bilateral, and whether it is
incomplete or complete. Alveolar clefts should also be noted carefully. In
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bilateral clefts, the premaxilla may be anteriorly displaced, which may require
intervention before surgery with naso-alveolar molding (NAM).
Evaluation
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It is vital for cleft care to involve an interprofessional team early on and to
evaluate the patient from head to toe for other medical comorbidities and
associated syndromes. Pediatric/neonatal intensive care teams are vital to the
early care of these children for required medical needs. Genetics consults for
patients in whom an associated syndrome is suspected are important. Any
abnormalities noted should receive indicated work-up. Speech-
language therapists and nutrition consults are usually required to teach parents
techniques to meet the special feeding needs of these children. When patients
do not meet feeding requirements for adequate nutrition, which is most
common when there is a concomitant cleft palate, feeding access is sometimes
required with the assistance of the pediatric surgery team. Establishment of
care with orthodontists and plastic surgeons or otolaryngologists who
specialize in patients with cleft lip deformity is important to assess the need
for interventions and follow these patients long term. [8][9] Today, antenatal
ultrasound can easily diagnose cleft deformity during the second trimester.
Treatment / Management
In neonates with a cleft lip the three major concerns are:
Difficulties with feeding
Risk of aspiration
Airway obstruction
Treatment of patients with cleft lip deformity is a long-term commitment.
Medical treatment will largely focus on requirements from any concomitant
congenital abnormalities and based on nutritional needs. Within the first few
weeks to months of life, NAM can be employed with assistance from an
orthodontist. This involves the creation of an orthodontic appliance that molds
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a protruding premaxillary segment and alveolar process into a more favorable
position. This allows for repositioning of the alveolar segments, medialization
of the alar base, and columellar lengthening, which allows for easier surgical
repair of cleft lip and nasal cleft deformity down the line. These require
frequent adjustments by the orthodontist. Other treatment adjuncts that assist
with decreasing the severity/width of the cleft early on are lip taping (often
performed in patients with less severe clefts) and lip adhesion (an
approximation of the cleft lip edges without changing lip landmarks or
disturbing tissue required for definitive closure, often used in patients with
wide clefts who are poor NAM candidates for social or geographic reasons).
Surgical intervention for initial cleft lip usually occurs at 3 to 5 months of
age. A good rule of thumb in deciding the age at which is it safe to perform
primary cleft lip repair is the "Rule of 10s.” If the infant is ten weeks old, 10
pounds, and hemoglobin has reached 10mg/dL, surgical repair should be safe
if no other comorbidities preclude it. There are many accepted surgical
techniques for primary repair of unilateral (Millard repair rotation
advancement, Fisher repair, and Mohler repair) and bilateral (Mulliken repair)
cleft lips, and the surgical details are out of the scope of this article. However,
common goals in all repairs are to re-establish a competent orbicularis oris
muscle, lengthen the philtrum and lip, and minimize visible scarring. In
primary cleft repair, some surgeons perform gingivoperiosteoplasty, which
involved the elevation of the mucoperiosteal flaps along an alveolar segment
with wide-undermining to promote bone growth along the periosteum, but
this is not a universal practice. [10][11] Because many of these children also
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have otitis media, it is important to have an ENT specialist involved in the
care. During the cleft lip repair, ventilation tubes are placed but children
continue to have eustachian tube dysfunction for many years.