Primary
management
Antenatal
diagnosis
An antenatal diagnosis of cleft lip, whether
unilateral or bilateral, is possible by ultrasound scan after 18 weeks of
gestation. Isolated cleft palate cannot be diagnosed on an antenatal scan. When
an antenatal diagnosis is confirmed, referral to a cleft surgeon is appropriate
for counselling to allay fears. Photographs of cleft lip shown to parents
‘before
Feeding
Most babies born with cleft lip and palate
feed well and thrive provided appropriate advice is given and support available.
Some mothers are successful in breastfeeding, particularly when the cleft is
incomplete and confined to the lip. Good feeding patterns can be established
with soft bottles (e.g. Mead Johnson) and modified teats (orthodontic, Nuyk).
Simple measures, such as enlarging the hole in the teat, often suffice. Feeding
plates, constructed from a dental impression of the upper jaw, are rarely
necessary to improve feeding. Some babies are provided with an active plate
which aims not only to improve feeding but also reduce the width of the cleft
lip and palate prior to surgery. The long-term benefit of such a regime remains
unproven.
Airway
Major respiratory obstruction is uncommon and
occurs exclusively in babies with Pierre Robin sequence. Hypoxic episodes during
sleep and feeding can be life threatening. Intermittent airway obstruction is
more frequent and managed by nursing the baby prone. More severe and
persistent airway compromise can be managed by ‘retained nasopharyngeal
intubation’ to maintain the airway. Surgical adhesion of the tongue to the
lower lip (labioglossopexy) in the first few days after birth is an alternative
but less commonly practised method of management.
Principles of
cleft surgery
The ultimate goal in cleft lip and palate
management is a patient with normal appearance of lip, nose and face, whose
speech is normal and whose dentition and facial growth fall within the range of
normal development.
Surgical
techniques are aimed to restore normal anatomy. With the exception of rare
conditions such as holoprosencephaly, there is no true hypoplasia of the
tissues involved either side of the cleft. There is, however, displacement,
deformation and underdevelopment of the muscles and facial skeleton. Emphasis is
placed on muscular reconstruction of the lip, nose and face, as well as muscles
of the soft palate. Normal or near-normal anatomy promotes normal function,
thereby encouraging normal growth and development of lip, nose, palate and
facial skeleton. An in-depth understanding of the anatomy of the cleft is
invaluable if the surgeon is to achieve normal, or near-normal, anatomical
reconstruction.
Anatomy of
cleft lip and palate
Cleft lip
The abnormalities in cleft lip are the direct
consequence of disruption of the muscles of the upper lip and nasolabial
Unilateral
cleft lip. In the unilateral cleft lip, the nasolabial and bilabial muscle rings
are disrupted on one side resulting in an asymmetrical deformity involving the
external nasal cartilages, nasal septum and anterior maxilla (premaxilla) (Fig.
37.3). These deformities influence the mucocutaneous tissues causing
displacement of nasal skin on to the lip and retraction of labial skin, as well
as changes to the vermilion and lip mucosa. All of these changes need to be
considered in planning the surgical repair of the unilateral cleft lip.
Bilateral
cleft lip. In the bilateral cleft lip, the deformity is more profound but
symmetrical. The two superior muscular rings are disrupted on both sides
producing a flaring of the nose (due to lack of nasolabial muscle continuity), a
protrusive premaxilla and an area of skin in front of the premaxilla, known as
the prolabium, devoid of muscle (Fig. 37.4). As in the unilateral cleft lip, the
muscular, cartilaginous and skeletal deformities influence the mucocutaneous
tissues, which must be respected in planning the repair of the bilateral cleft
lip.
Cleft palate
Embryologically, the primary palate consists of all anatomical structures anterior to the
incisive foramen, namely the alveolus and upper lip. The secondary
palate is defined as the remainder of the palate behind the incisive foramen
divided into the hard palate and, more posteriorly, the soft palate.
Cleft
palate results in failure of fusion of the two palatine shelves. This failure
may be confined to the soft palate alone or involve both hard and soft palate.
When the cleft of the hard palate remains attached to the nasal septum and vomer,
the cleft is termed incomplete. When
the nasal septum and
Soft
palate. In the normal soft palate, closure of the velopharynx, which is
essential for normal speech, is achieved by five different muscles functioning
in a complete but coordinated fashion. In general, the muscle fibres of the
soft palate are orientated transversely with no significant attachment to the
hard palate.
In
a cleft of the soft palate, the muscle fibres are orientated in an
anteroposterior direction, insetting into the posterior edge of the hard palate
(Fig. 37.5).
Hard
palate. The normal hard palate can be divided into three anatomical and
physiological zones (Fig. 37.6). The
In performing surgical closure of cleft palate
the changes associated with the cleft must be understood to obtain an