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 and after’ surgery are invaluable. Introduction to a parent support group and meeting parents of a child with a similar cleft who has undergone surgery may also be extremely helpful.

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 region. The facial muscles (Fig. 37.2) can be divided into the three muscular rings of Delaire.

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 vomer are completely separated from the palatine processes, the cleft palate is termed complete.

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 central palatal flbromucosa is very thin and lies directly below the floor of nose. The maxillary fibromucosa is thick and contains the greater palatine neurovascular bundle. The gingival fibromucosa lies more lateral and adjacent to the teeth.

In performing surgical closure of cleft palate the changes associated with the cleft must be understood to obtain an anatomical and functional repair. In complete cleft palate the median part of the palatal vault is absent and the palatal fibromucosa is reduced in size. The maxillary and gingival fibromucosa are not modified in thickness, width or position.