Congenital clefts of upper lip occur because of failure of fusion of the maxillary, medial, and lateral nasal processes. They vary from a notch in the upper lip to a cleft through the lip and the floor of the nose involving the alveolar ridge, or they may be complete cleft of the lip and the palate.

The cleft palate is divided into prepalatal and postpalatal clefts. The incisive foramen marks the boundary between the two. They both have different embryologic development. A prepalatal cleft involves the anterior palate, alveolus, lip, nostril floor, and ala nasi. Postpalatal clefts may extend anywhere from soft and hard palate to the incisive foramen. The third type is a submucosal cleft in which a bone defect exists without a mucosal defect. The most common cleft of the palate is a left complete cleft of the prepalatal and palatal structures. The second most common is a midline cleft of all the soft palate and part of the hard palate without a cleft in the prepalatal area. Various degrees of the cleft palate and lip are shown in Figure 42.1.

Major components of face develop between 4 and 7 weeks of fetal life. There are three mesodermal islands, one central and two lateral. Prepalatal clefts are caused by the failure of these mesodermal elements to develop and fuse. Palatal clefts are caused when the palatal ridges fail to migrate medially, contact, and fuse (Fig. 42.1).

Cleft lip and cleft palate may occur together or separately. A cleft lip with or without a cleft palate occurs in 1:1,000 births. A cleft palate alone occurs in approximately 1:2,500 births. A cleft lip with or without a cleft palate is more common in boys, whereas isolated cleft palate is more common in girls.

The highest incidence of a cleft lip and a cleft palate occurs among Asians (1.61:1,000 births), whereas the lowest incidence is in African Americans (0.3:1,000 births). The incidence in Whites is intermediate (0.9:1,000 births).

A cleft palate is responsible for some major physiologic disorders. The pharynx communicates more extensively with the nasal fossae and the oral cavity. The complex mechanisms of swallowing, breathing, hearing (through the eustachian tube), and speech are, therefore, impaired.

The presence of a cleft lip and palate in a neonate results in feeding difficulties. The neonate cannot suck because the cleft makes the creation of a negative pressure difficult. Breast-feeding is improbable and bottle-feeding is difficult.

Essentially, all children with a cleft palate have middle ear disease because abnormal anatomy of the nasopharynx adversely affects eustachian tube function. Chronic middle ear effusion produces varying degree of conductive hearing loss. Nasal septation between food and air is absent, creating a nonphysiologic mixing chamber in the nasopharynx. Not infrequently, this is associated with chronic rhinorrhea that must be distinguished preoperatively from infection.

Secondary defects of tooth development, growth of the ala nasi, and velopharyngeal function (contact between the soft palate and the posterior pharynx for speech and swallowing) can also occur.

Psychological problems may be considerable as this youngster approaches school age and peer association. The speech of these children is typically nasal with an inability to sound the so-called plosives (p/k/d/t) and fricatives (s/f).

Associated anomalies occur 30 times more frequently in the patient with isolated cleft palate than in the noncleft population.

The most common non-syndrome-related abnormalities are umbilical hernias, clubfoot, and limb and ear deformities.

The most common syndromes associated with a cleft lip and a cleft palate are the following:

This syndrome is characterized by retrognathia or micrognathia, glossoptosis, and airway obstruction. For facial cleft surgeons, Pierre Robin syndrome is defined by a triad of clinical criteria:

An incomplete cleft of the palate is associated with the syndrome in approximately 50% of these patients. In patients with micrognathia (small jaw) or retrognathia, the chin is posteriorly displaced causing the tongue to fall backward toward the posterior pharyngeal wall. This results in obstruction of the airway on inspiration. Crying or straining by these children can often keep the airway open. However, when the child relaxes or sleeps, airway obstruction occurs. Because of these respiratory problems, feeding may become very difficult. This can lead to a sequence of events: glossoptosis, airway obstruction, crying or straining with increased energy expenditure, and decreased oral intake. This vicious cycle of events if untreated can led to exhaustion, cardiac failure, and ultimately death.

It is highly complex disease process consisting of the following:

These patients need a pediatrician to maintain their overall health and a surgeon and an anesthesiologist to accomplish the various surgeries. They need a speech therapist to prevent or
overcome the speech deficiencies and an orthodontist for early orthodontic intervention to develop and maintain relatively normal bite and dentition. The care team should also include a geneticist, pediatric dentist, prosthodontist, psychologist, medical social worker, and public health nurse.

Functional goals of cleft palate surgery are normal speech, hearing, and maxillofacial growth. It is thought that speech and hearing are improved by early cleft palate repair (before 24 months of age) and that the delayed closure (after 4 years) is associated with less retardation of midfacial growth. An early two-stage palate repair is advocated in management of these patients. The recommended sequence involves closure of the soft palate at 3 to 6 months of age with secondary closure of the residual hard palate at 15 to 18 months of age. This sequence takes advantage of the early physiology and growth that occurs in the soft palate, which is vital in the development of speech. Furthermore, it avoids the potential pitfalls of the growth disturbance related to early periosteal undermining of palatal and vomerine tissue. This repair sequence also provides total palatal closure before speech evolves. If soft palate is repaired at the time of lip repair, an additional anesthetic is avoided.