Cleft lip and palate

Cleft palate is the most typical malformation of this kind in Finland. The extent of the cleft varies, but it always runs along the centerline of the palate. Cleft palate is the most common type among girls, while other types of cleft are more common among boys.

Submucous cleft palate  

A submucous cleft palate is a condition where the cleft is covered by a mucous membrane. Because the roof of the mouth looks intact, the only visible indications of a submucous cleft palate are a split uvula and a translucent line along the centerline of the palate. A submucous cleft palate does not always require surgical treatment.

Cleft soft palate  

A cleft soft palate is a cleft only at the back of the mouth, extending along all or part of the soft palate.

Cleft hard and soft palate  

This is the most common type of cleft palate, where the cleft, though variable in length, extends from the soft palate (at the back of the roof of the mouth) to the hard palate (at the front).

Both a cleft lip and a cleft lip and alveolus may vary in their extent and may occur on only one side or on both sides.

Cleft lip 

A cleft lip may vary in extent and may occur on only one side or on both sides. The mildest cases present only as a tiny dent at the site of the cleft, while the most severe cases can cause substantial asymmetry not only in the lip but also in the nostril on the side of the cleft. The bone structure, however, is unaffected in all cases.

Cleft lip and alveolus 

A cleft lip and alveolus extends across the lip and gums. It may be unilateral (on one side only, 2B), or bilateral (on both sides, 2A). A unilateral cleft lip and alveolus may, at its mildest, be similar in appearance to a simple cleft lip, but a cleft alveolus always includes an abnormality of the bone structure of the dental arch at the site of the cleft. In a severe case of cleft lip and alveolus, the cleft may be broad and extend to the nose and upper dental arch.

A cleft lip/palate may be of variable extent across the lip, gums and palate. It may be bilateral (on both sides) or unilateral (on one side).

A unilateral cleft lip and palate (Figure 1) often presents with significant asymmetry in the area of the nose, lip and upper dental arch.

With a bilateral cleft lip and palate (Figure 2), the central part of the upper jaw and upper dental arch is twisted up and forward. The tip of the nose is flat and broad.

Cleft lip, and cleft lip and alveolus

We perform corrective surgery on a cleft lip when the child is 3 to 6 months old. The corrective surgery for a bone transplant to correct the bone structure malformation under the gums (cleft alveolus) will be performed much later, at the age of 9 to 11 years.

Cleft palate

We perform corrective surgery to close up a cleft palate when the child is about 9 months old. We close the cleft by separating folds of mucous membrane on the soft and hard palates and by stitching the cleft shut.

Cleft lip and palate

Currently, we generally correct a cleft lip and palate in two separate surgical procedures. First, we close the cleft in the lip and the hard palate at the age of 4 months. We close the cleft in the soft palate at the age of 10 months. The corrective surgery for a cleft alveolus is performed at the age of 9 to 11 years.

Correcting the nose and/or lip 

In cases with an extensive cleft, whether unilateral or bilateral, shaping the nose is difficult and often requires further corrective surgery. In bilateral cases, it is almost always necessary to perform further corrective surgery on the nose to adjust the broad, flat profile of the nose. We correct the structure of the nose with cartilage grafts at the age of 6 years or later, but ultimately the nose and lips cannot be finally corrected until the child has stopped growing.

Surgery to improve speech

Children who have had a cleft palate may initially have less clear and more nasal speech than their peers. This generally corrects itself or can be remedied with speech therapy. However, about one in five of these children must later have surgery to narrow the velic passage to improve the quality of the speaking voice.

Bone graft

In cases of unilateral and bilateral cleft lip and palate and cleft lip and alveolus, we usually perform bone graft surgery to repair the structure of the alveolus at the age of 9 to 11 years (Figure 1). The purpose of this is to correct the bone deficiency in the structure of the alveolar ridge and the nasal floor and to ensure that the permanent teeth will erupt properly. We usually use spongy bone taken from the iliac crest as the graft. Orthodontic treatment is almost always required in connection with a bone graft operation.

Surgical treatment of jaw growth defects

Nearly half of the patients who have a unilateral or bilateral cleft lip and palate will develop a growth anomaly in the upper jaw and midface in adolescence. The related severe crossbite may significantly impede the functioning of the jaw and affect the appearance of the face.

Growth anomalies in the upper jaw cannot be corrected by orthodontic treatment alone; surgery is required to correct the bone imbalance between the mandibles.

Growth anomalies in the upper jaw are usually treated with a surgical procedure known as a Le Fort I osteotomy. We usually perform this operation once the child’s growth has ended. In the most severe cases, two operations may be required – one during growth and one towards the end of the growth period.

Sometimes surgery to the lower jaw is necessary for correcting the facial structure. In addition to correcting the bite, we focus particular attention on the appearance of the face and its proportions when performing an osteotomy on a cleft patient. The nose shape typical for cleft patients may be enhanced by a Le Fort I osteotomy. Because of this, we usually perform corrective rhinoplasty (nose surgery) at the same time.

Cases of cleft lip and/or palate involve a higher than normal rate of dental anomalies, bite anomalies and jaw growth anomalies. Nearly all children born with a cleft lip/palate require orthodontic treatment at school age because of this. They may also require dental prosthetics (replacing their own teeth with synthetic ones) and corrective surgery of the jaw to correct growth anomalies. Orthodontic treatment is also almost always required in connection with a bone graft operation.

Dental anomalies typical for cleft lip/palate cases include an abnormal number of teeth, and anomalies in tooth structure, formation, and eruption. These anomalies are more common for permanent teeth than for baby teeth.

The more extensive the cleft was, the more probable it is that there are anomalies in the permanent teeth. We can correct dental anomalies either by closing the space of missing teeth with orthodontic treatment or by installing prosthetics to fill in for the missing teeth.

Bite defects are also usually the more difficult the more extensive the cleft originally was. Typical bite defects include misalignment of teeth at the cleft site, crowding and maxillary deficiency, which may lead to a crossbite in the front or at the side.

Only some cleft patients require dental prosthetics. The majority of these are adolescents and young adults, but ageing patients also require new prosthetics and prosthetic repairs.

The patients with the greatest need for prosthetics are those who have had a cleft lip and alveolus or a cleft lip and palate, because these conditions usually cause the greatest changes in the bone structure of the dental arch and in the teeth. Dental prosthetics form part of the comprehensive treatment of cleft lip/palate.

The Pierre Robin sequence is a syndrome whose features include cleft lip and palate, a small jaw and breathing problems. The small jaw causes the tongue to fall back into the throat, constricting the airway. Breathing is difficult particularly when the infant is lying on his/her back. The infant also has excessive trouble breathing when feeding and gets tired quickly, hence not being able to eat enough.

If an infant is found to have a cleft palate and a small jaw, then breathing and weight development must be monitored. Oxygen saturation in the infant’s tissues can be monitored at the hospital or with an oximeter at home. Blood carbon dioxide levels can be measured with laboratory tests. If even a slight breathing problem is suspected, we recommend a test of breathing while sleeping, using polysomnography.

With mild breathing problems, positioning may be sufficient treatment (i.e. placing the infant in a prone or side position for sleeping); but in severe cases breathing may need to be assisted with nose tubes, a device in the mouth to push the tongue forward, surgically attaching the tongue to the lower gums or, in extreme cases, an opening made in the windpipe (tracheostomy). A nasogastric tube (from nose to stomach) may be needed as a temporary feeding aid.

An infant may initially seem normal and then develop breathing problems at 3 to 4 weeks. This usually corrects itself within a few months. 

Hemifacial microsomia is the second most common facial birth defect after cleft lip/palate. Its incidence is estimated to be from 1 in 3,500 to 1 in 5,600.

A typical case of hemifacial microsomia presents as bilateral or unilateral skewing of the jaw, a malformation of the ear, ear canal or inner ear, and facial soft tissue deficiencies.

In such a case, the infant may also have a lateral cleft at the corner of the mouth or, more rarely, a cleft lip or cleft lip and palate.

CATCH-22 syndrome gets its name from an acronym:

• C, cardiac: structural malformation in the heart, or heart defect;
• A, abnormal facies: malformations of the face typical for this syndrome;
• T, thymus; hypoplastic, underdeveloped, thymus, one of the endocrine glands;
• C, cleft: generally a cleft palate;
• H, hypoparathyreosis: decreased function of the parathyroid gland.

The number 22 refers to the cause of the syndrome, a microdeletion in chromosome number 22.
Symptoms and findings vary widely. Slight delays in speech development along with learning difficulties are also typical of this syndrome. Its incidence is estimated to be from 1 in 4,000 to 1 in 5,000.