It is the second most common malformation in newborns worldwide and initially gives many parents a shock: harelip. Perhaps you have also received this diagnosis and now need all the information you can get?

Here you will find everything you need to know.

Harelip Definition – What Is It?

The term harelip is used colloquially for orofacial clefts. The malformation (cleft formation) can occur on the lips, palate and jaw.

The extent of the cleft varies from person to person and may affect only one, two or all three of the above body parts. The cleft may be unilateral or bilateral. Doctors distinguish the following three types of clefts: Cleft lip or cleft lip and jaw.

About 20 percent of affected people have this type of cleft, the majority of them with unilateral expression. Terms used: cleft lip, cleft lip, cleft lip and jaw.

Cleft palate (soft palate cleft and hard and soft palate cleft). About 30 percent of those affected have such a pure cleft palate. It cannot be seen from the outside and is usually only discovered after birth. Used terms: cleft palate, palatal cleft palate, obsolete: Wolf cleft

Clefts Of The Lips, Palate, And Jaw

This complete cleft lip and palate is the most common subtype of orofacial clefts. It represents a combination of cleft palate and cleft lip and jaw and occurs in 50 percent of affected individuals.

The majority show a unilateral expression, usually on the left side. Terms used: cleft lip and palate (LKG cleft), cleft lip and palate (LKGS cleft).

All three types of clefts entail consequences for important functions such as breathing, swallowing, hearing and speaking. Healthy physical and psychological development is impaired, and growth and aesthetics are disturbed.

Surgical treatment is necessary. Medical professionals from different specialties are often involved in the treatment (pediatricians, oral and maxillofacial surgeons, orthodontists, dentists, ENT specialists, speech therapists and psychologists).

Important Information About The Term Harelip

The term harelip first appears in German-language medical literature in 1460.

And although zoomorphic terms are widely used in medical and colloquial language, and the term hasenscharte is still used in everyday speech, the term is now considered pejorative and obsolete.

More commonly, neutral terms such as cleft lip or cleft lip and palate are used instead. Medical professionals and health care providers do not use the term harelip because studies have shown that the term is associated with negative and inaccurate ideas.

Many adults with successfully operated orofacial clefts find the term harelip unpleasant.

Is The Diagnosis Of Harelip Made At Ultrasound Or Only At Birth?

A harelip can be detected by ultrasound diagnosis. During normal screening ultrasound, about half of the cases are detected.

This is because looking at the face is not a focus of screening and is not currently required by law.

If the maternity guidelines required this, more cases could possibly be detected early, as is the case in other European countries.

The other half of cases – and especially pure cleft palate – are not detected until after birth. Clefts on the palate are not visible from the outside, and a submucous (hidden) cleft palate often remains undetected even until language acquisition.

Parents who fear a malformation are advised by the DEGUM (German Society for Ultrasound in Medicine) to use fine diagnostic ultrasound to specifically look for abnormalities in the mouth area.

Experienced prenatal physicians can detect a facial anomaly from about the 14th week of gestation. In cases of increased risk or concrete suspicion, the health insurance company pays for the examination (corresponding referral from the gynecologist is necessary).

Those who pay for the fine-diagnostic examination out of their own pocket must expect costs of 200 to 400 euros.

How Often Does Cleft Lip Occur?

Harelip is the second most common congenital malformation worldwide. Only heart defects occur more frequently in newborns. Globally, up to 15 percent of children are born with orofacial clefts.

In Europe, one baby out of every 500 newborns has a harelip, or 0.2 percent.

In Germany, the figure is even lower: in this country, 0.15 percent of newborns are born with such anomalies (data from the German Society for Ultrasound in Medicine (DEGUM) from 2014).

Harelip: Cause And Development

The question of the cause of a harelip moves many parents. Especially if family planning has not yet been completed, the probability of recurrence is an important aspect.

So far, the exact cause of a cleft lip (cleft lip, cleft palate or cleft lip and palate) has not been conclusively clarified.

It is believed that several factors can cause such a deformity. There are “syndromic clefts” and “nonsyndromic clefts”. The latter are more common.

In “syndromal clefts”, there is an overriding syndrome (for example, Van der Woude syndrome or trisomy 13). An estimated 30 percent of those with cleft lip and palate have a syndrome.

Among patients with cleft palate, the figure is 50 percent. The syndrome can cause there to be other malformations of other organs.

For this reason, the other organs, such as the heart, should be looked at very closely even during the ultrasound examination. Because genetic factors play a role in “syndromal clefts,” relatives may also have clefts on the lips, jaw, or palate.

“Non-syndromal” clefts can occur due to external and internal influences.

Under discussion is, for example, malnutrition, stress, or the intake of medications and stimulants in early pregnancy (i.e. the phase during which the development of the face takes place). However, the effect of individual factors has not yet been definitively clarified.

A harelip develops approximately between the fifth and twelfth week of pregnancy. Basically, individual parts of the face initially develop separately from each other.

The nose, upper lip and jaw merge between the fifth and seventh weeks of pregnancy. During the 10th to 12th week of pregnancy, the individual parts of the palate unite and the palate is formed.

If there is a disruption of these developmental steps, or if the tissue ruptures again, the cleft formation occurs – harelip.

Harelip – Surgery And Treatment

Although there are a variety of cleft types and severities in harelip, the treatment process is largely uniform.

Timely closure of the various cleft segments is essential to allow normal development. Exactly what the treatment looks like depends on the cleft form/severity.

Here is an example of a typical treatment plan, based on the Regensburg University Hospital:

Before Birth

If it is known that a harelip is present: Education about the clinical picture, information about treatment options/treatment course.

Birth

The first examination takes place, lips, jaw and palate are closely examined. The parents receive further counseling. In children with cleft palate, an impression of the palate is made.

The impression is used to make a palate plate that separates the mouth and nose. This early orthodontic treatment starts shortly after birth.

Until 4 Months Of Age

The cleft lip is surgically closed (called labiaplasty). Often the nostril is also corrected, which is also usually not properly formed on the harelip side.

Final correction of the nose is done only after the completion of growth, if necessary. In children with a pure cleft lip, surgical treatment is often completed at this point.

Only rarely is a minor corrective surgery required later. The scar of cleft lip surgery reaches its final appearance after a good 12 months and is largely inconspicuous. An ear inspection also takes place.

Until 12 Months Of Age

Even before the acquisition of speech, the cleft palate is surgically closed (called cleft palate surgery). The oral cavity and nasopharynx are separated from each other to allow unhindered speech development.

Again, an ear inspection takes place. Children with a cleft palate often suffer from a ventilation disorder of the ears and suffer recurrent effusions of the middle ear.

So-called tympanic tubes, which require an incision of the eardrum for insertion, can help against this. The insertion of the tympanic tubes can be done during cleft lip surgery or cleft palate surgery, depending on the urgency.

The insertion is often combined with one of the surgeries in order to spare the child another anesthesia.

Until 6 Years Of Age

Control of tooth eruption, hearing test, speech evaluation, if necessary speech therapy, if necessary corrective surgery before school enrollment.

Modern treatment plans provide for normalization of function and appearance before school enrollment. For this purpose, regular checks of the development take place, therapies can be started early and possible corrections can be made.

Today, surgical corrective interventions are rare, but still occur. These include operations to improve speech (velopharyngeoplasty, VPP) and lengthening of the nasal bridge (columellaplasty).

Until 11 Years Of Age

Only for certain types of cleft jaw: cleft jaw is surgically closed using the patient’s own bone (called cleft jaw osteoplasty).

This may be followed by orthodontic treatment in which lateral incisors and/or canines are moved into the correct position.

Adults

Final surgical corrections after growth completion. Physical growth is usually completed between the ages of 16 and 18.

Then, for example, growth-related changes in surgical results can be corrected. Possible corrective surgeries include rhinoplasty, lip correction, jaw displacement surgery, and dental surgery and implants.

Is Harelip Visible Or Noticeable As An Adult?

Depending on how severe the deformity was, permanent – visible and/or audible features – may result.

However, this is not typical, as nowadays a comprehensive, interdisciplinary treatment concept is applied, in which aesthetics and function are normalized. However, it is important for parents to attend regular check-ups with their child and to keep therapy or surgery appointments.

Generally, surgical scars reach their final appearance after about a year and are inconspicuous in adulthood.

In addition, corrective surgery can be performed after the completion of the growth phase to achieve an all-around satisfactory result.