Do you know someone who has a long face, or do you have a long face?

There is a rare syndrome known as Long Face Syndrome.

Is it a problem? The answer is maybe.

We know that it can be challenging to find information about Long Face Syndrome and we would like to help provide educational material.

This blog post was written by an ear, nose and throat surgeon.

Long face syndrome. The common facial features and symptoms are shown. CamachoMD.com
Long face syndrome. The common facial features and symptoms are shown.

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Summary for Long face syndrome:

Long face syndrome, is a syndrome or disorder in which the face grows in an excess amount in the vertical dimension; this gives a “long” appearance to the face. This can be caused by chronic mouth breathing, especially in children who have been mouth breathing for years. Surgery should be a last resort in most cases. If the surgeon and the patient or the patient’s parents or guardians agree that surgery is in the best interest of a child, then typically the surgery will function to improve nasal breathing. In some cases, skeletal surgery can be performed to physically reduce the amount of bone in the face (upper and lower jaw surgery), then the face should look more normal as the surgeons should remove the extra height of the face to have more normal proportions.

What is long face syndrome?

Long face syndrome, is a syndrome or disorder in which the face grows in an excess amount in the vertical dimension; this gives a “long” appearance to the face.

This can be caused by chronic mouth breathing, especially in children who have been mouth breathing for years.

In 1976, Dr. Schendel and colleagues wrote a manuscript on the topic of long face syndrome in which they summarized the findings from 31 adults with the syndrome.[1]

Overall, Dr. Schendel reported that there was an excessive vertical growth of the maxilla, with two subtypes:

  • Dental open bite, and
  • Dental closed bite.[1]

The authors reported that the closed bite group had an increased in the lower jaw height (increased mandibular ramus height).[1]

What causes long face syndrome?

Long face syndrome causes can be controversial, however, a commonly accepted cause is chronic nasal obstruction. [2,3]

When humans switch from breathing through their nose to breathing through their nose and mouth, there is an increase in the length of the face in the vertical dimension.[3]

In some people, long face syndrome can be genetic and it has been proposed to occur even without nasal obstruction.

Long face syndrome in a child. Note the mouth is open so he can breathe. The airway is shown. The blue arrows show the path that the air takes in the upper airway. CamachoMD.com
Long face syndrome in a child. Note the mouth is open so he can breathe. The airway is shown. The blue arrows show the path that the air takes in the upper airway.

Who is more predisposed to developing long face syndrome (children or adults) who become new mouth breathers?

A child is more predisposed to developing long face syndrome since their face is still growing.

On the other hand, an adult who develops mouth breathing has already finalized their facial growth (craniofacial skeleton), therefore, the effect of mouth breathing on the shape of the face should be minimal.

How common is long face syndrome?

Long face syndrome is estimated to affect 22% of patients who are referred for surgical-orthodontic treatments.[4]

This estimate of 22% does not reflect the general population, therefore, likely the true estimate of long face syndrome is significantly less.

When should someone seek care for long face syndrome?

The best time to get treatment for long face syndrome is when the symptoms first start.

For example, if a child has nasal obstruction and is mouth breathing, then they should seek treatment.

The first treatment for mouth breathing is to manage medically with antihistamines (nasal or systemic), nasal sinus rinses, nasal steroids, and anti-leukotrienes.

Why do people get long face syndrome?

Nasal obstruction and congestion with the associated mouth breathing.

As the face is growing, it is known that the muscles of the face can alter the development and skeletal structures of the face, thus making it longer.[3]

The maxilla is known to grow and develop in a manner that is influenced by the environment.[5]

These changes will be more dramatic in a child since the face is growing. If an adult develops nasal obstruction such as from trauma or the development of allergic rhinitis, then there may not be any notable change to the face structure.

How is long face syndrome diagnosed?

The presence of a long face can be determined in two ways.

The first way is by looking at the person’s face and the second is by using measurements from an x-ray of the face (lateral cephalogram).

A study by Calvo-Henriquez and colleagues found that ear, nose and throat surgeons, as well as pediatricians, have low sensitivity and high specificity for identifying the early signs of vertical facial growth when compared to objective measurements with x-rays (cephalometric analysis).[2]

Is long face syndrome due to the upper jaw, the lower jaw or both?

Classically, long face syndrome is due to upper jaw (maxillary) elongation in the up and down (vertical) dimension.[6]

However, Dr. Schendel and colleagues have identified six variations of vertical maxillary excess.[6]

Overall, the possibilities for long face syndrome include:

  • Long vertical upper jaw (maxilla) with normal lower jaw (mandible or chin),
  • Normal upper jaw with a long lower jaw,
  • Long upper jaw and a long lower jaw, and
  • Long upper jaw with a long lower jaw.
Long face syndrome child side view (profile view). Note that the patient has his mouth open. CamachoMD.com
Long face syndrome child side view (profile view). Note that the patient has his mouth open.

Why do patients with long face syndrome have allergic shiners?

Allergic shiners are when there is a darkening (blue/gray/purple coloration) below the eyes in people.

There are multiple causes, including nasal congestion in which there is an accumulation of blood and other fluid below the eye.[7]

 It has been hypothesized that darkening below the eyes is due to the veins not draining properly (venous stasis).[7]

Why do patients with long face syndrome have allergic salute?

Patients with allergic rhinitis can have itchiness and since patients cannot reach the area to scratch the itch, they instead will push the tip of their nose up to help relieve the itchiness.

When patients push their nose upwards on a chronic basis, then a crease can form over the nose (just above where the nostrils meet the nose) and this crease is known as the allergic salute.

Why do patients with long face syndrome have an open mouth?

Patients with long face syndrome typically have nasal obstruction and congestion.

This obstruction and congestion cause patients to breathe through their mouths and they will spend a significant amount of time with their mouths open.

Sometimes patients will still breathe with their mouths open even after the nasal obstruction (adenoidectomy, turbinoplasty, etc.) is relieved and they do this more out of habit than because they have nasal congestion.

Why do patients with long face syndrome have a narrow face?

Long face syndrome. Front view. Mouth is open. Allergic shiners are seen. CamachoMD.com
Long face syndrome. Front view. Mouth is open. Allergic shiners are seen.

The mouth breathing and chronically open mouth mean that the lower jaw is pulling down on the upper jaw.

When the lower jaw pulls down on the upper jaw, this narrows the face because the trajectory of the pull is in the up and down (vertical) dimension of the face.

Over time, the soft tissue pulling of the upper jaw can cause the upper jaw to stretch instead of expanding in the normal up and down as well as sideways growth.

Why do patients with long face syndrome have their gums show?

When the patients breathe through their mouths, the soft tissue pulls the upper jaw down and this can lead to the gums of the upper jaw showing.

Why do patients with long face syndrome have a setback chin?

Some patients with long face syndrome also have a setback chin.

The primary feature of a patient with long face syndrome is that the maxilla is elongated, but in some cases, the mandible is set back.

Why do patients with long face syndrome have crowded teeth?

The palate is the floor of the nose or the roof of the mouth, and obstructed breathing can affect the shape.

Additionally, when a patient mouth breathes, the tongue no longer sits up against the palate and this can affect the shape.

Dental crowding with narrow upper jaw and narrow lower jaw with blue pointer showing crooked and overlapping teeth. The blue pointer demonstrates the dental crowding (crooked teeth). CamachoMD.com
Dental crowding with narrow upper jaw and narrow lower jaw with blue pointer showing crooked and overlapping teeth. The blue pointer demonstrates the dental crowding (crooked teeth).

Why do patients with long face syndrome snore?

Patients with long face syndrome often have nasal congestion if they have not been treated.

Because of the nasal congestion, the patients will often open their mouths during sleep and the soft tissues of the throat can vibrate and cause snoring.

Why do patients with long face syndrome feel sleepy or tired?

If a patient snores and/or has obstructive sleep apnea, then the obstructions of airflow could cause a decrease in oxygenation during sleep and/or frequent arousals or wake-ups after falling asleep and this can make the sleep be unrestful or unrefreshing.

What surgeries can be done for long face syndrome?

Surgery should be the last resort in most cases.

If the surgeon and the patient or the patient’s parents or guardians agree that surgery is in the best interest of a child, then typically the surgery will function to improve nasal breathing.

Adenoidectomy is a common surgery that is used to help improve nasal breathing.

Enlarge adenoids (the adenoid tissue in the back of the throat is shown in pink). CamachoMD.com
Enlarge adenoids (the adenoid tissue in the back of the throat is shown in pink).

If the child also has large inferior turbinates, then the surgeon might recommend an inferior turbinoplasty.

If the child is a teenager or an adult, then other areas that might be evaluated and treated include the upper and lower jaw themselves.

When a patient has jaw surgery it will likely involve cutting of the upper jaw (Leforte Osteotomy) in a way that a wedge is removed which allows the jaw to be shorter in the vertical dimension.

When the upper jaw is cut, then the lower jaw typically needs to be cut as well to allow for a proper bite (dental occlusion).

If the chin is also long in the front, which happens in some patients, then a cut can also be made in the chin to reduce the size.

Maxillomandibular advancement showing the split after the maxilla (upper jaw) and mandible (lower jaw) have been cut and moved forward. The plates and screws are shown in blue. CamachoMD.com
Maxillomandibular advancement showing the split after the maxilla (upper jaw) and mandible (lower jaw) have been cut and moved forward. The plates and screws are shown with blue color.

Overall, the goal is to improve nasal breathing so that the child or adult can breathe through their nose after surgery and this will significantly reduce or eliminate mouth breathing.

Do patients have normal facial proportions after surgery for long face syndrome?

In short, it depends on when the surgery is performed and what type of surgery is performed.

Three scenarios help further explain.

First scenario: a child has chronic mouth breathing and the child’s face is mild to moderately long and they are prepubescent (face is still growing).

The child has an adenoidectomy (with or without tonsillectomy), and after the surgery, the patient starts breathing through their nose and they no longer mouth breathe.

As the child grows, their face may develop into more normal proportions.

Second scenario: a child has chronic mouth breathing with a severely long face and is near puberty.

At this point, if a child has an adenoidectomy or other nasal surgery and breathes well through their nose after surgery, their face is almost done growing, so they would not look much different after surgery.

Third scenario: a teenager or an adult has long face syndrome and has skeletal surgery to physically reduce the amount of bone in the face (upper and lower jaw surgery), then the face should look more normal as the surgeons should remove the extra height of the face to have more normal proportions.

A research article summarizing the literature (a meta-analysis) found that with regard to total anterior facial height, that when patients have surgery to relieve nasal obstruction and they no longer mouth breathe, then there is a favorable effect because the surgery helps to prevent further lengthening of the face.[8]

With surgery of the upper jaw, the three advantages include:

Upward and forward anti-rotation of the lower jaw close open bite deformities, and

Achievement of a balanced and esthetic face (nasolabial angle, para nasal areas, and alar base width).[9]

Patients who have surgery also typically will undergo orthodontic treatment afterward (surgical-orthodontic correction[10]) to help improve both the function of the teeth (proper bite) and cosmetics of the teeth positioning.

References:

1. Schendel SA, Eisenfeld J, Bell WH, Epker BN, Mishelevich DJ (1976) The long face syndrome: vertical maxillary excess. American journal of orthodontics 70 (4):398-408

2. Calvo-Henriquez C, Martins-Neves S, Faraldo-Garcia A, Ruano-Ravina A, Rocha S, Mayo-Yanez M, Martinez-Capoccioni G (2019) Are pediatricians and otolaryngologists well prepared to identify early signs of vertical facial growth? International journal of pediatric otorhinolaryngology 119:161-165. doi:10.1016/j.ijporl.2019.01.035

3. Tourne LP (1990) The long face syndrome and impairment of the nasopharyngeal airway. The Angle orthodontist 60 (3):167-176. doi:10.1043/0003-3219(1990)060<0167:Tlfsai>2.0.Co;2

4. Bailey LJ, Haltiwanger LH, Blakey GH, Proffit WR (2001) Who seeks surgical-orthodontic treatment: a current review. The International journal of adult orthodontics and orthognathic surgery 16 (4):280-292

5. Solow B, Kreiborg S (1977) Soft-tissue stretching: a possible control factor in craniofacial morphogenesis. Scandinavian journal of dental research 85 (6):505-507

6. Schendel SA, Carlotti AE, Jr. (1985) Variations of total vertical maxillary excess. Journal of oral and maxillofacial surgery : official journal of the American Association of Oral and Maxillofacial Surgeons 43 (8):590-596

7. Chen CH, Lin YT, Wen CY, Wang LC, Lin KH, Chiu SH, Yang YH, Lee JH, Chiang BL (2009) Quantitative assessment of allergic shiners in children with allergic rhinitis. The Journal of allergy and clinical immunology 123 (3):665-671, 671.e661-666. doi:10.1016/j.jaci.2008.12.1108

8. do Nascimento RR, Masterson D, Trindade Mattos C, de Vasconcellos Vilella O (2018) Facial growth direction after surgical intervention to relieve mouth breathing: a systematic review and meta-analysis. Journal of orofacial orthopedics = Fortschritte der Kieferorthopadie : Organ/official journal Deutsche Gesellschaft fur Kieferorthopadie 79 (6):412-426. doi:10.1007/s00056-018-0155-z

9. Casteigt J, Dereudre B (1984) [Surgical approach of a vertical problem: the long face syndrome]. Revue de stomatologie et de chirurgie maxillo-faciale 85 (2):83-93

10. Capelozza Filho L, Cardoso Mde A, Reis SA, Mazzottini R (2006) Surgical-orthodontic correction of long-face syndrome. Journal of clinical orthodontics : JCO 40 (5):323-332; quiz 308