What is Binder Syndrome?

Binder Syndrome, also known as Maxillonasal Dysplasia, Maxillary Hypoplasia or Nasomaxillary Hypoplasia, is a rare congenital condition which affects the development of the nose and mid-face, resulting in a flattened appearance.

As it is present from birth, Binder Syndrome is caused by the underdevelopment of the facial bones and cartilage during pregnancy. This can result in breathing difficulties and may also affect eating, with a significant impact on comfort, health, and quality of life. Additionally, the distinctive appearance associated with Binder Syndrome may lead to self-consciousness.

The treatment and management of Binder Syndrome depend on the severity of the condition and its effects on an individual’s life. Babies who are born with Binder Syndrome will be monitored from birth to ensure that the most effective treatment plan is put in place at an early stage; however, as they grow, their concerns may change.

Cadogan Clinic’s team of expert Consultant Facial and ENT Surgeons has extensive experience in reconstructive surgical procedures to address many of the common concerns caused by Binder Syndrome.

Dysplasia vs Hypoplasia

Binder Syndrome is referred to in a number of different ways, and the medical wording may be confusing. The various terms all refer to the same lack of growth in the facial bones and tissues, but they each have slight differences in the anatomical and physiological meanings:

• Dysplasia refers to the abnormal development and organisation of cells, resulting in atypical size, shape or structure of tissues or organs
• Hypoplasia is the underdevelopment of organs or tissues caused by the full number of cells required not being present

Both Dysplasia and Hypoplasia may contribute to the causes of Binder Syndrome, depending on the exact nature of the condition.

Other medical terms which are used alongside hypoplasia and dysplasia refer to the affected parts of the face:

• ‘Naso’ or ‘Nasal’ refer to the nose
• ‘Maxill’ or ‘Maxillary’ both refer to the jaw and jawbone

These phrases may be combined in cases such as Maxillonasal and Nasomaxillary.

Understanding the medical terminology used in the various names of Binder Syndrome is important for gaining greater insight into how it is discussed.

What Are the Symptoms of Binder Syndrome?

Binder Syndrome includes external and internal symptoms. External Binder Syndrome symptoms are the most common and all affect the mid-section of the face, particularly the nose, mouth, and jawline.

The most common Binder Syndrome signs and symptoms include:

• Flat nose and upper lip
• Under projected nasal tip
• Protruding lower jaw
• Misaligned upper and lower teeth
• Crescent or triangular-shaped nostrils

The flattened nasal bridge and misaligned nasal structures that are typical of Binder Syndrome symptoms often result in a range of functional respiratory concerns regardless of the severity of the condition.

• Breathing difficulties
• Sleep disorders, such as snoring and sleep apnoea
• Chronic nasal congestion
• Recurrent nasal infections, such as sinusitis
• Headaches

Even in cases of mild Binder Syndrome, these functional concerns can affect wider health and quality of life, impacting participation in exercise and causing self-consciousness in social and professional environments.

Less common Binder Syndrome symptoms include:

• Cleft lip and palate
• Congenital heart defects
• Hearing problems
• Spinal abnormalities
• Crossed eyes (Strabismus)
• Intellectual disability

Although some Binder Syndrome signs and symptoms, such as a flattened nose, may be picked up immediately or shortly after birth, others may not be apparent until later in childhood. Close monitoring is essential to detect and manage concerns quickly and effectively.

What Does Binder Syndrome Look Like?

The visible symptoms of Binder Syndrome affect the shape and projection of the nose, cheeks and upper jaw. The predominant features are a flattened nasal bridge and an under projected nasal tip. This affects the overall balance of facial proportions.

What Causes Binder Syndrome?

Due to the rare nature of Binder Syndrome, its causes are not fully understood, and in most cases, it is not possible to establish an exact cause.

Some possible causes of Binder Syndrome that have been studied include:

• Genetics – In some cases, families have more than one child with Binder Syndrome, suggesting that genetics may play a part. However, the specific gene patterns involved have not been identified
• Lifestyle factors – Smoking and drinking alcohol during pregnancy may increase the risk of Binder Syndrome occurring
• Medications – Exposure to certain medications in utero, such as Phenytoin (an Epilepsy treatment) and Warfarin (a blood thinner), may have an effect
• Vitamin K deficiency – Vitamin K contributes to bone health, and deficiency (during pregnancy) may impact the development of bones and cartilage
• Injury during birth – Trauma or injury to the baby during birth may cause changes in the bone structures

Although an exact Binder Syndrome cause may not be confirmed, potential explanations may be explored to inform its treatment and management.

Binder Syndrome Diagnosis

Diagnosing Binder Syndrome as early as possible is crucial to effectively managing functional concerns, which may worsen or be more challenging to treat later in life.

Doctors may be able to initially diagnose Binder Syndrome based on a baby’s facial features, such as the presence of a flattened nose. However, this will usually be followed by a series of scans and tests to provide a more detailed view of the baby’s bone structure to confirm the diagnosis and determine the extent of the procedure.

Binder Syndrome ultrasound, CT, and MRI scans are typically part of the diagnostic process, but hearing, visual, and psychiatric testing may also be required at appropriate life stages to assess or rule out less common symptoms.

How to Treat Binder Syndrome

Binder Syndrome treatment for any facial concerns typically begins during adolescence. The severity of the condition will determine the available treatment plan and the time frame for completing it.

While the NHS do provide much initial Binder Syndrome treatment and reconstructive Binder Syndrome surgery, there are some limitations which may affect patients:

• Patients experiencing mild Binder Syndrome may find that their waiting times are longer, and that breathing difficulties and self-consciousness become overwhelming.
• Patients who have undergone Binder Syndrome surgery during adolescence may find that their results require refinement in adulthood due to changes in bone and muscle structures

Private healthcare provides a route which offers both timely treatment and the enhancement of prior outcomes.

Binder Syndrome Surgery

Several options are available for Binder Syndrome surgery. Generally, these procedures address the structure and function of the nose and facial tissues. Various types of Rhinoplasty surgery specifically target the lower nasal bridge, the weakened or misaligned septum and the flat nose appearance.

Initial reconstructive Rhinoplasty surgery is usually performed between the ages of 15 to 19, when the nose is fully developed.

Surgery to address Binder Syndrome includes:

Rhinoplasty

Rhinoplasty surgery involves repositioning and aligning the external tissues that make up the nose to alter its shape, size and projection.

As a Binder Syndrome treatment, Rhinoplasty is typically most suited to patients:

• With mild Binder Syndrome, who aren’t affected by functional concerns
• Who have had previous reconstructive surgery and wish to achieve aesthetic refinements to the shape and projection of their nose

Rhinoplasty is performed under general anaesthetic by a qualified Consultant ENT or Facial Plastic Surgeon and usually takes around two hours to complete. It will achieve a more refined nasal shape and position that are proportional to the rest of the face.

Septorhinoplasty

Septorhinoplasty is a comprehensive Rhinoplasty procedure that addresses the internal and external nasal structures, effectively treating functional and aesthetic concerns in a single procedure.

When performed as a Binder Syndrome treatment, Septorhinoplasty often involves the use of synthetic or cartilage grafts to reshape the nose and support the airways.

Septorhinoplasty is usually suited to patients:

• Who have not had previous Binder Syndrome surgery, and are looking for a comprehensive procedure to address aesthetic and functional concerns
• Who have experienced changes in their nasal structures since an initial reconstructive procedure, and wish to restore or improve previous surgical outcomes

Similar to Rhinoplasty, Septorhinoplasty is performed under general anaesthetic and takes up to three hours to complete. However, Septorhinoplasty is usually performed by a specialist Consultant ENT Surgeon with expert knowledge of the nose's function and anatomy.

Septorhinoplasty results in a balanced, proportionate nose and notable improvements in breathing, sleep disorders, and other associated functional concerns.

Septoplasty

Septoplasty is a purely functional Rhinoplasty procedure that addresses concerns affecting the septum, the cartilage in the middle of the nose that separates the two airways.

Septoplasty is a very simple procedure and suited to patients:

• With mild Binder Syndrome who wish to address functional breathing concerns without dramatically altering the appearance of their nose
• Who have previously had Binder Syndrome surgery, but specifically want to revise concerns affecting their septum, which may be affecting breathing
• Who want to effectively improve functional concerns, with a shorter downtime than other Rhinoplasty procedures

When performed as a standalone surgery, Septoplasty usually takes up to two hours to complete, and is performed under general anaesthetic by a Consultant ENT Surgeon.

Septoplasty surgery results in restored breathing, reduced symptoms of sleep disorders, and improved quality of life.

Fat Transfer to Face

For patients who experience more widespread symptoms of facial underdevelopment in the mid-face, mouth, and jaw, Fat Transfer techniques offer an effective option for building volume and definition.

Facial Fat Transfer can either be performed for patients who have had no prior Binder Syndrome surgery or for patients who wish to enhance previous surgical outcomes.

Excess fat is extracted from areas such as the abdomen, waist and thighs using Liposuction. It is then processed to remove impurities before being injected into the face.

Areas of the face which can benefit from Facial Fat Transfer when treating Binder Syndrome include:

• Cheeks
• Upper lip
• Jaw
• Philtrum (between the nose and upper lip)

The results of Fat Transfer to Face are permanent, and the regenerative properties of fat can also be harnessed to improve skin texture and tone, offering further facial rejuvenation.

Non-Surgical Binder Syndrome Treatment

In some cases, non-surgical treatments may provide an effective solution for patients with Binder Syndrome.

Non-surgical Binder Syndrome treatment options address aesthetic concerns but not functional ones, and therefore aren’t suitable for all patients.

Non-Surgical Rhinoplasty

Also known as a Liquid Rhinoplasty, Non-Surgical Rhinoplasty alters the shape of the nose for a more balanced appearance.

Dermal Fillers are injected into the skin of the nose to add volume and definition to carefully selected areas, changing the shape of the nose. When performed by surgeon with extensive experience of Non-Surgical Rhinoplasty, clever optical illusions can be achieved to prevent the nose from looking larger, although volume has been added.

Non-Surgical Rhinoplasty is suited to patients:

• With a flat nose and lower nasal bridge, which they wish to improve the appearance of, but who don’t have functional concerns which require treatment
• Who have had previous Binder Syndrome treatment and who want to refine their aesthetic results without further surgical procedures
• Who want to be able to understand the results they may be able to achieve from surgical results through a less involved procedure before they make a full commitment

Although Non-Surgical Rhinoplasty can’t address functional concerns, it remains a highly valuable treatment option for Binder Syndrome.

Dermal Fillers

Similar to Facial Fat Transfer, Dermal Fillers provide an option for increasing volume and definition in underdeveloped areas of the mid-face outside of the nose.

Dermal Fillers can be injected into most areas of the face and can address Binder Syndrome concerns in areas including:

• Cheeks
• Upper lip
• Jawline
• Philtrum (between the nose and upper lip

For patients seeking increased volume and definition in various areas of the face, Dermal Fillers are a versatile and effective treatment option. Patients should bear in mind that they aren’t a permanent solution, unlike Fat Transfer, and repeat treatments are required, usually every 6-12 months, depending on the area and the type of filler used.

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