Commonly associated with cleft lip or cleft palate, a facial cleft is a rare disorder where bone, skin, and soft tissue are absent or malformed in an area of the face. This condition can affect one or both sides of the face, and there is a wide range of cleft types. Furthermore, clefts can occur as an effect of another condition or on their own. The disorder can be mild to severe, and treatment will be based on the kind of cleft, degree of significance, and individual needs of the patient. Our experienced craniofacial plastic surgeon, Dr. Eric Payne, and our skilled multispecialty team utilize the most advanced techniques and technology available to treat facial clefts and to help patients achieve a more natural appearance and function.
When a child is born with a facial cleft, it can be a very emotional experience for parents. Our dedicated team of medical professionals offers support, information, and compassion during this time. If you have additional questions or wish to schedule a consultation, please contact us today.
What are facial clefts?
A facial cleft occurs during fetal development and is a space left in the soft tissue, bone, or both. This condition can affect the forehead, eyes, ears, cheeks, nose, mouth, or multiple areas of the face. The region of involvement typically can range as high as the hairline and as low as the jaw. Some clefts of the face are horizontal involving the corners of the mouth. This is known as macrostomia or enlarged mouth. If the cleft causes malformation of a feature, it is called dysplasia. If the bone and skin are split, this is known as a fissure. The Tessier classification system describes in detail the variety of facial clefts. In this system, bony and soft tissue clefts can be precisely characterized using a series of numbers, #0-14 and #30, that correspond to areas of the face, primarily the mouth, eye sockets, and nose. Other classification systems exist; however, Dr. Payne uses the Tessier system as it is the most widely recognized classification.
What are the different types of facial clefts?
In the Tessier classification, facial clefts are numbered 0-14 and can be more broadly categorized as midline, paramedian, orbital, and lateral clefts. These descriptions illustrate where on the face the clefts are positioned. Soft tissue clefts can be located in a slightly different location than clefts in the bone, which the Tessier system indicates. Tessier #7 cleft is the most common type.
Facial clefts that fall along the midline, or vertically up and down the center face, are called midline clefts. These clefts are designated Tessier number 0, which goes through the maxilla (upper jaw) and the nose, and Tessier number 14, which goes through the nose and the frontal bone (upper orbits and forehead). As you move away from the midline on the upper face (orbits and frontal bone), the numbers decrease, as you move away from the midline on the mid and lower face (nose and maxilla), the numbers increase. Frontonasal Dysplasia is a type of midline facial cleft. Some cases involve the brain and is described as Holoprosencephaly. A Tessier #30 cleft is vertical through the lower midline lip and/or mandible which is extremely rare.
Facial clefts that are located just off of the midline are called paramedian clefts, and they include Tessier number 1, 2, 12, and 13. Tessier number 1 and 2 affect the maxilla and the nose, with number 2 located further off from the midline than number 1. Tessier number 12 and 13 are located along the same line as number 1 and 2, respectively, but higher on the face, near the orbits. Both types of facial clefts are situated between the nose and frontal bone, with number 13 running closer to the midline than number 12.
Orbital clefts, as the name implies, affect the eye sockets. Facial clefts designated Tessier number 3, 4, 5, 9, 10, and 11 fall into this category. Tessier number 3, 4, and 5 involve the maxilla and the lower orbits, and number 9, 10, and 11 affect the upper orbits and either the forehead or the temple. Tessier number 11 is on the same line as number 3, number 10 is on the same line as number 4, and number 9 is on the same line as number 5.
Lateral indicates a horizontal rather than vertical direction. Tessier number 6, 7, and 8 are lateral clefts. Number 6 runs from the lower orbit to the upper cheekbone. Number 7 is located on the line from the ear to the corner of the mouth, and a cleft can begin at either end. Macrostomia, a condition wherein the mouth appears abnormally wide, is associated with a Tessier number 7 cleft, as is Treacher Collins syndrome. Number 8 goes from the outer corner of the eye and moves toward the ear. One of the signs of Goldenhar syndrome is a Tessier number 8 cleft.
How do facial clefts occur?
Just as there are a wide range of facial cleft types, so are there many reasons for why they can develop. In many instances the condition occurs as a sporadic, or random, event. This means that the malformation was not inherited nor was it the result of something the parents did or did not do during pregnancy. It is simply an unfortunate circumstance of unknown origin. In other cases, facial clefts can be caused by one or more genes that have mutated or been damaged, as a result of environmental factors, or both (multifactorial inheritance). Additionally, some facial clefts may be a sign of another condition, such as Treacher Collins syndrome, Binder syndrome, or Saethre-Chotzen syndrome. Dr. Payne will discuss the possible cause of your child’s facial cleft during the initial consultation. If genetics and/or environment are known or suspected to play a role, you may wish to speak with or be referred to a geneticist about testing.
Can facial clefts be passed on through the family?
Whether your child can pass on his or her facial cleft will depend on the cause of the disorder. If the cleft occurred as a result of a syndrome known or suspected of having a genetic link, then it is possible that your child could pass the disorder on when they have children or if you have more children. If there is a family history of facial clefts, this may also suggest that there is a hereditary element to the condition. Our skilled medical team will do their best to provide as much information as is known about your child’s condition and help guide you to additional resources as needed.
What signs and symptoms do facial clefts cause?
Depending on the type, severity, and location of the facial cleft, there can be a wide range of side effects. Significant clefts positioned higher on the face, including on or near the orbits (eye sockets) or forehead, can cause intellectual disabilities. More serious problems associated with clefts of the nose or mouth include difficulty eating and/or breathing. Each patient’s experience will be unique, and our physician will take the time to discuss your child’s cleft challenges with you during your consultations. It is also important to remember that there can be psychological effects as well as the physical concerns. Our team will work closely to make sure all issues are addressed. Broadly stated, facial clefts can cause the following signs and symptoms:
- Facial paralysis
- Facial asymmetry
- Flattened forehead
- Low and/or V-shaped hairline
- Eyelid defects (eyelid colombas)
- Vertical orbital dystopia (one eye is located higher than another)
- Ocular abnormalities
- Vision Impairment
- Hypertelorism (wide-set eyes)
- Nasal abnormalities
- Flattened or recessed cheeks
- Breathing problems
- Feeding problems
- Palate abnormalities
- Poor or delayed speech
- Reduced jaw size and projection
- TMD (temporomandibular joint disorder)
- Hearing impairment or loss
- Intellectual disability (rare)
- Central Nervous System abnormalities
What are the treatment options?
Treatment for facial clefts will vary dramatically from patient to patient depending on each child’s unique expression of the disorder. It’s important to plan the surgical approach early on, as many facial clefts will require multiple operations at different stages of development. Dr. Payne will go over each stage of reconstruction to help you become well-informed and understand the purpose of each procedure. We work with multiple specialist to achieve the best possible outcome. Your child’s care is our highest priority, and we seek to provide a compassionate and supportive environment throughout the experience.
In general, clefts will require facial reconstruction, typically of the bone, soft tissue, and skin. The first step will be to identify and correct any urgent problems that could affect breathing, eating, vision, and normal brain development. Typically a z-plasty type tissue rearrangement will be used, which is a technique that uses z-shaped incisions to help rearrange the facial structures such as the nose, eyelids, and mouth. Once a child is deemed old enough, bone grafts can be use to address foundational issues. Nasal clefts will require nasal reconstruction using cartilage (often from the rib, ear or donor cartilage) and skin rearrangement. A number of other techniques and procedures may be recommended based on the unique parameters of your child’s condition including free tissue transfer, fat grafting, and/or tissue expanders. Our surgeon’s goal is to achieve the best cosmetic and functional outcome possible, and many facial clefts can be corrected with exceptional results.
What is the recovery process like?
The recovery process will be different for every patient, as each child’s treatment plan and healing rate will vary. Furthermore, treatment plans often involve several stages of operations, and each surgery will have its own timeframe for downtime. Dr. Payne can provide more specific information pertaining to your child’s case during your consultations. Young children that undergo surgery typically may have to remain in the PICU (Pediatric Intensive Care Unit) for at least one to two days, followed by a short stay in the hospital before returning home. Rhinoplasty (nasal reconstruction) that requires bone grafting will also require an overnight stay in the hospital.
Recovery is a very important part of achieving the discussed outcome. Therefore, your child’s activities will be limited while they heal, and it’s important for you to help them manage this experience. Both children and adults can find this process stressful and emotional, but the final result can be very rewarding. Our skilled medical team is committed to helping you navigate this process from start to finish, and we are here to provide support and information as you need it.
If you have additional questions about facial clefts, or if you would like to schedule a consultation with Dr. Payne, please contact our office today.