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Clefts can occur in about one out of 600 newborn babies. Each cleft is unique in its appearance and severity. The cleft can affect the lip alone, palate alone or both the lip and palate. The cleft can occur on one side (unilateral) or both sides (bilateral) of the lip and/or palate. A cleft in the palate can affect the soft palate alone, hard palate alone or both the soft and hard palates. A cleft can affect the baby’s feeding, teeth, hearing, speech and nose.

What causes a cleft lip?

In most cases we are not sure why a cleft lip and/or palate (roof of the mouth) occurs. The condition is characterized by the sides of the lip and/or palate not coming together during the formation of the baby’s head and neck early in pregnancy. This incomplete development usually leaves an opening in these areas. Some clefts are related to certain medications the fetus was exposed to during pregnancy. Other times, cleft formation can be due to genetic reasons. 

Dr. Aboutanos – Pediatric Plastic Surgery

Pediatric Plastic Surgery - Dr. Aboutanos

Treatment of a Cleft Lip

An important part of your child’s treatment is locating a Cleft and Craniofacial Team. Several specialists are part of this team to help coordinate and treat your child over several years through your child’s development. The Cleft and Craniofacial Team at St. Mary’s Hospital is qualified and ready to take care of your child.

A cleft lip is usually repaired in the first months of your child’s life. A cleft palate is usually repaired in the first year of life.

The timing by which our Richmond pediatric plastic surgery is undergone may depend on your child’s conditions as well as the type of surgery your plastic surgeon will perform. Other important specialists your child may need to see are feeding and speech therapists, pediatric dentists, orthodontists and prosthodontists, oral surgeons, otolaryngologists (ENT) and audiologists, and psychologists. To learn more about the treatment of cleft lip and/or palate in Richmond, VA, please call 804.585.3420 to meet with one of our pediatric craniofacial surgeons.

Bon Secours’ blog on the Cleft and Craniofacial Team at St. Mary’s

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Velopharyngeal Insufficiency

Velopharyngeal insufficiency (VPI) occurs when air and sound escape from the child’s nose during speech. This produces speech that sounds nasal in quality. VPI may lead to hoarseness or fatigue of your child’s voice.

Causes:

Normal speech is produced when the soft palate closes off the nasal cavity, allowing the air to escape through the mouth. Velopharyngeal insufficiency is caused when the soft palate cannot block off the nasal cavity, letting the air go out through the nose.

Treatment:

The first step in diagnosing and treating velopharyngeal insufficiency is making sure that your child has normal hearing and no signs of an ear infection. The next step is taking your child to a speech therapist where he/she can listen to your child’s voice. Sometimes, speech therapy alone can improve your child’s speech disorder. In cases of severe speech disorders, surgery may be required. The timing and type of surgery depends on your child’s condition and on the plastic surgeon. Further speech therapy may be required after surgery. Our comprehensive team can offer an ENT surgeon, speech therapist and plastic surgeon who specialize in diagnosing and treating your child’s VPI.

To learn more about the treatment of velopharyngeal insufficiency in Richmond, VA, please call to meet with one of our craniofacial surgeons.

Pierre Robin Sequence

Pierre Robin sequence (PRS) is a disorder in which a baby is born with a small lower jaw (mandible), causing the tongue to fall back into the mouth and blocking the airway, which may lead to difficulty breathing. This condition will also cause the baby to have a cleft palate (open roof of mouth). 

The child will present with a very small or receding chin and the tongue will appear large. The cleft in the palate will have a U-shape, which is more typical of clefts formed due to Pierre Robin sequence.

Causes: Pierre Robin sequence happens during a series of events during the child’s development as an embryo (that is why it is called a sequence). The cause of these events is unknown.

PRS Treatment Options

The most important goal in treatment of Pierre Robin sequence is making sure the child is able to breathe adequately. This can be achieved by position changes, placement of nasal trumpets (tubes that keep the airway open), tongue-lip adhesion surgery, mandibular distraction or tracheostomy. It will also be important to make sure the child is able to feed without difficulty.

Our speech pathologist and feeding specialist will help your child every step of the way during this process.

The cleft palate is usually surgically repaired around 12 months of age. The timing of this surgery will depend on the child’s breathing and your surgeon. Your child may also need ear tubes placed, which will be determined by your child’s ENT surgeon. Your child will be followed by all the members of our dental team over the course of your child’s life.

To learn more about the treatment of Pierre Robin sequence, please call to meet with one of our craniofacial surgeons.

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Positional Plagiocephaly

Positional plagiocephaly refers to an abnormal head shape that can be a result of positional molding. It is important to distinguish positional plagiocephaly from a more serious condition called craniosynostosis.

Causes: Positional molding can occur as a result of your child sleeping on the back of his/her head, the baby’s prolonged position in the womb or from torticollis. The latter has been much more common as sleeping on the back, and not the belly, is the recommended position by the American Academy of Pediatrics. The change in head shape occurs because there is constant pressure to one side of the head causing it to be flat.

Children who are premature or the result of multiple birth pregnancy may have a higher incidence of plagiocephaly.

Treatment: Positional molding, if detected early enough, may improve on its own within the first six months of life with positional changes alone. If your child has torticollis, neck-stretching exercises will be provided in addition to the positional changes. It has been our experience that if the deformity is moderate or severe after six months of age, then helmet therapy might be indicated. Helmet therapy does not usually work after the child is 14-18 months of age. If helmet therapy is delayed after this age, extensive cranial surgery is the only way to fix an abnormal head shape.

If helmet therapy is prescribed, your child would need to wear the helmet 23 hours/day, everyday. It may be removed for short periods for airing or bathing. The average treatment time is three-four months. Most children are able to tolerate helmet therapy within the first days of use.

To learn more about positional plagiocephaly treatment in Richmond, VA, please call to meet with one of our craniofacial surgeons.

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Craniosynostosis

Craniosynostosis is a condition caused by early closure of one or more of the normally present bony gaps between the bones of the skull. These bony gaps are called cranial sutures. The cranial sutures allow the brain to grow and expand during the first year of life. Normally, these sutures close at different times in a patient’s life as brain growth stops. If the suture closes too early, the brain may not have adequate room to grow. Approximately one infant in every 1000 births will have early closure of a cranial suture.

Craniosynostosis does not improve with time and it can worsen with growth. This may result in a ridge in the area of the closed suture and a change in the shape of the skull. Some of the sutures that are affected are: metopic, coronal, sagittal and lambdoid. If more than one cranial suture is fused or closed, pressure inside the brain can become higher than normal. The diagnosis can be confirmed by simple examination of the child and in more complicated cases, XRAYS and/or a CT scan of the head.

Causes: In most cases, the reason for craniosynostosis is not clear and it is sporadic. However, some cases are related to certain medications the fetus may have been exposed to during pregnancy. Other times, craniosynostosis formation can be due to genetic reasons. If craniosynostosis is associated with other findings in the child, it may be part of a syndrome. In these cases, a geneticist may need to evaluate your child.

Treatment: Craniosynostosis is treated by performing surgery to the skull and forehead. The first goal is to open the closed sutures to prevent problems with high pressure in the brain and allow the brain to grow normally. The second goal is to obtain a normal shape of the skull and face. The timing and type of surgery your child will have depends on your child’s condition and your surgeon.

Our surgical team offers the most recent advances in open and minimally invasive correction of craniosynostosis. Our comprehensive team includes a geneticist, craniofacial plastic surgeon, pediatric neurosurgeon and pediatric ophthalmologist who will care for your child every step of the way.

To learn more about craniosynostosis treatment in Richmond, VA, please call to schedule a consultation with one of our craniofacial specialists.

Apert Syndrome

Apert syndrome is a genetic disorder which affects the child’s skull, face, hands and feet. This syndrome will affect the skull by fusion of both coronal sutures (craniosynostosis), causing an abnormal head shape and possibly an increase in the pressure on the brain. These skull changes lead to changes in the face, mainly underdevelopment of the upper and middle part of the face (midface hypoplasia). Facial features include widely spaced eyes, bulging of the eyes, an underdeveloped and ‘sunken-in’ appearance of the mid-face, and/or narrow roof of the mouth. This condition may also involve complex webbing (syndactyly) of the hands and feet.

Causes: Apert syndrome is a very rare genetic disorder. The incidence of children born with this disease can be 1 in 65,000 to 1 in 160,000. It can be passed on from the child’s parents through autosomal dominant inheritance. This means that only one parent needs to have the abnormal gene for the child to inherit the disease. The majority of cases are not inherited, which means that there was a new mutation in the child’s fibroblast growth factor receptor 2 gene, located on chromosome 10.

Treatment: Apert syndrome is a complex condition that requires multiple stages of treatment over the course of the patient’s lifetime. Craniosynostosis is treated by performing surgery to the skull. Oftentimes, the upper and middle face will require surgery as well. The first goal is to open the closed sutures to prevent problems with high pressure in the brain and allow the brain to grow normally. The second goal is to obtain a normal shape of the skull and face. The timing and type of surgery your child will have depends on your child’s condition and your surgeon. Your surgeon will also discuss the timing of hand and feet syndactyly release. Our comprehensive and specialized team includes a geneticist, craniofacial plastic surgeon, pediatric neurosurgeon, and dental specialists who will care for your child every step of the way.

To learn more about Apert syndrome treatment in Richmond, VA, please call to schedule an appointment with one of our craniofacial surgeons.

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Syndactyly

Syndactyly is a congenital condition in which a child is born with a fused finger and/or toe. The appearance of the fused finger or toe is variable and can have soft tissue and bone present.

Treatment: Separation of the fused digit, also called syndactyly release, is a common procedure performed to create a normal webspace. This surgery is typically performed when the child is older than three months of age. Syndactyly release is often combined with a skin graft procedure to put skin along the inner aspect of the separated digits. To learn more about syndactyly and syndactyly treatment in Richmond, VA, please call to schedule an appointment with one of our Richmond pediatric plastic surgeons.

Polydactyly

Polydactyly is a congenital condition in which a child is born with an extra finger and/or toe. The appearance of the extra finger or toe is variable and can have soft tissue and bone present.

Treatment: Removal of the accessory (extra) digit is common and is typically done when the child is older than three months of age.

To learn more about polydactyly and polydactyly treatment in Richmond, VA, please call to schedule an appointment with one of our Richmond pediatric plastic surgeons.

Crouzon Syndrome

Crouzon Syndrome is a genetic disorder which affects the child’s skull and face. This condition occurs when there is abnormal fusion between some of the bones of the skull and the bones of the face, therefore preventing normal growth. Such growth affects the shape of the head, the appearance of the face and the relationship of the teeth.

Causes: Crouzon Syndrome is a very rare genetic disorder. It can be passed on from the child’s parents through autosomal dominant inheritance. This means that only one parent needs to have the abnormal gene for the child to inherit the disease. The majority of cases are not inherited, which means that there was a new mutation in the child’s fibroblast growth factor receptor genes located on chromosome 10 or chromosome 4.

Treatment: Crouzon Syndrome is a complex condition that requires multiple stages of treatment over the course of the patient’s lifetime. Craniosynostosis is treated by performing surgery to the skull. Oftentimes, the upper and middle face will require surgery as well. The first goal is to open the closed sutures to prevent problems with high pressure in the brain and allow the brain to grow normally.

The second goal is to obtain a normal shape of the skull and face. The timing and type of surgery your child will have depends on your child’s condition and your surgeon. Our comprehensive and specialized team includes a geneticist, craniofacial plastic surgeon, pediatric neurosurgeon, and dental specialists who will care for your child every step of the way.

To learn more about Crouzon syndrome treatment in Richmond, VA, please call to schedule an appointment with one of our craniofacial surgeons.

Treacher Collins Syndrome

Treacher Collins Syndrome is a rare genetic disorder that leads to the underdevelopment of the bones and other tissues of the face. There can be a wide range of signs and physical characteristics of this disorder, from almost unnoticeable to severe. Craniofacial abnormalities typically involve the cheek bones, mouth, jaw, chin, ears and/or eyes. Individuals with Treacher Collins syndrome often have downward slanting eyes, few eyelashes, and a notch in the lower eyelids called a coloboma.

Affected individuals will also have underdeveloped zygomas (cheekbones) and small lower jaws. In addition, this condition may lead to hearing loss or cleft palate. In severe cases, underdevelopment of the facial bones may restrict an affected infant’s airway, causing potentially life-threatening respiratory problems.

Causes: Treacher Collins Syndrome is an autosomal dominant disorder, which means that only one parent needs to have the abnormal gene for the child to inherit the disease. Some of the cases are not inherited, which means that there was a new mutation in the child’s genome.

Treatment: Treacher Collins Syndrome is a complex condition that requires multiple stages of treatment over the course of the patient’s lifetime. Cleft palate repair is typically done after age 6 months but depends on the child’s breathing status. It is also important to diagnose hearing problems early therefore our speech language pathologist and ENT surgeons will evaluate the child to determine the need for a bone anchored hearing aid (BAHA). The child may also need surgeries to fix the bones of the middle of the face, the ears and the jaws. The timing of these surgeries will depend on your surgeons. Because the soft tissue of the face is underdeveloped, some children may benefit from fat grafting to improve their facial contour. To learn more about Treacher Collins Syndrome treatment in Richmond, VA, please call to schedule an appointment with one of our craniofacial surgeons.

Craniofacial (hemifacial) Microsomia

Craniofacial Microsomia is a congenital condition in which the lower half of the face does not develop or grow normally. It can affect one or both sides of the face. It can also be referred to as first and second brachial arch syndrome, oral-mandibular-auricular syndrome, lateral facial dysplasia, or otomandibular dysostosis. This condition has various levels of severity, but usually always includes the abnormal development of the ear, the lower jaw (mandible) and mouth. This is the second most common facial birth defect after cleft lip/palate.

Causes: The cause of craniofacial microsomia is unknown. It is a condition that begins during embryonic development.

Treatment: Craniofacial Microsomia is a complex condition that requires multiple stages of treatment over the course of the patient’s lifetime. Sometimes, surgical treatment to restore the child’s jaw position begins during late childhood or early adolescence. This will depend on the severity of the child’s condition, your dental team and your surgeon. Surgical treatment by using a rib graft or mandibular distraction may be performed in certain cases. Children with both sides of their face and ears affected will have difficulty hearing and may benefit from a bone anchored hearing aid (BAHA). Our speech language therapist and ENT surgeon can help evaluate your child’s need for this device and any other speech concerns.

To learn more about craniofacial microsomia treatment in Richmond, VA, please call to schedule a consultation with one of our craniofacial surgeons.

Hemangioma

Hemangiomas are raised birthmarks, normally red, blue or purple. Hemangiomas are clumps of blood vessels that did not grow normally and can appear in all different shapes and sizes over any part of the body. It is important to distinguish a hemangioma from a vascular malformation as this will determine the type of treatment the child might require. Our plastic surgeons can help make this distinction and determine the best course of treatment.

Causes: The cause of hemangioma is unknown. Most appear in the first weeks of life. They go through three stages which include: the proliferation phase, the resting phase and the involution phase. Half of all hemangiomas resolve by age 5 years.

Treatment: The majority of hemangiomas resolve on their own and do not require treatment. Some may require treatment with oral corticosteroids, beta blockers, laser therapy or surgical therapy. This is especially important if the hemangioma affects the child’s vision, breathing or feeding. Your child’s pediatric plastic surgeon can help determine which treatment course is best for your child and their condition.

To learn more about hemangiomas or vascular malformations, please call to schedule an appointment with one of our Richmond pediatric plastic surgeons.

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