The Center for Hemifacial Microsomia and Ear Deformities at Cohen Children’s is dedicated to correcting facial asymmetry and ear abnormalities. Underdevelopment of the eye sockets, cheek, jaw and ear can range from mild to severe. Our center utilizes the latest in 3D CAD/CAM technology to properly plan and execute the skeletal and soft tissue reconstruction to restore symmetry and the normal facial architecture of your child.
What we treat
At the Center for Hemifacial Microsomia and Ear Deformities, we provide treatment for a full range of facial asymmetry and ear abnormalities. These include:
Hemifacial macrosomia - Occurs in approximately one in 3,500 live births. Hemifacial microsomia is the second most common craniofacial anomaly after clefts. It is most commonly due to a malformation of the first and second branchial arches in utero, which the jaw is derived from. This condition is traditionally diagnosed according to the OMENS system, which reflect the abnormalities in the:
- Orbit (eye socket)
- Mandible (lower jaw)
- Nerve (VII, facial movement)
- Soft tissue (facial fat and musculature)
Patients with hemifacial microsomia often have ear abnormalities including malformed, small or absent ears as well as possible loss of conductive hearing due to lack of an ear canal. Sometimes only mild defects, such as skin tags, are the only sign of an ear deformity. Skin tags are typically excised at the age of 6 months, leaving a thin scar hidden in the shadows of your child’s face.
Severely malformed or absent ears require a more complex reconstruction which is often delayed until your child is 6 to 8 years old. This allows the cartilage to be big enough to be used to reconstruct your child’s ear. Alternatively, a synthetic ear (made of a plastic implant) can be placed earlier. The opening of the ear canal (meatoplasty) and possible hearing aid is more often not performed until after the reconstruction of the external ear.
Goldenhaar syndrome - Also known as oculo-auricular-vertebral syndrome, Goldenhaar syndrome is similar to hemifacial microsomia with the added abnormalities of spine and eye anomalies. Epibulbar dermoids, benign tumors in the eye, are common as well as malformations of the cervical spine. Goldenhaar syndrome may also affect both sides of face requiring extensive reconstruction.
Keloids - Keloids are tumors composed of scar tissue that may affect children who have a genetic predisposition. Ear lobule keloids are common in areas of piercings and can be very noticeable. Current treatment is a combination of excision, reconstruction and then postoperative low dose radiation. This maximally decreases the risk of recurrence. For complex keloids located in areas that are not amenable to radiation, we can offer dermal substitutes and epidermal grafts to reduce recurrence.
Ear trauma - Injury to the ear resulting from trauma or cancer may require reconstruction using a combination of local tissue and cartilage grafting. Cartilage may be obtained from the nasal septum or from the ribs (similar to microtia reconstruction). Multiple surgeries may be necessary to reproduce the intricate details and anatomy of the external ear.
Microtia - Microtia (small ear) or anotia (absent ear) may be present at birth either as isolated incidents or as part of hemifacial microsomia. Kidney abnormalities may also be present. In these cases, a renal ultrasound may be necessary. In addition to the external ear malformation, the ear canal is often absent, which can contribute to hearing loss.
A polymer implant, such as Medpor, or rib cartilage can be used to re-create the structural frame of the ear. It is preferred to use cartilage given its resistance to infection and longevity of the reconstruction. This reconstruction is usually postponed until at least age of 8 to allow for growth of the rib cage.
New ear framework is sculpted from the cartilages and buried underneath the skin where the new ear is to be created. Approximately three months later the “new ear” is elevated from underneath the skin and grafted to create the “normal” projection.
Alternatively, prosthetics are also available. The disadvantage is they are prone to fall off (since they are held on by glue) and degrade requiring expensive replacements. The cartilage-sculpted ear is the preferred permanent reconstruction recommended by most craniofacial surgeons and is more resistant to trauma and infection than synthetic ears.
Treatments offered at the Center for Hemifacial Microsomia and Ear Deformities include:
Ear molding - Ear molding is a nonsurgical procedure used to correct common deformities of the ear. Using an infant’s soft and pliable cartilage and silicone devices, doctors mold the ear into the proper shape. Ideally, the doctor will begin the process of molding the ear within two weeks of birth. The devices are placed in the office without any pain or need for anesthesia.
The molding device must be worn constantly for a period of two to six weeks for best results; older infants require longer molding times. We offer children a nonsurgical, scar-less alternative for correcting abnormally shaped ears. The following are common types of ear deformities that we treat:
- Lop ear (folded ear)
- Prominent ears (Dumbo ears)
- Stahl ear (Spock ear)
- Hidden ear (cryptotia)
The devices are placed on your child’s ear using an adhesive tape and often require reapplication within two weeks. After completion of treatment no further surgery or intervention is typically required.
Nerve reconstruction - The facial nerve may also be affected in hemifacial microsomia causing partial or total facial paralysis. The facial nerve is responsible for raising the eyebrow, closing the eyelids, smiling and moving the lips and neck. Static (fixed in a held position) or dynamic (restoring motion) procedures may be necessary to restore facial balance.
Soft tissue reconstruction - Difference in soft tissue volume also contributes to the facial asymmetry and new tissue must be introduced to equalize the two sides of the face. This can often be accomplished using fat grafting procedures where fat is harvested from the abdomen and thighs and injected into the area or using free tissue transfers (microsurgically transplanting tissue from one area of the body to another). The goal is to restore facial harmony and often multiple procedures may be required to achieve the best result.
Mandible reconstruction - A small lower jaw is known as mandibular hypoplasia and it can be isolated or part of a syndrome along with other deformities. One or both sides of the jaw can be involved. Treatment is aimed at lengthening the part of the jaw that is affected. This can be done surgically using either bone grafts or mandibular distraction. This will improve the facial aesthetics as well as the functional bite (occlusion). A 3D CT scan is usually required to help gain insight as to extent of the mandibular deformity.