The joy parents feel at the birth of their child or the delight they have during the toddler and preschool years can be overshadowed by concern when that child has an abnormality -- even one that’s correctable.

And yet, the outlook can be very promising for young patients with conditions such as cleft lip, cleft palate, craniosynostosis and microtia when they receive not only the surgery they need, but comprehensive follow-up care. At University Hospital, Dr. Samuel Rhee, a pediatric plastic surgeon and an assistant professor in the Division of Plastic and Reconstructive Surgery at New Jersey Medical School, is one of the team members who can help restore both form and function to children with certain birth defects.

Cleft lip and cleft palate are two different yet related conditions, in that they both involve a separation in a part of the body. As the names suggest, cleft lip occurs when there is a separation along the lip; cleft palate involves a separation in the roof of the mouth. “Usually, a cleft lip is observable at birth,” says Dr. Rhee. “While a cleft palate is also often noticeable then, sometimes it is only when the parents attempt to feed the baby and the breast milk or formula comes out of their child’s nose that they realize something might be out of the ordinary.”

Both cleft lip and cleft palate are very treatable with surgery, says Dr. Rhee, who performs the operations at University Hospital and also goes on humanitarian medical trips to assist cleft lip and palate patients in developing countries. Typically, he says, the operation to correct cleft lip is done when the baby is three months old, and for cleft palate, at nine months. In the weeks or months before the surgeries, special nipple feeder bottles and feeding techniques can be used to ensure that the baby receives proper nutrition. Presurgical taping techniques are also used in preparation for cleft lip surgery.

The goal of the surgeries for cleft lip and cleft palate is the same – to fill in the missing area. For a baby with cleft lip, tissue is taken from either side of the cleft and muscle is reattached to “build” a new lip. “Similarly, with cleft palate, we fill in the hole in the roof of the mouth with surrounding tissue,” he says. Both operations last about two hours and require an overnight hospital stay to ensure that the baby is eating and breathing properly.

stages of cleft lip surgery

The first surgery is not the last medical treatment cleft lip and cleft palate patients should have. Often with cleft palate, additional surgeries are needed later in life. But there’s more, says Dr. Rhee. “Patients with these conditions should be seen at regular intervals until they are 18, and they often require the expertise of other health care professionals. Children with cleft palate often are prone to developing ear infections, for example, and might need to have tubes placed in their ears. Some children’s speech is affected by their condition, and they benefit from speech therapy. Others may need to see an orthodontist or pediatric dentist to resolve oral health problems. Each child with cleft lip or cleft palate has very individual needs that are best met by a multidisciplinary team such as the one we have at University Hospital.”

When the Skull Fuses Too Quickly

Within about the first year of a child’s life, the brain doubles in size. If that’s not amazing enough, the skull bones that surround the body’s most complex organ are not one piece at infancy, but rather, several that fuse together over many months. “Sometimes, though, the fusion occurs too soon, while the brain is still growing,” says Dr. Rhee. “This condition, known as craniosynostosis, can have serious consequences if not treated at the right time – between six to nine months. High pressure can build up within the skull, which can cause developmental issues and less commonly, blindness or even death.”

The good news is that many cases of craniosynostosis are diagnosed early, particularly when the infant receives well-baby check-ups at the intervals recommended by pediatricians. At each visit, the doctor evaluates at what rate the baby’s skull bones are fusing.

Children diagnosed with craniosynostosis need reconstructive surgery, performed by a plastic surgeon and a pediatric neurosurgeon as part of a multidisciplinary team, to correct the fast-closing skull bones. “In surgery, the baby’s skull bones are released to permit for brain growth, then recontoured,” says Dr. Rhee. The operation lasts about four or five hours. Following the surgery, the baby spends one or two days in the pediatric intensive care unit, and then a total of about a week on the pediatric floor. “Children who have craniosynostosis surgery usually have excellent results, and oftentimes there is no further treatment needed,” he says.

Sometimes parents worry when their baby’s head appears to be misshapen, says Dr. Rhee, but most often, it’s because the infant favors one side to sleep or is consistently placed on his or her back to rest. The back sleep position was recommended several years ago after researchers made a connection between babies who slept on their bellies and Sudden Infant Death Syndrome (SIDS). Dr. Rhee encourages parents to follow pediatric recommendations on sleep positions, but to change the side the baby sleeps on each time. By sleeping consistently on one side, an infant’s malleable skull can become misshapen, a condition known as positional molding. “Most often, positional molding can be corrected by a special helmet, but the downside to that is that the baby needs to wear the helmet 24 hours a day for a certain period of time,” says Dr. Rhee.

Help for Underdeveloped Ears

A fetus’ ears and hearing develop early on in pregnancy. At about five weeks’ gestation, the ears begin to form, and by approximately the 24 th week, the hearing mechanisms are typically in place. Sometimes, however, the ears and possibly hearing do not develop as they should, and the baby is born without an outer ear or ears, or with only small parts of the organs. This congenital condition is known as microtia. Doctors are not certain what causes many ear and hearing conditions such as microtia, but decreased blood flow to the ear or ears during pregnancy, environmental factors and certain drugs taken by the mother are possibilities.

Microtia is observable at a child’s birth, says Dr. Rhee, but because the condition might also involve the middle and inner ear – and thus affect hearing – the infant should be evaluated by pediatric ear, nose and throat doctor and an audiologist. Upon discovery of a hearing loss, the ENT might recommend surgery to facilitate better hearing, such as the creation of an ear canal, if one is lacking.

Dr. Rhee’s primary role is to reconstruct the external ear or ears. He says the operation is usually not done until the child is between six and seven years old, because that is about when a typical child’s ears reach full growth. “If we reconstructed the ear when the child was younger, we’d only have to do a second operation a few years later,” he says.

To reconstruct the ears, Dr. Rhee needs a substance from which to “build.” His preference – and that of many other pediatric plastic surgeons – is to take a cartilage graft from the patient’s ribs. The initial operation involves harvesting the rib graft, followed by the actual reconstructive surgery. The operation takes between four and five hours and requires an overnight hospital stay. Other surgeries follow to refine the ear’s shape and position.

For a consultation or more information about the Division of Plastic and Reconstructive Surgery, you may contact Dr. Samuel Rhee at (973) 972-8092.