Cleft Lip and Palate Repair: An Expert Interview With John Girotto
Cleft Lip and Palate Repair: An Expert Interview With John Girotto
Editor's Note:
Cleft lip and palate are relatively common deformities, but only a few hundred reconstructive surgeons in the United States work with such patients. John A. Girotto, MD, not only cares for children with cleft lip and palate, but he is also the author of numerous articles on the topic. Dr. Girotto is Director of the Golisano Children's Hospital Cleft and Craniofacial Anomalies Center and Associate Professor in the Departments of Plastic Surgery, Neurosurgery, and Pediatrics at the University of Rochester. He recently spoke to Medscape's Pippa Wysong about the latest developments in cleft palate management.
Medscape: How common are cleft lip and palate and how big of a problem are they?
Dr. Girotto: Cleft lip and palate are both fairly common genetic birth defects. In the white population, cleft lip and palate occur in 1 in 700 to 1000 births. These defects are more common in Asian populations and much less common in the black population. Cleft lip and palate affect the fusion of the lip, the gumline, and the hard and soft palate of the roof of the mouth. Young children with open cleft palates have difficulty eating because the nose and the mouth communicate with each other. As these children get older, they will have difficulty with speech production and communication if their defects are not repaired. Cleft lip and palate can also affect the eustachian tubes and lead to poor hearing.
Medscape: What causes cleft lip and palate?
Dr. Girotto: Cleft lip and palate are almost always nonsyndromic or nonhereditary and occur randomly.
Medscape: Is there a particular age when it's best to treat cleft palate?
Dr. Girotto: It's best to fix a cleft palate before the production of meaningful speech. You want to fix it when the child starts to babble and begins to experiment with words and vocabulary. That's because you don't want the child to adopt bad habits to compensate for a wide-open palate; you want the child to learn how to speak clearly. There is some debate on this. Cleft palate can be fixed any time from 6 months of age to just over 1 year. If you wait longer than about 15 months, you start having problems with speech production. In my own practice, I like to fix cleft palate at about 10 months. Pressure-equalization tubes are placed at that time to help with hearing.
Medscape: You've performed studies about current surgical practices across the country. Are there issues related to different surgical practices?
Dr. Girotto: Two methods of repairing a cleft palate are widely practiced. One is the double-opposing Z-plasty, which helps with closure of the palate. The other is a straight-line repair with direct dissection of levator musculature and reorientation of that musculature in a similar coronal fashion.
The primary goal of both operations is anatomical reconstruction of the levator musculature of the soft palate. This goal is achieved either passively, with the Furlow Z-plasty technique, or a little more directly, with an intravelar veloplasty in a straight-line repair technique. It's the surgeon's preference as to which method to use.
Evidence that speech outcomes are better with the Furlow palatoplasty has been published. However, the oral fistula complications are higher with that technique, especially in inexperienced hands. The data are mediocre and largely come from retrospective or practitioner- and institution-specific studies.
Medscape: Should an otolaryngologist be consulted for these cases?
Dr. Girotto: It's not specialty-specific, but kids with cleft lip and palate should be treated by a large multidisciplinary team. When a child has both a cleft lip and a cleft palate, you need the input of specialists in speech pathology, dentistry, orthodontia, otolaryngology, and plastic surgery all working together, holistically, as the child develops from infancy all the way to adulthood. It's a long-term team effort.
Medscape: What are some of the special issues or challenges with particular types of cleft lip and palate?
Dr. Girotto: There are 2 or 3 different staging classification systems for cleft lip and palate. As a palate fuses in utero, it fuses posteriorly from the incisor foramena. It can fuse all the way back and leave just a bifid uvula. There could be a submucous cleft, whereby everything looks normal but the muscles are in the wrong orientation so they don't fuse.
The approach to management depends on whether there is a submucous cleft or an open cleft palate, either soft or hard palate, or both. If the palate is open, it needs a definitive repair -- either a Furlow repair or an intravelar veloplasty. With a submucous cleft, however, the child may still be able to produce good speech, so we often delay their treatment until they start producing speech. These children can be treated a little later in life, if necessary.
Medscape: Are there any special considerations for patients prior to surgery?
Dr. Girotto: Before surgery, it's all about eating and gaining weight. Kids with cleft palates cannot generate suction, so they generally cannot breastfeed. However, in underdeveloped countries, mothers are very active in expressing breastmilk and feeding these children, even children with wide-open clefts. In the United States, we suggest pumping and using a special squeezable bottle to feed these infants. One problem with this is that these infants aspirate a lot more air. This is linked to a higher incidence of gastroesophageal reflux in children with cleft lip and palate, but it's mostly a mechanical issue.
Medscape: Are there special postsurgery considerations?
Dr. Girotto: Once the palate is repaired, it's all about speech production and speech therapy over the next 2-3 years and seeing how they grow, develop, and learn to use their new anatomy. Immediately after surgery, the child is pretty sore for about 2 days, and eating is difficult for the first day or 2. But within 2 weeks, if you look in the child's mouth -- if the healing is normal -- you will hardly see any scarring. Many kids wear arm restraints for about 2 weeks so they don't put spoons, crackers, or other things in their mouths.
Medscape: Some cleft palate holes are quite large. Any tips for dealing with those?
Dr. Girotto: Generally, there's enough tissue present in the different layers that you can manipulate into place. But if there is a tissue absence, specifically at the junction of the hard and soft palate where things can be tight, AlloDerm® or donor cadaveric tissue has been advocated to help prevent fistula formation. I haven't found a need for this in my practice.
Medscape: Are cleft palate revisions common?
Dr. Girotto: The literature says that up to 20% of kids with a cleft palate will need some future surgery to help with speech production. They would still have velopharyngeal insufficiency or air escape behind the soft palate when they speak, which gives them a hypernasal-sounding voice. A variety of different procedures to make that vocal quality better have been described.
Medscape: Do surgeons ever need to do revisions because a child grows over time?
Dr. Girotto: Yes. The scar tissue associated with a cleft palate repair can limit the growth of the upper jaw such that patients can have an underbite as they start hitting their teenage and adult years. There's a role for oral surgery or orthognathic surgery to move the jaw forward, so you need to work with an orthodontist as well.
Medscape: What about other surgeries that these kids might need?
Dr. Girotto: Things need to be done in the right order. For instance, say someone first does a cosmetic operation, like a rhinoplasty. Then someone else goes in later to put the patient's teeth in the right position. They're moving the nose that somebody spent hours trying to make look good.
Medscape: What sorts of issues are there with patients in whom cleft lip and palate are part of a syndrome?
Dr. Girotto: Although rare, probably the most common is the 22q deletion syndrome. Those kids can have psychological problems as well as heart and vascular anomalies. It used to be called DeGeorge's syndrome or velocardiofacial syndrome, but when the gene was found, all of those syndromes were grouped together under the 22q deletion diagnosis. These kids are not any more difficult for the plastic surgeon, but to address the other issues, these kids need the services of a large children's hospital.
Medscape: Are there any new developments in the field?
Dr. Girotto: One area that is relatively new is the role of sleep apnea in these patients, which develops in about 50% to 60%. As kids with cleft lip and palate get a little older and midface scarring occurs, the midface might not grow as much, increasing the risk for sleep apnea. Basically, you need to have an increased suspicion for symptoms of sleep apnea in children with cleft lip and palate. Affected children are best served by a multidisciplinary team with a sleep specialist who can do sleep studies and provide treatment for apnea as children get older.
Medscape: What does the future hold for the treatment of cleft lip and palate?
Dr. Girotto: The biggest change will probably be in the study of sleep apnea and how we're going to take care of behavior and growth down the line. We'll continue to optimize speech outcomes so that these kids don't need revision surgeries. Ongoing research is exploring the genetic anomalies that lead to these birth defects, so who knows where that might lead.
Editor's Note:
Cleft lip and palate are relatively common deformities, but only a few hundred reconstructive surgeons in the United States work with such patients. John A. Girotto, MD, not only cares for children with cleft lip and palate, but he is also the author of numerous articles on the topic. Dr. Girotto is Director of the Golisano Children's Hospital Cleft and Craniofacial Anomalies Center and Associate Professor in the Departments of Plastic Surgery, Neurosurgery, and Pediatrics at the University of Rochester. He recently spoke to Medscape's Pippa Wysong about the latest developments in cleft palate management.
Medscape: How common are cleft lip and palate and how big of a problem are they?
Dr. Girotto: Cleft lip and palate are both fairly common genetic birth defects. In the white population, cleft lip and palate occur in 1 in 700 to 1000 births. These defects are more common in Asian populations and much less common in the black population. Cleft lip and palate affect the fusion of the lip, the gumline, and the hard and soft palate of the roof of the mouth. Young children with open cleft palates have difficulty eating because the nose and the mouth communicate with each other. As these children get older, they will have difficulty with speech production and communication if their defects are not repaired. Cleft lip and palate can also affect the eustachian tubes and lead to poor hearing.
Medscape: What causes cleft lip and palate?
Dr. Girotto: Cleft lip and palate are almost always nonsyndromic or nonhereditary and occur randomly.
Medscape: Is there a particular age when it's best to treat cleft palate?
Dr. Girotto: It's best to fix a cleft palate before the production of meaningful speech. You want to fix it when the child starts to babble and begins to experiment with words and vocabulary. That's because you don't want the child to adopt bad habits to compensate for a wide-open palate; you want the child to learn how to speak clearly. There is some debate on this. Cleft palate can be fixed any time from 6 months of age to just over 1 year. If you wait longer than about 15 months, you start having problems with speech production. In my own practice, I like to fix cleft palate at about 10 months. Pressure-equalization tubes are placed at that time to help with hearing.
Medscape: You've performed studies about current surgical practices across the country. Are there issues related to different surgical practices?
Dr. Girotto: Two methods of repairing a cleft palate are widely practiced. One is the double-opposing Z-plasty, which helps with closure of the palate. The other is a straight-line repair with direct dissection of levator musculature and reorientation of that musculature in a similar coronal fashion.
The primary goal of both operations is anatomical reconstruction of the levator musculature of the soft palate. This goal is achieved either passively, with the Furlow Z-plasty technique, or a little more directly, with an intravelar veloplasty in a straight-line repair technique. It's the surgeon's preference as to which method to use.
Evidence that speech outcomes are better with the Furlow palatoplasty has been published. However, the oral fistula complications are higher with that technique, especially in inexperienced hands. The data are mediocre and largely come from retrospective or practitioner- and institution-specific studies.
Medscape: Should an otolaryngologist be consulted for these cases?
Dr. Girotto: It's not specialty-specific, but kids with cleft lip and palate should be treated by a large multidisciplinary team. When a child has both a cleft lip and a cleft palate, you need the input of specialists in speech pathology, dentistry, orthodontia, otolaryngology, and plastic surgery all working together, holistically, as the child develops from infancy all the way to adulthood. It's a long-term team effort.
Medscape: What are some of the special issues or challenges with particular types of cleft lip and palate?
Dr. Girotto: There are 2 or 3 different staging classification systems for cleft lip and palate. As a palate fuses in utero, it fuses posteriorly from the incisor foramena. It can fuse all the way back and leave just a bifid uvula. There could be a submucous cleft, whereby everything looks normal but the muscles are in the wrong orientation so they don't fuse.
The approach to management depends on whether there is a submucous cleft or an open cleft palate, either soft or hard palate, or both. If the palate is open, it needs a definitive repair -- either a Furlow repair or an intravelar veloplasty. With a submucous cleft, however, the child may still be able to produce good speech, so we often delay their treatment until they start producing speech. These children can be treated a little later in life, if necessary.
Medscape: Are there any special considerations for patients prior to surgery?
Dr. Girotto: Before surgery, it's all about eating and gaining weight. Kids with cleft palates cannot generate suction, so they generally cannot breastfeed. However, in underdeveloped countries, mothers are very active in expressing breastmilk and feeding these children, even children with wide-open clefts. In the United States, we suggest pumping and using a special squeezable bottle to feed these infants. One problem with this is that these infants aspirate a lot more air. This is linked to a higher incidence of gastroesophageal reflux in children with cleft lip and palate, but it's mostly a mechanical issue.
Medscape: Are there special postsurgery considerations?
Dr. Girotto: Once the palate is repaired, it's all about speech production and speech therapy over the next 2-3 years and seeing how they grow, develop, and learn to use their new anatomy. Immediately after surgery, the child is pretty sore for about 2 days, and eating is difficult for the first day or 2. But within 2 weeks, if you look in the child's mouth -- if the healing is normal -- you will hardly see any scarring. Many kids wear arm restraints for about 2 weeks so they don't put spoons, crackers, or other things in their mouths.
Medscape: Some cleft palate holes are quite large. Any tips for dealing with those?
Dr. Girotto: Generally, there's enough tissue present in the different layers that you can manipulate into place. But if there is a tissue absence, specifically at the junction of the hard and soft palate where things can be tight, AlloDerm® or donor cadaveric tissue has been advocated to help prevent fistula formation. I haven't found a need for this in my practice.
Medscape: Are cleft palate revisions common?
Dr. Girotto: The literature says that up to 20% of kids with a cleft palate will need some future surgery to help with speech production. They would still have velopharyngeal insufficiency or air escape behind the soft palate when they speak, which gives them a hypernasal-sounding voice. A variety of different procedures to make that vocal quality better have been described.
Medscape: Do surgeons ever need to do revisions because a child grows over time?
Dr. Girotto: Yes. The scar tissue associated with a cleft palate repair can limit the growth of the upper jaw such that patients can have an underbite as they start hitting their teenage and adult years. There's a role for oral surgery or orthognathic surgery to move the jaw forward, so you need to work with an orthodontist as well.
Medscape: What about other surgeries that these kids might need?
Dr. Girotto: Things need to be done in the right order. For instance, say someone first does a cosmetic operation, like a rhinoplasty. Then someone else goes in later to put the patient's teeth in the right position. They're moving the nose that somebody spent hours trying to make look good.
Medscape: What sorts of issues are there with patients in whom cleft lip and palate are part of a syndrome?
Dr. Girotto: Although rare, probably the most common is the 22q deletion syndrome. Those kids can have psychological problems as well as heart and vascular anomalies. It used to be called DeGeorge's syndrome or velocardiofacial syndrome, but when the gene was found, all of those syndromes were grouped together under the 22q deletion diagnosis. These kids are not any more difficult for the plastic surgeon, but to address the other issues, these kids need the services of a large children's hospital.
Medscape: Are there any new developments in the field?
Dr. Girotto: One area that is relatively new is the role of sleep apnea in these patients, which develops in about 50% to 60%. As kids with cleft lip and palate get a little older and midface scarring occurs, the midface might not grow as much, increasing the risk for sleep apnea. Basically, you need to have an increased suspicion for symptoms of sleep apnea in children with cleft lip and palate. Affected children are best served by a multidisciplinary team with a sleep specialist who can do sleep studies and provide treatment for apnea as children get older.
Medscape: What does the future hold for the treatment of cleft lip and palate?
Dr. Girotto: The biggest change will probably be in the study of sleep apnea and how we're going to take care of behavior and growth down the line. We'll continue to optimize speech outcomes so that these kids don't need revision surgeries. Ongoing research is exploring the genetic anomalies that lead to these birth defects, so who knows where that might lead.
