Cleft Palate, Pierre Robin, and Tonsillectomy

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There are many things to sort out regarding your five year old. First, I compliment you on becoming well-informed about the many issues involved with your child. The Pierre Robin condition was first described as a syndrome, then later, termed as an anomalad, and currently, is known as the Pierre Robin sequence, denoting that there are a sequence of events involved that characterize the breathing and feeding problems associated with this condition. As you are well aware, the oral cavity, pharynx and lower jaw are expected to be quite small at birth, but do show improvement over time. Cleft palate is a common finding with Pierre Robin.

Due to the small pharynx and mandible, further surgery on the soft palate to provide a normal level of nasality in your daughter's voice would logically involve a palatal lengthening procedure as the first choice, such as the Furlow operation or an intravelar veloplasty. If even more surgery is indicated later on, a sphincter pharyngoplasty would be preferred over a pharyngeal flap in most instances with Pierre Robin. Creating a smaller sphincter allows for airflow through the nasopharynx for normal breathing. A pharyngeal flap, by contrast, can create an obstructive situation, especially where there is already a relatively small nasopharynx. If your daughter's soft palate moves actively in speech attempts, she would be considered a good candidate for palatal lengthening. If her soft palate does not exhibit much elevation during speech, a pharyngeal flap would be a reasonable choice since the flap does not depend on palatal movement but rather, movements of the lateral pharyngeal walls at the lateral portals of the flap.

You mentioned that a surgeon has attempted to close a palatal fistula, which I assume from your report was in the soft palate. Fistulae can occur in spite of the skill of even the best surgeon. I think you can appreciate the difficulty surgeons face in closing palates where there is a deficiency in tissue. A fistula in the hard palate can be especially difficult to repair. In the soft palate, closure of a fistula would not automatically involve a revision of the entire velum. I would encourage you not to wonder about the skill of the surgeon based upon the presence of a post-surgical fistula.

One final noteI do not agree with the surgical recommendation for a pharyngeal flap on a 5 year old with the Pierre robin sequence even with a mild expression of facial features. A palatal lengthening and tonsillectomy appears to me to represent the most conservative and potentially helpful procedure.

Now a few words about the tonsils. Since the airway is likely small in your child, and if the tonsils are large, which they normally can be at age 5, the tonsils can inhibit the full range of movement of the soft palate and can also obstruct the airway to some extent. Removing the tonsils should serve to enlarge the oral isthmus (area between the tonsils) and also allow for full elevation of the soft palate. The combination of palatal lengthening and tonsils removal can conceivably achieve the best speech result.

The conflicting information you mention about tonsils actually relates to discussions of tonsils and adenoids together. Both are lymphoid tissue masses in the pharynx. Every physician and dentist should know that an adenoidectomy is contraindicated in children with cleft palate since the adenoid mass serves as a target for soft palate contacts during speech. This is not the case, however, for the faucial tonsils. For some patients, adenoid tissue may need to be removed for medical reasons. If there is a question of the possibility of hypernasality resulting from an adenoidectomy, a lateral adenoidectomy (also called a peritubal adenoidectomy) can be done. The conflicting information about tonsils is likely linked to surgeons commonly referring to T&A's, or removal of tonsils and adenoids together, but they are separate tissue masses with a differing potential impact on speech. In the case of your daughter I would not worry about any conflicting information or problems from tonsillectomy and I feel comfortable with the knowledge and recommendations of the surgeon with combined ENT and plastics training and experience.

You also mentioned that your daughter has congenital lymphedema. Although this typically affects the extremities, the accumulation of interstitial fluid may present a concern for a tonsillectomy since tonsils are a part of the lymphatic system. Yes, your surgeon should be knowledgeable about lymphedema and can apprise you of the benefits or potential problems with tonsillectomy for your child.

In summary, I appreciate how confusing it can become to seek out several opinions and receive different surgical recommendations for how to proceed. From what you have reported in your questions here, I can recommend comfortably that I would opt for a palatal lengthening procedure at this point. Later, if additional surgery is needed, and if your daughter's palate moves well, a sphincter pharyngoplasty would be preferred over a pharyngeal flap.

If the information and opinions expressed here ease your concerns or help you decide on the next course of surgery, or provider, I will be happy. Good luck.

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Robert M. Mason, DMD, Ph.D. is a speech-language pathologist (CCC-ASHA Fellow), a dentist, and orthodontist. He is a Past President of the American Cleft Palate-Craniofacial Association, a professional, interdisciplinary organization specializing in problems associated with facial and oral deformities. Dr. Mason has studied and written extensively about orofacial examination, developmental problems related to the tongue, and the anatomy and physiology of the speech and hearing mechanisms. His reports have appeared in speech, dental, medical, and plastic surgical journals and texts. He is considered to be an expert in tongue thrusting, tongue tie, and other problems related to tongue functions and speech.