Submucous Cleft and Adenoidectomy: Issues and Concerns

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You have an ENT specialist who is appropriately addressing the middle ear problems with ear tubes. The ENT is also aware of a possible submucous cleft and knows that a bifid uvula is a characteristic associated with submucous clefting. I don't doubt his finding that no submucous cleft of the hard palate was identified by palpation. I also agree that it is likely that your child will need surgical removal of the adenoid mass, at least around the eustachian tubes, since this is apparently, and logically, contributing to the problem in the middle ear. But there is more to this story.

Not all submucous clefts of the palate are alike. Some are discrete and may show no clear intraoral features. As you may know, a submucous cleft can occur in the hard palate and soft palate, or only in the soft palate. A typical finding with submucous clefts is a pale blue zona (a zona pellucida) extending down the midline from hard palate to soft palate. Where there is a deficiency in bone at the posterior of the hard palate (a notching or an inverted V-shaped defect in bone), the muscles of the soft palate course forward to attach onto the anteriorly-displaced palatal aponeurosis rather than attaching into one another at the midline aponeurosis of the soft palate. Where this situation is present, the overall length of the soft palate is usually deficient and unable to maintain an appropriate contact in speech with the posterior wall of the pharynx.

The adenoid mass in your daughter may serve as a contact site for the soft palate during speech. As the adenoid mass spontaneously decreases in size over time (a process referred to as involution of the adenoid), velopharyngeal incompetence and a persistent hypernasality may develop if there is a short soft palate.

From your report of the findings by your ENT, it may be that the bifid uvula is an isolated condition. A bifid uvula is found in 1 of 76 individuals in the normal population. However, your report of the development of some transient hypernasality raises concerns about the adequacy of your daughter's soft palate to achieve velopharyngeal closure, and as well the recommendation of an adenoidectomy; the result of which could be a persistent hypernasal voice that would require a corrective pharyngoplasty. With these potential problems in mind, I will make several suggestions that may hopefully ease or further illuminate your appropriate concerns.

First, a few suggestions about further assessment for a possible submucous cleft. During a sustained "ah", you should try to identify by intraoral inspection where the soft palate dimples or buckles inward. This is revealed only during function. You can use a tongueblade to press down on the base of the tongue. This will reveal the uvula and also will gag your daughter, providing a good view of the palatal dimple. The palatal dimple is normally seen just above the uvula, or about 80% of the way back on the soft palate from the end of the hard palate to the uvula. If the dimple is displaced forward on the soft palate - toward the 50% range from hard palate to the uvula, this would suggest a submucous cleft. The dimple is an indicator of the attachment area of the levator palatini muscles in the soft palate. Any palatal tissue between the dimple and the uvula would not be useful in achieving velopharyngeal closure since tissues beyond the dimple hang pendulously.

A submucous cleft may also show other discrete signs such as a lack of full elevation due to tethering on the nasal surface of the palate by extra connective tissue strands. This characteristic of some discrete submucous clefts would be identified by nasendoscopy. Normally, a soft palate should elevate up to the plane of the hard palate as seen by intraoral inspection; however, with large adenoids, full palatal elevation may not be possible.

Your ENT specialist is obviously well versed to advise you about the adenoids. While the adenoids can grow to the extent that they would be considered large for a 2 year 8 month old, large adenoids are not uncommon at this age. The key feature of the adenoid mass for your daughter is encroachment of adenoidal tissue around the eustachian tubes which are found on the lateral walls of the pharynx. This tissue encroachment can interfere with the normal ventilation process of the middle ear and lead to middle ear problems.

Where hypernasality is already reported, along with the presence of a bifid uvula, extreme care should be exercised in recommending removal of adenoids. I like your ENT's decision to delay consideration of an adenoidectomy at present. While an adenoidectomy may become a medical necessity at some point, delaying consideration for an adenoidectomy provides time to more fully evaluate your daughter's mouth and pharynx.

There are alternatives to a complete adenoidectomy which your ENT specialist is well aware of and may recommend at an appropriate time. In instances where a total adenoidectomy has the potential to result in severe and persistent hypernasality, a lateral adenoidectomy (also called a peritubal adenoidectomy) can be accomplished. This involves a dissection procedure to remove adenoidal tissue around the eustachian tubes while retaining a midline strip of adenoids that can continue to serve as a target for velopharyngeal closure. In some ENT centers, a lateral adenoidectomy is now being done nasendoscopically. On the other hand, if your child continues to exhibit hypernasality over the next year, your ENT may decide to remove all of the adenoids knowing that she will need a pharyngoplasty subsequent to adenoidectomy. He would advise you of this possibility prior to the adenoidectomy. For now, I appreciate his wait and see attitude.

While I do not doubt the competence of your ENT specialist, I would suggest that you seek an additional diagnostic evaluation for a submucous cleft at a cleft palate center. It would be helpful to have a radiographic image of the pharynx to view the outline of the adenoid mass and to obtain additional information about the anatomical characteristics of your daughter's palates. This additional evaluation is recommended since you raised some concern about your ENT deciding that a submucous cleft was not present based only upon palpation of the posterior surface of the hard palate. An assessment at a cleft palate center should provide the additional information that you desire.

While every parent wants the best for their child, I urge you to be patient and not push for an adenoidectomy in the near future. I agree with your ENT specialist in this regard. I also suggest that you provide your ENT with regular reports of your daughter's successes or difficulties with feeding, breathing and speech. This is certainly important information that can be used in the decision about the timing of adenoid removal.

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.