MEDICAL PRACTICE

Submucous cleft and the general practitioner R. B. Lowry, m.b., b.ch., f.r.c.p.[c], A. D. Courtemanche, m.d., f.r.c.s.[c] and C. MacDonald, m.a., Vancouver, B.C.

Summary: Submucous cleft palate latente, la lesion peut etre revelee is a permanent feature after this opera¬ refers to a situation where the soft par une adenoidectomie: en effet les tion. Such patients probably have a palate is largely composed of mucosa vegetations adenoides ont jusque la submucous cleft palate. Their soft with little or no muscle. The defect constitue un facteur compensatoire palate contains little or no muscle, is often not obvious on inspection of permettant la fermeture palatopha- being composed almost exclusively of the mouth and . There is ryngienne. II importe done que les mucosa, and therefore is unable to considerable clinical variation, with medecins qui pratiquent I'amygdalec- effect palatopharyngeal closure. Fig. 1 speech ranging from normal or minimal tomie et I'adenoidectomie connaissent summarizes the signs and symptoms nasality to severe nasality and defective bien les signes et symptdmes qui which should lead a physician to sus- articulation. Many patients who have peuvent mener au diagnostic. pect this disorder. Bifid uvula is a latent submucous cleft palate have frequent but not an invariable finding, the condition unmasked by an Many physicians are familiar with the and a variety of other palatal morpho- because the adenoid fact that occasional patients may have logic variations may be seen.1 pad had served as a compensatory hypernasal speech following a tonsillec¬ factor in effecting palatopharyngeal tomy and adenoidectomy. Usually this Effects on speech closure. All physicians who perform is temporary; however, there are a few tonsillectomy and adenoidectomy patients in whom the hypernasal speech Normally the closes off should be aware of the signs and symptoms which may suggest the diagnosis. Resume: La fissure palatine sous- muqueuse et I'omnipraticien Dans la fissure palatine sous- muqueuse, le palais mol est principalement compose de muqueuse et ne comporte pas ou ne comporte guere de muscle. La fissure est rarement evidente par un simple examen de la bouche et du pharynx. Quant au langage articule, il est frappe de divers troubles dont le nasonnement qui peut etre minimal ou severe et une articulation vicieuse. Chez de nombreux malades, dont la fissure palatine sous-muqueuse est simplement

From the Department of Paediatrics (Divisions of Medical Genetics and Audiology & Speech Sciences) and the Department of Surgery (Division of Plastic Surgery), University of British Columbia, and the Cleft Palate Programme, Children's Hospital Diagnostic Centre, Vancouver, B.C. This work was supported in part by MRC Grant no. MA-3415. Reprint requests to: Dr. R. B. Lowry, 715 West 12th Ave., Vancouver 9, B.C. FIG. 1.The symptoms and signs of submucous cleft palate. CMA JOURNAL/NOVEMBER 17, 1973/VOL. 109 995 the nasal cavity from the oral cavity (i.e. 15 to 18 months) should be based referred for evaluation to a unit spe- in the production of certain speech on a diagnosis of palatopharyngeal in- cifically dealing with the cleft palate, sounds and in swallowing. The degree competence, not merely on a diagnosis or, if this is unavailable, to a plastic of speech defect will depend on the of submucous cleft palate. A recent surgeon and speech pathologist, since extent of the submucous cleft. If it is study4 suggests that there is no correla- personnel from these disciplines prob- extensive, then the child's speech may tion between the extent of the mus- ably have the most experience in this be no different from that in a case of cular cleft and speech proficiency. area. It is well known that patients overt cleft palate. Less severe sub- However, a short palate and/ or one with cleft palate have an increased mucous clefts may cause minimal nasa- with reduced mobility often results in frequency of otitis media, and those lity partly because of the degree of speech defects. The failure of pala- with submucous cleft palate are no ex- movement of the soft palate and partly topharyngeal closure can often be ception.7 However, in the interests of because the adenoid pad may act as a demonstrated by radiologic methods good speech, and since the indications compensatory factor by reducing the (Fig. 2). for tonsillectomy and adenoidectomy distance that must be bridged for pala- If there is a distinct probability that are seldom urgent, it is preferable to topharyngeal closure. Children with hypernasal speech will result from conserve the adenoid pad and treat the minimal nasality due to a submucous palatopharyngeal incompetence, then otitis media by other methods, thereby cleft palate can develop severe and surgery is indicated. The procedure of preventing the speech disorder that may often permanent nasality following choice is the Wardill V-Y repair as re- follow operation. adenoidectomy.2 The mucosa covering commended by Calnan5 in 1954. In an the cleft acts as a drumhead which older child with established hypernasal transmits vibrations from mouth to speech, particularly if an adenoidec- References nose,3 and nasality is particularly no- tomy has been performed, this may not 1. LOWRY RB: Sex-linked cleft palate in a ticeable on high vowels such as ee/i/ be sufficient to produce adequate pala- British Columbia Indian family. Pediatrics 46: 123, 1970 and oo/u/. There may be additional topharyngeal closure during speech and 2. GRABB WC, et al: Cleft Lip and Palate. Boston; Little, Brown; 1971 speech errors such as (a) weak produc- serious consideration should be given to 3. PERKINS WH: Speech Pathology. St. Louis, tion of plosive sounds (p and b); (b) combining the palatal surgery with a CV Mosby, 1971 4. WEATHERLEY-WHITE RC, SAKURA CY JR. nasal grimacing (alar pinching) during Hynes pharyngoplasty.' BRENNER LD, et al: Submucous cleft palate. Its incidence, natural history, and indications the production of most consonants, and It is vital therefore to approach each for treatment. Plast Reconstr Surg 49: 297, (c) presence of non-speech sounds such patient with submucous cleft palate 1972 5. CALNAN J: Submucous cleft palate. Br J Plast as glottal stops (tiny cough-like sounds) individually with respect to the need Surg 5: 264, 1954 6. HYNES W: Observations on pharyngoplasty. and pharyngeal fricatives. for operation. A muscular cleft alone Br J Plast Surg 20: 244, 1967 does not demand surgical intervention; 7. BERGsTROM L, HEMEN WAY WG: Otologic problems in submucous cleft palate. South Surgical aspects one must look for further signs of Med J 64: 1172, 1971 palatopharyngeal incompetence such as Is operation indicated when a sub- a short palate and/or one with inade- mucous cleft palate is diagnosed in quate mobility. infancy? Advocates of early surgical management overlook the fact that Comment some children with a submucous cleft palate do not have palatopharyngeal Many pediatricians, otologists and incompetence and do not invariably family doctors are not aware of this develop speech defects.4 Conversely, entity. All physicians who perform ton- it is well recognized that surgery per- sillectomy and adenoidectomy should formed after faulty speech is established be aware of the signs and symptoms is often not rewarding. which may suggest the diagnosis, and if The decision to operate in infancy it is suspected the patient should be

FIG. 2-Lateral radiographs of posterior pharynx showing (left) soft palate at rest and (right) on phonation. a =adenoid pad, b = velopharyngeal space, c = soft palate (velum), d-vertebral body. Patient has submucous cleft palate. Note failure of closure of the velopharyngeal space during phonation. CMA JOURNAL/NOVEMBER 17, 1973/VOL. 109 997