Pediatric dentistry plays an important part in the orthodontic treatment of patients with rare orofacial diseases. Interactions between these two disciplines are numerous and particularly noteworthy in the following pathologies:

1. cleft lip and palate;

2. ectodermal dysplasia;

3. amelogenesis imperfecta;

4. dentinogenesis imperfecta.

After reviewing the main characteristics of these pathologies, we will highlight the fundamental role of pediatric dentistry in the early diagnosis, the prevention and the dental care throughout the orthodontic treatment. A close cooperation between the orthodontist and the pediatric dentist is a key factor to a successful treatment.

Parents want their children’s’ faces, the most visible part of their bodies, one that marks their identity throughout life, to be perfect. To satisfy this understandably urgent desire, a high quality of primary treatment for cleft lip and palate is essential and must satisfy a double objective: restore normal morphology and normal function. The functional, morphological, and esthetic prognoses depend on the character of the defect, whether it stands alone or is associated in a syndrome with other malformations. Important sequellae flow from the quality of the initial repair, as a consequence of the surgery and other therapies as well as from the deformity itself.

Before the year 2000, the Maxillo-facial and Plastic Surgery Service at the Armand Trousseau Hospital of the Pierre and Marie Curie Faculty of Medicine adhered to the protocol that Malek had described, making an initial repair of the soft palate at 3 months and then a cheiloplasty, with upper and lower triangles, and closure of the hard palate at 6 months. Since then we have adopted the more functional approach that Talmant described, integrating systematic nasal surgery and the type of lip surgery that Millard suggested without leaving any residual exposed bone after closure of the osseous cleft. We then perform gingivo-periosteal surgery with bone grafts on patients when they were between 4 and 6 years of age, after orthodontic therapy had been completed. This constitutes the last stage of primary treatment.

The therapeutic approach we have been using on our service, which has evolved of over the last 20 years, has come to define its principal objective as integration of extensive rehabilitation into the very first stages of our multi-disciplinary therapy so as to minimize the establishment of faulty functioning of phonation, lip competence, and ventilation while avoiding any intervention that would have a harmful impact on facial growth. This multi-disciplinary approach, which integrates surgical evaluation and protocol, is indispensable and fully justifies treatment of patients with cleft lip and palate at accredited centers.

Over the last 50 years, the dominant conception of the etiology of facial clefts postulated that they resulted from a mesodermal deficit that accounted for the primary hypoplasia and the unpredictable nature of subsequent oro-facial growth. Because of this notion, rigid dogmatic interdictions, with no allowance for nuanced treatment adjustments were issued banning sub-periosteal and sub-perichondrial dissections as well as the undertaking of any nasal surgery during the growth period. These prohibitions have had a grave braking effect on the development of treatment for cleft lip and palate patients.

With a new comprehension of the important role that the dynamics of fetal ventilation plays in the development of the airways and its influence on maxillary growth researchers no longer have any doubt that normal growth mechanisms operating under abnormal anatomic conditions created by the cleft are the actual causes of the malformations and the facial growth deficit suffered by children born with clefts. It now seems likely that the nasal septum plays only a passive role in growth, as Moss believed, rather than the active one that Scott as well as Delaire defined, calling it the driving force of maxillary development. They advocated re-inserting the lateral muscles on the anterior nasal spine. But practitioners who limit their functional approach to this procedure risk being deceived. In addition this focusing on muscles distracts us from what is essential, nasal ventilation, whose rehabilitation beginning with the first operation and preservation throughout treatment are absolutely necessary to ensure the normal unfolding of a cleft palate patient’s facial growth.

To achieve this goal surgeons must complete anatomic reconstruction with precisely timed, ambitious surgery carefully designed to control scar formation. Whether their procedure is unilateral or bilateral surgeons make this correction of the nasal malformation best if they operate when patients are six months old and close the lip defect at the same time. During this procedure surgeons close the soft palate defect using the Sommerlad technique taking advantage of the shrinking of the hard palate gap that closes enough to allow for simple, complete surgical elimination of the cleft in two layers without leaving dense scar tissue or denuded areas. By the time patients are 4 years old orthodontists have expanded the anterior maxilla to establish good canine function and surgeons can close the alveolar cleft with a gingivoperiosteoplasty and an iliac graft. Each procedure conserves the width of the piriform orifice, the nasal fossae and the anterior, incisor-canine sector of the arch. Nasal ventilation, as well as symmetrical, centered mastication, is required for normal facial growth.

It is common for international organizations to provide surgical corrective care to vulnerable populations in developing countries. However, a current worsening of the overall surgical burden of disease in developing countries reflects an increasing lack of sufficient numbers of trained healthcare personnel, and renders outside volunteer assistance more desirable and crucial than ever. Unfortunately, program evaluation and monitoring, including outcome indices and measures of effectiveness, is not measured commonly. In 2005, Operation Smile International implemented an electronic medical record system that helps monitor a number of critical indices during surgical missions that are essential for quality assurance reviews. This record system also provided an opportunity to retrospectively evaluate cases from previous missions. Review of data sets from >8,000 cases in 2005 and 2006 has provided crucial information regarding the priority of surgery, perioperative and operative complications, and surgical program development.

The most common procedure provided was unilateral cleft lip repair, followed closely by cleft palate. A majority of these interventions occurred for patients who were older than routinely provided for in the western world. The average child treated had an age:weight ratio at or below the [US] Centers for Disease Control and Prevention (CDC) 50th percentile, with a small percentage falling below the CDC 20th percentile. A majority of children had acceptable levels of hemoglobin, but the relative decreased age:weight ratio nonetheless can reflect mild malnutrition. Complications requiring medical intervention were seen in 1.2% of cases in 2005 and 1.0% in 2006. Thirty percent were reported as anesthesia complications, and 61% reported as surgical complications. One death was reported, but occurred after discharge outside the perioperative period. Complication rates are similar to rates reported in the US and UK and emphasizes the importance of standardization with uniform indices to compare quality performance and equity of care. This study offers an important example of the importance of collecting, analyzing, and reporting measures of effectiveness in all surgical settings.

The purpose of this study was to compare the prevalence of ear, nose and particularly voice problems in groups of children with cleft palate (CP) and with unilateral cleft lip, alveolus and palate (UCLP). On the basis of history, regular otorhinolaryngological examinations and hearing tests, the prevalence of different pathologies was assessed in 80 CP children (35 boys and 45 girls) and 73 UCLP children (47 boys and 26 girls). Ear pathology was reported in 53.8 per cent of CP children and in 58.9 per cent of UCLP children. Nasal breathing was impaired in 14 CP (17.5 per cent) and 36 UCLP (49.3 per cent) children. Dysphonia was detected in 12.5 per cent of CP and 12.3 per cent of UCLP children. In 9.2 per cent of all cleft children, functional voice disorder caused a hoarse voice. Two-thirds of cleft children with functional dysphonia had protracted hearing loss. Therefore, ENT specialists must take an active role early in the treatment of children with clefts.

This report highlights innovative ways of overcoming difficulties in delivering effective continuous positive airway pressure in an extremely preterm baby with bilateral cleft lip and cleft palate.

Tympanometry was analysed according to cleft type with respect to age in 239 cleft palate patients (57 with bilateral cleft lip and palate (BCLP)), 122 with unilateral cleft lip and palate (UCLP) and 60 with isolated cleft palate (ICP)). The frequency of type B tympanograms was 46.5 per cent in the BCLP group, 50.6 per cent in the UCLP group and 58.3 per cent in the ICP group. Type A tympanograms were more frequently observed in older patients (median age 11 years) in comparison to type B (median age five years; p<0.001) and type C (median age six years; p<0.001). The total sample showed an age-related decrease in the frequency of type B tympanograms (rs = −0.3942; p<0.001). The frequency of type A tympanograms increased significantly with age (rs = 0.4263; p<0.001), whereas type C was not correlated with age. In the UCLP group, the frequency of type B tympanograms decreased with age (rs = −0.4430; p<0.001), the decrease being faster than in the BCLP group (rs = −0.3186; p = 0.001) and the ICP group (rs = −0.3378; p<0.0001)). Type B tympanograms had the highest correlation with a hearing loss of 21–40 dB at mean hearing level at speech frequencies (MHLSF) (rs = 0.4574; p<0.001), a lower correlation with a hearing loss of 11–20 dB (rs = 0.2184; p = 0.02) and the lowest correlation with hearing loss above 40 dB. At the ages of one to three, the frequency of type B is higher in UCLP patients than in the BCLP and ICP groups, decreasing at seven to 12, increasing again at 13 to 15, and thereafter showing a continuous decrease. In the BCLP group, the frequency of type B increased significantly at the ages of four to six and then decreased continuously from seven to 18. In ICP patients, the changes in the frequency of type B with age are not significant until the age of 15; at the age of seven, it is higher than in the BCLP and ICP groups. The type C tympanogram is not typically found in cleft palate patients and its frequency is not correlated with age.

It can be presumed that each type of cleft lip and palate, due to its characteristic conditions in the epipharynx, will favour a different mode of pathophysiological development of middle-ear disease.

Otitis media with effusion is almost universal in children with cleft palate and can delay speech, language and educational development by causing hearing loss. Grommet insertion at the time of cleft palate repair is common. There is debate about whether the benefits of grommets outweigh the risk of complications. A postal questionnaire was used to investigate parental perceptions of middle-ear ventilation via grommet insertion in children attending the multidisciplinary cleft palate clinic. These children's case notes were reviewed. Many children had speech and language delay, but parents thought this improved after grommet insertion. Overall parents were pleased with the results. This confirms that grommets have an important part to play in the management of children with cleft palate.

Otitis media with effusion (OME) is almost universal in children born with a cleft palate. Early placement of a ventilation tube to alleviate hearing problems is common. A retrospective study has been carried out to assess whether the practice of tube placement only for definite clinical indications is successful in terms of subsequent hearing levels and speech and language development. This was assessed by a case note review, analysis of speech therapy data and by means of a special follow-up clinic.

There was no difference in speech development between those treated with tube insertion for OME and those untreated. Audiological thresholds were worse in the treated group. A similar number in each group required regular speech therapy. More abnormal otological findings were present at follow up in those who had tubes inserted, some of these were directly attributable to the presence of tubes.

A conservative management of OME in cleft palate children, with tube insertion for only definite clinical indications, is an appropriate management, and will lead to fewer otological complications of tube insertion.