The influence of three different primary treatment protocols on 5-year-old maxillary growth in patients with complete unilateral cleft lip and palate.

The study evaluated the effects of three different primary treatment protocols on maxillary growth in patients aged 5 years with complete unilateral cleft lip and palate (UCLP). The secondary objective was to assess the influence of initial cleft severity, family history of class III, and status of permanent lateral incisor on maxillary growth. In total, 54 patients with non-syndromic complete UCLP were included and grouped as follows: group An underwent lip adhesion, cheilorhinoplasty associated with tibial periosteal graft for hard palate repair, and finally veloplasty; group B underwent lip adhesion, then cheilorhinoplasty with intravelar veloplasty, and finally a hard-palate repair; group C underwent cheilorhinoplasty with intravelar veloplasty and then a hard-palate repair. Five-year maxillary growth was assessed on dental models, both clinically and digitally. No difference was found with GOSLON-Yardstick scoring. Five-year measurements showed that group C tended to have the best maxillary arch morphology (p = 0.012). Initial cleft severity did not impact maxillary growth, but status of permanent lateral incisor and family history of class III did (p = 0.019 and p = 0.004, respectively). In patients aged 5 years, the two-stage approach appeared to be the least detrimental to growth development. Predictive factors for growth retardation included the absence of lateral incisor and a family history of class III.

"Oris Duplicatio"-Duplication of the Oral Cavity a Rare Malformation in a 10-Month-Old Child: State of the Science and Management.

The authors report the case of a 10-month-old child with total oral duplication. The authors review this pathology and the diagnostic and treatment modality through the few cases described in scientific history. The discussed etiopathogeny of this rare malformation is detailed in this work.

Aesthetic and psychosocial impact of dentofacial appearance after primary rhinoplasty for cleft lip and palate.

The primary aim of this study was to demonstrate whether primary rhinoplasty shows aesthetic and psychosocial advantages for children with a complete unilateral cleft lip and palate. The second aim was to determine the satisfaction levels concerning the dentofacial appearance. Group A corresponded to patients from a center specialised in primary cheilo-rhinoplasty with 20 years' experience and Group B to patients who did not benefit from primary rhinoplasty. Children and their parents filled in a custom-designed satisfaction questionnaire on dentofacial appearance and its psychosocial impact. The variables studied were the main criterion (the nose) and secondary criteria (the upper lip, the smile, the profile and the face as a whole). 56 families consented to be involved in the study. The children did not rate statistically differently their social relationships if they had primary rhinoplasty or not. Parents however expressed very different views. They considered the nasal appearance of the children who had primary rhinoplasty as statistically more attractive and evaluated their psychosocial experience as significantly better. For the other parts of the face, in both groups, satisfaction levels of dentofacial appearance and psychosocial comfort were good (scores above 80/100). Yet, 44% of the families would go for further interventions, especially concerning the nose (13% of whom were in Group A and 42% in Group B). Within the limitations of this study, primary rhinoplasty seems to improve the patient's well-being and social life and, therefore, should be considered whenever appropriate.

Microdeletion del(22)(q12.1) excluding the MN1 gene in a patient with craniofacial anomalies.

Several studies have recently reported that 22q12.1 deletions encompassing the MN1 gene are associated with craniofacial anomalies. These observations are consistent with the hypothesis that MN1 haploinsufficiency may be solely responsible for craniofacial anomalies and/or cleft palate. We report here the case of a 4-year-old boy presenting with global developmental delay and craniofacial anomalies including severe maxillary protrusion and retromicrognathia. Array-CGH detected a 2.4 Mb de novo deletion of chromosome 22q12.1 which did not encompass the MN1 gene thought to be the main pathological candidate in 22q12.1 deletions. This observation, combined with data from other patients from the Database of Chromosomal Imbalance and Phenotype in Humans Using Ensemble Resources (DECIPHER), suggests that other gene(s) in the 22q12.1 region are likely involved in craniofacial anomalies and/or may contribute to the phenotypic variability observed in patients with MN1 deletion.

Evaluation of 5-year-old children with complete cleft lip and palate: Multicenter study. Part 2: Functional results.

BACKGROUND AND PURPOSE: Cleft surgery is marked by all the controversies and the multiplication of protocols, as it has been shown by the Eurocleft study. The objective of this pilot study is to start a comparison and analyzing procedure between primary surgical protocols in French centers. METHODS: Four French centers with different primary surgical protocols for cleft lip and palate repair, have accepted to be involved in this retrospective study. In each center, 20 consecutive patients with complete cleft lip and palate (10 UCLP, 10 BCLP per center), non syndromic, have been evaluated at a mean age of 5 [range, 4-6]. In this second part, maxillary growth and palatine morphology were assessed on clinical examination and on dental casts (Goslon score). Speech was also evaluated clinically (Borel-maisonny classification) and by Aerophonoscope. RESULTS: Veau-Wardill-Killner palatoplasty involves a higher rate of transversal maxillary deficiency and retromaxillary. The fistula rate is statistically lower with tibial periosteum graft hard palate closure but this technique seems to give retromaxillary. Malek and Talmant two-stage-palatoplasty techniques reach Goslon scores of 1 or 2. Considering speech, Sommerlad intravelar veloplasty got higher outcomes. CONCLUSIONS: Primary results. Extension to other centers required. The two-stage palatoplasty, including a Sommerlad intravelar veloplasty seems to have the less negative impact on maxillary growth, and to give good speech outcomes. LEVEL OF EVIDENCE: Therapeutic study. Level III/retrospective multicenter comparative study.

Evaluation of 5-year-old children with complete cleft lip and palate: Multicenter study. Part 1: Lip and nose aesthetic results.

BACKGROUND AND PURPOSE: Cleft surgery is marked by all the controversies and the multiplication of protocols, as it has been shown by the Eurocleft study. The objective of this pilot study is to start a comparison and analyzing procedure between primary surgical protocols in French centers. METHODS: Four French centers with different primary surgical protocols for cleft lip and palate repair, have accepted to be involved in this retrospective study. In each center, 20 consecutive patients with complete cleft lip and palate (10 UCLP and 10 BCLP per center), non syndromic, have been evaluated at a mean age of 5 [4,6]. In this first part, the aesthetic results of nose and lip repair were assessed based on the scale established by Mortier et al. (1997). RESULTS: Considering nose outcome, primary cleft repair surgery including a nasal dissection gives a statistically significant benefit in terms of septum deviation. Considering lip result, muscular dehiscence rate is significantly higher in BCLP patients with a two-stage lip closure. The centers using Millard one-stage lip closure do not have uniform results. For UCLP patients, the quality of scar is not statistically different between Skoog and Millard techniques. CONCLUSIONS: Primary results based on a simple, reproducible evaluation protocol. Extension to other centers required. LEVEL OF EVIDENCE: Therapeutic study. Level III/retrospective multicenter comparative study.