Crouzon syndrome is one of the most common craniosynostosis facial syndromes caused by a mutation in the fibroblast growth factor receptor 2 (FGFR2) gene. Less commonly, there is a mutation of the FGFR3 gene which results in Crouzon syndrome syndrome with acanthosis nigricans. It involves the premature fusion of sutures of the cranial vault, base, orbital and maxillary region. The clinical presentation of this congenital deformity depends on the pattern and timing of sutural fusion. The present report describes the features and management of this syndrome in an 18-year-old woman. The patient presented with a hypoplastic maxilla, deficient midface, exorbitism due to shallow orbits, severe crowding and bilateral crossbite. A multidisciplinary approach involving orthodontics and surgical intervention with distraction osteogenesis brought about marked improvement in the facial profile, occlusion and upper airway. The aesthetics and function were greatly enhanced, and the results were found to be stable at the end of three years.

Objective: To analyze the effects of presurgical nasoalveolar molding (PNAM) in newborns with cleft lip and palate and evaluate its postsurgical stability at 1-year follow-up using a nasal stent. Design: Prospective clinical trial. Setting: Department of Orthodontics, Maulana Azad Institute of Dental Sciences, Delhi. Patients: Patients with cleft lip and palate of 0 to 6 months. Sample size 25 recruited, 16 satisfactorily completed the procedure. Interventions: Presurgical nasoalveolar molding done as indicated in all the patients. Postsurgical external nasal stents were given for 1 year to maintain the nasal correction. Main Outcome Measures: Changes in cleft defect and maxillary arch were recorded pre- and post-PNAM. Changes in nasal morphological parameters assessed pre- and post-PNAM and 1 year after surgical repair following the use of nasal stent. Results: There was a significant reduction in cleft defect assessed both intraorally ( 4.16 mm) and extra orally ( 4.42 mm) at the end of PNAM therapy (average treatment time 4 months). The nasal morphology improved with an increase in columella height (1.5 mm) and reduction in columella width. Bialar width reduced (2.90 mm) with an increase in nostril height ( 2.10 mm). Better symmetry was achieved in all cases as the nostril height and width difference changed significantly pre- and post-nasoalveolar molding. The percentage increase in columella height was greater for infants less than 6 weeks. Conclusions: Presurgical nasoalveolar molding considerably reduces the cleft gap and improves arch form making surgical union easier along with improved nasal morphology which can be maintained at 1-year postsurgery by use of a postsurgical external nasal stent.

Background:The setback of having a child with cleft lip and palate deeply affects the parents psychology. The Pre Surgical Infant Orthopaedics(PSIO) is a promising procedure for improvement in facial morphology prior to primary lip repair. Objective: The current study examines the perception of effects of PSIO procedures on the facial appearance of newborns with cleft lip/palate by their mothers and finds its correlation if any, with the change in psychosocial status of the mothers. Study design: The mothers of 50 infants(0-6 months) born with cleft lip/palate rated the nasal morphology, extent of cleft defect and overall facial morphology on a Likert's scale before and after PSIO procedure. This was correlated with changes in mother's depression, anxiety and stress levels by using the validated Hindi-version of Depression Anxiety and Stress scale(DASS-42) index before and after PSIO. Results: The mothers reported a significant improvement in all the morphological parameters for their infants with PSIO which correlated well with significant reduction in the DASS scores from 22.54(severe) to 7.10(normal) for depression, 20.64(extremely severe) to 6.46(normal) for anxiety and 24.7(severe) to 8.4(normal) for stress. Conclusions: The changes in facial morphology by PSIO procedures are well perceived by mothers and significantly improves their depression, anxiety and stress levels.

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Restoration of missing oral tissues in patients with cleft lip and palate is the biggest challenge in their rehabilitation. In this report, we described the postdistraction rehabilitation and 1-year follow-up of the regenerated bone by implant-supported prosthesis and orthodontic tooth movement. A novel distraction appliance regenerated natural bone which is compatible for both implant placement and orthodontic tooth movement, thereby restoring adequate function and esthetics.