Van der Woude syndrome: Management in the mixed dentition
A female patient aged 6 years and 7 months, reported to our department with the chief complaint of malaligned upper and lower front teeth. The patient did not give any history of medical conditions. Patient's parents were extremely concerned about the patient's esthetics and speech defects. Lip repair was done at the age of 3 months. The extra-oral examination revealed a surgical scar on the upper lip, bilateral symmetrical lower lip pits, obtuse nasolabial angle and a concave profile due to recessive upper lip and everted lower lip [Figures and ].\nA bilateral cleft of the alveolus was observed intra-orally with an overhanging mobile premaxilla. An oronasal fistula was present in the cleft on the right side. Posterior cross-bite on both the sides confirmed a collapsed maxillary arch. Mild crowding with retroclination of the anteriors was noticed in the lower arch. The patient was in mixed dentition stage with permanent central incisors still in erupting stage [].\nOrthpantomograph [] showed congenitally missing upper lateral incisors. Also, lower second premolar tooth buds were missing.\nThe treatment was carried out in two phases.\nPhase I of the treatment consisted of expansion of the maxillary arch to correct the posterior cross-bite []. Expansion was continued for 6 months until the posterior cross-bite was overcorrected and the quad helix was retained in place. Total expansion achieved was approximately of about 5 mm. Maxillary central incisors and canines were bonded and 0.016 niti arch-wire was ligated to prevent an anterior cross-bite from developing []. A posterior bite block was given in the lower arch to raise the bite.\nPhase II of the treatment involved secondary alveolar bone grafting to provide adequate bone support for the eruption of canines. At the cleft site, mucoperiosteal flap was elevated and nasal floor was exposed. A pouch was created for receiving the graft. For the bone graft, iliac crest was exposed and cancellous bone was harvested. The bone graft was then placed in the pouch and the flap edges were approximated and sutures placed. The oronasal fistula on the right side of the cleft was completely obliterated by the graft.\nNo tooth movement was initiated for a period of 6 months post-surgery. On reviewing the case after 6 months, it was decided that maxillary protraction would not be required as the profile had become sufficiently straight. However, to maintain the arch width and to facilitate unhindered growth of maxilla till the permanent canines erupted, functional regulator-3 (FR-3) was delivered [Figures and ].\nIt was observed that the premaxilla had immobilized completely and the profile had improved esthetically to a considerable extent [Figures and ]. The patient was followed-up regularly for a period of 6 months after which she had to discontinue the treatment at our institution as her parents had migrated.