Use of an Orthodontic and Otolaryngological Approach in an Infant with Holoprosencephaly.

Holoprosencephaly is a complex human brain malformation resulting from incomplete cleavage of the prosencephalon into both hemispheres. Congenital nasal pyriform aperture stenosis (CNPAS) is sometimes found in patients with mild forms of holoprosencephaly. Surgical treatment is required. Low-invasive surgical approaches involve balloon dilation of the pyriform opening. We present the case of an 8-day-old girl diagnosed with holoprosencephaly, CNPAS, and the presence of a solitary median maxillary central incisor. Once examined by neonatologist, geneticist, pneumologist, otolaryngologist, and pediatric dentist, a combined otolaryngological-orthodontic approach was used. The obstruction of the right nasal cavity was treated by widening the nasal cavities and stabilizing them with a balloon dilation technique. After surgery, the respiratory space was increased by applying a neonatal palatal expander plate (NPEP) considering the palatal deformity: ogival shaped, anterior vertex growth direction, reduction of transverse diameters. The NPEP promoted distraction of the median palatine suture and assisted the nasal dilation. Therefore, after the insertion of NPEP, the physiological sucking-swallowing mechanism was activated. In infants with CNPAS, NPEP can be useful to ensure the safe stability of nasal dilation. A multidisciplinary approach is fundamental. In our experience, the close collaboration between an otolaryngologist and orthodontist is essential for the management of the patient with CNPAS.

Effects of Rapid Palatal Expansion on the Upper Airway Space in Children with Obstructive Sleep Apnea (OSA): A Case-Control Study.

Obstructive Sleep Apnea (OSA) in children needs a multidisciplinary approach. Even if the first-line treatment of pediatric OSA is adenotonsillectomy, nowadays rapid palatal expansion (RPE) is considered a valid additional treatment. The aim of this study is to evaluate cephalometric changes in upper airways dimensions after rapid palatal expansion (RPE) in children suffering from Obstructive Sleep Apnea (OSA). A total of 37 children (range age 4-10 years) with diagnosis of OSA referred to Dentistry Unit of Bambino Gesù Children's Research Hospital IRCCS (Rome, Italy) were included in this pre-post study and underwent lateral radiographs at the start (T0) and at the end (T1) of a RPE treatment. Inclusion criteria were: diagnosis of OSA confirmed by cardiorespiratory polygraphy (AHI > 1) or pulse oximetry (McGill = >2), skeletal maxillary contraction evaluated by presence of posterior crossbite. A control group of 39 untreated patients (range age 4-11 years), in good general health, was set up. A paired T-test was used to investigate the statistical differences between T0 and T1 values in both groups. The results showed a statistically significant increase of nasopharyngeal width in the treated group after RPE treatment. Moreover, the angle that identifies mandibular divergence compared to palatal plane (PP-MP°) was significantly reduced. In the control group, no statistically significant differences were observed. The present study showed that RPE treatment determines a significant sagittal space increase in the upper airways space and a counterclockwise mandibular growth in children with OSA compared to a control group. These results suggest that a widening of the nasal cavities induced by RPE may support a return to physiological nasal breathing and promote a counterclockwise mandibular growth in children. This evidence confirms the crucial role of the orthodontist in the management of OSA in pediatric patients.