Background and Clinical Significance: Binder syndrome or maxillonasal dysplasia is a rare developmental disorder affecting the anterior maxilla and nasal complex, characterized by midfacial hypoplasia, a flattened nasal bridge, and increased nasofrontal angle. Case Presentation: We present a case series of seven fetuses diagnosed with Binder phenotype through targeted ultrasound examination at our prenatal diagnosis center during the SARS-CoV-2 pandemic, between September 2021 and July 2023, including the first case described in the literature before 14 weeks. The median gestational age at diagnosis was 21 weeks. Ultrasound features included flattened fetal facial profile, increased nasofrontal angle (>143°), verticalized nasal bones and widened maxillary alveolar arch. Five cases presented as isolated anomalies, while two showed associated findings including growth restriction and polyhydramnios. Invasive prenatal diagnosis was offered in all cases, with three patients consenting to amniocentesis, all revealing normal karyotype and chromosomal microarray. Pregnancy outcomes varied: three patients opted for termination of pregnancy, one case resulted in intrauterine fetal demise, one delivered prematurely with confirmed postnatal phenotype, and two continued pregnancy with normal delivery. Conclusions: This relatively high case frequency within a short timeframe suggests that Binder syndrome, while rare, may not be as uncommon as previously reported. Accurate ultrasound diagnosis combined with comprehensive genetic counseling enables appropriate pregnancy management and optimal perinatal outcomes.

Our study aimed to evaluate a stepwise treatment of class III malocclusion accompanied with flat nasal deformity, using orthodontics, orthognathic surgery, and rhinoplasty, as well as stability during long-term follow-up. In total, 27 patients with nasomaxillary hypoplasia and skeletal class III malocclusion were enrolled in this study. All patients had accepted orthodontic and orthognathic surgery, followed by rhinoplasty with costal cartilage as the second surgical procedure. Clinical results were evaluated by radiography, medical photography, questionnaire, and cephalometric analysis. All patients were satisfied with the surgical results and no serious complications occurred. During the follow-up, the patients showed well-corrected midface contour and nasal projection, and stable occlusion. The costal cartilage grafts were well fixed, without obvious absorption deformation. Lateral cephalometric analysis and overlay results showed that the postoperative point A had advanced approximately 5.20 ± 1.43 mm and the SNA angle had increased by approximately 5.59 ± 2.86°. Soft-tissue measurements showed a 14.22 ± 6.56° decrease in the facial lobe, while the nasolabial angle had increased by 16.83 ± 6.65° postoperatively. The results suggested that stepwise orthodontic-orthognathic surgery and rhinoplasty produce a predictable and stable result in long-term follow-up.

Binder phenotype (BP) is a term used to define a form of maxillo-nasal dysplasia defined by the presence of flattened nose, verticalized nasal bones, and retrusion of the maxilla (BP triad). This facial anomaly can be associated with other anomalies in both genetic and acquired conditions as well as in isolation. This systematic review aimed to summarize the antenatal findings, maternal and obstetrics characteristics as well as the obstetrics and neonatal outcomes of all cases of BP diagnosed in utero. According to the PRISMA statement, we conducted a systematic review of the literatures to identify all the Binder phenotype cases diagnosed antenatally. We extracted and summarized obstetrics and neonatal characteristics of each study, including our case report. We identified a total of 47 cases of BP. The median gestational age at diagnosis was 23 weeks. BP was associated with other skeletal or non-skeletal anomalies in all cases apart from six confirmed to be isolated. Respiratory distress syndrome was observed in 12 neonates and in 18 cases BP was one of the clinical findings of chondrodysplasia punctata X-linked. BP is often associated with other fetal defects; however, when isolated, BP seems to have good obstetrics and neonatal outcomes. In fetuses with apparent isolated BP at midtrimester, genetic testing with fluorescence in situ hybridization (FISH) for ARSE deletion can be offered for CDPX1 diagnosis while exome sequencing may be more informative in cases where a syndromic condition is suspected. Lastly, BP per se is associated with a higher risk of respiratory distress syndrome and feeding difficulties.

Binder syndrome (maxillonasal dysplasia) is an uncommon congenital craniofacial condition. It is marked by distinctive facial characteristics including a flat, vertically oriented nose, maxillary underdevelopment, malocclusion, and nasal bone irregularities. This case study introduces an inventive strategy for addressing congenitally absent anterior teeth in a patient diagnosed with Binder syndrome. Our treatment approach combined orthodontic interventions and prosthetic restorations to enhance both aesthetics and function. This report explores the diagnostic and therapeutic aspects of the case, underscoring the encountered challenges and the ultimately successful outcome. This approach provides valuable insights into managing dental anomalies linked to Binder syndrome, emphasizing the necessity of a multidisciplinary approach for comprehensive patient care. A suitable strategy for adult patients might be slow maxillary expansion. This case report is about a rare case of maxillonasal dysplasia managed with the esthetic replacement of anterior teeth.

Binder's syndrome is a rare congenital deformity characterized by midface hypoplasia, particularly around the nasomaxillary area. Genetic etiology or developmental failure caused by prenatal exposure to teratological agents has been considered. In this article, we present 3 related rhesus monkeys born with orofacial deformities similar to those found in infants with the Binder phenotype. For the first time, a primate biomodel for this condition is presented. The clinical description and association with management and environmental factors are discussed. These findings reinforce the knowledge about the relationship between possible vitamin K metabolism interference and Binder's syndrome.