Binder phenotype: clinical and etiological heterogeneity of the so-called Binder maxillonasal dysplasia in prenatally diagnosed cases, and review of the literature.

OBJECTIVE: Prenatal Binder profile is a well known clinical phenotype, defined by a flat profile without nasal eminence, contrasting with nasal bones of normal length. Binder profile results of a hypoplasia of the nasal pyramid (sometimes referred to as maxillonasal dysplasia). We report 8 fetuses prenatally diagnosed as Binder phenotype, and discuss their postnatal diagnoses. METHODS: Ultrasonographic detailed measurements in 2D and 3D were done on the 8 fetuses with Binder profile, and were compared with postnatal phenotype. RESULTS: All fetuses have an association of verticalized nasal bones, abnormal convexity of the maxilla, and some degree of chondrodysplasia punctata. The final diagnoses included fetal warfarin syndrome (one patient), infantile sialic acid storage (one patient), probable Keutel syndrome (one patient), and five unclassifiable types of chondrodysplasia punctata. CONCLUSION: This series demonstrates the heterogeneity of prenatally diagnosed Binder phenotype, and the presence of chondrodysplasia punctata in all cases. An anomaly of vitamin K metabolism, possibly due to environmental factors, is suspected in these mild chondrodysplasia punctata. We recommend considering early prophylactic vitamin K supplementation in every suspected acquired vitamin K deficiency including incoercible vomiting of the pregnancy.

Arteriovenous malformations of the dental arcades. The place of endovascular therapy: results in 12 cases are presented.

Twelve arteriovenous malformations of the dental arcades (AVMDAs) (seven mandibular and five maxillary) were seen in our institution between 1977 and 1997. All these lesions consisted of true arteriovenous shunts (of either nidus or fistulous type) involving the bone, with or without soft tissue extension. Haemorrhage was present in eight patients (67%); either torrential, necessitating emergency embolization, or recurrent and progressive. Teeth instability was detected in all patients and was the origin of the bleeding. All lesions were embolized. Lesions in nine patients were embolized with Polyvinyl Alcohol Particles (PVA): this helped to stabilize the situation but could not avoid recurrences in all patients, necessitating complementary embolizations and or surgery. The use of acrylic glue (N-Butyl-Cyano-Acrylate [NBCA] Histoacryl) as the embolic agent has changed the results obtained tremendously. Eight patients have been treated with NBCA (five as complementary therapy to PVA during later sessions and three at the first attempt); injection either via the transarterial route or direct transcutaneous puncture (four patients) achieved a cure in four of these lesions (34%) with stability at long-term follow-up of all the other AVMs. Embolization with glue represents the therapy of choice in these sometimes life-threatening lesions, achieving a cure if directed towards the osseous venous lakes. Surgery, often leading to facial mutilation and necessitating massive reconstruction should be avoided nowadays, at least as the initial therapy.