Familial syndrome with abnormal molar roots and other ectodermal and mesodermal anomalies.
Pyramidal Molar Roots with Juvenile Glaucoma and Unusual Upper Lip; Juvenile Glaucoma with Unusual Upper Lip and Dental Roots Syndrome.
Unknown, but extremely rare.
The combination of dental, cutaneous, ocular, and bone abnormalities has been first described by Ackerman et al in 1973 in 20 members of a non-consanguineous family of English-German ancestry. Affected patients showed various abnormalities of the molar roots (pyramidal, mesotaurodont, hypertaurodont, or fused roots with often a single root canal only). Other, although widely inconsistent, ectodermal and mesodermal anomalies included unusual shape of the upper lip lacking cupid’s bow with thickening and widening of the philtrum, entropium of the lower eyelids, juvenile glaucoma, clinodactyly, syndactyly (hand or foot), scant body hair with only vellus hair in the beard area, and horizontal ridging of fingernails with distal onychoschizia.
Precautions before anesthesia
Assess dental anomalies with regards to direct laryngoscopy. Inquire about glaucoma and efficacy of treatment. Otherwise do routine preoperative assessment.
Although some of the adult patients in the original description had a fair number of missing molars, it was not explicitly noted that the abnormal roots are weaker than the normal ones. Nevertheless, it seems prudent to assume that these molars are at higher risk of injury during laryngoscopy or stage II of the anesthetic (clinching of the teeth). Hence, a soft bite-block may be beneficial. Succinylcholine has the theoretical potential to increase intraocular pressure (IOP) and its benefits should therefore be weighed against the risk of further increasing IOP in the presence of glaucoma. Ample lubrication of the eyes during anesthesia is recommended.
Other conditions to be considered
☞Lacrimo-Auriculo-Dento-Digital Syndrome (LADD Syndrome; Levy Hollister Syndrome; Limb Malformations-Dento-Digital Syndrome): Inherited syndrome with eye, ear, teeth, and hand abnormalities and a potential for cardiac defects.
☞Oculodentodigital Syndrome (Oculo-Dento-Osseous Dysplasia; ODOD Syndrome): Form of bone dysplasia characterized by microphthalmia, microsomia, large jaw, and hypoplasia of the dental enamel. Other possible features include syndactyly, clinodactyly, and glaucoma.
A: Taurodont, pyramidal and fused molar roots associated with other anomalies in a kindred. Am J Phys Anthropol