Oropharyngeal teratoma, oral duplication, cervical diplomyelia and anencephaly in a 22-week fetus: A review of the craniofacial teratoma syndrome.

BACKGROUND: Oropharyngeal teratoma may occur by itself or together with other craniofacial malformations, most commonly cleft palate. Oropharyngeal teratoma may be also seen in association with frontonasal dysplasia and/or various degrees of craniofacial duplication. The nosology of these sporadic disorders is poorly defined. CASE AND REVIEW: We report on a 22-week fetus with a protruding nasopharyngeal teratoma, partial oral duplication, anencephaly, multiple costo-vertebral segmentation defects, and cervical diplomyelia. A review of the literature identified 48 patients published from 1931 to 2013 with co-existing clefting and duplication anomalies of the cephalic pole. Thoracic and abdominal midline anomalies were reported 13 times. CONCLUSION: The term "craniofacial teratoma syndrome" is introduced to define this phenotype as a recognizable developmental field defect of the cephalic pole. Developmental pathogenesis is discussed with a focus on pleiotropy and stereotaxis. The observation of midline findings suggestive of holoprosencephaly in a few previously reported cases suggests a role for the sonic hedgehog signaling pathway in this malformation pattern.

Quantitative analysis of an inhibitory gradient field in the hydrozoan stolon.

An inhibitory field which emanates from the mobile tips of elongating stolons of colonial hydroids has been identified and analyzed. It extends proximally with decreasing intensity for about 400-700 µm and ensures that branching sites occur at appropriate distances along the stolon. The local strengths of inhibition within the field have been measured with a new method which permits high temporal and spatial resolution. Kinetic studies reveal three characteristics. First, inhibition decays rather rapidly after removal of its source. The half life is about 30 min. Second, loss of inhibition immediately triggers initiation of future tip formation. Third, restoration and spreading of inhibition are slow processes which take 8-24 h to recover 90% of the original inhibitory levels.