The oral manifestations of cyclopia

Sedano, Heddie O.; Gorlin, Robert J.

doi:10.1016/0030-4220(63)90321-9

Cited by 30 publications

(10 citation statements)

References 48 publications

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“…Finally it is worthy of note that the mesenchymal component of the maxillary incisor tooth buds are derived from the median nasal processes. As would be expected, maxillary incisor formation fails in cyclopia, only with an atypical midline tooth which is sometimes present (Sedano and Gorlin, 1963;Latham, 1971;Gardner and Lim, 1971;Fleming et al, 1990). In this study the maxilla was definitely abnormal, showing abnormally narrow maxillary trapezoid with aberrant incisor tooth germs, while the mandible showed minimum hypoplasia.…”

Section: Discussionsupporting

confidence: 67%

Craniofacial Dysmorphia of Frontal Proboscis in Holoprosencephaly

Lee

Suh

1993

Congenital Anomalies

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We have examined eleven cases of holoprosencephaly with special emphasis on the frontal proboscis. Although the nostril appears blind ended grossly, the proboscis has a single slender midline nostril lined with squamous mucosa containing numerous sebaceous glands. It is actually connected to a poorly formed but definite nasal cavity microscopically. The inner portion of the nostril tunnel is lined by olfactory mucosa containing numerous subjacent secretary glands. Despite the absence of olfactory bulbs and tracts the cartilaginous nasal capsule is identified along with abnormal nasal cavity. However, the nasal capsule is hypoplastic, and has an aberrant nasal septum and no concha. In severe form of holoprosencephaly, i.e., cyclopia, the nasal capsule is noted above the level of the ethmoid plate, in which case the nasal cavity dose not communicate with nasopharynx because it is too distant from the hypoplastic maxilla. Therefore, choanal atresia is resulted. Although unique maxillary bone comprises the whole upper jaw, the failure of fusion between vomeronasal septum and palatal processes in holoprosencephaly results in abnormal maxillary bone which is characterized by a narrow palate without palatal arch form. Nevertheless, through serial microscopic sections we could find a space between the posterior maxilla and the sphenoid bone, which is composed of adipose tissue and is similar in shape to the nasal capsule. This space is thought to be made for the nasal capsule during early developmental period. The present study suggests that the frontal proboscis in holoprosencephaly is an undescended nose. In most cases it is connected to the aberrant nasal cavity which is located beneath the proboscis. The level of proboscis descends gradually toward normal nose position as the severity of holoprosencephaly decreased from cyclopia, ethmocephaly, cebocephaly, to premaxillary agenesis. Key words: frontal proboscis, holoprosencephaly, craniofacial dysmorphia

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Section: Discussionsupporting

confidence: 67%

Craniofacial Dysmorphia of Frontal Proboscis in Holoprosencephaly

Lee

Suh

1993

Congenital Anomalies

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“…The data indicate that the median tooth in cyclopia has arisen from the maxillary processes. Sedano and Gorlin [1963] expressed a similar opinion.The fusion of the inferomedial parts of the maxillary processes to form a median tooth is consistent with the fusion of the superomedial parts of the maxillary processes to form a median lacrimal fossa. Given the size of the fused median tooth one might suggest that the lateral and smaller part of the normal lateral incisor is a development of the maxillary process.…”

Section: Discussionsupporting

confidence: 54%

Upper Jaw in Human Cyclopia

McGrath

1989

Cells Tissues Organs

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In human cyclopia the upper jaw forms a solid bony mass between the median orbit and the oral cavity. The skeletal elements forming the upper jaw have been studied in serial sections through the median third of the head in 3 perinatal human specimens presenting with a median orbit and proboscis. One head was sectioned in the sagittal plane and 2 in the coronal plane. The upper jaw has also been studied in a dried cyclops skull and in a desiccated cyclops head in which the roof of the orbit had been removed. The data obtained demonstrate the particular contributions made by the lacrimal bones the maxillae and the palatine bones to the upper jaw in human cyclopia. The effects of the absence of the frontonasal process contribution and of the absence of the nasal cavity on the upper jaw in cyclopia are considered.

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“…Finally, the spectacular association of cyclopia and iniencephaly was noted by Sedano and Gorlin (1963; case 2) and by Warkany (1971).…”

Section: Central Nervous Systemmentioning

confidence: 78%

Holoprosencephaly: Clinical, anatomic, and molecular dimensions

Cohen

2006

Birth Defects Research

191

192

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Holoprosencephaly is addressed under the following headings: alobar, semilobar, and lobar holoprosencephaly; arrhinencephaly; agenesis of the corpus callosum; pituitary abnormalities; hindbrain abnormalities; syntelencephaly; aprosencephaly/atelencephaly; neural tube defects; facial anomalies; median cleft lip; minor facial anomalies; single maxillary central incisor; holoprosencephaly-like phenotype; epidemiology; genetic causes of holoprosencephaly; teratogenic causes of holoprosencephaly; SHH mutations; ZIC2 mutations; SIX3 mutations; TGIF mutations; PTCH mutations; GLI2 mutations; FAST1 mutations; TDGF1 mutations; and DHCR7 mutations.

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The oral manifestations of cyclopia

Cited by 30 publications

References 48 publications

Craniofacial Dysmorphia of Frontal Proboscis in Holoprosencephaly

Craniofacial Dysmorphia of Frontal Proboscis in Holoprosencephaly

Upper Jaw in Human Cyclopia

Holoprosencephaly: Clinical, anatomic, and molecular dimensions

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