Indiana University-Purdue University Indianapolis (IUPUI) / Frontonasal malformation, FNM, was first described by Hoppe
in 1859. FNM is an anomaly that is characterized by ocular hypertelorism, broad nasal root, lack of a nasal tip, V-shaped hair,
prolongation onto the forehead (widow's peak), anterior cranium
bifidum occultum, median facial cleft affecting the nose, upper lip,
and/or palate, and uni- or bilateral clefting of the ala nasi. The
anomalies noted in FNM may be explained as a single malformation.
If the nasal capsule fails to develop properly, the primitive brain
vesicle fills the space normally occupied by the capsule, thus
producing anterior cranium bifidum occultum, an arrest in the
positioning of the eyes, and a lack of formation of the nasal tip. The
condition presents clinically with variable expressions as sporadic
cases and infrequently in familial cases. The present study is the first
attempting to quantify and characterize FNM via anatomic
radiographic measurements. The lateral (LA) and posterior-anterior
(PA) cephalometric radiographs of twenty-four individuals, both
sporadic and familial, with FNM were analyzed for comparison of
linear and angular measurements with previously published data of a
"normal," i.e. unaffected, population standard. Usual and customary
cephalometric points were identified and located, then digitized into
the computer. Twenty-nine measurements included the previously
diagnosed anomalous features of hypertelorism, medial nasal cavity,
and palatal shelves, as well as other facial features. The radiographs
of individuals with FNM have anatomic features that are unusual and
distinct to the specific malformation. The data from this research
suggest that patients with FNM, regardless of a genetic or sporadic
predisposition, have a midface deficiency in height and depth, an
increased interorbital width with possible increased orbital socket
width, and a longer zygomatic buttress. Also, the familial cases tend
to have a flatter cranial base than the sporadic cases. Furthermore,
the familial patients might be a different type of FNM since this
subgroup shows narrower zygomatic widths. The patients with
surgical procedures demonstrated improvement different from the
growth of those patients who did not have surgery. The hypothesis
that the facies of a patient with frontonasal malformation is different
from the "normal" control population is supported by this research.
The differences between the familial and sporadic patients tend to
support the general theory that genetic predisposition is less severe
than FNM that occurs randomly.
| Identifer | oai:union.ndltd.org:IUPUI/oai:scholarworks.iupui.edu:1805/4368 |
| Date | January 1994 |
| Creators | Hiester, John David, 1964- |
| Contributors | Bixler, David, Hathaway, Ronald R., Sadove, A. Michael, Shanks, James C., Avery, David R. |
| Source Sets | Indiana University-Purdue University Indianapolis |
| Language | en_US |
| Detected Language | English |
| Type | Thesis |
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